Many DBA patients need regular blood transfusions. This blood contains the red blood cells they cannot produce themselves, which themselves contain a protein called haemoglobin. Iron is a crucial component of this haemoglobin. Iron is not easily absorbed into nor excreted from the body, but in people with properly functioning bone marrow the iron is reused when new red cells are produced from old red cells as they die in a natural cycle of renewal. However, this does not occur in DBA patients and those patients who need regular transfusions can accumulate iron in their bodies. This can be toxic at high levels and dangerous to organs in the body.
Chelation therapy means taking drugs which help remove some of this excess iron from the body.
One common chelation drug is Deferoxamine (Desferal) which is taken over a period of many hours (often overnight) by insertion of a small needle under the skin (subcutaneously), commonly 5-7 nights a week. Most patients (or parents) do this themselves at home.
Another, more recent, chelation drug is Deferasirox (Exjade) which can be taken orally.
These are the two iron chelation drugs mainly used in the UK. Both of these drugs are only prescribed after consultation with medical professionals, and either may be more or less appropriate for individual cases. Patients should discuss this with their consultant.
Some form of effective chelation therapy is essential for DBA patients on long-term blood transfusions.
For further information a full guide on Chelation prepared by the CDC (a US organisation) is available.
DBA UK is not a medical charity and is not qualified to give medical advice on DBA. Please talk with your doctor or health care provider if you are worried about iron chelation. However, if you would like to talk to other DBA patients and families who may have been in the same position as you then please contact us.