Materiały źródłowe

• #1 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 decreased risk for injury from possible bleeding through appropriate prophylactic measures.

• #2 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. […] 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.

• #3 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.

• #4 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.

• #5 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.

• #6 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. […] 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, thus decreasing overall joint deterioration and improving patients’ 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 B without inhibitors be treated prophylactically rather than through episodic management of bleeding events.

• #7 7 Hemophilia Nursing Care Plans – Nurseslabs

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

  Therapeutic interventions and nursing actions for patients with hemophilia may include: […] 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. This can result in a greater risk of physical injuries, such as bruises, cuts, and joint bleeds, which can lead to pain, limited mobility, and other complications. […] 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. This treatment can also be provided in the home. […] Clients with hemophilia may experience acute pain due to hemarthrosis (bleeding into joints) or traumatic muscle injury, which can occur as a result of injury or trauma. Hemophilia causes a deficiency of blood clotting factors, leading to prolonged bleeding, inflammation, and pain, which can occur spontaneously or after physical activity or injury.

• #8 7 Hemophilia Nursing Care Plans – Nurseslabs

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

  Clients with hemophilia may experience impaired physical mobility due to hemarthrosis (bleeding into joints), pain, and discomfort. Hemarthrosis can lead to swelling and inflammation, which can cause restricted movement and reduced range of motion. Pain and discomfort can also limit mobility and affect a persons ability to engage in physical activities. […] Children with hemophilia and their families may experience compromised family coping due to the chronic and unpredictable nature of the disease, as well as the financial and emotional stressors that may accompany it. The need for frequent medical care, potential complications, and limitations on physical activity can also impact the familys ability to cope with the challenges of the condition. […] Nurses assess and monitor potential complications for children with hemophilia to detect early signs of bleeding, infections, or other complications, allowing for prompt intervention and prevention of further complications, and ensuring the childs safety and well-being by providing timely and appropriate care.

• #9 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 family coping, preventing bleeding, and preventing injury. […] Goals are met as evidenced by decreased pain, maintained optimal physical mobility, effective family coping, 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.

• #10 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. […] 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.

• #11 Treating and Managing a Bleed | The Haemophilia Society

  https://haemophilia.org.uk/bleeding-disorders/haemophilia-a-and-b/treating-managing-a-bleed/

  Different types of treatments are available for managing haemophilia and may vary depending on how severe it is. Prompt, effective treatment and prevention of bleeding significantly reduces the risk of complications and disruptions to school, work and family life. […] Treatment will be needed by everyone with haemophilia if they are bleeding. It will also be necessary to prevent bleeding if they need an operation or have an injury where there is a risk of bleeding. […] Bleeding must be treated as soon as possible. Prompt treatment helps relieve pain quickly, shortens recovery time and reduces the chance of permanent damage. […] Treatment with regular infusions of clotting factor aims to prevent the long-term damage caused by bleeding into joints and muscles. Prophylaxis stops the bleeding that occurs without any obvious injury, often called spontaneous bleeding. It also reduces the risk of bleeding from minor injuries.

• #12 Haemophilia

  https://www.nhs.uk/conditions/haemophilia/

  Haemophilia is usually treated with injections of clotting factor to replace the missing clotting factor in your blood. […] It can also be treated with other medicines including emicizumab, desmopressin and tranexamic acid. […] Haemophilia is treated in 2 main ways: If you have severe haemophilia, you’ll usually need injections up to 3 times a week to prevent bleeding (preventative or prophylactic treatment). You can usually do these injections yourself at home. […] If you have mild or moderate haemophilia, you’ll usually only need treatment to stop or reduce bleeding after it starts (on-demand treatment). You may also need treatment before having surgery or dental work. […] Your haemophilia team will usually show you how to treat bleeding yourself at home, with clotting factor injections or other medicines.

