Treatments for primary immunodeficiency involve preventing and treating infections, boosting the immune system, and treating the underlying cause of the immune problem. In some cases, primary immune disorders are linked to a serious illness, such as an autoimmune disorder or cancer, which also needs to be treated.
- Treating infections. Infections require rapid and aggressive treatment with antibiotics. Infections that don't respond may require hospitalization and intravenous (IV) antibiotics.
- Preventing infections. Some people need long-term antibiotics to prevent respiratory infections and associated permanent damage to the lungs and ears. Children with primary immunodeficiency may not be able to have vaccines containing live viruses, such as oral polio and measles-mumps-rubella.
Treating symptoms. Medications such as ibuprofen (Advil, Motrin IB, others) for pain and fever, decongestants for sinus congestion, and expectorants to thin mucus in the airways may help relieve symptoms caused by infections.
Postural drainage — using gravity and light blows to the chest to clear the lungs — may help relieve the discomfort of repeated (chronic) respiratory infections.
Treatment to boost the immune system
- Immunoglobulin therapy. Immunoglobulin consists of antibody proteins needed for the immune system to fight infections. It can either be injected into a vein through an IV line or inserted underneath the skin (subcutaneous infusion). IV treatment is needed every few weeks, and subcutaneous infusion is needed once or twice a week.
- Gamma interferon therapy. Interferons are naturally occurring substances that fight viruses and stimulate immune system cells. Gamma interferon is a manufactured (synthetic) substance given as an injection in the thigh or arm three times a week. It's used to treat chronic granulomatous disease, one form of primary immunodeficiency.
- Growth factors. When immune deficiency is caused by a lack of certain white blood cells, growth factor therapy can help increase the levels of immune-strengthening white blood cells.
Stem cell transplantation
Stem cell transplantation offers a permanent cure for several forms of life-threatening immunodeficiency. Normal stem cells are transferred to the person with immunodeficiency, giving him or her a normally functioning immune system. Stem cells can be harvested through bone marrow, or they can be obtained from the placenta at birth (cord blood banking).
The stem cell donor — usually a parent or other close relative — must have body tissues that are a close biological match to those of the person with primary immunodeficiency. Even with a good match, however, stem cell transplants don't always work.
The treatment often requires that functioning immune cells be destroyed using chemotherapy or radiation before the transplants, leaving the transplant recipient temporarily even more vulnerable to infection.
Jan. 20, 2015
- Primary immunodeficiency. National Institute of Child Health and Human Development. http://www.nichd.nih.gov/publications/pubs/Pages/primary_immuno.aspx. Accessed Nov. 23, 2014.
- About primary immunodeficiencies. Immune Deficiency Foundation. http://primaryimmune.org/about-primary-immunodeficiencies/. Accessed Dec. 2, 2014.
- Pasternack MS. Approach to the adult with recurrent infections. http://www.uptodate.com/home. Accessed Nov. 23, 2014.
- Stiehm ER. Approach to the child with recurrent infections. http://www.uptodate.com/home. Accessed Nov. 23, 2014.
- Primary immunodeficiency diseases. American Academy of Allergy, Asthma and Immunology. http://www.aaaai.org/conditions-and-treatments/primary-immunodeficiency-disease.aspx. Accessed Nov. 23, 2014.
- Berger M. Immune globulin therapy in primary immunodeficiency. http://www.uptodate.com/home. Accessed Nov. 23, 2014.
- General care. Immune Deficiency Foundation. http://primaryimmune.org/about-primary-immunodeficiencies/relevant-info/general-care/. Accessed Nov. 3, 2014.