Materiały źródłowe

• #1 Preventing Sickle Cell Anemia Complications in Children | VitalSigns | CDC

  https://www.cdc.gov/vitalsigns/sickle-cell-anemia/index.html

  Sickle cell anemia is the most severe form of sickle cell disease, a group of inherited red blood cell disorders causing unusually shaped, hard, and sticky red blood cells. These cells clump together, blocking blood flow carrying oxygen through the body. Sickle cell anemia, which primarily affects Black or African American people, is associated with a shorter life span and life-threatening complications that can affect all parts of the body. These complications cause pain and suffering. Sickle cell anemia is a common cause of childhood stroke. […] There are screenings and treatments available to ease children’s suffering from sickle cell anemia. Two recommended healthcare measures to prevent complications in children with sickle cell anemia are: […] Transcranial doppler (TCD) ultrasound screening, which identifies children with increased risk for stroke. […] Hydroxyurea therapy, which reduces the occurrence of several complications, including severe acute pain episodes and acute chest syndrome, which can result in lung injury and trouble breathing.

• #2 Sickle-Cell Disease: a strategy for the WHO African Region

  https://iris.who.int/handle/10665/1682

  1. Sickle-cell Disease (SCD) is an inherited disorder of haemoglobin. It is the most prevalent genetic disease in the WHO African Region. In many countries, 10%-40% of the population carries the sickle-cell gene resulting in estimated SCD prevalence of at least 2%. […] 4. This strategy provides a set of public health interventions to reduce the burden of SCD in the African Region through improved awareness, disease prevention and early detection. The interventions include improvements in health-care provision; effective clinical, laboratory, diagnostic and imaging facilities adapted to different levels of the health system; screening of newborns; training of health workers and development of protocols; genetic counselling and testing; accessibility to health care; establishment of patient support groups; advocacy; and research.

• #3 Sickle-Cell Disease: a strategy for the WHO African Region

  https://iris.who.int/handle/10665/1682

  1. Sickle-cell Disease (SCD) is an inherited disorder of haemoglobin. It is the most prevalent genetic disease in the WHO African Region. In many countries, 10%-40% of the population carries the sickle-cell gene resulting in estimated SCD prevalence of at least 2%. […] 4. This strategy provides a set of public health interventions to reduce the burden of SCD in the African Region through improved awareness, disease prevention and early detection. The interventions include improvements in health-care provision; effective clinical, laboratory, diagnostic and imaging facilities adapted to different levels of the health system; screening of newborns; training of health workers and development of protocols; genetic counselling and testing; accessibility to health care; establishment of patient support groups; advocacy; and research.

• #4 Sickle Cell Disease (SCD): Types, Symptoms & Causes

  https://my.clevelandclinic.org/health/diseases/12100-sickle-cell-disease

  You cant prevent sickle cell disease because its a genetic condition. If youre pregnant, consider talking to your provider about genetic testing or genetic counseling.

• #5 Sickle Cell Anemia: Symptoms, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/4579-sickle-cell-anemia

  Sickle cell anemia is an inherited disorder that you cant prevent. You can have a blood test to find out if you have the genetic change that causes sickle cell anemia. […] Starting in 2006, all babies born in the U.S. have sickle cell anemia tests right after theyre born. Early diagnosis and treatment are why fewer babies and young children born in the United States die of sickle cell anemia.

• #6 Sickle-Cell Disease: a strategy for the WHO African Region

  https://iris.who.int/handle/10665/1682

  1. Sickle-cell Disease (SCD) is an inherited disorder of haemoglobin. It is the most prevalent genetic disease in the WHO African Region. In many countries, 10%-40% of the population carries the sickle-cell gene resulting in estimated SCD prevalence of at least 2%. […] 4. This strategy provides a set of public health interventions to reduce the burden of SCD in the African Region through improved awareness, disease prevention and early detection. The interventions include improvements in health-care provision; effective clinical, laboratory, diagnostic and imaging facilities adapted to different levels of the health system; screening of newborns; training of health workers and development of protocols; genetic counselling and testing; accessibility to health care; establishment of patient support groups; advocacy; and research.

• #7 Prevention and Treatment of SCD Complications | Sickle Cell Disease (SCD) | CDC

  https://www.cdc.gov/sickle-cell/about/prevention-and-treatment.html

  The focus of sickle cell disease (SCD) management is preventing and treating pain episodes and complications. […] Prevention strategies include lifestyle behaviors, medical screenings, and interventions to prevent infections and other complications. […] Several medications can be taken regularly to prevent or reduce the occurrence of complications. […] Simple steps to prevent harmful infections include the following: Wash your hands often. Washing hands with soap and clean water many times each day is one of the best ways people with SCD, their family members, and other caregivers can help prevent an infection. […] Vaccines can protect against harmful infections. It is important that children with SCD get all regular childhood vaccines. […] Penicillin greatly reduces the risk of infections in people with HbSS (the most common form of SCD in which people inherit two hemoglobin S genes) and has been shown to be even more effective when it is started earlier.

• #8

  https://www.healthychildren.org/English/health-issues/conditions/chronic/Pages/sickle-cell-disease-in-children.aspx

  Learn more about sickle cell disease and how early and ongoing care and treatment helps manage symptoms and prevent complications. […] Prompt diagnosis before infants show any symptoms allows infants with sickle cell disease to get early treatment and parents to learn about ways to help lower the risk of serious health problems. […] National guidelines recommend newborns diagnosed with severe sickle cell disease (SS and S beta zero thalassemia) receive antibiotics by mouth twice a day until they are 5 years old. […] Research shows that children with sickle cell anemia given twice daily penicillin (an antibiotic) had 84% less risk of Streptococcus pneumoniae bacterial infection, which can cause serious conditions like pneumonia and meningitis. […] Early diagnosis and starting antibiotics shortly after birth for those with severe forms of sickle cell have greatly improved childhood survival. […] The U.S. Food Drug Administration (FDA) has approved medications that can help prevent sickle cell complications in children. These include hydroxyurea (ideally started at 9 months of age) and L-glutamine (may start at 5 years of age).

• #9 Sickle Cell Anemia: Symptoms, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/4579-sickle-cell-anemia

  Sickle cell anemia is an inherited disorder that you cant prevent. You can have a blood test to find out if you have the genetic change that causes sickle cell anemia. […] Starting in 2006, all babies born in the U.S. have sickle cell anemia tests right after theyre born. Early diagnosis and treatment are why fewer babies and young children born in the United States die of sickle cell anemia.

• #10 Penicillin Prophylaxis in Children with Sickle Cell Disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3018247/

  Children who have sickle cell disease and are under the age of five years are at increased risk of life-threatening pneumococcal infection due to absent or non-functional spleens and a decreased immune response. To prevent pneumococcal infection, the American Academy of Pediatrics recommends the use of penicillin prophylaxis in children with sickle cell disease under the age of five and in older children who have had a previous severe pneumococcal infection or have functional/surgical asplenia. […] Following the addition of penicillin prophylaxis for SCD patients younger than 5 years of age, the rate of infection for S. pneumoniae has decreased to 1.5 events per 100 patient-years. […] Penicillin prophylaxis and vaccines against various serotypes of S. pneumoniae have been used to prevent infection with S. pneumoniae in SCD patients.

• #11 Management of Sickle Cell Disease: Recommendations from the 2014 Expert Panel Report | AAFP

  https://www.aafp.org/pubs/afp/issues/2015/1215/p1069.html

  Family physicians are the primary and sometimes only health care resource for families affected by sickle cell disease. […] Children with sickle cell anemia should receive prophylactic penicillin from birth through at least five years of age, and all persons with sickle cell disease require vaccination to prevent invasive pneumococcal disease. […] Annual screening with transcranial Doppler ultrasonography is recommended for all children with sickle cell disease beginning at two years of age and continuing through adolescence to evaluate the risk of stroke and to initiate transfusion therapy in those at high risk. […] Offer hydroxyurea therapy to infants, children, and adolescents with sickle cell anemia regardless of clinical severity to reduce sickle cell disease-related complications.

• #12 Penicillin Prophylaxis in Children with Sickle Cell Disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3018247/

  The authors concluded penicillin prophylaxis would drastically reduce the risk of pneumococcal infection in children with SCA, especially those under the age of three. […] In the PROPS study, all patients received penicillin V potassium 125 mg twice daily. This has become the dose recommended for children younger than 5 years of age. […] It is recommended that all children younger than 5 years with SCD take daily prophylactic antibiotics. All newborns screened for SCD and those who test positive should be started on prophylactic penicillin as early as possible. […] Children with recurrent invasive pneumococcal infections should receive penicillin prophylaxis indefinitely. […] Education for families of children with sickle cell disease is paramount to the successful management of a child with SCD.

• #13 Management of Sickle Cell Disease: Recommendations from the 2014 Expert Panel Report | AAFP

  https://www.aafp.org/pubs/afp/issues/2015/1215/p1069.html

  Use an individualized prescribing and monitoring protocol or a sickle cell disease-specific protocol whenever possible to promote rapid, effective, and safe analgesic management and resolution of vasoocclusive crises in children and adults. […] Administer prophylactic oral penicillin (125 mg twice daily for children younger than three years; 250 mg twice daily for those three years and older) until at least five years of age in all children who are homozygous for sickle hemoglobin. […] Screen children with sickle cell anemia annually with transcranial Doppler ultrasonography from two until at least 16 years of age. […] Pregnant or breastfeeding women should discontinue hydroxyurea therapy. […] Offer hydroxyurea therapy for infants nine to 42 months of age and for older children and adolescents with sickle cell anemia, regardless of clinical severity, to reduce sickle cell disease-related complications.

• #14 Sickle Cell Disease (SCD) Treatment & Management: Approach Considerations, Hydroxyurea Therapy, Transfusion

  https://emedicine.medscape.com/article/205926-treatment

  Prevention of infection also improves chances of survival in SCD. In the adult patient, all infections must be treated promptly with broad-spectrum antibiotics. Once a causative organism is identified, therapy is tailored according to its antibiotic sensitivity. […] Penicillin prophylaxis significantly reduces the incidence of infection with encapsulated organisms in particular, S pneumoniae and may decrease the mortality rate. Begin at age 2 months with 125 mg bid of penicillin V or G; at 3 years, increase the dose to 250 mg bid. Prophylaxis should continue until age 5 years or the early teens. […] Transfusion therapy, aimed at keeping the proportion of HbS below 30%, is now considered standard care for primary and secondary stroke prevention in children with SCD. The Stroke Prevention Trial in Sickle Cell Anemia (STOP) showed that regular blood transfusions produced a marked (90%) reduction in first stroke in asymptomatic high-risk children who had 2 abnormal transcranial Doppler (TCD) studies with velocities of 200 cm/s or greater. […] Erythrocytapheresis is now increasingly used as an alternative to simple transfusion. This procedure allows rapid reduction of HbS concentrations to less than 30% without significantly increasing total hemoglobin concentration post transfusion.

