Achalasia is a rare neurological condition of the oesophagus where the nerves controlling the oesophagus are damaged and no longer transmit signals to the muscles. This causes weak peristalsis or a lack of peristalsis. The lower oesophageal sphincter no longer relaxes effectively to let food pass through to the stomach. Food builds-up in the lower part of the oesophagus. This build-up of food causes stretching of the oesophagus. The cause is unknown. Tests to prove that the patient has achalasia are: Manometry, barium X-ray, and gastroscopy.
Symptoms may include:
Difficulty with swallowing food and liquid; heartburn, spasms and chest pain, vomiting or regurgitation of food, coughing, as well as night time choking.
Treatment options are:
Dilation of the lower oesophageal sphincter, Heller Myotomy, and Botox injections.
Note: this information is written especially for the patient and his/her caregiver. Where possible simple English has been used to make it easier to understand. It is hoped that it will give the patient’s perspective to the condition. I am indebted to the Yahoo.com Achalasia Support Group, for the information which does not fall into my own experience of the condition. Special thanks to Jerry Hastings, Peter Scott, Maggi Smith, and Sandra Holt-Smith, for their contributions and editorial comments - Joan Pearse]
What is achalasia ? How does Achalasia affect the patient??
A person is said to have Achalasia when the nerves which control the working of the oesophagus [the tube that connects the mouth to the stomach] no longer work. The word achalasia comes from the Greek “a”- failure or absence + “chalasis”- relaxation = failure of relaxation. Because of this:
The food that is swallowed does not go down to the stomach easily because there is no peristalsis [movement of muscles in the oesophagus pushing the food down]
The lower oesophageal sphincter (‘LES’)or ‘valve’ [the muscle that opens and closes the opening from the oesophagus to the stomach] also does not work. This muscle can no longer relax and open as and when it should. It does not always work properly and later as the condition progresses remains closed.
Because the sphincter no longer opens properly, food cannot go into the stomach, and it builds up in the oesophagus. - This causes the oesophagus to stretch and after some time it remains stretched and damaged.. - Old food stays in the bottom end of the oesophagus.
Primary Achalasia is that which originated in the oesophagus.
Achalasia is called - ‘Classic Achalasia’ when there is virtually no contraction or peristalsis in the body of the oesophagus - ‘Vigorous Achalasia’ when the action of the muscles of the oesophagus are ineffective and spasms occur rather than co-ordinated muscle action. - ‘Idiopathic Achalasia’ when the cause in unknown
Secondary Achalasia or Pseudo Achalasia occurs when other diseases cause the symptoms eg. Cancer.
Cause of Achalasia
The cause of the nerve damage has not been decided on. These are causes that are being looked at:
Viral and other infections
Autoimmune reactions [the body acts against itself]
Inherited disorder.[passed from parent to child]
Not all patients have the same symptoms – each patient has their own specific group of symptoms.
Some symptoms that patients with Achalasia have:
Difficulty in swallowing food. - usually solid food and later also liquids. Sometimes liquids are a problem first before other food.
When food gets ‘stuck’ there is usually some discomfort - there may be pain or spasm. There may be a feeling of pressure or choking.
Severe spasm or “Non-cardiac chest pain” occurs in some people and can be severe. Spasm of the sphincter may be severe, and feel like a heartattack. The pain can be over the sternum [breast bone] and go through the left shoulder, or the back, neck, roof of the mouth and teeth. It is often mistaken for heartburn. Special medication is usually effective for this pain, but some can manage to prevent the attack by drinking large amounts of water or by eating certain snack foods.
Most patients get “heartburn” early in the disease but may not later on - because of nerve damage it may not be felt. - it is usually caused by the acid [lactic acid, carboxylic or other acid] which is made by the fermenting undigested food which collects in the bottom of the oesophagus. - acid from food, drinks, medication or alcohol are also irritant and a cause of heartburn
Different food affects different people – what one person cannot swallow may be easy for another. – the foods that often cause a problem are pasta, potatoes, rice, meat, raw vegetables.
Regurgitation of the food ‘stuck’ in the oesophagus may be necessary to feel relief. Regurgitation often happens with out trying. Some people use regurgitation to clear the oesophagus. [vomiting happens when food is thrown up from the stomach]
Regurgitation of food may also happen when the patient bends down, or lies down.
