They said there was only a fifty percent chance he would survive
After a stomach pain and a black out in his home, then 13-year old Will was rushed to a local hospital in New Jersey, where the doctors discovered a volvulus and most of his small intestine was removed.
“95% of my small intestine was deceased because the blood flow was restricted for many hours.”
Depending on parenteral support to live
Will was diagnosed with short bowel syndrome. He began receiving total parenteral support (PS), a lifesaving therapy for patients with intestinal failure that provides all the elements of nutrition and hydration needed to live via intravenous infusion. Will was doing PS infusions seven nights a week and the pump would run for 12 hours. He would have to carry his PS hanging from a pole, limiting his freedom to leave the house, and limiting his ability to go to school.
“I didn’t have the capability to finish the school year. I was still weak. I lost about 40 pounds in the hospital, so I was still trying to gain that weight back. It was difficult to function all day.”
Being a teenager with short bowel syndrome was tough. He experienced problems with his liver and infections. He was unable to participate in sports with his friends because he was prone for frequent dehydration.
Will felt that people saw him as normal on the outside but on the inside, he could see all the scars and the tubes.
Optimizing parenteral support for better quality of life
With his medical team, Will’s PS has been optimized from 12 hours for 7 days a week, to only 8 hours for 2 days a week. Going to 8 hours gave Will a much better quality of life, as he could sleep and not having to carry around the pole the following morning.
Will’s goal is to be free of PS, or otherwise to reduce as much as possible. He is determined to prove others wrong about the impediments of his disease.
“I like when people tell me I can’t do something because then I go out and try to prove them wrong. Some doctors told me I was never going to eat or drink again. I ended up doing that. They told me I wasn’t going to come off of PS completely: I’m almost there.”
Short bowel syndrome (SBS)
SBS is a life-threatening and complex and severe chronic condition associated with reduced or complete loss of intestinal function. In adults, the main underlying causes of SBS are major intestinal surgery following Crohn’s disease, ischemia, radiation damage or surgery.
It is estimated that 20,000-40,000 people are affected by SBS in the U.S. and Europe. The most severely affected are dependent on daily parenteral support. This requires them to be connected to infusion lines and pumps, which pose significant restrictions on their ability to engage in daily activities.
Sources: Jeppesen P. Expert Opin Orphan Drugs; 1:515-25; Transparency Market Research; Short Bowel Syndrome Market, 2017