Materiały źródłowe

• #1 What every nurse should know about hemophilia

  https://www.myamericannurse.com/what-every-nurse-should-know-about-hemophilia/

  What every nurse should know about hemophilia […] One diagnosis that requires particularly complex care is a bleeding disorder, such as hemophilia. […] Learning about bleeding disorders can vastly improve the care you provide and increase patients confidence in your care. […] Prolonged bleeding has multiple causes. This article discusses hereditary bleeding disordersspecifically hemophilia A and hemophilia B. […] Hemophilia occurs in two major typesA and B. […] Hemophilia A, a deficiency of coagulation factor VIII, occurs in approximately 1 in 10,000 males. […] Hemophilia B, a deficiency of coagulation factor IX, occurs in approximately 1 in 50,000 males. […] Hemophilia is categorized as mild, moderate, or severe. […] For many years, blood or plasma transfusions were the main treatment for hemophilia.

• #2 Nursing Care Plan (NCP) for Hemophilia | Free NURSING.com Courses

  https://nursing.com/lesson/nursing-care-plan-for-hemophilia

  Hemophilia is an inherited bleeding disorder caused by mutations in the genes that encode clotting factors, specifically Factor VIII (Hemophilia A) or Factor IX (Hemophilia B). […] Individuals with hemophilia have a deficiency or absence of clotting factors (Factor VIII in Hemophilia A, Factor IX in Hemophilia B), critical components in the blood clotting cascade. […] The primary goal is to prevent and minimize bleeding episodes by ensuring adequate levels of clotting factors through appropriate treatments such as factor replacement therapy. […] Achieve and maintain optimal levels of clotting factors to facilitate normal blood clotting and prevent spontaneous bleeding or bleeding following injury. […] Educate patients and their families about the importance of adherence to treatment plans, recognizing early signs of bleeding, and taking preventive measures. Empower individuals to actively participate in their care and advocate for their health needs.

• #3 5 Hemophilia Nursing Care… – Planning Nursing Care – NCPFacebookShared with Public

  https://www.facebook.com/PlanningNursingCare/posts/5-hemophilia-nursing-care-planshttpsplanningnursingcareblogspotcom2020055-hemoph/131150585216883/

  Hemophilia, an X-linked disorder, is a congenital inherited bleeding disorder of the clotting mechanism cause by an abnormal gene that produces a defective clotting factor protein with little or no clotting ability. The two most common forms of this disorder are Classic hemophilia (type A) which is caused by the lack of factor VIII and Christmas disease (type B) is caused by the deficiency in factor IX. Since both of these disorders are X-linked, the female is the carrier and the disorder is manifested only in males. […] Hemophilia is classified into the following three groups, based on the severity of factor deficiency, mild (5-50%), moderate (1-5%) and severe (1%). Hemophiliacs are at risk for prolonged bleeding or hemorrhage as a result of minor trauma. Individuals with severe hemophilia, or less than 1% clotting factor, are also at risk to suffer from spontaneous bleeding without trauma or more severe prolonged bleeding after trauma. […] The nursing care plan goals for a client with hemophilia may include absence of complications and pain, prevention of injury and bleeding, improved physical mobility, and understanding of the disease condition and its management.

• #4 Hemophilia Nursing Care Planning and Management Study Guide – Nurseslabs

  https://nurseslabs.com/hemophilia/

  Hemophilia nursing care includes individualized care, patient education, and a multidisciplinary approach in handling individuals living with this complex bleeding disorder. […] Nursing care for a child with hemophilia includes assessment of history and physical examination. […] The major nursing diagnoses are acute pain related to traumatic injury to the muscles, impaired physical injury related to pain and discomfort with the onset of bleeding episodes, compromised family coping related to incorrect and inadequate information or understanding, risk for bleeding related to decreased concentration of clotting factors circulating in the blood, and risk for injury related to decreased clotting factor. […] The major goals are that the child will experience decreased pain, maintain optimal physical mobility, cope effectively with the illness, and have a decreased risk for injury from possible bleeding through appropriate prophylactic measures.

• #5 HAEMOPHILIA AND NURSING CARE | Hematology, Transfusion and Cell Therapy

  https://www.htct.com.br/en-haemophilia-and-nursing-care-articulo-S2531137921011044

  Hemophilia is a rare, inherited, X-chromosome-linked bleeding disorder resulting from a deficiency of clotting factor VIII (hemophilia A) or factor IX (hemophilia B). […] Nurses may be involved in providing direct clinical care, education, support and self-management for patients and their families. […] In this presentation we will talk about important aspects of hemophilia: pathophysiology, nursing care and concern, treatment pathway and patient education.

• #6 Hemophilia Nursing Care Planning and Management Study Guide – Nurseslabs

  https://nurseslabs.com/hemophilia/

  Hemophilia nursing care includes individualized care, patient education, and a multidisciplinary approach in handling individuals living with this complex bleeding disorder. […] Nursing care for a child with hemophilia includes assessment of history and physical examination. […] The major nursing diagnoses are acute pain related to traumatic injury to the muscles, impaired physical injury related to pain and discomfort with the onset of bleeding episodes, compromised family coping related to incorrect and inadequate information or understanding, risk for bleeding related to decreased concentration of clotting factors circulating in the blood, and risk for injury related to decreased clotting factor. […] The major goals are that the child will experience decreased pain, maintain optimal physical mobility, cope effectively with the illness, and have a decreased risk for injury from possible bleeding through appropriate prophylactic measures.

• #7 nursing care plan of hemophilia

  https://www.yumpu.com/en/document/view/22642693/nursing-care-plan-of-hemophilia

  Hemophilia is a disease of the blood coagulation due to congenital deficiency of clotting factor VIII children (hemophilia A) or factor IX (hemophilia B). […] Nursing Assessment 1. Assessment of neurological system a. Examination of the head b. Pupillary reaction c. Level of consciousness d. Tendon reflexes e. Sensory Function 2. Hematology a. Public Display b. Skin: (pallor, petechiae, bruising, bleeding from the mucous membranes or wounds or puncture vein injection) c. Abdomen (enlarged liver, spleen) 3. Assess the child’s verbal and nonverbal behavior that indicates pain 4. Assess where relevant to assess the extent of bleeding and widespread damage where sensory and motor nerves. 5. Assess the child’s ability to perform self-care activities (eg, brushing teeth) 6. Assess the child’s developmental level 7. Assess readiness of children and families for repatriation and managing capabilities of home treatment programs. 8. Assess vital signs (BP, N, S, Rr).

• #8 nursing care plan of hemophilia

  https://www.yumpu.com/en/document/view/22642693/nursing-care-plan-of-hemophilia

  Hemophilia is a disease of the blood coagulation due to congenital deficiency of clotting factor VIII children (hemophilia A) or factor IX (hemophilia B). […] Nursing Assessment 1. Assessment of neurological system a. Examination of the head b. Pupillary reaction c. Level of consciousness d. Tendon reflexes e. Sensory Function 2. Hematology a. Public Display b. Skin: (pallor, petechiae, bruising, bleeding from the mucous membranes or wounds or puncture vein injection) c. Abdomen (enlarged liver, spleen) 3. Assess the child’s verbal and nonverbal behavior that indicates pain 4. Assess where relevant to assess the extent of bleeding and widespread damage where sensory and motor nerves. 5. Assess the child’s ability to perform self-care activities (eg, brushing teeth) 6. Assess the child’s developmental level 7. Assess readiness of children and families for repatriation and managing capabilities of home treatment programs. 8. Assess vital signs (BP, N, S, Rr).

• #9 nursing care plan of hemophilia

  https://www.yumpu.com/en/document/view/22642693/nursing-care-plan-of-hemophilia

  Hemophilia is a disease of the blood coagulation due to congenital deficiency of clotting factor VIII children (hemophilia A) or factor IX (hemophilia B). […] Nursing Assessment 1. Assessment of neurological system a. Examination of the head b. Pupillary reaction c. Level of consciousness d. Tendon reflexes e. Sensory Function 2. Hematology a. Public Display b. Skin: (pallor, petechiae, bruising, bleeding from the mucous membranes or wounds or puncture vein injection) c. Abdomen (enlarged liver, spleen) 3. Assess the child’s verbal and nonverbal behavior that indicates pain 4. Assess where relevant to assess the extent of bleeding and widespread damage where sensory and motor nerves. 5. Assess the child’s ability to perform self-care activities (eg, brushing teeth) 6. Assess the child’s developmental level 7. Assess readiness of children and families for repatriation and managing capabilities of home treatment programs. 8. Assess vital signs (BP, N, S, Rr).

• #10 nursing care plan of hemophilia

  https://www.yumpu.com/en/document/view/22642693/nursing-care-plan-of-hemophilia

  Hemophilia is a disease of the blood coagulation due to congenital deficiency of clotting factor VIII children (hemophilia A) or factor IX (hemophilia B). […] Nursing Assessment 1. Assessment of neurological system a. Examination of the head b. Pupillary reaction c. Level of consciousness d. Tendon reflexes e. Sensory Function 2. Hematology a. Public Display b. Skin: (pallor, petechiae, bruising, bleeding from the mucous membranes or wounds or puncture vein injection) c. Abdomen (enlarged liver, spleen) 3. Assess the child’s verbal and nonverbal behavior that indicates pain 4. Assess where relevant to assess the extent of bleeding and widespread damage where sensory and motor nerves. 5. Assess the child’s ability to perform self-care activities (eg, brushing teeth) 6. Assess the child’s developmental level 7. Assess readiness of children and families for repatriation and managing capabilities of home treatment programs. 8. Assess vital signs (BP, N, S, Rr).

• #11 nursing care plan of hemophilia

  https://www.yumpu.com/en/document/view/22642693/nursing-care-plan-of-hemophilia

  Hemophilia is a disease of the blood coagulation due to congenital deficiency of clotting factor VIII children (hemophilia A) or factor IX (hemophilia B). […] Nursing Assessment 1. Assessment of neurological system a. Examination of the head b. Pupillary reaction c. Level of consciousness d. Tendon reflexes e. Sensory Function 2. Hematology a. Public Display b. Skin: (pallor, petechiae, bruising, bleeding from the mucous membranes or wounds or puncture vein injection) c. Abdomen (enlarged liver, spleen) 3. Assess the child’s verbal and nonverbal behavior that indicates pain 4. Assess where relevant to assess the extent of bleeding and widespread damage where sensory and motor nerves. 5. Assess the child’s ability to perform self-care activities (eg, brushing teeth) 6. Assess the child’s developmental level 7. Assess readiness of children and families for repatriation and managing capabilities of home treatment programs. 8. Assess vital signs (BP, N, S, Rr).

• #12 nursing care plan of hemophilia

  https://www.yumpu.com/en/document/view/22642693/nursing-care-plan-of-hemophilia

  Hemophilia is a disease of the blood coagulation due to congenital deficiency of clotting factor VIII children (hemophilia A) or factor IX (hemophilia B). […] Nursing Assessment 1. Assessment of neurological system a. Examination of the head b. Pupillary reaction c. Level of consciousness d. Tendon reflexes e. Sensory Function 2. Hematology a. Public Display b. Skin: (pallor, petechiae, bruising, bleeding from the mucous membranes or wounds or puncture vein injection) c. Abdomen (enlarged liver, spleen) 3. Assess the child’s verbal and nonverbal behavior that indicates pain 4. Assess where relevant to assess the extent of bleeding and widespread damage where sensory and motor nerves. 5. Assess the child’s ability to perform self-care activities (eg, brushing teeth) 6. Assess the child’s developmental level 7. Assess readiness of children and families for repatriation and managing capabilities of home treatment programs. 8. Assess vital signs (BP, N, S, Rr).

• #13 nursing care plan of hemophilia

  https://www.yumpu.com/en/document/view/22642693/nursing-care-plan-of-hemophilia

  Hemophilia is a disease of the blood coagulation due to congenital deficiency of clotting factor VIII children (hemophilia A) or factor IX (hemophilia B). […] Nursing Assessment 1. Assessment of neurological system a. Examination of the head b. Pupillary reaction c. Level of consciousness d. Tendon reflexes e. Sensory Function 2. Hematology a. Public Display b. Skin: (pallor, petechiae, bruising, bleeding from the mucous membranes or wounds or puncture vein injection) c. Abdomen (enlarged liver, spleen) 3. Assess the child’s verbal and nonverbal behavior that indicates pain 4. Assess where relevant to assess the extent of bleeding and widespread damage where sensory and motor nerves. 5. Assess the child’s ability to perform self-care activities (eg, brushing teeth) 6. Assess the child’s developmental level 7. Assess readiness of children and families for repatriation and managing capabilities of home treatment programs. 8. Assess vital signs (BP, N, S, Rr).

• #14 Hemophilia Nursing Care Planning and Management Study Guide – Nurseslabs

  https://nurseslabs.com/hemophilia/

  Hemophilia nursing care includes individualized care, patient education, and a multidisciplinary approach in handling individuals living with this complex bleeding disorder. […] Nursing care for a child with hemophilia includes assessment of history and physical examination. […] The major nursing diagnoses are acute pain related to traumatic injury to the muscles, impaired physical injury related to pain and discomfort with the onset of bleeding episodes, compromised family coping related to incorrect and inadequate information or understanding, risk for bleeding related to decreased concentration of clotting factors circulating in the blood, and risk for injury related to decreased clotting factor. […] The major goals are that the child will experience decreased pain, maintain optimal physical mobility, cope effectively with the illness, and have a decreased risk for injury from possible bleeding through appropriate prophylactic measures.

