Published: May 25, 2021
Patients with sickle cell disease (SCD) are at high risk for a broad range of acute and chronic conditions affecting many of the body’s systems and carrying a high association with morbidity.
Given the broad range of potential manifestations of the disease, coordinating care among primary care providers, specialists, emergency departments, laboratories and hospitals can dramatically improve outcomes.
“Treating the sickle cell disease can improve outcomes for the many potential complications of SCD, but getting ahead of the complications by identifying those at risk and with early signs can lead to much better outcomes,” said Ashok B. Raj, M.D., a pediatric hematologist/oncologist and medical director of Norton Children’s Cancer Institute, affiliated with the UofL School of Medicine.
Dr. Raj is also director of the pediatric erythrocytapheresis program at Norton Children’s Cancer Institute.
The following is modified from the National Institutes of Health’s Evidence-Based Management of Sickle Cell Disease; Expert Panel Report, 2014
Young children with sickle cell anemia (SCA) have a very high risk for septicemia and meningitis due to defective spleen function.
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Sickle cell nephropathy is a major complication of SCD, causing tubular and medullary dysfunction. Glomerular enlargement, perihilar focal segmental glomerulosclerosis and global sclerosis are the most common.
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Individuals with the HbSS form of SCD often have significantly lower diastolic, systolic and mean blood pressure.
All SCD patients, and especially those with HbSC (one sickle cell gene “S” from one parent and one abnormal hemoglobin gene “C” from the other), are at risk for retinal disease. One study found proliferative retinopathy in 4.3% of children with SCD. Another study in Jamaica found visual acuity loss attributed to retinopathy was in 10% of untreated eyes.
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Stroke is one of the most common and devastating complications of SCD. Without efforts to prevent stroke, about 10% of children with SCA will have overt stroke. Overt stroke recurs in most children with SCA who do not receive chronic transfusions or successful hematopoietic stem cell transplantation.
Adults with SCA have a high risk of both ischemic and hemorrhagic stroke.
Of children with SCD, 20% to 48% have respiratory conditions A study of individuals with SCA and asthma had a more than twofold higher risk of mortality than patients with just SCA.
Assess children and adults with SCD for signs and symptoms of respiratory conditions such as asthma, chronic obstructive pulmonary disorder (COPD), restrictive lung disease or obstructive sleep apnea.
Perform further assessment, including pulmonary function tests to determine the cause and treatment plan in children and adults with SCD found to have signs or symptoms of respiratory conditions.
There is an increased risk of adverse pregnancy outcomes in SCD patients as well as risk of maternal morbidity and mortality.
For patients with SCD:
Hormonal contraceptives may decrease menstrual blood flow, leading to higher hemoglobin levels. Progestin-only hormonal contraceptives lower the risk of thromboembolism compared with use of estrogen-containing contraceptives and have been shown to be safe for patients with SCD.
Intrauterine devices (IUDs) and intrauterine implants carry modest risks associated with the insertion procedure, while sterilization carries risks associated with the surgical procedure. There is no evidence that IUDs pose an increased risk for patients with SCD.
An overemphasis on treating SCD may lead to some patients failing to get recommended clinical preventive services.
Newborn screening
Supplemental immunization schedule for children with SCD, per Centers for Disease Control and Prevention: