Primary immunodeficiency disorders (PIDD) diagnostic & treatment center

When a child has an infection or virus, the illness typically runs its course – and the child bounces right back into action. But for children with primary immunodeficiency diseases (PI), bouncing back is not that simple. Primary immunodeficiency disorders (PIDD) are conditions in which the immune system is weakened or impaired, leaving individuals more susceptible to infections and illnesses. The multidisciplinary team of pediatric specialists at Advocate Children’s Hospital is highly experienced in caring for children with PIDD and committed to improving their quality of life.

The immune system is a complex, finely balanced system that defends against infections. If any part of this system is inherently weak or out of balance, such as with PIDD, a child becomes susceptible to a large variety of serious, and sometimes fatal, infections and autoimmune problems. Many of these are from common organisms that, for a child with a fully functioning immune system, would usually cause a low-grade infection or even no noticeable symptoms.

Children with primary immunodeficiency face their own distinct needs and challenges. Our dedicated team creates personalized treatment plans for your child, meeting monthly to discuss the best course of care for PIDD. While primary immunodeficiency is a chronic condition that children will live with for the rest of their lives, our specialists are committed to helping children grow up to live full, independent lives.

What is primary immunodeficiency disease?

Primary immunodeficiency diseases (PI) are conditions that prevent the immune system from functioning properly. Children with PI are born without some of the body’s immune defenses, therefore making them more susceptible to germs that cause infections. Children living with PI often suffer from infections that fail to respond to medications – even antibiotics.

More than 300 types of primary immunodeficiency are known. Each type of PIDD varies in severity and symptoms for each person. In addition to infections, children with PI are susceptible to other conditions, such as chronic lung disease and autoimmune disorders like arthritis.

Signs & symptoms of PI disease

Signs and symptoms of primary immunodeficiency vary from patient to patient. Some forms of primary immunodeficiency are very mild and go unnoticed for years. Other forms of PIDD are more severe and discovered at birth. The most common sign of PIDD in children is an increased vulnerability to infections that fail to respond to typical treatments.

A child may have a primary immunodeficiency with symptoms such as:

• Recurrent ear, lung, skin, or blood infections
• Autoimmune disorders affecting joints, skin, and the gastrointestinal tract
• Blood disorders, unexplained anemia, low white cell counts, and low platelet counts
• Chronic diarrhea
• Delayed growth and development

Treatment for primary immunodeficiency disease

Treatments for PI disease vary depending upon the illness. Options can include targeting specific infections or different forms of therapy to boost the immune system.

Children at Advocate Children’s Hospital have access to the full range of treatments for primary immunodeficiency, such as:

• Antibiotics and other medications to prevent and manage infections
• Immunoglobulin therapy to boost the immune system
• Gamma interferon therapy to improve neutrophil function
• Therapy to increase immune-strengthening white blood cell levels
• Monoclonal antibody therapy like Abatacept
• Evaluation for stem cell transplantation to cure some forms of immunodeficiency disease

Why choose Advocate Children's Hospital – Oak Lawn?

Advocate Children’s Hospital is a recognized diagnostic and treatment center for children with primary immunodeficiency. Our primary immunodeficiency disease program is one of two in the Chicago area that are designated a Jeffery Modell Foundation PI center for diagnosis and treatment.

We also provide leadership in monitoring and studying specific PI illnesses through the Immunodeficiency Foundation. Almost all the children we treat are in the USIDNET registry. USIDNET (United States Immunodeficiency Network) is an organization established to advance scientific research in PI disease. We are a member of the Clinical Immunology Society and actively collaborate with the National Institute of Health for some rare PI disorders. Advocate Children’s Hospital is also at the forefront of discovering new information about primary immunodeficiency diseases and treatments.

Our team under the multidisciplinary program for the DiGeorge Syndrome treats many children with DiGeorge Syndrome. The team includes cardiology physicians from our world-renowned pediatric cardiology program, developmental pediatricians, geneticists, speech pathologists, physical therapists, child psychologists, and nutritionists.

Children who are referred to Advocate Children’s Hospital with a confirmed diagnosis of PI disease, as well as those who are suspected of having PI, receive comprehensive evaluation and management. Physicians at Advocate Children’s Hospital realize that early detection is critical to each child’s long-term health, so they raise awareness among local pediatricians, nurses, residents, and other medical care providers through education and training. Our physicians are trained to recognize the warning signs of primary immunodeficiency and watch for them as children are admitted to the hospital.

Who will be on my child's care team?

Advocate Children’s Hospital features staff with extensive awareness and knowledge of PI disease. Working together and meeting monthly to determine the best course of treatment for each child with PI disease, our exceptional team includes:

• Pediatric immunologist: Treats various diseases and abnormalities of the immune system.
• Pediatric infectious disease specialist: Specializes in treating difficult infections. The specialist treats a wide range of infectious and immunologic diseases caused by bacteria, viruses, fungi, and parasites.
• Pediatric cardiologist: Treats minor to complex heart problems in babies and children.
• Pediatric gastroenterologist: Treats children with digestive, liver, and nutritional problems, such as food allergies, inflammatory bowel disease, or pancreatitis.
• Pediatric hematologist/oncologist: Specially trained to treat blood diseases.
• Pediatric pulmonologist: Diagnoses and treats lung conditions and diseases, such as asthma.
• Child psychologist: Studies the mental, social, and emotional development of a child with PI disease.
• Genetic counselor: Works with families to coordinate testing, interprets results, and reviews all additional testing, surveillance, surgical, or research options available.
• Registered dietician: A food and nutrition expert who works in the treatment and prevention of various diseases by developing a diet plan for your child.
• Research nurse coordinator: Oversees the clinical care of children with PI.
• Social Worker: Supports your child and your entire family by working with you to understand the disease. The social worker helps the family understand the disease and provides counseling about decisions that need to be made.

Preventing infections

Although primary immunodeficiency diseases are genetic and cannot be prevented, parents can take the following steps to prevent their children with weakened immune systems from getting infections:

• Hygiene: Ensure that your child washes their hands regularly and brushes their teeth twice a day
• Nutrition: Provide a healthy, balanced diet for your child
• Exposure: Avoid crowds and people with colds or other infections
• Medications: Make sure your child takes their medications regularly
• Vaccination: Find out which vaccinations your child should receive