• #15 Hemophilia Nursing Care Planning and Management Study Guide – Nurseslabs

  https://nurseslabs.com/hemophilia/

  Hemophilia nursing care includes individualized care, patient education, and a multidisciplinary approach in handling individuals living with this complex bleeding disorder. […] Nursing care for a child with hemophilia includes assessment of history and physical examination. […] The major nursing diagnoses are acute pain related to traumatic injury to the muscles, impaired physical injury related to pain and discomfort with the onset of bleeding episodes, compromised family coping related to incorrect and inadequate information or understanding, risk for bleeding related to decreased concentration of clotting factors circulating in the blood, and risk for injury related to decreased clotting factor. […] The major goals are that the child will experience decreased pain, maintain optimal physical mobility, cope effectively with the illness, and have a decreased risk for injury from possible bleeding through appropriate prophylactic measures.

• #16 7 Hemophilia Nursing Care Plans – Nurseslabs

  https://nurseslabs.com/hemophilia-nursing-care-plans/

  Hemophilia is a rare inherited bleeding disorder that affects the bodys ability to form clots. This can lead to excessive bleeding even from minor injuries, and can be life-threatening in some cases. As a nurse, it is essential to understand the nursing care plans and nursing diagnosis that are required to care for patients with hemophilia. […] The nursing care plan goals for a client with hemophilia may include the absence of complications and pain, prevention of injury and bleeding, improved physical mobility, and understanding of the disease condition and its management. […] The following are the nursing priorities for patients with hemophilia: Bleeding prevention and management, Monitor for signs of bleeding complications, Administration of clotting factor replacement therapy, Injury prevention and self-care measures, Pain management during bleeding episodes, Promote physical mobility and joint protection, Ensure compliance with treatment plans and medication regimens.

• #17 Hemophilia Nursing Care Planning and Management Study Guide – Nurseslabs

  https://nurseslabs.com/hemophilia/

  The nursing interventions for a child with hemophilia include relieving pain, maintaining optimal physical mobility, assisting in the coping of the family, preventing bleeding, and preventing injury. […] Goals are met as evidenced by decreased pain, maintained optimal physical mobility, effective coping by the family, and decreased risk for injury from possible bleeding. […] Documentation in a child with hemophilia includes baseline and subsequent assessment findings, individual cultural or religious restrictions, plan of care, teaching plan, clients’ responses to teachings, and attainment or progress toward the desired outcome.

• #18 7 Hemophilia Nursing Care Plans – Nurseslabs

  https://nurseslabs.com/hemophilia-nursing-care-plans/

  A nursing diagnosis helps in identifying and addressing specific patient needs and responses related to hemophilia. By conducting a comprehensive assessment, nurses can formulate an accurate nursing diagnosis that guides the development of a customized care plan. […] Therapeutic interventions and nursing actions for patients with hemophilia may include: Preventing Bleeding Risk and Injury, Managing Acute Pain, Improving Physical Mobility, Promoting Effective Family Coping, Assessing and Monitoring for Potential Complications, Administering Medications and Pharmacologic Support, Monitoring Laboratory and Diagnostic Procedures. […] Children with hemophilia are at risk for injury due to the decreased concentration of clotting factors in their blood, which leads to impaired blood clotting and prolonged bleeding after injury or trauma.

• #19 7 Hemophilia Nursing Care Plans – Nurseslabs

  https://nurseslabs.com/hemophilia-nursing-care-plans/

  Provide replacement therapy for deficient clotting factors. Replacement of factors is the primary treatment for bleeding. Treatment includes factor VIII, which is an essential clotting factor needed to convert prothrombin to thrombin. […] Educate the family and affected child about the signs and symptoms of bleeding, and appropriate interventions to control bleeding at home. […] Teach parents related to home health maintenance: The affected child should receive all routine immunizations (use the subcutaneous route, recommend pressure and an elastic bandage after injections).

• #20 Nursing Care Plan (NCP) for Hemophilia | Free NURSING.com Courses

  https://nursing.com/lesson/nursing-care-plan-for-hemophilia

  Administer clotting factor replacement therapy as prescribed to manage and prevent bleeding episodes. Monitor levels regularly and adjust the dosage as needed. […] Implement pain management strategies, such as pharmacological interventions and non-pharmacological approaches, to address pain associated with bleeding episodes or joint damage. […] Provide ongoing education to the individual and their family about hemophilia, treatment options, and self-care practices. Include counseling to address psychosocial aspects and promote mental well-being. […] Regularly assess clotting factor levels to ensure that replacement therapy maintains levels within the target range. Adjust treatment plans based on monitoring results. […] Evaluate the frequency and severity of bleeding episodes over time. A reduction in the number of bleeding episodes indicates the effectiveness of the treatment plan.

• #21 Hemophilia Nursing Diagnosis & Care Plan – NurseStudy.Net

  https://nursestudy.net/hemophilia-nursing-diagnosis/

  Monitor for bleeding signs. Rationale: Early detection allows prompt intervention. Administer factor replacement as prescribed. Rationale: Maintains therapeutic factor levels. Teach injury prevention. Rationale: Reduces bleeding risk. […] The patient will maintain optimal joint function. The patient will participate in appropriate physical activities. The patient will demonstrate proper body mechanics. […] Provide comprehensive education. Rationale: Improves self-management. Demonstrate treatment procedures. Rationale: Ensures proper technique. Review emergency protocols. Rationale: Prepares for complications. […] The patient will maintain the treatment schedule. The patient will utilize support resources. The patient will demonstrate improved compliance. […] The patient will express decreased anxiety. The patient will utilize coping strategies. The patient will participate in support activities.

• #22 Hemophilia Nursing Diagnosis & Care Plan – NurseStudy.Net

  https://nursestudy.net/hemophilia-nursing-diagnosis/

  Monitor for bleeding signs. Rationale: Early detection allows prompt intervention. Administer factor replacement as prescribed. Rationale: Maintains therapeutic factor levels. Teach injury prevention. Rationale: Reduces bleeding risk. […] The patient will maintain optimal joint function. The patient will participate in appropriate physical activities. The patient will demonstrate proper body mechanics. […] Provide comprehensive education. Rationale: Improves self-management. Demonstrate treatment procedures. Rationale: Ensures proper technique. Review emergency protocols. Rationale: Prepares for complications. […] The patient will maintain the treatment schedule. The patient will utilize support resources. The patient will demonstrate improved compliance. […] The patient will express decreased anxiety. The patient will utilize coping strategies. The patient will participate in support activities.

• #23 Hemophilia: Treatment, Preventing Bleeding and Daily Care

  https://www.nationwidechildrens.org/conditions/hemophilia

  Here are some things you can do to help prevent bleeding: […] Be sure to tell the doctor, dentist or nurse your child has hemophilia. […] Be sure immunizations are given under the skin (subcutaneous) instead of into a muscle (intramuscular) and are given with a smaller needle. The staff should apply pressure and ice to the injection site for 5 to 10 minutes after the shot is given. This prevents bleeding. […] Do not let your child get intramuscular (IM) injections (shots). […] Never let a doctor or dentist do any procedure that involves opening the skin until you have talked with the Hemophilia team. […] People with bleeding disorders must talk to their doctor or nurse before taking medicine that can affect their platelets. These include aspirin, ibuprofen and naproxen. Always ask your hemophilia team before giving your child a medicine.

• #24 Hemophilia: Treatment, Preventing Bleeding and Daily Care

  https://www.nationwidechildrens.org/conditions/hemophilia

  Here are some things you can do to help prevent bleeding: […] Be sure to tell the doctor, dentist or nurse your child has hemophilia. […] Be sure immunizations are given under the skin (subcutaneous) instead of into a muscle (intramuscular) and are given with a smaller needle. The staff should apply pressure and ice to the injection site for 5 to 10 minutes after the shot is given. This prevents bleeding. […] Do not let your child get intramuscular (IM) injections (shots). […] Never let a doctor or dentist do any procedure that involves opening the skin until you have talked with the Hemophilia team. […] People with bleeding disorders must talk to their doctor or nurse before taking medicine that can affect their platelets. These include aspirin, ibuprofen and naproxen. Always ask your hemophilia team before giving your child a medicine.

• #25 Hemophilia: Treatment, Preventing Bleeding and Daily Care

  https://www.nationwidechildrens.org/conditions/hemophilia

  Here are some things you can do to help prevent bleeding: […] Be sure to tell the doctor, dentist or nurse your child has hemophilia. […] Be sure immunizations are given under the skin (subcutaneous) instead of into a muscle (intramuscular) and are given with a smaller needle. The staff should apply pressure and ice to the injection site for 5 to 10 minutes after the shot is given. This prevents bleeding. […] Do not let your child get intramuscular (IM) injections (shots). […] Never let a doctor or dentist do any procedure that involves opening the skin until you have talked with the Hemophilia team. […] People with bleeding disorders must talk to their doctor or nurse before taking medicine that can affect their platelets. These include aspirin, ibuprofen and naproxen. Always ask your hemophilia team before giving your child a medicine.

• #26 Hemophilia: Treatment, Preventing Bleeding and Daily Care

  https://www.nationwidechildrens.org/conditions/hemophilia

  Here are some things you can do to help prevent bleeding: […] Be sure to tell the doctor, dentist or nurse your child has hemophilia. […] Be sure immunizations are given under the skin (subcutaneous) instead of into a muscle (intramuscular) and are given with a smaller needle. The staff should apply pressure and ice to the injection site for 5 to 10 minutes after the shot is given. This prevents bleeding. […] Do not let your child get intramuscular (IM) injections (shots). […] Never let a doctor or dentist do any procedure that involves opening the skin until you have talked with the Hemophilia team. […] People with bleeding disorders must talk to their doctor or nurse before taking medicine that can affect their platelets. These include aspirin, ibuprofen and naproxen. Always ask your hemophilia team before giving your child a medicine.

• #27 Hemophilia – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hemophilia/diagnosis-treatment/drc-20373333

  Avoid certain pain medications. Drugs that can make bleeding worse include aspirin and ibuprofen (Advil, Motrin IB, others). Instead, use acetaminophen (Tylenol, others), which is a safer alternative for mild pain relief. […] Avoid blood-thinning medications. Medications that prevent blood from clotting include heparin, warfarin (Jantoven), clopidogrel (Plavix), prasugrel (Effient), ticagrelor (Brilinta), rivaroxaban (Xarelto), apixaban (Eliquis), edoxaban (Savaysa) and dabigatran (Pradaxa). […] Practice good dental hygiene. The goal is to prevent tooth and gum disease, which can lead to excessive bleeding. […] Get vaccinations. People with hemophilia should receive recommended vaccinations at the appropriate ages, as well as hepatitis A and B. […] Protect your child from injuries that could cause bleeding. Kneepads, elbow pads, helmets and safety belts all help prevent injuries from falls and other accidents.

• #28 Nursing Care Plan (NCP) for Hemophilia | Free NURSING.com Courses

  https://nursing.com/lesson/nursing-care-plan-for-hemophilia

  Administer clotting factor replacement therapy as prescribed to manage and prevent bleeding episodes. Monitor levels regularly and adjust the dosage as needed. […] Implement pain management strategies, such as pharmacological interventions and non-pharmacological approaches, to address pain associated with bleeding episodes or joint damage. […] Provide ongoing education to the individual and their family about hemophilia, treatment options, and self-care practices. Include counseling to address psychosocial aspects and promote mental well-being. […] Regularly assess clotting factor levels to ensure that replacement therapy maintains levels within the target range. Adjust treatment plans based on monitoring results. […] Evaluate the frequency and severity of bleeding episodes over time. A reduction in the number of bleeding episodes indicates the effectiveness of the treatment plan.

• #29 Hemophilia – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hemophilia/diagnosis-treatment/drc-20373333

  Avoid certain pain medications. Drugs that can make bleeding worse include aspirin and ibuprofen (Advil, Motrin IB, others). Instead, use acetaminophen (Tylenol, others), which is a safer alternative for mild pain relief. […] Avoid blood-thinning medications. Medications that prevent blood from clotting include heparin, warfarin (Jantoven), clopidogrel (Plavix), prasugrel (Effient), ticagrelor (Brilinta), rivaroxaban (Xarelto), apixaban (Eliquis), edoxaban (Savaysa) and dabigatran (Pradaxa). […] Practice good dental hygiene. The goal is to prevent tooth and gum disease, which can lead to excessive bleeding. […] Get vaccinations. People with hemophilia should receive recommended vaccinations at the appropriate ages, as well as hepatitis A and B. […] Protect your child from injuries that could cause bleeding. Kneepads, elbow pads, helmets and safety belts all help prevent injuries from falls and other accidents.

• #30 Hemophilia | PPT

  https://www.slideshare.net/slideshow/hemophilia-5734428/5734428

  Replace clotting factor and blood product to increase patient blood clotting. […] Nursing diagnosis Pain related bleeding into tissue Goal Patient will verbalize that pain is relieved to a satisfactory level. […] Administer opioid (morphine) as prescribed to control pain from severe to moderate. […] Monitor sedation and respiratory status of the patient receiving opioid of pain because opioid can cause depress respiratory center of the brain.

• #31 Hemophilia | PPT

  https://www.slideshare.net/slideshow/hemophilia-5734428/5734428

  Replace clotting factor and blood product to increase patient blood clotting. […] Nursing diagnosis Pain related bleeding into tissue Goal Patient will verbalize that pain is relieved to a satisfactory level. […] Administer opioid (morphine) as prescribed to control pain from severe to moderate. […] Monitor sedation and respiratory status of the patient receiving opioid of pain because opioid can cause depress respiratory center of the brain.

