NH is a 2.5 year old boy born full-term with a history of left congenital diaphragmatic hernia repair, pulmonary hypoplasia, gastroesophageal reflux disease, dysphagia and oral aversion. He was placed on ECMO for 7 days and a ventilator for an additional 27 days. Prior to 6 months of age he also had a nissen fundoplication surgery and gastrostomy tube placement including exploratory laparotomy and lysis of adhesions. He developed a problem with retching after receiving his nissen. He has had multiple revisions of the feeding tube due to leaking and granulation tissue and then conversion to a GJ tube secondary to feeding intolerance. His diaphragm reherniated prior to 12 months of age requiring a repeat primary repair with a patch. He has required resiting of his GJ tube and the prior site closed resulting requiring him to be NPO for several weeks. There have been multiple replacements of the GJ tube whic parents described as traumatic for him. Prior to starting treatment he was being given Nutren Jr and Kids Boost Essentials 1.5 into the g-tube (daytime bolus and overnight) but progress with oral feeding had been minimal. NH was recommended for feeding treatment using a transdisciplinary approach involving collaborative care across providers of occupational therapy, speech therapy, psychology and nutrition.
NH cried with any attempt of therapists coming near him or even looking at him. He was observed while his parents attempted a meal. He sat in his mother’s lap for most of the first session due to refusal to sit in the booster seat. For the next two sessions he tolerated sitting for part of the meal in a Tripp Trapp chair but preferred to be in his mother’s lap. His parents provided verbal cues for him to take bites which he refused. They also tried handing bites to him and attempted to feed him, but all efforts were refused by either turning his head away, pushing away, keeping lips pursed closed, saying no and/or crying. He did not consume any food or drink during this observation. He did take a few small sips of water independently from an open cup but volume was insignificant. Baseline with therapist feeding were not conducted due to NH’s extreme anxiety about the environment and separation from his parents.
Summary of Progress:
Medical: During week 1 NH was noted to be a very fussy child who generally acted like he did not feel well with random outbursts of screaming without any provocation. His medication included Zantac (2ml) 3 times per day. After consultation with the CDH team that had been providing is care, Zantac was reduced to twice per day and Prevacid was added once per day (15mg) via g-tube in the evening 20-30minutes before his overnight tube feeding. Because of loose stools, retching and report of previous reactions to dairy his formula was slowly converted from Boost Kids Essential 1.5 to Elecare during the first week. Retching during meals was an ongoing problem despite efforts at slow stretching. During the fourth week, following additional consultation the CDH team, Prevacid was increased to15mg twice per day and Zantac (3ml) was given twice per day. To rule out a hernia or problem with his diaphragm patch a MBS, esophagram, UGI, and small bowel follow through were completed with up to 8oz combined orally & via g-tube for the study. No retching occurred during the study. His diaphragm patch and nissen fundoplication were intact. Follow-up with a motility specialist was recommended.
Oral Motor: Due to significant defensives to touch to the face, NH required two weeks of gradual desensitization paired with positive reinforcement in order to tolerate an oral motor assessment. Findings showed non productive patterns in almost all areas. Oral motor exercises were performed prior to each feeding session. Significant functional and measurable improvements were noted in the following areas with ongoing oral motor intervention and home program: Lower lip protrusion, elongation and strength, bilateral lateral tongue movement to the lower gum and cheek, tongue tip elevation, bilateral cheek range of motion and strength, bilateral jaw strength and patterns for chewing.
