Frequently Asked Questions

Q: What is Diamond Blackfan Anemia?

A: Diamond Blackfan Anemia (or DBA) is a rare blood anemia disorder in which the bone marrow does not produce red blood cells. The rest of their blood cells (the platelets and the white blood cells) are normal.

Q: How many people have DBA?

A: There are about 25-35 new cases of DBA per year in the United States and Canada. Only about 600 people in the US and Canada have DBA. It’s estimated to be 7 cases for every million children born in the US and Canada.

Q: Is there a cure for DBA?

A: The only cure available for DBA is bone marrow transplantation, which replaces the person’s defective bone marrow with healthy marrow. However, transplantation is a difficult procedure to go through and it doesn’t always work. It is usually reserved for people whom steroid medications and blood transfusions don’t help. 20% of patients with DBA go into spontaneous remission. That’s what Irelynn’s family prays for her.

Q: How did Irelynn get DBA?

A: Irelynn was born with her own genetic mutation of DBA (RSP26). Her father, mother & sister do not carry DBA. Irelynn has a 50% chance of passing DBA onto her children.

Q: How often does Irelynn need blood?

A: Irelynn receives blood transfusions every 3 weeks. She’s had 70+ blood transfusions to date.

More About 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.

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.