• #32 Nursing Care Plan (NCP) for Hemophilia | Free NURSING.com Courses

  https://nursing.com/lesson/nursing-care-plan-for-hemophilia?quiz-view=open

  Administer clotting factor replacement therapy as prescribed to manage and prevent bleeding episodes. Monitor levels regularly and adjust the dosage as needed. […] Implement pain management strategies, such as pharmacological interventions and non-pharmacological approaches, to address pain associated with bleeding episodes or joint damage. […] Collaborate with physical therapists to develop and implement joint protection strategies and exercises. Encourage regular physical therapy to maintain joint mobility and prevent complications like hemophilic arthropathy. […] Develop an emergency plan for managing unexpected bleeding episodes. Educate the patient and caregivers on recognizing signs of severe bleeding and the steps to take, including when to seek immediate medical attention. […] Regularly assess clotting factor levels to ensure that replacement therapy maintains levels within the target range. Adjust treatment plans based on monitoring results. […] Evaluate the frequency and severity of bleeding episodes over time. A reduction in the number of bleeding episodes indicates the effectiveness of the treatment plan.

• #33 Hemophilia – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hemophilia/diagnosis-treatment/drc-20373333

  Clot-preserving medications. Also known as anti-fibrinolytics, these medications help prevent clots from breaking down. […] Fibrin sealants. These can be applied directly to wound sites to promote clotting and healing. Fibrin sealants are especially useful for dental work. […] Physical therapy. It can ease signs and symptoms if internal bleeding has damaged your joints. Severe damage might require surgery. […] First aid for minor cuts. Using pressure and a bandage will generally take care of the bleeding. For small areas of bleeding beneath the skin, use an ice pack. Ice pops can be used to slow down minor bleeding in the mouth. […] To avoid excessive bleeding and protect your joints: Exercise regularly. Activities such as swimming, bicycle riding and walking can build muscles while protecting joints.

• #34 Hemophilia: Treatment, Preventing Bleeding and Daily Care

  https://www.nationwidechildrens.org/conditions/hemophilia

  Children with hemophilia should not play contact sports such as football, wrestling, boxing or hockey. If you have any questions about your child playing sports, be sure to talk with your hemophilia team. […] Early treatment of bleeding is especially important for people with hemophilia. They should follow these good rules for early treatment: […] Take factor right away if you have an injury. This is especially important if a similar injury caused a bleed in the past. […] Take factor as soon as you think you are bleeding. […] Take factor as soon as you see or feel any signs of bleeding such as tingling, bubbling, warmth or swelling. […] If you are not sure you are bleeding, take factor anyway. […] Your child should wear a medical identification bracelet or necklace. You can get it through your treatment center or local hemophilia chapter. It states that your child has hemophilia so if an emergency occurs, the doctor will know.

• #35 7 Hemophilia Nursing Care Plans – Nurseslabs

  https://nurseslabs.com/hemophilia-nursing-care-plans/

  Provide replacement therapy for deficient clotting factors. Replacement of factors is the primary treatment for bleeding. Treatment includes factor VIII, which is an essential clotting factor needed to convert prothrombin to thrombin. […] Educate the family and affected child about the signs and symptoms of bleeding, and appropriate interventions to control bleeding at home. […] Teach parents related to home health maintenance: The affected child should receive all routine immunizations (use the subcutaneous route, recommend pressure and an elastic bandage after injections).

• #36 Nursing Care Plan (NCP) for Hemophilia | Free NURSING.com Courses

  https://nursing.com/lesson/nursing-care-plan-for-hemophilia

  Administer clotting factor replacement therapy as prescribed to manage and prevent bleeding episodes. Monitor levels regularly and adjust the dosage as needed. […] Implement pain management strategies, such as pharmacological interventions and non-pharmacological approaches, to address pain associated with bleeding episodes or joint damage. […] Provide ongoing education to the individual and their family about hemophilia, treatment options, and self-care practices. Include counseling to address psychosocial aspects and promote mental well-being. […] Regularly assess clotting factor levels to ensure that replacement therapy maintains levels within the target range. Adjust treatment plans based on monitoring results. […] Evaluate the frequency and severity of bleeding episodes over time. A reduction in the number of bleeding episodes indicates the effectiveness of the treatment plan.

• #37 Hemophilia Nursing Diagnosis & Care Plan – NurseStudy.Net

  https://nursestudy.net/hemophilia-nursing-diagnosis/

  Monitor for bleeding signs. Rationale: Early detection allows prompt intervention. Administer factor replacement as prescribed. Rationale: Maintains therapeutic factor levels. Teach injury prevention. Rationale: Reduces bleeding risk. […] The patient will maintain optimal joint function. The patient will participate in appropriate physical activities. The patient will demonstrate proper body mechanics. […] Provide comprehensive education. Rationale: Improves self-management. Demonstrate treatment procedures. Rationale: Ensures proper technique. Review emergency protocols. Rationale: Prepares for complications. […] The patient will maintain the treatment schedule. The patient will utilize support resources. The patient will demonstrate improved compliance. […] The patient will express decreased anxiety. The patient will utilize coping strategies. The patient will participate in support activities.

• #38 Hemophilia Nursing Diagnosis & Care Plan – NurseStudy.Net

  https://nursestudy.net/hemophilia-nursing-diagnosis/

  Monitor for bleeding signs. Rationale: Early detection allows prompt intervention. Administer factor replacement as prescribed. Rationale: Maintains therapeutic factor levels. Teach injury prevention. Rationale: Reduces bleeding risk. […] The patient will maintain optimal joint function. The patient will participate in appropriate physical activities. The patient will demonstrate proper body mechanics. […] Provide comprehensive education. Rationale: Improves self-management. Demonstrate treatment procedures. Rationale: Ensures proper technique. Review emergency protocols. Rationale: Prepares for complications. […] The patient will maintain the treatment schedule. The patient will utilize support resources. The patient will demonstrate improved compliance. […] The patient will express decreased anxiety. The patient will utilize coping strategies. The patient will participate in support activities.

• #39 Nursing Care Plan (NCP) for Hemophilia | Free NURSING.com Courses

  https://nursing.com/lesson/nursing-care-plan-for-hemophilia

  Administer clotting factor replacement therapy as prescribed to manage and prevent bleeding episodes. Monitor levels regularly and adjust the dosage as needed. […] Implement pain management strategies, such as pharmacological interventions and non-pharmacological approaches, to address pain associated with bleeding episodes or joint damage. […] Provide ongoing education to the individual and their family about hemophilia, treatment options, and self-care practices. Include counseling to address psychosocial aspects and promote mental well-being. […] Regularly assess clotting factor levels to ensure that replacement therapy maintains levels within the target range. Adjust treatment plans based on monitoring results. […] Evaluate the frequency and severity of bleeding episodes over time. A reduction in the number of bleeding episodes indicates the effectiveness of the treatment plan.

• #40 Hemophilia – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK551607/

  Hemophilia A and B are the most common severe hereditary hemorrhagic disorders. […] The principal aim of care should be to avoid and treat bleeding. […] The patient should receive treatment in a comprehensive treatment center where interprofessional services are offered at all times to the patients and their families. […] This activity reviews the epidemiology, natural history, evaluation, and management of hemophilia and also highlights the role of the interprofessional team in evaluating, managing, and improving care for patients with this condition. […] The hemophilia treatment strategy is primarily divided into two categories – management of acute bleeding and prophylaxis. […] The fundamental concept of management of a diagnosed or confirmed acute bleeding in hemophilia is to achieve quick and aggressive hemostasis, preferably within two hours of the onset of symptoms and correction of coagulopathy, but these measures should not be delayed even if diagnostic tests are pending or if physical symptoms are not present.

• #41 Hemophilia – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hemophilia/diagnosis-treatment/drc-20373333

  Our caring team of Mayo Clinic experts can help you with your hemophilia-related health concerns […] The main treatment for severe hemophilia involves replacing the clotting factor you need through a tube in a vein. […] This replacement therapy can be given to treat a bleeding episode in progress. It can also be given on a regular schedule at home to help prevent bleeding episodes. Some people receive continuous replacement therapy. […] Other therapies include: Desmopressin. In some forms of mild hemophilia, this hormone can stimulate the body to release more clotting factor. It can be injected slowly into a vein or used as a nasal spray. […] Emicizumab (Hemlibra). This is a newer drug that doesn’t include clotting factors. This drug can help prevent bleeding episodes in people with hemophilia A.

• #42 Hemophilia B (Factor IX Deficiency) Treatment & Management: Approach Considerations, Prehospital Care, Emergency Department Care

  https://emedicine.medscape.com/article/779434-treatment

  Patients may be treated with prophylaxis or with intermittent, on-demand therapy for bleeding events. Prophylaxis has been shown in many studies to prevent or at least reduce the progression of damage to target sites, such as joints. […] Indeed, the International Society on Thrombosis and Haemostasis (ISTH) Hemophilia Guideline Panel recommends that patients with severe and moderately severe congenital hemophilia B without inhibitors be treated prophylactically rather than through episodic management of bleeding events. […] In most developed countries with access to recombinant product, prophylaxis is primary (ie, therapy is started in patients as young as 1 y and continues into adolescence). […] Prophylactic replacement is used to maintain a measurable FIX level at all times, with the goal of avoiding hemarthrosis and the vicious cycle of repetitive bleeding and inflammation that results in destructive arthritis.

• #43 Hemophilia A (Factor VIII Deficiency) Treatment & Management: Approach Considerations, Prehospital Care, Emergency Department Care

  https://emedicine.medscape.com/article/779322-treatment

  Patients can be treated with prophylaxis or with intermittent, on-demand therapy for bleeding events. Prophylaxis has been shown in many studies to prevent or at least reduce the progression of damage to target sites, such as joints. […] According to a review of six randomized, controlled trials, preventive therapy started early in childhood, as compared with on-demand treatment, can reduce total bleeds and bleeding into joints, resulting in decreased overall joint deterioration and improved quality of life. […] Indeed, the International Society on Thrombosis and Haemostasis (ISTH) Hemophilia Guideline Panel recommends that patients with severe and moderately severe congenital hemophilia A without inhibitors be treated prophylactically rather than through episodic management of bleeding events.

• #44 Hemophilia B (Factor IX Deficiency) Treatment & Management: Approach Considerations, Prehospital Care, Emergency Department Care

  https://emedicine.medscape.com/article/779434-treatment

  Patients may be treated with prophylaxis or with intermittent, on-demand therapy for bleeding events. Prophylaxis has been shown in many studies to prevent or at least reduce the progression of damage to target sites, such as joints. […] Indeed, the International Society on Thrombosis and Haemostasis (ISTH) Hemophilia Guideline Panel recommends that patients with severe and moderately severe congenital hemophilia B without inhibitors be treated prophylactically rather than through episodic management of bleeding events. […] In most developed countries with access to recombinant product, prophylaxis is primary (ie, therapy is started in patients as young as 1 y and continues into adolescence). […] Prophylactic replacement is used to maintain a measurable FIX level at all times, with the goal of avoiding hemarthrosis and the vicious cycle of repetitive bleeding and inflammation that results in destructive arthritis.

• #45 Hemophilia – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hemophilia/diagnosis-treatment/drc-20373333

  Our caring team of Mayo Clinic experts can help you with your hemophilia-related health concerns […] The main treatment for severe hemophilia involves replacing the clotting factor you need through a tube in a vein. […] This replacement therapy can be given to treat a bleeding episode in progress. It can also be given on a regular schedule at home to help prevent bleeding episodes. Some people receive continuous replacement therapy. […] Other therapies include: Desmopressin. In some forms of mild hemophilia, this hormone can stimulate the body to release more clotting factor. It can be injected slowly into a vein or used as a nasal spray. […] Emicizumab (Hemlibra). This is a newer drug that doesn’t include clotting factors. This drug can help prevent bleeding episodes in people with hemophilia A.

• #46 Treatment of Hemophilia | Hemophilia | CDC

  https://www.cdc.gov/hemophilia/treatment/index.html

  Hemlibra works by replacing the function of factor VIII, rather than replacing the missing clotting factor VIII directly. […] DDAVP and Stimate are medications that are similar to a hormone that occurs naturally in the body. […] Amicar is a medication that can be given through a vein or by mouth (as a pill or a liquid). […] Roctavian is a gene therapy that was approved by the FDA on June 29, 2023, for the treatment of severe hemophilia A (factor VIII deficiency) in adults. […] Hemgenix is a gene therapy that was approved by the FDA on November 22, 2022, for the treatment of hemophilia B (factor IX deficiency) in adults.

• #47 7 Hemophilia Nursing Care Plans – Nurseslabs

  https://nurseslabs.com/hemophilia-nursing-care-plans/

  Provide replacement therapy for deficient clotting factors. Replacement of factors is the primary treatment for bleeding. Treatment includes factor VIII, which is an essential clotting factor needed to convert prothrombin to thrombin. […] Educate the family and affected child about the signs and symptoms of bleeding, and appropriate interventions to control bleeding at home. […] Teach parents related to home health maintenance: The affected child should receive all routine immunizations (use the subcutaneous route, recommend pressure and an elastic bandage after injections).

• #48 Hemophilia: Treatment, Preventing Bleeding and Daily Care

  https://www.nationwidechildrens.org/conditions/hemophilia

  Regular tooth brushing, flossing and dental care are important. When he goes to the dentist, be sure to tell the dentist your child has hemophilia. […] You can learn how to give clotting factor at home. Talk to the Hemophilia team about this. […] As your child gets older and more mature, he can learn to give the factor to himself. […] When your child is diagnosed with hemophilia, you will meet with the Hemophilia Team (doctor, nurse, physical therapist, psychologist, genetic counselor and social worker) to talk about your child’s care. […] Your child will have regular appointments at the Comprehensive Hemophilia Center. This clinic provides specialists in hematology, dentistry, orthopedic surgery, social work, clinical nursing, research nursing and physical therapy. […] The longer you wait, the more severe the bleeding can become. Delay can increase the amount of damage and the length of treatment. […] If you have any questions, be sure to call the Hemophilia Team.

