Materiały źródłowe

• #1 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.

• #2 Sickle cell anemia – Diagnosis & treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/diagnosis-treatment/drc-20355882

  During global health threats, such as the COVID-19 pandemic, people with sickle cell anemia should take extra precautions. These include staying at home as much as possible and for those who are eligible, getting vaccinated. […] Management of sickle cell anemia is usually aimed at avoiding pain episodes, relieving symptoms and preventing complications. Treatments might include medicines and blood transfusions. […] Regular blood transfusions can decrease stroke risk. […] Clinical trials are ongoing to address stem cell transplantation in adults and gene therapies.

• #3 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. […] The authors concluded penicillin prophylaxis would drastically reduce the risk of pneumococcal infection in children with SCA, especially those under the age of three. […] 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 less than 5 years of age should receive oral penicillin 125 mg twice daily, and the dose should be increased to 250 mg twice daily for children older than 5 years. […] Education for families of children with sickle cell disease is paramount to the successful management of a child with SCD.

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

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

  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. […] Offer hydroxyurea therapy to infants, children, and adolescents with sickle cell anemia regardless of clinical severity to reduce sickle cell disease-related complications. […] 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. […] Initiate hydroxyurea therapy in adults with sickle cell anemia who have one or more of the following: three or more moderate to severe pain crises in a 12-month period; daily sickle cell pain that affects quality of life; severe or recurrent acute chest syndrome; or severe symptomatic chronic anemia.

• #5 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.

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

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

  In patients with sickle cell anemia, preoperative transfusion therapy to increase hemoglobin levels to 10 g/dL […] Regular blood transfusions are used for primary and secondary stroke prevention in children with SCD. […] 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. […] Hydroxyurea may decrease the frequency and severity of pain episodes. […] Penicillin prophylaxis significantly reduces the incidence of infection with encapsulated organisms in particular, S pneumoniae and may decrease the mortality rate.

• #7 Sickle cell anemia – Diagnosis & treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/diagnosis-treatment/drc-20355882

  Children with sickle cell anemia might receive penicillin from about 2 months old to 5 years old, or longer. This medicine can help prevent infections, such as pneumonia, which can be life-threatening to children with sickle cell anemia. […] Adults who have sickle cell anemia might need to take penicillin throughout their lives if they’ve had pneumonia or surgery to remove the spleen. […] Childhood vaccinations are important for preventing disease in all children. Vaccinations are even more important for children with sickle cell anemia because their infections can be severe. […] Your child’s healthcare team should make sure that your child gets all the recommended childhood vaccinations. These include vaccines against pneumonia, meningitis, hepatitis B and a yearly flu shot. Vaccines also are important for adults with sickle cell anemia.

• #8 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. Dosing and monitoring protocols are outlined in the CANHAEM consensus statement.

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

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

  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. […] Ensure that all persons with SCD have been vaccinated against Streptococcus pneumoniae. […] Ensure completion of pneumococcal vaccination series before discontinuation. […] 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) TCD findings to a subspecialist with expertise in long-term transfusion therapy aimed at preventing stroke.

• #10 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.

• #11 Sickle Cell Anemia | Sepsis Alliance

  https://www.sepsis.org/sepsisand/sickle-cell-anemia/

  Infection prevention for people with sickle cell anemia is essential. There are standard infection prevention procedures, such as frequent and thorough hand washing, getting appropriate vaccines, and avoiding people who are sick. But in addition, people with sickle cell anemia should: […] The CDC reported fewer deaths among children with sickle cell anemia 42% fewer when the pneumococcal vaccine became available in the U.S. The vaccine helps reduce infections like pneumococcal pneumonia and pneumococcal meningitis. […] Children with sickle cell anemia often take preventative (prophylactic) penicillin every day up to the age of five. This is to prevent infections. If they cant take penicillin, erythromycin is another option.

