My child was diagnosed with X-linked agammaglobulinemia in 2008. He as been on antibiotics but now they are no longer effective, and in our country we don't have intravenous immunoglobulin (IVIG). What should I do to keep my child?
Managing X-linked agammaglobulinemia (XLA) mainly consists of preventing infections and treating infections aggressively when they do occur. Sudden infections in individuals with XLA are usually treated with antibiotics that are taken for at least twice as long as taken in healthy individuals. Preventing bacterial infections is very important for people with XLA. Gammaglobulin (a type of protein in the blood that contains antibodies to prevent or fight infections) is the main treatment for people with XLA. In the past, most people received this by intravenous (IV) infusion every two to four weeks. However, in the last few years, an increasing number of people have been receiving it by weekly subcutaneous injections. The choice of whether to receive it intravenously or by injection may just depend on what is most convenient for the doctor and/or patient. Sometimes, people with XLA have a reaction to gammaglobulin, which may include headaches, chills, backache, or nausea. These reactions are more likely to occur when they have a viral infection or when the brand of gammaglobulin has been changed. Some centers use chronic prophylactic antibiotics (continuous use of antibiotics) to prevent bacterial infections.
Aggressive use of antibiotics lower the chance of chronic sinusitis and lung disease, which are common complications in individuals with XLA. Early diagnosis and treatment of bowel infections may decrease the risk of inflammatory bowel disease (IBD). Furthermore, children with XLA should not be given live viral vaccines. For example, they should be given inactivated polio vaccine (IPV) rather than the oral polio vaccine. The siblings of children with XLA should also be given inactivated polio vaccine (IPV) rather than oral polio vaccine in order to avoid infecting their affected sibling with live virus.
Last updated: 8/3/2011
What can I do if intravenous infusion of gammaglobulin is not available where I live?
Although gammaglobulin is the treatment of choice for XLA, there may be alternatives to intravenous gammaglobulin. In the last few years, an increasing number of people have been receiving gammaglobulin treatment by weekly subcutaneous injections. This is an injection (like a shot) given in the fatty layer of tissue just under the skin. Injection may also have the advantage of providing IgG levels that are relatively constant, compared with the highs and lows of levels that may occur with IV therapy. Another advantage is that injections may be given at home. Disadvantages of having injections may include discomfort where the injections are given, and the potential lack of medical supervision by doctors (because coming to the hospital for infusions may no longer be necessary). Fresh frozen plasma (FFP), used in blood transfusions, has been used in the past but carries the major risk of potential transmission of infectious diseases.
If you do not have access to gammaglobulin or other treatments where you live, it is recommended that you contact the various support groups for XLA that we have listed on our Web site. You can see the contact information for these support groups by clicking here. These groups may be able to help you either locate a physician or treatment center that is accessible, or provide you with information about how to obtain gammaglobulin or other treatments for XLA.
Last updated: 8/3/2011
We hope this information is helpful. We strongly recommend you discuss this information with your doctor. If you still have questions, please