• #49 Hemophilia: Treatment, Preventing Bleeding and Daily Care

  https://www.nationwidechildrens.org/conditions/hemophilia

  Children with hemophilia should not play contact sports such as football, wrestling, boxing or hockey. If you have any questions about your child playing sports, be sure to talk with your hemophilia team. […] Early treatment of bleeding is especially important for people with hemophilia. They should follow these good rules for early treatment: […] Take factor right away if you have an injury. This is especially important if a similar injury caused a bleed in the past. […] Take factor as soon as you think you are bleeding. […] Take factor as soon as you see or feel any signs of bleeding such as tingling, bubbling, warmth or swelling. […] If you are not sure you are bleeding, take factor anyway. […] Your child should wear a medical identification bracelet or necklace. You can get it through your treatment center or local hemophilia chapter. It states that your child has hemophilia so if an emergency occurs, the doctor will know.

• #50 Hemophilia: Treatment, Preventing Bleeding and Daily Care

  https://www.nationwidechildrens.org/conditions/hemophilia

  Children with hemophilia should not play contact sports such as football, wrestling, boxing or hockey. If you have any questions about your child playing sports, be sure to talk with your hemophilia team. […] Early treatment of bleeding is especially important for people with hemophilia. They should follow these good rules for early treatment: […] Take factor right away if you have an injury. This is especially important if a similar injury caused a bleed in the past. […] Take factor as soon as you think you are bleeding. […] Take factor as soon as you see or feel any signs of bleeding such as tingling, bubbling, warmth or swelling. […] If you are not sure you are bleeding, take factor anyway. […] Your child should wear a medical identification bracelet or necklace. You can get it through your treatment center or local hemophilia chapter. It states that your child has hemophilia so if an emergency occurs, the doctor will know.

• #51 Hemophilia: Handling Bleeds (for Parents) | Nemours KidsHealth

  https://kidshealth.org/en/parents/hemophilia-bleeds.html

  Hemophilia is a rare disease that prevents blood from clotting as it should. Kids with hemophilia can bleed easily or longer than normal. So it’s important for parents to know how to handle bleeding when it happens. […] Most bleeding from minor injuries can be treated at home. More serious injuries may need treatment in the doctor’s office, hemophilia treatment center, or emergency room. […] Call your care team if: The area is very painful. The bruise is getting bigger. The bruise makes movement painful. […] If the bleeding does not stop within 20 minutes, call your care team. […] If the bleeding does not stop after 20 minutes, call your doctor. Follow your doctor’s instructions for: giving factor replacement therapy (which gives the body the clotting factor it needs) going to the doctor’s office, hemophilia treatment center, or emergency room.

• #52 Hemophilia: Handling Bleeds (for Parents) | Nemours KidsHealth

  https://kidshealth.org/en/parents/hemophilia-bleeds.html

  Hemophilia is a rare disease that prevents blood from clotting as it should. Kids with hemophilia can bleed easily or longer than normal. So it’s important for parents to know how to handle bleeding when it happens. […] Most bleeding from minor injuries can be treated at home. More serious injuries may need treatment in the doctor’s office, hemophilia treatment center, or emergency room. […] Call your care team if: The area is very painful. The bruise is getting bigger. The bruise makes movement painful. […] If the bleeding does not stop within 20 minutes, call your care team. […] If the bleeding does not stop after 20 minutes, call your doctor. Follow your doctor’s instructions for: giving factor replacement therapy (which gives the body the clotting factor it needs) going to the doctor’s office, hemophilia treatment center, or emergency room.

• #53 Hemophilia: Handling Bleeds (for Parents) | Nemours KidsHealth

  https://kidshealth.org/en/parents/hemophilia-bleeds.html

  Call your doctor right away. Follow the doctor’s instructions for: giving factor replacement therapy as soon as possible putting ice on the area giving medicine that is safe for pain, such as acetaminophen (Tylenol or store brand). […] If your child has an injury to the head, chest, neck, or belly, call your doctor right away. Follow the doctor’s instructions for: giving factor replacement therapy at home right away going to the emergency room or calling an ambulance. […] Go to the emergency room if your child: has bleeding that is not stopping even after following your doctor’s instructions.

• #54 Hemophilia: Treatment, Preventing Bleeding and Daily Care

  https://www.nationwidechildrens.org/conditions/hemophilia

  Children with hemophilia should not play contact sports such as football, wrestling, boxing or hockey. If you have any questions about your child playing sports, be sure to talk with your hemophilia team. […] Early treatment of bleeding is especially important for people with hemophilia. They should follow these good rules for early treatment: […] Take factor right away if you have an injury. This is especially important if a similar injury caused a bleed in the past. […] Take factor as soon as you think you are bleeding. […] Take factor as soon as you see or feel any signs of bleeding such as tingling, bubbling, warmth or swelling. […] If you are not sure you are bleeding, take factor anyway. […] Your child should wear a medical identification bracelet or necklace. You can get it through your treatment center or local hemophilia chapter. It states that your child has hemophilia so if an emergency occurs, the doctor will know.

• #55 Hemophilia: Treatment, Preventing Bleeding and Daily Care

  https://www.nationwidechildrens.org/conditions/hemophilia

  Helping Hand Logo […] The key to best outcomes is to prevent and treat bleeding. You do this by making good choices of activities and wearing good safety equipment such as seat belts, bike helmets and sports safety gear. If your child has bleeding, he may be given Factor VIII (8), IX (9), IV (into a vein). Refer to Helping Hand HH-II-17, IV Therapy. For mucosal bleeding (mouth/nose) he might be given an antifibrinolytic medicine (Amicar/Lysteda). Refer to Helping Hand HH-V-258, Tranexamic Acid (Lysteda). […] Your child may need one or more doses of the clotting factor. Your child will receive the factor in the clinic, the hospital or as part of a home treatment program. Where the treatment is done depends on the extent of the bleeding and its location. For some children the nurse coordinator can arrange a home treatment program.

• #56 Hemophilia – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hemophilia/diagnosis-treatment/drc-20373333

  Avoid certain pain medications. Drugs that can make bleeding worse include aspirin and ibuprofen (Advil, Motrin IB, others). Instead, use acetaminophen (Tylenol, others), which is a safer alternative for mild pain relief. […] Avoid blood-thinning medications. Medications that prevent blood from clotting include heparin, warfarin (Jantoven), clopidogrel (Plavix), prasugrel (Effient), ticagrelor (Brilinta), rivaroxaban (Xarelto), apixaban (Eliquis), edoxaban (Savaysa) and dabigatran (Pradaxa). […] Practice good dental hygiene. The goal is to prevent tooth and gum disease, which can lead to excessive bleeding. […] Get vaccinations. People with hemophilia should receive recommended vaccinations at the appropriate ages, as well as hepatitis A and B. […] Protect your child from injuries that could cause bleeding. Kneepads, elbow pads, helmets and safety belts all help prevent injuries from falls and other accidents.

• #57 Hemophilia – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hemophilia/diagnosis-treatment/drc-20373333

  Avoid certain pain medications. Drugs that can make bleeding worse include aspirin and ibuprofen (Advil, Motrin IB, others). Instead, use acetaminophen (Tylenol, others), which is a safer alternative for mild pain relief. […] Avoid blood-thinning medications. Medications that prevent blood from clotting include heparin, warfarin (Jantoven), clopidogrel (Plavix), prasugrel (Effient), ticagrelor (Brilinta), rivaroxaban (Xarelto), apixaban (Eliquis), edoxaban (Savaysa) and dabigatran (Pradaxa). […] Practice good dental hygiene. The goal is to prevent tooth and gum disease, which can lead to excessive bleeding. […] Get vaccinations. People with hemophilia should receive recommended vaccinations at the appropriate ages, as well as hepatitis A and B. […] Protect your child from injuries that could cause bleeding. Kneepads, elbow pads, helmets and safety belts all help prevent injuries from falls and other accidents.

• #58 Hemophilia: Treatment, Preventing Bleeding and Daily Care

  https://www.nationwidechildrens.org/conditions/hemophilia

  Regular tooth brushing, flossing and dental care are important. When he goes to the dentist, be sure to tell the dentist your child has hemophilia. […] You can learn how to give clotting factor at home. Talk to the Hemophilia team about this. […] As your child gets older and more mature, he can learn to give the factor to himself. […] When your child is diagnosed with hemophilia, you will meet with the Hemophilia Team (doctor, nurse, physical therapist, psychologist, genetic counselor and social worker) to talk about your child’s care. […] Your child will have regular appointments at the Comprehensive Hemophilia Center. This clinic provides specialists in hematology, dentistry, orthopedic surgery, social work, clinical nursing, research nursing and physical therapy. […] The longer you wait, the more severe the bleeding can become. Delay can increase the amount of damage and the length of treatment. […] If you have any questions, be sure to call the Hemophilia Team.

• #59 Hemophilia: Treatment, Preventing Bleeding and Daily Care

  https://www.nationwidechildrens.org/conditions/hemophilia

  Here are some things you can do to help prevent bleeding: […] Be sure to tell the doctor, dentist or nurse your child has hemophilia. […] Be sure immunizations are given under the skin (subcutaneous) instead of into a muscle (intramuscular) and are given with a smaller needle. The staff should apply pressure and ice to the injection site for 5 to 10 minutes after the shot is given. This prevents bleeding. […] Do not let your child get intramuscular (IM) injections (shots). […] Never let a doctor or dentist do any procedure that involves opening the skin until you have talked with the Hemophilia team. […] People with bleeding disorders must talk to their doctor or nurse before taking medicine that can affect their platelets. These include aspirin, ibuprofen and naproxen. Always ask your hemophilia team before giving your child a medicine.

• #60 Hemophilia: Treatment, Preventing Bleeding and Daily Care

  https://www.nationwidechildrens.org/conditions/hemophilia

  Children with hemophilia should not play contact sports such as football, wrestling, boxing or hockey. If you have any questions about your child playing sports, be sure to talk with your hemophilia team. […] Early treatment of bleeding is especially important for people with hemophilia. They should follow these good rules for early treatment: […] Take factor right away if you have an injury. This is especially important if a similar injury caused a bleed in the past. […] Take factor as soon as you think you are bleeding. […] Take factor as soon as you see or feel any signs of bleeding such as tingling, bubbling, warmth or swelling. […] If you are not sure you are bleeding, take factor anyway. […] Your child should wear a medical identification bracelet or necklace. You can get it through your treatment center or local hemophilia chapter. It states that your child has hemophilia so if an emergency occurs, the doctor will know.

• #61 Hemophilia – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hemophilia/diagnosis-treatment/drc-20373333

  To help you and your child cope with hemophilia: Get a medical alert bracelet. This lets medical personnel know that you or your child has hemophilia, and the type of clotting factor that’s best in case of an emergency. […] Talk with a counselor. Striking the right balance between keeping your child safe and encouraging as much activity as possible can be tricky. […] Let people know. Be sure to tell anyone who will be taking care of your child babysitters, child care workers, relatives, friends and teachers about your child’s condition.

• #62 Hemophilia: Treatment, Preventing Bleeding and Daily Care

  https://www.nationwidechildrens.org/conditions/hemophilia

  Regular tooth brushing, flossing and dental care are important. When he goes to the dentist, be sure to tell the dentist your child has hemophilia. […] You can learn how to give clotting factor at home. Talk to the Hemophilia team about this. […] As your child gets older and more mature, he can learn to give the factor to himself. […] When your child is diagnosed with hemophilia, you will meet with the Hemophilia Team (doctor, nurse, physical therapist, psychologist, genetic counselor and social worker) to talk about your child’s care. […] Your child will have regular appointments at the Comprehensive Hemophilia Center. This clinic provides specialists in hematology, dentistry, orthopedic surgery, social work, clinical nursing, research nursing and physical therapy. […] The longer you wait, the more severe the bleeding can become. Delay can increase the amount of damage and the length of treatment. […] If you have any questions, be sure to call the Hemophilia Team.

• #63 Guidelines for Emergency Department : Resources : Hemophilia Foundation of Northern California

  https://www.hemofoundation.org/resources/guidelines-for-emergency-department.html

  For individuals with inhibitors (antibodies to factor VIII or IX), treatment decisions may be more complicated. […] If a patient with hemophilia or other bleeding disorder or the parent of a patient with a bleeding disorder brings clotting factor concentrate with them to the emergency department, allow them to utilize it. […] Emergency departments must have ready access to factor replacement products so that they are available within one hour of the patient’s arrival. […] Factor replacement must be administered intravenously by IV push over 1-2 minutes or per label instructions. […] In any suspected bleeding emergency in which the clotting factor level of a patient with hemophilia is unknown, the factor level should be assumed to be 0%. […] Intramuscular injections, including immunizations, should be avoided whenever possible. […] If you are advised by the hematology staff to go to the ER, take a copy of these MASAC recommendations, and the letter from your hematologist. […] Be sure that a dose of the treatment product is given before any procedure, such as lumbar puncture (LP), IM injection, head scan, or any surgery.

• #64 Nursing Care Plan (NCP) for Hemophilia | Free NURSING.com Courses

  https://nursing.com/lesson/nursing-care-plan-for-hemophilia

  Administer clotting factor replacement therapy as prescribed to manage and prevent bleeding episodes. Monitor levels regularly and adjust the dosage as needed. […] Implement pain management strategies, such as pharmacological interventions and non-pharmacological approaches, to address pain associated with bleeding episodes or joint damage. […] Provide ongoing education to the individual and their family about hemophilia, treatment options, and self-care practices. Include counseling to address psychosocial aspects and promote mental well-being. […] Regularly assess clotting factor levels to ensure that replacement therapy maintains levels within the target range. Adjust treatment plans based on monitoring results. […] Evaluate the frequency and severity of bleeding episodes over time. A reduction in the number of bleeding episodes indicates the effectiveness of the treatment plan.

