WHAT IS SICKLE CELL DISEASE?
Sickle cell disease (SCD) is an inherited, potentially incapacitating blood disorder. it is prevalent not only in the Caribbean and the African-American region, but also in the Mediterranean, the Middle East and India.
Patients with sickle cell disease go through episodes of excruciating pain due to blockage of small blood vessels. Long-term complications can affect any organ, ranging from visual impairment to kidney damage or stroke. Patients are also susceptible to severe infections.
Early diagnosis and proper care can prevent suffering and handicap. Special emphasis needs to be placed on adequate pain management. Pain crises need to be treated promptly and efficiently, since delay or insufficient pain treatment worsens the “sickling” and, in turn, pain and organ damage.
HOW DO YOU GET SICKLE CELL DISEASE?
Sickle cell disease is inherited. The normal hemoglobin is called HbA; the “Sickle Hb” is called HbS. There is also HbC, which is very similar to HbS, and a number of other, less common abnormal Hb’s. A healthy person inherits one gene coding for HbA from his mother and one gene for coding for HbA from his father and will, therefore, test HbAA. A person who has inherited one normal gene coding for HbA and one abnormal gene coding for HbS (or HbC), will test HbAS (or HbAC) and carries the sickle trait. A person who inherited two genes coding for HbS (or HbC), tests HbSS (or HbSC) and has sickle cell disease. Unfortunately, in this situation, each of your children has a 25% risk of being born with sickle cell disease.
WHAT PROBLEMS CAN SICKLE PATIENTS EXPERIENCE?
Problems are mostly due to the red blood cells’ tendency to shred fast (anemia) or to their tendency to “sickle”, thus blocking the flow of blood and oxygen to the tissue (occlusive problems).
Below is a list of possible problems. This list does not aim to be complete. We have not put it together in order to cause you alarm, but in order to alert you to warning signs, so that danger is recognized early and damage can be prevented.
- Vaso-occlusive episodes
- Splenic Sequestration
- Acute Chest Syndrome
- Abdominal problems
IS THERE A PERMANENT CURE?
At this point in time, the only permanent cure for sickle cell disease is a bone marrow transplant. This means, the patient’s bone marrow needs to be destroyed, after which he receives bone marrow from a donor. The procedure is still not entirely satisfactory. It is difficult, risky, not always successful and extremely expensive. We are placing our hopes for the future on the development of gene therapy. Presently, this is not an option – yet. However, for certain patients with serious complications or numerous pain crises, there are treatment options which help to improve their situation. Hydroxyurea, a medication taken by mouth, is one of those options. If the need arises, your doctor will discuss those with you.
Interview with Dr. Hadeed discussing SCD on the Healthy Perspectives television program.
ABOUT THE ANTIGUA & BARBUDA SICKLE CELL ASSOCIATION
The Antigua and Barbuda Sickle Cell Association in its present form operates since 2010. It is a non-profit organization with the explicit goal of improving the lives of Sickle Cell patients and their families. Our mission is to improve care for patients, educate patients, health care providers and general public about the condition and its management, to establish a statistic data base, to counsel patients and persons carrying the Sickle Trait.
ABSCA has only about 20 members, consisting mainly of persons who have first-hand experience with SCD, be it as a parent or as a patient. ABSCA’s president, Dr. Edda Hadeed, is a pediatrician and neonatologist who joined the association end of 2013. Dr. Hadeed manages children with SCD on a regular basis and collaborates with Dr. R. Dickerhoff, Duesseldorf University, a renowned expert in the management of SCD.
To be a unique client and family support; an organization operating in a specialized Clinic/Education Centre for clients and families with Sickle Cell Disorders.
To assist clients in receiving total care and their entitlements and also to understand Sickle Cell Disorders; hence achieving the skills to take good care of themselves.
To administer high quality care and support, educating clients and their families about Sickle Cell Disorders.