Hirschsprung's disease is a condition of the large intestine that causes severe constipation and intestinal obstruction.

The constipation comes from the lack of nerve cells in part or all of the large intestine. Hirschsprung's disease is mainly found in infants but sometimes a less severe case develops in older children. An adult case of the disease is extremely rare.

Symptoms About 80 percent of children who have Hirschsprung's disease show symptoms within the first six months of life. Children who have only a short segment of intestine lacking nerve cells may not show symptoms for up to several months or years. The most common symptoms of the disease are: no bowel movement in the first 48 hours of life, gradual abdominal bloating, gradual onset of vomiting and a fever. Children who do not show early symptoms may have constipation that becomes worse over time, a loss of appetite, delayed growth and passing small, watery stools.

Diagnosis In order to determine whether a child has Hirschsprung's disease, a doctor will perform tests that may include an abdominal X-ray, a barium enema, an anorectal manometry or a biopsy of the rectum or large intestine. The abdominal X-ray may show a lack of stool in the large intestine or near the rectum and dilated segments on both the large and small intestine. Barium, which is a metallic, chalky liquid, is given into the rectum as an enema to coat the organs so that they show up on an X-ray. This will show whether there are any narrowed areas, blockages or dilated intestines above the obstruction. An anorectal manometry is a test that measures nerve reflexes that are missing in Hirschsprung's disease, and the biopsy of the rectum or large intestine is a test that takes a small sample of the cells in the rectum or large intestine to be examined under a microscope. Treatment Two surgeries may be performed to correct Hirschsprung's disease --- ostomy surgery and pull-through surgery. Soon after a child is diagnosed as having the disease, she will undergo one of these procedures to remove the affected section. Although most children with Hirschsprung's may not need ostomy surgery, a child who has been very sick may need to undergo the surgery so his health can improve before he undergoes the pull-through procedure. Ostomy allows the stool to leave the body through an opening in the abdomen. The surgeon will take out the diseased segment of the large intestine, and the healthy end of it will be moved to the opening of the abdomen, where a stoma is created. A stoma is created by rolling the intestine end back on itself, similar to a shirt cuff, and stitching it to the abdominal wall. An ostomy pouch is attached to the stoma and is worn outside the body to collect the stool. The pullthrough procedure is when the surgeon removes the affected segment of the large intestine and connects the healthy segment to the anus. Diet and Nutrition After the pull-through procedure is performed, children will need to drink more fluids to avoid the risk of dehydration. Because the colon absorbs water from food in the last stages of digestion, when a portion of the colon is removed, the child may have trouble absorbing enough water. Drinking adequate amounts will help the child stay hydrated and may help constipation. Some infants may need to be fed through feeding tubes for awhile. The tube allows an infant's formula or milk to be pumped directly into the stomach or small intestine. Breast milk is recommended due its known laxative effect. If a child is under 12 months of age and is still breastfed, remember that once food is introduced, the frequency and consistency of the stool will change because the breast milk will no longer have its laxative effect. For babies between 4 and 8 months old, some foods they can eat to help avoid constipation are baby oatmeal, baby prunes, green beans, blueberries, apple juice and carrots. Babies between 8 and 10 months old can have canned peas, fresh strawberries, watermelon, honeydew melon, peeled grapes, kiwi, whole-wheat toast, waffles and breads, whole-wheat macaroni and cheese or pasta, steamed broccoli, green beans, cauliflower and green peas. Avoid the following for babies 4 months and older: rice cereal, applesauce, papayas, tangerines, yams, oranges and potatoes without skins. For patients with an ostomy bag, include complex carbohydrates, which can be found in foods like whole-wheat cereals, breads and pasta. Make sure to give plenty of caffeine-free fluids to stay hydrated, and avoid all fruit juices and sugary drinks. Juices offer little to no nutritional benefits and can increase stool and ostomy output. Avoid sources of simple sugars, such as jams, jellies, snack cakes, fruit roll-ups and candies. Large amounts of high-fat dairy products may cause constipation in some children and have no effect in others. Be sure to provide enough dairy foods to meet the calcium requirements needed for the age. For children between 0 and 6 months, 210 mg of calcium a day is needed; for children 7 to 12 months, 270 mg; and for children 1 to 3 years, 500 mg.

