Born with biliary atresia, a rare gastrointestinal disorder, Aaron Jackson has undergone 17 surgical procedures and 5 major surgeries, including a liver transplant and a liver and kidney transplant. Today, with a smile nearly as broad as his experience, he is bringing hope and advice to the transplant community

by Joe Rumney

Early diagnosis and surgery

When Aaron was born in San Dimas Community Hospital, California on 20 December 1983, it was clear that something wasn’t quite right; his parents and the doctors caring for him noticed he had jaundiced eyes and pale skin.

With the assigned delivery doctor on holiday, a stand-in recognised Aaron’s symptoms straight away, having seen a colleague’s notes about a condition called biliary atresia. Soon after, Aaron was examined and then tested. The doctor’s suspicions were confirmed—it was biliary atresia.

Biliary atresia is a rare gastrointestinal disorder in which the bile ducts outside the liver are missing or damaged, so that bile and waste products accumulate. Most symptoms appear by the time a child is two years old. The skin and the whites of the eye become yellowish, and there is sometimes scarring of the liver.¹

There was no time for his parents to dwell on the diagnosis, Aaron was admitted to Children’s Hospital Los Angeles, where he would have his first surgery, the Kasai procedure, at just three months. The Kasai procedure, first developed in 1951, involves removing the damaged bile ducts and gallbladder, then using a segment of the child’s small intestine to take its place. Aaron says that the operation is much more commonly performed today and carries less risk than when he had his.

Defying expectations

Doctors said that Aaron would have learning difficulties, stunted growth and a weakened body. None of those things happened. “In fact, at one point they wanted me to be the poster child for liver disease. I had been so sick that they wanted to take my picture, put me on a poster, and use my appearance to raise money.”. But his recovery was so good that he ended up looking too healthy! “I had gotten too fat and I smiled way too much.”

Despite what the doctors had forecast, Aaron regularly played sports and relished physical activity, his favourites being soccer, basketball, baseball and golf. He was only limited when playing contact sports as he had to be more cautious because of his condition.

“I excelled in school. I excelled at sports, and my frame was perfectly fine. Everything was almost the exact opposite of what they had described.”

However, through his school years, Aaron battled with low self-confidence, because of what was planted in his mind early on.

As a result of the Kasai procedure, Aaron lived with an enlarged spleen during his childhood, giving him a pronounced abdomen, which made him an easy target for bullying. Other students often made remarks towards him, saying he looked “pregnant”. When playing contact sports, such as his much-loved baseball, Aaron would wear a large plastic contraption that would fit under his top to protect his torso from impact which could cause fatal internal bleeding.

Wearing such large protection left him feeling distanced from his peers. “It was a real challenge, but I think that made me stronger.” Despite these challenges, Aaron found the strength to excel in his education, achieving A and B grades.

Luckily, Aaron had a good support network with his friends and family, allowing him to cope with his struggles at school and to become the positive person he is today. With time he realised he was no different from other people his age. He could see his worth but just needed to work that little bit harder for success.

2017: the year that everything changed

Aaron graduated from college with an economics degree and was beginning to build a comfortable life for himself. He had an apartment and a job at a large bank while still enjoying playing sports at the weekend.

“Usually for people who have biliary atresia, they’re in and out of the hospital, and they experience certain medical setbacks that mean they have to take it easy in life. I didn’t have any of that, it just felt like normal living for me.”

Aaron says that everything in his life was going as well as it could, “but something was telling me not to get too comfortable: ‘You need to keep in the back of your mind that at some point, you’re going to need a transplant.’” In 2017, everything changed.

Aaron went about his day with his usual routines but became more and more exhausted as the day progressed. Not realising the connection to his biliary atresia, he assumed he was overworked and just needed more exercise.

Then he started to notice more symptoms. His hair was falling out, there were noticeable changes on his skin, and like when he was born, his eyes were jaundiced. “When those things started to happen, I thought, ‘Okay, this is a little bit more serious.’”

At Ronald Reagan UCLA Medical Center (UCLA), his MELD (model for end-stage liver disease) score was reading the same levels, generally around 10 to 14, as they had when Aaron was feeling well. He wouldn’t be accepted for a liver transplant until the score increased.

As time went on, he felt the effects of liver deterioration, but his doctors didn’t deem him unwell enough to start the transplant process. “I was living in limbo. I was just feeling this level of depression and trying to figure out what exactly was going on with my body.”

This waiting period led Aaron down a dark path in his life—not prepared for the physical and mental toll on his health, and he lived in this ‘limbo’ state for several months. In hindsight, Aaron realises how he projected himself onto the world had changed.

“I went into a barber shop just trying to get a haircut and the staff made fun of me saying that I look like a werewolf with my yellow eyes. At the time, you have to save face and just laugh it off, but I was hurt by that. They had no idea.”

Experiencing poor health and working in a toxic environment, Aaron decided to take leave of absence from his job. “I had people working with me who didn’t really understand what I was going through and they held my health problems against me.”

“I’ve learned a great deal about resilience and determination, and what it takes to really dedicate yourself to yourself.”