• #65 Nursing Care Plan (NCP) for Hemophilia | Free NURSING.com Courses

  https://nursing.com/lesson/nursing-care-plan-for-hemophilia

  Administer clotting factor replacement therapy as prescribed to manage and prevent bleeding episodes. Monitor levels regularly and adjust the dosage as needed. […] Implement pain management strategies, such as pharmacological interventions and non-pharmacological approaches, to address pain associated with bleeding episodes or joint damage. […] Provide ongoing education to the individual and their family about hemophilia, treatment options, and self-care practices. Include counseling to address psychosocial aspects and promote mental well-being. […] Regularly assess clotting factor levels to ensure that replacement therapy maintains levels within the target range. Adjust treatment plans based on monitoring results. […] Evaluate the frequency and severity of bleeding episodes over time. A reduction in the number of bleeding episodes indicates the effectiveness of the treatment plan.

• #66 Nurses’ Guide to Bleeding Disorders | Comprehensive Care | NBDF

  https://www.bleeding.org/healthcare-professionals/allied-healthcare/nursing/nurses-guide-to-bleeding-disorders

  The Nurses’ Guide to Bleeding Disorders provides comprehensive information as well as practical ideas to assist nurses at all levels in caring for patients with bleeding disorders. […] Topics covered in the guide include hemophilia and von Willebrand disease therapy, women and bleeding disorders, rare coagulopathies, prophylaxis, venous access devices, emergency room care, orthopedic complications and treatment, hepatitis and HIV/AIDS, inhibitors to factor VIII and factor IX, enhancing development in the child with a bleeding disorder, travel and vacation planning, insurance coverage, and wellness. […] Nurses Guide Hepatitis and Hemophilia Author: Leticia Riley, RN, BSN; Mack Womack, RN, BSN; (Original Author: Cheryl Brower, RN, MSPH) # of Pages: 20 […] Nurses Guide Orthopedic Complications and Treatment Author: Susan Geraghty, RN, MBA # of Pages: 10 […] Nurses Guide Emergency Room Care Author: Leticia Riley, RN, BSN; Mack Womack, RN, BSN; Susan Zappa, RN, CPN; (Original Author: Nancy Roy, RN, MSN, ARNP, FNP) # of Pages: 15 […] Nurses Guide Prophylaxis Author: Joni Osip, RN, MS; Kerry Hansen, RN, BS # of Pages: 6

• #67 Nursing Care Plan (NCP) for Hemophilia | Free NURSING.com Courses

  https://nursing.com/lesson/nursing-care-plan-for-hemophilia

  Administer clotting factor replacement therapy as prescribed to manage and prevent bleeding episodes. Monitor levels regularly and adjust the dosage as needed. […] Implement pain management strategies, such as pharmacological interventions and non-pharmacological approaches, to address pain associated with bleeding episodes or joint damage. […] Provide ongoing education to the individual and their family about hemophilia, treatment options, and self-care practices. Include counseling to address psychosocial aspects and promote mental well-being. […] Regularly assess clotting factor levels to ensure that replacement therapy maintains levels within the target range. Adjust treatment plans based on monitoring results. […] Evaluate the frequency and severity of bleeding episodes over time. A reduction in the number of bleeding episodes indicates the effectiveness of the treatment plan.

• #68 Hemophilia Nursing Diagnosis & Care Plan – NurseStudy.Net

  https://nursestudy.net/hemophilia-nursing-diagnosis/

  Monitor for bleeding signs. Rationale: Early detection allows prompt intervention. Administer factor replacement as prescribed. Rationale: Maintains therapeutic factor levels. Teach injury prevention. Rationale: Reduces bleeding risk. […] The patient will maintain optimal joint function. The patient will participate in appropriate physical activities. The patient will demonstrate proper body mechanics. […] Provide comprehensive education. Rationale: Improves self-management. Demonstrate treatment procedures. Rationale: Ensures proper technique. Review emergency protocols. Rationale: Prepares for complications. […] The patient will maintain the treatment schedule. The patient will utilize support resources. The patient will demonstrate improved compliance. […] The patient will express decreased anxiety. The patient will utilize coping strategies. The patient will participate in support activities.

• #69 Comprehensive Medical Care for Bleeding Disorders | NBDF

  https://www.bleeding.org/healthcare-professionals/guidelines-on-care/comprehensive-medical-care

  The hematologists, nurses, social workers, and physical therapists not only help people with bleeding disorders with their medical care issues, but also lend tremendous emotional support. […] People who go to HTCs will not only find state-of-the art medical care, but also benefit from an experienced, caring staff that takes time to develop comprehensive treatment care plans for patients and families. […] The treatment center not only provides specialty care but can also act as a resource to your regular family physician or dentist. […] The nurse is probably the person you will see most frequently. […] You are an important member of the treatment team. The staff needs your input to develop a plan of care that will ensure your remain healthy, active, and able to live successfully with added challenge of a bleeding disorder.

• #70 Hemophilia A (Factor VIII Deficiency) Treatment & Management: Approach Considerations, Prehospital Care, Emergency Department Care

  https://emedicine.medscape.com/article/779322-treatment

  The treatment of hemophilia may involve prophylaxis, management of bleeding episodes, treatment of factor VIII (FVIII) inhibitors, and treatment and rehabilitation of hemophilia synovitis. Use of factor replacement products and other medications, including pain medications, is typically required. […] Treatment of patients with hemophilia ideally should be provided through a comprehensive hemophilia care center. These centers, which are found in many US cities, follow a multidisciplinary approach, with specialists in hematology, orthopedics, dentistry, and surgery; nurses; physiotherapists; social workers; and related allied health professionals. Patients treated at comprehensive care clinics have been shown to have better access to care, less morbidity, and better overall outcome. […] Ambulatory replacement therapy for bleeding episodes is essential for preventing chronic arthropathy and deformities. Home treatment and infusion by the family or patient is possible in most cases. Prompt and appropriate treatment of hemorrhage is important to prevent long-term complications and disability.

• #71 Hemophilia B (Factor IX Deficiency) Treatment & Management: Approach Considerations, Prehospital Care, Emergency Department Care

  https://emedicine.medscape.com/article/779434-treatment

  The treatment of hemophilia may involve management of hemostasis, management of bleeding episodes, use of factor replacement products and medications, treatment of patients with factor inhibitors, and treatment and rehabilitation of patients with hemophilic synovitis. […] Treatment of patients with hemophilia ideally should be provided through a comprehensive hemophilia care center. These centers follow a multidisciplinary approach, with specialists in hematology, orthopedics, dentistry, and surgery; nurses; physiotherapists; social workers; and related allied health professionals. Patients treated at comprehensive care clinics have been shown to have better access to care, less morbidity, and better overall outcome. […] Ambulatory replacement therapy for bleeding episodes is essential for preventing chronic arthropathy and deformities. Home treatment and infusion by the family or patient is possible in most cases. Prompt and appropriate treatment of hemorrhage is important to prevent long-term complications and disability.

• #72 Hemophilia A (Factor VIII Deficiency) Treatment & Management: Approach Considerations, Prehospital Care, Emergency Department Care

  https://emedicine.medscape.com/article/779322-treatment

  The treatment of hemophilia may involve prophylaxis, management of bleeding episodes, treatment of factor VIII (FVIII) inhibitors, and treatment and rehabilitation of hemophilia synovitis. Use of factor replacement products and other medications, including pain medications, is typically required. […] Treatment of patients with hemophilia ideally should be provided through a comprehensive hemophilia care center. These centers, which are found in many US cities, follow a multidisciplinary approach, with specialists in hematology, orthopedics, dentistry, and surgery; nurses; physiotherapists; social workers; and related allied health professionals. Patients treated at comprehensive care clinics have been shown to have better access to care, less morbidity, and better overall outcome. […] Ambulatory replacement therapy for bleeding episodes is essential for preventing chronic arthropathy and deformities. Home treatment and infusion by the family or patient is possible in most cases. Prompt and appropriate treatment of hemorrhage is important to prevent long-term complications and disability.

• #73 Hemophilia A (Factor VIII Deficiency) Treatment & Management: Approach Considerations, Prehospital Care, Emergency Department Care

  https://emedicine.medscape.com/article/779322-treatment

  The treatment of hemophilia may involve prophylaxis, management of bleeding episodes, treatment of factor VIII (FVIII) inhibitors, and treatment and rehabilitation of hemophilia synovitis. Use of factor replacement products and other medications, including pain medications, is typically required. […] Treatment of patients with hemophilia ideally should be provided through a comprehensive hemophilia care center. These centers, which are found in many US cities, follow a multidisciplinary approach, with specialists in hematology, orthopedics, dentistry, and surgery; nurses; physiotherapists; social workers; and related allied health professionals. Patients treated at comprehensive care clinics have been shown to have better access to care, less morbidity, and better overall outcome. […] Ambulatory replacement therapy for bleeding episodes is essential for preventing chronic arthropathy and deformities. Home treatment and infusion by the family or patient is possible in most cases. Prompt and appropriate treatment of hemorrhage is important to prevent long-term complications and disability.

• #74 Hemophilia and Thrombophilia Care | Rush

  https://www.rush.edu/services/hemophilia-and-thrombophilia-care

  Our highly experienced team helps children and adults with bleeding disorders pursue full, active lives. […] At Rush, you will have a multidisciplinary team of blood disorders specialists in your corner. We’ll work with you to help you understand your condition and gain the confidence you need to manage it. […] We take a holistic approach to treating blood disorders. You or your child will may also work with the following specialties: […] You’ll have access to all the medical, psychological, social and supportive services you need. […] Rush has hematology providers in downtown Chicago, Aurora/Fox Valley and Hoffman Estates. […] Positive patient outcomes and quality of life: Research has found that people with bleeding disorders who go to hemophilia treatment centers like the one at Rush are 40% less likely to be admitted to the hospital for bleeding complications.

• #75 About Comprehensive Hemophilia Care | Vanderbilt Health Nashville, TN

  https://www.vanderbilthealth.com/patient-resource/about-comprehensive-hemophilia-care

  Studies by the Centers for Disease Control (CDC) show that people with hemophilia who receive medical care through a designated hemophilia treatment center (HTC) have a significantly reduced risk of bleeding complications, better school and work attendance and improved emotional health and well-being. […] Comprehensive care is designed to provide an integrated and multidisciplinary approach to patients of all ages and their families. […] The nurse will talk to you about home treatment records, your bleeding history, and any other health problems you might be having. […] If you are ready for home infusions, the nurse will make an appointment for training. […] The nurse will also provide you with any educational materials that you might need. […] The social worker will see you to discuss issues that might be affecting your ability to care for yourself or your child. […] The social worker will also talk to you about insurance, school issues, employment and other psychosocial concerns.

• #76 Treatment of Hemophilia | Hemophilia | CDC

  https://www.cdc.gov/hemophilia/treatment/index.html

  If you have hemophilia, you need treatment so your blood can clot. […] Hemophilia treatment centers specialize in caring for people with bleeding disorders. […] Healthcare providers typically prescribe treatment products for episodic care or prophylactic care. […] People with hemophilia can learn how to perform infusions themselves so that they can stop bleeding episodes and, by performing the infusions on a regular basis, can even prevent most bleeding episodes. […] Good quality medical care from healthcare providers who know a lot about the disorder can help people with hemophilia prevent some serious problems. […] This type of specialty care is at a comprehensive hemophilia treatment center (HTC). […] The team consists of physicians (hematologists or blood specialists), nurses, social workers, physical therapists, and other healthcare providers who are specialized in the care of people with bleeding disorders.

• #77 Comprehensive Medical Care for Bleeding Disorders | NBDF

  https://www.bleeding.org/healthcare-professionals/guidelines-on-care/comprehensive-medical-care

  The hematologists, nurses, social workers, and physical therapists not only help people with bleeding disorders with their medical care issues, but also lend tremendous emotional support. […] People who go to HTCs will not only find state-of-the art medical care, but also benefit from an experienced, caring staff that takes time to develop comprehensive treatment care plans for patients and families. […] The treatment center not only provides specialty care but can also act as a resource to your regular family physician or dentist. […] The nurse is probably the person you will see most frequently. […] You are an important member of the treatment team. The staff needs your input to develop a plan of care that will ensure your remain healthy, active, and able to live successfully with added challenge of a bleeding disorder.

• #78 Center for Bleeding and Clotting Disorders | UC San Diego Health

  https://health.ucsd.edu/care/blood-disorders/hemophilia-thrombosis/

  The Center for Bleeding and Clotting Disorders (CBCD) is an innovative, multidisciplinary program catering to the needs of adult and adolescent patients and their families with hemophilia, von Willebrand Disease, and other bleeding and thrombotic disorders. […] We provide you with personalized, comprehensive services under the directorship of Dr. Annette von Drygalski. […] Home infusion training with factor concentrates. […] Supply and delivery of factor concentrates (through our Total Hemophilia Care homecare program). […] Emergency care for bleeds. […] Inhibitor management. […] Coordination of care with specialists in infectious disease and hepatology (through UC San Diego’s Owen Clinic and Liver Center). […] Support for women with bleeding disorders and heavy menstrual bleeding.

• #79 Center for Bleeding and Clotting Disorders | UC San Diego Health

  https://health.ucsd.edu/care/blood-disorders/hemophilia-thrombosis/

  Social work services including psychological support, financial and insurance counseling for patients and family members. […] Coordination of home factor infusion for dental care and surgical procedures. […] Guided transition of care for adolescents with bleeding and clotting disorders into young adulthood in close partnership with the hemophilia/thrombosis program at Rady Children’s Hospital San Diego. […] Around-the-clock availability of highly trained physicians and nurses. […] The Hemophilia Research Program at UC San Diego Health is directed at advancing new therapies, evaluating novel aspects of hemophilia care, and creating new knowledge about the evolving needs of aging patients with hemophilia. […] Our patients have the opportunity to participate in cutting-edge research and contribute to scientific and medical knowledge in a variety of innovative ways that could not only help them but also help the larger community of affected patients.

