Achalasia Symptoms and Treatment
Achalasia is a rare chronic disease of the esophagus.People who are affected suffer primarily from difficulty in swallowing. Other typical achalasia symptoms are belching of undigested chyme from the esophagus, pain behind the breastbone, weight loss and bad breath.
What is Achalasia ?
Achalasia is a disease of the gullet (esophagus), in which the persons concerned have difficulty in swallowing. Contraction movements of the esophagus are usually exactly synchronized with the timing of the opening of the lower sphincter.It ends at the lower end then relaxes at the right time of the sphincter and the food can reach the stomach.
In achalasia esophagus does not precisely matche to the lower sphincter contraction movements (peristalsis). Secondly, the sphincter is permanently tense and it can not expand sufficiently.
As a consequence of the chyme is not normally transported by the disturbed peristalsis down the esophagus. It also permanently builds up in front of the strained lower esophageal sphincter and causes the typical symptoms of achalasia occur. These mainly include; difficulty swallowing of solid food (dysphagia) and regurgitation of undigested food particles from the esophagus into the oropharynx.
Who is Affected from Achalasia?
Achalasia is a rare disease. Annually one in 100,000 people suffer achalasia. Most affected people who are in middle age( between age of 30 and 50 years). But sometimes it can be in children, adolescents or in elderly people. If an achalasia in childhood, a genetic cause is called ‘’ Triple A syndrome’’ is often responsible.
Primary and Secondary Achalasia
Doctors distinguish the disease as primary and secondary achalasia. The primary achalasia is the most common form.There can be no clear causes of primary achalasia .
In the rare secondary achalasia , there are clear causes such as esophageal cancer or Chagas disease. These diseases damage the nerves in the region of the esophagus and this leads to normal function of the esophageal contractions and of the esophagus sphincter is impaired. It is typical of the secondary achalasia that dysphagia compared to primary achalasia grow much faster. often affected by the secondary achalasia elderly.
The typical achalasia symptoms are difficulty swallowing (dysphagia) and regurgitation of undigested food (regurgitation). There are also other symptoms such as pain behind the breastbone, weight loss and bad breath.
- Achalasia symptoms – Difficulty swallowing ; Initially, the symptoms are usually only mild and occur only sporadically. Patients who in the early phase of the disease feel trouble to swallow solid food. So they feel as foods and drinks get stuck in the throat during swallowing. An increased fluid intake causes the food better can reach the stomach. In addition, some sufferers experience a feeling of pressure in the chest area behind the breastbone. In more advanced stages and liquids can not be swallowed, or can be swallowed only with difficulty.This situation provides very significant problems to people who are concerned. Firstly, the swallowing disorder is psychologically distressing and it leads to reduce their physical performance.
- Achalasia symptoms – Regurgitation of undigested food particles ; In the advanced stage achalasia results in unintentional regurgitation. It often arrives undigested food particles from the esophagus back into the oral cavity. The spontaneous regurgitation of undigested food particles occurs most frequently when patient is lying down. Some patients suffer a feeling of fullness and also need to vomit.
Many patients swallow at up exhausted undigested food as it passes into the trachea (aspiration). This happens especially at night when the patient lie. The reflux of food can lead to nighttime coughing. In addition, the leftovers can cause pneumonia in the trachea and the bronchi (Aspiration).
Other Symptoms of Achalasia
The decrease in body weight is slow in primary achalasia over months or years and usually is not more than ten percent of the original body weight. In secondary achalasia , weight loss may be more pronounced and also progress in a much shorter period.
Some patients also have severe pain behind the sternum (retrosternal pain) that especially occur during swallowing because of their achalasia. If pain is very strong in foreground, the doctors call this as “hypermotile achalasia”.
Achalasia Causes and Risk Factors
The act of swallowing is a complex, finely tuned process, which requires a precise timing tuned control of the muscles of the esophagus by nerve impulses. The causes of primary achalasia are not yet fully understood. There is evidence that achalasia is caused by a destruction of certain nerves and nerve centers (ganglion cells) produced in the region of the esophagus.
In particular,it is called myenteric (Auerbach plexus) which seems to be affected. This is a fine network of nerves in the muscular wall of the esophagus, stomach and intestines.
Science has a more specific idea why nerve cells die: Typical causes of secondary achalasia are esophageal cancer (especially the cardia) and Chagas disease. In both diseases the nerve cells are damaged in the wall of the esophagus. Chagas disease is a parasitic disease transmitted by bed bugs, which occurs mainly in Central and South America .
Achalasia also can be inherited in some cases ;
If children and young people are affected by achalasia, a genetic cause is often responsible.