• #13 Treating and Managing a Bleed | The Haemophilia Society

  https://haemophilia.org.uk/bleeding-disorders/haemophilia-a-and-b/treating-managing-a-bleed/

  Most people with severe haemophilia and some with moderate haemophilia learn to treat themselves/their children with clotting factor at home. Home treatment has many advantages: makes regular treatment possible, treatment can be given as soon as possible after the bleeding starts, there is less disruption to home, school and working life, greater independence and control for the parent/person with haemophilia, reduced need for hospital attendance, early treatment reduces recovery time and risk of permanent damage. […] At first, a specialist haemophilia nurse teaches parents how to treat their child. Children will learn how to self-treat in time, and most can do this by the time they reach secondary school.

• #14 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.

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

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

  Increasing evidence associates hemophilia with low bone mineral density; consequently, careful assessment and management of fracture risk are recommended. Regular exercise, fall prevention strategies, and optimization of calcium and vitamin D intake are recommended, along with prophylactic factor replacement therapy in severe hemophilia. […] Patients whose condition and bleeding are stabilized should be transferred to a comprehensive hemophilia care center for further treatment and monitoring. These centers offer a multidisciplinary approach by specialists experienced in hemophilia. […] 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. […] Hemophilic chronic arthropathy is painful. Narcotic agents have been used, but their benefit for long-term therapy is limited by side effects, the development of tolerance, and the risk of addiction. […] Nonsteroidal anti-inflammatory drugs (NSAIDs) can be effective in managing acute and chronic arthritic pain, but significant caution must be used in dose and frequency due to concerns for increased risk of bleeding.

• #16 Haemophilia

  https://www.rch.org.au/clinicalguide/guideline_index/haemophilia/

  For muscle and joint bleeds P.R.I.C.E will limit bleeding and reduce pain. Initiate on arrival […] Children with haemophilia will require frequent venous access, this can be a major fear and stressor, so minimisation of unsuccessful or distressing venepuncture is essential […] Do not use products containing aspirin or NSAIDS (eg ibuprofen, diclofenac) as they may worsen bleeding […] Consider consultation with local paediatric team when assessing any child with haemophilia with bleeding episodes. […] Consider transfer when child requiring care beyond the level of comfort of the local hospital or treating medical team […] Consider discharge when no active bleeding and appropriate follow-up is arranged.

• #17 For parents | The Haemophilia Society

  https://haemophilia.org.uk/support/day-day-living/bleeding-disorders-and-school/for-parents/

  Before school starts, set up a meeting with a few of the key staff, like your childs teacher, the school nurse if there is one, the gym teacher, and someone from administration to make sure they know that a child with a bleeding disorder is under their supervision. […] Though parents often know quite a lot about their childs bleeding disorder, its better to bring in a health care professional, usually a haemophilia nurse, who has expertise in hemophilia/bleeding disorders to talk with teachers and the school nurse. […] It is important that a school care plan is in place for children with bleeding disorders. A school care plan can be obtained from the childs haemophilia centre. […] The plan should include the schools immediate treatment instructions, such as using P.R.I.C.E. (Protection, rest, ice, compression, elevation) for bleeds, and factor infusion to help stop the bleed. […] Your childs healthcare team can also outline possible activities they should avoid or when to use safety tools like a helmet.

• #18 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.

• #19 Haemophilia

  https://www.nhs.uk/conditions/haemophilia/

  If you can, treat any bleeding, including bleeding into joints or muscles, as soon as possible. This will reduce pain, recovery time and the risk of permanent damage. […] Treatment reduces the risk of complications. […] If you or your child have haemophilia, your haemophilia team will be there to support you throughout your treatment.

• #20 Hemophilia – StatPearls – NCBI Bookshelf

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

  Prophylactic treatment has several advantages. […] Prophylaxis is usually started at an early age to prevent and reduce the risk of joint bleeding and the development of hemophilic arthropathy. […] The most common complication of hemophilia is joint bleeding, which can cause significant morbidity and requires early prevention and treatment before chronic degenerative changes set in. […] Pain management remains the most important aspect of controlling the patient’s symptoms. […] Physical activity is of utmost importance in patients with hemophilia to ensure physical fitness, muscle strengthening, maintaining healthy body weight, bone density, and proper muscle development. […] It is also crucial to make sure that patients are compliant with prophylactic treatment schedules to reduce the incidence of joint bleeds and improve the quality of life. […] Hemophilia requires a complete interprofessional effort at an even higher level than most other conditions; when executed properly, this approach can lead to a higher quality of life and life expectancy.

