Immune Deficiency Disorders: Treatment

People living with immune deficiency can lead active and full lives. The goal of treating an immune deficiency is to help you regain or maintain control of your life. 

For many people this may include:

• Participating in work, school, family and social activities

• Decreasing the number and severity of infections

• Having few, if any, side effects from medications and other treatments

• Feeling good about yourself and your treatment program 

Your doctor may prescribe medications or other treatments to make you feel better and to protect you from foreign invaders. Medications and treatments must be chosen for your individual needs and may be adjusted as your needs change.

Medications and treatments for immune deficiencies include:

• Antimicrobial therapy to fight and prevent infections

• Immune globulin replacement therapy

• Vaccinations

• Specialized immune globulins

• Hematopoietic cell transplant

• Gene therapy

• Enzyme replacement therapy

• Biologics

Antimicrobial Therapy

Antimicrobials, such as antibiotics and antifungals, are medications that fight bacterial or fungal infections. Your doctor may perform additional testing, such as imaging (X-ray or CT scan), or obtain cultures from the infected area to determine if a pathogen, bacteria, virus or fungus is there and to identify it. Your doctor also can test how responsive your infection is to various antimicrobial medications so that she or he can choose the one most likely to kill the invader. This is called determining the sensitivity.

Antimicrobial therapy also may be used to prevent infections. Your doctor may recommend that you take antimicrobial medications daily or a few times per week even when you are feeling well to help you prevent infections.

Talk with your doctor about the potential side effects of antimicrobials.  

Immune Globulin Replacement Therapy

People who are unable to produce adequate amounts of functional antibodies or immune globulins may benefit from immune globulin replacement therapy. There are four different types of immune globulins: IgG, IgA, IgM and IgE. Antibody replacement with gamma globulin replaces IgG in the blood. This can be given both intravenously (IVIG) or under the skin, called subcutaneously (SCIG).

Gamma globulin is made of pooled antibodies from the serum, a component of blood, of healthy people. In each treatment, antibodies from at least 1,000 donors provide protection against a wide variety of foreign invaders. The blood is carefully tested and discarded if there is evidence of contagious diseases such as hepatitis or HIV. Talk with your doctor if you have any questions or concerns.

Because supplemental IgG therapy (IVIG or SCIG) contains minimal amounts of IgA or IgM, their protective functions are not replaced. People with immune deficiencies may continue to have trouble with certain types of infections despite supplemental IgG therapy. However, treatment with supplemental IgG and early treatment of infections help many people with immune deficiencies lead active and full lives by preventing the most common infections. These are infections that the majority of people are vaccinated against. Because people with immune deficiencies may not mount strong protective immune responses to vaccines, immune globulin replacement can take the place of this. 

Specific Immune Globulins

Pathogen-specific immune globulins may be used in some immunocompromised people for prophylaxis against potentially life-threatening infection from the offending pathogen. For example, monoclonal antibody treatment against respiratory syncytial virus (RSV) is used to prevent RSV infection in high-risk populations. Other examples include using specific IgG to provide protection in the case of Hepatitis B, botulis, or rabies exposure. Lastly, targeted immune globulins are being used to treat COVID-19 in certain groups of people.

Other Treatments

Other treatments are available for some types of immune deficiencies. Bone marrow transplants, hematopoietic cell transplantation, thymus transplantation, gene therapy, enzyme replacement and biologic cytokine inhibitors are examples of other treatments. Researchers in immunology are experimenting with gene therapy and other treatments that will be available in the future. 

Helpful Hints for Remembering Medications

• Plan to take your medications at the same time as a daily activity like waking up, brushing teeth, eating meals and going to bed.

• Use a medication checklist or worksheet to record when medications are taken. Place the checklist someplace visible to use as a reminder.

• Pack medications in pillboxes to help you remember to take them.

Preventing Infections

There are things you can do to prevent infection. They include:

• Avoiding people who have a cold or other infection.

• Wearing a mask in indoor spaces.

• Washing your hands with soap before eating, after outings and after using the bathroom.

  • Although hand-washing seems simple, make sure it is effective. Use liquid soap and scrub your hands using plenty of lather for 10-15 seconds. Rinse your hands and dry them completely.

  • If a sink is not available, use alcohol-based hand sanitizer over the entire surface of your hands until dry.

• Clean cuts and scrapes right away with warm, soapy water. Apply antibacterial cream or ointment and cover the cut or scrape with a fresh bandage at least daily. Tell your doctor if any redness or drainage develops.

• Brush your teeth after meals, and floss at least daily. Have a dental exam every six months. Tooth decay and gum disease are types of infection.

• Keep your vaccinations up to date. You should receive your yearly influenza vaccination, as well as vaccination for the most common causes of pneumonia. COVID vaccination also is essential. Getting vaccinated may lessen the severity of symptoms or prevent these infections altogether.

If you notice any of the following, you may have an infection:

• Fever

• Chills

• Nasal congestion or discharge

• Cough

• Diarrhea

• Vomiting

• Tenderness or pain

• Unusual discharge

• Swelling or redness on the skin

If you suspect you have an infection of any kind, call your doctor right away.

COVID-19 and Immune Disorders

Patients with immune deficiency and immune system disorders are at increased risk of severe and life-threatening COVID-19.

All patients should receive one of the available COVID-19 vaccines and a booster dose as this is the best protection from hospitalization and death from the virus. Most patients with immune deficiency and immune dysregulation do not make completely protective antibody responses to vaccinations. However, the other parts of the immune system will be active in responding to vaccination. This adds the extra protection that people with immune deficiency need to fight this deadly disease. Also, if you are on immune globulin replacement therapy, making sure you do not miss any doses is very important to keeping yourself protected from COVID-19.

Almost all patients with immune deficiency are eligible to receive monoclonal antibody infusion therapy immediately if they are exposed to COVID-19 or if they are in the very early stages of infection. Let your doctor know immediately if you test positive for COVID-19.

Patients with immune deficiency should physically distance themselves from others and wear a mask in accordance with CDC guidelines. You should avoid being around people who are sick.

See the latest COVID-19 updates from the Immune Deficiency Foundation.