Hirschsprung's Disease

What is Hirschsprung's Disease?

Hirschsprung's Disease, also known as aganglionic megacolon or congenital megacolon, is a disease of the large intestine that usually occurs in children. It is an abnormality that develops before the child is born where the nerve cells in the large intestine stop growing to the end of the digestive tract. In a normal large intestine, nerve cells known as ganglion cells, are located at the last part of the small intestine to the end of the large intestine. Ganglion cells are responsible for making the muscles in the large intestine push waste to the anus where it leaves the body. With these important cells missing, a person with Hirschsprung's suffers constipation while other people with the disease are unable to have a bowel movement at all. The stool creates a blockage in the intestine and if not treated, can fill the large intestine and cause infection, a bursting of the colon and even death. Even though the cause is unknown, it is known that the disease is not caused by the mother's habits and that it can sometimes be heredity. Hirschsprung's Disease is also seen in association with some chromosome abnormalities, such as Down's Syndrome and other rare disorders. It can be either a short segment disease, meaning only part of the colon, or a long segment disease, meaning the diseased section has reached to, or has included, the small intestine.


What Are The Symptoms I Should Look For In My Child?


The symptoms of Hirschsprung's Disease occur mostly in infants in the first 6 weeks of life or they can occur later in infancy or childhood depending on the extent of the disease. Symptoms may vary within individuals; however, the most common symptoms are:

* Not being able to pass stool in the first 48 hours of life

* Vomiting

* Bloating of the abdomen

* Constipation

* Fever

* Poor weight gain

* Loss of appetite

* Passing small, watery stools

* Malabsorbtion

These symptoms may also resemble other medical conditions that need prompt attention and therefore it is advised that you take your child to see their pediatrician for an adequate diagnosis.


What Kind Of Tests Do Doctors Use To Diagnose Hirschsprung's Disease?


The physician will examine the child and obtain a medical history then other tests may be done. These test may include an abdominal x-ray, a barium enema, an anal manometry and a biopsy of the large intestine or rectum.

* Abdominal x-rays show a picture of the bowel and the amount of stool in the bowel.

* Barium enemas are procedures in which barium is given into a rectum as an enema. Barium is a metallic, chemical, chalky, liquid used to coat the inside of organs so that they will show up on an x-ray. After the enema is given, an x-ray is taken to show abnormalities such as blockages, dialated intestine above the blockages or narrowed areas.

* An anal manometry is a balloon study in which a balloon type device is inserted into the rectum. This device is used to enlarge the anus to test the nerve reflexes which would be missing in Hirschsprung's Disease.

* Biopsies of the large intestine and rectum, sometimes performed under sedation, are tests that take samples of cells from the large intestine or rectum. These samples are put under a microscope for further examination.


What Is The Treatment For Hirschsprung's Disease?

Treatment for Hirschsprung's Disease depends on the medical history, overall health, and the extent of the problem as well as the child's age. Other factors include the child's tolerances for certain procedures, medications or therapies as well as expectations for the course of the disorder. Surgery is the only treatment for Hirschsprung's Disease where the abnormal section of the bowel is removed; such as the case with short segent disease. This procedure, called a Bowel Reconstruction, is done in two stages. The first stage consists of creating a colostomy. A colostomy brings a portion of the large intestine through the abdominal wall to carry feces out of the body. The bowel contents are therefore discharged into a waste bag. After the child is healthy enough, the second stage of the procedure is performed to close the colostomy, remove the diseased portion of bowel, and to connect the healthy bowel to the anus. Colostomies in this case can be temporary or permanent; depending on how much the bowel is affected. If it is a long segment disease, then an ileostomy is performed. The entire large intestine is removed and a portion of the small intestine is brought out through the abdominal wall to carry feces outside the body. Like a colostomy, the waste of an ileostomy is also discharged into a specially made waste bag.


The prognosis after treatment is very good in children with Hirschsprung's Disease. With the diseased portion gone, they go on to lead normal healthy and happy lives.

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Digestive System Disorders

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Colon polyps are tissues that grow inside the large intestine and are common in individuals 60 years and older. Even though polyps are mostly non-cancerous, they do have the potential to become cancerous if they are not removed. Polyps vary from size and shape from a small mushroon shape with a stalk about the size of a pea to large flat ones the size of a golf ball and can appear anywhere inside the colon.

Age is a contributing factor in the risk of colon polyps. However, other factors can contribute to the development of colon polyps and cancer. Among these factors include:

Poor Eating Habits: A diet that is low in fiber and high in fat is a contributor to colon cancer because cancer-causing substances (carcinogens) in foods when consumed stay in the bowel longer. Fiber provides bulk that moves your stool more quickly through your bowel and therefore eliminates the carcinogens more quickly.