Feeding Treatment Progression/Plan:
Week 1: The first week of treatment started with NH adjusting to sitting in the Tripp Trapp Chair with the seatbelt fastened, playing with toys and remaining seated until the timer rang. His mother sat next to him and therapist gradually moved closer to him in the room during the first day. Oral motor exercises were initiated with his mother touching his upper and lower lip with approximations toward lip stretches. By the second day of therapy, desensitization to the therapist wearing gloves was introduced. A dry cup, squeeze bottle and EZ spoon touched to his lips became part of the meal with even rotation. Touches to the lips gradually worked up to acceptance of the squeeze bottle straw and spoon into his mouth. A continuous reinforcement protocol with 1:1 schedule using video and toys was utilized after each bite/acceptance. The length of treatment sessions was increased from 10 to 15 minutes. By the third day of treatment water was placed in the cup, squeeze bottle and on the spoon prior to presentation. Sessions focused on acceptance of the spoon into his mouth. He initially demonstrated consistent swallowing of the water between 1-3g per trial from the squeeze bottle with small tubing. As NH demonstrated improved tolerance for therapy, meal sessions were increased from 15 to 25 minutes. By the end of the first treatment week he was consuming 19-22g of water per session.
Week 2: NH showed remarkable improvement in desensitization. He was able to transition to the feeding seat without crying; tolerated oral motor exercises to the face and inside his mouth, and his mother did not need to sit right next to him. Titration of Elecare into therapy sessions starting with 1cc dilution in 4oz of water and gradually increased ratio until achieved 100% by the end of the week. Elecare was presented on the EZ spoon and also in the squeeze bottle with small tubing straw. Handling was needed to help facilitate lip closure for clearing the spoon and swallowing due to his inefficient upper and lower lip strength and range of motion. Meal time was increased to 30 minutes in addition to time spent on oral motor exercises (total 45-60min) which continued for the remainder therapy and home practice. Total volume of Elecare averaged 2oz from the squeeze bottle and 3-8g from the spoon.
Week 3: Introduction of Stage II fruits and sweet potato on the EZ spoon was initiated. Elecare (100% concentration) continued to be presented in the squeeze bottle with small tubing straw. NH demonstrated understanding of continuous reinforcement protocol and was motivated by the anticipated positive reinforcement after each trial. The volume of Elecare consumed increased (ranging from 36g-84g per meal) as did volume of puree (ranged from 6g-21g per meal). NH continued to be volume sensitivity with reflux symptoms, discomfort and retching noted when higher amounts were consumed. This also seems to occur after meals 2 and 4 even without increased volume at these meals. Retching overnight continued per parent report.
Week 4: Spoon size was upgraded from and EZ spoon to the First Years Take-and-Toss Infant spoon. This allowed for a larger bolus to be given of the stage 2 foods. Crunchy dissolvable foods wrapped in organza mesh were introduced for exploration of chewing. By end of the week he tolerated Gerber puffs (no longer wrapped in mesh) using placement to molars. He was instructed to chew up to the count of ten. Vertical chewing patterns were noted bilaterally. Rate of presentations of baby food and drink was slowed as needed to minimize retching. Volume for Elecare increased (ranging from 61g-78g per meal) as did volume of puree (ranging from 10g-27g per meal). NH continued to have volume sensitivity with reflux symptoms, discomfort and retching with higher volumes. No overnight retching was noted over the week. The CDH team was contacted and Prevacid was increased to 15mg twice per day along with Zantac 3ml twice per day.
Weeks 5&6: A wider variety of stage 2 foods and mixed dinner combinations boosted with Elecare were introduced. Straw size for the squeeze bottle was upgraded to the large straw to allow for greater volume of Elecare per trial. Volume for Elecare consumed remained relatively unchanged however NH demonstrated occasional self initiated sucking from the straw of the squeeze bottle. Volume of puree consumed continued to improve slightly.
Week 7: NH was transitioned from baby foods to smooth pureed table foods which provided more calories and better nutrition. The spoon was once again upgraded to the larger Take and Toss toddler spoon. Soft solids and solid foods were introduced this week. They were placed on his molars to assist in facilitating a functional chewing pattern and decrease tongue mashing and suckling of food. Attempts were made to increased average volume consumed to greater than 3 ounces per meal. Total volume (combined drink and puree) ranged from 68-175g per meal.
