Interventions for Sickle cell anemia/disorder

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Age Cohort: < 5 years

Health Promotion	Disease Prevention	Diagnostic	Curative	Rehabilitative	Palliative
Community Level
Community education and awareness on sickle cell disease * Community education and sensitization with links to civil society organizations, parents’ groups, schools, and school clubs Sensitize and use local governance structures to establish community-based intervention program	Genetic counselling for carriers of haemoglobin disorders, including premarital counselling Prophylaxis for infection, pneumococcal vaccines, oral penicillin, Insecticide treated bed nets, Prophylaxis for Malaria Folate supplementation with folic acid	Recognition of Sickle cell disease signs and symptoms	Immediate transfer of severe anaemia to a higher-level health care facility	Physiotherapy: exercise, breathing exercises and massages	Non-opioid analgesia for effective control of mild to moderate- pain Symptomatic non-pharmacological treatment Emotional, social, spiritual support for parents
Primary Care
Community education and awareness on sickle cell disease * Community education and sensitization with links to civil society organizations, parents’ groups, schools, and school clubs Sensitize and use local governance structures to establish community-based intervention program	Genetic counselling for carriers of haemoglobin disorders, including premarital counselling Prophylaxis for infection, pneumococcal vaccines, oral penicillin Insecticide treated bed nets Prophylaxis for Malaria Folate supplementation with folic acid	Prenatal screening using rapid point of care test	Management of pain Referral to the next higher level Folic acid	Physiotherapy, exercise, breathing exercises and massages	Non-opioid analgesia for effective control of mild to moderate- pain Symptomatic non-pharmacological treatment Emotional, social, spiritual support for parents
Referral Facility: General
Parent/care takers education and awareness on sickle cell disease, physical activity and need of being mobile, breathing techniques	Genetic counselling for carriers of haemoglobin disorders, including premarital counselling Prophylaxis for infection, pneumococcal vaccines, oral penicillin,	History Physical examination Pre-natal Screening Neonatal screening X-ray, Ultrasound Electrocardiogram (ECG)	Chemoprophylaxis IV fluids Supplemental oxygen IM analgesics IM/IV antibiotics Blood and blood product transfusion Referral to the next higher level	Physiotherapy: exercise, breathing exercises and massages	Opioid analgesia for effective control of moderate- to severe pain Symptomatic non-pharmacological treatment Emotional, social, spiritual support for parents
Referral Facility: Specialist
Parent/care takers education and awareness on sickle cell disease, physical activity and need of being mobile, breathing techniques	Genetic counselling for carriers of haemoglobin disorders, including premarital counselling Prophylaxis for infection, pneumococcal vaccines, oral penicillin,	History Physical examination Pre-natal screening Neonatal screening Incentive spirometry X-ray, Ultrasound, Computed tomography (CT) Electrocardiogram (ECG)	Chemoprophylaxis Oral hydroxyurea IV fluids Supplemental oxygen IV/IM analgesics IV/IM antibiotics Blood and blood product transfusion Splenectomy Red cell exchange transfusion	Physiotherapy: exercise, breathing exercises and massages	Opioid analgesia for effective control of moderate- to severe pain Symptomatic non-pharmacological treatment Emotional, social, spiritual support for parents