• #12 Sickle Cell Disease | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/sickle-cell-disease

  Most children with sickle cell disease must: […] Take preventative dosing penicillin until at least age 5 […] Complete all routine childhood vaccinations and additional sickle cell specific vaccinations […] Maintain vigilance regarding fevers and signs of infection. […] Hydroxyurea is an oral medication that causes the body to produce fetal hemoglobin (HbF), a type of hemoglobin normally found only in fetuses and very young children. Increasing the healthy fetal hemoglobin can significantly reduce the side effects and complications of sickle cell disease. […] Blood transfusions may be given acutely in order to treat severe anemia, acute chest syndrome, and other life-threatening complications of sickle cell disease. Children with sickle cell disease may receive chronic blood transfusions to prevent complications such as stroke.

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

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

  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. […] Far too few patients are receiving these potentially lifesaving prevention measures, recommended by an expert panel in 2014. […] Everyone can help improve care for people with sickle cell anemia by taking steps to address racism and prejudice. The healthcare system can promote tailored strategies to reduce barriers and increase TCD screening and hydroxyurea use among children with sickle cell anemia.

• #14 Hydroxyurea significantly reduces infections in children with sickle cell anemia: IU News

  https://news.iu.edu/live/news/33918-hydroxyurea-significantly-reduces-infections-in

  Hydroxyurea significantly reduces infections in children with sickle cell anemia. […] Hydroxyurea treatment resulted in a remarkable 60% reduction in severe or invasive infections, including malaria, bacteremia, respiratory tract infections and gastroenteritis, among Ugandan children with sickle cell anemia. […] Our investigation provides powerful justifications for hydroxyurea’s use in children with sickle cell anemia in Africa. […] We hope our evidence will encourage ministries of health to continue supporting and expanding access to hydroxyurea for young patients who can greatly benefit from the treatment. […] We believe incorporating hydroxyurea treatment as the standard of care for sickle cell anemia across Africa will not only reduce infections but will more importantly save countless lives.

• #15 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. […] The goals of treatment are symptom control and management of disease complications. Treatment strategies include the following seven goals: […] Prevention and treatment of infections […] 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)

• #16 JMIR Research Protocols – Primary Prevention of Stroke in Children With Sickle Cell Anemia in Nigeria: Protocol for a Mixed Methods Implementation Study in a Community Hospital

  https://www.researchprotocols.org/2022/6/e37927/

  In Nigeria, approximately 150,000 children with sickle cell anemia (SCA) are born annually, accounting for more than half of all SCA births worldwide. Without intervention, about 11% of children with SCA will develop a stroke before their 20th birthday. Evidence-based practices for primary stroke prevention include screening for abnormal transcranial Doppler (TCD) measurements coupled with regular blood transfusion therapy for at least one year, followed by hydroxyurea (HU) therapy indefinitely. […] In high-income countries, evidence-based practices for primary stroke prevention in children with SCA involve screening for abnormal transcranial Doppler (TCD) ultrasound velocity coupled with regular blood transfusion therapy for at least one year followed by hydroxyurea (HU) therapy indefinitely. This strategy has decreased the risk of stroke by 92%, leading to a 10-fold drop in stroke incidence from 0.67 to 0.06 strokes per 100 patient-years.

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

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

  Yearly visits to an eye doctor beginning at age 10 to look for damage to the retina (the part of your eye that senses light and sends images to your brain) are important for people with SCD to avoid vision loss. […] Children who are at risk for stroke can be identified using a special type of exam called transcranial Doppler ultrasound (TCD). […] If the child is found to have an abnormal TCD, a doctor might recommend frequent blood transfusions (a procedure in which new blood is put into a person’s body through a small plastic tube inserted into a person’s blood vessels) to help prevent a stroke. […] Blood transfusions may be used to treat severe anemia. […] A sudden worsening of anemia resulting from infection or enlargement of the spleen (an organ in the upper left side of the abdomen) is a common reason for a transfusion.