Cows Milk Breast-feeding is the preferred method of feeding infants. It provides unique nutritional benefits to the infant, as well as psychological benefits to both mother and child. Breast milk may also protect the infant from infection, and possible allergies. The nutrients provided through breast milk are all that is needed in the first four to six months. Women are encouraged to breast-feed for as long as it is possible to do so. Alternatives to Breast-Feeding If breast-feeding is not possible or is not chosen, there are other acceptable options. When and what type of milk should be introduced is outlined in this article. All of the following are acceptable options - but some more than others. Iron-Fortified Formulas are the recommended infant formulas during the first nine months of age. Premature infants, or infants on solids who are not eating an iron-rich diet, may be at greater risk for developing iron deficiency anemia. Therefore, it may be best to keep these children on iron-fortified formula up to nine to twelve months of age. Standard Milk-Based Formulas are an acceptable option in the first nine months of life. While they do contain some iron, there is not as much as is found in the iron-fortified type. Still, if your child is eating an iron-rich diet by six months of age, this is not a concern. Evaporated Whole Milk Formula is the least acceptable option for infants. The heat treatment of evaporated milk changes the protein to make it easier for infants to digest. However, the milk still must be diluted to lower the protein and mineral concentration and sugar must be added to increase the energy content. The type of fat found in this formula is less well absorbed than the type found in the formulas described above. As well, it contains low amounts of linoleic acid, a type of fat which is essential to health. As long as milk is the only source of energy to the infant, his/her intake of this nutrient will be low. Finally, the iron content of evaporated milk is also low making this formula a less desirable option than those outlined above. Note: While it is not recommended, if this formula is chosen, it should be prepared in a 1:2 ratio of milk to water. That is 1 oz., or 30 mL, evaporated milk to 2 oz., or 60 mL, water mixed with 2 tbsp. or 30 mL of sugar. At six months, this can be changed to a 1:1 ratio. This means 1 oz, or 30 mL, evaporated milk to each 1 oz., or 30 mL, of water. At this concentration, the sugar can be discontinued. Whole cows' Milk can be introduced after nine months of age. For infants under six months, cows' milk protein is hard to digest, the fat is poorly absorbed, and the mineral content is too high. For infants under nine months, reliance on cows' milk as the main source of energy may not provide enough iron. By nine months, the infant's intestinal tract has matured, and whole cows' milk may be introduced. At this stage, the infant should be consuming iron-rich foods such as iron fortified cereals, and vegetables and fruit containing vitamin C. Iron deficiency anemia should be less of a concern at this point. Once consuming cows' milk, only whole (homo) cows' milk is acceptable until two years of age. Cows Milk Tradition best forgotten When it comes to nutritional practices and beliefs, many women seem to place great faith in our tradition. Though there is much that is important and useful in our folklore, in matters of health it is always safe to err on the side of caution. This maxim is particularly relevant in the context of the use of animal milk for infants. In our land, when an infant is weaned from mothers milk, it has traditionally been the practice to replace the mothers milk with cows milk. This can have unpleasant consequences. One of the most common food allergies happens to be the allergy to milk (both cows and buffalos milk). Data suggests that cow milk allergy affects more than 2 per cent of the infant population all over the world. The reason for developing this allergy seems to be quite straightforward.

Animal milk is usually the first foreign protein that is encountered by an infant. In fact, the American Academy of Pediatrics advises against consumption of cows milk (or buffalos milk) in the first year of an infants life. Such animal milk is erroneously considered an appropriate replacement for breast milk or infant formula milk. There are many reasons as to why such milk is not the best choice. It does not provide adequate Vitamin C or iron and on the other hand has excessive sodium and protein. Feeding an infant who is less than one year old with cows milk increases the risk of anaemia and cow milk allergy Once an individual develops the cow milk allergy, it makes one susceptible to other allergies as well. This is the reason why even a soya formula often does not work for a child with cow milk allergy. The only solution, in such a situation, is to provide a chicken or pulse-based feed, which can be prepared at home. The following are the symptoms by which one may know if a child suffers from milk allergy: Excessive colic Recurrent diarrhoea Nausea, abdominal pain Rash, hives and eczema Chronic running nose Nasal stuffiness / recurrent ear infections Failure to thrive Confusions surrounding Milk Allergy There is also a great deal of confusion about what exactly is milk allergy. The common perception is to confuse lactose intolerance with milk allergies. Lactose intolerance is the inability to digest milk sugar (lactose) due to a deficiency of an enzyme (Lactase), whereas food allergies are those reactions where the bodys immune system reacts to the protein in the food by producing antibodies. This is exactly the same situation when the body reacts to an antigen. If a person experiences adverse symptoms without producing antibodies, then the reaction cannot be labeled as an allergy. Thus, one should not confuse lactose intolerance with milk allergy. Children who are allergic to milk can usually take lactose safely. In fact, in a recent double-blind placebo-controlled study, children with proven milk allergies were fed lactose without adverse reactions. Cow milk allergies usually get better as children grow up (usually by about 3 years). Lactose intolerance usually gets worse, although lactose intolerant children can handle small amounts of lactose happily. Interestingly, a child who was being fed solely on his mothers milk developed an allergy that had all the features of cow milk allergy. Further investigation revealed that small amounts of cow milk protein from the milk consumed by the mother was being passed through breast milk to the infant, causing the symptoms. As soon as the mother excluded all dairy products from her diet, the symptoms disappeared. Of course, she was asked to take calcium supplements and she continued to breast feed.