A lot of froth – a mixture of saliva, mucous and air – is found in the oesophagus, especially when food gets stuck, or has trouble getting down. - there appears to be a lot of extra saliva
The patient may have a cough which cannot be explained. The cough usually happens because food and liquid is regurgitated during rest when the patient is lying down [at night or daytime], and food gets into the lungs. - the body tries to get rid of this by coughing.
Weigh loss may happen, especially in the later stages of the disease. - some patients with Achalasia may be overweight because the only type of food they can eat is fattening.
Burping/belching – some people have a problem because of burping – often quite loudly. Sometimes it sounds ‘squeaky’ or like water bubbling down a drain. It is uncontrollable and not related to meal times – it is probably due to the food trapped in the lower oesophagus which is now fermenting.
Tests and examinations:
A special barium x-ray is taken. The patient swallows liquid barium, and the flow of this can be seen on a video screen. Still pictures of this are taken for the record. A video recording can be made. – sometimes the patient is asked to swallow something solid containing barium. This can either be a pill or barium mixed into some bread, egg or cornflakes. – the barium is not unpleasant to swallow and the test is painless, but can take some time as they watch the barium moving through to the stomach.
Manometry: Manometry is a test measuring the pressures inside the oesophagus. These pressures give an indication of how well the muscles of the oesophagus are working i.e. The strength of the peristalsis and how well the lower oesophageal sphincter relaxes. These tests make sure there are no other diseases causing the problem. - a thin tube is passed down the patient’s nose or mouth and into the oesophagus. This tube contains a sensor. - mouthfuls of water are swallowed and pressure readings are taken as the tube is pushed downwards and into the lower oesophageal sphincter and pulled out again - the test is not painful but a bit uncomfortable and can be embarrassing as the patient wants to vomit up the tube. Gagging can be reduced by the use of anaesthetic sprays or gels. - make sure you have a supply of tissues and just relax. It is soon over.
A gastroscopy is an examination done under deep sedation. The gastroenterologist examines the oesophagus using a gastroscope [a special flexible tube containing a camera and small instruments] to look inside. The gastroscope is passed down the throat into the oesophagus once the patient is asleep.
The doctor will take a detailed history of your symptoms.
There are four options:
1.Conservative treatment [treatment with medicines]
learning to live with the condition
this means using medication to control spasm and acid
medicines which control spasms: Calcium Channel blockers (nefidipine [Isordil,Verapamil] and others] and nitroglycerin. Certain anti-depressant and anti-convulsant medications may work for some people.
2. Surgery – is the recommended treatment when conservative management fails
A Heller’s Myotomy is usually done. This means that the lower oesophageal sphincter muscle is cut across to break the spasm, and reduce the pressure of the blockage.
Myotomy is the most successful procedure and can give long lasting relief, but there is a possibility that it may not work and may have to be repeated.
A fundoplication or wrap may be done as well as a Myotomy – this means that a piece of the fundus (upper stomach) is wrapped around the oesophageal sphincter in such a way that it prevents the stomach acid from going into the oesophagus. This procedure may be successful, but it may also cause problems if it is made too tight.
Surgery may be done the traditional way with an ‘open’ laparotomy (cut in the abdominal (stomach) wall or a laparoscopy with the use of a laparoscope. A laparoscope is a highly complex instrument and is used to do the surgery through 5 small cuts in the abdomen - It is less invasive and the patient usually goes home after a couple of days. With open surgery the patient is in hospital for about 5-7days.
Recovery from surgery depends on the patient but with a laparoscopy the patient may go back to work earlier – about 2 weeks. With open surgery [laparotomy] the recovery time is longer – 4-8 weeks.
Surgery should be considered when the oesophagus appears to be stretching.
Surgery is said to be more successful if done early rather than later when the oesophagus has already stretched.
Surgery is the preferred treatment for younger patients.
3. Dilatation of the lower oesophageal sphincter:
Dilation can be successful and is used mostly when surgery is not possible or wanted.
The number of dilatations that can be done safely are limited as the risk of perforation or tearing increases with each successive dilatation.
The effect of a dilatation may last for years, months or weeks or it may not work.