• #18 Sickle Cell Disease | Nutrition Guide for Clinicians

  https://nutritionguide.pcrm.org/nutritionguide/view/Nutrition_Guide_for_Clinicians/1342072/all/Sickle_Cell_Disease

  Comprehensive and multidisciplinary care is essential. Education of both patient and family may help prevent complications of the disease. […] Pneumococcal prophylaxis (oral penicillin V 125-250 mg twice daily) should be taken continuously by children with sickle cell anemia until age 5. Children with a history of splenectomy or severe pneumonia may need further prophylaxis. […] Folic acid should be taken in doses of 1 mg daily. Additionally, an iron-free multivitamin is recommended to ensure adequate intake of nutrients that are commonly deficient in those with SCD. Iron overload and subsequent oxidative stress may cause depletion of antioxidant vitamins. […] Transcranial Doppler may identify children at risk for stroke. Those at higher risk should be offered a chronic blood transfusion program. […] Routine eye exams should monitor for proliferative retinopathy. […] Assessment for chronic complications, including chronic lung and kidney disease, should be performed periodically, especially in older children and adults.

• #19 Sickle cell disease – Wikipedia

  https://en.wikipedia.org/wiki/Sickle_cell_disease

  The care of people with sickle cell disease may include infection prevention with vaccination and antibiotics, high fluid intake, folic acid supplementation, and pain medication. […] Pneumococcal infection is a leading cause of death among children with SCD; penicillin is recommended daily during the first 5 years of life in order to minimise the risk of infection. […] People with SCD are recommended to receive all vaccinations which are recommended by health authorities in order to avoid serious infection which might trigger a sickling crisis. […] In children, preventive RBC transfusion therapy has been shown to reduce the risk of first stroke or silent stroke when transcranial Doppler ultrasonography shows abnormal cerebral blood flow.

• #20 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.

• #21

  https://myhealth.alberta.ca/Health/pages/conditions.aspx?hwid=uh1427

  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. […] Stay safe at high altitudes and during air travel. Low oxygen levels caused by high altitudes and plane flights can cause problems.

• #22 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:

• #23 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.

• #24 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. Regular physical activity is good, but do not allow children to exercise or play so much that they become really worn out. Staying hydrated during any physical activity is important. […] 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.

• #25 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.

• #26 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.

• #27 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.

• #28 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.

• #29 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.

• #30 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.

• #31 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. […] Therefore, the steps of IVF treatment outlined in our IVF/ICSI treatment page will also apply here. […] For patients over the age of 35, due to their lowered ovarian reserves, we recommend that they consider having two IVF cycles so that we can combine two sets of embryos obtained from both cycles. […] 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.

• #32 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.

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

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

  5. Success in implementing identified interventions will depend on the commitment of Member States to integrate SCD prevention and control in national health plans, and provide an environment conducive for various stakeholders to contribute to the reduction of SCD prevalence, morbidity and mortality.

• #34 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.

• #35 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.

• #36

  https://sickle.nhm.gov.in/home/about

  The overall aim is to enable access to affordable and quality health care to all SCD patients, and to lower the prevalence through awareness, change of practices and screening interventions. […] Objectives of the Mission: Provision of affordable and accessible care to all SCD patients […] To ensure quality of care for SCD patients […] To reduce the prevalence of SCD. […] Strategy: The strategy emphasizes on THREE pillars: Health promotion- Awareness generation pre-marital genetic counselling […] Prevention: Universal screening and early detection. […] At INDIVIDUAL/Household level- Individuals with known or detected SCDs would also be encouraged to register on Sickle Cell Disease Support Corner, to bridge the gap between patients and health care services in tribal areas. […] At Community level: Platforms such as monthly Village Health Sanitation and Nutrition Committee (VHSNC)/ Mahila Arogya Samiti (MAS) meetings, Village/Urban Health Sanitation and Nutrition Days (V/UHSND) meetings at Anganwadis, Jan Arogya Samitis in AB-HWC, Arogya Sabha, Self-Help Groups (SHG), youth clubs, parent-teachers meetings in schools, etc., shall be leveraged to sensitize people on the importance of sickle cell disease and screening service available at AB-HWCs.

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