• #21 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. […] 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. […] 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. […] 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.

• #22 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.

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

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

  Provide comprehensive education. Rationale: Improves self-management. Demonstrate treatment procedures. Rationale: Ensures proper technique. Review emergency protocols. Rationale: Prepares for complications. […] The patient will verbalize understanding of the condition, The patient will demonstrate proper treatment techniques, The patient will identify emergencies. […] Develop treatment schedule. Rationale: Promotes adherence. Connect with support resources. Rationale: Provides assistance. Monitor compliance. Rationale: Ensures treatment effectiveness. […] The patient will maintain the treatment schedule, The patient will utilize support resources, The patient will demonstrate improved compliance. […] Provide emotional support. Rationale: Reduces anxiety. Teach coping strategies. Rationale: Improves stress management. Connect with support groups. Rationale: Builds support network. […] The patient will express decreased anxiety, The patient will utilize coping strategies, The patient will participate in support activities.

• #24 The changing role of the specialist haemophilia nurse | Nursing Times

  https://www.nursingtimes.net/haematology/the-changing-role-of-the-specialist-haemophilia-nurse-09-08-2021/

  Specialist nurses may be involved in providing direct clinical care, education, support and self-management for patients and their families. […] Specialist nurses educate patients and caregivers on how to recognise bleeds and other aspects of self-care (including bleed prevention, mitigating risks and managing acute bleeds) and promote concordance with care plans. […] Patient advocacy is another important role. This includes acting as the first contact for patients and families in a crisis and bringing in other team members or agencies as required. […] Haemophilia nursing roles are continuing to develop worldwide and many nurses practise with a high degree of autonomy. […] The transformation of haemophilia from fatal disease to manageable long-term condition is changing the role of the specialist haemophilia nurse. Haemophilia care is evolving and, as a result, the haemophilia nurses role is likely to become less clinically focused and have a greater emphasis on psychosocial support, including motivation, shared decision-making, support and self-advocacy for patients.

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

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

  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. […] 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. […] 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. […] 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.

• #26 Hemophilia (for Parents) | Nemours KidsHealth

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

  Hemophilia treatment centers can provide full care to kids with hemophilia. […] The medical care team for kids with hemophilia usually includes: a nurse who specializes in conditions related to bleeding. […] The main treatment for hemophilia is factor replacement therapy. This gives the body the clotting factor it needs. […] Help your child with hemophilia get the best care and avoid bleeding problems as much as possible. […] Enroll your child in a hemophilia treatment center. […] Work with your care team so you know the exact steps to take if your child is injured or bleeding. […] Most people lead full, healthy lives with careful management of their condition.

• #27 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/

  Children who have severe haemophilia may need a central venous access device for prophylactic infusions; this can pose an infection risk and care may fall under that of the community paediatric team. […] All UK patients diagnosed with haemophilia should be registered with a haemophilia comprehensive care centre that provides core multidisciplinary care.

• #28 The changing role of the specialist haemophilia nurse | Nursing Times

  https://www.nursingtimes.net/haematology/the-changing-role-of-the-specialist-haemophilia-nurse-09-08-2021/

  Haemophilia nurses coordinate and provide comprehensive care. As models of haemophilia care and management evolve, their role is changing to include more psychosocial care […] Specialist haemophilia nurses are pivotal in providing comprehensive care to people with haemophilia and related bleeding disorders, and in coordinating care with other members of the multidisciplinary team across different care settings, including patients own homes. This article focuses on the role of the haemophilia nurse and how this is changing as new models of care and management evolve. […] Comprehensive care aims to promote physical health, psychosocial wellbeing, and quality of life for people with haemophilia, and to reduce morbidity and mortality. […] A fundamental part of comprehensive care is access to a multidisciplinary team, whose core members include a medical director, nurse coordinator, musculoskeletal and psychosocial experts and laboratory specialists. It is recommended that a haemophilia nurse specialist coordinates care provided by the multidisciplinary team, educates patients and their families, provides training for home therapy and other aspects of care, and assesses patients and institutes initial care where appropriate.