Age Cohort: 5 - 11 years

Health Promotion	Disease Prevention	Diagnostic	Curative	Rehabilitative	Palliative
Community Level
Community education and awareness on sickle cell disease * Community education and sensitization with links to civil society organizations, parents’ groups, schools, and school clubs Sensitize and use local governance structures to establish community-based intervention program	Genetic counselling for carriers of haemoglobin disorders, including premarital counselling Prophylaxis for infection, pneumococcal vaccines, oral penicillin, Insecticide treated bed nets, Prophylaxis for Malaria Folate supplementation with folic acid	Recognition of sickle cell disease signs and symptoms	Immediate transfer of severe anaemia to a higher-level health care facility	Physiotherapy: aerobic exercises, breathing exercises, and massages	Non-opioid analgesia for effective control of mild to moderate- pain Symptomatic non-pharmacological treatment Emotional, social, spiritual support
Primary Care
Community education and awareness on sickle cell disease * Community education and sensitization with links to civil society organizations, parents’ groups, schools, and school clubs Sensitize and use local governance structures to establish community-based intervention program	Insecticide treated bed nets, Prophylaxis for Malaria Folate supplementation with folic acid	Prenatal screening using rapid point of care test	Management of pain Referral to the next higher level Folic acid	Opioid analgesia for effective control of moderate-to severe pain Symptomatic non-pharmacological treatment Emotional, social, spiritual support Wound care for stasis ulcer
Referral Facility: General
Patient education and awareness on sickle cell disease, physical activity and need of being mobile, breathing techniques	Genetic counselling for carriers of haemoglobin disorders, including premarital counselling Prophylaxis for infection, pneumococcal vaccines, oral penicillin, Prophylaxis for Malaria Folate supplementation with folic acid	History Physical examination Pre-natal Screening Neonatal screening X-ray, Ultrasound Electrocardiogram (ECG)	Condition-specific nutrition assessment and counselling Chemoprophylaxis for sickle cell disease Oral hydroxyurea for prevention of vaso-occlusive crises Oral or intravenous fluid rehydration Supplemental oxygen Analgesics for pain relief Antibiotics for bacterial infections Blood and blood product transfusion Referral to the next level facility	Physiotherapy: aerobic exercises, breathing exercises, and massages	Opioid analgesia for effective control of moderate-to severe pain Symptomatic non-pharmacological treatment Emotional, social, spiritual support Wound care for stasis ulcer
Referral Facility: Specialist
Patient education and awareness on sickle cell disease, physical activity and need of being mobile, breathing techniques	Genetic counselling for carriers of haemoglobin disorders, including premarital counselling Prophylaxis for infection, pneumococcal vaccines, oral penicillin, Prophylaxis for Malaria Folate supplementation with folic acid	History Physical examination Pre-natal screening Incentive spirometry X-ray, ultrasound, CT scan Electrocardiogram (ECG)	Chemoprophylaxis Oral hydroxyurea IV fluids Supplemental oxygen IV/IM analgesics IV/IM antibiotics Blood and blood product transfusion Splenectomy Red cell exchange transfusion	Physiotherapy: aerobic exercises, breathing exercises, and massages	Opioid analgesia for effective control of moderate-to severe pain Symptomatic non-pharmacological treatment Emotional, social, spiritual support Wound care for stasis ulcer