Inactive Lifestyle: Physical activity such as exercise helps in the digestion of food by moving the waste through the bowel faster. Therefore, when a person is inactive, the food stays in the bowel longer thus creating the possibility of polyps. It is noted that inactive people are more likely to develop colon cancer but not rectal cancer.

Inflammatory Intestinal Conditions: People with conditions such as Crohn's disease and ulcerative colitis are more likely that those without the conditions to develop colon polyps and cancer.

Overweight or Obesity:The excess fat in the obese encourages the growth of the cells in the colon and the rectum. Many health problems, which also include several types of cancer including colon cancer, are linked to obesity.

Smoking: It is a widely known fact that smoking causes many deadly lung diseases such as lung cancer and emphysema. It is also known that smoking, especially when consuming excess amounts of alcohol, increases the risk of colon polyps and cancer. It is estimated that people who smoke 20 cigarettes or more a day have 250 percent more polyps than nonsmokers do.

Family History: The risk of getting colon polyps and cancer increses when a parent, sibling or other relative has the disease. Even though some are heredity, other cases of colon cancer can also be passed down through similar diet or lifestyle factors as well as simular exposure to environmental carcinogens.

Symptoms of colon polyps are sometimes not noticed and some individuals find out if they have them when a physician examines their bowel. Others however, notice symptoms such as blood in the stool and/or rectal bleeding, fatigue associated with anemia, abdominal pain, constipation or diarrhea. Several tests for colon polyps and cancer include a rectal examination, a barium enema, colonoscopy, a virtual colonoscopy and a sigmoidoscopy.

Treatment for polyps includes the removal of them the same time a colonoscopy is performed. Follow up colonoscopies should be done on patients with polyps within 3 to 5 years to see if the polyps have returned.

Avoiding smoking and excessive alcohol intake, maintaining a healthy body weight, eating a diet low in fat and high in fiber, fruits and vegetables reduces the risk of getting colon polyps. Regular colonoscopies after the age of 50 are also recommended to reduce risk.

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Digestive System Disorders: Ulcers

An ulcer is an open sore, commonly found in the mucous membranes of the body. Canker sores are one form of ulcer, that occurs in the mouth. If an ulcer occurs in the lining of the stomach or duodenum, then it is called a peptic ulcer, which is probably what most people think of when the hear the word ulcer.

Peptic ulcers do not necessarily cause any noticeable symptoms, but when they do, the most common symptom is a burning pain between the breastbone and navel. Other symptoms of a peptic ulcer may include belching, nausea, loss of appetite, loss of weight, and a tired and weak feeling.

It used to be thought that ulcers were caused by stress and/or spicy food. Now, however, primary cause of stomach ulcers is believed to be a bacteria, called Helicobacter pylori. This bacteria lives in the mucous lining of the stomach, and usually causes no problems. But sometimes, this bacteria weakens the mucous coating of the stomach, which protects it from the acids present in the stomach. These acids then start to irritate the stomach, eventually causing an ulcer. Some factors that may cause or aggravate peptic ulcers are: smoking, excessive alcohol consumption, regular use of pain relievers, and stress. Although stress does not cause ulcers, it may aggravate the symptoms, and delay the healing of an ulcer.

As with any digestive disorder, if you think you have an ulcer, you should see a doctor for a professional diagnosis and treatment.

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Digestive System Disorders: Celiac Disease

Perhaps you may have seen several food products in the store that state "gluten free" on the labels. Or you may also have heard of the term gluten free diet and might wonder what it is. Gluten is a protein substance that is often found in wheat, rye, and barley as well as other products. In people with celiac disease, this protein is the cause of many undesirable symptoms that are painful. In the lining of the small intestine, finger like protrusions, called villi, normally allow nutrients from food to be absorbed into the bloodstream. In celiac disease, these villi become damaged or destroyed upon the ingestion of gluten. Regardless of the quantity of food eaten, a person can become malnourished without healthy villi. Since the immune system is responsible for damaging the small intestine in the condition, Celiac Disease is considered an auto immune disorder as well as a malabsorbtion disorder due to the nutrients not being absorbed. It is a genetic condition which can be triggered by surgery, pregnancy, severe emotional stress or viral infection. Celiac disease is also known as gluten-sensitive enteropathy, celiac sprue or nontropical sprue.