• #29 Haemophilia | Better Health Channel

  https://www.betterhealth.vic.gov.au/health/conditionsandtreatments/haemophilia

  Haemophilia is treated by injecting clotting factor concentrate into a vein which either prevents or reduces bleeding. […] Once you have been trained by health professionals, you can do this at home. […] With treatment and support from their haemophilia treatment centre, most people with haemophilia can live relatively normal healthy lives. […] Your local haemophilia treatment centre team will help you learn how to recognize a bleed and deal with it promptly, and how to prevent and rehabilitate injuries and bleeds. […] Specialist haemophilia treatment services have a team of health professionals with expertise in providing treatment and care to people with bleeding disorders including haemophilia. […] The team includes: haematologists: doctors who specialise in blood disorders, haemophilia nurses, social workers or counsellors, physiotherapists, other specialist health professionals.

• #30 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.

• #31 7 Hemophilia Nursing Care Plans – Nurseslabs

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

  Medications used in hemophilia include clotting factor concentrates (Factor VIII for Hemophilia A and Factor IX for Hemophilia B), desmopressin (DDAVP), antifibrinolytics, pain medications, and prophylactic antibiotics, which are administered to replace deficient clotting factors, promote clot stability, manage pain, prevent bleeding, and protect against infections, respectively. […] Laboratory procedures used in hemophilia include activated partial thromboplastin time (aPTT), factor VIII assay (for Hemophilia A), factor IX assay (for Hemophilia B), complete blood count (CBC), von Willebrand factor assay, and bleeding time test, which help assess clotting factor levels, measure clotting times, evaluate platelet function, and differentiate between different types of bleeding disorders.

• #32 Access for everyone: Learning to live with haemophilia

  https://www.octapharma.com/news/patient-stories/2024/access-for-everyone-learning-to-live-with-haemophilia

  Recurrent bleeding into joints is one of the most severe consequences of this disease, as it reduces movement and causes both chronic pain and stiffness. […] Living in an area where knowledge about haemophilia and its diagnosis are uncommon made getting proper care difficult, he remembers. […] When I use human coagulation factor VIII concentrate, it really supports my daily activities and I can do sports any time.

• #33

  https://haematologica.org/article/view/7644

  Repeated joint bleeds cause morphological changes and eventually lead to crippling arthropathy. […] HRQoL measurement may be most important from the payers perspective. […] Real-world evidence (RWE) holds the key to gathering the necessary information about all aspects of care, including clinical and patient-relevant outcomes, therapy profiles in real-life practice, as well as patient preferences. […] To satisfy the demand for higher levels of performance for reimbursement and market access in hemophilia, the ability to generate and access RWE will be essential. […] Randomized trials will remain the cornerstone for demonstrating the efficacy of medical technologies. The key challenge for reimbursement and market access in hemophilia as a rare disease will be to develop RWE approaches through national and international collaboration, thereby reducing the uncertainty for patients, physicians, payers, and other decision makers.

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

  https://emedicine.medscape.com/article/779434-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. […] 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. This goal is achieved by administering factor 2-3 times a week. […] 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.

• #35 Bleeding Disorders • European Haemophilia Consortium (EHC)

  https://www.ehc.eu/bleeding-disorders/

  The EHC strives both at the European and national levels to increase the awareness and advocate for people with bleeding disorders. […] When they have access to adequate treatment, people with bleeding disorders are able to lead normal and fruitful lives. However, limited access to treatment and care can have drastic consequences on the lives of those affected by these conditions. […] The challenges faced by these patients relate to a lack of access to adequate treatment (which is often expensive) and specialised healthcare services (which may be located only in one or two cities in a given country). […] At the moment it is believed that prophylactic treatment is the best solution to prevent joint bleeds. Unfortunately, this is often seen as an expensive course of action. However, the EHC believes that investing in the prevention of joint bleeds is the optimal course of treatment.

