Hirschsprung's Disease

WHAT YOU SHOULD KNOW

Present from birth, Hirschsprung's disease is typically discovered in infancy. It results from absence of the nerves that control activity in the large intestine (colon). Lacking these nerves, the intestine fails to move waste along in the normal manner, allowing it to build up into a massive obstruction. The disorder is easily detected and can be corrected by surgery.

Causes

The cause of Hirschsprung's disease is unknown, but it is believed to be due to a defect in development brought on by maternal fever early in pregnancy. The problem may also be inherited.

Signs/Symptoms

Although this disorder is congenital, it may not become apparent until the child is between four and 10 months of age. Typical warning signs include severe constipation, abdominal pain and swelling, a lack of appetite, and a decreased growth rate. Bowel movements are infrequent and the stools are hard. Newborns experiencing these symptoms may cry with their legs drawn up to their chest, and may begin vomiting shortly after birth.

Care

Two operations are needed to correct this problem. The first surgical procedure, a colostomy, is aimed at quickly restoring normal digestive function through removal of the intestinal obstruction. During this operation, the lower end of the functioning part of the intestine is connected to an opening, called a stoma, made in the abdominal wall. All waste will leave the body through this opening and be deposited into a collection bag, called a stoma appliance.

The second surgery, called a resection, is performed when the child is older and stronger---usually between nine and 12 months of age, or 15 and 20 pounds. In this operation, the surgeon removes the abnormal section of the intestine and permanently connects the functioning remainder to the anus, thereby allowing normal bowel movements.

If the intestine is only mildly dysfunctional, the disease may not be detected until the child is older. Mild cases can be treated with enemas, stool softeners, and a low residue diet.

Risks

The longer this disorder is left untreated, the greater are the chances of the child developing toxic enterocolitis, a potentially fatal inflammation of the intestine.

Call Your Doctor If...

• Your baby is straining to produce bowel movements or the bowel movements are infrequent and hard.
• The infant has no appetite for more than 1 day.

Seek Care Immediately If...

• You notice that the child's abdomen is tight or swollen.
• The child develops severe diarrhea.

IF YOU'RE HEADING FOR THE HOSPITAL

What to Expect While You're There

You may encounter the following procedures and equipment during the child's stay:

• Taking Vital Signs: These include temperature, pulse, blood pressure, and respiration. A stethoscope is used to listen to the heart and lungs. The tests may be performed hourly.
• IV: A tube placed in a vein for giving medications or liquids. It will either be capped or have tubing connected to it.
• X-rays: Prior to this type of surgery, x-ray pictures of the following areas and organs may be needed:
• • chest
  • kidneys
  • gastrointestinal system
• Blood Tests: Samples are taken from a vein in the child's hand or the bend in the elbow. When blood gases must also be evaluated, additional samples may be drawn from the wrist, elbow, or groin.
• Urine Tests: The child's urine will be tested for any abnormalities.
• ECG: Also known as a heart monitor, an electrocardiograph or an EKG. Patches are placed on the child's chest and then hooked up to a TV-type screen that shows a trace of each heartbeat.
• Surgery: An incision is made in the abdomen. The colon is severed and the end of the healthy section is stitched to the abdomen around an opening, or stoma. The other end is stitched closed. For the next few months, bowel movements will pass through the open end of the colon into a stoma appliance, which adheres to the skin and forms a tight seal around the opening.
• Colostomy Home Care Instructions: Before the child is discharged, you will receive explicit instructions from your doctor on how to care for your child during this time. Instructions will vary somewhat depending upon the age of the child. You may be asked to practice certain care procedures prior to discharge.

After You Leave

• Care at home following the operation will be stressful for both you and the baby. The more you know, the easier the care will be. Specifically, you should be given instructions on:
• • how to change the stoma appliance
  • how often the stoma appliance needs to be changed
  • how to monitor fluid loss through the stoma appliance
  • how to take care of the child's skin at the site of the stoma appliance
  • about special dietary requirements (increased fluid is necessary because colon absorption is decreased; a low residue diet is needed to decrease the bulk of the stool)
  • how to take care of the stoma appliance
  • how to control odor
  • how to detect signs of complications, which include ribbon-like stools, diarrhea, constipation, and bleeding
• Change the baby's diapers frequently.
• Wash the skin around the stoma with clear water daily.
• Keep the area of the stoma open to air as much as possible.
• Your doctor will prescribe an ointment or dressing to use on the skin to protect it from secretions and reduce the chance of infection. One application will typically keep the child clean and dry for 24 hours. You may also use karaya gum, Maalox, or aluminum paste to protect the skin from secretions. Check the area frequently.

Call Your Doctor If...

• The child develops a fever.
• You notice swelling, redness, drainage, or bleeding at the surgical site.
• You notice signs of other complications, such as diarrhea, constipation, vomiting, or abdominal pain.