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World PI Week 2023
Texas Children’s Raises Awareness of Primary Immunodeficiency
April 22-29 is World PI Week, and people around the world are joining forces to raise awareness of primary immunodeficiency (PI). The ultimate goal is to improve care and quality of life for patients and their families.
During World PI Week 2023, four key themes are being emphasized: diagnosis, treatment and care, research and innovation and medical education. PI occurs when immune systems do not function properly, leaving people more prone to repeat infections as well as auto-immunity, allergies, malignancy — and even death if untreated. There are over 480 identified forms of PI, and they impact the lives of an estimated 10 million people worldwide.
That’s why early screening and diagnosis are so crucial — they can mean the difference between life and death for patients.
Texas Children’s leads the way
Texas Children’s Hospital maintains one of the largest primary immunodeficiency services in the United States and is the main referral site for more than half of the state of Texas as well as large portions of Oklahoma, Louisiana and Northern Mexico. In 2013, Texas Children's established the Jeffrey Modell Diagnostic and Research Center in the Department of Allergy and Immunology with a gift from the Jeffrey Modell Foundation, a global non-profit organization dedicated to PI research, physician education, patient support, public awareness, advocacy and newborn screening.
The Jeffrey Modell Foundation was established after Fred and Vicki Modell lost their son, Jeffrey, at the age of 15 in 1986 from complications of his PI. Since Jeffrey’s death, the Modells have successfully testified before U.S. Congress on numerous occasions to put PI on the national research agenda and have sponsored physician education conferences in cities around the world.
The Jeffrey Modell Center at Texas Children’s is integrated with the William T. Shearer Center for Human Immunobiology, a scientific and clinical collaborative aimed at obtaining deep insights from the human immune system, and offers advanced research diagnostic evaluation to patients with a suspected PI.
Q&A with Dr. Lisa Forbes Satter
Dr. Lisa Forbes Satter is Director of the Jeffrey Modell Center at Texas Children’s Hospital, Medical Director of the William T. Shearer Center for Human Immunobiology and Associate Professor of Pediatrics in the Section of Immunology, Allergy and Retrovirology at Baylor College of Medicine.
Below, she answers questions about PI diseases and World PI Week:
What is primary immunodeficiency and what are some examples?
Primary immunodeficiency (PI) diseases occur when part of the immune system is missing or does not function properly. These diseases occur primarily due to inherited genetic abnormalities that can affect any person regardless of age, race, ethnicity or gender. Some examples of PI include antibody deficiency where the immune system makes an inadequate antibody response to an infection or vaccine. Another example is severe combined immunodeficiency (SCID) in which there is an absence of a key immune cell called a T cell. T cells are very important in defending against viruses and fungal infections. David Vetter, known to the world as the Bubble Boy, was from The Woodlands and was treated here at Texas Children’s.
What are some common symptoms of PI?
The most common symptoms of PI are recurrent infections. These are usually severe and can require multiple courses of antibiotics/antimicrobials or hospitalization. Infections can show up in the ears, sinuses, lungs, intestines and even the brain and spinal cord. Some PIs also have non-infectious features including swollen lymph nodes, a large spleen, autoimmune disease-like inflammatory bowel disease or inflammation of the blood vessels.
If a parent is concerned a child might have PI, what are the first steps that should be taken?
Parents concerned their child may have PI should talk to their pediatrician and discuss their concerns. Primary care providers are on the frontline and are key partners referring patients for further evaluation. Identifying a clear pattern of symptoms and getting a referral to an allergist/immunologist to perform a detailed immune workup can lead to a diagnosis and identification of therapies and treatment plans to help improve the patient’s health and well-being.
What can be done to improve the lives of patients with PI?
We are living in an exciting time in medicine with the growth of clinical genetic testing to aid in precise diagnosis in addition to the expansion of our therapeutic “toolbox” with the growing number of biologic therapies to personalize medicine. The Jeffrey Modell Network and the Immune Deficiency Foundation have wonderful patient resources. They foster community awareness and ways to improve overall health in partnership with providers to treat the patient’s disease and improve their quality of life.
What’s new on the horizon at Texas Children’s Jeffrey Modell Diagnostic and Research Center and William T. Shearer Center for Human Immunobiology?
We have an innovative and dedicated team of physicians and scientists working on diagnosis, treatment and discovery. Additionally, Texas Children’s Hospital has a new Immune Disorder Clinic Network., which includes specialists who have expertise in treating immune-mediated diseases and who partner across the hospital with our colleagues in a series of multidisciplinary clinics to treat our patients more efficiently and effectively. In the William T. Shearer Center for Human Immunobiology, our research and immunogenetics team is working tirelessly on new disease discovery, new ways to use therapies in immune diseases and new ways to modulate the immune system to better treat our patients.
For questions regarding the Immune Disorder Clinic Network, email firstname.lastname@example.org.
Learn about the Immune Deficiency Foundation and learn more about World PI Week to access materials and ideas to help you raise awareness and take part in the efforts of the primary immunodeficiency community globally!