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

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

  Assess for signs of active bleeding, Monitor vital signs, Check the skin for bruising, Evaluate joint function, Document bleeding episodes. […] 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 remain free from serious bleeding episodes, The patient will demonstrate the proper factor replacement technique, The patient will identify early signs of bleeding. […] Assess joint function regularly. Rationale: Monitors disease progression. Implement a safe exercise program. Rationale: Maintains joint function. Teach proper body mechanics. Rationale: Prevents injury. […] The patient will maintain optimal joint function, The patient will participate in appropriate physical activities, The patient will demonstrate proper body mechanics.

• #37 What Is Hemophilia?

  https://my.clevelandclinic.org/health/diseases/14083-hemophilia

  Hemophilia happens when you don’t have enough clotting factors to help control how much you bleed. […] Healthcare providers treat this condition by replacing the missing clotting factors. […] Healthcare providers treat hemophilia by boosting clotting factor levels or replacing missing clotting factors (replacement therapy). […] In replacement therapy, you receive human plasma concentrates or lab-made (recombinant) clotting factors. […] If you have severe hemophilia and frequent bleeding episodes, your healthcare provider may prescribe prophylactic factor infusions to prevent bleeding. […] Some people who have replacement therapy develop antibodies (called inhibitors) that attack the clotting factors. Healthcare providers address this with a technique called immune tolerance induction (ITI). ITI involves giving clotting factors given on a daily basis to bring down inhibitor levels. ITI may be a long-term treatment, and some people may need this treatment for months or years. […] At this time, healthcare providers can’t cure hemophilia. They can provide treatment to prevent or reduce hemophilia symptoms. And they can make recommendations for steps you can take to limit hemophilia’s impact on daily life.

• #38

  https://link.springer.com/article/10.1007/s40140-024-00635-y

  The duration and intensity of factor support perioperatively depend on many factors, including the type of surgery (and thus, the bleeding risk), provider discretion regarding intensity of factor dose and frequency postoperatively, presence of inhibitors, etc. […] In patients with hemophilia A, the required dose of CFC (in IU) to raise FVIII to the desired level (in IU/dL) is calculated by the following formula: FVIII dose (IU) = body weight (kg) × desired rise in FVIII activity (IU/dL) × 0.5. […] In the presence of inhibitor titers greater than 5 BU, CFC treatment is ineffective and alternative bypassing agents must be used for hemostasis. […] While the most severe forms of hemophilia typically affect males, females with an abnormal copy of FVIII or FIX, deemed carriers, can also demonstrate mild, moderate, or even severe hemophilia symptoms depending on the lyonization of their X chromosomes and/or the presence of a second coagulation defect.

• #39 Evolution of Haemophilia Care in Europe: 10 years of the principles of care | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-020-01456-y

  The European principles of care in haemophilia marked their first decade in 2018. […] The 10-year-old European principles had a significant impact on the development of care for haemophilia and related bleeding disorders in Europe. […] Monitoring adherence to, and impact of, the European Principles of Care significantly assists in tracking developments and highlighting gaps. […] In 2008, an interdisciplinary group of European clinicians with patient organisation input published 10 principles of haemophilia care that highlighted the importance of access to a multidisciplinary team of specialists in comprehensive care centres (CCCs) and haemophilia treatment centres (HTCs), a supply of safe clotting factor concentrates for use in home treatment and prophylaxis programmes, and a national patient register collecting treatment statistics.

• #40 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 introduces a functional gene into living tissue, providing cells with the ability to produce a missing or nonfunctional protein. 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. Because both hemophilia A and B are each caused by a variation in a single gene, gene therapy holds the potential to directly address the pathophysiologic basis and may allow the body to produce the missing factor. 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.

