People with sickle cell disease require deliberate efforts to coordinate their care between communities, health centres, district hospitals, regional referrals, and national referral hospitals as well as a private practice to improve their health and well-being.
People living with sickle cell disease are at increased risk of developing multiorgan acute and chronic damage that can span from acute chest syndrome to overt stroke, as well as acute and chronic kidney injury.
Much as the treatment of sickle cell disease may relieve most of these complications – it doesn’t entirely prevent them. It is where screening of these complications becomes paramount. However, screening should not only be cost-effective, but it should also be accurate, associated with minimal harm, and should have proved that early intervention is beneficial.
Because we do not screen every condition, recommendations come in to help guide the practising health care provider in providing the best quality health care services there’s. They aim at helping people living with sickle cell disease achieve an optimally healthy lifestyle.
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We have adopted the recommendations below from the National Heart, Lung, and Blood Institute expert panel for evidence-based management of sickle cell disease. The Uganda Clinical Guidelines have most of this information in a more summarized form with doses for the practising clinician as low as a health centre II. Let’s get this started.
Sickle cell anaemia (SCA) describes the clinically similar disorders HbSS or HbSβ°-thalassemia.
Sickle cell disease (SCD) refers to all disease genotypes. They include SCA and compound heterozygous disorders like HbSC, HbSD, and HbSβ+- thalassemia. The carrier state for haemoglobin S (HbAS or sickle cell trait) is not a form of SCD.
Recommendations about the Prevention of Invasive Pneumococcal Infection.
- Give oral penicillin prophylaxis (125 mg for age <3 years and 250 mg for three years and above) twice daily until age 5 in all children with HbSS. Discontinue prophylactic penicillin in children with HbSS at five years unless they have ad a splenectomy or invasive pneumococcal infection. When discontinuing penicillin prophylaxis at age 5, it is crucial to ensure that the child has completed the recommended pneumococcal vaccination series, and if not, complete the series immediately.
- Consider withholding penicillin prophylaxis for children with HbSC disease and HbSβ+- thalassemia. Only continue it if they have had a splenectomy.
- Ensure that people of all ages with SCD have had vaccines against Streptococcus pneumoniae.
- Remind people with SCD, their families, and caregivers to seek immediate medical attention whenever fever (temperature greater than 38.5°C) occurs, due to the risk for severe bacterial infection.
Screening for Renal Disease.
1. Screen all individuals with SCD, beginning by age 10, for proteinuria: If the result is negative, repeat screening annually. When positive, perform a first-morning void urine albumin-creatinine ratio and if abnormal, consult with or refer to a renal specialist.
Recommendations about Electrocardiogram Screening
1. We do not recommend routine ECG screening is in children and adults with SCD.
Screening for Hypertension
- In adults with SCD, screen for hypertension and treatment to lower systolic blood pressure ≤130 and diastolic blood pressure ≤80 according to ‘The Eighth Report of the Joint National Committee on Prevention, Detection, Evaluation, and Treatment of High Blood Pressure’ (JNC 8).
- In children with SCD, measure blood pressure, and evaluate and treat hypertension following recommendations from either the national guidelines or ‘Fourth of Fifth Report on the Diagnosis, Evaluation, and Treatment of High Blood Pressure in Children and Adolescents.’
Screening for Retinopathy
- All people with SCD should have a detailed eye examination to evaluate for retinopathy that begins at ten years.
- For people having a typical dilated retinal examination, re-screen at 1-2year intervals.
- Refer people with suspected retinopathy to a retinal specialist.
- about screening for Risk of Stroke Using Neuroimaging
- In children with SCA, do annual screening with transcranial Doppler (TCD) according to the methods employed in the STOP studies (read about it here), beginning at age two and continue until at least age 16.
- In children with conditional (170-199 cm/Sec) or elevated (>200 cm/Sec) TCD results, refer to a specialist in chronic transfusion therapy aimed at preventing stroke.
- In children with genotypes other than the SCA, do not perform screening with TCD.
- In asymptomatic children with SCD, do not screen with MRI or CT.
