Sickle cell anemia is a genetic blood disorder is characterized by a depletion of red blood cells that deliver oxygen via the circulatory system. Sickle genes cause the body to produce mutated red blood cells. These sickle cells die much faster than normal red blood cells, causing a chronic deficit in blood supply to oxygenate the body.
Another problem created by sickle cells is that they are crescent-shaped rather than rounded, and they tend to cling together. They can become lodged in the smaller blood vessels, creating a sort of traffic jam that prevents oxygen from reaching parts of the body. A lack of oxygen can be life-threatening and extremely painful. The following are common symptoms experienced by patients with sickle cell anemia, and although many are characteristic of certain ages they may not necessarily age-exclusive.
Children
Sickle cell is present at birth and almost all states in the U.S. screen newborns. Symptoms may not manifest until three to five months after birth. Infants and children with sickle cell anemia are especially vulnerable to infections; they will require more doctor visits and more vaccines, including yearly flu shots. An additional precautionary measure, your child may take penicillin or erythromycin daily until age five.
Because infections are so threatening to patients with sickle cell anemia, fevers should not be managed with over-the-counter medications but should be treated by your child's doctor. Routine ultrasound tests of the brain may reveal the likelihood of having a stroke. In this case, some children are given periodic blood transfusions as a precautionary measure.
The hands and feet often become painfully swollen when they are not getting enough oxygen; this is called dactylitis, or hand and foot syndrome. Due to chronic illness, chronically low oxygen levels, or appetite suppression from symptoms or medications, your child may grow at a slower-than-normal pace. Bladder control may also be delayed.
Teenagers
One characteristic of teenagers with sickle cell anemia is the delayed onset of puberty. Typically puberty may occur two to three years later than their peers. Some teenage boys experience priapism, or painful, long-lasting erections. When erections last longer than one hour, medical attention should be sought. Delayed puberty, smaller physical statures, uncertainty of life, and chronic pain and illness may contribute to psychosocial problems during what is already an emotionally turbulent stage of life. After years of periodic crises, organ damage may occur by this time.
Adults
After years of chronic pain, inflammation, and lack of oxygen, some degree of tissue damage may occur. The retinas in the back of the eye may become damaged and result in blindness. The heart may become enlarged from years of being overworked as it pumps harder to ensure blood reaches every part of the body, and pulmonary hypertension may result. Tissue breakdown may occur, causing skin ulcers. Men also experience priapism.
The Center for Disease Control recommends seeking emergency medical care for the following symptoms:
- Fever above 101 degrees Fahrenheit
- Chest pain
- Difficulty breathing
- Abdominal swelling
- Severe headache
- Sudden weakness or loss of feeling or movement
- Seizure
- Painful erection of the penis that lasts more than four hours
