Achalasia – what you need to know but were afraid to ask!

Achalasia is a disorder of the gullet (oesophagus) where it loses the ability to move food along. The valve at the end of the gullet also fails to open and allow food to pass into your stomach.

Achalasia

Achalasia

As a result, food gets stuck in your gullet and is often brought back up.

A ring of muscle called the lower oesophageal (cardiac) sphincter keeps the opening from the gullet to the stomach shut tight to prevent acid reflux (acidic stomach content moving back up into the gullet).

Normally, this muscle relaxes when you swallow to allow the food to pass into your stomach. In achalasia, this muscle does not relax properly and the end of your gullet becomes blocked with food.

Achalasia is an uncommon condition that affects about 6,000 people in Britain. It is sometimes known as cardiospasm.

What are the symptoms?

Symptoms of achalasia may start at any time of life and usually come on gradually.

Most people with achalasia have dysphagia, a condition where they find it difficult and sometimes painful to swallow food. This tends to get worse over a couple of years.


It may cause you to bring back up undigested food shortly after meals and some of the vomited food may have been held up in your gullet for some time.

Bringing up undigested food can lead to choking and coughing fits, chest pain and heartburn.

Occasionally, vomit may dribble out of your mouth and stain the pillow during the night. If it trickles down your windpipe, it can cause repeated chest infections and even pneumonia.

You may experience gradual but significant weight loss.

However, in some people achalasia causes no symptoms and is only discovered when a chest X-ray or other investigation is performed for another reason.

What is the cause?

Achalasia is caused by damage to and loss of the nerves in the gullet wall. The reason for this is unknown, although a viral infection earlier in life may be partly responsible.

Achalasia may also be associated with having an autoimmune condition, where the immune system attacks healthy cells, tissue and organs. One recent study found people with achalasia are significantly more likely to have an autoimmune condition such as Sjogren’s syndrome, lupus or uveitis.

Although achalasia can occur at any age, it is more common in middle-aged or older adults.

There is no evidence to suggest that achalasia is an inherited illness. Women with achalasia can have a normal pregnancy and there’s no reason why their children will not develop normally.

How is it diagnosed?

If your GP thinks you have achalasia, you will be referred to hospital to have some diagnostic tests performed.

Barium swallow

A barium swallow involves drinking a white liquid containing the chemical barium, which allows the gullet to be seen and videoed on an X-ray.

In achalasia, the exit at the lower end of your gullet never opens properly, which causes a delay in barium passing into your stomach.

An ordinary chest X-ray may show a wide gullet.

Endoscopy

A flexible instrument called an endoscope is passed down your throat to allow the doctor to look directly at the lining of your gullet and stomach. Trapped food will be visible.

The endoscope can be passed through the tight muscle at the bottom of your gullet and into your stomach to check there is no other disorder of the stomach.

Read more about having an endoscopy.

Manometry

Manometry measures pressure waves in your gullet. A small plastic tube is passed into your gullet through your mouth or nose and the pressure at different points in your gullet is measured.

In achalasia, there are usually weak or absent contractions of the gullet and sustained high pressure in the muscle at the lower end of the gullet. The high pressure means the muscle does not relax in response to swallowing, causing symptoms of achalasia.

How is it treated?

The aim of treatment is to open the lower oesophageal sphincter muscle so food can pass into the stomach easily. The underlying disease cannot be cured but there are various ways to relieve symptoms which can improve swallowing and eating.

Dilatation (stretching the muscle)

A balloon (about 3-4cm in diameter) is used to stretch and disrupt the muscle fibres of the sphincter muscle at the lower end of your gullet.

This usually improves swallowing but may need to be performed several times or repeated after one or more years. Balloon dilatation does carry the risk of oesophageal rupture which may require emergency surgery.

Surgery

Under general anaesthetic the gullet is accessed through the abdomen (tummy) or, rarely, the chest. The muscle fibres of the lower oesophageal sphincter that fail to relax are divided. This usually leads to a permanent improvement in swallowing.

The operation is now performed by keyhole surgery (laparoscopy) and only requires an overnight stay in hospital.

Recovering from treatment

There are a few things you can do after dilatation or surgery to reduce symptoms:

  • chew your food well
  • take your time eating
  • drink plenty of fluids with your meals
  • always eat food sitting upright
  • use several pillows or raise the head of your bed so that you sleep fairly upright, which prevents stomach acid rising into your gullet through the weakened valve and causing heartburn

If heartburn develops after treatment, consult your GP as medication may be needed to reduce the acid reflux. Sometimes your surgeon may suggest you take this routinely to prevent problems after surgery. Read about treatments for acid reflux.