• #80 Guidelines for Emergency Department : Resources : Hemophilia Foundation of Northern California

  https://www.hemofoundation.org/resources/guidelines-for-emergency-department.html

  Patients with bleeding disorders who present to an emergency department for care should receive appropriate, expeditious management. […] Individuals with bleeding disorders should be triaged urgently as delays in administering appropriate therapy, such as infusion of factor concentrate, can significantly affect morbidity and mortality. […] Consultation with the patient’s primary provider of bleeding disorder care, in most cases a hematologist, is strongly advised. […] Administration of clotting factor replacement to the patient should not be delayed waiting for a consultation. […] Treatment for a suspected bleeding episode is based on clinical history. […] If the patient or the parent of a patient suspects that occult bleeding is occurring, administer clotting factor replacement.

• #81 Guidelines for Emergency Department : Resources : Hemophilia Foundation of Northern California

  https://www.hemofoundation.org/resources/guidelines-for-emergency-department.html

  Patients with bleeding disorders who present to an emergency department for care should receive appropriate, expeditious management. […] Individuals with bleeding disorders should be triaged urgently as delays in administering appropriate therapy, such as infusion of factor concentrate, can significantly affect morbidity and mortality. […] Consultation with the patient’s primary provider of bleeding disorder care, in most cases a hematologist, is strongly advised. […] Administration of clotting factor replacement to the patient should not be delayed waiting for a consultation. […] Treatment for a suspected bleeding episode is based on clinical history. […] If the patient or the parent of a patient suspects that occult bleeding is occurring, administer clotting factor replacement.

• #82 Guidelines for Emergency Department : Resources : Hemophilia Foundation of Northern California

  https://www.hemofoundation.org/resources/guidelines-for-emergency-department.html

  Patients with bleeding disorders who present to an emergency department for care should receive appropriate, expeditious management. […] Individuals with bleeding disorders should be triaged urgently as delays in administering appropriate therapy, such as infusion of factor concentrate, can significantly affect morbidity and mortality. […] Consultation with the patient’s primary provider of bleeding disorder care, in most cases a hematologist, is strongly advised. […] Administration of clotting factor replacement to the patient should not be delayed waiting for a consultation. […] Treatment for a suspected bleeding episode is based on clinical history. […] If the patient or the parent of a patient suspects that occult bleeding is occurring, administer clotting factor replacement.

• #83 Guidelines for Emergency Department : Resources : Hemophilia Foundation of Northern California

  https://www.hemofoundation.org/resources/guidelines-for-emergency-department.html

  Patients with bleeding disorders who present to an emergency department for care should receive appropriate, expeditious management. […] Individuals with bleeding disorders should be triaged urgently as delays in administering appropriate therapy, such as infusion of factor concentrate, can significantly affect morbidity and mortality. […] Consultation with the patient’s primary provider of bleeding disorder care, in most cases a hematologist, is strongly advised. […] Administration of clotting factor replacement to the patient should not be delayed waiting for a consultation. […] Treatment for a suspected bleeding episode is based on clinical history. […] If the patient or the parent of a patient suspects that occult bleeding is occurring, administer clotting factor replacement.

• #84 Guidelines for Emergency Department : Resources : Hemophilia Foundation of Northern California

  https://www.hemofoundation.org/resources/guidelines-for-emergency-department.html

  Patients with bleeding disorders who present to an emergency department for care should receive appropriate, expeditious management. […] Individuals with bleeding disorders should be triaged urgently as delays in administering appropriate therapy, such as infusion of factor concentrate, can significantly affect morbidity and mortality. […] Consultation with the patient’s primary provider of bleeding disorder care, in most cases a hematologist, is strongly advised. […] Administration of clotting factor replacement to the patient should not be delayed waiting for a consultation. […] Treatment for a suspected bleeding episode is based on clinical history. […] If the patient or the parent of a patient suspects that occult bleeding is occurring, administer clotting factor replacement.

• #85 Guidelines for Emergency Department : Resources : Hemophilia Foundation of Northern California

  https://www.hemofoundation.org/resources/guidelines-for-emergency-department.html

  Patients with bleeding disorders who present to an emergency department for care should receive appropriate, expeditious management. […] Individuals with bleeding disorders should be triaged urgently as delays in administering appropriate therapy, such as infusion of factor concentrate, can significantly affect morbidity and mortality. […] Consultation with the patient’s primary provider of bleeding disorder care, in most cases a hematologist, is strongly advised. […] Administration of clotting factor replacement to the patient should not be delayed waiting for a consultation. […] Treatment for a suspected bleeding episode is based on clinical history. […] If the patient or the parent of a patient suspects that occult bleeding is occurring, administer clotting factor replacement.

• #86 Guidelines for Emergency Department : Resources : Hemophilia Foundation of Northern California

  https://www.hemofoundation.org/resources/guidelines-for-emergency-department.html

  Clotting factor replacement therapy should be given before any diagnostic studies (X-rays, CT scans etc.) are performed to evaluate a suspected bleeding problem, especially in the case of head trauma or suspected intracranial hemorrhage. […] For patients with hemophilia who have illnesses or disorders that necessitate an invasive procedure (lumbar puncture, arterial blood gas, arthrocentesis, etc.) or surgery, factor replacement therapy or bypass therapy to 100% must be administered in the emergency department prior to the planned procedure or surgery. […] Treatment should not be delayed waiting for test results which may take several hours. […] Indications for Factor Replacement Therapy include: 1) Suspected bleeding into a joint or muscle. 2) Any significant injury to the head, neck, mouth or eyes or evidence of bleeding in these areas. 3) Any new or unusual headache, particularly one following trauma. 4) Severe pain or swelling at any site. 5) All open wounds requiring surgical closure, wound adhesive, or steri-strips. 6) History of an accident or trauma that might result in internal bleeding. 7) Any invasive procedure or surgery. 8) Heavy or persistent bleeding from any site. 9) Gastrointestinal bleeding leading to moderate to severe anemia. 10) Acute fractures, dislocations and sprains. 11) Heavy menstrual bleeding leading to moderate to severe anemia or volume instability.

• #87 Guidelines for Emergency Department : Resources : Hemophilia Foundation of Northern California

  https://www.hemofoundation.org/resources/guidelines-for-emergency-department.html

  For individuals with inhibitors (antibodies to factor VIII or IX), treatment decisions may be more complicated. […] If a patient with hemophilia or other bleeding disorder or the parent of a patient with a bleeding disorder brings clotting factor concentrate with them to the emergency department, allow them to utilize it. […] Emergency departments must have ready access to factor replacement products so that they are available within one hour of the patient’s arrival. […] Factor replacement must be administered intravenously by IV push over 1-2 minutes or per label instructions. […] In any suspected bleeding emergency in which the clotting factor level of a patient with hemophilia is unknown, the factor level should be assumed to be 0%. […] Intramuscular injections, including immunizations, should be avoided whenever possible. […] If you are advised by the hematology staff to go to the ER, take a copy of these MASAC recommendations, and the letter from your hematologist. […] Be sure that a dose of the treatment product is given before any procedure, such as lumbar puncture (LP), IM injection, head scan, or any surgery.

• #88 Guidelines for Emergency Department : Resources : Hemophilia Foundation of Northern California

  https://www.hemofoundation.org/resources/guidelines-for-emergency-department.html

  Clotting factor replacement therapy should be given before any diagnostic studies (X-rays, CT scans etc.) are performed to evaluate a suspected bleeding problem, especially in the case of head trauma or suspected intracranial hemorrhage. […] For patients with hemophilia who have illnesses or disorders that necessitate an invasive procedure (lumbar puncture, arterial blood gas, arthrocentesis, etc.) or surgery, factor replacement therapy or bypass therapy to 100% must be administered in the emergency department prior to the planned procedure or surgery. […] Treatment should not be delayed waiting for test results which may take several hours. […] Indications for Factor Replacement Therapy include: 1) Suspected bleeding into a joint or muscle. 2) Any significant injury to the head, neck, mouth or eyes or evidence of bleeding in these areas. 3) Any new or unusual headache, particularly one following trauma. 4) Severe pain or swelling at any site. 5) All open wounds requiring surgical closure, wound adhesive, or steri-strips. 6) History of an accident or trauma that might result in internal bleeding. 7) Any invasive procedure or surgery. 8) Heavy or persistent bleeding from any site. 9) Gastrointestinal bleeding leading to moderate to severe anemia. 10) Acute fractures, dislocations and sprains. 11) Heavy menstrual bleeding leading to moderate to severe anemia or volume instability.

• #89 Hemophilia A (Factor VIII Deficiency) Treatment & Management: Approach Considerations, Prehospital Care, Emergency Department Care

  https://emedicine.medscape.com/article/779322-treatment

  In most developed countries with access to recombinant product, prophylaxis is primary (ie, therapy is started in patients as young as 1 y and continues into adolescence). A cost-benefit analysis indicates that this approach reduces overall factor use and significantly reduces morbidity. […] For prophylaxis, dosing is designed to maintain trough levels of 2% or higher. This usually requires the administration of FVIII three times per week. Individualized therapy (ie, tailored prophylaxis) has been also used with success; the best approach has yet to be determined. […] The treatment of patients with inhibitors of FVIII is difficult. […] In patients who develop synovitis from joint bleeds, injection of radioisotopes into the joint to ablate the synovium (radiosynovectomy) can be used to decrease bleeding, slow progression of cartilage and bone damage, and prevent arthropathy.

• #90 Caring for patients with inherited bleeding disorders | Nursing Times

  https://www.nursingtimes.net/haematology/caring-for-patients-with-inherited-bleeding-disorders-04-09-2020/

  Complications: Approximately 30% of patients with severe haemophilia A develop antibodies to factor VIII, known as inhibitors, following treatment. In haemophilia B, it is approximately 2%. Although rare, inhibitors in haemophilia B can be challenging to deal with as patients can develop an anaphylactic reaction. […] Blood tests: Patients with a bleeding disorder may require regular blood tests to determine their factor levels to ensure they are receiving treatment at an optimal dose and frequency. It is important to ensure that the sample bottles are appropriately filled as over and under-filled tubes cannot be processed. In my experience, coagulation laboratories have a very close relationship with the haemophilia team, facilitating a quick turnaround of clotting results. […] Surgery: The key to successful surgical management of a patient with a bleeding disorder is a multidisciplinary approach. With careful planning, most surgical and invasive procedures can be carried out safely in people with bleeding disorders. The haemostasis surgical plan includes arrangements for escalation in the event of unexpected bleeding problems and is communicated to all staff involved in the patients care throughout the surgical journey. Additionally, please seek advice from the haemophilia team regarding post-operative thromboprophylaxis requirements. Guidelines regarding pre-operative analgesia and factor replacement should be followed, for example, medications which have an anti-platelet effect should be avoided. Some patients with inherited bleeding disorders are deemed as being at risk of variant Creutzfeldt-Jakob disease (vCJD) because they might historically have been treated with contaminated plasma products. National guidelines recommend a number of infection control measures including using single-use surgical instruments and performing the procedure at the end of a surgical list.

• #91 Caring for patients with inherited bleeding disorders | Nursing Times

  https://www.nursingtimes.net/haematology/caring-for-patients-with-inherited-bleeding-disorders-04-09-2020/

  Complications: Approximately 30% of patients with severe haemophilia A develop antibodies to factor VIII, known as inhibitors, following treatment. In haemophilia B, it is approximately 2%. Although rare, inhibitors in haemophilia B can be challenging to deal with as patients can develop an anaphylactic reaction. […] Blood tests: Patients with a bleeding disorder may require regular blood tests to determine their factor levels to ensure they are receiving treatment at an optimal dose and frequency. It is important to ensure that the sample bottles are appropriately filled as over and under-filled tubes cannot be processed. In my experience, coagulation laboratories have a very close relationship with the haemophilia team, facilitating a quick turnaround of clotting results. […] Surgery: The key to successful surgical management of a patient with a bleeding disorder is a multidisciplinary approach. With careful planning, most surgical and invasive procedures can be carried out safely in people with bleeding disorders. The haemostasis surgical plan includes arrangements for escalation in the event of unexpected bleeding problems and is communicated to all staff involved in the patients care throughout the surgical journey. Additionally, please seek advice from the haemophilia team regarding post-operative thromboprophylaxis requirements. Guidelines regarding pre-operative analgesia and factor replacement should be followed, for example, medications which have an anti-platelet effect should be avoided. Some patients with inherited bleeding disorders are deemed as being at risk of variant Creutzfeldt-Jakob disease (vCJD) because they might historically have been treated with contaminated plasma products. National guidelines recommend a number of infection control measures including using single-use surgical instruments and performing the procedure at the end of a surgical list.

• #92 Hemophilia B (Factor IX Deficiency) Treatment & Management: Approach Considerations, Prehospital Care, Emergency Department Care

  https://emedicine.medscape.com/article/779434-treatment

  The main goal of prophylactic treatment is to prevent bleeding symptoms and organ damage, in particular to joints. Hemophilic arthropathy that results from recurrent or target joint bleeding can be prevented by this method. […] Consensus recommendations on the management of hemophilia B by an international group of hematology experts include the following with regard to prophylaxis and factor treatment choice: Consider FIX prophylaxis in all people with severe hemophilia B, including those who have non-severe disease according to their basal FIX levels but with a severe bleeding phenotype; initiate prophylaxis as early as possible (ie, before the onset of joint bleeding) and do not interrupt it. […] Pain management can be challenging in patients with severe hemophilia. Acute bleeding in joints and soft tissues can be extremely painful. This requires immediate analgesic relief.