• #15 Penicillin Prophylaxis in Children with Sickle Cell Disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3018247/

  The authors concluded penicillin prophylaxis would drastically reduce the risk of pneumococcal infection in children with SCA, especially those under the age of three. […] In the PROPS study, all patients received penicillin V potassium 125 mg twice daily. This has become the dose recommended for children younger than 5 years of age. […] It is recommended that all children younger than 5 years with SCD take daily prophylactic antibiotics. All newborns screened for SCD and those who test positive should be started on prophylactic penicillin as early as possible. […] Children with recurrent invasive pneumococcal infections should receive penicillin prophylaxis indefinitely. […] Education for families of children with sickle cell disease is paramount to the successful management of a child with SCD.

• #16 Antibiotics for Sickle Cell DiseaseShare to Facebookprint pageBookmark for latercaret iconFollow us on facebookFollow us on instagramFollow us on facebookFollow us on linkedincaret icon

  https://sickle-cell.com/penicillin

  Children should be started on penicillin prophylaxis once the diagnosis is established or at least by 2 months of age. To prevent infections, children with SCD should take oral penicillin until they are 5 years old. […] Pneumococcus infections can quickly become severe in children with SCD. Because of this, preventing infections is usually more effective than treating them. Children with SCD should take penicillin from 2 months old to 5 years old. This is often called penicillin “prophylaxis” because it is taken to prevent an infection. […] Penicillin greatly reduces the risk of infections in people with SCD. […] Early diagnosis and penicillin treatment have reduced this to only 3 percent. […] Sticking to a penicillin prophylaxis plan is often difficult. Children with SCD have a higher risk of infection when they do not take penicillin. Infections can happen quickly even after 1 missed dose (10 to 20 hours without penicillin). Continued education to parents and caregivers about the importance of penicillin has helped improve rates of people sticking to the plan.

• #17 Antibiotic Prophylaxis for Children with Sickle Cell Anemia

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7252515/

  Children with sickle cell anemia (SCA) are at increased risk for invasive pneumococcal disease (IPD); antibiotic prophylaxis significantly reduces this risk. Our objective was to calculate the proportion of children with SCA who received 300 days of antibiotic prophylaxis and identify predictors of such receipt. […] Daily receipt of penicillin is an effective method to reduce the incidence of IPD among children with SCA. The Prophylactic Penicillin Study demonstrated an 84% reduction in the risk of infection among children that received daily penicillin as compared to those receiving placebo. […] Despite national recommendations and proven life-saving benefit, antibiotic prophylaxis rates are low among children with SCA. […] Our findings indicate that a substantial gap exists between use of prophylactic antibiotics among children with SCA and NHLBI recommendations, which indicate penicillin prophylaxis until age 5.

• #18 Acute complications in children with sickle cell disease: Prevention and management | Canadian Paediatric Society

  https://cps.ca/en/documents/position/acute-complications-with-sickle-cell

  Daily prophylactic penicillin VK or amoxicillin should be prescribed for all children with SCD from 2 months to 5 years of age. Duration of prophylaxis may be extended for children who have had a surgical splenectomy or a history of invasive bacterial infections, or whose immunizations are not up to date. Cotrimoxazole or erythromycin are alternatives in cases of penicillin allergy. […] High quality studies have shown that hydroxyurea use can significantly reduce risk for acute chest syndrome (ACS), vaso-occlusive episodes (VOE), transfusions, hospitalization, and mortality. Treatment risks and benefits should be discussed with families and the medication offered to all children 9 months of age. Hydroxyurea is now standard of care for all patients with HbSS and HbSB0 thalassemia. While experience with paediatric HbSC patients is limited, hydroxyurea should be considered for symptomatic cases.

• #19 MARAC Statement: Penicillin Shortage – Sickle Cell Disease Association of America Inc.

  https://www.sicklecelldisease.org/2023/02/07/marac-statement-penicillin-shortage/

  Prioritize sickle cell disease as an immune-compromised condition that has specific guidelines stating the necessity for penicillin prophylaxis. […] Make sure your child has all immunizations as recommended for sickle cell disease. SCDAA MARAC strongly encourages full immunization, especially pneumococcal vaccines PCV and PPSV-23. […] In the United States, penicillin prophylaxis is written into public health policy, National Institutes of Health guidelines and even state laws. Brazil, Canada, France, Italy, Jamaica, Ghana, Nigeria, Tanzania, Uganda, the United Kingdom and other countries have penicillin prophylaxis in their national guidelines for sickle cell disease. […] Penicillin prophylaxis has an excellent track record and has been used to provide safe care to thousands of children for over 30 years. Studies have showed that giving penicillin prophylaxis continuously does not cause harmful bacteria to become antibiotic resistant. On-and-off periods of penicillin prophylaxis could cause antibiotic resistant bacteria to emerge. Penicillin was chosen for prophylaxis because it is focused on the pneumococcal bacteria that can cause problems in sickle cell disease.

• #20 Penicillin Prophylaxis in Patients With Sickle Cell Disease Beyond Age 5 Years in: The Journal of Pediatric Pharmacology and Therapeutics Volume 28: Issue 6 | Journal of Pediatric Pharmacology and Therapeutics

  https://jppt.kglmeridian.com/view/journals/jppt/28/6/article-p519.xml

  The Prophylaxis with oral penicillin in children with sickle cell anemia (PROPS) trial established the efficacy of antimicrobial prophylaxis with penicillin at preventing IPD, and the follow-up PROPS-II study demonstrated the safety of discontinuation in patients following their fifth birthday (absent any prior IPD or splenectomy). On the basis of these 2 studies, the national Evidence-Based Management of SCD Expert Consensus Report recommends the discontinuation of penicillin prophylaxis at age 5 years, with the exclusion of patients with surgical asplenia or a prior invasive pneumococcal infection. […] The results found in this study support appropriate de-prescribing of antimicrobial prophylaxis in patients with SCD who are not at high risk for IPD. Further multicenter studies are needed to evaluate the effects of antimicrobial prophylaxis with alternative agents on antibiotic resistance, examine provider rationale for continuation of antimicrobial prophylaxis, and evaluate the quality of life effects (e.g., medication adherence and adverse drug reactions) of antimicrobial prophylaxis.

• #21 Penicillin Prophylaxis in Children with Sickle Cell Disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3018247/

  The authors concluded penicillin prophylaxis would drastically reduce the risk of pneumococcal infection in children with SCA, especially those under the age of three. […] In the PROPS study, all patients received penicillin V potassium 125 mg twice daily. This has become the dose recommended for children younger than 5 years of age. […] It is recommended that all children younger than 5 years with SCD take daily prophylactic antibiotics. All newborns screened for SCD and those who test positive should be started on prophylactic penicillin as early as possible. […] Children with recurrent invasive pneumococcal infections should receive penicillin prophylaxis indefinitely. […] Education for families of children with sickle cell disease is paramount to the successful management of a child with SCD.

• #22 Antibiotic Prophylaxis for Children with Sickle Cell Anemia

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7252515/

  Children with sickle cell anemia (SCA) are at increased risk for invasive pneumococcal disease (IPD); antibiotic prophylaxis significantly reduces this risk. Our objective was to calculate the proportion of children with SCA who received 300 days of antibiotic prophylaxis and identify predictors of such receipt. […] Daily receipt of penicillin is an effective method to reduce the incidence of IPD among children with SCA. The Prophylactic Penicillin Study demonstrated an 84% reduction in the risk of infection among children that received daily penicillin as compared to those receiving placebo. […] Despite national recommendations and proven life-saving benefit, antibiotic prophylaxis rates are low among children with SCA. […] Our findings indicate that a substantial gap exists between use of prophylactic antibiotics among children with SCA and NHLBI recommendations, which indicate penicillin prophylaxis until age 5.

• #23 Antibiotic Prophylaxis for Children with Sickle Cell Anemia

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7252515/

  Given the consistent finding that antibiotic prophylaxis rates are low among children with SCA, development of practical and effective interventions are key. […] Despite long-standing national recommendations, antibiotic prophylaxis against IPD remains low among children with SCA, and efforts aimed at increasing adherence are urgently needed.

• #24 Management of Sickle Cell Disease: Recommendations from the 2014 Expert Panel Report | AAFP

  https://www.aafp.org/pubs/afp/issues/2015/1215/p1069.html

  Ensure that all persons with SCD have been vaccinated against Streptococcus pneumoniae. […] Ensure that children 6 to 18 years of age who have functional or anatomic asplenia have received at least 1 dose of 13-valent pneumococcal conjugate vaccine. […] Screen annually with TCD according to methods used in the STOP studies, beginning at 2 years of age and continuing until at least 16 years of age. […] Refer children with conditional (170 to 199 cm per second) or elevated (200 cm per second or greater) TCD findings to a subspecialist with expertise in long-term transfusion therapy aimed at preventing stroke.

• #25 Acute complications in children with sickle cell disease: Prevention and management | Canadian Paediatric Society

  https://cps.ca/en/documents/position/acute-complications-with-sickle-cell

  SCD was part of NBS in seven provinces and two territories in 2021. Its inclusion has not only helped reduce SCD-related infant mortality rates, but allows for earlier referral, parent education, preventive strategies, and genetic counselling. […] While the routine Canadian vaccination schedule includes partial immunization against pneumococcal disease and immunization against meningococcal type C bacterial infection, it is imperative that children with SCD receive enhanced vaccination against these encapsulated bacteria. Specifically, children with SCD should receive the 13-valent pneumococcal conjugate and polysaccharide vaccines against Streptococcus pneumoniae, and both conjugated quadrivalent meningococcal (A,C,W,Y) and serogroup B vaccines targeting Neisseria meningitidis. Further, an extra booster dose against Haemophilus influenzae type B (Hib), immunization against hepatitis A and B, and annual influenza vaccines are all recommended. In the context of travel, vaccination against Salmonella typhi and malaria prophylaxis should be offered.

