SCTPN STORY OF THE DAY
ABNORMAL HEMOGLOBIN TRAITS | THALASSEMIA | "SICKLE CELL ANEMIA AND EXJADE"
Sickle Cell Disease is a genetically inherited blood disorder prevalent among people of African, Caribbean, Central/South American, East Indian, Middle Eastern, Mediterranean, Asian and Southeast Asian ancestry. Sickle cell trait and disease is indigenous to all these populations because of malaria. These genetic mutations developed over thousands of years as a result of the human body’s natural immune response against malaria.
The disease process results in severe anemia, oxygen deprivation, poor circulation and extreme pain. Painful episodes known as “crisis” are disabling and debilitating. Complications associated with sickle cell disease are:
- Enlargement and damage of the spleen (life threatening, especially in children).
- Dysfunction or acute failure of the liver or kidneys.
- Damage to tissue and bone due to diminished blood flow and loss of oxygen.Organs most commonly affected are the heart, lungs, brain, shoulders and hips bones as well as upper and lower extremities - resulting in the development of arm and leg ulcers.
- Stroke due to infarction of blood vessels.
In some cases, beginning as early as the age of 6 months, individuals with sickle cell disease can experience frequent, severe painful episodes that require multiple hospitalizations for treatment. It is common to spend weeks, sometimes months in the hospital for excruciating crisis episodes, especially when accompanied by other complications. Even in the case of adequate medical care, severe disability or death may result in childhood and early adult years.
THALASSEMIA
Thalassemia, also known as Cooley’s Anemia is a genetic disorder, which produces severe anemia due the low production of beta (β)-globin proteins in the hemoglobin molecule of red blood cells. There are two types of thalassemia, alpha (a) thalassemia and beta (β) thalassemia. They are present in Africa, S.E. Asia, Southern China, India, Greece, Italy and the Middle Eastern countries. β thalassemia symptoms include jaundice, extreme fatigue, enlargement of the heart and liver, bone deformation, damage to organs and organ systems. Individuals with β thalassemia are also prone to developing diabetes. Children diagnosed with β thalassemia require regular blood transfusions to maintain good health and survive. a Thalassemia also known as hemoglobin H disease (Hb AH) is a milder condition.
SICKLE CELL ANEMIA AND EXJADE™—CLAUDETTE GOULBOURNE
IRON DISORDERS INSTITUTE NEWSLETTER | VOLUME 7, ISSUE 2 | MARCH/APRIL, 2007 Iron Patients - Their Own StoriesI am presently participating in a four year oral chelation study because of very high iron overload resulting from blood transfu-sions for sickle cell anemia. Monthly trans-fusions were the solution to relieve years of experiencing acute chest syndrome, which later developed into pulmonary hyperten-sion. It was imperative that I do something to relieve my system of this excess iron be-fore the iron damaged any of my organs.
My physician suggested using a Desferal® infusion for remov-ing the excess iron. My thinking at that time was that Des-feral® was invasive and too time consuming. My physician continued to stress the danger that existed because of high iron level. I was later informed about a study by Novaritis for the oral chelation ICL670, which seemed much more reason-able to work with because it is taken orally rather than intra-venously.
I realize that every study has its pros and cons, which must always be considered. Some of my concerns were changes in vision, hearing, and possible kidney and liver damage. I have noticed that over the years I have been in the study there have been changes in my vision and hearing.
I am tested every six months to determine if organ damage has occurred from the Exjade™. My weight is measured so that the appropriate dose of Exjade™ can be administered. Additionally, I have monthly blood work to measure decreases in iron. READ MORE on page 2