Our Story
Adam is a student at Arizona State University studying mathematics and statistics. Drawing from statistics terminology, he was born with an outlier health condition – a rare problem with his immune system called common variable immunodeficiency. About 1 in 25,000 have CVID.
It is worth noting that CVID is considered a primary immune deficiency, so is not acquired by an infection. In fact, the opposite is true. CVID causes infections. People with this immune disease (specifically B-cells that make antibodies) do not mount a strong enough defense against infections, cancer and autoimmune diseases.
Adam didn’t show any symptoms until he was 17 years old. That is when his liver started to fail from primary sclerosing cholangitis. After a liver transplant, the pathology lab found diffuse large B-cell lymphoma in his original liver. These two conditions rarely go together. The two co-existing conditions are so rare, it caught the attention of the Phoenix Children’s Hospital’s Allergy and Immunology team.
In a Zoom telemedicine appointment in early 2022, Adam was told he had CVID and would need another transplant. Ironically, he was sick in bed with a fever at the time. After a five-point confirmation of CVID that included a genetic test, Adam was listed for a bone-marrow transplant (also called a stem cell transplant).
Adam’s older brother David turned out to also be a genetic carrier of CVID, so he was unable to donate his cells. Instead, Adam’s doctors turned to the Be The Match database. There were half a dozen excellent matches for him. After waiting all year for his donor cells, Adam received a stem cell transplant Dec. 21, 2022.
The first sign: Liver disease
In the summer of 2018, Adam was lucky enough to be invited to accompany two different friends on their family vacations. He texted his mother while on the first of two trips saying he was extremely itchy. We had 24 hours to see a doctor, give him an antibiotic and get him on his way for the second trip. He had amazing experiences that summer, but the itching continued and at times was unbearable.
He continued to suffer from uncontrollable itching into the beginning of his junior year of high school. Thinking it must be an allergy, he visited our allergist. After results from a blood test came back, a prescribing nurse told Adam that his liver enzymes were extremely high. This was causing the itching, but she didn’t know why. We now know this was a symptom of his liver dysfunction.
In October, Adam was hospitalized with jaundice. A liver biopsy indicated that his liver had been injured. Two months later, the jaundice had not gone away. Adam was back in the hospital. This time, he was diagnosed with primary sclerosing cholangitis (PSC). The bile ducts of the liver were both strictured and enlarged. Doctors were surprised at the fast pace of his decline in such a short time, as this is uncharacteristic of the condition.
By January 2019, Adam was on the liver transplant list at Phoenix Children’s Hospital. Adam’s bile ducts had to be kept open with stents, that served like straws. The stents allowed the bile to flow out of his liver. Doctors soon realized he was stent dependent. When the stents were in, Adam was able to resume his normal activities. When the stents were removed or displaced, Adam got cholangitis, a very serious infection. While liver enzymes that factor into ranking transplant recipient scores reflect the health of the liver, they do not adequately account for the bile duct issues Adam was experiencing.
Due to Adam’s age, he was considered an adult. The summer before his senior year, Adam’s hepatologist suggested he also be listed at Mayo Clinic to increase his chances for transplant. In addition to working with cadaver-organ transplants – like PCH – Mayo also has a live donor program. Interestingly, Adam’s PSC perplexed the medical experts at both Phoenix Children’s Hospital and the Mayo Clinic. Fortunately, in late 2019, PCH made the case for him to receive exception points. This was easier because Adam was listed as a pediatric patient.
He received a high score of 20, while in the adult world he was only an 8. Starting in December, with the higher score he started to receive organ offers. We had two dry runs, meaning Adam was admitted and prepped only to find out that the livers were not of good quality, so they would not be suitable for an 18-year-old with decades before him.
Both teams suspected cancer to be causing the rapid change, but nothing could be detected. By early 2020, his condition started to worsen. In fact, he was hospitalized with infections monthly for the first three months of the year. He was successfully transplanted April 3, 2020, at age 18 at Phoenix Children’s Hospital and began his three months of isolation as part of the healing regimen.
A second sign: Cancer
After transplant, Adam’s removed liver was sent to a pathology lab. The liver tested positive for diffuse large B-cell lymphoma. His hepatologist says she only could find reference to five documented cases of PSC and lymphoma. We are hopeful due to the amount of testing for cancer done at Mayo Clinic, that the lymphoma was successfully and completely removed with his old liver.
To be sure it doesn’t come back or hasn’t spread, Adam underwent four rounds of chemotherapy.
About Adam
Overall, Adam is social with close friends, rather shy with those he doesn’t know, and very determined at his core. He is a good listener, a good friend, quick with a smile and the support you need. Somewhere along the way, Adam became a beacon of positivity. He shares his silver lining way of seeing a situation that encourages others. But to understand Adam, you need to get a picture of his determination. To do this, we have to go back to his adolescence.
Adam has always enjoyed good food. At around age 13, Adam was hefty, having gained enough weight that started to change his self-perception. After a difficult year of teasing and self-doubt, Adam decided to take action and lose weight.
He changed the way he ate, logging each meal in MyFitnessPal, weighing himself and taking photos each morning. He started running. A year later, Adam had dropped 60 pounds and continued to lose weight. Thanks to some amazing mentors – several years his senior – Adam learned the ropes around the neighborhood gym and began lifting weights. He continues working out every day when his health allows it.
Adam is supported by his mother, father, older brother, grandparents and friends.