If you have been diagnosed with primary immune disorder, also called primary immunodeficiency disease, you may have questions about what kinds of treatments are available to you.
There are over 150 recognized forms of primary immune disorders, and all of them can cause a weakened immune system due to the body’s inability to make enough antibodies. When you don’t have enough antibodies to fight infection, you are more vulnerable to opportunistic infections which may be recurring and difficult to treat.
These infections may include recurrent upper respiratory, gastrointestinal, or skin infections that do not respond to treatment. You may finish a course of antibiotics only to find that you are not feeling any better or have even gotten worse. In children, delayed growth and development may also signal the presence of primary immunodeficiency. Anemia, low platelet counts, and autoimmune disorders such as lupus and rheumatoid arthritis may also indicate the presence of the disorder.
If you are concerned that you or a child may have immune system deficiencies, talk to your primary care provider or pediatrician right away. If he or she suspects an immune disorder you will probably be referred to an immunologist who specializes in diagnosing and treating the disease.
Antibiotic treatment for primary immune disorder
If you have a compromised immune system due to an immune disorder, you may be at greater risk of opportunistic infections, which are difficult to treat and may worsen quickly. For example, a common cold can quickly turn into bronchitis or pneumonia. Sometimes your doctor will ask you to take antibiotics long-term to prevent these types of infections from occurring. In some cases, antibiotics may need to given intravenously while you are being monitored in the hospital. Long-term preventative antibiotics are often recommended for children with primary immune disorder since they are especially prone to infections. Chronic upper respiratory and ear infections can lead to permanent lung and ear damage when not managed appropriately. It’s important to know that immunocompromised children should not receive vaccines containing live viruses, such as measles-mumps-rubella (MMR) or oral polio.
People who are unable to produce their own antibodies may benefit from immunoglobulin (Ig) therapy. Ig comes from the plasma of healthy donors and contains antibodies to many different types of bacteria and viruses. Plasma donors are screened for contagious diseases according to the standards of the American Association of Blood Banks and the U.S. Food and Drug Administration. The Ig is then extracted from the donor plasma before being given to the patient.
If your doctor recommends Ig therapy, you should know that it offers temporary immunity and must be repeated periodically in order to maintain its effectiveness. Your Ig infusion is administered either intravenously (through a vein) or subcutaneously (through the skin) and usually takes between two to four hours. Typically, intravenous infusions need to be repeated approximately every three to four weeks but may be administered more frequently depending on your age and doctor’s recommendation. Subcutaneous infusions require more frequent administration, normally once or twice per week. You will probably need to continue Ig therapy throughout your life in order to keep your immune system healthy.
Some frequently reported side effects of Ig therapy include muscle or joint aches, low-grade fever, and headaches. In some cases, allergic symptoms such as chest tightness and hives can occur. These can usually be relieved with an antihistamine such as Benadryl.
Stem Cell Therapy: A Potential Cure for Primary Immune Disorder
You may have heard about a newer treatment known as stem cell therapy for primary immune disorder. Stem cells are a type of cell usually present in bone marrow that can divide over and over to produce more specialized types of cells, such as the white blood cells needed to maintain a strong immune system. Hematopoietic stem cell transplantation (HSCT) is effective in certain types of severe primary immune disorder. Although bone marrow has traditionally been the source of stem cells, more and more parents are now choosing to store their newborns’ cord blood which is an excellent source of stem cells.
If you are a candidate for stem cell therapy and you do not produce enough healthy stem cells of your own, a donor must be found. A healthy donor’s stem cells can help replace your deficient immune system with a functional one, thereby curing the disorder. HSCT can be a very effective treatment and cure for many patients. The transplantation itself is relatively simple and is similar to receiving a blood transfusion. However, you should talk with your doctor about all potential risks and benefits before choosing HSCT.
One possible complication is that your body may still have enough of a functioning immune system to attack or reject the donor cells as “foreign.” Prior to undergoing HSCT, you will likely need chemotherapy or radiation treatment to further weaken your remaining immune system so that it does not reject the donor cells. These treatments come with side effects of their own. Anemia and persistent bleeding are common after chemotherapy. Blisters in the mouth are another side effect which could make it difficult for you to consume adequate fluid and nutrition. Chemotherapy will also leave your immune system in an extremely weakened state and at greater risk for opportunistic infections.
A second complication can arise when the donor’s antibodies perceive your own tissues as foreign and attack them. Medications to suppress inflammation and antibody activation are commonly used to prevent this. It is also important for you and your donor to be a good match. Your siblings have a 25% chance of being a donor match for you. If you do not have a family member who is a match, your doctor can also assist with finding a suitable match through the Bone Marrow Donors Worldwide database.
If you have a severe primary immune disorder, stem cell therapy offers great hope for a cure. Because of the risks involved, it is usually recommended only in the most severe cases. Talk with your doctor about whether stem cell therapy or HSCT may work for you.