About Diamond Blackfan Anemia (DBA):
For those of you who wonder exactly what DBA is that Irelynn has, here’s a wonderful video that has been made by DBA in the United Kingdom. The video gives some of the challenges that our family faces and raises great awareness about blood donation. Thanks to all who take the time to watch. http://animoto.com/play/FBKxRFf6A1o898WHHthLjQ
Diamond Blackfan Anemia was named for Dr. Louis K. Diamond and Dr. Kenneth D. Blackfan, the first doctors who documented cases of the disease in the 1930s.
Most DBA patients are diagnosed within the first year of life. Althought DBA is present at birth, it can be difficult to identify. DBA is not caused by a lack of iron or other nutrients.
The two most common forms of treatment are blood transfusions and corticosteroid medication.
In a blood transfusion, a person receives healthy red blood cells from another person. With DBA, how often transfusions are needed varies from person to person. Transfusions can be needed as often as every 3 to 5 weeks. Red blood cell transfusions have many possible negative side effects, such as iron overload. Iron overload can occur in the liver, heart, and endocrine organs which can cause more complicated health problems such as liver or heart failure, diabetes, thyroid issues, etc. A close watch on iron is monitored and most all patients are on chelation medicine to remove the excess iron from their organs. DBA patients may choose to undergo the risky procedure of a stem cell transplant (also referred to as a bone marrow transplant).
Corticosteroids, or oral prednisone, are drugs used to treat DBA and are one of the most successful treatments. Although, some people with DBA may not respond to steroid treatment. Other DBA treatment options are being studied, but to date none work as well as corticosteroids or transfusion therapy. One day, a safe, reliable cure, perhaps using gene therapy, might be possible. However, this is still many years away.
The DBAR defines “remission” as an adequate hemoglobin level without any treatment, lasting 6 months, independent of prior therapy. The actuarial likelihood of remission is 20% by age 25 years, with 72% experiencing a remission during the first decade of life. Some patients have experienced more than one remission in their lifetime.
People with DBA can live long, healthy, active lives if they get good medical care and live a healthy lifestyle. As long as their hemoglobin levels are high enough (hemoglobin is the substance in the red blood cells that supplies oxygen to all cells in the body), people with DBA can take part in all activities, usually without limitations.