- Triple-A syndrome (AAA syndrome)
- Adrenal insufficiency
- Down syndrome
- Familial visceral neuropathy
- Microcephaly syndrome
Achalasia Investigation and Diagnosis
You provide the physician already valuable information about your current state of health (medical history) and a detailed description of your symptoms.
Attending physician could ask you the following questions:
- Do you have difficulty swallowing, such as the feeling that you get stuck food in your throat?
- Does this feeling increase when you drink plenty of liquid?
- Do you occasionally need to undigested food residues burp?
- Does it hurt when you swallow?
- Have you lost weight?
- Do you have halitosis?
Then the doctor will particularly examine your abdomen and the upper body and light in the mouth and throat to see possible changes. Doctor will also scan your neck.Doctor will check enlarged lymph nodes in the neck and also check your thyroid scan.
Supplementary examinations of suspected achalasia
A achalasia can often be diagnosed by typical symptoms in combination with imaging techniques such as Esophageal Reflection. Optionally, it can also be checked with the oesophageal manometry to see the function of the lower esophageal sphincter.
Esophagoscopy and Gastroscopy
Reflection on an endoscope can judge the mucosa structure in the esophagus and stomach. In addition, the mirroring is the exclusion of other diseases of the esophagus and stomach, such as inflammation, scarring or cancer. The patient must not eat and drink six hours before the examination, so that the doctor has a clear view on the mucous membranes during the investigation.
A achalasia treatment is necessary if symptoms are caused by the disorder.There are different options avaliable to alleviate the achalasia symptoms. Use of drugs or specific interventions can usually help to decrease symptoms. The goal of therapy is to reduce the increased pressure of the lower esophageal sphincter. However, a complete cure is hardly possible, because once nerve cellsare damaged it is limited to regenerate .
Achalasia – Drugs
Drug therapy helps only about ten percent of patients. The drug nifedipine – originally a drug for the treatment of high blood pressure, ensures a relaxation of the esophageal sphincter. The active substance group of nitrates has a similar effect. The drugs are taken about 30 minutes before food intake. Thus, the lower esophageal sphincter relaxes in time and the food can easily reach the stomach.
Achalasia – Specific interventions
The narrowed junction between the esophagus and stomach can be widen by various methods.Balloon method is one of choice. Young patients with achalasia is an exception , where surgery is usually better. An injection with botulinum toxin directly into the lower esophagus sphincter is also often carried out. Surgical expansion of the transition between the esophagus and stomach is performed in only a few cases.
Balloon Expansion (balloon)
The narrowing of the transition from the esophagus and stomach can be stretched with the aid of a balloon. The balloon can be done during a gastroscopy, so it is no surgery needed. The doctor pushes a thin tube through the mouth into the esophagus prior to the constriction (stenosis) at the entrance to the stomach which small balloon inflates at the end of the hose. In rare cases (about five percent), in the engagement complications the esophagus or cardia tear. Penetrating bacteria into the wound, an inflammation of the esophagus may occur. Furthermore, the balloon needs to be repeated in about half of the cases after a few years.
The injection of diluted botulinum toxin (Botox) into the narrowed esophageal sphincter can be carried out in during a gastroscopy. Most people know Botox as a nerve paralyzing poison of aesthetic medicine. In esophageal sphincter that blocks the nerves, whereupon the sphincter relaxes. This type of achalasia therapy improves symptoms in 90 percent of those affected. However, many patients develop again achalasia symptoms after a few months later.
If the patient can not be sufficiently helped with the above measures, also an operation could be used. This is useful especially in young patients, because in this age group, the balloon often works poorly
Achalasia: Clinical course and Prognosis
If there is chronic achalasia d isease , it doesnt recover with a spontaneous way. The typical problem of achalasia’’ swallowing ‘’takes usually over years or even decades. However, With the help of different treatment options symptoms can usually be relieved sufficiently.
Complications of Achalasia
Untreated an achalasia can lead to increasing expansion (dilatation) of the esophagus. In extreme cases, a so-called megaesophagus is formed which this is no longer able to transport the chyme from the mouth to the stomach. The increased regurgitation can trigger later in an inflammation of the esophagus (esophagitis) or complications of the lungs (cough, hoarseness up to pneumonia).
The achalasia is associated with an increased risk of esophageal cancer (esophageal cancer). The risk of achalasia patients are falling ill with a malignant tumor of the esophagus. This is 30 times higher than in healthy individuals. This is due to the fact that at a constant stress and irritation of the esophagus mucosa constantly need to make new cells to repair esophageal mucosa. The increased rate of cell division means an increased risk of malignant transformation of cells. Achalasia patients should be examined regularly even after successful treatment.