• #26 Living with Sickle Cell Disease – Prevention of Infection – Alder Hey Children’s Hospital Trust

  https://www.alderhey.nhs.uk/conditions/patient-information-leaflets/living-with-sickle-cell-disease-prevention-of-infection/

  People with Sickle Cell disease are more prone to catching infections like pneumococcal, meningitis, osteomyelitis, salmonella and septicaemia. […] Good hygiene can help to prevent infections. Ensure that your child washes their hands regularly. […] Giving your child Penicillin medicine twice a day has been shown to reduce their chance of catching pneumococcal infections. […] Make sure that your child gets all their recommended immunisations. […] Your child should receive all their routine childhood immunisations at the ages recommended. In addition to those immunisations your child will need additional vaccines that your sickle cell team will advise you about and let your primary health care team know when they are due. […] The extra vaccines are: Flu vaccine (influenza vaccine) every year after six months of age. Everyone in the household should also get this immunisation every year. […] Pneumococcal vaccine (called Pneumovax) at two years of age and a booster every five years after that. […] Hepatitis B. […] Meningitis ACWY […] Meningitis B (if your child has not received this as part of their routine immunisations).

• #27 Acute complications in children with sickle cell disease: Prevention and management | Canadian Paediatric Society

  https://cps.ca/en/documents/position/acute-complications-with-sickle-cell

  SCD was part of NBS in seven provinces and two territories in 2021. Its inclusion has not only helped reduce SCD-related infant mortality rates, but allows for earlier referral, parent education, preventive strategies, and genetic counselling. […] While the routine Canadian vaccination schedule includes partial immunization against pneumococcal disease and immunization against meningococcal type C bacterial infection, it is imperative that children with SCD receive enhanced vaccination against these encapsulated bacteria. Specifically, children with SCD should receive the 13-valent pneumococcal conjugate and polysaccharide vaccines against Streptococcus pneumoniae, and both conjugated quadrivalent meningococcal (A,C,W,Y) and serogroup B vaccines targeting Neisseria meningitidis. Further, an extra booster dose against Haemophilus influenzae type B (Hib), immunization against hepatitis A and B, and annual influenza vaccines are all recommended. In the context of travel, vaccination against Salmonella typhi and malaria prophylaxis should be offered.

• #28 Acute complications in children with sickle cell disease: Prevention and management | Canadian Paediatric Society

  https://cps.ca/en/documents/position/acute-complications-with-sickle-cell

  SCD was part of NBS in seven provinces and two territories in 2021. Its inclusion has not only helped reduce SCD-related infant mortality rates, but allows for earlier referral, parent education, preventive strategies, and genetic counselling. […] While the routine Canadian vaccination schedule includes partial immunization against pneumococcal disease and immunization against meningococcal type C bacterial infection, it is imperative that children with SCD receive enhanced vaccination against these encapsulated bacteria. Specifically, children with SCD should receive the 13-valent pneumococcal conjugate and polysaccharide vaccines against Streptococcus pneumoniae, and both conjugated quadrivalent meningococcal (A,C,W,Y) and serogroup B vaccines targeting Neisseria meningitidis. Further, an extra booster dose against Haemophilus influenzae type B (Hib), immunization against hepatitis A and B, and annual influenza vaccines are all recommended. In the context of travel, vaccination against Salmonella typhi and malaria prophylaxis should be offered.

• #29 Guidelines on sickle cell disease: primary stroke prevention in children and adolescents. Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular Guidelines project: Associação Médica Brasileira – 2021

  https://www.htct.com.br/en-guidelines-on-sickle-cell-disease-articulo-S2531137921012748

  Guidelines on sickle cell disease: primary stroke prevention in children and adolescents. […] This article presents the guidelines on sickle cell disease: primary stroke prevention in children and adolescents. […] Stroke is a significant cause of morbidity and mortality in individuals with SCD, leading to severe motor and neurocognitive sequelae. […] Studies have been conducted to identify concurrent risk factors for cerebrovascular disease, and the detection of high cerebral blood flow velocity (CBFV) in arterial segments of the polygon of Willis by conventional transcranial doppler (TCD) has been confirmed as the major risk factor for the development of ischemic stroke in children and adolescents with SCA. […] Many advances have been made in treating SCD and in the primary prevention of stroke, such as chronic packed red blood cell (pRBC) transfusions, drugs that induce fetal hemoglobin (HbF), and hematopoietic stem cell transplantation.

• #30 Management of Sickle Cell Disease: Recommendations from the 2014 Expert Panel Report | AAFP

  https://www.aafp.org/pubs/afp/issues/2015/1215/p1069.html

  Use an individualized prescribing and monitoring protocol or a sickle cell disease-specific protocol whenever possible to promote rapid, effective, and safe analgesic management and resolution of vasoocclusive crises in children and adults. […] Administer prophylactic oral penicillin (125 mg twice daily for children younger than three years; 250 mg twice daily for those three years and older) until at least five years of age in all children who are homozygous for sickle hemoglobin. […] Screen children with sickle cell anemia annually with transcranial Doppler ultrasonography from two until at least 16 years of age. […] Pregnant or breastfeeding women should discontinue hydroxyurea therapy. […] Offer hydroxyurea therapy for infants nine to 42 months of age and for older children and adolescents with sickle cell anemia, regardless of clinical severity, to reduce sickle cell disease-related complications.

• #31 Management of Sickle Cell Disease: Recommendations from the 2014 Expert Panel Report | AAFP

  https://www.aafp.org/pubs/afp/issues/2015/1215/p1069.html

  Ensure that all persons with SCD have been vaccinated against Streptococcus pneumoniae. […] Ensure that children 6 to 18 years of age who have functional or anatomic asplenia have received at least 1 dose of 13-valent pneumococcal conjugate vaccine. […] Screen annually with TCD according to methods used in the STOP studies, beginning at 2 years of age and continuing until at least 16 years of age. […] Refer children with conditional (170 to 199 cm per second) or elevated (200 cm per second or greater) TCD findings to a subspecialist with expertise in long-term transfusion therapy aimed at preventing stroke.

• #32 Preventing Sickle Cell Anemia Complications in Children | VitalSigns | CDC

  https://www.cdc.gov/vitalsigns/sickle-cell-anemia/index.html

  Sickle cell anemia is the most severe form of sickle cell disease, a group of inherited red blood cell disorders causing unusually shaped, hard, and sticky red blood cells. These cells clump together, blocking blood flow carrying oxygen through the body. Sickle cell anemia, which primarily affects Black or African American people, is associated with a shorter life span and life-threatening complications that can affect all parts of the body. These complications cause pain and suffering. Sickle cell anemia is a common cause of childhood stroke. […] There are screenings and treatments available to ease children’s suffering from sickle cell anemia. Two recommended healthcare measures to prevent complications in children with sickle cell anemia are: […] Transcranial doppler (TCD) ultrasound screening, which identifies children with increased risk for stroke. […] Hydroxyurea therapy, which reduces the occurrence of several complications, including severe acute pain episodes and acute chest syndrome, which can result in lung injury and trouble breathing.

• #33 Guidelines on sickle cell disease: primary stroke prevention in children and adolescents. Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular Guidelines project: Associação Médica Brasileira – 2021

  https://www.htct.com.br/en-guidelines-on-sickle-cell-disease-articulo-S2531137921012748

  Conventional transcranial doppler (TCD) should be periodically performed in children with sickle cell disease (HbSS/HbS0), aged between two and 16 years, by measuring the maximum velocity of the middle cerebral artery and internal carotid artery to identify patients at risk for stroke. […] The STOP trial was designed to assess the incidence of stroke (ischemic or hemorrhagic) in SCD (HbSS, HbS0) patients without previous stroke, but with a high risk to develop it due to abnormal TCD 200 cm/s), who are on pRBC transfusions regimen to reduce the HbS. […] A meta-analysis confirmed the reduction in stroke (primary prevention) in children and adolescents with SCD (HbSS, HbS0) and abnormal TCD velocity ( 200 cm/s) receiving regular pRBC transfusions. […] Patients with sickle cell disease (HbSS, HbS0), age between 2-16 years and abnormal TCD velocity ( 200 cm/s) should receive chronic packed red blood cell transfusions to maintain HbS.

• #34 Management of Sickle Cell Disease: Recommendations from the 2014 Expert Panel Report | AAFP

  https://www.aafp.org/pubs/afp/issues/2015/1215/p1069.html

  Ensure that all persons with SCD have been vaccinated against Streptococcus pneumoniae. […] Ensure that children 6 to 18 years of age who have functional or anatomic asplenia have received at least 1 dose of 13-valent pneumococcal conjugate vaccine. […] Screen annually with TCD according to methods used in the STOP studies, beginning at 2 years of age and continuing until at least 16 years of age. […] Refer children with conditional (170 to 199 cm per second) or elevated (200 cm per second or greater) TCD findings to a subspecialist with expertise in long-term transfusion therapy aimed at preventing stroke.

• #35 Management of Sickle Cell Disease: Recommendations from the 2014 Expert Panel Report | AAFP

  https://www.aafp.org/pubs/afp/issues/2015/1215/p1069.html

  Ensure that all persons with SCD have been vaccinated against Streptococcus pneumoniae. […] Ensure that children 6 to 18 years of age who have functional or anatomic asplenia have received at least 1 dose of 13-valent pneumococcal conjugate vaccine. […] Screen annually with TCD according to methods used in the STOP studies, beginning at 2 years of age and continuing until at least 16 years of age. […] Refer children with conditional (170 to 199 cm per second) or elevated (200 cm per second or greater) TCD findings to a subspecialist with expertise in long-term transfusion therapy aimed at preventing stroke.

• #36 Sickle Cell Disease (SCD) Treatment & Management: Approach Considerations, Hydroxyurea Therapy, Transfusion

  https://emedicine.medscape.com/article/205926-treatment

  Prevention of infection also improves chances of survival in SCD. In the adult patient, all infections must be treated promptly with broad-spectrum antibiotics. Once a causative organism is identified, therapy is tailored according to its antibiotic sensitivity. […] Penicillin prophylaxis significantly reduces the incidence of infection with encapsulated organisms in particular, S pneumoniae and may decrease the mortality rate. Begin at age 2 months with 125 mg bid of penicillin V or G; at 3 years, increase the dose to 250 mg bid. Prophylaxis should continue until age 5 years or the early teens. […] Transfusion therapy, aimed at keeping the proportion of HbS below 30%, is now considered standard care for primary and secondary stroke prevention in children with SCD. The Stroke Prevention Trial in Sickle Cell Anemia (STOP) showed that regular blood transfusions produced a marked (90%) reduction in first stroke in asymptomatic high-risk children who had 2 abnormal transcranial Doppler (TCD) studies with velocities of 200 cm/s or greater. […] Erythrocytapheresis is now increasingly used as an alternative to simple transfusion. This procedure allows rapid reduction of HbS concentrations to less than 30% without significantly increasing total hemoglobin concentration post transfusion.