4. Botox :
Botulism toxin is used to paralyse the muscle of the oesophageal sphincter.
The effect of Botox is variable and may or may not be effective. There is a decrease of effectiveness with each injection
Botox needs to be repeated sometimes every 6 months or sooner
The long term safety of Botox is not yet established.
Most specialist doctors now no longer recommend it’s use in younger patients for achalasia.
Is there a cure for achalasia?
The cause of achalasia has not been discovered and there is no cure. The disease is progressive [it gets a little worse] with time. The treatment used depends on what stage you are at and hopefully treatment will ease the problems. Because people who have laparoscopic surgery get better quicker and the results are usually good, surgery is now used for more patients in the early stages. They may remain with few problems for several years. Surgery may have to be repeated at a later stage or followed up with a dilatation. As a last resort the oesophagus may be removed altogether.
How to cope with Achalasia
Achalasia is with you for the rest of your life, so the sooner you learn to live with your condition the better for you and your family. You have to work out what works best for you. What may be helpful to some one else may not work for you. Here are some of the things others do to help them cope:
Tell your close friends and family about achalasia. It will help you cope with the condition and they can be more supportive if they understand the facts about the condition. You will find that most people are interested – but don’t go on and on about it!
Drink lots of water with your food - some times even with liquid food ! - some use water at room temperature, others need iced water or hot water - when you go to a restaurant arrange with your waiter to see that you always have a full glass of water or a jug of water. - carry a bottle or bottles of water with you.
Some find gulping air and water useful to force the food down
Others cannot drink water with meals at all !
Cut your food up into tiny pieces and chew it well
Some can only eat minced meat and mashed soft food. - get yourself a liquidizer or blender.
Use lots of gravy and sauces
Have small amounts of food more often.
Learn to make nutritious home made soups – you may need to put the soup through the blender .
There are lots of food supplements you can drink e.g. ENSURE, BUILD UP, etc.
Baby food can be a useful emergency food
Adult or children’s Vitamin and Mineral syrup [Vidaylin] and pain syrup [Stopayne Liquid or Kidease] are useful when you have difficulty swallowing pills. Some medicine comes in powder or effervescent form.
Wrap pills in margarine to make them easy to swallow. - discuss this with your doctor or pharmacist. Some pills may not dissolve when they should if they are coated with margarine.
Eat sitting upright – let gravity help the food go down – stay upright for several hours after eating.
Others find it best not to lie down at all for 4-5 hours after a meal.
Try and be as relaxed as possible when you eat. Some find a glass of wine helps them relax but most people will find wine too acidic.
Stress seems to play a part in making the condition worse. Learn stress relieving and coping strategies.
If you feel that some food is stuck and the pressure is mounting inside you, rather make a dignified trip to the bathroom than leave it till you start to heave and have to run for it.
Froth in your throat can be a real problem – try to spit it out in the toilet without loosing what you have eaten. This takes a bit of practice.
You cannot always say when the food will come back up so be prepared and carry a suitable plastic bag in your handbag or briefcase in case you are caught out - for example in the car.
Raise the head of your bed on bricks or blocks – 6"(15cm) or more height is needed. [One or two standard bricks] This will help prevent regurgitation at night. - some find a wedge pillow useful. you may find sleeping in a recliner chair helpful.
Ant-acids can be useful if you have a lot of acid and heartburn. Remember that certain ant-acids [ aluminium hydroxide] block the absorption of certain medication [e.g. thyroxin] so do not take ant-acids for two hours before or after the medicine.
Your doctor may prescribe medicine which blocks the formation of acid in your stomach.[eg. Nexium, Lanzor, Losec] Some acid [e.g. lactic, carboxylic or other acid] is formed from the old food sitting in your oesophagus and this medication will not work for that. - after a dilatation or myotomy you may have acid reflux (acid coming up) from your stomach - anti-acid or acid blocking medicines can reduce the amount of acid produced by the stomach but not that produced in the lower oesophagus by fermenting food. - a fundoplication (special wrap over the lower oesophageal sphincter is often done to prevent acid-reflux from happening when a myotomy is done. After a myotomy the sphincter cannot close as before and remains open)
What to ask your doctor:
What is achalasia and how did I get this condition?
What causes it ?
