For persons with a primary immunodeficiency, infection is a constant concern, and if left untreated, can lead to severe illness and hospitalization.
In a recent Education Forum, Dr. Niraj Patel described how to manage infections through identification, treatment, and prevention. Dr. Patel is Chief of the Division on Infectious Disease and Immunology at Levine Children’s Hospital in Charlotte, North Carolina and Clinical Associate Professor of Pediatrics. He is the director of the Charlotte Immunodeficiency Center and Co-director of the Immunodeficiency Multidisciplinary Clinic.
Many persons with PI experience recurrent infections such as sinusitis, ear infection, pneumonia, bronchitis, meningitis, thrush/yeast infections, bone or joint infections, urinary tract infections, ocular infections, and skin infections.
“Some of these are less common but to a clinician, some of these, like sepsis, that’s a clue to me and other health care providers that may point to a PI,” said Dr. Patel.
Those with PI also suffer from repeated gastrointestinal infections, which sometimes are not given enough attention, said Dr. Patel.
“A vast number of PI patients have gastrointestinal symptoms. Some have infections of the GI tract, while others have autoimmune disease like inflammatory bowel disease. I think this is one of the most common complaints - diarrhea, abdominal pain, reflux - so look out for recurrent GI symptoms,” he said.
While PI is a contributing factor to infection, PI can also be accompanied by other conditions like asthma, allergies, anatomic abnormalities, and secondary immunodeficiencies – which all weaken the immune system and make a person more susceptible to infection.
In a 2007 IDF survey, over half of persons with PI surveyed had other serious chronic diseases like chronic lung disease, asthma, arthritis, fibromyalgia, sinusitis, depression, and cancer.
“The more you get recurrent infections, the more you are at risk for getting serious chronic diseases. Ultimately, the problem is you can get recurrent infections that end up causing end-organ damage, so that typically can be irreversible,” he said.
“Be aware that PI is just not a single problem. It really can have other associated risks and issues.”
Dr. Patel discussed a range of strategies to treat and prevent infection, including the following:
Other ways to prevent infection include:
Vaccines are also important in maintaining health. The pneumonia shot not only prevents pneumonia but also ear infection and sinusitis. Dr. Patel also recommended the flu shot (in most cases the benefits outweigh the risks, he said) and any other vaccines that providers think are effective, though some forms of PI do not respond to vaccines.
Working to ensure communication between subspecialists is key to successful treatment and prevention. In addition to their primary care provider, a person with PI might see an immunologist, an allergist, an ear, nose, and throat specialist, a rheumatologist (for autoimmune complications, swollen joints, Lupus, or Hashimoto’s Disease), a hematologist/oncologist (for cancer or blood-related disorders), a gastroenterologist, or a pulmonologist.
“It’s very important to identify a home-base person because if you have questions or problems, you need someone to reach out to. It can be a primary care provider or your immunologist – whoever you feel the most comfortable with. That person is there to coordinate amongst the various subspecialties,” said Dr. Patel.
“It does no good if your PCP has one idea, and then your ENT has another and your immunologist has another, but none of them really communicate.”
Dr. Patel also stressed that persons with PI should follow-up with their doctors to ensure effectiveness of treatment, and bring all paperwork and test results to appointments.
“I can’t emphasize enough - I think we need to be our own advocates. Be proactive. Do not be dismissed. If you are dismissed, you go back or seek another solution,” he said.
To listen to the full presentation of “Managing Recurrent Infections,” click here.
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