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Parent and Caregiver Handbook for Sickle Cell

Appendix: Adulthood and what medical issues to expect

Thankfully, most people with sickle cell disease live into middle or late adulthood. As more is learned about sickle cell disease, people with the disease live longer. People with sickle cell disease can have full, long lives with their own families and interesting jobs.

Taking care of health and medical needs can improve how people feel and thrive. With better understanding of sickle cell disease and treatments, hopefully your child will be well positioned to begin adulthood in the best health possible.

Adults with sickle cell disease may feel well but have progressive silent damage. Ongoing care includes screening for these problems and trying to prevent them.

An adult with sickle cell disease should have a primary care doctor. That doctor is not usually not a sickle cell specialist. They should be supported by a comprehensive sickle cell care center to address questions and complications. Adults should be seen at a comprehensive sickle cell care center at least once a year.

The problems adults have may require consultation with different specialists. The sickle cell care center should help coordinate those visits. The primary care doctor and sickle cell team should make sure that routine visits include monitoring organs (kidneys, lungs, bones, eyes, heart, or brain) for signs of damage. Over time, sickle cells can affect most body organs. Just like in children, in adults if signs of damage are found, proper treatment can be started early.

Not all, but some, adults with sickle cell disease have major health problems related to their disease. The most common problems that are monitored include the risk of pulmonary hypertension, chronic lung disease, kidney damage, avascular necrosis of the hip, silent stroke, and eye problems. Other problems may include hypertension, heart disease, chronic pain, leg ulcers, and iron overload.

As the patient gets older, their medical complications may get worse. Below are a few more details on organ damage in adulthood:

  • Lungs. Adults who have repeated episodes of acute chest syndrome and severe asthma or both may develop chronic lung disease. Monitoring lung function is helpful in diagnosing progression of lung damage. In addition, adults are at risk for clots in the lungs. Any hospitalization of an adult with sickle cell disease requires medication to prevent clots. Monitoring the lung function and heart echocardiogram will detect the amount of lung injury and the development of pulmonary hypertension. Medical therapy can be started and possibly chronic transfusion. Smoking in sickle cell adults should be avoided because it may aggravate the lung injury
  • Kidneys. Monitoring the protein in the urine and kidney blood tests will enable early treatment of early kidney disease. Despite adequate therapy, some patients eventually develop kidney failure and will need dialysis or kidney transplantation. 
  • Bones. The chance of getting avascular necrosis in the hip increases with age. Early identification and care may decrease the progression of the hip. However hip replacement is not unusual in older sickle cell patients because of severe pain. It usually helps relieve pain and improve movement.
  • Brain. Strokes can occur at any age in sickle cell disease. The incidence has decreased since we started screening for strokes with TCD in children. Adults, however, may continue to have silent infarction that could lead to learning disabilities. Transfusion and rehabilitation help most people who have had strokes regain their abilities.
  • Eyes. Fortunately, blindness rarely happens. It can be prevented by yearly check-ups with an ophthalmologist (eye doctor) who knows about sickle cell disease.
  • Heart. As with all adults, older adults with sickle cell disease may develop congestive heart failure when the heart muscle doesn't pump blood as well as it should. High blood pressure (pulmonary hypertension) can happen when the pressure in the blood vessels leading from the heart to the lungs is too high. Medicine and transfusions may help with these age-related conditions.

This information is provided courtesy of the University of San Francisco, Benioff Children's Hospital Oakland Sickle Cell Center.​

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