Question

What is the incidence and prevalence of Hirschsprung’s disease?

Review treatment options for these children?

What kind of feeding issues or treatments could benefit these children, especially if they have short gut syndrome (TPN? Enteral versus parenteral nutrition?)

Develop 3 nursing diagnosis with interventions for a child with Hirschsprung’s Disease.

Answer 1

Ans) The incidence of Hirschsprung's disease was found to be 0.140 per 1000 live births (1:7,165) with a male: female ratio of 4.1:1 in short segment, and 2.4:1 in long segment Hirschsprung's disease (p = 0.36).

- Hirschsprung's disease is a very serious condition. But if found quickly, it can almost always be cured by surgery. Doctors will usually do one of two types of surgery: Pull-through procedure: This surgery simply cuts out the part of the large intestine with the missing nerve cells.

- Nursing interventions for a child with aganglionic megacolon (hirschaprung disease) include:

Promote skin integrity. When performing routine colostomy care. give careful attention to the area around the colostomy; record and report redness, irritation, and rashy appearances of the skin around the stoma; prepare the skin with skin-toughening preparations that strengthen it and provide better adhesion of the appliance.
Promote comfort. Observe for signs of pain, such as crying, pulse and respiration rate increases, restlessness, guarding of the abdomen, or drawing up the legs; administer analgesics as ordered; additional nursing measures that can be used are changing the child’s position, holding the child when possible, stroking, cuddling, and engaging in age-appropriate activities.
Maintain fluid balance. Accurate intake and output determinations and reporting the character, amount, and consistency of stools help determine when the child may have oral feedings; to monitor fluid loss, record and report the drainage from the NG tube every 8 hours; and immediately report any unusual drainage, such as bright-red bleeding.
Provide oral and nasal care. Perform good mouth care at least every 4 hours; at the same time, gently clean the nares to relieve any irritation from the NG tube.
Provide family teaching. Show the family caregiver how to care for the colostomy at home; discuss topics such as devices and their use, daily irrigation, and skin care; the caregivers should demonstrate their understanding by caring for the colostomy under the supervision of nursing personnel several days before discharge.