Current Information *J, a 2 week old Caucasian female, was diagnosed with Hirschsprung’s Disease. Hirschsprung’s Disease is a congenital condition where the large intestine becomes obstructed due to poor muscle movement in the bowel. Failure to pass meconium within the first 36 hours after birth indicated a bowel obstruction, and an exploratory laparotomy with sigmoid colon and ileal biopsy supported evidence of Hirschsprung’s Disease. Her recovery from ileostomy surgery and nutritional status is being evaluated for discharge.
An ostomy pouch was used for the drainage of the intestinal waste, and a mucous fistula allowed passing of mucous and gasses from the non- functioning portion of the colon. The patient has no known drug allergies. Bacitracin 500units/g topical ointment is scheduled, and applied generously twice daily to the peristomal erythema surrounding the mucous fistula.
Medications as needed include acetaminophen 37. 5mg every 6 hours 1 to 3 times daily, and hydrocodone-acetaminophen 0. 8ml every 4 hours for pain via nasogastric tube. Lanolin topical is also available, 1 application as needed. She receives enteral feedings of Similac Sensitive formula via nasogastric tube every 3 hours, and successfully passing gas and intestinal waste through ostomy pouch.
The patient’s mother reports 2-3 wet diapers every 4 hours. History *Patient was born vaginally at term weighing 2. 7kg, and measured 48cm in length. The patient’s mother breast fed for the first 48 hours. It was reported that there was difficulty latching on for feedings, the patient was switched to formula feedings. No meconium was passed within 36 hours after birth. Abdominal x-rays showed marked dilation in the bowel with no gas in the rectum. Ileal biopsy study showed the resected tissue had no ganglionic cells. An exploratory laparotomy was performed to rule out other intra-abdominal injuries.
Patient underwent end ileostomy with mucous fistula surgery; the small intestine is connected to the stoma for intestinal waste drainage, and the remaining bowel is able to release gas and drain mucous through the mucous fistula.
Chest x-rays verified initial placement of the endotracheal tube, and nasogastric tube while the patient was intubated. Assessment *Patient physical assessment highlights included distended abdomen, hypoactive bowel sounds, and erythema surrounding borders of surgical incision/mucous fistula. Vital signs include heart rate 162bpm, blood pressure 131/81mmHg, oxygen saturation 95%, temperature 98.
7°F, and respirations 22. She weighed 2. 68kg, and had small/thin extremities. Posterior and anterior fontanels are soft and flat. Pupils are equal round and reactive to light. Mucous membranes are pink and moist. Skin color is normal for ethnicity and dry. Radial and pedal pulses are strong and equal, and skin turgor is elastic on extremities. Capillary refill was less than 2. Behavior was appropriate and patient was easily consoled by family, and she smiled and cooed appropriately. Ostomy collected 80g of yellow, green, liquid, pasty effluent. Urine output was 52ml. Intake via nasogastric tube was 81ml, and 59ml bottle fed.
Patient Management Actual or Potential Problem *Patient’s problems include poor weight gain due to malabsorption and dehydration, and leukocytosis. Potential risks are infection, diarrhea, nausea, vomiting, anorexia, failure to thrive and slow growth and development. Post ileostomy complications such as enterocolitis, and stoma prolapse are risks also. Plan of Care *Patient is discharged, and patient’s mother is denying any home health assistance. She states that she and the patient’s father will be independently caring for J at home.
Family education on normal stoma appearance (red/pink, moist, and shiny), normal effluent consistency and color (liquid, thick, pasty, greenish-yellow), and cleaning the ostomy bag was provided. Advise the family to use special soap for ostomy bag, and to never use deodorant or fragrance inside the bag. Teach family that some foods that may block the stoma are raw pineapple, nuts and seeds, celery, popcorn, corn, dried fruits, mushrooms, chunky relishes, coconut, and some Chinese vegetables. Problem Status * Improvement on gaseous distention was shown by the abdomen being softer and patient’s ability to be easily consoled.
A noted improvement in appetite, and amount of effluent passed was made. Peristomal skin is less irritated, and healing well. Family verbalizes understanding of home care, diet, and when to see their physician. Recommendations * Refer family to various local pediatricians that would be most fitting for them due to the recent retirement of their former pediatrician.
Provide additional information of recommended dates for follow up appointments. Pamphlets are given to further educate the family on the Hirschsprung’s Disease process, and available support groups such as Hirschsprung’s Disease Family Support Group, and Hirschsprung’s & Motility Disorders Support Network.