Week 8: NH continued to take soft solids and solid foods in addition to pureed foods. Attempts were made to consistently increase average volume consumed to greater than 4 ounces per meal. Total volume (combined drink and puree) ranged from 101-176g per meal. He was able to achieve 52-58% of his daily caloric needs by mouth. NH had reaction to milk including a rash around his mouth and hives after being fed small sips of milk shake at home over the weekend.
Nutrition: NH was receiving 60ml bolus feedings of Nutren Jr without fiber 5-6 times per day upon initial assessment. He also received 800-850 ml total overnight of Nutren Jr plus 237ml of Kids Boost Essentials 1.5 at a rate of 65ml/hr. His recommended daily caloric needs were estimated at 1250-1350kcal per day. Prior to receiving therapy his parents adjusted his tube feedings based on his degree of retching on any given day and weight gain had been poor and his oral intake was negligible. During the first week of therapy he had not taken any calories orally. Over the course of the first week his formula was changed to Elecare in an effort to improve tolerance and decrease retching episodes. By week 4 he was able to consume 342 kcal/day by mouth across his 4 therapy sessions. By week 8, average volume consumed increased to 620 kcal/day orally which met 52-58% of his daily caloric needs by mouth. NH was able to gain 5.13 grams per day over the course of these 8 weeks which met expected growth velocity of 5-6 g/day while going through the process of reducing tube feedings.
Parent Training: By the second week of therapy, NH’s mother was trained in the structured meal protocol including the timer, supportive seating, administration of the reinforcement schedule as well as use of the squeeze bottle and EZ spoon. She was trained in all oral motor exercises by the 3rd week. Parent training was integrated into daily therapy sessions throughout the admission. NH’s mother replicated what she had learned daily by feeding him in the evenings and over the weekends.
Ongoing Treatment Recommendations:
- Continue outpatient feeding therapy 1-3 times per week with First Steps as well as feeding therapy with psychologist via telemedicine due to traveling distance. If his First Steps therapist was not able to meet the need for increased frequency or did not see ongoing progress then 1-2 times per week therapy with OT/SLP via telemedicine could be added. The goal would be to continue collaborative care with disciplines working together.
- Further evaluate of food intolerance due to observed reaction to milk, odor of maple syrup, intermittent skin rashes, intermittent edema, retching, GI discomfort and ongoing concerns about his bowel movements. Suggested starting with blood test for reactions to milk – casein & whey, soy, egg, peanut, wheat, corn.
- Continue Prevacid and Zantac to manage GI irritation and symptoms of discomfort.
- Referral was made to Pediatric GI specialist for evaluation of motility and reasons for ongoing retching. Rule out possible low gastric pain threshold or visceral hyperalgesia as reason for volume sensitive retching. A possible trial of medication such as Neurontin (gabapentin) or Elavil (amitriptyline) was suggested to see if this would relieve symptoms and allows for better tolerance of higher volume during meals.
- Meal schedule of 3 meals and 2 snacks or 4-5 small meals per day. These should include smooth pureed table food and Elecare 30cal per ounce as well as ~1oz or less of soft table foods (mainly for practicing chewing skills). Meals should be fed evenly rotating through food and drink along with using the structured meal protocol and positive reinforcement after each bite/drink. Use the squeeze bottle for Elecare and the toddler spoon for purees. Meal duration 30min.
- Continue Elecare mixed at 30cal/ounce for meals. Adjust overnight feedings of Elecare 30cal/ounce based on oral intake during the day for a total goal of 1350-1400cal/day. Overnight feedings can be mixed less than 30cal/oz to decrease osmolality when daily oral intake is greater than 500cal. Do not recommend a formula change until food intolerance and GI issues are well managed.
- Monitor weight closely with weekly weight checks either at home or PCP office, especially if continuing to decrease overnight tube feedings.
- Continue the prescribed Beckman Oral motor Exercises 3-5 times per day. Recommended exercises include: Upper and lower lip stretch, side-to-side lip stretch (upper & lower), diagonal nasal bridge stretch, Z-stretch, mini-C stretches, gum massage, lateral pressure to the tongue, cheek stretches with posterior stretch, resistive chewing.