We will discuss what causes sickle cell anemia, what the symptoms are, what is done to treat this disease, and tests available.
- It’s inherited. The inherited gene causes the body to make abnormal hemoglobin “S.” Normal hgb is “A.”
- One inherited gene will cause Sickle Cell Trait which can be passed on to the next generation. Two genes will cause Sickle Cell Disease.
- The disease is present at birth, but babies usually don’t show symptoms until about four months old.
- The abnormal hemoglobin (hgb) causes the red blood cells to be crescent shaped, like a sickle. Normal red cells are doughnut shaped. These sickle shaped red blood cells clump together and get caught in blood vessels.
- The abnormal hemoglobin (hgb) causes anemia. Hemoglobin is made of protein and iron. Hgb “S” does not have as much iron as normal hgb.
- The most common symptom is sudden pain. This is due to the abnormal red cells getting caught in the blood vessels in the limbs and organs.
- Fatigue. This is due to the anemia.
- Chest pain. This is caused by the cells getting trapped in the lungs, or an infection.
- Cold hands and feet
- Infections. These are due to the spleen being damaged. Your spleen helps to fight infections.
- A red cell analysis(CBC or ABC), including studying the cells under a microscope, will show the shape of the cells.
- The “Sickle Cell Test” determines if abnormal hgb is present in the blood. A deoxygenating agent is added to the blood. A technologist examines the sample under the microscope to look for sickled cells. This shows if the patient has either the trait or the disease.
- Hemoglobin electrophoresis determines the type of hgb by passing an electric charge through a solution of the patient’s blood. Different hgb’s travel different distances.
- Pain management
- IV fluids. It’s important for the patient to drink lots of fluids to help prevent a sickle cell crisis.
- Antibiotics for infection
- Vaccination against pneumonia
- Blood transfusion
- Medication to prevent a painful crisis. There are possible serious side effects to the medication, however.
- Possible bone marrow transplant
Carriers of the trait may choose to consult a genetic counselor. Scientists are currently researching gene therapy. They’re also searching for new medicines, with hope that their research will provide better treatment for this disease.