White Plains girl bat­tles rare di­ges­tive dis­or­der

Only 400 cases of Su­pe­rior Me­sen­teric Artery Syn­drome doc­u­mented

Maryland Independent - - Front Page - By JAMIE ANFENSON-COMEAU jan­fen­son-comeau@somd­news.com Twit­ter: @JamieACIndyNews

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.”

The Red­mon fam­ily in their White Plains home: front, from left, Trin­ity, 5, Micah, 7, and Jay­lah, 8, and in back from left, par­ents James and Tif­fany.

STAFF PHOTOS BY JAMIE ANFENSON-COMEAU

Jay­lah Red­mon, a third grader at J.C. Parks Ele­men­tary School, colors in her room. Jay­lah has a rare gas­trovas­cu­lar dis­or­der, Su­pe­rior Me­sen­teric Artery Syn­drome, and is cur­rently on a liq­uid diet.

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