• #37 Sickle Cell Disease (SCD) Treatment & Management: Approach Considerations, Hydroxyurea Therapy, Transfusion

  https://emedicine.medscape.com/article/205926-treatment

  Prevention of infection also improves chances of survival in SCD. In the adult patient, all infections must be treated promptly with broad-spectrum antibiotics. Once a causative organism is identified, therapy is tailored according to its antibiotic sensitivity. […] Penicillin prophylaxis significantly reduces the incidence of infection with encapsulated organisms in particular, S pneumoniae and may decrease the mortality rate. Begin at age 2 months with 125 mg bid of penicillin V or G; at 3 years, increase the dose to 250 mg bid. Prophylaxis should continue until age 5 years or the early teens. […] Transfusion therapy, aimed at keeping the proportion of HbS below 30%, is now considered standard care for primary and secondary stroke prevention in children with SCD. The Stroke Prevention Trial in Sickle Cell Anemia (STOP) showed that regular blood transfusions produced a marked (90%) reduction in first stroke in asymptomatic high-risk children who had 2 abnormal transcranial Doppler (TCD) studies with velocities of 200 cm/s or greater. […] Erythrocytapheresis is now increasingly used as an alternative to simple transfusion. This procedure allows rapid reduction of HbS concentrations to less than 30% without significantly increasing total hemoglobin concentration post transfusion.

• #38 Sickle Cell Disease (SCD) Treatment & Management: Approach Considerations, Hydroxyurea Therapy, Transfusion

  https://emedicine.medscape.com/article/205926-treatment

  Prevention of infection also improves chances of survival in SCD. In the adult patient, all infections must be treated promptly with broad-spectrum antibiotics. Once a causative organism is identified, therapy is tailored according to its antibiotic sensitivity. […] Penicillin prophylaxis significantly reduces the incidence of infection with encapsulated organisms in particular, S pneumoniae and may decrease the mortality rate. Begin at age 2 months with 125 mg bid of penicillin V or G; at 3 years, increase the dose to 250 mg bid. Prophylaxis should continue until age 5 years or the early teens. […] Transfusion therapy, aimed at keeping the proportion of HbS below 30%, is now considered standard care for primary and secondary stroke prevention in children with SCD. The Stroke Prevention Trial in Sickle Cell Anemia (STOP) showed that regular blood transfusions produced a marked (90%) reduction in first stroke in asymptomatic high-risk children who had 2 abnormal transcranial Doppler (TCD) studies with velocities of 200 cm/s or greater. […] Erythrocytapheresis is now increasingly used as an alternative to simple transfusion. This procedure allows rapid reduction of HbS concentrations to less than 30% without significantly increasing total hemoglobin concentration post transfusion.

• #39 Preventing Sickle Cell Anemia Complications in Children | VitalSigns | CDC

  https://www.cdc.gov/vitalsigns/sickle-cell-anemia/index.html

  Sickle cell anemia is the most severe form of sickle cell disease, a group of inherited red blood cell disorders causing unusually shaped, hard, and sticky red blood cells. These cells clump together, blocking blood flow carrying oxygen through the body. Sickle cell anemia, which primarily affects Black or African American people, is associated with a shorter life span and life-threatening complications that can affect all parts of the body. These complications cause pain and suffering. Sickle cell anemia is a common cause of childhood stroke. […] There are screenings and treatments available to ease children’s suffering from sickle cell anemia. Two recommended healthcare measures to prevent complications in children with sickle cell anemia are: […] Transcranial doppler (TCD) ultrasound screening, which identifies children with increased risk for stroke. […] Hydroxyurea therapy, which reduces the occurrence of several complications, including severe acute pain episodes and acute chest syndrome, which can result in lung injury and trouble breathing.

• #40 Guidelines on sickle cell disease: primary stroke prevention in children and adolescents. Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular Guidelines project: Associação Médica Brasileira – 2021

  https://www.htct.com.br/en-guidelines-on-sickle-cell-disease-articulo-S2531137921012748

  Hydroxyurea (HU) is a cytostatic and myelosuppressive agent that acts directly on the pathophysiological mechanism of SCD, raising HbF level and improving blood flow through reduced cellular adhesion. […] Given the results obtained so far, it is possible to observe the curative potential of HSCT in patients with SCA and its role in preventing stroke. […] Patients with sickle cell anemia and abnormal conventional transcranial doppler (200 cm/s) should undergo HLA testing to search for family donors (parents and siblings).

• #41 Management of Sickle Cell Disease: Recommendations from the 2014 Expert Panel Report | AAFP

  https://www.aafp.org/pubs/afp/issues/2015/1215/p1069.html

  Use an individualized prescribing and monitoring protocol or a sickle cell disease-specific protocol whenever possible to promote rapid, effective, and safe analgesic management and resolution of vasoocclusive crises in children and adults. […] Administer prophylactic oral penicillin (125 mg twice daily for children younger than three years; 250 mg twice daily for those three years and older) until at least five years of age in all children who are homozygous for sickle hemoglobin. […] Screen children with sickle cell anemia annually with transcranial Doppler ultrasonography from two until at least 16 years of age. […] Pregnant or breastfeeding women should discontinue hydroxyurea therapy. […] Offer hydroxyurea therapy for infants nine to 42 months of age and for older children and adolescents with sickle cell anemia, regardless of clinical severity, to reduce sickle cell disease-related complications.

• #42 Acute complications in children with sickle cell disease: Prevention and management | Canadian Paediatric Society

  https://cps.ca/en/documents/position/acute-complications-with-sickle-cell

  Daily prophylactic penicillin VK or amoxicillin should be prescribed for all children with SCD from 2 months to 5 years of age. Duration of prophylaxis may be extended for children who have had a surgical splenectomy or a history of invasive bacterial infections, or whose immunizations are not up to date. Cotrimoxazole or erythromycin are alternatives in cases of penicillin allergy. […] High quality studies have shown that hydroxyurea use can significantly reduce risk for acute chest syndrome (ACS), vaso-occlusive episodes (VOE), transfusions, hospitalization, and mortality. Treatment risks and benefits should be discussed with families and the medication offered to all children 9 months of age. Hydroxyurea is now standard of care for all patients with HbSS and HbSB0 thalassemia. While experience with paediatric HbSC patients is limited, hydroxyurea should be considered for symptomatic cases.

• #43 Acute complications in children with sickle cell disease: Prevention and management | Canadian Paediatric Society

  https://cps.ca/en/documents/position/acute-complications-with-sickle-cell

  Daily prophylactic penicillin VK or amoxicillin should be prescribed for all children with SCD from 2 months to 5 years of age. Duration of prophylaxis may be extended for children who have had a surgical splenectomy or a history of invasive bacterial infections, or whose immunizations are not up to date. Cotrimoxazole or erythromycin are alternatives in cases of penicillin allergy. […] High quality studies have shown that hydroxyurea use can significantly reduce risk for acute chest syndrome (ACS), vaso-occlusive episodes (VOE), transfusions, hospitalization, and mortality. Treatment risks and benefits should be discussed with families and the medication offered to all children 9 months of age. Hydroxyurea is now standard of care for all patients with HbSS and HbSB0 thalassemia. While experience with paediatric HbSC patients is limited, hydroxyurea should be considered for symptomatic cases.

• #44 Management of Sickle Cell Disease: Recommendations from the 2014 Expert Panel Report | AAFP

  https://www.aafp.org/pubs/afp/issues/2015/1215/p1069.html

  Use an individualized prescribing and monitoring protocol or a sickle cell disease-specific protocol whenever possible to promote rapid, effective, and safe analgesic management and resolution of vasoocclusive crises in children and adults. […] Administer prophylactic oral penicillin (125 mg twice daily for children younger than three years; 250 mg twice daily for those three years and older) until at least five years of age in all children who are homozygous for sickle hemoglobin. […] Screen children with sickle cell anemia annually with transcranial Doppler ultrasonography from two until at least 16 years of age. […] Pregnant or breastfeeding women should discontinue hydroxyurea therapy. […] Offer hydroxyurea therapy for infants nine to 42 months of age and for older children and adolescents with sickle cell anemia, regardless of clinical severity, to reduce sickle cell disease-related complications.

• #45

  https://myhealth.alberta.ca/Health/pages/conditions.aspx?hwid=uh1427&lang=en-ca

  Here are some things you can do to help prevent problems when you or your child has sickle cell disease. […] Avoid infections. Make sure that you and your child get all the recommended vaccines on schedule. […] To help your child with sickle cell disease, make sure that your child takes antibiotics until age 5. […] Drink water and other fluids to prevent dehydration. […] Eat healthy foods. Healthy eating helps keep your body’s immune system strong. […] Don’t smoke. Smoking and second-hand smoke reduce the amount of oxygen in your bloodstream. […] Exercise safely. […] Stay safe at high altitudes and during air travel. Low oxygen levels caused by high altitudes and plane flights can cause problems. […] Recognize serious symptoms. Talk to your doctor about what symptoms to watch for.

• #46 Advances in the Management of Sickle Cell Disease

  https://www.uspharmacist.com/article/advances-in-the-management-of-sickle-cell-disease

  Hydroxyurea and blood transfusions have been the mainstays in the prevention of anemia and vaso-occlusive crises in sickle cell disease. Hydroxyurea is an antimetabolite proven to reduce the incidence of vaso-occlusive pain crises and the need for blood transfusions in sickle cell patients. […] Blood transfusions are also used chronically to prophylactically to treat patients who are at an increased risk for recurrent vaso-occlusive events. Since 1998, hydroxyurea has been used first-line to prevent the occurrence of vaso-occlusive pain crises in sickle cell patients. […] Crizanlizumab, L-glutamine, and voxelotor reduced the incidence of vaso-occlusive events, and voxelotor was also beneficial in reducing anemia and hemolysis in sickle cell patients. New therapies, specifically L-glutamine and voxelotor, may be used as adjunct therapy to hydroxyurea or in place of hydroxyurea if intolerable.

• #47 Advances in the Management of Sickle Cell Disease

  https://www.uspharmacist.com/article/advances-in-the-management-of-sickle-cell-disease

  The most notable addition to therapies indicated for the prevention of vaso-occlusive crises is Adakveo (crizanlizumab). Crizanlizumab is a monoclonal antibody that inhibits P-selectin. […] In 2017, Endari (L-glutamine) was approved to reduce the frequency of pain crises in sickle cell patients older than age 5 years who experience two or more pain crises a year. […] Voxelotor is a HgS polymerization inhibitor that blocks the initial step in the production of HgS and works to stabilize oxygenation to reduce sickling of hemoglobin.

