Living with FPIES

Haley Sylvester is considered medically complex because by the age of three she had been diagnosed with a range of medical conditions, including acute food protein-induced enterocolitis syndrome (FPIES), epilepsy, Sandifer syndrome, and mild to moderate autism spectrum disorder, among others. Haley’s autism causes her to fixate on things often spending her playtime rearranging toys into lines or stacks according to size. Laura, Haley’s mother, said it is not uncommon for her to go into the playroom and see that Haley has formed rows or groupings of some of her toys.
Jennie Cameron, Haley’s full-time nurse, adjusts Haley’s shirt after starting one of her four daily feedings on Nov. 11. Although Haley is not able to digest food regularly because of her condition, she “eats” a PurAmino Hypoallergenic amino acid-based formula though a tube that connects to her stomach, known as a GI tube. The formula is contained in a bag Haley wears in a backpack during her feedings allowing her to play and be active while eating.
Jennie washes out Haley’s backpack feeding pouch after a feeding on Nov 11. Haley ingests hypoallergenic amino-acid formula, the building blocks of the proteins her body can’t naturally break down, through her backpack pouch with a GI tube in her stomach.
A schedule of Haley’s daily feedings and medical injections is posted on the side of the refrigerator, above her backlog which holds the feeding pump. The Sylvester’s live by a rigorous schedule modeled around Haley’s needs for medication and food.
Haley’s list of diagnoses include mild to moderate autism spectrum disorder, gastroesophageal reflux disease, acid reflux, epilepsy, Sandifer syndrome, gastroparesis, and periodic limb movement disorder. Treating this host of conditions results in Haley taking 10 different medicines using 36 syringes daily, and although the Sylvesters get help from Medicaid they still typically spend several thousand dollars a month out of pocket.
Jennie spends the days working as Haley Sylvester’s full-time nurse. Because of Haley’s young age, in addition to her behaviors stemming from her autism, she can easily become fixated on objects. If she were to find a crumb of food laying on the ground and become interested in it she will pick it up, sometimes trying to eat it. However doing so results in a violent reaction by her body, so Jennie and Haley’s family members have to always watch her carefully.
Laura Sylvester embraces her daughter Haley during an afternoon play session on Nov. 11.
Haley and her mother Laura enjoy an afternoon of play together on Nov. 11.
Laura shows her day-planner, filled with reminders about doctor’s appointments and records of what happened. Haley’s medically complex condition results in numerous doctors appointments and daily feeding/medication schedules.
Haley’s frequent reflux problems present noticeable discomfort for her on a regular basis. One moment she is vibrant and running, the next her face glows bright red as her stomach audibly gurgles. When she is having a reaction she wraps herself in her favorite blanket for comfort, alternating between periods of stillness while bearing the pain and writhing and tossing when the pain is too much.
Laura holds printouts of images of a rash outbreak caused by Haley’s FPIES taken during a sigmoidoscopy. These images were taken while Haley had been fasting, and revealed that even when Haley has not had contact with protein her internal systems are poised to start a negative reaction. Haley’s case of FPIES is more severe than some other cases.
No matter how Haley is feeling, seeing her sister always brings a smile to her face.
Hannah and Haley change into matching bedroom shoes shortly after Hannah arrives from school on Nov. 11.
Haley sits with her mother Laura during a Dec. 12 visit to pediatric dentist Jennifer Turner’s office, while they watch sister Hannah get a checkup and cleaning from Dr. Turner.
Haley looks into the camera after a visit to the dentist’s office on Dec. 12. Although her various medical complexities make her life more difficult than the average three year-old, Haley is a normal, curious kid, vibrant, striving, and full of curiosity.

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