Can it be cured ?
Can I pass it on to my children ?
What treatment options are there?
Which treatment do you recommend for me?
Does medical management work? [taking pills]
How many successful dilatations have you done?
How long have you been doing dilatations?
Have you had any which ruptured? How many?
How many myotomies have you done?
How many of these were successful ?
How many needed further treatment [e.g. Dilatation]?
For how long have you been doing myotomies.?
Do you do a fundoplication ? How many of these are successful?
Why do you prefer doing fundoplication ? Do I have any choice?
Have you had any problems with these loosening or tightening?
If I have endoscopic surgery [using a laparoscope] how long will I be in hospital ? - how long will I be off work
If I have an open laparotomy how long will I be in hospital ? - how long will I be off work?
How soon after surgery can I start to eat normally?
For how long must I have liquids /eat soft food?
What are the costs ? Will my medical aid / insurance cover all the costs ?
Which hospitals does the surgeon work in?
Do you need pre-hospital approval?
Will I have to pay the surgeon / anaesthetist myself and then claim?
May I contact a few of your previous patients for discussion and support?
Your doctor should be willing to answer all these questions
Note: A gastroenterologist does the tests and a dilatation
- a surgeon does the surgery. There is a lot of information available on the Internet. Go to www://google.com.and type "achalasia" into the search box. You will get about 27 pages of articles about Achalasia.
There is also an Internet support group for people with Achalasia. http://www.groups.yahoo.com/group/achalasia/ Please join this group where you will meet others like yourself. You will get plenty of support and good advice from others who have been dealing with the problems for a long time. The Website has lots of information and links to other sources of information.
This information is intended for general information and reference for patients and their care givers. It is not a substitute for specialist professional medical assessment, advice, and diagnosis. It contains information from the experiences of other persons who have achalasia and this varies greatly. Any hints taken from this pamphlet are taken at your own risk. Always seek the advice of your doctor.
References and recommended reading:
1. Achalasia. In The Merck Manual (Seventeenth Edition.,1999); Beers M H, Berkow R,(Editors) Gastrointestinal
Disorders; Motor Disorders, chapter 20, page 230. Merck Research Laboratories, Whitehouse Station, N.J
The references below are from the Internet:
2. DeBackey M: Achalasia: Department of Surgery, Baylor College of Medicine. Houston.2003
3. Digestive Disease Centre – Public Information Site, Medical University of South Carolina: Digestive Problems – Oesophagus o Achalasia.
6. Achalasia: Harvard Medical School, Patient information. 2003.
7. Vaezi M.F, Richter JE,: Diagnosis and management of Achalasia. Practice Guidelines American College of Gastroenterology, Centre for swallowing and oesophageal Disorders, Department of Gastroenterology, The Cleveland Clinic, Cleveland Ohio.1999.American Journal of Gastroenterology Vol.94, No.12, Pages 3406-3412.
8. Oelschlager B, Pellegrini C.A: Surgical management of Achalasia; University of Washington School of Medicine. Medscape General Medicine 5 (4),2003/
9. Ferguson M: Achalasia and Oesophageal Motility Disorders. Society of Thoracic Surgeons Patient Information.
10. Maish M S, DeMeester S. R :Laparoscopic Myotomy and Fundoplication for Achalasia. University of Southern Carolina. CTNET Experts Techniques.
11. Yashohan S. et.al Electrical Stimulation of the vagus nerve restores motility in an animal model of achalasia ;Journal of Gastrointestinal Surgery Vol.7 No 7. Pages 843-849, November 2003.
12. Urbach D, Hansen P. D, et.al.; A decision making analysis of the optimal initial approach to achalasia: laparoscopic Heller Myotomy with partial fundoplication, thorascopic Heller Myotomy, pneumatic dilatation, or botulinum toxin injection. Journal of Gastrointestinal Surgery Vol.5 No.2, Page 192-205 (March 2001).
13. Beileveldt K, Piush G, Anjana A: Abnormal Esophageal Motility, Esophageal Function: A Primer for Clinicians, Virtual Hospital .
14. Klaus Bielefeldt K, Heartburn and Achalasia ?, Case-based Learning in Gastroenterology and Hepatology: Esophageal Disorders, Virtual Hospital
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