• #48 Sickle Cell Crisis: Symptoms, Causes, Treatment, Prevention

  https://www.webmd.com/a-to-z-guides/sickle-cell-crisis

  There are a few drugs that can help. The drug called L-glutamine oral powder (Endari) has proven to help prevent these crises from occurring and thus preventing hospitalizations. […] Crizanlizumab-tmca (Adakveo) helps stop your blood cells from sticking together and blocking blood vessels, which isn’t just painful, but can damage organs. […] Theres no sure way, but you can lower your odds: […] It also helps to keep yourself as healthy as possible:

• #49 Advances in the Management of Sickle Cell Disease

  https://www.uspharmacist.com/article/advances-in-the-management-of-sickle-cell-disease

  The most notable addition to therapies indicated for the prevention of vaso-occlusive crises is Adakveo (crizanlizumab). Crizanlizumab is a monoclonal antibody that inhibits P-selectin. […] In 2017, Endari (L-glutamine) was approved to reduce the frequency of pain crises in sickle cell patients older than age 5 years who experience two or more pain crises a year. […] Voxelotor is a HgS polymerization inhibitor that blocks the initial step in the production of HgS and works to stabilize oxygenation to reduce sickling of hemoglobin.

• #50 Advances in the Management of Sickle Cell Disease

  https://www.uspharmacist.com/article/advances-in-the-management-of-sickle-cell-disease

  The most notable addition to therapies indicated for the prevention of vaso-occlusive crises is Adakveo (crizanlizumab). Crizanlizumab is a monoclonal antibody that inhibits P-selectin. […] In 2017, Endari (L-glutamine) was approved to reduce the frequency of pain crises in sickle cell patients older than age 5 years who experience two or more pain crises a year. […] Voxelotor is a HgS polymerization inhibitor that blocks the initial step in the production of HgS and works to stabilize oxygenation to reduce sickling of hemoglobin.

• #51 Advances in the Management of Sickle Cell Disease

  https://www.uspharmacist.com/article/advances-in-the-management-of-sickle-cell-disease

  Hydroxyurea and blood transfusions have been the mainstays in the prevention of anemia and vaso-occlusive crises in sickle cell disease. Hydroxyurea is an antimetabolite proven to reduce the incidence of vaso-occlusive pain crises and the need for blood transfusions in sickle cell patients. […] Blood transfusions are also used chronically to prophylactically to treat patients who are at an increased risk for recurrent vaso-occlusive events. Since 1998, hydroxyurea has been used first-line to prevent the occurrence of vaso-occlusive pain crises in sickle cell patients. […] Crizanlizumab, L-glutamine, and voxelotor reduced the incidence of vaso-occlusive events, and voxelotor was also beneficial in reducing anemia and hemolysis in sickle cell patients. New therapies, specifically L-glutamine and voxelotor, may be used as adjunct therapy to hydroxyurea or in place of hydroxyurea if intolerable.

• #52

  https://myhealth.alberta.ca/Health/pages/conditions.aspx?hwid=uh1427&lang=en-ca

  Here are some things you can do to help prevent problems when you or your child has sickle cell disease. […] Avoid infections. Make sure that you and your child get all the recommended vaccines on schedule. […] To help your child with sickle cell disease, make sure that your child takes antibiotics until age 5. […] Drink water and other fluids to prevent dehydration. […] Eat healthy foods. Healthy eating helps keep your body’s immune system strong. […] Don’t smoke. Smoking and second-hand smoke reduce the amount of oxygen in your bloodstream. […] Exercise safely. […] Stay safe at high altitudes and during air travel. Low oxygen levels caused by high altitudes and plane flights can cause problems. […] Recognize serious symptoms. Talk to your doctor about what symptoms to watch for.

• #53 Sickle Cell Disease (SCD) Treatment & Management: Approach Considerations, Hydroxyurea Therapy, Transfusion

  https://emedicine.medscape.com/article/205926-treatment

  Prevention of infection also improves chances of survival in SCD. In the adult patient, all infections must be treated promptly with broad-spectrum antibiotics. Once a causative organism is identified, therapy is tailored according to its antibiotic sensitivity. […] Penicillin prophylaxis significantly reduces the incidence of infection with encapsulated organisms in particular, S pneumoniae and may decrease the mortality rate. Begin at age 2 months with 125 mg bid of penicillin V or G; at 3 years, increase the dose to 250 mg bid. Prophylaxis should continue until age 5 years or the early teens. […] Transfusion therapy, aimed at keeping the proportion of HbS below 30%, is now considered standard care for primary and secondary stroke prevention in children with SCD. The Stroke Prevention Trial in Sickle Cell Anemia (STOP) showed that regular blood transfusions produced a marked (90%) reduction in first stroke in asymptomatic high-risk children who had 2 abnormal transcranial Doppler (TCD) studies with velocities of 200 cm/s or greater. […] Erythrocytapheresis is now increasingly used as an alternative to simple transfusion. This procedure allows rapid reduction of HbS concentrations to less than 30% without significantly increasing total hemoglobin concentration post transfusion.

• #54 Study Shows Low Enoxaparin VTE Prophylaxis Adherence for Patients With Sickle Cell Disease During Hospitalization – Hematology Advisor

  https://www.hematologyadvisor.com/news/sickle-cell-disease-low-enoxaparin-vte-prophylaxis-adherence-treatment/

  A recent study of hospitalized patients with sickle cell disease (SCD) showed a low rate of adherence to enoxaparin for venous thromboembolism (VTE) prophylaxis. […] Despite the high rates of VTE in hospitalized patients with SCD, there are no disease-specific guidelines for VTE prophylaxis in this population, the researchers explained in their report. […] VTE is a serious complication in patients with SCD and it is vital for the care team to assess if medications are being administered as prescribed, the researchers wrote in their report. They advised discussing with the patient, during each admission, the risks of VTE and possible prophylaxis options. The whole care team can play a part in making sure patients are receiving the best agent to prevent detrimental outcomes, they concluded.

• #55 Study Shows Low Enoxaparin VTE Prophylaxis Adherence for Patients With Sickle Cell Disease During Hospitalization – Hematology Advisor

  https://www.hematologyadvisor.com/news/sickle-cell-disease-low-enoxaparin-vte-prophylaxis-adherence-treatment/

  A recent study of hospitalized patients with sickle cell disease (SCD) showed a low rate of adherence to enoxaparin for venous thromboembolism (VTE) prophylaxis. […] Despite the high rates of VTE in hospitalized patients with SCD, there are no disease-specific guidelines for VTE prophylaxis in this population, the researchers explained in their report. […] VTE is a serious complication in patients with SCD and it is vital for the care team to assess if medications are being administered as prescribed, the researchers wrote in their report. They advised discussing with the patient, during each admission, the risks of VTE and possible prophylaxis options. The whole care team can play a part in making sure patients are receiving the best agent to prevent detrimental outcomes, they concluded.

• #56 Sickle cell crisis prevention for children | Norton Children’s Louisville, Ky.

  https://nortonchildrens.com/news/sickle-cell-crisis-prevention/

  The best defense for a sickle cell crisis is prevention. Here are sickle cell crisis prevention tips for families. […] Sickle cell crisis prevention is important for families of children with sickle cell disease. […] To prevent a sickle cell crisis, families can: Take sickle cell medications as prescribed: Preventing sickle cell crises starts with a child taking all medications as prescribed for sickle cell disease, including disease-modifying therapies such as hydroxyurea. […] Try to limit infection exposure: Children with sickle cell disease can be more at risk for complications of infections and bacteria such as the flu and salmonella. Staying up to date on vaccines, including the flu shot, and practicing good hygiene, including hand-washing, can help limit exposure to germs that cause infections.

• #57 Sickle cell crisis prevention for children | Norton Children’s Louisville, Ky.

  https://nortonchildrens.com/news/sickle-cell-crisis-prevention/

  Eat well and stay hydrated: Eating a balanced diet and drinking plenty of water can help children with sickle cell disease stay healthy overall and prevent dehydration. Dehydration can help to bring on a sickle cell crisis. […] Get good rest: Staying well rested can help children prevent sickle cell crises. […] Stay active: Children with sickle cell disease benefit from physical activity with breaks incorporated. […] Dress for the weather: Sickle cell crises can be caused by exposure to extreme temperatures, both hot and cold. […] Manage any other conditions: Should a child have any other conditions, such as diabetes, make sure they are managed well as part of their overall health care. […] Avoid high altitudes: Should you need to fly, always travel in commercial airplanes. […] Avoid smoking and alcohol: Teens and young adults with sickle cell disease should not experiment with alcohol or smoking. […] Try to stay positive and avoid stress: Managing any chronic condition can be difficult for children and families. Stress can cause a lot of physical reactions in the body that could trigger a sickle cell crisis.

• #58 Practical Tips for Preventing a Sickle Cell Crisis | AAFP

  https://www.aafp.org/pubs/afp/issues/2000/0301/p1363.html

  Most of the time, you won’t know what caused your sickle cell crisis. A crisis usually has more than one cause. However, you can do several things that might keep a crisis from occurring: […] Don’t drink a lot of alcohol. […] Don’t smoke. If you do smoke, quit. […] Exercise regularly but not so much that you become really tired. When you exercise, drink lots of fluids. […] Drink at least eight 12-ounce glasses of water a day during warm weather. […] Reduce or avoid stress. Talk to your doctor if you’re depressed or have problems with your family or job. […] Treat any infection as soon as it occurs. When in doubt, see your doctor. […] Wear warm clothes outside in cold weather and inside in air-conditioned rooms during hot weather. Also, don’t swim in cold water. […] Try to be positive about yourself.

• #59

  https://www.nhs.uk/conditions/sickle-cell-disease/living-with/

  There are a number of things you can do, and precautions you can take, to stay as healthy as possible if you have sickle cell disease. […] You’ll usually be given antibiotics and advised to have vaccinations to help prevent most serious infections, but there are also things you can do to reduce your risk. […] Make sure you speak to your GP or healthcare team if you’re planning to travel aboard, as this may mean you need extra medication or vaccinations. […] It’s important to let your healthcare team know if you need to have an operation under general anaesthetic at any point. […] This is because general anaesthetic can cause problems for people with sickle cell disease, including an increased risk of experiencing a sickle cell crisis. […] You may need close monitoring during surgery to make sure you’re getting enough fluids and oxygen and are kept warm. […] Sometimes you may need a blood transfusion beforehand to reduce the risk of complications.

