Understanding and Preventing Possible Complications
Data from stroke studies have shown that 33% of children with HbSS or HbSC disease had evidence of “silent” infarcts and cerebral atrophy (brain changes). In a study of 312 children ages 6-14, all 16 children with a clinical history of stroke also had abnormal MRI scans. Of the children without a history of stroke, about 10% had evidence of infarcts, and 5% had evidence of atrophy.
Proliferative sickle retinopathy, a complication of blockage of the vessels in the eye, may result in vision loss if undiagnosed or untreated. Individuals with Hemoglobin SC disease are at greater risk for this sickle cell complication than are individuals with Hemoglobin SS disease. Regular eye exams (annually for Hemoglobin SC patients and every other year for Hemoglobin SS patients) are an important component of monitoring for early changes.
The cause of leg ulcers is unknown, but it is presumed to be the result of blockage of the small blood vessels of the leg and surrounding tissue death. Most begin as small scabbed areas over the ankles and more rarely over the front of the leg. These ulcers often take months to heal, and they do recur. Because they are difficult to heal, they often get worse without proper medical care to cover large surface areas, sometimes completely around the leg.
Outcomes may be influenced by proper gentle debridement (removed of dead tissue), control of local edema (swelling), treatment of infection, identification and correction of nutritional deficiencies, and prevention of significant anemia.
Avascular necrosis (AVN) is the lack of blood flow to a bone causing bone death. AVN can affect patients with all types of sickle cell disease and typically involves the hips, shoulder or knees. AVN is commonly seen in patients with HbSS between 25 and 35 years, and slightly older than that for patients with HbSC or Sβ+thalassemia. However, AVN can also occur in the early teen years. Evaluation with X-ray or MRI is required to make the diagnosis.
Pulmonary hypertension is an elevated pressure in the blood flow through the lungs thought to be the result of repeated episodes of sickling in the lungs and can lead to heart issues. Symptoms of pulmonary hypertension may include shortness of breath with activity, rapid heart rate, rapid breathing, and dizziness.
Sickling occurs in the kidney more readily than any other organ. This can result in gradual kidney disease. Screening for early signs of decreased kidney function is recommended.
Children and young adults with sickle cell disease are more likely to have problems with bedwetting (enuresis). This is mainly because their kidneys have less ability to concentrate urine and instead make large amounts of dilute urine. The dilute urine fills the bladder quickly causing bedwetting when they are asleep.
Pica is an eating disorder marked by the consumption of items that are not food (e.g., paper, dirt, ice). This condition is not well understood but may be related to nutritional deficiencies or degree of anemia. Individuals with sickle cell disease may have a higher incidence of pica than the general population. Distraction techniques and open communication are important tools for managing this disorder.