Primary Immune Deficiency Disorder

Medically reviewed by Drugs.com. Last updated on Oct 6, 2024.

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What is a primary immune deficiency disorder (PIDD)?

A PIDD means your immune system did not develop correctly, or does not work correctly. This increases your risk for infections and certain cancers. Infections you had before, such as tuberculosis, may also become active again. Your immune system may also attack healthy cells instead of harmful cells.

What are the signs and symptoms of a PIDD?

• Infections that happen often, especially in your airway, such as sinus infections or pneumonia
• More infections than other people around you
• Infections that cause more severe illness or last longer than in other people
• Bacterial infections that do not get better with antibiotics
• Wounds that do not heal well

How is a PIDD diagnosed?

• A health exam can help your healthcare provider diagnose a PIDD. Your healthcare provider will examine you and ask about your symptoms. Your provider will ask about recent infections or other health problems you have had. Tell your provider about where you have had infections and how long they lasted. Also tell your provider if you have any allergies, such as a food allergy. Your provider will also ask if anyone in your family has a PIDD.
• Blood tests may be used to diagnose the specific kind of PIDD you have. A blood test will show how many antibodies you have. Antibodies are proteins that help fight infection. The number of blood cells you have will also be shown. Too many or too few blood cells can mean your immune system is not working correctly.

How is a PIDD treated?

Treatment may include controlling infections, helping the immune system work better, or treating the cause of the PIDD. You may also need to have an autoimmune disease or cancer treated. You may need any of the following, depending on the kind of PIDD you have:

• Antibiotics prevent or fight a bacterial infection. You may need strong antibiotics given in the hospital if your body does not respond to antibiotics you take at home. You may also need to take antibiotics for a long period of time to prevent infections that cause permanent lung or ear damage.
• Medicines may be given to help strengthen your immune system.
• A bone marrow transplant may be used to increase the number of healthy white blood cells.

What can I do to manage a PIDD?

The following are ways you can help prevent infection and protect or build your immune system:

• Wash your hands often. This can help prevent infection and the spread of germs. Use soap and warm water. Wash your hands after you change a child's diaper and before you prepare or eat food. Use an alcohol-based hand rub if soap and water are not available. Carry gel with you when you leave the house. Dry your hands before you use hand rub. Rub your hands together until all of the liquid has dried.
• Avoid crowds during flu season. Flu season is from late October to the middle of March. Do not have close contact with someone who is sick. Stay 3 to 6 feet away from people when you are in public. Ask friends and family to visit only when they are not sick.
• Ask about vaccines you may need. Get the flu vaccine every year when it becomes available. You may also need the pneumonia vaccine every 5 years. Ask your healthcare provider for more information about vaccines.
• Eat a variety of healthy foods. Healthy foods help your immune system work better. Healthy foods include fruits, vegetables, whole-grain breads, fish, lean meats, low-fat dairy products, and cooked beans.
• Exercise as directed. Exercise can help keep your lungs clear. This will help decrease your risk for a lung infection. Do not exercise outdoors in cold weather or in an area that has pollution.
• Protect your mouth from germs that lead to infection. Brush your teeth at least 2 times per day. Floss every day. See your dentist at least every 6 months.
• Do not smoke. Nicotine and other chemicals in cigarettes and cigars can cause lung damage. Ask your healthcare provider for information if you currently smoke and need help to quit. E-cigarettes or smokeless tobacco still contain nicotine. Talk to your healthcare provider before you use these products.
• Ask if you need to be screened for cancer. A PIDD can increase your risk for certain cancers. Regular screening can help find cancer early.

When should I seek immediate care?

• You have sudden or severe trouble breathing.

When should I contact my healthcare provider?

• You have a fever.
• You have new or worsening symptoms.
• You have questions or concerns about your condition or care.

Care Agreement

You have the right to help plan your care. Learn about your health condition and how it may be treated. Discuss treatment options with your healthcare providers to decide what care you want to receive. You always have the right to refuse treatment. The above information is an educational aid only. It is not intended as medical advice for individual conditions or treatments. Talk to your doctor, nurse or pharmacist before following any medical regimen to see if it is safe and effective for you.

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