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Achalasia is a rare disease of the muscle of the esophagus


Acid Reflux -- Esophageal Spasms

Gastroesophageal Reflux Disease (GERD, Acid Reflux)

Acid-Alkaline Balance by Karl Loren

Dr. Weston Price -- On Acid -- Alkaline Balance

Endoscopy & Gastroscopy

Abnormal Propulsion of Food

Achalasia is a rare disease of the muscle of the esophagus

Mass Near The Esophagus -- Cancer?  Benign?

Esophagus Cancer Mortality

 


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Achalasia: Answers to Frequently Asked Questions

Achalasia is a rare disease of the muscle

 

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The "heated cholesterol" Fraud?
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Muscle Testing -- Kinesiology -- Valid Or Not?

Dr. Julian Whitaker Claims Oral Chelation Is No Good!  What Do You Say? What About Seasilver?  Or  The Latest MLM?
The Q2 Machine:  Mysterious Science Pulls In Greedy Suckers What About Coral Calcium?  Mr. Barefoot?
Milk!  The (Now) Dangerous Food! How To Tell If MSM Is The Real  Stuff!

Comparing Clathration with Chelation

Is It True? That You Refuse To Sell Anything To Anyone Taking Certain Drugs?
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Low Body Temperature -- Wilson's Syndrome How Can I Help Persuade My Friend To Use Alternative Methods?
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The Bio terrorism Act Of 2002 -- The Beginning Of The Need For Recognition of Change

Acid Reflux
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Mental Causation Of Heart Disease

My Husband Just Had A Massive Heart Attack In The ER!

Oral Chelation Frauds

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Achalasia: Answers to Frequently Asked Questions

What is the definition of achalasia?

Lay: Achalasia is a disorder of swallowing resulting from the inability of the muscle of the esophagus (food pipe) to relax, preventing food and liquids to get to the stomach. The muscle affected is called the lower esophageal sphincter: this is a ring of muscle at the junction of the esophagus and stomach, which normally stays shut (to prevent stomach acid from coming back) and opens during swallowing to allow food down. In patients with achalasia, this ring stays shut. In addition, the rest of the esophagus loses its ability to pump material down (a process called peristalsis). This combination of defects results in great difficulty in swallowing.

 

 

Scientific: Achalasia is a disorder of the esophagus characterized by failure of the lower esophageal sphincter (LES) to relax and aperistalsis of the esophageal body.

Are there different types of achalasia?

Achalasia can very rarely occur from other disease states (such as cancer), when it is known as secondary achalasia or pseudoachalasia. However, by far the commonest type is primary achalasia which implies that the disease originates in the esophagus itself. This is the type that is commonly implied when the term achalasia is used without qualification. Another common adjective used for this kind of achalasia is "idiopathic" which means that we do not know what causes this disorder. Finally, primary achalasia can also be classified as "classic" (in which there is virtually no contraction seen in the body of the esophagus) or "vigorous" (in which there is activity in the body of the esophagus but this is largely ineffective "spasm" rather than true coordinated pump-like activity or peristalsis).

What percentage of the population has it?

Achalasia is a relatively uncommon disorder of esophageal motility with a prevalence estimated at about 10 in 10,000 and an incidence rate in the range of 0.5 new cases per year per 100,000 population.

What is the age distribution?

Although achalasia has also been described in very young children and the very old, it is uncommon before the age of 25, with a clear-cut age-related increase thereafter. Most commonly the disease occurs in middle adult life (30-60 years of age) and affects both sexes and all races nearly equally.

What causes it?

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Theoretically, achalasia could result from a problem in the swallowing center in the brain, the (vagus) nerves that carry impulses from that center to the esophagus or within the nerve cells residing in the esophagus itself. There is good evidence to suggest that the major problem lies in the nerve cells of the esophagus. These nerves are of two broad types: those that cause the sphincter (or other muscle) to relax or open up and those that cause it contract or tighten up. Normally, the sphincter muscle is in a state of balance between these two opposing sets of nerves. In achalasia, most of the nerves responsible for relaxation are lost, resulting in a shift in the balance towards contraction- hence, the failure of the LES to relax or open up with swallowing.

 

 

What are the symptoms of achalasia?

The major symptoms of achalasia are difficulty in swallowing (dysphagia) and bringing back material from the esophagus (regurgitation). The difficulty in swallowing usually involves both liquids and solids from the onset and is often felt as an obstruction to passage of material in the lower chest. Regurgitation of food can occur spontaneously but particularly when the patient is lying down or bends over. It can occasionally be associated with spells of coughing or even pneumonia if the regurgitated material goes down the airways. The third common symptom is chest pain: this can be associated with swallowing but often occurs spontaneously. The chest pain can be severe or mild. Finally, some patients will also complain of "heartburn", although this is relatively rare.

It is not surprising that patients will lose weight and run the risk of becoming significantly malnourished.

How is it diagnosed?

