Information About Sickle Cell Disease

Sickle cell disease is an inherited form of anemia – a condition in which red blood cells are unable to carry oxygen throughout the body. For most, red blood cells are round and can move easily through blood vessels, but the red blood cells in people with sickle cell disease are crescent, or half-moon shaped. These irregular shaped cells can get stuck in blood vessels, which can slow or block the flow of oxygen to certain parts of the body.

In addition to being irregular in shape, sickle cells are fragile and break apart easily. Normal red blood cells live an average of four months before they die and need to be replaced. Sickle-shaped cells however only live an average of 20 days. The result of this shortage of blood cells is a loss of energy and general sense of fatigue.
Other symptoms of sickle cell disease include:

• Hand-Foot Syndrome – Often the first sign of sickle cell disease. It is caused by a lack of blood flow to the hands and feet

• Episodes of Pain – Referred to as a “crisis”, these episodes of pain occur when blood flow is blocked to the chest, abdomen, and joints. The frequency and duration of the episodes vary from person to person, but in severe cases, they can result in hospitalization.

• Frequent Infections and Fever– Sickle Cell can cause damage to the spleen, an organ that fights infection, making those with sickle cell at greater risk of developing an infection and an accompanying fever.

• Changes in Skin – People with sickle cell disease can develop a yellow tint to their skin or the whites of their eyes. Skin and nail beds can often become pale.

• Delayed growth – By not receiving enough oxygen rich red blood cells, those with sickle cell disease may also not get the necessary nutrients essential for growth.

The risk of inheriting sickle cell disease is a genetic one. For a baby to be born with it, both parents must carry the sickle cell gene. Doctors can diagnose sickle cell disease before a child is born. Couples who are at risk for passing on this disease to their children may want to talk with a genetic counselor about prenatal testing. The sickle cell gene is more common in families that come from Africa, India, Carribbean islands, and Central and South America.

To determine if you have sickle cell disease, your doctor can order a test to check for hemoglobin S, the defective form of hemoglobin that underlies sickle cell anemia. Further tests can confirm the existence of one gene (carrying the sickle cell trait) or two genes (sickle cell anemia). For those who have sickle cell anemia, treatment is aimed at treating the symptoms and avoiding crisis. Regular check-ups to monitor your red blood cell count are important. Medications are available to reduce pain and prevent complications can be prescribed, and blood transfusions, supplemental oxygen and even bone marrow transplants may also be necessary.

Jamaica Hospital serves a culturally rich and diverse population. Many members of our community are from the parts of the world most often affected by sickle cell disease. We encourage anyone living with sickle cell disease to carefully manage their condition. The hospital also recommends all potential parents to be tested for the sickle cell trait.

All content of this newsletter is intended for general information purposes only and is not intended or implied to be a substitute for professional medical advice, diagnosis or treatment. Please consult a medical professional before adopting any of the suggestions on this page. You must never disregard professional medical advice or delay seeking medical treatment based upon any content of this newsletter. PROMPTLY CONSULT YOUR PHYSICIAN OR CALL 911 IF YOU BELIEVE YOU HAVE A MEDICAL EMERGENCY.