Tyler's Intestinal Malrotation Story
My son Tyler was born with Down syndrome and autism. Because of this, his verbal communication has always been limited.
When Tyler was born he didn’t have regular bowel movements and frequently experienced abdominal distension. His pediatrician felt these symptoms were a result of the low muscle tone that is associated with Down syndrome. We tried various tactics to increase his motility but didn’t have any success. This was in the days before the internet, so I went to the library to do my own research. I found a book with one sentence that said if a baby with Down syndrome suffered from constipation they should be screened for Hirschsprung's disease. I didn’t even call the pediatrician, I went straight to the geneticist at Texas Children’s Hospital. At eight-months-old Tyler was diagnosed with Hirschsprung’s disease, a congenital condition in which the intestinal nerves fail to develop properly. At 10-months-old he had a laparoscopic pull-through for Hirschsprung’s.
After surgery Tyler was much better, but he continued to have mild gastrointestinal issues. Over the years he experienced symptoms such as multiple bowel movements some days and constipation on others. He had several X-rays done over the years which only showed excess bowel gas. Tyler’s gastroenterologist performed a colonoscopy, an endoscopy and an upper GI series. None of them lead to any answers.
At 14-years-old Tyler’s GI issues intensified. He began having episodes where he would dry heave and double over in pain. I noticed he would position himself with the arm of the couch pressing into his tummy, as if the pressure helped his pain. The episodes typically only lasted a few minutes. As they became more frequent I began to worry. I brought Tyler to the pediatrician several times, but by the time we arrived his symptoms had always resolved. He’d be so happy to see his doctor, which probably made me look a little crazy.
Tyler finally started to become aggressive in an attempt to communicate to us that something was wrong during his episodes of pain. His pediatrician referred us to a new gastroenterologist who felt Tyler’s symptoms were the result of swallowed air. We were told to give Tyler probiotics and put him on a low fodmap diet. I knew he needed more than a diet change.
The next time Tyler had an episode of pain we decided to take him to the emergency department at Texas Children’s Hospital. They did an X-ray, but it looked normal. A bump had popped up near Tyler’s belly button during this episode, so the surgical resident thought Tyler might have a hernia. He advised us to talk to Tyler’s doctor the following week.
The next day Tyler refused to eat anything. I was so frustrated by the lack of answers that I phoned Tyler’s pediatrician and said I needed help advocating for him. I knew something was wrong but felt like I was out of options. The pediatrician called the GI who scheduled him for a barium enema.
Before we had even arrived home after the barium enema, Tyler’s gastroenterologist called to say the test indicated Tyler had some kind of intestinal blockage. They were unsure what was causing the obstruction, but they did not think it was an emergency. They encouraged us to meet with the surgical team later that week. They also said if Tyler showed any signs of pain or vomiting we should head straight to the hospital.
That night Tyler woke me up and asked if I would take him to the doctor. Immediately after asking be began vomiting green bile. We knew what to do and headed straight for the hospital.
The surgical team prepped Tyler for surgery. They were still unsure what was causing Tyler’s obstruction, but they were worried it was cancer. When Tyler got out of surgery several hours later, we learned he didn’t have cancer, he had intestinal malrotation and a partial volvulus. His small intestine had become wrapped around his colon which was mobile. They placed a cecostomy for bowel management and Tyler spent the next 21 days in the hospital.
After being diagnosed with intestinal malrotation we hoped Tyler’s GI issues were behind us, but six months later he woke up vomiting yellow bile. Tyler had developed adhesions that were constricting his bowels and had caused another volvulus. This time surgery didn’t go as smoothly.
Tyler was admitted to the hospital with an NG tube in an attempt to decompress his obstruction. As they monitored him, Tyler's skin became very pale and his lips turned grey. The resident took one look at Tyler and left to page the surgeon. She arrived moments later and they rushed Tyler to the operating room.
As I watched them rush Tyler away, I had a horrible feeling in my gut that when the surgeon came back she was going to tell me Tyler had died. My husband was at work and I was there by myself. I was so scared as I waited for an update. The surgeon came out as soon as she could to tell me Tyler was in septic shock and had coded on the operating table. They had been able to revive him, but they didn’t know if he would make it.
Tyler had six surgeries in the next six days. He spent the next several weeks in the ICU on life support. We never left Tyler’s side while he was in ICU. We didn’t want to miss a single doctor or shift change. Our faith, friends, pastor and church family were the only things that got us through.
It was such a relief to finally bring Tyler home, but a month after Tyler was discharged he developed another adhesion, and had to undergo another surgery. Eighteen months later Tyler’s doctors felt he was ready for his colostomy reversal. The colostomy reversal went well initially, but five months later Tyler developed adhesions and needed surgery again.
Tyler’s doctors believe he has adhesion related disorder (ARD), which means that as his body heals it makes adhesions more rapidly than the average person. We are seven months out from his most recent surgery and I hold my breath every day. I get anxiety when he burps, gets bloated or doesn’t have a good bowel movement. I wonder constantly if the next time could be the time we lose him.
Even though it was difficult to get an intestinal malrotation diagnosis for Tyler initially, we love the surgical team at Texas Children’s Hospital. They have fought so hard for our son. They adore him and have truly become like family to us. As hard as it is to constantly wonder if Tyler will get sick again, it helps to know his surgical team is just a phone call or email away.
If you would like to share your experience with intestinal malrotation, please send us an email for more information.