GUIDING STEPS FOR SICKLE CELL PATIENT’S
People with sickle cell disease (SCD) start to have signs of the disease during the first year of life, usually around 5 months of age. Symptoms and complications of SCD are different for each person and can range from mild to severe.
The reason that infants don’t show symptoms at birth is because baby or fetal hemoglobin protects the red blood cells from sickling. When the infant is around 4 to 5 months of age, the baby or fetal hemoglobin is replaced by sickle hemoglobin and the cells begin to sickle.
SCD is a disease that worsens over time. Treatments are available that can prevent complications and lengthen the lives of those who have this condition. These treatment options can be different for each person depending on the symptoms and severity.
Hydroxyurea (pronounced hye droks ee yoor EE a) is a medicine that has been shown to decrease several complications of SCD. Stem cell transplants (A stem cell transplant, also called a bone marrow transplant, is a procedure that infuses healthy cells, called stem cells, into the body to replace damaged or diseased bone marrow. Bone marrow is the center of the bone where blood cells are made) from bone marrow or blood of healthy donors are increasingly being used to successfully cure SCD. Complications from hydroxyurea therapy and stem cell transplants are rare but can be serious or life-threatening. People with SCD and their families should ask their doctors about the benefits and risks of each.
ANEMIA IS WHAT KIND OF DISEASE?
Anemia is a very common complication of SCD. With SCD, the red blood cells die early. This means there are not enough healthy red blood cells to carry oxygen throughout the body. When this happens, a person might have:
- Dizziness and lightheadedness
- A fast heart rate
- Difficulty breathing
- Pale skin color
- Jaundice (yellow color to the skin and whites of the eyes)
- Slow growth
- Delayed puberty
Blood transfusions are used to treat severe anemia. A sudden worsening of anemia resulting from infection or enlargement of the spleen is a common reason for a transfusion. Multiple blood transfusions, however, might cause health problems because of the iron content of blood. Iron overload, called hemosiderosis, can damage liver, heart, pancreas and other organs, leading to diseases such as diabetes mellitus. Iron chelation therapy should be started in patients with SCD receiving regular blood transfusions to reduce excess iron levels.