Achalasia is a rare swallowing condition that prevents the food you swallow from reaching your stomach properly. It affects the muscles in your oesophagus, the food pipe that runs from your throat, down through your chest and to your stomach.
The muscles within the oesophagus that perform peristalsis are normally automatically controlled by a complex, interlinked system of nerve cells, or neurons. Some of these are inhibitory, some excitatory according to whether they suppress muscle activity or start it. Achalasia is related to the degeneration and malfunctioning of a specific type of nerve cell called ganglia that send signals to the brain to operate the muscles. The loss of function of these neurons impairs the normal motility (the passage of food through the digestive system) of the oesophagus and dilation of the LOS – resulting in an oesophagus that cannot channel food into the stomach properly.
The causes of the degeneration of these nerves and therefore of achalasia are not clear, and research into the causes of achalasia has so far been inconclusive. They are likely to be different for individuals.
It is believed that infections from the varicella zoster virus (linked with chicken pox and shingles) or a side effect from measles may be contributory factors.
With achalasia, the muscles in the oesophagus do not work properly. They are either weak, do not tighten and relax in the right order, or do not work at all. The LOS, located just above your stomach, often fails to open. Food and drink then block back into your oesophagus and can remain there for a long time, sometimes hours or days. This food build-up can create pressure, similar to a blocked hosepipe, until the food either trickles down into your stomach, or is brought back up through your mouth (regurgitation). This is not caused by a fault in the muscles themselves but seems to be a problem with the nerve endings that send signals to the brain to operate those muscles.
Achalasia is rare, and statistics are not precise. The number of people diagnosed each year (incidence) is similar in most countries, and is estimated as between 0.7 to 1.6 per 100,000 inhabitants per year.
The UK population of 66.8 million would therefore, in theory, generate between 467-1,069 diagnoses annually. The prevalence (number of people continuing to be affected by the condition after diagnosis) is regarded internationally as about 10 in 100,000 and is estimated by the NHS to be around 6,000 in the UK. The latest high-resolution manometry techniques may be contributing to more cases being diagnosed.
It is most common between the ages of 30 and 60 years, but it can develop at any age. Some young children are diagnosed with achalasia, and, rarely, it can be present from birth. A study found only 228 UK children under 16 years diagnosed with achalasia in a 10-year period until 2008 but with a rise in the annual rate within that decade.
Achalasia is sometimes described as an auto-immune disease, meaning that the body’s immune system may have started to react differently. There may be occasional cases of achalasia that may be associated with auto-immune disease, or occur in patients who already have auto-immune diseases.
As far as we understand it, this is not of the scale of some other conditions which are actually autoimmune in aetiology (how they are caused), and where, for instance, treatment would entail prescribing immune-suppressant drugs or steroids. Furthermore this does not normally mean that if you have achalasia your immunity from general infections is affected.
Because achalasia is rare, it is not likely that your GP will have had much experience at dealing with the condition. Swallowing problems can have a number of different causes. Swallowing can be affected by stress and tension so it is important to distinguish between general tension that causes swallowing problems rather than a condition like achalasia that may be an underlying cause of some anxiety and stress around the process of eating food. Having achalasia, when a person wants to eat and swallow normally but the food sticks in the oesophagus, is different from an eating disorder like anorexia.
A diagnosis of achalasia depends on a series of tests normally given by a gastroenterologist in a specialist Upper GI (Upper Gastrointestional tract) clinic in a hospital.
Achalasia is a long-term condition that cannot be cured at present, but there are treatments that can improve the symptoms. Treatments can range from lifestyle changes to surgery. Doctors should look at the patient as a whole, and take into account their overall physical health rather than being solely governed by test results.
See Treatment options here
As with many medical conditions, treatment will tend to be better where there is a specialist centre with a higher volume of relevant cases. There are specialist Upper GI treatment centres within the UK, and some general district hospitals where surgeons have a special interest in achalasia.
Most diagnoses are undertaken by gastroenterologists and physiologists who use manometry testing. Dilatation and sometimes POEM procedures can be undertaken by gastroenterologists; the best centres involve collaboration between gastroenterologists, surgeons and other practitioners so as to enable to the best treatment to be given for the individual patient.
People with achalasia may need to take medication for other reasons, and if so may need to take extra care because pills may simply remain in your oesophagus rather than reaching your stomach and then being absorbed into your body through your digestive system. The chemicals from the pills might damage your oesophagus which is different in nature from your stomach lining.
Many medications can be given in liquid or powder form. Your pharmacist can advise you about this, and your doctor might need to specify this on the prescription.
If you need to take medication in tablet or capsule form, take them, subject to the medication information leaflet, before meals with plenty of water to improve the chances of them passing into your stomach.
Some people do not need to take medication related to achalasia, but information is included below about some associated medication that is not directly aimed at achalasia itself, but may be given to reduce the symptoms in various ways, eg to reduce reflux, or to relax certain groups of muscles to combat spasms.
For severe cases of pain, you should beware of liquid opiates because of the addiction and constipation issues, and should seek specialist intervention to diagnose the cause of the pain.
As always, you should seek medical advice before taking medication, not least because any medication may have unwanted side effects. Some of the medication mentioned on this website may need to be prescribed by a specialist, but might then be able to be continued by a GP.
Oesophageal Candida is not a direct symptom but can be a fairly commonassociated problem. This is due to food being held up in the oesophagus and as with any similar condition that causes stasis can lead to immuno-suppression and damage to the mucosa because of the fermentation of food, plus if you are a bit under nourished, run down or have been on antibiotics due to aspiration pneumonia – all these things can lead to a risk of candida. It’s easy to treat (though the side effects of the antifungal drugs need to be monitored) but hard to diagnose without an endoscopy ( sometimes possible to get a clinical diagnosis based on symptoms and mouth swabs).
Further research is planned into causes and potential treatments of achalasia including Medical Treatment of Spasms: (Development from our earlier spasms survey, with Stefan Antonowicz, Imperial College)
Most important thing with spasms is to make sure there’s not an obstruction, e.g. failed myotomy, scar tissue, or is it painful reflux – e.g. following a POEM. If not those two things then consider medical treatment for spasms. If spasms are especially at night this suggests reflux.
Achalasia Action have produced a lot of advice on how to eat better to avoid or manage spasms and what might help when you have one – see Living with Achalasia booklet, available on the website. It has been suggested that cannabis/ CBD might help but no research on this to date.
The nutritional content is the same but liquidising means that fibre is lost – if constipation becomes a problem then add a fibre supplement to the smoothie. Soluble v insoluble fibre – this depends on the type of bonds making up carbs – the bonds are are stronger in insoluble fibre so help with roughage in poo whilst soluble fibres are easier to digest: this is something to consider i.e. which type of fibre is more suitable for your digestive problems.
POEM could leave children open to having reflux for life. It would be better to have a myotomy with a wrap in the first place and to avoid having to have a follow up wrap to deal with the reflux. Endoscopic procedures are not necessarily any less invasive than keyhole surgery.
Surgeons do not generally treat children under 16 unless they have undertaken additional paediatric training and have a licence so usually there needs to be a collaboration between a paediatric surgeon and a surgeon specialising in achalasia. Whilst it is advisable to wait until 16 it can be a problem if the child has lots of dilations in the meantime.
See how Achalasia can be treated