Symptoms of celiac disease are varied and affects people in different ways and therefore might be very difficult to diagnose. It can mimic such diseases as Crohn's Disease, irritable bowel syndrome, parasite infections, anemia, gastric ulcers, skin disorders or even a nervous condition. Some people with Celiac may have no gastrointestinal symptoms at all, some develop symptoms as children, while others develop it as adults. It is also noted that the symptoms can occur in the digestive system while other symptoms occur elsewhere in the body. Among the wide variety of symptoms of celiac include the following:

- chronic diarrhea
- recurring abdominal bloating and pain
- pale, foul-smelling, or fatty stool
- gas
- fatigue
- weight loss / weight gain
- unexplained anemia
- osteoporosis, osteopenia
- bone or joint pain
- infertility, recurrent miscarriage
- missed menstrual periods (often because of excessive weight loss)
- seizures
- tingling numbness in the legs (from nerve damage)
- muscle cramps
- behavioral changes
- pale sores inside the mouth, called aphthous ulcers
- tooth discoloration or loss of enamel
- itchy skin rash called dermatitis herpetiformis
- failure to thrive in infants
- delayed growth

Before the diagnostic tests for celiac disease, it is advisable that the patient should continue to eat a regular diet that includes foods with gluten, such as breads and pastas for an accurate diagnosis. Several tests for celiac disease would include a blood tests that measures anti-tissue transglutaminase (tTGA), IgA anti-endomysium antibodies (AEA), Immunoglobulin A (IgA) and possible a Complete Blood Count (CBC) (to look for anemia). If celiac disease is suspected due to the symptoms and the blood tests, more tests would be done for conformation. These tests can be done by examining the tissue (in particular, the villi), which is acquired through a biopsy of the small intestine. Other tests might include a CRP (C-Reactive protein) to evaluate inflammation, a CMP (complete metabolic panel) to determine electrolyte, protein, and calcium levels, and to verify the status of the kidney and liver, Vitamin D, E, and B12 levels to measure vitamin deficiencies, an evaluation of stool fat, to help evaluate malabsorption as well as an ESR (erythrocyte sedimentation rate) to evaluate inflammation.

Treatment begins immediately once the diagnosis has been made. Unlike many conditions, celiac disease does not require any special medications, frequent visits to the doctor or surgery. The only treatment that is known is the "gluten free" diet; meaning the elimination of foods that contain the protein gluten. This elimination is lifelong commitment and can be difficult at first, but the results of good health are very rewarding. Some of the foods that contain gluten include those that contain wheat or wheat flour. However, there are several more foods that need to be avoided as well due to the fact that they still contain gluten. There are still many products available that are gluten free which the person can still enjoy.

Celiac Disease
Celiac Disease

People who visit their physician for symptoms of acid reflux are given tests to confirm diagnosis. Tests include an examination of the throat and larynx, x-rays, esophageal acid testing, biopsies and an endoscopy (a tube that is inserted down the throat and into the stomach). Endoscopies can also identify the complications of GERD such as ulcers, Barrett's esophagus and strictures.

Once the diagnosis of acid reflux has been made, several treatment options are available for the sufferer. among these treatments include:

Antacids: These antacids neutralize the acid in the stomach therefore leaving no acid to reflux. It is recommended to take these one hour after meals or just before the symptoms of reflux begin after a meal.

Lifestyle changes: A change in eating habits may be helpful for those with GERD. It is also one of the easiest of treatments. Change in eating habits includes smaller and earlier evening meals so the food would have already passed by bedtime. The patient should also avoid certain foods that can aggravate the condition. Among these foods include caffeine drinks, peppermint, fatty foods, alcohol and chocolate. If the patient is a smoker, it is also advised that the patient should quit smoking (although the patient should quit smoking anyhow, whether suffering from GERD or not). Other foods that might aggravate the condition include spicy or acid containing foods such as soft drinks citrus and tomato juices.

Histamine antagonists: Histamine antagonists are the first medications developed for more effective and convenient treatment of acid-related diseases. These medicines work by blocking histamine from stimulating the acid-producing cells. Histamine is an important chemical that stimulates acid production in the stomach. Histamine antagonists are best taken 30 minutes before a meal. They can also be taken at night before going to bed. The four known Histamine antagonist include Tagamet, Axid, Pepcid and Zantac.

Proton pump inhibitors: Proton pump inhibitors block the secretion of acid in the stomach by the acid producing cells. This medicine can also be used when the patient suffers from complications such as ulcers, Barrett's esophagus and strictures. It is also given when histamine antagonists do not work. Proton pump inhibitors are usually taken when the stomach is most actively producing acid, which occurs after meals. Such proton pump inhibitors include Prevacid, Aciphex,Nexium, Protonix and Prilosec.

Pro-motility drugs: Pro-motility drugs work by stimulating the muscles of the digestive tract from esophagus to the small intestine and/or colon to speed up the stomach emptying process. These are taken 30 minutes before a meal and at bedtime. One such drug that has been approved for GERD is Raglan.