• #60

  https://www.nhs.uk/conditions/sickle-cell-disease/living-with/

  There are a number of things you can do, and precautions you can take, to stay as healthy as possible if you have sickle cell disease. […] You’ll usually be given antibiotics and advised to have vaccinations to help prevent most serious infections, but there are also things you can do to reduce your risk. […] Make sure you speak to your GP or healthcare team if you’re planning to travel aboard, as this may mean you need extra medication or vaccinations. […] It’s important to let your healthcare team know if you need to have an operation under general anaesthetic at any point. […] This is because general anaesthetic can cause problems for people with sickle cell disease, including an increased risk of experiencing a sickle cell crisis. […] You may need close monitoring during surgery to make sure you’re getting enough fluids and oxygen and are kept warm. […] Sometimes you may need a blood transfusion beforehand to reduce the risk of complications.

• #61

  https://www.nhs.uk/conditions/sickle-cell-disease/living-with/

  There are a number of things you can do, and precautions you can take, to stay as healthy as possible if you have sickle cell disease. […] You’ll usually be given antibiotics and advised to have vaccinations to help prevent most serious infections, but there are also things you can do to reduce your risk. […] Make sure you speak to your GP or healthcare team if you’re planning to travel aboard, as this may mean you need extra medication or vaccinations. […] It’s important to let your healthcare team know if you need to have an operation under general anaesthetic at any point. […] This is because general anaesthetic can cause problems for people with sickle cell disease, including an increased risk of experiencing a sickle cell crisis. […] You may need close monitoring during surgery to make sure you’re getting enough fluids and oxygen and are kept warm. […] Sometimes you may need a blood transfusion beforehand to reduce the risk of complications.

• #62 Sickle Cell Disease (SCD) Treatment & Management: Approach Considerations, Hydroxyurea Therapy, Transfusion

  https://emedicine.medscape.com/article/205926-treatment

  The National Institutes of Health advises that optimal care for patients with sickle cell disease (SCD), including preventive care, is best achieved through treatment in clinics that specialize in the care of SCD. All patients with SCD should have a principal health care provider, who should either be a hematologist or be in frequent consultation with one. […] An expert panel has released evidence-based guidelines for the treatment of SCD, including a strong recommendation that hydroxyurea and long-term, periodic blood transfusions should be used more often to treat patients. Other recommendations include the following: Use of daily oral prophylactic penicillin up to age 5; Annual transcranial Doppler examinations between the ages of 2 and 16 years in patients with sickle cell anemia; Long-term transfusion therapy to prevent stroke in children with abnormal transcranial Doppler velocity (200 cm/s); In patients with sickle cell anemia, preoperative transfusion therapy to increase hemoglobin levels to 10 g/dL; Rapid initiation of opioids for the treatment of severe pain associated with a vaso-occlusive crisis; Use of analgesics and physical therapy for the treatment of avascular necrosis.

• #63 What Is Sickle Cell Anemia? Symptoms, Causes, Diagnosis, Treatment, and Prevention

  https://www.everydayhealth.com/sickle-cell-anemia/guide/

  There is no known way to prevent sickle cell anemia. However, you can find out if you carry the gene mutation through a simple blood test. If you don’t have sickle cell anemia but people in your family do, you may have inherited one sickle cell gene; this is referred to as sickle cell trait. If you carry the gene, your chances increase for having a child with either the disease or sickle cell trait. […] To be born with sickle cell anemia, a child must inherit two sickle cell gene mutations, one from each parent. […] If you carry the mutated gene and your partner carries the mutated gene, there is a 1 in 4 chance that your child will have sickle cell anemia; a 1 in 2 chance they will become a carrier; and a 1 in 4 chance they will not acquire the disease. […] If one of you carries the trait, there is a 1 in 2 chance that your child will also carry the trait but there is no chance they can get the disease.

• #64 Screening and Prevention of Sickle Cell Disease | Hematology-Oncology Associates of CNY

  https://www.hoacny.com/patient-resources/blood-disorders/what-sickle-cell-disease/screening-and-prevention-sickle-cell

  People who do not know whether they carry an abnormal hemoglobin gene can ask their doctor to have their blood tested. […] Couples who are planning to have children and know that they are at risk of having a child with sickle cell disease (SCD) may want to meet with a genetics counselor. A genetics counselor can answer questions about the risk and explain the choices that are available.

• #65 Sickle cell anemia – Symptoms & causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/symptoms-causes/syc-20355876

  If you carry the sickle cell trait, it can help to see a genetic counselor before you get pregnant. A counselor can help you understand your risk of having a child with sickle cell anemia. You also can learn about possible treatments, preventive measures and reproductive options.

• #66 What Is Sickle Cell Anemia? Symptoms, Causes, Diagnosis, Treatment, and Prevention

  https://www.everydayhealth.com/sickle-cell-anemia/guide/

  Genetic counselors can help you review your family history, navigate your blood test results, and guide you in making informed decisions with regard to family planning. […] A few simple steps can help prevent and reduce the number of pain crises: Drinking plenty of water, avoiding getting too hot or cold, avoiding exposure to high altitudes, avoiding places or situations where there might be low oxygen levels, such as mountain climbing or extremely intense exercise.

• #67 Screening and Prevention of Sickle Cell Disease | Hematology-Oncology Associates of CNY

  https://www.hoacny.com/patient-resources/blood-disorders/what-sickle-cell-disease/screening-and-prevention-sickle-cell

  People who do not know whether they carry an abnormal hemoglobin gene can ask their doctor to have their blood tested. […] Couples who are planning to have children and know that they are at risk of having a child with sickle cell disease (SCD) may want to meet with a genetics counselor. A genetics counselor can answer questions about the risk and explain the choices that are available.

• #68 What Is Sickle Cell Anemia? Symptoms, Causes, Diagnosis, Treatment, and Prevention

  https://www.everydayhealth.com/sickle-cell-anemia/guide/

  There is no known way to prevent sickle cell anemia. However, you can find out if you carry the gene mutation through a simple blood test. If you don’t have sickle cell anemia but people in your family do, you may have inherited one sickle cell gene; this is referred to as sickle cell trait. If you carry the gene, your chances increase for having a child with either the disease or sickle cell trait. […] To be born with sickle cell anemia, a child must inherit two sickle cell gene mutations, one from each parent. […] If you carry the mutated gene and your partner carries the mutated gene, there is a 1 in 4 chance that your child will have sickle cell anemia; a 1 in 2 chance they will become a carrier; and a 1 in 4 chance they will not acquire the disease. […] If one of you carries the trait, there is a 1 in 2 chance that your child will also carry the trait but there is no chance they can get the disease.

• #69 Sickle Cell Disease | WHO | Regional Office for Africa

  https://www.afro.who.int/health-topics/sickle-cell-disease

  Sickle-cell disease can be managed by simple procedures including: high fluid intake, healthy diet, folic acid supplementation, pain medication, vaccination and antibiotics for the prevention and treatment of infections, a number of other therapeutic measures. […] The most cost-effective strategy for reducing the burden of haemoglobin disorders is to complement disease management with prevention programmes. Inexpensive and reliable blood tests can identify couples at risk for having affected children. This screening is especially opportune before marriage or pregnancy, allowing couples to discuss the health of their family. Subsequent genetic counselling informs trait carriers of risks that the condition may be passed along to their children, the treatment needed, if affected by a haemoglobin disorder, and the possible options for the couple.

• #70 Beliefs negatively mediate adolescents’ and adults’ knowledge about sickle cell disease prevention and relationship choices; a one-center exploratory study in Central Region, Ghana | Archives of Public Health | Full Text

  https://archpublichealth.biomedcentral.com/articles/10.1186/s13690-024-01379-1

  Sickle cell disease (SCD) is scientifically preventable through intentional spousal selection. […] One of the effective interventional strategies that could be harnessed in SCD prevention is educational efforts targeting young people who are about starting to make reproductive decisions. […] Interventional strategies that prioritize increased knowledge to address long-held misconceptions and attitudes about SCD, and their attendant reproductive consequences could hypothetically yield greater chances of reducing SCD burden. […] An overwhelming majority (91.2%) of participants were in favor of mandatory sickle cell testing for all couples prior to marriage. […] Moreover, the majority of participants (87.8%) indicated that sickle cell testing for all newborns should be mandatory. […] Additionally, 60.1% of the participants disagreed with the notion that having a sickle cell condition should not deter them from marrying someone else with the same condition.

• #71 Sickle Cell Disease (Sickle Cell Anemia) – Causes & Types

  https://www.webmd.com/a-to-z-guides/what-is-sickle-cell-disease

  Sickle cell disease is a genetic condition that people are born with, so there isn’t a way to prevent it. By law, all babies born in the U.S. are screened for sickle cell at birth. A child must inherit a sickle cell gene from both parents to develop the disease. Genetic testing allows doctors to diagnose sickle cell disease in as few as 10 weeks of pregnancy. Early diagnosis is important for managing the disease because children can develop symptoms early in life.

• #72 Complications & Treatments – Sickle Cell Disease Association of America Inc.

  https://www.sicklecelldisease.org/treatments/

  Health maintenance for patients with sickle cell disease starts with early diagnosis, preferably in the newborn period and includes penicillin prophylaxis, vaccination against pneumococcus bacteria and folic acid supplementation. […] Preventative care and screening can help you stay healthy and keep you out of the hospital. Examples include sticking to an appointment schedule with all your providers hematologist, primary care provider, optometrist, etc; staying up to date on vaccines and making it a priority to get your flu shot and COVID-19 booster every year; completing routine tests and screenings as recommended by your provider; and following treatment instructions carefully and asking questions if they come up. […] Individuals with SCD who wish to have children may need extra support as they consider starting a family and/or navigate a pregnancy. Starting discussions with your provider early can help you make informed choices about family planning. Genetic counseling can help you and your partner understand your unique situation and how it may impact your future children. Those who are already pregnant may wish to pursue advanced genetic tests that can tell you whether your baby has sickle cell prior to their birth.

• #73 Sickle Cell Anemia: Symptoms, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/4579-sickle-cell-anemia

  Sickle cell anemia is an inherited disorder that you cant prevent. You can have a blood test to find out if you have the genetic change that causes sickle cell anemia. […] Starting in 2006, all babies born in the U.S. have sickle cell anemia tests right after theyre born. Early diagnosis and treatment are why fewer babies and young children born in the United States die of sickle cell anemia.

