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A few years ago, Cynthia Perry removed a piece of medical tape from her shoulder and found the skin underneath bright red and hot to the touch. Worried she had a severe skin infection, Perry headed for the emergency department (ED).
The ED nurse seemed unconcerned because Perry had no fever. She mistakenly referred to Perry’s diagnosis of common variable immune deficiency (CVID) as an "autoimmune condition," even though Perry explained that it was a primary immunodeficiency (PI). The nurse refused to call a doctor and instead summoned a physician's assistant (PA) to see Perry. The PA wanted to give Perry oral antibiotics, but she convinced him to administer intravenous (IV) antibiotics. After providing treatment, the PA sent her home. Exhausted, Perry called the staff at her infectious disease doctor’s clinic, who then contacted the local university ED and alerted them to expect Perry.
"The head of the ED at the university came down to see me, and I said, ‘What do you think is going on?’ She said, ‘I don't know, but I think it's an infection,’" said Perry. "They gave me two doses of a reserve antibiotic, which is a last resort for drug-resistant infections, put me on a 24-hour sepsis watch, and gave me a prescription for 10 more days of antibiotics. They were furious that the other hospital had discharged me."
Perry’s experience isn’t unique. Many people with PI find that when they go to the ED for a serious health issue, health providers aren’t familiar with PI. They don’t understand that patients may present with fewer or no symptoms or unusual blood work results because their immune systems are compromised and they don’t launch a normal immune response to bacterial, viral, or fungal infections.
"The issue with patients who have PI is that their response to an infection isn't always going to look like a healthy person having an infection. In fact, there may be a really serious infection going on without the outward signs," said Dr. Morna Dorsey, director of the University of California, San Francisco (UCSF) Pediatric Allergy and Immunology Center, at the 2023 Immune Deficiency Foundation (IDF) virtual conference. "In many individuals with PI, those pathways [to an inflammatory response] are blunted. Fever may not be there, or white [blood cell] count may not be elevated."
Dr. Taylor Fitch, a Children’s Healthcare of Atlanta (CHOA) pediatric hematologist/oncologist specializing in treating children with PI, said that ED clinicians may not know how to proceed with treatment for infections in people with PI.
"Familiarity with these genetic disorders is a challenge. Patients will come in, and they'll say they have this rare condition, which means something to them and to me, but isn't as clear to the providers that see them, and they may not understand the nuances and the complexity of that diagnosis," said Fitch.
A low-grade fever in a child without PI is considered a virus until proven otherwise, but for immunodeficient patients, that could be a sign of bacteremia, or bacteria in the bloodstream, said Fitch. People with PI often look well and have normal vital signs that mask the severity of the illness. But caregivers of children with PI and PI patients themselves know when they are very sick.
"These patients and these families know what badness looks like to them because this is not their first rodeo. They've done this many, many times. So, coming into the ED saying, ‘Oh, I feel bacteremic,’ and their heart rate is normal, that can be a little bit perplexing to providers," said Fitch.
"For providers who are used to people who are very sick looking very sick, our patients can smolder, smolder, smolder, and then fall apart. And I think that can be a challenge that [the patients] face, where they feel like they're being dismissed or sent home without a complete or comprehensive evaluation."
Common symptoms that may require a visit to the ER for someone with PI include:
If you have a PI or a child with PI, be prepared before you go to the ED. One of the most effective ways to communicate to ED providers that you have a PI is through a letter prepared by your immunologist, infectious disease doctor, or other specialist. The letter should state the name of your specific type of PI, the kinds of infections that you may have and their signs and symptoms (no fever but elevated white blood count, for example), recommended tests, and the contact information for the doctor.
Perry sometimes visits the ED up to six times a year, and has noticed a marked difference in her treatment since she’s had a letter from her doctor explaining her diagnosis.
"The letter is super effective," said Perry. "If you're not feeling well, it can be really hard to advocate for yourself. The letter is very helpful in that. I feel like my doctor is always with me when I can whip out that letter and say, ‘Look at this.’"
You may also keep the IDF I Am Immunocompromised emergency card in your wallet as an alternative. The card can be downloaded or ordered and contains the patient’s name, birthdate, language, emergency contact, and physician information.
Another critical piece of information for ED providers is a medical summary. The medical summary should be a short, one-page (if possible) list of dates of surgeries, complications, infections leading to hospitalizations, treatments and outcomes, medications, allergies, and specialists’ names. The summary saves time and allows the provider to gain valuable insight into a patient with a quick scan.
Accompany the medical summary with a brief verbal explanation of your PI and how you may not present with typical findings, such as a high temperature, high heart rate, or abnormal bloodwork. Provide the basics:
Another recommendation is to either call your immunologist before you go to the ED so that they can call the ED ahead of time and explain your condition, or have the ED call your immunologist when you get there. If the ED refuses to call your immunologist, call the immunologist yourself and ask that they reach out to the ED.
"The nurses and doctors treating you or your child, they're your best friends at that moment, and so try to explain your concerns. Explain the situation. But if you're not really getting that two-way feedback, then you can push back a little further without being too strong in your demands. If it’s really not getting through, I think going outside of that circle of communication and then having someone else discuss it with them would be totally fine," said Dorsey.
Other items to bring with you to the ED include a go-bag with toiletries, snacks, drinks, toys, books, phone, portable charger, and headphones. You may also want to include protective face masks, hand sanitizer, and daily medication in case you are waiting for a while.
Perry said that she’s learned that the ED is a good resource for stabilizing health, but not necessarily for a diagnosis. The ED is helpful for quick lab work, IV fluids, and IV antibiotics. They are part of your medical team but are limited in what they can do.
"Here's the thing to keep in mind about ERs just in general, and why I don't get frustrated with them and they don't get mad at me when I show up. They’ll say, ‘It was great that you came in, and it absolutely makes sense to go infection hunting, but then we're going to turn you back to your outside doctor to figure it all out. We're happy to run the acute labs for you so that you can get answers. You need them done right now because you're in a flare or you feel like you're septic. But we're not the ones to make those decisions about diagnosis. You're too complicated," said Perry, who also has mast cell activation syndrome (MCAS) and autoimmune conditions.
Even though it can be frustrating if an ED provider is not acknowledging the severity of your illness, remain calm and respectful when communicating with them. Suggestions work better than demands.
"In any situation, someone has something to offer. It may not be perfect, it may not be everything you want, but there is something that the person can offer for your specific situation," said Dorsey.
If you can’t find the treatment you need at one ED, visit another one, preferably one connected to a major hospital or university.
"If my patients are really getting into trouble with the ED, I tell my patients to contact me directly on my cell. The CHOA system doctors can access our notes, but not those outside of the system," said Fitch.
"If their local facility is not willing to call me or reach out or is unwilling to do what they've been told to do, they're instructed to come to our ED, and I don't have anyone in our ED system that does not take our patients seriously. I think they've seen what happens when they don't listen to our recommendations."
You can order or download this card, fill it out, and keep it in your wallet so that it's easy for emergency care providers to find important information about your condition.
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