PXE and Swallowing Difficulties


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By Mary Krieger, R.N.

Swallowing is a basic human function that most of us take for granted. But swallowing is actually a complex process in which 50 pairs of muscles and many different nerves move food from the mouth down into the stomach. And it is an essential function because we must take in food and liquids to survive. The medical term for difficulty with swallowing is dysphagia. This term comes from the Greek words dys- (with difficulty) and phagia (to eat).

There are no studies to date (November 2004) in the medical literature which clearly link dysphagia with PXE. However, many individuals who have PXE also report difficulties with swallowing. It may be helpful to review the events that occur in "normal" swallowing.

Normal Swallowing

There are three stages in the process of swallowing:

1) The tongue moves food around in the mouth and 32 adult teeth grind the food into the proper size for swallowing. Saliva is mixed in to soften and moisten the food to make swallowing easier. Then the tongue collects this soft round mass of food, called a bolus, and moves it to the back of the throat (pharynx). During this time, the lips seal and the tongue pushes up to the roof of the mouth in a front to back motion.

2) The second phase of swallowing is involuntary and happens in approximately 1 second. But it consists of a series of intricate, coordinated events. As the food bolus moves through the pharynx, the passage that leads upward to the nasal cavity (nasopharynx) closes off to prevent food from moving up into the nose. Similarly, the voice box (larynx) closes tightly and breathing stops to keep food and liquids from entering the lungs. Finally, a ringlike band of muscle fibers (sphincter) at the opening to the esophagus relaxes and allows the bolus to pass through.

3) The esophagus is a hollow, muscular tube about 10 inches in length that connects the pharynx to the stomach. It has sphincters at the upper and lower borders. The upper sphincter prevents air from entering the esophagus during breathing and the lower sphincter prevents food and acid in the stomach backing up into the esophagus. Once the food enters the esophagus, it is passed along to the stomach by peristalsis, a wave-like series of muscle contractions and relaxation.

Difficult Swallowing

Dysphagia or difficulty in swallowing can occur at any age, but it seems to be more common in older adults. It occurs if there is a problem with any of the structures or organs involved in the swallowing process: teeth, tongue, mouth, throat, or esophagus. The problems may be mechanical (anatomic) like a tumor or a narrowing of the esophagus by scar tissue (stricture). Or, the problems could be due to impaired motor (neuromuscular) function, as seen in Parkinson disease, multiple sclerosis, or stroke. Some of the signs and symptoms of dysphagia are:
* Pain or pressure in the chest
* Coughing or choking while eating
* Frequent heartburn
* Feeling of food getting stuck in the throat or chest
* Food or stomach acid backing up into the throat (regurgitation)

Tests to Diagnose Swallowing Problems
1) Standard or modified barium swallow - This test involves drinking a barium solution which makes the throat and esophagus more visible on x-ray. It can help to pinpoint the site of the swallowing problem.
2) Upper GI endoscopy - A thin, flexible lighted instrument is passed down the throat to allow the physician to directly view the esophagus.
3) Esophageal manometry - This highly sensitive test measures the pressure of the muscle contractions in the esophagus during swallowing.
4) Chest CT or MRI may also be used.

General Guidelines for Managing Swallowing Difficulties
* Chew food very well
* Eat slowly
* Take small sips of fluid with meals
* Eat smaller, more frequent meals
* Remain upright while eating and for 30 minutes afterward
* Avoid foods that trigger swallowing problems
* Sleep with the head of the bed elevated 6-8 inches on blocks--using pillows to elevate the head during sleep are not as effective

A Special Note on GERD (Gastroesophageal Reflux Disease)

Gastroesophageal reflux disease (GERD) occurs when the lower esophageal sphincter does not close properly and stomach contents or acid leak back up into the esophagus. When stomach acid touches the lining of the esophagus, it can cause a burning sensation which is known as heartburn. No one knows the exact cause of GERD, but several factors may contribute to this condition: obesity, alcohol use, smoking, pregnancy, hiatal hernia.

Certain foods may trigger reflux events: citrus fruits (like oranges), chocolate, caffeine drinks, fatty and fried foods, garlic, onions, mint flavorings (like peppermint), spicy foods, and tomato-based foods (like spaghetti).

Occasional heartburn may not be serious, but if you have heartburn more than twice a week, you should consult your physician.


Dysphagia. National Institute on Deafness and Other Communication Disorders, National Institutes of Health. http://www.nidcd.nih.gov/health/voice/dysph.asp

Heartburn, Hiatal Hernia, and Gastroesophageal Reflux Disease (GERD.) National Digestive Diseases Information Clearinghouse, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health. http://digestive.niddk.nih.gov/ddiseases/pubs/gerd/index.htm

For reliable information on these and other medical conditions, visit the MedlinePlus web site: http://medlineplus.gov/