Sickle cell anemia causes pain in infants' hands and feet. When you get older, you will have to face having recurring pain crises, problems with your spleen, numerous infections, hip pain and the possibility of having seizures and strokes. All of this is due to a small change in the hemoglobin of a red blood cell--instead of the amino acid glutamine, they have the amino acid valine. This substitution results in a hemoglobin that hardens red blood cells and forms them into a sickle shape. According to the National Heart Lung and Blood Institute, 70,000 to 100,000 Americans have sickle cell anemia.
Pain in Hands and Feet
Symptoms generally don't develop in infants younger than 4 months. The type of hemoglobin newborn babies have will not go into a sickle shape. The first type of pain that a young child will feel is called sickle dactylitis, or inflammation, swelling and pain in the hands and feet.
Pain Crises
When you are a little older, if you become dehydrated, get infections or receive low amounts of oxygen, you will experience pain crises. This pain will be due to the sickle cells obstructing the blood vessels, not letting the red blood cells and the oxygen that they carry pass. Another name for this type of pain crisis is vaso-occlusive crisis, because the sickle cells occlude (obstruct) the blood vessels. If they obstruct the small blood vessels in your lungs, you also may have chest pain, cough, shortness of breath and fever. This is called an acute chest syndrome. If the sickle cells obstruct the blood vessels to the kidney, you will have (flank) pain in your lower back, according to The Merck Manuals.
Spleen
Sickle cells also cause problems in the spleen, because their shape will interfere with the normal flow of blood inside the organ. This causes the spleen to increase in size, which can cause shock because not enough blood is circulating throughout your body. This is called a splenic sequestration crisis, because the red blood cells are sequestered or held inside the spleen. The spleen also can be damaged from not getting enough oxygen itself, causing it to shrink in size by the time you are around 6 years old, according to the "Handbook of Pathophysiology."
Infections
With sickle cell anemia, you are at risk for getting serious, painful infections. With a spleen that is not properly functioning, you are susceptible to getting infections from H. influenzae, S. pneumoniae and N. meningitides--bacteria that cause pneumonia and meningitis. You also are at risk of getting osteomyelitis, a painful bone infection. And you may be infected by parvovirus B19, which causes what is called an aplastic crisis, in which your bone marrow will either stop making as many red blood cells or not make any at all.
Avascular Necrosis of the Femoral Head
This term is used when there is an obstruction in the arteries that prevents blood and oxygen from reaching the thigh bone. Not getting the blood supply (avascular) to the femur (which is the thigh bone) causes necrosis (the type of damage from not receiving blood and oxygen). If this happens, you will experience a lot of hip pain, the "Handbook of Pathophysiology" explains.
Seizures and Strokes
If the sickle cells occlude the blood vessels in the brain, the brain will not receive the oxygen that it needs to function. This can lead to seizures as well as strokes, and at an early age. Many times, this problem can be fatal.
References
- "Handbook of Pathophysiology"; Elizabeth Corwin, MSN, Ph.D., FNP; 2000
- National Heart Lung and Blood Institute: Sickle Cell Anemia
- The Merck Manuals: Sickle Cell Anemia
