Overview of Sickle Cell Disease
This disease is a group of inherited disorders that produces an abnormal type of hemoglobin that causes red blood cells to take on a sickle shape during deoxygenation. Sickling of red blood cells leads to lysis that results in anemia and vaso-occlusion. Sickled red blood cells form clots in organs, joints, and bones that produce painful episodes or crises. Sickle cell episodes can lead to damage to the spleen, with anemia leading to infections and systemic complications. Clients may have frequent crises that require ongoing medical management and complications. Infants are routinely screened for this disease. Clients are counseled to control their activities and stressors that may trigger a crises episode. Daily activities are difficult to complete if a client with sickle cell disease has chronic anemia.1,2
Comorbidities to Consider
Keys to Examination of Clients
Assess the results of blood tests for hematocrit and hemoglobin levels and reticulocyte and white blood cell counts as well as tests for liver and renal function.
Perform a thorough medical history to understand clients' history of sickling crises and the complications from the disease process.
Determine if clients have any persistent joint or mobility problems.
Recommended Baseline Testing of Fitness Levels
Type: Aerobic and recreational activities
Intensity: Brief episodes of high-intensity activities
Frequency: Three to seven times per week
Aerobic activities and recreational activities are most appropriate for these clients and should begin at low intensities. Clients should consider their exercise activity to be enjoyable and not an excessive stressor to their lifestyle. Young children may choose to participate in activities that require brief episodes of sprinting, such as soccer or baseball, and will need to rest when they feel fatigued.1,3 Young people should be encouraged to participate in school and recreational activities to allow for normal development and normal social interactions.3,4 They will need to be able to rest when fatigued and have regular access to hydration. They will need more time for acclimation to excessive heat, cold, and higher-altitude conditions. They should avoid long periods of underwater swimming or scuba diving.1,4