Treatment

Treatment for PID is varied depending on the type of PID, and severity.Treatments for PID involve:

  • treatments to boost the immune system
  • managing infections
  • treatment to cure PID
  • boosting the immune system and
  • treating the underlying cause of the immune problem

Treatment to boost the immune system

Immunoglobulin replacement therapy

Immunoglobulin consists of antibody proteins needed for the immune system to fight infections. Immunoglobulin replacement therapy is used in the treatment of several immune deficiencies.

  • IVIg – Intravenous immunoglobulin is administered by a nurse intravenously in a hospital infusion ward every 2 to 4 weeks to maintain sufficient levels of immunoglobulin.
  • SCIg – Subcutaneous immunoglobulin is self-administered by the patient using a pump and small needle(s) inserted under the skin at home.

More information about Immunoglobulin replacement therapy

Managing infections

Antibiotics

Infections are typically treated with antibiotics. In cases where infections don’t respond to standard medications, hospitalisation and treatment with intravenous  antibiotics may be necessary. Some PID patients need to take antibiotics long term to prevent infections from occurring and to prevent permanent damage to the lungs and ears.

Steroids

For severe inflammation, corticosteroids may be necessary

Treating symptoms

Patients may need medications to relieve symptoms caused by infections, such as panadol and ibuprofen for pain and fever, decongestants for sinus congestion, and expectorants toclear airways.

Immunomodulation

Gamma interferon therapy. Interferons are naturally occurring substances that fight viruses and stimulate immune system cells. Gamma interferon is a man-made (synthetic) substance and is given as an injection in the thigh or arm three times a week. It is used to treat chronic granulomatous disease.

PEG-ADA

Injections of specially treated, purified enzyme Adenosine Deaminase (ADA). It is used in the treatment of SCID.

Bone Marrow transplant

Stem cell / Bone marrow transplantation

For a few vary rare Primary Immune Deficiencies (e.g. Chronic Granulomatous Disease), bone marrow transplant can result in a cure.

With this treatment, normal stem cells are transferred to the person with PID, giving them a normally functioning immune system. They are harvested through bone marrow, or they can be obtained from the placenta at birth (cord blood). To be successful, the donor — usually a parent or other close relative — must have body tissues that are a close biological match to those of the person with PID. Stem cells that aren’t a good match may be rejected by the immune system.

Even with a good match, stem cell transplants don’t always work. The treatment often requires that any functioning immune cells be destroyed using chemotherapy or radiation prior to the transplant, leaving the recipient even more vulnerable to infection temporarily.

Future treatments

Gene therapy

Researchers hope this treatment will one day be a cure for PID and many other conditions. Gene therapy replaces defective genes with working genes. A harmless virus is used to carry the genes into the body’s cells. In turn, the newly introduced genes trigger the production of healthy immune system enzymes and proteins. Experts have identified many of the genes that cause PIDs — but there are many issues. E.g. some of the missing or defective genes are only activated during the early development of the immune system, so even if scientists can figure out how to get that gene where it needs to be, it would also have to trigger the development of the missing functions. Although the technique has shown promise in some initial trials, gene therapy is still experimental.

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