Although achalasia can be suspected on the basis of clinical history alone, patients usually undergo a series of tests to confirm the diagnosis. These are as follows:

  1. A barium study of the esophagus: this consists of having the patient swallow barium paste or liquid and then having a series of X-rays taken of the esophagus. Variations of this test include cine-esophagograms or video-esophagograms in which the X-rays events are recorded continuously on film or video.
  2. Upper Endoscopy (EGD): this test consists of having a "seeing" instrument called an endoscope inserted through the mouth and into the esophagus and stomach. This test is an outpatient procedure but is usually done under mild sedation. It is necessary to make sure there is no other cause of your symptoms.
  3. Esophageal manometry. This test consists of insertion of a special tube through the nose down into the esophagus for recording pressures. This test allows physicians to directly test the function of the LES and the body of the esophagus. It usually takes about an hour to do.
  4. Other tests such as radionuclide swallowing tests are sometimes ordered and give doctors some idea about the severity of the problem. They may also be useful in following the response to treatment.

Can I die from achalasia by choking to death?

Theoretically patients can have pieces of food and liquids go down the airways, resulting in severe difficulty in breathing. However, the chances of this causing death are very unlikely. Most often when this occurs, patients will complain of coughing at night or develop pneumonia. This does not happen very often these days because of earlier medical attention.

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What are the long term effects of living with Achalasia?

The long term effects of untreated achalasia are poor nutrition with its consequences and a small risk of developing cancer of the esophagus.

Will achalasia reverse itself and disappear after a period of time?

Achalasia does not reverse itself spontaneously.

Tips and tricks of dealing with achalasia.

What are the types of treatment and the pros and cons of each?

Achalasia is not a curable disease yet. However, there are a variety of treatments available that can effectively relieve symptoms and help patients resume a relatively normal lifestyle. The most important cause of symptoms in achalasia appears to be the lower esophageal sphincter- this is a ring of muscle at the junction of the esophagus and the stomach that normally opens with swallowing to allow material to pass into the stomach. In achalasia, this muscle stays shut and therefore causes food to get held up and eventually start backing up into the throat.

All present therapies for achalasia seek to lower the pressure in this ring of muscle, thereby overcoming the resistance to the passage of food (see figure). This can be done by one of the following means:

1. Traditional drugs: these act directly on the muscle, causing it to relax. Examples include nitroglycerin (also used for heart problems) and nifedipine.

2. Balloon (also known as pneumatic) dilation. This involves the insertion of a large balloon into the esophagus at the time of endoscopy. The balloon is positioned across the LES and then inflated quickly with the aim of causing a controlled tear of the muscle. This is usually an outpatient procedure.

3. Surgery (myotomy). This involves exposing the LES surgically and then cutting the muscle directly. In the past, this involved a major operation but nowadays is increasingly being done via "key-hole" surgery (laparascopic or videoscopic myotomy) with much reduced discomfort and length of stay.

4. Botulinum toxin injection. This is also an endoscopic procedure and requires the injection of this drug (botulinum toxin) into the LES. The toxin causes the muscle to be partially paralyzed.
 

 

Questions And Answers About Treatment
No treatment method has clear cut superiority over others in all aspects.
The long-term results (beyond 10 years) are hard to predict with any means of treatment.


Questions Patients Should Ask The Physician

What is the short term effectiveness of treatments?
 

   Overall > 40-50 years of age < 40-50 years of age
 Dilation  70%  70-80% 30-40%
BoTx  70%  80%  40%
 Surgery  80-90%  80-90%  80-90%

 

What immediate adverse effects can I expect?

What will it take to get me through the next five years?

What about after the first 5 years?

Questions The Physician Should Ask You

 

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Do you want the single most permanent method of palliation and are willing to be hospitalized for a few days with moderate discomfort?

Do you want the next most permanent method of palliation and are willing to take the small risk of perforation ?

Do you want the most innocuous procedure that provides effective relief but are willing to undergo a 15 minute endoscopic procedure every year, with the understanding that the long-term consequences are not fully known?


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Achalasia

Achalasia SIZE=+1 COLOR=blue>Outlooks: Achalasia is a rare disease of the muscle of the esophagus. The cause of achalasia is unknown.

Common symptoms include difficulty swallowing and chest pain. Achalasia can be diagnosed by x-ray, endoscopy, or esophageal manometry. Achalasia may increase the risk of cancer of the esophagus.

Treatments include oral medications, dilation or stretching of the esophagus, surgery, and injection of muscle-relaxing medicines directly into the esophagus.

What is achalasia? Achalasia is a rare disease of the muscle of the esophagus (swallowing tube) which is usually diagnosed in young adults. The term achalasia means "failure to relax" and refers to one of the abnormalities of the esophagus seen in the disease, specifically, the inability of the muscle at the lower end of the esophagus (the lower esophageal sphincter) to open and let food pass into the stomach.