You should also see your GP if you still have swallowing difficulties or are continuing to lose weight.

It’s normal for chest pain to persist for a while after treatment – drinking cold water often gives relief.

Cancer risk

If the gullet contains a large amount of food that does not pass into the stomach in the normal way, the risk of cancer of the oesophagus (gullet) is slightly increased.

The increased risk is likely to be most significant in long-term untreated achalasia. It’s therefore important to get appropriate treatment for achalasia straight away, even if your symptoms are not bothering you.

According to Cancer Research UK, compared with the general population:

  • men with achalasia have an eight to 16 times higher risk of oesophageal cancer
  • women with achalasia have a 20 times higher risk of one particular type of oesophageal cancer (adenocarcinoma)

However, cancer of the oesophagus is very uncommon and although your risk is slightly increased, it remains highly unlikely.

Cancer Research. Prof Dean Fennell, leading cancer researcher, gets us up to date with the latest cancer and Mesothelioma research


Prof Dean Fennell

Prof Dean Fennell

We are very proud to publish our latest interview with a  leading clinician.  Today we are interviewing  Prof Dean Fennell one of the world’s leading cancer researchers.   Prof Dean Fennell is currently Chair of Thoracic Medical Oncology at the University of Leicester. Top find out more about Prof Fennel and his work please go to his web page – http://www2.le.ac.uk/departments/csmm/people/professor-dean-fennell

In this interview Prof Fennell tells us more about his current research into Mesothelioma.  You can read our previous article on the subject here https://patienttalk.org/calling-the-cancer-community-new-study-harnessing-ground-breaking-science-offers-hope-to-mesothelioma-patients/

PATIENTTALK.ORG What is Mesothelioma and how does it differ from other types of lung cancer?

PROF FENNELL Well Mesothelioma is a cancer that is unique and it is unique because it is one that we know is caused by a specific substance.  Just in the same way that people would associate tobacco with the majority of lung cancers.  Mesothelioma is almost invariably associated with the substance asbestos.  This is a mineral which was used for its phenomenal insulating properties dating back as far as the Romans actually but the problem has been that with the use of asbestos in the 20th Century those individuals that have come into contact with it have been exposed to essentially a carcinogen that causes this disease and it take around thirty years to form.  I suppose the main thing that defines it in terms of how it behaves compared with lung cancer is that this is not a cancer that flies around the body and spreads rapidly to distant parts of the body.  It tends to stay within the chest.  It tends to create a straightjacketing effect around the lung and really the symptoms of this disease are related in part to the fact that this is confined to the chest rather than spreading to say the bone or the brain or other parts of the body.

PATIENTTALK.ORG How common is it?

PROF FENNELL I think this is a problem that we are facing with Mesothelioma.  We are in the wake of an epidemic.  The epidemic is not just a national emergency it’s part of a worldwide problem that we are facing.  Particularly in Europe and this because of the timing I think of the exposure.  The maximum exposure was seen in the order of around thirty or so years ago and when just now we are able to see a dramatic rise in the incidence.  So we see around 200,000 patients a year at the moment.  We think the rate is going to increase over the next decade it may extend beyond that but certainly up until the middle of the 21st Century we anticipate that the numbers will be much higher than historically seen, certainly before the seventies.

PATIENTTALK.ORG What is the typical treatment for Mesothelioma and the typical prognosis?

PROF FENNELL Well there is only one, what we can define as NICE approved standard of care for Mesothelioma.  About ten years ago a trial was conducted and the result of that trial is today’s standard therapy.  This is combination chemotherapy.  Patients will receive usually four to six cycles of that chemotherapy and following that chemotherapy that’s really it as far as the HS goes for many centres because there is no standard of care in the second line we are really guessing at what treatments to offer patients and this is really why we need major advances in terms of new drug based treatments for this cancer.

PATIENTTALK.ORG Can you explain what exactly is a clinical trial?  What the objectives of this study are and the risks involved?

PROF FENNELL In general a clinical trial is the formalised assessment of a new agent and the assessment is very often conducted particularly when wanting to establish how effective the drug is by randomising the drug compared to either the existing standard of care or if there isn’t one to a placebo which matches the experimental medication almost exactly.  So it is impossible for either the investigator or for the patient to know which is which.  It’s only when the analysis of the trial is done that one can work out, de-convolute the data and establish whether the drug was superior to nothing at all.  This is a very important mechanism for validating new drugs and what is considered to be the best standard of care but clinical trials are changing and particularly because our understanding of the cancer is changing dramatically we are now looking at the possibility or certain drugs and we have seen examples of this in lung cancer, examples of drugs that are so effective that a randomised trial simply isn’t necessary.  There is more than enough historical data out there to tell us that a new drug may be far superior to existing standards and therefore eastern US, this is not the case in Europe at the moment but within the US it was possible recently with lung cancer to approve a trial on a very, very small number of patients who were treated with very dramatic effects.

