Peter was a full term infant born with a high arch of his palate, a small jaw (micrognathia) and difficulty breathing through his nose which made him mouth breath (unusual for a baby). He also had an extra thumb on one hand (polydactly), webbed toes (syndactly), a flat broad forehead, and gastroesophageal reflux. He was later diagnosed with Smith Lemli Opitz Syndrome.
Feeding: Right from the beginning, Peter looked as if he wanted to eat. He would suck in short spurts before pausing to take a breath, and he would fall asleep before finishing a feeding. We tried several things to make feedings easier for him. We positioned him on his side for feedings because this would allow gravity to pull the tongue to the side and free up his airway to breathe. We used a Haberman feeder, a special bottle that allows the feeder to squeeze milk into the baby?s mouth. We provided cheek support to help him get a better seal on the nipple so that he wouldn?t have to work so hard during feedings. These interventions only helped Peter a little bit and he continued to struggle through less than ? an ounce of formula per feeding receiving the rest through a tube in his mouth (oral-gastric feeding tube). During feedings he snorted, gasped, pulled off the nipple, and appeared to have difficulty breathing.
A modified barium swallow study (x-ray of his swallowing) showed that when he became tired during a feeding, a small amount of formula was aspirated into his lungs. It also showed that his tongue would fall back against the back of his throat causing a block to his breathing. A bronchoscopy (test done where a small tube with a camera on the end is placed into the airway and lungs to look for abnormalities) revealed narrow nasal passages. Peter was sent home for 2 months to work on feeding but did not get any better. Finally, a feeding tube was placed in his stomach (g-tube) along with a Nissen fundoplication (surgery to prevent gastroesophageal reflux by tightening the top of the stomach so that food can?t go back up). A procedure called a lip-tongue adhesion was done which anchored his tongue to his lip with several stitches to stop the tongue from falling back in the airway. He still did not progress with his oral feeds. He was irritable, fussy, had frequent desaturations (he wasn?t able to take in the oxygen he needed) and was tired much of the day.
After much discussion, it was then decided to do a tracheotomy. A small plastic tube would be positioned in his neck to allow for breathing bypassing his upper airway where his structures seemed to be getting in the way. He was now 4 months old. After healing from the surgery, Peter appeared to be feeling better. At his first feeding, he took 2 ounces easily from his Mother and has progressed well from there. He began smiling, fed well, and became a more happy baby.
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