In addition, the muscle of the lower half of the esophagus does not contract normally to propel food down the esophagus and into the stomach. Both of these abnormalities result in food sticking in the esophagus after it is swallowed. What is the cause of achalasia? The cause of achalasia is unknown.

Theories on cause include infection, heredity or an abnormality of the immune system which causes the body itself to damage the esophagus. None of these potential causes has been proven.

When the muscle of the lower esophagus is examined under the microscope, inflammation is seen, and a less than normal amount of nerves that control the muscle is present. It is believed that the nerves which are lacking are those that cause the lower esophageal sphincter to relax. As a result, the sphincter does not relax but remains contracted or narrowed.

What are the symptoms of achalasia?

The symptoms of achalasia are difficulty swallowing and, sometimes, chest pain. Regurgitation of food that is trapped in the esophagus can occur, and this can lead to coughing or breathing problems when the regurgitated food enters the throat or lungs.

How is achalasia diagnosed?

The diagnosis of achalasia usually is made by an x-ray study called a video-esophagram in which video x-rays of the esophagus are taken after barium is swallowed. The barium fills the esophagus, and the emptying of the barium into the stomach can be observed. In achalasia, the video-esophagram shows that the esophagus is dilated (enlarged or widened), with a characteristic tapered narrowing of the lower end sometimes likened to a "bird¦s beak."

In addition, the barium stays in the esophagus longer than normal before passing into the stomach. Another test, esophageal manometry, can demonstrate specifically the abnormalities of muscle function that are characteristic of achalasia, that is, the failure of the muscle to contract with swallowing and the failure of the lower sphincter to relax.

For manometry, a thin tube that measures the pressure generated by the contracting esophageal muscle is passed through the nose and into the esophagus. In a patient with achalasia, no wave of pressure due to muscular contraction is seen in the lower half of the esophagus after a swallow, and the pressure within the contracted sphincter does not relax with the swallow. An advantage of manometry is that it can diagnose achalasia early in its course at a time in which the video-esophagram may be normal.

Endoscopy is also a helpful tool in the diagnosis of achalasia. Endoscopy is a procedure in which a flexible tube with a camera on the end is swallowed. The camera provides direct visualization of the inside of the esophagus. Endoscopy is important because it excludes the presence of esophageal cancer, another serious disease of the esophagus that can obstruct the passage of food and dilate the esophagus.

What are the developments and complications of achalasia? A typical patient with achalasia has symptoms for approximately two years before the diagnosis finally is determined. The frequent delay in diagnosis is due to the mild and vague symptoms in the early stages of the disease that often do not cause the patient to seek medical attention. These symptoms include mild chest discomfort, indigestion, or slight difficulty with swallowing.

As the disease progresses, more prominent chest pain, difficulty eating, regurgitation of food, weight loss, and breathing problems appear, which typically lead to testing and diagnosis.

Complications of achalasia include inflammation of the swallowing tube, called esophagitis, which is caused by the irritating food and fluids that collect and remain in the esophagus for prolonged periods of time.

Of potential concern is the possibility that there is an increased occurrence of cancer of the esophagus in patients with achalasia. Some physicians feel that effective treatment of achalasia may reduce the risk for cancer, but this has not been proved.

What is the treatment for achalasia? Treatments for achalasia include oral medications, dilation or stretching of the lower esophageal sphincter, and surgery to cut the sphincter. A newer approach involves injection of medications into the sphincter to loosen the muscle. Oral medications that help to relax the lower esophageal sphincter include groups of drugs called nitrates and calcium-channel blockers.

Although some patients with achalasia have improvement of symptoms with medications, many experience side-effects of the medications. By themselves, medications taken by mouth are likely to provide only short-term and not long-term relief of the symptoms of achalasia.

The lower esophageal sphincter also may be treated directly. Dilation of the lower esophageal sphincter is done by having the patient swallow a tube with a balloon on the end.

The balloon is placed across the sphincter with the help of x-ray, and the balloon is blown up suddenly. The goal is to stretch· actually to tear--the sphincter. Surgical treatment (called myotomy) cuts the muscle of the lower sphincter and thereby reduces the pressure in the muscle. A newer endoscopic treatment is the injection of medicine called botulinum toxin into the lower sphincter to weaken the muscle. Preliminary studies have shown that the use of botulinum toxin is safe, but the effect on the esophagus may be temporary, and additional botulinum toxin therapy may be necessary. Since botulinum toxin therapy is relatively new, it is not known how well such therapy will work over a period of many years.

Dilation of the esophagus causes rupture of the esophagus in one out of every 20 patients. While healing a rupture often requires only careful observation, surgery frequently is necessary to repair the tear.

Dilation also may not be effective permanently, and a second dilation commonly is needed.

What are the newer directions in the management of achalasia? Achieving simpler, safer, and more permanent treatments is the major goal in the management of achalasia. Surgery is a major procedure which involves prolonged recovery time. Recently, laparoscopic surgical techniques for myotomy have been developed that reduce recovery time and the amount of discomfort following surgery.
 

 

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