Spherocytosis
Spherocytosis is a condition in which an abnormality is caused in the red blood cell membrane. While healthy blood cells are shaped like flattened, indented discs, these abnormal membranes lead to sphere-shaped red blood cells and the premature breakdown of those cells.
Red blood cells which are suffering from spherocytosis are basically smaller, rounder in shape, and more fragile than healthy red blood cells. The rounded shape causes the red blood cells to get caught in the spleen, where they easily break down.
The different cases of spherocytosis can be mild with minor symptoms or severe with symptoms that quickly surface. These symptoms can be raised after the happening of some specific types of infections. Severe cases may be diagnosed in childhood, while those with mild symptoms may not be diagnosed until adulthood.
Prevention
Because spherocytosis is an inherited condition, it is not possible to prevent the disease. However, egular screening of individuals at high risk can prevent the risk of complications of the disease with early treatment.
Symptoms
Symptoms of Spherocytosis
Spherocytosis can cause:
- Yellowing of the skin or whites of the eyes i.e. known as jaundice
- Pallor
- Shortness of breath
- Fatigue
- Weakness
In children, irritability and moodiness
Treatment
Treatment of Spherocytosis
Just have a talk with your doctor about the best treatment plan for you. Treatment options include:
Folic Acid Supplementation
Folic acid 1 mg/day and consideration for blood transfusions are suggested during the time of severe anemia.
Surgery
Surgical extraction of the spleen can treat the anemia. The abnormal shape of blood cells remains, but the blood cells are no longer destroyed in the spleen.
Currently, meningococcal, Haemophilus, and pneumococcal vaccines are administered several weeks before splenectomy. Lifetime penicillin prophylaxis is suggested after surgery to prevent dangerous infections. The surgery is not advised for children who are under 5 years old. There is a lifetime risk of serious and potentially life-threatening infections.
