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Diagnosing & Treating
Primary Immune Deficiency

Treating PI

There are a number of treatment options available for patients diagnosed with Primary Immune Deficiency. Chronic infections can be treated with long-term appropriate antibiotics, preventing hearing loss or permanent breathing problems. One of the most common treatments, antibody replacement therapy, is helpful for a range of Primary Immune Deficiencies. There are also other therapies, such as PEG-ADA and gamma interferon, which may be helpful with certain types of immune diseases. Bone marrow transplants may also be considered for some patients.

Antibody Replacement Therapy

For many people with primary immune deficiency, the cause of their PI disease is a lack of the cells that produce protective antibodies. Antibodies are proteins that are produced by white blood cells, which are important cells of the immune system. Antibodies, also called immunoglobulins, help the body destroy the germs that cause infections. Although there are several types of immunoglobulins, the main immunoglobulin in the blood and tissues of the body is immunoglobulin G or IgG.

An important treatment option for Primary Immune Deficiency is infusions of antibodies in large doses. Called antibody replacement therapy, this treatment is given intravenously, which means through a needle directly into a vein. The antibodies have been collected from the plasma of healthy donors and purified through a special process so that the therapy is safe and effective. This purified plasma treatment contains antibodies that can fight different types of germs. Most of the antibodies in this replacement therapy are the IgG class of antibody, also called immunoglobulin or gamma globulin. The treatment is known as 'intravenous immunoglobulin therapy, 'IVIG,' or sometimes 'intravenous gamma globulin.'

IVIG therapy can make a real difference for many people with primary immune deficiency. Effective IVIG therapy helps reduce the frequency and severity of infections in people with a PI disease.

Adverse Reactions

The majority of adverse reactions are mild, transient, and self-limited and do not require discontinuation of therapy.³ These include the following: headache, myalgia, fever, chills, backache, chest pain, nausea, and/or vomiting. Fortunately, most patients with these complaints respond to a reduced infusion rate.⁴ Premedication with acetaminophen, antihistamines, or occasionally steroids also decreases side effects.⁴ Severe reactions occur very infrequently and usually do not contraindicate further IVIG therapy.¹ These reactions are usually related with the use of higher doses of IVIG and include renal failure, thrombotic events, hypotension, congestive heart failure and aseptic meningitis.¹ Most patients at risk for these adverse reactions have an identifiable risk factor.

Effective Therapy

Overall, gamma globulin is an effective therapy for primary immune deficiency because it reduces the number of sick days and serious infections, and the amount of antibiotic needed to fight infections. ⁷

Other treatment alternatives for PI exist and research into the next generation of treatments is in progress.

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