White Plains girl battles rare digestive disorder
Only 400 cases of Superior Mesenteric Artery Syndrome documented
Jaylah Redmon, 8, is much like other girls her age. Vibrant and outgoing, she likes to color and paint, watch television and play games on her tablet. But Jaylah is living with a rare medical condition that sets her apart; she’s on a liquid-only diet, as she can’t keep down solid food.
“When I first saw my sisters eating hot dogs, I started crying, because hot dogs are my favorite food. On the second day, they had something else that was my favorite, and I cried, but then Mommy explained it to me and then I stopped crying,” Jaylah said.
Jaylah, a third grader at J.C. Parks Elementary School, was diagnosed last spring with Superior Mesenteric Artery Syndrome, a rare condition that causes the abdominal aorta and superior mesenteric artery to compress the duodenum of her small intestine, preventing food from passing through her stomach into her intestines.
Jaylah can only digest liquid food, and then only in small amounts; everything else causes pain and vomiting, her mother Tiffany Redmon said.
“Basically there’s a blockage in her stomach area and it takes it a while for liquids to even seep through, which is why she throws up, because there’s nowhere else for it to go. It can’t go down, so it comes back up,” Tiffany said.
Although the condition has a huge impact on her life, Jaylah looks to the positive.
“I’m lucky, I can have popsicles at school,” Jaylah said. “And I can have soup, but people are going to have to take all the stuff out. But I get to drink Ensures.”
The condition, also known as Wilkie syndrome, is considered one of the rarest gastrovascular disorders — there are approximately 400 cases documented in English literature, according to radiopaedia.org, an online encyclopedia of radiology.
Jaylah was initially vomiting once a week, but doctors thought it might be nerves as the family had recently moved to White Plains from Waldorf. The frequency of vomiting increased, however. Jaylah had an upper gastrointestinal scan which identified the problem, her mother said.
“We had never heard of it,” Tiffany said. “I asked her doctor if he had any other kids who had this, and he said that since he has been at Children’s [National Medical Center], there’s only been one other case.”
Her doctor recommended she eat several small meals a day, as well as drink two Ensure nutritional beverages, Tiffany said.
But her condition continued to worsen, and by late July, she was vomiting almost every meal.
Jaylah was admitted to the hospital Aug. 3 after losing 13 pounds in one month — more than 17 percent of her body weight.
Her mother said Jaylah kept her spirits up, despite what was happening to her.
“She was still her happy self, even though she couldn’t keep anything down and she had no energy,” Tiffany said.
Following an endoscopy and biopsy of her stomach and duodenum, a nasojejunal feeding tube, or NJ tube, was inserted through Jaylah’s nose into her small intestine to provide nutrition. Jaylah said the tube was the worst pain she has ever felt.
“That hurt even more in my life, than when a baby swing hit me. A baby swing. Or once when a ball hit me in the head and I fell down, and I had a knot on my head,” Jaylah said. “The tube hurt more.”
The tube kept coming out, however, and had to be reinserted three times.
“That is the hardest thing that you can see your child going through,” Tiffany said. “She said she didn’t like the tube, but she couldn’t keep anything down.”
Eventually, Jaylah was able to keep down small amounts of fluids, a little at a time, and released from the hospital Aug. 12.
Now Jaylah can only have very thin liquids, such as thin milkshakes, popsicles and chicken broth, with all the solid parts strained out. She has several Ensure Plus nutrition shakes every day. If Jaylah continues to do well with the fluids, she may be able to move on to try very soft foods, her mother said.
“As long as we take it slow, we can slowly move to soft foods. If she cannot handle that at all, she will have to have a GJ tube or a PEG tube,” Tiffany said, referring to a Percutaneous Endoscopic Gastronomy tube, or feeding tube.
SMAS sometimes occurs as a complication of scoliosis surgery, anorexia or trauma; the actor Christopher Reeve suffered from SMAS after his spinal cord injury. It typically affects girls and young women between 10 and 30 years old. Sometimes, as in Jaylah’s case, the cause of the condition is unknown.
There is no cure for SMAS; doctors told the family there is a possibility she may outgrow it over time, but no one knows for sure, her father James Redmon said.
Surgery could be performed to correct the condition, although it’s not always successful.
“She wouldn’t be able to have those surgeries until she’s 20, or if she gets to the point where she has lost a massive amount of weight, they may attempt the surgery,” Tiffany said.
She said she has spoken with Jaylah’s school, and the staff has been very supportive in setting up a program for Jaylah which will allow her to drink small amounts of Ensure and other liquid food during the day and avoid activities that will make it harder for her to keep the fluids down.
James said it is painful to watch his daughter struggle with a condition and not be able to do anything about it.
“It’s hard as a father,” he said. “My daughter’s crying that she’s hungry, and I’ve got a fridge full of food, and I can’t do anything about it.”
Jaylah’s mother said that because the condition is so rare, many doctors are unaware of it, and people may be going undiagnosed with the condition.
“I just want to get it out there, so that if other people are having this problem — the throwing up, the feeling full, the stabbing pains, the discomfort — they’ll know what to look for,” she said.
She has created a Facebook page, “Jaylah’s Fight with Superior Mesenteric Artery Syndrome,” to give the community updates on Jaylah’s progress. She also hopes to create a support group and a walk to inform others about the condition.
“Jaylah’s my little fighter,” Tiffany said. “I want to scream, I want to cry, but I want to stay strong for her. We’re going to figure out a way to beat this.”