- In asymptomatic adults with SCD, do not screen with neuroimaging (TCD, MRI, or CT).
Recommendations about Screening for Pulmonary Disease
- In children and adults with SCD, assess for signs and symptoms of respiratory problems, such as asthma, COPD, restrictive lung disease, or obstructive sleep apnoea, by history and physical examination.
- In children and adults with SCD found to have signs or symptoms of respiratory problems by history and physical examination, further assessment, which includes pulmonary function tests, is recommended to determine the cause and develop a plan to address the issues.
- Do not screen asymptomatic children and adults with pulmonary function tests.
Recommendations about Reproductive Counselling.
Specific for women or men with SCD
- Encourage each woman, man, and the couple affected by SCD to have a reproductive life plan.
- As a part of primary care visits, provide risk assessment and educational and health promotion, counselling (or refer to individuals with expertise in these disciplines) to all women and men of childbearing age to reduce reproductive risk and improve pregnancy outcomes. Provide contraceptive counselling, if desired, to prevent unintended pregnancy, and if pregnancy is needed, provide preconception counselling.
- If the spouse of a man or woman with SCD has unknown SCD or thalassemia status, refer them for hemoglobinopathy screening.
- After testing, refer couples who are at risk for having a potentially affected fetus and neonate for genetic counselling.
Specifically for women with SCD
- Test women with SCD who have been transfused and are anticipating pregnancy for red cell alloantibodies.
- If a woman has red cell alloantibodies, test her partner for the corresponding red cell antigen(s).
- If the partner tests positive for the corresponding red cell antigen(s), counsel the woman and her partner about the risks of hemolytic disease in the fetus and neonate, how it is treated, or refer them to a maternal-fetal specialist who can provide this education.
- Counsel women with SCD and their partners or refer for counselling about the following:
- Pregnancy in women with SCD is considered high risk, and there’s an increased risk of adverse pregnancy outcomes like fetal (intrauterine) growth restriction, preterm delivery, and stillbirth.
- Additional fetal surveillance is vital during pregnancy.
- There are increased risks to a woman’s health during pregnancy. These risks include an increased frequency of pain crises and an increased risk of thrombosis, infections, preeclampsia, and death relative to women who do not have SCD.
For women who require chronic opioid therapy during pregnancy, there is an increased risk of neonatal withdrawal in their newborns.
Recommendations about Contraception
- Progestin-only contraceptives (pills, injections, and implants), levonorgestrel IUDs, and barrier methods have no restrictions or concerns for use in women with SCD.
- If the benefits outweigh the risks, women with SCD may use the combined hormonal contraceptives (pills, patches, and rings).
Recommendations about immunization
- All individuals with SCD should receive immunizations according to ACIP, or UNEPI or national clinical guidelines, whichever suits best unless they have a personal contraindication as noted in clinical guidelines.
- Because of their increased susceptibility to invasive pneumococcal disease, all infants with SCD should receive the complete series of the 13-valent conjugate pneumococcal vaccine series beginning shortly after birth and the 23-valent pneumococcal polysaccharide vaccine at two years, with a second dose at five years*
*There’s a shred of relevant evidence that pediatric patients should discontinue prophylactic penicillin at five years provided that their immunizations are up to date. However, this would mean they would be at potential risk of infection by invasive pneumococcal if they would have to wait for additional coverage until age 7. The experts who designed these recommendations dictate from experience that giving the second dose at five years ensures adequate coverage.
Recommendation about Malaria prophylaxis
- All people living with SCD should receive antimalarial prophylaxis in the form of sulphadoxine-pyrimethamine (SP) commonly known as Fansidar.
- For those with known allergy to sulfur-containing medications, they can receive erythromycin.
NOTE: Please refer to your regional clinical guidelines for appropriate and proper management. Every patient is a distinct individual and not an opportunity to implement these recommendations. You remain with the absolute right to use your clinical acumen to provide evidence-based care to the best of your ability.
Thanks for reading, come back for the next article about the management of acute complications of SCD.