Effects of Sickle Cell Anemia

The effects of sickle cell anemia cause pain and damage to the organs. Irregularly shaped red blood cells become sluggish, and move more slowly, causing clumping. Red blood cells are normally round, and carry oxygen rich blood. Oxygen is carried by hemoglobin in red blood cells, but in individuals with sickle cell anemia, abnormal hemoglobin levels cause red blood cells to take on a C shape. Red blood cells that are abnormally shaped, as in sickle cell anemia, die before new ones can be produced.

Symptoms

• Sickle cell anemia is inherited and is present at birth. Symptoms vary from mild to severe, and may not appear until four months of age. Pallor, cool extremities, shortness of breath from lack of hemoglobin and oxygen to the lungs, dizziness, fever and headache are symptoms. Generalized pain accompanies "sickle cell crisis", and may require hospitalization. Pain might appear suddenly. Some individuals experience constant pain, interspersed with severe episodes that can destroy quality of life.

Effects

• Sickle cell anemia can destroy the organs of the body. Pain and swelling in the toes, feet and ankles might be one of the first signs of sickle cell anemia. Blocked blood vessels can also cause pain in the hands. Sickle cells can block blood flow to other organs, including the spleen, lungs, brain, eyes and the blood vessels that supply the heart and lungs. Infection and pneumonia are possible.
  Eye damage, disability from hemorrhagic or ischemic stroke (from lack of oxygen to the brain), enlargement of the spleen, and pulmonary artery hypertension (increased pressure in the lungs) are possible effects of sickle cell anemia. Leg ulcers can occur from poor blood flow to the skin. Kidney failure can occur. Red blood cell destruction that releases too much bilirubin into the bloodstream can lead to gallstones and gallbladder attacks that cause nausea and abdominal pain.

Causes

• Sickle cell anemia is inherited--it comes from both parents. You may possess a sickle cell trait meaning you will not have the disease, but have a genetic possibility of passing the gene to your children.

Diagnosis

• By law, newborns in the United States are automatically screened for sickle cell anemia. If the test is positive, it is repeated for confirmation. Follow-up is important, and should be done within the first two months after birth. The blood test can also reveal the presence of the sickle cell trait. Early diagnosis is important for outcomes. Genetic testing from amniotic fluid can tell if your baby will have sickle cell anemia, and can be performed before birth, within the first three months of pregnancy.

Treatment

• Individuals with sickle cell anemia can experience complications if dehydration occurs. During a sickle cell crisis, when pain becomes acute, hospital care may be needed. Intravenous fluids are administered. Over the counter pain medication, such as Tylenol and ibuprofen can reduce sickle cell pain. Severe pain may require narcotic pain medication.
  Bone marrow transplant has been used as a treatment in children with severe sickle cell anemia.
  Hydroxyurea is a medication used that can prevent a sickle cell crisis. Studies are promising, but the long-term effects are not known, and the medication can have serious side effects. Close monitoring of patients taking hydoxyurea for sickle cell anemia is important. Blood transfusions may be necessary when enlargement of the spleen or infection are present from sickle cell anemia.
  Newer medications used to treat sickle cell anemia include nitric oxide to keep blood vessels relaxed and improve blood flow, butyric acid to raise hemoglobin levels, and decitidine that also raises hemoglobin levels. Maintaining proper hydration, eating a healthy diet, staying warm, exercising and sleeping well can help with treatment and the management of sickle cell anemia.