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. 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 doctor/ 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.
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.
Medication
The muscle at the lower end of your gullet may
be temporarily relaxed by medication. Tablets such as nitrates or nifedipine
can sometimes produce a brief improvement in swallowing but are not effective
in all patients. They can be helpful while a more permanent treatment is
planned. These tablets may cause headaches, but this
usually improves with continued treatment.
Dilatation (stretching the muscle)
This is done under a sedative or general anaesthetic (where you are put
to sleep). 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.
Botulinum toxin (Botox injection)
Botox causes relaxation of the muscle fibres. It
can be injected painlessly into the lower oesophageal sphincter muscle through
an endoscope. This is usually effective for a few months and
occasionally for a few years, but has to be repeated. Botox can be used
for temporary relief in people who are not able to have other treatments.
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 doctor/ 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. 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.
Source: NHS Choices