Losing his brightness

Just a year earlier, Aaron was bright, happy and upbeat, but by the end of 2017, he was unrecognisable. His hair was falling out, his skin lost its brightness, and his eyes became so jaundiced that he avoided eye contact with everyone he saw. He would ask himself: “Why aren’t I sick enough for a transplant? ‘What can I do to get sicker?’ I want it to be over.”

Aaron explains that when the liver fails, the ammonia levels in the brain increase. Because of this he suffered from hallucinations and mood swings. “I would snap at family members and I’d become very angry. I would act in a way that was completely out of my character.” Eventually, his symptoms became so bad that he isolated himself from his friends and family. His weight would fluctuate from being overloaded with fluid. He also had constant itching from hives (an itchy rash) and couldn’t sleep at night or even be comfortable in the daytime.

One day, after his MELD score had finally increased, Aaron woke with a strong mindset. Not content on giving up hope, he decided he would try to go for a walk around the block to prove he had the power to overcome his ill-health. “I felt like I had a little bit of flight left in me.” Despite his attitude, Aaron couldn’t walk far at all. “I came back home, sat down on the couch and just started crying. I felt like I didn’t know what was next for me.”

Later that day, Aaron’s parents found him in bed, “kind of out of it”, and very combative. Aaron was so confused that he refused to get into the car to go to hospital, and when they arrived, it took four security guards and two nurses to get him to a ward. Everything after that is a blur; Aaron only remembers waking up two weeks later.

Aaron’s transplant journey

When Aaron woke up in December 2019, he realised he had lost the majority of his vision. “I could only see a few inches in front of me, and everything else was completely blurry.” It was now more important than ever to have a transplant.

As a child, Aaron would consider having a transplant a future problem, but suddenly it had become his reality—on 9 December, he had his first liver transplant. Post-surgery, keen to get back on his feet, Aaron told the nurses he’d like to build up an exercise routine. Unfortunately, his muscles had wasted away completely, and he didn’t have the strength to walk, or even stand.

Just one week later, Aaron’s new liver began to fail, and his kidneys started to fail too. Placed back on the transplant list, his health deteriorated rapidly. The ward nurses caring for him were always positive, giving him hope that he’d have a new liver for his birthday. “I think they knew in the back of their mind that there was no possibility, they were just giving me hope, which is what I needed while I was in there.”

When the New Year came, Aaron’s body had become septic and he needed emergency surgery. At that point, he had become “the sickest person in California on the transplant waiting list”. Aaron needed that transplant, or there was a chance he wouldn’t make it.

In mid-January, his health deteriorated—fast. Not only had his transplanted liver failed, but his kidneys were rapidly deteriorating, too. Nurses prepared his family for the worst. Without another transplant, there was nothing more that could be done to save Aaron.

Then, only two days later, Aaron finally received not only a liver transplant, but a new kidney too, and this time the transplants were a success. The jaundice in his eyes disappeared almost immediately and his eyesight returned. “After the successful transplant, my mindset was all about happiness, life, family, and just enjoying the fruits of life. It was an interesting transition.”

After 17 surgical procedures and five major surgeries, Aaron’s life could start again—after such a long time on the ward, even standing up required physiotherapy. Performing tasks like brushing his teeth or getting changed were now challenges to be overcome. Despite these challenges, his mental state was better than ever. “My clarity of mind was just through the roof.”

“Each day I was getting a little bit stronger, a little bit better. I never turned down physical therapy or occupational therapy, even on those mornings when I felt sick. I was just, like, you’ve got to do this.”

Positivity and love

Aaron has realised the importance of support from his time competing in the Transplant Games of America. “In many social circles, you talk to people and the first question they have for you is ‘Where do you work? Or what do you do for a living?’ They immediately just want to know where they can place you in terms of social status. But at the Transplant Games, it was more what’s your journey? What are you going through now? It was a lot of positivity and love.” He achieved a silver and a bronze medal and is planning to attend the games in 2024.

Aaron feels it’s important to talk openly about the areas of the transplantation journey that aren’t explained enough, such as the medical and financial needs that arise. Aaron believes there needs to be more focus on the symptoms that he wasn’t aware of when he first needed a transplant, especially itchy skin, yellowing eyes and hair loss. “When the patients hear about the things that I went through, sometimes it gives them hope,” he says.

As an ambassador for Donate Life, Aaron visits schools and hospitals to speak about organ donation in the United States, trying to overcome a lack of awareness: “The challenge right now is getting people to become aware that they can be an organ donor.”

“I talk about how important it is to become a donor, and what a great help it would be if we could just be there for each other as humankind. When you leave, you’re not going to need those organs anymore.”

Aaron also serves as a committee member for the UCLA Health Transplant Patient and Family Committee. The committee was started in 2020 to gain insights from patients and their families and caregivers, so that UCLA Health can continually improve the transplant patient experience. 

Having dealt with a lifetime of challenges, Aaron Jackson has become a resilient and determined advocate for the biliary atresia community, having discovered that mental and physical health are tightly linked. With the valued support of friends and family, Aaron faces the world with a positive attitude and a smile, continuing to spread awareness of organ donation for transplant patients.

References

[1] https://rarediseases.org/rare-diseases/extrahepatic-biliary-atresia/

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