Hypogammaglobulinemia: Dylan’s Story

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Dylan, an active second grader in Monmouth, NJ, who’s had his first infection-free year in his entire life thanks to COVID-19.

“It’s been a bit of silver-lining to a very dark cloud,” said Dylan’s mom, Tara. “All the social distancing, mask wearing and hand washing we’re doing to prevent COVID-19, has actually helped my son remain healthy.”

Dylan and his family Dylan (far right) and his family Dylan has been a patient at Children’s Hospital of Philadelphia (CHOP) and the CHOP Care Network since he was a few days old. He’s seen pediatricians at CHOP Primary Care in Paoli and High Point, as well as numerous specialists from Pulmonology, ENT, Endocrinology, Allergy and Immunology at CHOP’s Philadelphia campus. Today, despite moving more than an hour away, Tara says she and her husband, Joe, continue to bring Dylan to CHOP because of the “quality of care he’s received.”

CHOP has quite literally changed Dylan’s life,” Tara says. “They’ve given us answers when we didn’t know any existed and worked with us to develop the best treatments for his needs.

Multiple infections, pneumonia and more

Dylan’s health challenges began when he was a baby. At about a year old, he was diagnosed with asthma and began nebulizer treatments at home. Then, Dylan began to develop frequent ear infections; so many in fact, that his mom says she lost count long ago.

When he began socializing in his neighborhood and at school, Tara noticed Dylan was unusually susceptible to any bacterial or viral infection around. Any minor ailment, such as sniffles or a cough – things most kids would recover from in a few days – seemed to knock Dylan down for the count.

He suffered multiple bouts of cold, flu, strep throat, sinus infections and had pneumonia a whopping six times – three of which required hospitalization – before he was 6 years old.

Tara, who has another son three years older than Dylan, knew something out of the ordinary was going on and sought help from CHOP. Along with his pediatricians, Dylan was also evaluated by specialists.

Investigating the cause of illnesses

With so many illnesses in such a short time, Dylan’s pulmonologist, Jeffrey M. Ewig, MD, began wondering whether there was a larger underlying cause for Dylan’s many ailments. He ordered a bevy of specialized tests, searching for clues that might lead the multidisciplinary team to an answer.

It worked: Dylan’s blood work revealed his immunoglobulin (IG) and pneumococcal levels were low – both of which made Dylan more likely to have extreme reactions to common illnesses. Dr. Ewig referred the family to the Division of Allergy and Immunology for further evaluation.

Rare immune disorder diagnosed

Dylan sleeping in hospital bed In February 2020, Dylan and his family met with Juhee Lee, MD, an attending physician in the Immunology Program. She evaluated all of Dylan’s test results and diagnosed him with hypogammaglobulinemia, a rare immune disorder that prevents the body from making enough antibodies (called immunoglobulins) to identify and fight off foreign invaders like viruses and bacteria.

“It was a relief to know there was a name for what he has,” Tara says. “And to know we were at a place that would help us get answers so Dylan could have a healthy future.”

Dr. Lee explained that without enough antibodies, Dylan would be more likely to get infections – as he had for years – and ordered a pneumococcal booster shot in an effort to bolster his immune system.

So far, Dylan has shown a positive response to the booster. It’s kept the “protective” levels in his immune system functioning – although at less-than-ideal levels.

Dr. Lee continues to closely monitor Dylan for any changes in his health, and a follow-up appointment is set for the Spring. If Dylan’s immune system continues to decline, it may be a sign he has common variable immune deficiency (CVID), a chronic condition that requires life-long treatment and can lead to more significant health concerns.

For now, Dylan’s family is committed to remaining vigilant for any possible signs of infection and getting him immediate help if needed.

Closer to home, with some health benefits

In March 2020, COVID-19 hit the United States – shuttering schools, businesses and transit for all but the most essential services. Dylan and his family retreated into their home to wait out the storm. For the remainder of the school year, Dylan attended virtually with his peers. Though he had to remain close to home, he enjoyed building marble rollercoasters, playing catch and video games, listening to music and hanging out with his family and their two dogs.

When school began in-person lessons in the fall 2020, Tara called Dr. Lee for advice. “Dr. Lee has been really wonderful,” Tara says. “Anytime his school plan changed – from virtual to every other day, to 5 half days a week – she was always there for us, to reassure us and give us guidance in finding the right answer for Dylan.”

Dylan playing baseball Dylan now attends school every morning and completes his core studies, then comes home for lunch and continues virtual learning in the afternoons – the same schedule as his peers. He also plays for his town’s 7-and-younger travel baseball team and recently had the opportunity to pitch for his team.

“Dylan knows his body doesn’t respond the same as other kids when he’s exposed to germs, so he’s been really good about following COVID-19 precautions,” Tara says. “All the restrictions have been a blessing to us in a way. It’s given Dylan a chance to stay healthy, but not feel different than his peers. Essentially, the entire world has played a role in his first infection-free year, and we can’t help but feel grateful for the contribution of others for this success.”

At Dylan’s next scheduled appointment in Spring 2021, his family knows Dr. Lee might discuss the possibility of starting Dylan on long-term, infusion maintenance medications to keep future infections at bay.

“I’m confident that when the world starts opening up, CHOP will help us navigate each new challenge that presents itself and recommend the best long-term treatment for Dylan,” Tara says.

Next Steps

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