Foam barriers: These medications are tablets that include antacids with a foaming agent. When the tablet is taken and it reaches the stomach, it turns into foam that floats on the top of the liquid contents of the stomach. This foam serves as a barrier to the reflux of the liquid. When the acid comes in contact with the foam, it neutralizes. These foam barriers are taken after meals and the known foam barrier that is prescribed is Gaviscon.

Surgery: Sometimes surgery is an option when all other treatments are ineffective. It is very effective at relieving the symptoms of GERD. Most patients will experience relief for 5 to 10 years.

Heartburn can be only a minor nuisance to many people. However, many have what is called chronic heartburn that should be treated. For heartburn to be considered chronic, symptoms would occur 2 or more times a week. If you are experiencing the symptoms of acid reflux, please see your physician for diagnosis and a treatment that is right for you.

Acid Reflux
Acid Reflux

In the stomach, there is an amount of liquid content that helps digest food. This content contains an enzyme called pepsin, that helps in the digestion of proteins, and acid. In acid reflux (also known as gastroesophageal reflux disease, or GERD), this liquid backs up (refluxes) into the esophagus, causing heartburn, regurgitation and even nausea in those suffering from GERD. In certain people, the liquid may also consists of bile, that was refluxed into the stomach from the duodenum. This condition is chronic and can inflame and damage the esophagus.

There are several conditions and factors that make people more susceptible to acid reflux. For example, in pregnancy the increased pressure in the abdomen caused by the growing fetus, in conjunction with the elevated hormones in the mother tends to lower the pressure in the lower esophageal sphincter. This action is known to increase reflux. People who also suffer from connective tissue diseases as well as scleroderma are also prone to GERD. Other factors include hiatal hernias, defective esophageal contractions, slower emptying of the stomach and abnormalities of the lower esophageal sphincter.

Symptoms of acid reflux are uncomfortable and painful, and should not be ignored over a period of time. As mentioned earlier, GERD is chronic and can cause damage to the esophagus. People who suffer from this condition have the following symptoms, which can vary within individuals.

Heartburn: Heartburn is a burning pain in the middle of the chest, behind the sternum. Heartburn commonly after meals, and is due to the stimulation of the nerve fibers in the esophagus caused by acid in the esophagus. Heartburn can also occur when laying down due to the effects of gravity and the stomach, therefore acid is returned to the stomach more slowly. The severity of heartburn goes from frequent and/or severe for several weeks and months to less frequent and/or severe, perhaps absent, over a period of several weeks and months following. Heartburn is a chronic problem that almost always returns.

Regurgitation: Regurgitation happens when larger quantities of liquid (and sometimes food),are refluxed and reach the upper esophagus. The reflux liquid gets through the upper esophageal sphincter, which normally prevents esophageal contents from backing up into the throat; resulting in an acid taste. If there are larger quantities getting though, patients may find their mouths full of the liquid or food, which might be included in the liquid. Acid induced erosions of the teeth can also result from this symptom of acid reflux.

Nausea: A rare symptoms of acid reflux, the frequency and/or the severity of acid reflux might induce such vomiting. Acid reflux (or GERD) is one of the first conditions suspected when unexplained vomiting is a symptom.

Knowing the symptoms of GERD is the first step to treating the condition. If left untreated, then damage to the esophagus may occur. If you think you may be suffering acid reflux, it is important for you to see a doctor, and get a proper diagnosis, and talk about treatment options.

Acid Reflux Disease
GERD

On occasion, the colon develops weak spots, where small pouches bulge outwards, much like a weak spot on an inner tube. These pouches are called diverticula. When you have these weak spots in the colon, the condition is called diverticulosis. In about 10-25 percent of people who have diverticulosis, the diverticula may get inflamed or infected. This condition is called diverticulitus.

It is believed that diverticulosis is caused by a low fiber diet, which makes stool harder to pass, which causes increased pressure in the colon. This increased pressure causes the weak spots in the colon to bulge, forming the diverticula.

There may be no symptoms from diverticulosis, although there might be. Symptoms include mild cramps, bloating, and constipation.

Symptoms of diverticulitis include abdominal pain, particularly in the lower left part of the abdomen. Cramping and constipation may also occur. If the diverticulitis is caused by an infection, then the symptoms may also include fever, chills, and nausea.

Diverticulitis may be mild, or it could be serious. Mild forms may require nothing more than rest, and just allowing the body to fight off the infection on it's own. For more serious cases of diverticulitis, antibiotics may be prescribed, and in extreme cases, surgery may be necessary to remove the affected portions of the colon.

Increasing the amount of fiber in the diet may help to reduce the symptoms of diverticulosis, and help to prevent diverticulitis. Fiber helps to soften stool, and help it to pass through the colon more easily.

Diverticulitis
Diverticulitis