Age Cohort: 25 - 59 years

Health Promotion	Disease Prevention	Diagnostic	Curative	Rehabilitative	Palliative
Community Level
Community engagement [community sensitization on screening for sickle cell disease, myths associated with sickle cell disease] Community education and awareness regarding the transmission of disease, stigma related to disease and carrier states, and informing the community about appropriate prevention options * Community education and sensitization with links to civil society organizations, parents’ groups, schools, and school clubs Sensitize and use local governance structures to establish community-based intervention program	Marriage counselling for genetics for carriers of haemoglobin disorders Counselling on avoidance of triggers for vaso-occlusive crisis Counselling on disease management and warning signs and when to seek treatment Vaccination: pneumococcal conjugate		Recognition of danger signs and referral for management
Primary Care
Community engagement [community sensitization on screening for sickle cell disease, myths associated with sickle cell disease] Community education and awareness regarding the transmission of disease, stigma related to disease and carrier states, and informing the community about appropriate prevention options * Community education and sensitization with links to civil society organizations, parents’ groups, schools, and school clubs Sensitize and use local governance structures to establish community-based intervention program	Genetic counselling for carriers of haemoglobin disorders, including premarital counselling Education patients and caregivers about sickle cell disease including on warning signs and what to do in acute conditions before coming to the hospital. Adequate hydration by teaching the patients to drink enough fluids to make their urine clear Prophylaxis for infection, pneumococcal vaccines, oral penicillin, use of insecticide treated bed nets, and anti-malaria Folate supplementation with folic acid		History and physical examination for sickle cell disease Screening for sickle cell disease using rapid point of care test Condition-specific nutrition assessment and counselling Incentive spirometer to help improve lung function Routine malaria chemoprophylaxis non-steroidal anti-inflammatory drugs (NSAIDs) for relieving the inflammatory component of infarctive (vaso-occlusive) bone pains Oral antibiotics to prevent and treat invasive pneumococcal diseases Pulse oximetry to reliably estimate arterial oxygen saturation Referral (for acute complication and for initial diagnosis)
Referral Facility: General
Community engagement [community sensitization on screening for sickle cell disease, myths associated with sickle cell disease] Community education and awareness regarding the transmission of disease, stigma related to disease and carrier states, and informing the community about appropriate prevention options * Community education and sensitization with links to civil society organizations, parents’ groups, schools, and school clubs Sensitize and use local governance structures to establish community-based intervention program	Genetic counselling for carriers of haemoglobin disorders, including premarital counselling Education patients and caregivers about sickle cell disease including on warning signs and what to do in acute conditions before coming to the hospital. Adequate hydration by teaching the patients to drink enough fluids to make their urine clear Prophylaxis for infection, pneumococcal vaccines including Hep. B, oral penicillin, use of insecticide treated bed nets, and anti-malaria Folate supplementation with folic acid		History and physical examination for sickle cell disease Screening for sickle cell disease using rapid point of care test Condition-specific nutrition assessment and counselling History and physical examination *Basic and advanced laboratory tests Investigations such as X-ray, Electrocardiogram (ECG) Incentive spirometry for sickle cell disease Chemoprophylaxis for sickle cell disease Oral hydroxyurea for prevention of vaso-occlusive crises Oral or intravenous fluid rehydration Supplemental oxygen Analgesics for pain relief Antibiotics for bacterial infections Blood and blood product transfusion Referral to the next higher level
Referral Facility: Specialist
Community engagement [community sensitization on screening for sickle cell disease, myths associated with sickle cell disease] Community education and awareness regarding the transmission of disease, stigma related to disease and carrier states, and informing the community about appropriate prevention options * Community education and sensitization with links to civil society organizations, parents’ groups, schools, and school clubs Sensitize and use local governance structures to establish community-based intervention program	Adequate hydration by teaching the patients to drink enough fluids to make their urine clear Prophylaxis for infection, pneumococcal vaccines including Hep. B, oral penicillin, use of insecticide treated bed nets, and anti-malaria Folate supplementation with folic acid		History and physical examination for sickle cell disease Condition-specific nutrition assessment and counselling Basic and advanced laboratory tests Investigations such as X-ray, Electrocardiogram (ECG) Incentive spirometry for sickle cell disease Chemoprophylaxis for sickle cell disease Oral hydroxyurea for prevention of vaso-occlusive crises Oral or intravenous fluid rehydration Supplemental oxygen Analgesics for pain relief Antibiotics for bacterial infections Blood and blood product transfusion Splenectomy for splenic sequestration crisis Red cell exchange transfusion

Age Cohort: 60+ years

Health Promotion	Disease Prevention	Diagnostic	Curative	Rehabilitative	Palliative
Community Level
Community engagement [community sensitization on screening for sickle cell disease, myths associated with sickle cell disease] Community education and awareness regarding the transmission of disease, stigma related to disease and carrier states, and informing the community about appropriate prevention options * Community education and sensitization with links to civil society organizations, parents’ groups, schools, and school clubs Sensitize and use local governance structures to establish community-based intervention program	Marriage counselling for genetics for carriers of haemoglobin disorders Counselling on avoidance of triggers for vaso-occlusive crisis Counselling on disease management and warning signs and when to seek treatment Vaccination: pneumococcal conjugate		Recognition of danger signs and referral for management
Primary Care
Health education and awareness regarding the transmission of disease, stigma related to disease and carrier states	Genetic counselling for carriers of haemoglobin disorders, including premarital counselling Education patients and caregivers about sickle cell disease including on warning signs and what to do in acute conditions before coming to the hospital. Adequate hydration by teaching the patients to drink enough fluids to make their urine clear Prophylaxis for infection, pneumococcal vaccines, oral penicillin, use of insecticide treated bed nets, and anti-malaria Folate supplementation with folic acid		Condition-specific nutrition assessment and counselling Incentive spirometer to help improve lung function Routine malaria chemoprophylaxis Non-steroidal anti-inflammatory drugs (NSAIDs) for relieving the inflammatory component of infarctive (vaso-occlusive) bone pains Oral antibiotics to prevent and treat invasive pneumococcal diseases Pulse oximetry to reliably estimate arterial oxygen saturation Referral (for acute complication and for initial diagnosis)
Referral Facility: General
Patient education and awareness regarding the transmission of disease, stigma related to disease and carrier states	Genetic counselling for carriers of haemoglobin disorders, including premarital counselling Education patients and caregivers about sickle cell disease including on warning signs and what to do in acute conditions before coming to the hospital. Adequate hydration by teaching the patients to drink enough fluids to make their urine clear Prophylaxis for infection, pneumococcal vaccines including Hep. B, oral penicillin, use of insecticide treated bed nets, and anti-malaria Folate supplementation with folic acid		Condition-specific nutrition assessment and counselling Chemoprophylaxis for sickle cell disease Oral hydroxyurea for prevention of vaso-occlusive crises Oral or intravenous fluid rehydration Supplemental oxygen Analgesics for pain relief Antibiotics for bacterial infections Blood and blood product transfusion Referral to the next level facility
Referral Facility: Specialist
Patient education and awareness regarding the transmission of disease, stigma related to disease and carrier states	Adequate hydration by teaching the patients to drink enough fluids to make their urine clear Prophylaxis for infection, pneumococcal vaccines including Hep. B, oral penicillin, use of insecticide treated bed nets, and anti-malaria Folate supplementation with folic acid