• #74

  https://www.healthychildren.org/English/health-issues/conditions/chronic/Pages/sickle-cell-disease-in-children.aspx

  Learn more about sickle cell disease and how early and ongoing care and treatment helps manage symptoms and prevent complications. […] Prompt diagnosis before infants show any symptoms allows infants with sickle cell disease to get early treatment and parents to learn about ways to help lower the risk of serious health problems. […] National guidelines recommend newborns diagnosed with severe sickle cell disease (SS and S beta zero thalassemia) receive antibiotics by mouth twice a day until they are 5 years old. […] Research shows that children with sickle cell anemia given twice daily penicillin (an antibiotic) had 84% less risk of Streptococcus pneumoniae bacterial infection, which can cause serious conditions like pneumonia and meningitis. […] Early diagnosis and starting antibiotics shortly after birth for those with severe forms of sickle cell have greatly improved childhood survival. […] The U.S. Food Drug Administration (FDA) has approved medications that can help prevent sickle cell complications in children. These include hydroxyurea (ideally started at 9 months of age) and L-glutamine (may start at 5 years of age).

• #75 Prevention of Sickle Cell Disease | PatientsEngage

  https://www.patientsengage.com/conditions/sickle-cell-disease/prevention

  There is no known way to prevent this genetic disease. […] Avoid consanguineous marriages and encourage blood testing before marriage. […] Screening tests such as Prenatal diagnosis or new born screening can be done to check if baby has sickle cell disease for prompt intervention and treatment. […] We must keep check on symptoms for sickle cell disease and early diagnosis of it.

• #76 Sickle Cell Disease, a Review

  https://www.mdpi.com/2673-6357/3/2/24

  Sickle cell disease can be prevented prenatally, diagnosed in utero or in the newborn period by screening, or be detected at any time during life. […] Newborn screening at birth is used to detect sickle cell disease, prior to the development of any symptoms. This has been shown to be effective in reducing death. […] Vaccination with pneumococcal, meningococcal, and influenza, in addition to general recommended immunizations, reduces occurrence of major infections. […] Prevention and treatment of infections. […] Prevention of the complications including various organ damages associated with the disease. […] Prevention of stroke. […] Early detection and treatment of pulmonary hypertension.

• #77 Sickle Cell Disease Prevention – North Cyprus IVF

  https://www.lowcostivf.net/sickle-cell-disease-prevention/

  Sickle cell anemia, or sometimes referred to as Sickle cell disease is an inherited type of anemia where red blood cells (which are responsible for carrying oxygen from the lungs to the rest of the body) are sickle shaped. […] At this point, one can ask themselves, can sickle cell disease be treated? Or at least prevented? Luckily, with advancements in technology and medicine, it is possible to completely avoid passing sickle cell anemia down to your children! Pre-Implantation Genetic Diagnosis allows us to screen embryos for majority of genetic disorders as well as single gene disorders such as the sickle cell anemia. […] PGD technique is offered as part of an IVF treatment program. Therefore, if you are at risk of passing sickle cell down to your offspring, IVF treatment will be required in order to prevent the next generation from sickle cell disease.

• #78 Sickle Cell Disease Prevention – North Cyprus IVF

  https://www.lowcostivf.net/sickle-cell-disease-prevention/

  Sickle cell anemia, or sometimes referred to as Sickle cell disease is an inherited type of anemia where red blood cells (which are responsible for carrying oxygen from the lungs to the rest of the body) are sickle shaped. […] At this point, one can ask themselves, can sickle cell disease be treated? Or at least prevented? Luckily, with advancements in technology and medicine, it is possible to completely avoid passing sickle cell anemia down to your children! Pre-Implantation Genetic Diagnosis allows us to screen embryos for majority of genetic disorders as well as single gene disorders such as the sickle cell anemia. […] PGD technique is offered as part of an IVF treatment program. Therefore, if you are at risk of passing sickle cell down to your offspring, IVF treatment will be required in order to prevent the next generation from sickle cell disease.

• #79 Sickle Cell Disease Prevention – North Cyprus IVF

  https://www.lowcostivf.net/sickle-cell-disease-prevention/

  Processing the blood samples take about two months. Once we have finished designing the PGD probe, then we will contact you to prepare you for your treatment. […] The initial parts of the treatment are very similar to standard IVF/ICSI treatment where the female patient uses medication to stimulate her ovaries in order to produce multiple eggs. […] Sickle cell disease prevention is offered as part of an IVF treatment which involves Pre-Implantation Genetic Diagnosis (PGD) on the embryos which will be created in-vitro after the IVF protocol.

• #80 Frontiers | Sickle cell disease: a comparative perspective on global and national initiatives

  https://www.frontiersin.org/journals/hematology/articles/10.3389/frhem.2024.1457158/full

  Sickle cell disease (SCD) is a genetic disorder caused by mutations in the β-globin chain of hemoglobin, leading to abnormal red blood cells, severe pain, anemia, organ damage, and increased infection risk. […] Comprehensive healthcare management (CHCM) emphasizes patient education, nutrition, prophylactic antibiotics, and early intervention to reduce morbidity and improve quality of life. […] National initiatives, such as Nigeria’s National Sickle Cell Disease Control Program and India’s National Sickle Cell Anaemia Elimination Mission, aim to improve outcomes through early screening, public education, and enhanced healthcare access. […] The WHO’s guidance frameworks for SCD in Africa and globally aim to enhance care through strategic policies and advocacy. […] The SICKLE technical package integrates interventions, education, and community empowerment.

• #81 Frontiers | Sickle cell disease: a comparative perspective on global and national initiatives

  https://www.frontiersin.org/journals/hematology/articles/10.3389/frhem.2024.1457158/full

  These frameworks emphasize early diagnosis through newborn screening, public education, and improved access to healthcare services. […] Nigeria’s National Sickle Cell Disease Control Program has made substantial strides in improving the management and outcomes of sickle cell disease (SCD). This program focuses on comprehensive strategies, including early screening, public education, and enhanced healthcare access. […] The National Sickle Cell Anaemia Elimination Mission, launched by the Prime Minister of India on July 1, 2023, in Shahdol, Madhya Pradesh, integrates with existing National Health Mission mechanisms to optimize resources and avoid duplication of efforts. […] The ongoing national mission aims to screen approximately seventy million people from fiscal year 2023-24 to 2025-26, achieving significant milestones such as screening over thirty-three million seven hundred thousand individuals, confirming nine hundred seventy-five thousand individuals as sickle cell trait carriers, diagnosing one hundred forty thousand with SCD, and verifying the status of over three hundred fifty-nine thousand individuals.

• #82 Frontiers | Sickle cell disease: a comparative perspective on global and national initiatives

  https://www.frontiersin.org/journals/hematology/articles/10.3389/frhem.2024.1457158/full

  Sickle cell disease (SCD) is a genetic disorder caused by mutations in the β-globin chain of hemoglobin, leading to abnormal red blood cells, severe pain, anemia, organ damage, and increased infection risk. […] Comprehensive healthcare management (CHCM) emphasizes patient education, nutrition, prophylactic antibiotics, and early intervention to reduce morbidity and improve quality of life. […] National initiatives, such as Nigeria’s National Sickle Cell Disease Control Program and India’s National Sickle Cell Anaemia Elimination Mission, aim to improve outcomes through early screening, public education, and enhanced healthcare access. […] The WHO’s guidance frameworks for SCD in Africa and globally aim to enhance care through strategic policies and advocacy. […] The SICKLE technical package integrates interventions, education, and community empowerment.

• #83 Frontiers | Sickle cell disease: a comparative perspective on global and national initiatives

  https://www.frontiersin.org/journals/hematology/articles/10.3389/frhem.2024.1457158/full

  These frameworks emphasize early diagnosis through newborn screening, public education, and improved access to healthcare services. […] Nigeria’s National Sickle Cell Disease Control Program has made substantial strides in improving the management and outcomes of sickle cell disease (SCD). This program focuses on comprehensive strategies, including early screening, public education, and enhanced healthcare access. […] The National Sickle Cell Anaemia Elimination Mission, launched by the Prime Minister of India on July 1, 2023, in Shahdol, Madhya Pradesh, integrates with existing National Health Mission mechanisms to optimize resources and avoid duplication of efforts. […] The ongoing national mission aims to screen approximately seventy million people from fiscal year 2023-24 to 2025-26, achieving significant milestones such as screening over thirty-three million seven hundred thousand individuals, confirming nine hundred seventy-five thousand individuals as sickle cell trait carriers, diagnosing one hundred forty thousand with SCD, and verifying the status of over three hundred fifty-nine thousand individuals.

• #84 Burkina Faso Prevention diagnosis and treatment of sickle cell disease

  https://www.fondationpierrefabre.org/en/our-programmes/combatting-sickle-cell-disease/prevention-diagnosis-treatment-sickle-cell-disease/

  As one of the worlds 10 least-developed countries, Burkina Faso has a record rate of sickle cell disease: 2% of newborns will suffer from major syndromes. Yet no national programme has been implemented to fight this scourge. As a result, most cases are discovered too late, based on complications from the disease. […] The first objective is to raise awareness by creating a dedicated information centre. The second is to improve access to care by creating a pilot centre inside Saint Camille Hospital in Ouagadougou, the main activities of which will be doctors visits, a day clinic and orthopaedic surgery. The last is to offer systematic neonatal diagnosis in the Saint Camille Hospital maternity ward. Because only screening done before the age of six months can effectively reduce the Sickle cell disease mortality rate.

• #85 Burkina Faso Prevention diagnosis and treatment of sickle cell disease

  https://www.fondationpierrefabre.org/en/our-programmes/combatting-sickle-cell-disease/prevention-diagnosis-treatment-sickle-cell-disease/

  To train healthcare professionals, reinforce screening and improve treatments. […] Roll out awareness-raising initiatives. […] The second phase of the programme aims to contribute to the definition of a national policy to combat sickle-cell disease. […] This multi-country project must address the major challenge of reducing morbidity and mortality linked to sickle cell disease by acting on several levels: Detect, treat, train, raise awareness and support public authorities.

• #86 Frontiers | Sickle cell disease: a comparative perspective on global and national initiatives

  https://www.frontiersin.org/journals/hematology/articles/10.3389/frhem.2024.1457158/full

  Sickle cell disease (SCD) is a genetic disorder caused by mutations in the β-globin chain of hemoglobin, leading to abnormal red blood cells, severe pain, anemia, organ damage, and increased infection risk. […] Comprehensive healthcare management (CHCM) emphasizes patient education, nutrition, prophylactic antibiotics, and early intervention to reduce morbidity and improve quality of life. […] National initiatives, such as Nigeria’s National Sickle Cell Disease Control Program and India’s National Sickle Cell Anaemia Elimination Mission, aim to improve outcomes through early screening, public education, and enhanced healthcare access. […] The WHO’s guidance frameworks for SCD in Africa and globally aim to enhance care through strategic policies and advocacy. […] The SICKLE technical package integrates interventions, education, and community empowerment.