PATIENTTALK.ORG And what is the objective of this particular study?

PROF FENNELL Aside from chemotherapy, maybe four to six treatments of the standard chemotherapy, is the standard of care and when you stop the treatment the cancer comes back.  So the main purpose of this trial is to evaluate a new type of approach for treating Mesothelioma. It’s sort of a two hit approach­.  You have the chemotherapy first of all that gets initial control of the cancer and then you stop the cell that normally causes the cancer coming back, these are the cancer stem cells, you stop them from growing by using a specific stem cell targetting drug and one of the drugs that we are looking at here seems to have that property, that it can target stem cells. In the process of doing this, what you’re doing is targeting a minority of cells within the cancer with low toxicity. As a consequence this drug can be administered and administered until such time as the drug has to stop because the cancer’s grown again but the main objective is to try and see if the cancer can be suppressed for longer. Where this type of approach that’s been looked at in other cancer’s such as lung cancer the results have been very impressive. This has led to a change in practice where maintenance therapy this continuation second hit as it were has been shown to extend benefit for patients.

PATIENTTALK.ORG What would be the risks involved?


PROF FENNELL Well I think risk of any drug trial must always be balanced against the benefits. The risks by and large are always associated with side effects.  Of course many people will be aware that chemotherapy has an associated well recognise constellation of side effects, nausea for example or other unpleasant side effects.  We can do a lot to control those sufficient that patients even in some cases can get back to work or travel that sort of thing whilst they are on chemotherapy.  With a new drug, I think the trend that we are seeing is a very encouraging one that a lot of the new agents seem to be much less toxic, much more tolerable and orally available.  They can be taken as a tablet and as an injection.  So I think the first and most important risk for a patient is what will the drug do to me in terms of harm, in terms of side effects and I think with the drugs that we are looking at in this particular trial I think that’s actually a relatively low risk.  The other risk of course do I get the drug and if I don’t what happens?  This is an inherent part of any randomised study and certainly something patients will always need to consider when entering a randomised controlled trial.

PATIENTTALK.ORG You mentioned the risks are quite low with this one but how are patients, participants protected from harm?

PROF FENNELL With clinical trials of any sort now because of the very significant legislation that’s based around what was termed the EU directive of the trials there is an extremely vigilant monitoring process that takes place to look for side effects, to look for any adverse effects that could arise very early on in the study.  What I think is extremely important is that a drug which is the one that we are evaluating for Mesothelioma or any drug that is going forward into what we would describe as a more advanced trial, randomised trial has been explored extensively in safety testing and so we have a fair understanding of the tolerance of this drug and the safety of the drug before we even enter a trial like this.  Nevertheless as you expand the number of patients who receive the drug there are potentially rare side effects that you are to look for and so the pharmacovigilance process is a very efficient one at detecting and reporting these.

PATIENTTALK.ORG And why should people participate in this study?

PROF FENNELL Well I think the first thing I have to say actually is that I don’t think any patient must participate just to use that term. All clinical trials, all forms of clinical research are really there I think to offer patients an option and the option is whether or not to receive the drug that may or may not, I say may not have an additional clinical advantage over and above the standard of care. Many patients will consider this very carefully and based on what we have mentioned which is the toxicity vs safely issue, the possibility and rational behind whether this is a drug that could work. I think patients will make that choice and patients are always very often given time to make these decisions. What I think is really critical for patients is that they need to be made aware that these options exist in the first place and so providing information about clinical trials – whether it is the one that we are currently involved with or other trials which are ongoing in the UK. I think patients need to know that these exist so that they can get access to what could be life enhancing treatment.

PATIENTTALK.ORG What are the exact criteria you require for the study?

PROF FENNELL Well patients who are being treated with chemotherapy who are in the process of being treated with chemotherapy and who are benefitting from it. What I mean by that is there are maybe a fifth of patients who will have the chemotherapy in whom the cancer is programmed not to respond. These are primary chemo-resistant cancers. Patients who have chemo-sensitive tumours actually have either a response or shrinkage of the cancer or a stable disease. These are the patients who would be eligible for any for any form of maintenance treatment such as the one we are exploring currently. So if a patient is on treatment and feels that things are going well and their doctor feels that things are going well, this is definitely a trial to at least consider.