• #41 About Hemophilia | Hemophilia | CDC

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

  Hemophilia treatment centers are comprehensive clinics that treat people who have bleeding disorders. […] Good quality medical care from healthcare providers who know a lot about the disorder can help prevent some serious problems. This type of specialty care can be found at a comprehensive hemophilia treatment center (HTC). An HTC not only provides care to address all issues related to the disorder, but also provides health education that helps people with hemophilia stay healthy. Use the HTC directory to find a treatment center near you. […] The standard way to treat both hemophilia A and B is to replace the missing blood clotting factor so that the blood can clot properly. One way to do this is by infusing (injecting into a vein) commercially prepared clotting factor concentrates. People with hemophilia can learn how to perform these infusions themselves so that they can stop bleeding episodes and, by performing the infusions on a regular basis (called prophylaxis), can even prevent most bleeding episodes. […] Gene therapy is also an option for people with hemophilia A and B. Gene therapy is done by injecting copies of the gene that help the body make more clotting factor to prevent bleeding.

• #42 What every nurse should know about hemophilia

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

  As nurses, we know theres no such thing as a simple patient. Essentially, every patient has more than one diagnosis. And as the U.S. population ages, our patients will have longer lists of chronic diagnoses and medicationsand their nursing care will grow even more complex. […] 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. […] Nursing considerations for hospital patients with hemophilia vary with the reason for admission. Those admitted for a problem unrelated to their bleeding disorder nonetheless need to be monitored for bleeding. Urge the physician to obtain a hematology consult. […] Monitor the patient for signs and symptoms of bleeding.

• #43

  https://www.roche.com/solutions/focus-areas/haematology/haemophilia

  By working toward a shared vision of Treatment for All, the WFH and all of its partners, including Roche, aim to address gaps in healthcare and bring innovative treatment solutions to patients living with haemophilia. […] Learn more about the impact of haemophilia in infants and the importance of early diagnosis and prophylactic treatment from birth. […] Learn more about this gap in haemophilia care, and how it can be addressed. […] Dr Gallia Levy, Associate Group Medical Director at Roche, shares her perspective on the need for sustained bleeding protection in haemophilia A. […] We celebrate the WFHs Humanitarian Aid Program, and its mission of improving access to treatment in developing countries. […] How Patient-Reported Outcomes are helping to redefine haemophilia care.

• #44 The existing scenario of haemophilia care in Canada and China – A review | Hematology, Transfusion and Cell Therapy

  https://www.elsevier.es/pt-revista-hematology-transfusion-cell-therapy-396-articulo-the-existing-scenario-haemophilia-care-S2531137919301348

  Hemophilia is an X-linked recessive genetic disorder which affects approximately 400,000 people globally. […] Although developed countries have sufficient treatment products available, they are burdened by the higher expectation of outcomes, coupled with insufficient supportive care to monitor adherence and outcomes and to implement regular follow-up. […] In contrast, developing regions may not have ready access to factor replacement, but have developed excellent physiotherapy and rehabilitation programs. […] The World Federation of Hemophilia (WFH) guidelines, introduced in 2005 and updated in 2012, offer practical recommendations to diagnose and manage hemophilia, providing guidance on managing complications, such as musculoskeletal issues, inhibitors and transfusion-related infections.

• #45 Optimizing haemophilia care | JBM

  https://www.dovepress.com/optimizing-haemophilia-care-in-resource-limited-countries-current-chal-peer-reviewed-fulltext-article-JBM

  About 75% of persons with hemophilia live in the developing world and do not have access to routine care due to many barriers. […] There are a lot of challenges associated with hemophilia care in resource-limited settings, ranging from financial to organisational and government commitments. […] A participative approach involving all stakeholders is key to optimizing care in resource-limited settings. […] Care for PLWH begins with appropriate diagnosis. […] Once diagnosed, clinical care and follow-up is indispensable. […] The WFH recommends that care in PLWH be multidisciplinary requiring hematologists, paediatricians, dentists, physiotherapists, rheumatologists, radiologists, nurse coordinators, psychologists, and more. […] Continuous patient and family education also contributes significantly to proper care for PLWH.