• #87 Sickle Cell Disease | WHO | Regional Office for Africa

  https://www.afro.who.int/health-topics/sickle-cell-disease

  WHO will: increase awareness of the international community of the global burden of these disorders; promote equitable access to health services; provide technical support to countries for the prevention and management of these disorders; and promote and support research to improve quality of life for those affected.

• #88 Sickle Cell Disease | WHO | Regional Office for Africa

  https://www.afro.who.int/health-topics/sickle-cell-disease

  Sickle-cell disease can be managed by simple procedures including: high fluid intake, healthy diet, folic acid supplementation, pain medication, vaccination and antibiotics for the prevention and treatment of infections, a number of other therapeutic measures. […] The most cost-effective strategy for reducing the burden of haemoglobin disorders is to complement disease management with prevention programmes. Inexpensive and reliable blood tests can identify couples at risk for having affected children. This screening is especially opportune before marriage or pregnancy, allowing couples to discuss the health of their family. Subsequent genetic counselling informs trait carriers of risks that the condition may be passed along to their children, the treatment needed, if affected by a haemoglobin disorder, and the possible options for the couple.

• #89 Penicillin Prophylaxis in Children with Sickle Cell Disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3018247/

  The authors concluded penicillin prophylaxis would drastically reduce the risk of pneumococcal infection in children with SCA, especially those under the age of three. […] In the PROPS study, all patients received penicillin V potassium 125 mg twice daily. This has become the dose recommended for children younger than 5 years of age. […] It is recommended that all children younger than 5 years with SCD take daily prophylactic antibiotics. All newborns screened for SCD and those who test positive should be started on prophylactic penicillin as early as possible. […] Children with recurrent invasive pneumococcal infections should receive penicillin prophylaxis indefinitely. […] Education for families of children with sickle cell disease is paramount to the successful management of a child with SCD.

• #90 Preventing Sickle Cell Anemia Complications in Children | VitalSigns | CDC

  https://www.cdc.gov/vitalsigns/sickle-cell-anemia/index.html

  Childhood stroke and other sickle cell anemia complications are preventable—not inevitable. Everyone has a role to play in prevention. Consider actions to increase TCD screening and hydroxyurea use among children with sickle cell anemia. […] Healthcare Providers […] Implement actions that track TCD screenings and follow-up in electronic health records. […] Integrate screening into a single, comprehensive sickle cell visit whenever possible. […] Educate patients and families about screening. […] Patients and Families […] Learn the importance of getting an annual TCD screening. […] Talk to your provider about TCD results and next steps if the results indicate a greater stroke risk. […] Community-Based Organizations/Partners […] Develop patient and provider resources to improve understanding of the importance of annual TCD screening to prevent childhood stroke. […] Connect patients and families with resources and tools to schedule screening appointments, and support transportation needs by identifying financial assistance.

• #91 Preventing Sickle Cell Anemia Complications in Children | VitalSigns | CDC

  https://www.cdc.gov/vitalsigns/sickle-cell-anemia/index.html

  Healthcare Providers […] Become familiar with guidelines on prescribing hydroxyurea and studies showing the safety of hydroxyurea. […] Address practice barriers, such as lack of support staff or time to provide hydroxyurea counseling. Incorporate reminders into the electronic health record. […] Patients and Families […] Learn about hydroxyurea and take as directed. Talk to your provider about the importance of regular monitoring and pros and cons compared with other treatments. […] Community-Based Organizations/Partners […] Share patient resources about the benefits and safety of hydroxyurea. […] Develop tools to help patients take medication as directed (for example, mobile apps with reminders). […] Develop provider resources to increase familiarity with hydroxyurea safety and prescribing guidelines.

• #92 Preventing Sickle Cell Anemia Complications in Children | VitalSigns | CDC

  https://www.cdc.gov/vitalsigns/sickle-cell-anemia/index.html

  Childhood stroke and other sickle cell anemia complications are preventable—not inevitable. Everyone has a role to play in prevention. Consider actions to increase TCD screening and hydroxyurea use among children with sickle cell anemia. […] Healthcare Providers […] Implement actions that track TCD screenings and follow-up in electronic health records. […] Integrate screening into a single, comprehensive sickle cell visit whenever possible. […] Educate patients and families about screening. […] Patients and Families […] Learn the importance of getting an annual TCD screening. […] Talk to your provider about TCD results and next steps if the results indicate a greater stroke risk. […] Community-Based Organizations/Partners […] Develop patient and provider resources to improve understanding of the importance of annual TCD screening to prevent childhood stroke. […] Connect patients and families with resources and tools to schedule screening appointments, and support transportation needs by identifying financial assistance.

• #93 Preventing Sickle Cell Anemia Complications in Children | VitalSigns | CDC

  https://www.cdc.gov/vitalsigns/sickle-cell-anemia/index.html

  Healthcare Providers […] Become familiar with guidelines on prescribing hydroxyurea and studies showing the safety of hydroxyurea. […] Address practice barriers, such as lack of support staff or time to provide hydroxyurea counseling. Incorporate reminders into the electronic health record. […] Patients and Families […] Learn about hydroxyurea and take as directed. Talk to your provider about the importance of regular monitoring and pros and cons compared with other treatments. […] Community-Based Organizations/Partners […] Share patient resources about the benefits and safety of hydroxyurea. […] Develop tools to help patients take medication as directed (for example, mobile apps with reminders). […] Develop provider resources to increase familiarity with hydroxyurea safety and prescribing guidelines.

• #94 Prevention and Treatment of SCD Complications | Sickle Cell Disease (SCD) | CDC

  https://www.cdc.gov/sickle-cell/about/prevention-and-treatment.html

  The focus of sickle cell disease (SCD) management is preventing and treating pain episodes and complications. […] Prevention strategies include lifestyle behaviors, medical screenings, and interventions to prevent infections and other complications. […] Several medications can be taken regularly to prevent or reduce the occurrence of complications. […] Simple steps to prevent harmful infections include the following: Wash your hands often. Washing hands with soap and clean water many times each day is one of the best ways people with SCD, their family members, and other caregivers can help prevent an infection. […] Vaccines can protect against harmful infections. It is important that children with SCD get all regular childhood vaccines. […] Penicillin greatly reduces the risk of infections in people with HbSS (the most common form of SCD in which people inherit two hemoglobin S genes) and has been shown to be even more effective when it is started earlier.

• #95 Sickle Cell Disease, a Review

  https://www.mdpi.com/2673-6357/3/2/24

  Sickle cell disease can be prevented prenatally, diagnosed in utero or in the newborn period by screening, or be detected at any time during life. […] Newborn screening at birth is used to detect sickle cell disease, prior to the development of any symptoms. This has been shown to be effective in reducing death. […] Vaccination with pneumococcal, meningococcal, and influenza, in addition to general recommended immunizations, reduces occurrence of major infections. […] Prevention and treatment of infections. […] Prevention of the complications including various organ damages associated with the disease. […] Prevention of stroke. […] Early detection and treatment of pulmonary hypertension.

• #96 Beliefs negatively mediate adolescents’ and adults’ knowledge about sickle cell disease prevention and relationship choices; a one-center exploratory study in Central Region, Ghana | Archives of Public Health | Full Text

  https://archpublichealth.biomedcentral.com/articles/10.1186/s13690-024-01379-1

  Sickle cell disease (SCD) is scientifically preventable through intentional spousal selection. […] One of the effective interventional strategies that could be harnessed in SCD prevention is educational efforts targeting young people who are about starting to make reproductive decisions. […] Interventional strategies that prioritize increased knowledge to address long-held misconceptions and attitudes about SCD, and their attendant reproductive consequences could hypothetically yield greater chances of reducing SCD burden. […] An overwhelming majority (91.2%) of participants were in favor of mandatory sickle cell testing for all couples prior to marriage. […] Moreover, the majority of participants (87.8%) indicated that sickle cell testing for all newborns should be mandatory. […] Additionally, 60.1% of the participants disagreed with the notion that having a sickle cell condition should not deter them from marrying someone else with the same condition.

• #97 Why I prefer sickle cell disease prevention to the search for a curecaret iconEnvelope icon

  https://sicklecellanemianews.com/columns/why-i-prefer-sickle-cell-disease-prevention-search-cure/

  Prevention is the most effective strategy to reduce incidence, mortality rates […] The reason is because prevention is paramount to reducing the incidence of sickle cell disease worldwide. […] I still support sickle cell disease prevention instead of gambling and waiting for a universal cure that might never come. […] It is crucial to be aware of your genotype and select a compatible partner if you are unable to pursue other methods to prevent the birth of children with sickle cell disease. […] Choosing a partner with a compatible genotype is the most effective and economical way for people from low socioeconomic regions. […] Prevention emerges as the most effective strategy to reduce the incidence and mortality rates of sickle cell disease, especially considering the progressive nature of the disorder and the impact of sickle cell-related complications on quality of life.

• #98 Preventing Sickle Cell Anemia Complications in Children | VitalSigns | CDC

  https://www.cdc.gov/vitalsigns/sickle-cell-anemia/index.html

  Far too few patients are receiving these potentially lifesaving prevention measures, recommended by an expert panel in 2014. […] The healthcare system can promote tailored strategies to reduce barriers and increase TCD screening and hydroxyurea use among children with sickle cell anemia. […] Preventing complications among children with sickle cell anemia includes recognizing where racism exists in the healthcare setting. It also means deciding to take steps to combat it. Healthcare providers can educate themselves, their colleagues, and the institutions they work with about the specialized needs of people with sickle cell anemia, including how racism is a barrier to better health. They can advocate for and listen to their patients to better understand their needs and support them on their journey to better health.

• #99 Burkina Faso Prevention diagnosis and treatment of sickle cell disease

  https://www.fondationpierrefabre.org/en/our-programmes/combatting-sickle-cell-disease/prevention-diagnosis-treatment-sickle-cell-disease/

  To train healthcare professionals, reinforce screening and improve treatments. […] Roll out awareness-raising initiatives. […] The second phase of the programme aims to contribute to the definition of a national policy to combat sickle-cell disease. […] This multi-country project must address the major challenge of reducing morbidity and mortality linked to sickle cell disease by acting on several levels: Detect, treat, train, raise awareness and support public authorities.

Zobacz więcej