PATIENTTALK.ORG And what would a participant need to do to take part?

PROF FENNELL I think we are doing our best to communicate with all doctors throughout the country who treat Mesothelioma so that they are aware of the option to enter a maintenance therapy trial but patients should be empowered with the knowledge that these trials exist and if they are keen to know more, certainly find out more. And that can be done through the information resources that are present through Cancer Research UK and in particular Mesothelioma UK. There are people that one can speak to actually through Mesothelioma UK who are more than happy to provide information about for example where the trial is taking place locally.

PATIENTTALK.ORG What one piece of advice would you give to somebody who has been diagnosed with Mesothelioma?

PROF FENNELL I think that this is a devastating diagnosis to receive and although the initial news the patient may receive may be extremely nihilistic; I think it is important to be aware that we do have new trials which are coming, new trials which are presently active.  It is quite clear I think, and the Government supports this notion, that clinical trials associate with better outcomes for patients whether it is through closer monitoring or the actual implementation of new agents and as such patients should not feel that they are not entitled to get access to these. It is something that a patient should really enquire about and that it something that can only be for the greater good. If these trials are positive then the faster we can enrol them, the faster we can get the data, the faster we can get the results and if positive hopefully make these medicines for everybody.

PATIENTTALK.ORG So what is the typical prognosis?

PROF FENNELL Patients are often told that survival following diagnosis of Mesothelioma is about a year. Now that of course will vary between many patients – some may have a much better prognosis than that. We are very interested in trying to understand the genetics behind those sub groups of Mesotheliomas – some can be really quite explosive and grow very very quickly indeed and those are associated with prognosis maybe in the order of several months, maybe even weeks.

Calling the cancer community – New study, harnessing ground-breaking science, offers hope to mesothelioma patients.


  • Study will evaluate a new drug that suppresses cancer stem cells, a ground-breaking new
    Mesothelioma and cancer research

    Mesothelioma and cancer research

    science, as potential new treatment for pleural mesothelioma; an aggressive cancer that affects the lining of the lungs, and is strongly linked to asbestos exposure

  • In the UK, the number of deaths caused by the disease each year is growing and according to latest figures it is the most rapidly increasing cancer amongst women in the UK (3rd most for men)[i],[ii]
  • Despite the UK’s control on asbestos in 1985, the number of mesothelioma patients is set to rise sharply over the coming years as mesothelioma can take between 30-40 years from exposure to asbestos to develop[iii]
  • Mesothelioma is a highly aggressive disease, and even on currently approved treatment, the average survival time is only 12 months[iv]
  • There is no cure; the only approved chemotherapy regimen prolonged life by 2.8 months from 9.1 to 11.9 months[v]
  • Most at risk include builders, construction workers, railway workers, plumbers and gas fitters, dock workers,  electricians, roofers, painters and decorators and other tradespeople, along with their spouses[vi]
  • Lord Saatchi will be presenting his medical innovation bill at a public consultation on Monday 24th at the House of Lords. The bill is designed to enable doctors to innovate new treatments and cures for cancer and other diseases.
  • Should the bill be passed, its aim is to facilitate patients access to innovative treatments and clinical trials , like this one in cancer stem cell suppression.
  • On the International Rare Diseases Day , Friday (28th February). We want to focus the spotlight on the new developments in treatment options for patients fighting mesothelioma . Shifting the attention from the litigation around asbestos to raising awareness of the ongoing and continued research required for this disease.

Pleural mesothelioma, is an aggressive form of lung cancer strongly linked to asbestos exposure, which has very limited treatment options and is associated with poor patient survival, with most having less than a year left to live once diagnosed.iv

Eight leading UK cancer research centres are participating in a ground-breaking new study to evaluate a potential new treatment targeting cancer stem cells, and measure if the life expectancy of these patients could be prolonged. Cancer stem cells are often resistant to conventional chemotherapy, By targeting the cancer stem cells, it is hoped that the growth of the mesothelioma can be slowed or stopped, and a new treatment option for patients could be established. (Trials are taking place at Leicester Royal Infirmary, Addenbrooke’s Hospital, Cambridge, Velindre Hospital, Cardiff, Broomfield Hospital, Chelmsford, Kings College, London and St. Batholomew’s Hospital, London, Wythenshaw Hospital, Manchester and Southampton General Hospital.)