• #93 Haemophilia: diagnosis, management and nursing care of patients | Nursing Times

  https://www.nursingtimes.net/haematology/haemophilia-diagnosis-management-and-nursing-care-of-patients-13-09-2021/

  Nurses and other health professionals should liaise with the specialist haemophilia team about all aspects of care for patients with haemophilia, regardless of the care setting or how patients present. […] In haemophilia, bleeding is often internal, and nurses need to be able to recognise the signs. […] Patients with haemophilia who are on surgical wards will need infusions to prevent potentially life-threatening bleeds. […] Pain management is also more complex non-steroid anti-inflammatory drugs cannot be used because they make bleeding worse. […] Toddlers and young children presenting with problems in weight-bearing joints can be in severe pain; if a child is distressed, screaming and unwilling to use the affected limb, nurses should not attempt to pull it straight to demonstrate mobility.

• #94 Caring for patients with inherited bleeding disorders | Nursing Times

  https://www.nursingtimes.net/haematology/caring-for-patients-with-inherited-bleeding-disorders-04-09-2020/

  Complications: Approximately 30% of patients with severe haemophilia A develop antibodies to factor VIII, known as inhibitors, following treatment. In haemophilia B, it is approximately 2%. Although rare, inhibitors in haemophilia B can be challenging to deal with as patients can develop an anaphylactic reaction. […] Blood tests: Patients with a bleeding disorder may require regular blood tests to determine their factor levels to ensure they are receiving treatment at an optimal dose and frequency. It is important to ensure that the sample bottles are appropriately filled as over and under-filled tubes cannot be processed. In my experience, coagulation laboratories have a very close relationship with the haemophilia team, facilitating a quick turnaround of clotting results. […] Surgery: The key to successful surgical management of a patient with a bleeding disorder is a multidisciplinary approach. With careful planning, most surgical and invasive procedures can be carried out safely in people with bleeding disorders. The haemostasis surgical plan includes arrangements for escalation in the event of unexpected bleeding problems and is communicated to all staff involved in the patients care throughout the surgical journey. Additionally, please seek advice from the haemophilia team regarding post-operative thromboprophylaxis requirements. Guidelines regarding pre-operative analgesia and factor replacement should be followed, for example, medications which have an anti-platelet effect should be avoided. Some patients with inherited bleeding disorders are deemed as being at risk of variant Creutzfeldt-Jakob disease (vCJD) because they might historically have been treated with contaminated plasma products. National guidelines recommend a number of infection control measures including using single-use surgical instruments and performing the procedure at the end of a surgical list.

• #95 Caring for patients with inherited bleeding disorders | Nursing Times

  https://www.nursingtimes.net/haematology/caring-for-patients-with-inherited-bleeding-disorders-04-09-2020/

  Complications: Approximately 30% of patients with severe haemophilia A develop antibodies to factor VIII, known as inhibitors, following treatment. In haemophilia B, it is approximately 2%. Although rare, inhibitors in haemophilia B can be challenging to deal with as patients can develop an anaphylactic reaction. […] Blood tests: Patients with a bleeding disorder may require regular blood tests to determine their factor levels to ensure they are receiving treatment at an optimal dose and frequency. It is important to ensure that the sample bottles are appropriately filled as over and under-filled tubes cannot be processed. In my experience, coagulation laboratories have a very close relationship with the haemophilia team, facilitating a quick turnaround of clotting results. […] Surgery: The key to successful surgical management of a patient with a bleeding disorder is a multidisciplinary approach. With careful planning, most surgical and invasive procedures can be carried out safely in people with bleeding disorders. The haemostasis surgical plan includes arrangements for escalation in the event of unexpected bleeding problems and is communicated to all staff involved in the patients care throughout the surgical journey. Additionally, please seek advice from the haemophilia team regarding post-operative thromboprophylaxis requirements. Guidelines regarding pre-operative analgesia and factor replacement should be followed, for example, medications which have an anti-platelet effect should be avoided. Some patients with inherited bleeding disorders are deemed as being at risk of variant Creutzfeldt-Jakob disease (vCJD) because they might historically have been treated with contaminated plasma products. National guidelines recommend a number of infection control measures including using single-use surgical instruments and performing the procedure at the end of a surgical list.

• #96 Improving mental well-being in the hemophilia communityEnvelope icon

  https://hemophilianewstoday.com/columns/improving-mental-well-being-hemophilia-community/

  I’ve lived with bleeding disorders all my life. I’ve infused at home, navigated emergency rooms, and advocated for better care for myself and others. But when I was invited to participate in a global hackathon on mental well-being in the hemophilia community, I was struck by something that often goes unsaid: how rarely we talk about the emotional burden of living with a bleeding disorder. […] Mental health is still stigmatized, even in communities like ours that are built around shared challenges. And when you have a condition that’s already misunderstood, it can feel risky to add one more layer of vulnerability. […] The project was grounded in sobering data. A global survey conducted by Sanofi and The Harris Poll found that more than half of people with hemophilia rated their mental health as fair or poor. Yet 71% of those same people told their doctors they were coping just fine. That disconnect hit me hard. I’ve done the same: put on a brave face during appointments, even when I was quietly struggling.

• #97 Learn About The Latest Developments In Hemophilia Care

  https://hemhorizonhcp.com/

  The modern age of hemophilia care began in the 1970s with the availability of factor replacement concentrates and continues to evolve to this day. Hemophilia, a bleeding disorder with a history of persistent treatment burden, is characterized by deficiencies in specific clotting factors that prevent blood from clotting naturally (hemostasis). Normal clotting is impeded in hemophilia because the genes that encode for the necessary clotting factor VIII or factor IX contain mutations that result in deficient or nonfunctioning factor proteins. For people living with severe or moderate hemophilia, bleeding can occur spontaneously or from an injury. If left untreated, internal bleeding into organs may affect a patient’s life expectancy. Today, the landscape of treatment continues to evolve with innovations designed to reduce treatment burden, addressing the needs of patients throughout their treatment journey. Current access to prophylaxis and comprehensive care have made life expectancy for patients with hemophilia close to that of the general population. As patients are living longer and treatment goals have grown more ambitious, the standard of care has continued to evolve. Today’s patients seek the freedom to participate fully in daily living activities with a reduced treatment burden. Treatment limitations and patient burden persist despite the availability of current standard-of-care options. Bleeds, joint disease, and mobility problems still occur in patients with hemophilia. Joint damage can occur despite prophylaxis, indicating that patients undergoing prophylactic therapy may still experience clinical and subclinical bleeding. The development of inhibitors continues to complicate management and limit treatment options. IV access issues, injection site reactions, weight-based dosing, treatment preparation, and the timing and frequency of injections can all contribute to the burdens for patients and caregivers. Emerging approaches may offer improved pharmacokinetics with simpler dosing administration, which in turn may help reduce treatment burden. Emerging treatment modalities have the potential to impact mode and frequency of administration, risk and management of inhibitors, and therapeutic monitoring, all key aspects of hemophilia care. Gene therapy for hemophilia involves the use of a viral vector to deliver the gene to liver cells. Liver cells are targeted because they are responsible for synthesis of factor VIII and factor IX. Gene therapy aims to meet key unmet goals of achieving steady-state clotting factor levels after a one-time infusion, which may offer long-term bleed protection. This liver-directed gene therapy is being studied for its potential for sustained therapeutic benefit, although it is not yet clear how long the effects of gene therapy may last. An agent that inhibits TFPI has the potential to block TFPI’s normal anticoagulation properties. TFPI inhibition offers the potential to achieve clotting even when factor VIII or factor IX is absent or deficient. An agent that reduces antithrombin production has the potential to promote sufficient thrombin production to rebalance the process and may allow a clot to form when factor VIII or factor IX is absent or deficient. When factor VIII or factor IX is absent or deficient, an agent that inhibits APC activity may allow for sufficient thrombin generation to rebalance the coagulation cascade and allow a clot to form.

• #98 Learn About The Latest Developments In Hemophilia Care

  https://hemhorizonhcp.com/

  The modern age of hemophilia care began in the 1970s with the availability of factor replacement concentrates and continues to evolve to this day. Hemophilia, a bleeding disorder with a history of persistent treatment burden, is characterized by deficiencies in specific clotting factors that prevent blood from clotting naturally (hemostasis). Normal clotting is impeded in hemophilia because the genes that encode for the necessary clotting factor VIII or factor IX contain mutations that result in deficient or nonfunctioning factor proteins. For people living with severe or moderate hemophilia, bleeding can occur spontaneously or from an injury. If left untreated, internal bleeding into organs may affect a patient’s life expectancy. Today, the landscape of treatment continues to evolve with innovations designed to reduce treatment burden, addressing the needs of patients throughout their treatment journey. Current access to prophylaxis and comprehensive care have made life expectancy for patients with hemophilia close to that of the general population. As patients are living longer and treatment goals have grown more ambitious, the standard of care has continued to evolve. Today’s patients seek the freedom to participate fully in daily living activities with a reduced treatment burden. Treatment limitations and patient burden persist despite the availability of current standard-of-care options. Bleeds, joint disease, and mobility problems still occur in patients with hemophilia. Joint damage can occur despite prophylaxis, indicating that patients undergoing prophylactic therapy may still experience clinical and subclinical bleeding. The development of inhibitors continues to complicate management and limit treatment options. IV access issues, injection site reactions, weight-based dosing, treatment preparation, and the timing and frequency of injections can all contribute to the burdens for patients and caregivers. Emerging approaches may offer improved pharmacokinetics with simpler dosing administration, which in turn may help reduce treatment burden. Emerging treatment modalities have the potential to impact mode and frequency of administration, risk and management of inhibitors, and therapeutic monitoring, all key aspects of hemophilia care. Gene therapy for hemophilia involves the use of a viral vector to deliver the gene to liver cells. Liver cells are targeted because they are responsible for synthesis of factor VIII and factor IX. Gene therapy aims to meet key unmet goals of achieving steady-state clotting factor levels after a one-time infusion, which may offer long-term bleed protection. This liver-directed gene therapy is being studied for its potential for sustained therapeutic benefit, although it is not yet clear how long the effects of gene therapy may last. An agent that inhibits TFPI has the potential to block TFPI’s normal anticoagulation properties. TFPI inhibition offers the potential to achieve clotting even when factor VIII or factor IX is absent or deficient. An agent that reduces antithrombin production has the potential to promote sufficient thrombin production to rebalance the process and may allow a clot to form when factor VIII or factor IX is absent or deficient. When factor VIII or factor IX is absent or deficient, an agent that inhibits APC activity may allow for sufficient thrombin generation to rebalance the coagulation cascade and allow a clot to form.

• #99 Learn About The Latest Developments In Hemophilia Care

  https://hemhorizonhcp.com/

  The modern age of hemophilia care began in the 1970s with the availability of factor replacement concentrates and continues to evolve to this day. Hemophilia, a bleeding disorder with a history of persistent treatment burden, is characterized by deficiencies in specific clotting factors that prevent blood from clotting naturally (hemostasis). Normal clotting is impeded in hemophilia because the genes that encode for the necessary clotting factor VIII or factor IX contain mutations that result in deficient or nonfunctioning factor proteins. For people living with severe or moderate hemophilia, bleeding can occur spontaneously or from an injury. If left untreated, internal bleeding into organs may affect a patient’s life expectancy. Today, the landscape of treatment continues to evolve with innovations designed to reduce treatment burden, addressing the needs of patients throughout their treatment journey. Current access to prophylaxis and comprehensive care have made life expectancy for patients with hemophilia close to that of the general population. As patients are living longer and treatment goals have grown more ambitious, the standard of care has continued to evolve. Today’s patients seek the freedom to participate fully in daily living activities with a reduced treatment burden. Treatment limitations and patient burden persist despite the availability of current standard-of-care options. Bleeds, joint disease, and mobility problems still occur in patients with hemophilia. Joint damage can occur despite prophylaxis, indicating that patients undergoing prophylactic therapy may still experience clinical and subclinical bleeding. The development of inhibitors continues to complicate management and limit treatment options. IV access issues, injection site reactions, weight-based dosing, treatment preparation, and the timing and frequency of injections can all contribute to the burdens for patients and caregivers. Emerging approaches may offer improved pharmacokinetics with simpler dosing administration, which in turn may help reduce treatment burden. Emerging treatment modalities have the potential to impact mode and frequency of administration, risk and management of inhibitors, and therapeutic monitoring, all key aspects of hemophilia care. Gene therapy for hemophilia involves the use of a viral vector to deliver the gene to liver cells. Liver cells are targeted because they are responsible for synthesis of factor VIII and factor IX. Gene therapy aims to meet key unmet goals of achieving steady-state clotting factor levels after a one-time infusion, which may offer long-term bleed protection. This liver-directed gene therapy is being studied for its potential for sustained therapeutic benefit, although it is not yet clear how long the effects of gene therapy may last. An agent that inhibits TFPI has the potential to block TFPI’s normal anticoagulation properties. TFPI inhibition offers the potential to achieve clotting even when factor VIII or factor IX is absent or deficient. An agent that reduces antithrombin production has the potential to promote sufficient thrombin production to rebalance the process and may allow a clot to form when factor VIII or factor IX is absent or deficient. When factor VIII or factor IX is absent or deficient, an agent that inhibits APC activity may allow for sufficient thrombin generation to rebalance the coagulation cascade and allow a clot to form.

