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Primary Immune Deficiency Disorder in Children

Medically reviewed by Last updated on Jan 5, 2023.

What is a primary immune deficiency disorder (PIDD)?

A PIDD means your child's immune system did not develop correctly, or does not work correctly. The immune system protects the body from infection and some kinds of cancer. PIDD increases your child's risk for infections and certain cancers. Infections your child had before, such as tuberculosis, may become active again. Your child's immune system may also attack healthy cells instead of harmful cells. Most PIDDs in children are genetic disorders. This means it was passed to your child by one or both parents. A PIDD may be diagnosed shortly after your baby's birth, or it may not show for months or years.

What are the signs and symptoms of a PIDD?

  • More infections than other children
  • Infections that cause more severe illness than in other children
  • Bacterial infections that do not get better with antibiotics
  • Not gaining weight or growing as fast as he or she should because of infections that happen often
  • Wounds that do not heal well

How is a PIDD diagnosed?

Your child's healthcare provider will examine your child. Tell the provider if anyone in your child's family has a PIDD. The provider will ask if your child is sick often or has wounds that do not heal well. If your child is young, he or she might not have been exposed to many viruses or bacteria. This may make it hard for your provider to know if your child has PIDD. Keep a record of all your older child's illnesses and allergies, including food allergies. Include when the illness started and stopped, and if medicine treated infections properly. Your child may also need any of the following:

  • Blood tests may be used to diagnose the specific kind of PIDD your child has. A blood test will show how many antibodies your child has. Antibodies are proteins that help fight infection. The number of blood cells your child has will also be shown. Too many or too few blood cells can mean your immune system is not working correctly.
  • X-ray CT, MRI, or ultrasound may be used to check the size of your child's thymus. The thymus gland makes cells that help your child's body fight infections. A large thymus may mean your child has a PIDD.

How is a PIDD treated?

Treatment may include controlling infections, helping the immune system work better, or treating the cause of the PIDD. Your child may also need to have an autoimmune disease or other problem treated. Your child may need any of the following, depending on the kind of PIDD he or she has:

  • Antibiotics prevent or fight a bacterial infection. Your child may need strong antibiotics given in the hospital if his or her body does not respond to antibiotics taken at home. Your child may also need to take antibiotics for a long period of time to prevent infections.
  • Medicines may be given to help strengthen your child's immune system.
  • A bone marrow transplant may be used to increase the number of healthy white blood cells.

What can I do to help manage my child's PIDD?

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

  • Ask about vaccines your child may need. Vaccines help protect your child from infections. Infections can be serious in children who have a PIDD. Your child may not be able to get vaccines that contain live viruses, such as the oral polio vaccine. Your child's immune system may not be able to prevent an infection from the live vaccine.
  • Have your child wash his or her hands often. This can help prevent infection and the spread of germs. Anyone who cares for your child should also wash often. Use soap and warm water. Everyone should wash their hands after changing a child's diaper and before preparing or eating food. Use an alcohol-based hand gel if soap and water are not available. Have your child bring gel when he or she leaves the house. Teach your child to dry his or her hands before using hand rub. Rub hands together until all of the liquid has dried.
  • Keep your child away from crowds during flu season. Flu season is from late October to the middle of March. Do not let anyone who is sick care for your child. Keep your child 3 to 6 feet away from people when he or she is in public. Ask friends and family to visit only when they are not sick.
  • Offer your child a variety of healthy foods. Healthy foods help your child's immune system work better. Healthy foods include fruits, vegetables, whole-grain breads, fish, lean meats, low-fat dairy products, and cooked beans. If your baby drinks formula, mix it with distilled water only to help prevent infection.
  • Encourage your child to exercise. Exercise can help keep your child's lungs clear. This will help decrease his or her risk for a lung infection. Do not let your child exercise outdoors in cold weather or in an area that has pollution.
  • Protect your child's mouth from germs that lead to infection. Have your child brush and floss his or her teeth at least 2 times per day. Help your young child brush and floss. Ask your child's dentist what kind of toothpaste your child should use. You can rub a wet cloth over your baby's teeth to clean them. Take your child to the dentist at least every 6 months.
  • Talk to your adolescent about not smoking. Nicotine and other chemicals in cigarettes and cigars can cause lung damage. Ask your adolescent's healthcare provider for information if he or she currently smokes and needs help to quit. E-cigarettes or smokeless tobacco still contain nicotine. Talk to your healthcare provider before your adolescent uses these products.
  • Ask if your child needs to be screened for cancer. A PIDD can increase your child's risk for certain cancers. Regular screening can help find cancer early. Screening might not begin until your child is an adult.

When should I seek immediate care?

  • Your child has sudden or severe trouble breathing.

When should I contact my child's healthcare provider?

  • Your child has a fever.
  • Your child has new or worsening symptoms.
  • You have questions or concerns about your child's condition or care.

Care Agreement

You have the right to help plan your child's care. Learn about your child's health condition and how it may be treated. Discuss treatment options with your child's healthcare providers to decide what care you want for your child. 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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