Age Cohort: 12 - 24 years

Health Promotion	Disease Prevention	Diagnostic	Curative	Rehabilitative	Palliative
Community Level
Community education and awareness on sickle cell disease Community education and sensitization with links to civil society organizations, parents’ groups, schools, and school clubs Sensitize and use local governance structures to establish community-based intervention program	Insecticide treated bed nets, Prophylaxis for Malaria Folate supplementation with folic acid	Recognition of sickle cell disease signs and symptoms	Immediate transfer of severe anaemia to a higher-level health care facility	Physiotherapy: aerobic exercises, breathing exercises, and massages
Primary Care
Health education and awareness on sickle cell disease	Insecticide treated bed nets, Prophylaxis for Malaria Folate supplementation with folic acid Prophylaxis for infection, pneumococcal vaccines, oral penicillin,	Prenatal screening using rapid point of care test	Management of pain Referral to the next higher level Folic acid	Physiotherapy: aerobic exercises, breathing exercises, and massages	Opioid analgesia for effective control of moderate-to severe pain Symptomatic non-pharmacological treatment Emotional, social, spiritual support Wound care for stasis ulcer
Referral Facility: General
Opioid analgesia for effective control of moderate-to severe pain Symptomatic non-pharmacological treatment Emotional, social, spiritual support Wound care for stasis ulcer	Genetic counselling for carriers of haemoglobin disorders, including premarital counselling Prophylaxis for infection, pneumococcal vaccines, oral penicillin, Prophylaxis for Malaria Folate supplementation with folic acid	History Physical examination Pre-natal Screening X-ray, Ultrasound Electrocardiogram (ECG)	Condition-specific nutrition assessment and counselling Chemoprophylaxis for sickle cell disease Oral hydroxyurea for prevention of vaso-occlusive crises Oral or intravenous fluid rehydration Supplemental oxygen Analgesics for pain relief Antibiotics for bacterial infections Blood and blood product transfusion Referral to the next level facility	Physiotherapy: aerobic exercises, breathing exercises, and massages	Opioid analgesia for effective control of moderate-to severe pain Symptomatic non-pharmacological treatment Emotional, social, spiritual support Wound care for stasis ulcer
Referral Facility: Specialist
Patient education and awareness on sickle cell disease, physical activity and need of being mobile, breathing techniques	Genetic counselling for carriers of haemoglobin disorders, including premarital counselling Prophylaxis for infection, pneumococcal vaccines, oral penicillin, Prophylaxis for Malaria Folate supplementation with folic acid	History Physical examination Pre-natal screening Neonatal screening Incentive spirometry X-ray, ultrasound, CT scan Electrocardiogram (ECG)	Chemoprophylaxis Oral hydroxyurea IV fluids Supplemental oxygen IV/IM analgesics IV/IM antibiotics Blood and blood product transfusion Splenectomy Red cell exchange transfusion	Physiotherapy: aerobic exercises, breathing exercises, and massages	Opioid analgesia for effective control of moderate-to severe pain Symptomatic non-pharmacological treatment Emotional, social, spiritual support Wound care for stasis ulcer