• #100 Learn About The Latest Developments In Hemophilia Care

  https://hemhorizonhcp.com/

  The modern age of hemophilia care began in the 1970s with the availability of factor replacement concentrates and continues to evolve to this day. Hemophilia, a bleeding disorder with a history of persistent treatment burden, is characterized by deficiencies in specific clotting factors that prevent blood from clotting naturally (hemostasis). Normal clotting is impeded in hemophilia because the genes that encode for the necessary clotting factor VIII or factor IX contain mutations that result in deficient or nonfunctioning factor proteins. For people living with severe or moderate hemophilia, bleeding can occur spontaneously or from an injury. If left untreated, internal bleeding into organs may affect a patient’s life expectancy. Today, the landscape of treatment continues to evolve with innovations designed to reduce treatment burden, addressing the needs of patients throughout their treatment journey. Current access to prophylaxis and comprehensive care have made life expectancy for patients with hemophilia close to that of the general population. As patients are living longer and treatment goals have grown more ambitious, the standard of care has continued to evolve. Today’s patients seek the freedom to participate fully in daily living activities with a reduced treatment burden. Treatment limitations and patient burden persist despite the availability of current standard-of-care options. Bleeds, joint disease, and mobility problems still occur in patients with hemophilia. Joint damage can occur despite prophylaxis, indicating that patients undergoing prophylactic therapy may still experience clinical and subclinical bleeding. The development of inhibitors continues to complicate management and limit treatment options. IV access issues, injection site reactions, weight-based dosing, treatment preparation, and the timing and frequency of injections can all contribute to the burdens for patients and caregivers. Emerging approaches may offer improved pharmacokinetics with simpler dosing administration, which in turn may help reduce treatment burden. Emerging treatment modalities have the potential to impact mode and frequency of administration, risk and management of inhibitors, and therapeutic monitoring, all key aspects of hemophilia care. Gene therapy for hemophilia involves the use of a viral vector to deliver the gene to liver cells. Liver cells are targeted because they are responsible for synthesis of factor VIII and factor IX. Gene therapy aims to meet key unmet goals of achieving steady-state clotting factor levels after a one-time infusion, which may offer long-term bleed protection. This liver-directed gene therapy is being studied for its potential for sustained therapeutic benefit, although it is not yet clear how long the effects of gene therapy may last. An agent that inhibits TFPI has the potential to block TFPI’s normal anticoagulation properties. TFPI inhibition offers the potential to achieve clotting even when factor VIII or factor IX is absent or deficient. An agent that reduces antithrombin production has the potential to promote sufficient thrombin production to rebalance the process and may allow a clot to form when factor VIII or factor IX is absent or deficient. When factor VIII or factor IX is absent or deficient, an agent that inhibits APC activity may allow for sufficient thrombin generation to rebalance the coagulation cascade and allow a clot to form.

• #101 Learn About The Latest Developments In Hemophilia Care

  https://hemhorizonhcp.com/

  The modern age of hemophilia care began in the 1970s with the availability of factor replacement concentrates and continues to evolve to this day. Hemophilia, a bleeding disorder with a history of persistent treatment burden, is characterized by deficiencies in specific clotting factors that prevent blood from clotting naturally (hemostasis). Normal clotting is impeded in hemophilia because the genes that encode for the necessary clotting factor VIII or factor IX contain mutations that result in deficient or nonfunctioning factor proteins. For people living with severe or moderate hemophilia, bleeding can occur spontaneously or from an injury. If left untreated, internal bleeding into organs may affect a patient’s life expectancy. Today, the landscape of treatment continues to evolve with innovations designed to reduce treatment burden, addressing the needs of patients throughout their treatment journey. Current access to prophylaxis and comprehensive care have made life expectancy for patients with hemophilia close to that of the general population. As patients are living longer and treatment goals have grown more ambitious, the standard of care has continued to evolve. Today’s patients seek the freedom to participate fully in daily living activities with a reduced treatment burden. Treatment limitations and patient burden persist despite the availability of current standard-of-care options. Bleeds, joint disease, and mobility problems still occur in patients with hemophilia. Joint damage can occur despite prophylaxis, indicating that patients undergoing prophylactic therapy may still experience clinical and subclinical bleeding. The development of inhibitors continues to complicate management and limit treatment options. IV access issues, injection site reactions, weight-based dosing, treatment preparation, and the timing and frequency of injections can all contribute to the burdens for patients and caregivers. Emerging approaches may offer improved pharmacokinetics with simpler dosing administration, which in turn may help reduce treatment burden. Emerging treatment modalities have the potential to impact mode and frequency of administration, risk and management of inhibitors, and therapeutic monitoring, all key aspects of hemophilia care. Gene therapy for hemophilia involves the use of a viral vector to deliver the gene to liver cells. Liver cells are targeted because they are responsible for synthesis of factor VIII and factor IX. Gene therapy aims to meet key unmet goals of achieving steady-state clotting factor levels after a one-time infusion, which may offer long-term bleed protection. This liver-directed gene therapy is being studied for its potential for sustained therapeutic benefit, although it is not yet clear how long the effects of gene therapy may last. An agent that inhibits TFPI has the potential to block TFPI’s normal anticoagulation properties. TFPI inhibition offers the potential to achieve clotting even when factor VIII or factor IX is absent or deficient. An agent that reduces antithrombin production has the potential to promote sufficient thrombin production to rebalance the process and may allow a clot to form when factor VIII or factor IX is absent or deficient. When factor VIII or factor IX is absent or deficient, an agent that inhibits APC activity may allow for sufficient thrombin generation to rebalance the coagulation cascade and allow a clot to form.

• #102 Learn About The Latest Developments In Hemophilia Care

  https://hemhorizonhcp.com/

  The modern age of hemophilia care began in the 1970s with the availability of factor replacement concentrates and continues to evolve to this day. Hemophilia, a bleeding disorder with a history of persistent treatment burden, is characterized by deficiencies in specific clotting factors that prevent blood from clotting naturally (hemostasis). Normal clotting is impeded in hemophilia because the genes that encode for the necessary clotting factor VIII or factor IX contain mutations that result in deficient or nonfunctioning factor proteins. For people living with severe or moderate hemophilia, bleeding can occur spontaneously or from an injury. If left untreated, internal bleeding into organs may affect a patient’s life expectancy. Today, the landscape of treatment continues to evolve with innovations designed to reduce treatment burden, addressing the needs of patients throughout their treatment journey. Current access to prophylaxis and comprehensive care have made life expectancy for patients with hemophilia close to that of the general population. As patients are living longer and treatment goals have grown more ambitious, the standard of care has continued to evolve. Today’s patients seek the freedom to participate fully in daily living activities with a reduced treatment burden. Treatment limitations and patient burden persist despite the availability of current standard-of-care options. Bleeds, joint disease, and mobility problems still occur in patients with hemophilia. Joint damage can occur despite prophylaxis, indicating that patients undergoing prophylactic therapy may still experience clinical and subclinical bleeding. The development of inhibitors continues to complicate management and limit treatment options. IV access issues, injection site reactions, weight-based dosing, treatment preparation, and the timing and frequency of injections can all contribute to the burdens for patients and caregivers. Emerging approaches may offer improved pharmacokinetics with simpler dosing administration, which in turn may help reduce treatment burden. Emerging treatment modalities have the potential to impact mode and frequency of administration, risk and management of inhibitors, and therapeutic monitoring, all key aspects of hemophilia care. Gene therapy for hemophilia involves the use of a viral vector to deliver the gene to liver cells. Liver cells are targeted because they are responsible for synthesis of factor VIII and factor IX. Gene therapy aims to meet key unmet goals of achieving steady-state clotting factor levels after a one-time infusion, which may offer long-term bleed protection. This liver-directed gene therapy is being studied for its potential for sustained therapeutic benefit, although it is not yet clear how long the effects of gene therapy may last. An agent that inhibits TFPI has the potential to block TFPI’s normal anticoagulation properties. TFPI inhibition offers the potential to achieve clotting even when factor VIII or factor IX is absent or deficient. An agent that reduces antithrombin production has the potential to promote sufficient thrombin production to rebalance the process and may allow a clot to form when factor VIII or factor IX is absent or deficient. When factor VIII or factor IX is absent or deficient, an agent that inhibits APC activity may allow for sufficient thrombin generation to rebalance the coagulation cascade and allow a clot to form.

• #103 Treatment of Hemophilia | Hemophilia | CDC

  https://www.cdc.gov/hemophilia/treatment/index.html

  Hemlibra works by replacing the function of factor VIII, rather than replacing the missing clotting factor VIII directly. […] DDAVP and Stimate are medications that are similar to a hormone that occurs naturally in the body. […] Amicar is a medication that can be given through a vein or by mouth (as a pill or a liquid). […] Roctavian is a gene therapy that was approved by the FDA on June 29, 2023, for the treatment of severe hemophilia A (factor VIII deficiency) in adults. […] Hemgenix is a gene therapy that was approved by the FDA on November 22, 2022, for the treatment of hemophilia B (factor IX deficiency) in adults.

• #104 Treatment of Hemophilia | Hemophilia | CDC

  https://www.cdc.gov/hemophilia/treatment/index.html

  Hemlibra works by replacing the function of factor VIII, rather than replacing the missing clotting factor VIII directly. […] DDAVP and Stimate are medications that are similar to a hormone that occurs naturally in the body. […] Amicar is a medication that can be given through a vein or by mouth (as a pill or a liquid). […] Roctavian is a gene therapy that was approved by the FDA on June 29, 2023, for the treatment of severe hemophilia A (factor VIII deficiency) in adults. […] Hemgenix is a gene therapy that was approved by the FDA on November 22, 2022, for the treatment of hemophilia B (factor IX deficiency) in adults.

• #105 Multiyear Follow-up Study Shows Positive Results for Hemophilia B Gene Therapy

  https://www.managedhealthcareexecutive.com/view/multiyear-follow-up-study-shows-positive-results-for-hemophilia-b-gene-therapy

  Results of a follow-up study of people with hemophilia B who had been treated with Beqvez (fidanacogene elaparvovec), a gene therapy, showed sustained factor IX activity over three to six years, and during that period, only low-grade adverse effects. […] Current treatment involves recurrent intravenous infusions of various forms of factor IX to control bleeding episodes. […] As a one-time treatment, Beqvez is indicated for the treatment of adults with moderate to severe hemophilia B who currently use factor IX prophylaxis therapy or have current or historical life-threatening hemorrhage or have repeated, serious spontaneous bleeding episodes and do not have neutralizing antibodies to adeno-associated viral vector capsid as detected by an FDA-approved test. […] The low-grade adverse events were largely observed in the first year post-infusion.

• #106 Haemophilia: diagnosis, management and nursing care of patients | Nursing Times

  https://www.nursingtimes.net/haematology/haemophilia-diagnosis-management-and-nursing-care-of-patients-13-09-2021/

  Haemophilia is a lifelong disorder causing recurrent and spontaneous bleeds. […] Advances in the treatment and care of haemophilia have transformed this rare bleeding disorder from a fatal disease into a long-term condition, with which people are increasingly living into old age. […] All UK patients diagnosed with haemophilia should be registered with a haemophilia comprehensive care centre that provides core multidisciplinary care from doctors, nurses, physiotherapists, psychosocial carers and coagulation laboratory staff, who liaise with non-haemophilia specialists including dentists and surgeons when necessary. […] Clinical nurse specialists in haemophilia practice at advanced levels have a pivotal role in comprehensive care as part of the multidisciplinary team, including promoting evidence-based care, advocacy and self-management skills for people with haemophilia.

• #107 Haemophilia: diagnosis, management and nursing care of patients | Nursing Times

  https://www.nursingtimes.net/haematology/haemophilia-diagnosis-management-and-nursing-care-of-patients-13-09-2021/

  Nurses and other health professionals should liaise with the specialist haemophilia team about all aspects of care for patients with haemophilia, regardless of the care setting or how patients present. […] In haemophilia, bleeding is often internal, and nurses need to be able to recognise the signs. […] Patients with haemophilia who are on surgical wards will need infusions to prevent potentially life-threatening bleeds. […] Pain management is also more complex non-steroid anti-inflammatory drugs cannot be used because they make bleeding worse. […] Toddlers and young children presenting with problems in weight-bearing joints can be in severe pain; if a child is distressed, screaming and unwilling to use the affected limb, nurses should not attempt to pull it straight to demonstrate mobility.

• #108 Caring for patients with inherited bleeding disorders | Nursing Times

  https://www.nursingtimes.net/haematology/caring-for-patients-with-inherited-bleeding-disorders-04-09-2020/

  The haemophilia specialist nurses will visit patients when they are on a ward, sometimes to take bloods or teach patients to self-infuse, and other times simply to offer support. We can also advise and support the ward/department team, for example, demonstrate how to prepare the product before administration, and can help with discharge planning. We also provide informal teaching sessions and resources. The Bleeding Disorders Academy is a valuable resource for nurses, particularly for those new to bleeding disorders.

• #109 What every nurse should know about hemophilia

  https://www.myamericannurse.com/what-every-nurse-should-know-about-hemophilia/

  Until recently, many people with hemophilia suffered joint damage due to recurring internal joint bleeds. […] Hormone therapy (typically oral contraception) is used to treat heavy menses in hemophilia carriers or patients with other bleeding disorders. […] Nursing considerations for hospital patients with hemophilia vary with the reason for admission. […] Monitor the patient for signs and symptoms of bleeding. […] Surgical patients with hemophilia require close monitoring, factor administration before the procedure, and consideration of deep vein thrombosis prophylaxis. […] Many patients with hemophilia and their families receive comprehensive care through a hemophilia treatment center thats part of a national network of family-centered hemophilia treatment centers. […] Most children and many adults with severe hemophilia treat themselves prophylactically with factor infusions, infusing factor three times weekly to keep enough in their blood to prevent bleeding and joint damage. […] Having accurate, up-to-date information about signs, symptoms, treatments, and nursing implications of bleeding disorders helps ensure they receive proper care and gives them confidence in their care providers.

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