• #46 Optimizing haemophilia care | JBM

  https://www.dovepress.com/optimizing-haemophilia-care-in-resource-limited-countries-current-chal-peer-reviewed-fulltext-article-JBM

  The Twinning programs organized by the WFH between experienced Hemophilia Treatment Centers and emerging ones have been very successful. […] Currently, with the collaboration of World Federation of Hemophilias Humanitarian Aid Program, there is increased access to factor VIII and IX concentrates, and marked improvement in care for PLWH. […] The organization of hemophilia care delivery in many African countries is at its early stages. […] Developing a patients society (National Members Organisation) and a bleeding disorder registry are very important steps towards ensuring adequate treatment for PLWH in resource-limited settings, as is the case in developed countries. […] The coordinated delivery of comprehensive care by multidisciplinary teams of healthcare professionals should be encouraged.

• #47 Treatment and Care | World Federation of Hemophilia

  https://wfh.org/treatment-and-care/

  The WFH SDM Tool provides a framework to guide PWH through the thought process regarding treatment goals and preferences, provides evidence-based and balanced education on expected outcomes by treatment class, and helps PWH navigate the complexities of the current treatment options. […] The WFH works to provide the most up-to-date and evidence-based standards of care for the global bleeding disorders community. […] The WFH Guidelines for the Management of Hemophilia, 3rd edition was developed by an international panel of expert healthcare professionals (HCPs) and people with hemophilia (PWH) and published in August 2020. The objective of the publication is to set the standard of care globally and work to ensure all HCPs and PWH have access to the latest and most up-to-date treatment recommendations, ultimately producing optimal health outcomes for PWH, minimizing harm, and reducing variations in clinical care globally.

• #48 Evolution of Haemophilia Care in Europe: 10 years of the principles of care | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-020-01456-y

  The importance of registries is generally understood and accepted since good data collection leads to maximal treatment benefits. […] A strong network of CCCs and HTCs ensures structure and strategic organisation and leads to the provision of optimum levels of treatment and care. […] Multiple stakeholder-partnership in national decision-making is the most efficient path to optimum delivery of care. […] Calling for safe and effective concentrates, applying a metric of national IU/per capita and recommending its incremental growth over time, has had obvious success. […] The principle of access to home treatment is amongst the most successful with year-on-year reports of increased access. […] Access to prophylaxis for children and adults has increased in all regions. […] Specialist services such as dentistry, physiotherapy, obstetrics and gynaecology, genetic counselling and psychosocial support should be mainstay components of all haemophilia centres, yet between 2009 and 2018 access to these services was inconsistent in all regions. […] Clear gaps and inconsistent inhibitor treatment persist for patients across Europe. […] Monitoring adherence to, and the impact of, the European principles of care and the EDQM recommendations significantly assists in tracking developments and highlighting gaps.

• #49 What every nurse should know about hemophilia

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

  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.

• #50 Sanofi’s Hemophilia Treatment goes Beyond the Bleed

  https://www.sanofi.com/en/magazine/your-health/its-time-to-look-beyond-the-bleed

  Hemophilia can affect nearly every aspect of a persons life, but these daily impacts are often overlooked, and current solutions rarely go beyond medical care. Sanofi is committed to working with the hemophilia community to shed light on the unmet needs of people living with hemophilia and to advance the hemophilia care paradigm to go beyond treating bleeds. […] Bleeding episodes are just one part of living with hemophilia. Too often, people living with hemophilia and their loved ones experience anxiety and fear about bleeding episodes. What they want most is to live their lives free from the burdens and limitations of this disorder. They strive to be seen, heard, and ultimately, understood beyond the box that having hemophilia often puts them in. […] Conducting daily activities without discomfort or worry of a bleed, and reducing impact on personal relationships are among the top goals for managing their hemophilia.

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