Agammaglobulinemia, x-linked

Treatment

What are the first steps after an initial diagnosis of X-linked agammaglobulinemia?

After a diagnosis of X-linked agammaglobulinemia is confirmed, families should seek a medical consultation with a physician such as an immunologist with experience in treating the disorder. Treatment with gammaglobulin replacement therapy should begin as soon as possible. Families should work with a medical team to develop a plan to treat the disorder. Other initial steps will depend on how the disorder is affecting a person. Families should see a genetic counselor to help understand the genetic aspects of this disorder. Genetic counselors can be found on the National Society of Genetic Counselors website.

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Is there a treatment for X-linked agammaglobulinemia?

What is gammaglobulin replacement therapy for X-linked agammaglobulinemia?

Which formulation should I use to treat X-linked agammaglobulinemia?

What are the side effects of gammaglobulin replacement therapy for X-linked agammaglobulinemia?

Is there a treatment for X-linked agammaglobulinemia?

Currently there is no cure for X-linked agammaglobulinemia (XLA), but it can be effectively treated. People with XLA can be given the antibodies that they are missing, usually by having them injected into the bloodstream (intravenously) or below the skin (subcutaneously). These antibodies will help to protect the body from infection. This treatment should be started as early as possible. People with XLA receive this treatment regularly for the rest of their lives. The treatment is called intravenous immunoglobulin or IVIG. Some chronic infections may still happen despite this treatment. For example, some people may still get chronic sinus infections.

It is important that people with XLA receive prompt antibiotic therapy when they develop an infection. It is recommended that they remain on antibiotics for longer than people without the disorder. Some people with XLA will receive prophylactic antibiotic therapy. This means that they'll receive antibiotics even if they do not have signs of an infection. This is done to try and prevent bacterial infections from happening.

Live viral vaccines, particularly oral polio vaccine, should be avoided. It is also important to avoid live vaccines for people who are living with someone who has XLA so disease from the live vaccine is not transmitted to a person with XLA.

References
What is gammaglobulin replacement therapy for X-linked agammaglobulinemia?

The main treatment for people with X-linked agammaglobulinemia (XLA) is gammaglobulin replacement therapy. This treatment is also called immunoglobulin therapy or antibody therapy. People with XLA lack antibodies, the specialized proteins in the blood that help the body to fight off bacteria, virus, and other foreign substances. This therapy replaces the missing antibodies, which gives people with XLA some protection from infections. These antibodies are given via injection (also call infusion). The antibodies remain in circulation until they are used up, usually a few weeks. Another infusion will then be given. People with XLA will need these infusions for the rest of their lives.

There are two ways to deliver these donated antibodies to people with X-linked agammaglobulinemia, injected directly into a vein (intravenously) or injected just below the skin (subcutaneously). Intravenous infusion, or IVIG, is usually given every 2-4 weeks; subcutaneous infusion, or SCIG, is required weekly.

The Immune Deficiency Foundation has a Patient & Family Handbook that discusses gammaglobulin therapy.

The International Patient Organisation for Primary Immunodeficiencies offers a pamphlet on this topic called Immunoglobulin Replacement Therapy: One Size Does Not Fit All.

References
Which formulation should I use to treat X-linked agammaglobulinemia?

People with X-linked agammaglobulinemia are treated by receiving infusions (injections) of the antibodies they are missing. These replacement antibodies come from healthy donors and are injected directly into the bloodstream or below the skin. There are several different brands of antibody replacement formulations. Your doctor will prescribe you or your child one of these formulations. No one brand has been proven superior to others. The brands are different, however, and they are not interchangeable and cannot be mixed. It is important to know the specific brand that you or your child has been prescribed. Any changes should be made only after discussing it with your physicians.

References
What are the side effects of gammaglobulin replacement therapy for X-linked agammaglobulinemia?

Antibody replacement therapy is safe and effective for most people with X-linked agammaglobulinemia. Some people may have minor side effects like headaches, low-grade fever, chills, rash, muscle aches or abdominal pain. Some people may not be able to tolerate intravenous therapy and will have fewer side-effects on subcutaneous therapy. If you have concerns about any type of treatment, you should discuss them with your doctors.

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