Despite the UK’s control on asbestos in 1985, the number of mesothelioma patients is set to continue to rise sharply as mesothelioma can take between 30-40 years from exposure to asbestos to develop. In the UK, the number of deaths caused by the disease each year has grown to 2,543 in 2010 and it is the most rapidly increasing cancer amongst women in the UK (3rd most for men)[vii],ii

It is estimated that more than 9 out of 10 men and more than 8 out of 10 women with mesothelioma have been in contact with asbestos.vii,[viii]

Most at risk of developing the disease include builders, construction workers, railway workers, plumbers and gas fitters, dock workers, electricians, roofers, painters and decorators amongst other tradespeople and workers who have had exposure to asbestos.vi As the data on the increasing incidence in women show, the demographics are changing as asbestos is found in many places and secondary exposure is considered to be a great risk moving forward.

 

For patients who are diagnosed with mesothelioma, there are very limited treatment options beyond the one chemotherapy approved. By targeting cancer stems cells it is hoped that it will be possible to prolong patient survival and improve quality of life.



[ii] GLOBAL PANORAMA ON MESOTHELIOMA 2009. Laurie Kazan-Allen. Presented at Mesothelioma Symposium Chamber of Deputies. São Paulo, Brazil, November 2009

[iii] Health and Safety Executive (HSE). Mesothelioma in Great Britain 2013. http://www.hse.gov.uk/STATISTICS/causdis/mesothelioma/mesothelioma.pdf

[iv] Cancer Research UK. Statistics and Outlook: http://www.cancerresearchuk.org/cancer-help/type/mesothelioma/treatment/statistics-and-outlook-for-mesothelioma#gen Accessed 08 November 2013

[v] Vogelsang, NJ et al., Phase III study of pemetrexed in combination with cisplatin versus cisplatin alone in patients with malignant pleural mesothelioma. J Clin Oncol. 2003 Jul 15;21(14):2636-44.

[vi]http://www.mesothelioma.uk.com/editorimages/Factsheets/Concerned%20about%20Asbestos%20Exposure%20June%202010%20downloadable.pdf

[vii] Cancer Research UK. Mesothelioma Risks and Causes. http://www.cancerresearchuk.org/cancer-help/type/mesothelioma/about/mesothelioma-risks-and-causes#how   Accessed 27 November 2013

[viii] The Asbestos Center: http://www.asbestos.com/mesothelioma/uk/  Accessed 27 November 2013

 


(An estimated 350-400patients from eleven countries worldwide will participate in the study. In the UK, participants are currently being recruited at eight research centres:

  • Leicester Royal Infirmary, Leicester
  • Addenbrooke’s Hospital, Cambridge
  • Velindre Hospital, Cardiff
  • Broomfield Hospital, Chelmsford
  • King’s College, London
  • St. Bartholomew’s Hospital, London
  • Wythenshaw Hospital, Manchester
  • Southampton General Hospital, Southampton

About Malignant Pleural Mesothelioma

Malignant pleural mesothelioma is an aggressive form of cancer that occurs in the mesothelium, the thin layer of tissue that covers the lungs. Since the 1960s, it has been known that the disease can be triggered by the inhalation of asbestos fibres; an insulating material which was used widely in the building and manufacturing industries up until 1985 when the UK issued a ban on asbestos.vii It is estimated that in the UK more than 9 out of 10 men and more than 8 out of 10 women with mesothelioma have been in contact with asbestos and the number of deaths caused by the disease each year has grown to 2,543 in 2010 and is one of the highest increasing rates of incidence in the world.vii,viii This number is set to continue to rise sharply over the next 20 years, with a peak coming in 2020.vii According to the World Health Organization, a total of 59,000 deaths occur worldwide each year due to mesothelioma. Most mesotheliomas begin as one or more nodules that progressively grow to form a solid coating of tumour surrounding the lung leading to eventual suffocation and death. A high percentage of mesotheliomas contain cancer stem cells which are generally resistant to the currently available treatment options for advanced mesothelioma.

 

Most at risk professions:

  • Carpenters and joiners
  • Boiler, pipe and heating workers
  • Shipbuilding/shipyard workers
  • Navy seamen
  • Dockworkers
  • Laggers and sprayers
  • Mechanics
  • Plumbers and gasfitters
  • Roofers
  • Construction and demolition workers
  • Painters and decorators
  • Electricians
  • Railway workers and coach construction
  • Engineers
  • Power station workers
  • Laboratory and research
  • Asbestos manufacture and sales
  • And the spouses of the at risk professions ( from exposure to their work wear when washing the clothing)

WEBLINKS                  

www.mesothelioma.uk.com

www.cancerresearchuk.org