Persons with primary immunodeficiencies do not appear to be at any higher risk of mortality from COVID than those without PI. That is one of the many messages and critical pieces of information imparted at the most recent IDF Forum, “COVID-19: An Update.”
Dr. Kathleen Sullivan, Chief of the Division of Allergy and Immunology at Children’s Hospital of Philadelphia, and Dr. Maria Jimena Gutierrez, Assistant Professor of Pediatrics and an allergist/immunologist/pediatric rheumatologist at Johns Hopkins Children’s Center, presented the forum on Oct. 5. The doctors allowed hundreds of questions from the PI community about COVID to shape the content of their presentation.
Since the pandemic began, the question of whether persons with PI are more vulnerable to the virus is a central concern. Dr. Sullivan said that the risk factors for severe COVID, such as advanced age, are the same in persons with PI as in the general population.
“I’m going to paint a picture that is largely reassuring, which is not to say that I’m minimizing the anxiety and risks. I don’t want anyone to get the virus – not anyone at all, but, in fact, it’s largely good news about people with PI,” said Dr. Sullivan.
“Overall, I’m going to say that the risks are not dramatically elevated in people with the classic PI… but I don’t want to minimize that there’s been a lot of pain in the PI community.”
For example, if you have CVID and are older than 65 and have chronic lung disease, you have a higher mortality rate if you get COVID (10 percent) – but it’s the same mortality rate as a person who is over 65 and has a chronic lung condition and doesn’t have a PI who gets COVID.
There are some exceptions. If you have a PI that compromises your ability to make interferon, then your risks of severe disease are elevated beyond the average person who doesn’t have a PI. Interferon is a hormone that stimulates cells to block viruses.
Those with a PI that affects their interferon do not have recurrent infections and were only identified with a PI upon a COVID diagnosis.
“In the folks who recover, there’s a lot more interferon-stimulated genes than in the ones who go on to get severe disease,” explained Dr. Sullivan.
“The people with PI who have antibodies to interferon have more severe disease. Antibodies can be protective, but they can also get in the way. Also, people with interferon pathway mutations have more severe disease.”
The best way to protect yourself against severe disease is to receive a vaccine. Should you develop a breakthrough case after vaccination, seek treatment with antiviral medications and monoclonal antibodies within 10 days of diagnosis to lessen the effects of the infection.
The current trend with COVID, explained Dr. Gutierrez, is that the new delta variant is now accounting for 99 percent of cases, and delta is nearly twice as contagious as the original virus. The U.S. had its lowest point with infections in June 2021 but infections are now spiking because of delta.
“It’s gotten smarter about evading the vaccine. The vaccine is slightly less effective against this one,” said Dr. Gutierrez. “But the main message is – the vaccine remains very, very high in effectiveness against severe infections.”
The unvaccinated population is now driving the hospitalizations and deaths from COVID, with the latest data showing that only 3 percent of those hospitalizations are coming from breakthrough cases. Among the breakthrough case hospitalizations, 70 percent are people aged 65 and older. In addition, 44 percent are persons who are immunocompromised due to primary or secondary causes.
Studies have shown that immunity to COVID is waning after vaccination and that a booster is necessary for certain populations. Though studies are still underway to determine how well those with a PI respond to a vaccine, the doctors said that some will need a booster, starting a month after their last dose. The doctors recommend consulting with your immunologist about the specific recommendations and best timing for a booster in your case.
The vaccine booster is the same strength and composition as previous doses of COVID vaccine and is widely available at any pharmacy. The doctors recommend matching the brand of vaccine you had for your first two doses but that is not entirely necessary.
“Try and match it, but if you have reason to switch it up, don’t sweat it,” said Dr. Sullivan.
The doctors cautioned against getting more than one booster.
“I would remind everyone that patients with CVID - 80 percent make antibodies after the vaccine. If you don’t respond to other vaccines, there’s still a good chance that you will respond to these vaccines. These are very powerful vaccines,” said Dr. Sullivan.
“It’s a relatively small slice of pie where we don’t expect people to make antibody response to vaccine. From the data we have so far, that would be largely men with XLA and a small subset of people with CVID. If you have no B cells at all in our body, I would not keep getting more and more doses.”
Dr. Gutierrez added that even those with XLA and CVID haves signs of T cell response which can protect them, and that the PI community should stay tuned for future COVID-19 presentation updates as larger studies of vaccine effectiveness in PI patients are underway.
Measuring antibodies after vaccines to determine if the vaccine is working should also be avoided.
“Antibody testing in the U.S. and around the world is a complete and utter mess right now. It is not standardized. Every company stood up their own testing. They don’t use the same metrics. They don’t use the same measure and if you look at the labels, it is not the same because people are measuring them in different ways,” said Dr. Sullivan. “So, I really discourage people from getting antibody testing right now”
The doctors do recommend persons with PI:
- Get vaccinated
- Take precautions such as masking, social distancing, handwashing and disinfecting high touch areas
- Vaccinate close contacts
- Seek early treatment with monoclonal antibodies for early infections or prophylaxis
- Get a third vaccine dose
- Address any mental health concerns
The doctors also noted that while children do better with COVID than adults, the delta variant is causing more children to battle severe disease. The following are some recommendations for caring for children during the pandemic:
- Get the vaccine now for children 12 and older
- Vaccinate children ages 5 to 11 as soon as a vaccine is approved
- Put children in pods or outside for school lunchtimes
- Provide children with KN95 masks for extra protection
Finally, for those still isolating at home with no face-to-face human interaction, the doctors said it’s time to assess your local virus transmission, ease off on the strict measures, and determine what level of risk you are willing to accept. Both doctors urge patients to move beyond their fear and interact with the outside world, within limits.
“People with PI have been told their entire life by their immunologists and their doctors, ‘Oh my gosh, you’re fragile, you have to worry about every exposure.’ You’ve been told that your whole life and now there’s this scary virus that shows up and it seems very threatening because you can’t measure it,” said Dr. Sullivan.
“You don’t know exactly what the risk is but certainly the data do not suggest that people with PI are unduly susceptible to ICU or mortality.
“Again, let me say, that doesn’t mean I want people to get COVID, of course I don’t. But if you just step back and say, ‘Are my risks higher than my neighbor who is the same age who doesn’t have PI?’ Unless you have an interferon defect, your risks are roughly the same as your neighbor who is the same age. With that framework, what risks do you accept?”
Venture outdoors for a walk first, a low-risk endeavor, and then go to those long overdue doctor and dentist appointments in-person.
“Pick something like that and take some baby steps and see how you feel and then gradually try to liberalize to the point where you meet your own risk tolerance,” said Dr. Sullivan.
To listen to the entire presentation, click here.