What is Achalasia?

Achalasia is defined as a rare esophageal motility disorder that involves the smooth muscle layer of the esophagus (the muscular tube that transports food from your mouth to your stomach) and lower oesophageal sphincter. In patients with achalasia, the esophagus muscles do not contract properly which prevents foods and drinks from passing into the stomach, which can get stuck and be brought back up leading to vomiting or choking.

Which age and gender are more affected by this condition?

This disorder typically affects people between the second to the fifth decade of life with a peak incidence between the ages of 30 to 60 years.

Men and women are found to be equally affected. Symptoms may also be found in children after the age of 10 years.

No particular race or ethnic group is more affected than others and familial predisposition is rarely seen.

What are the causes leading to Achalasia?

It is a rare disorder but the following factors have been found to cause it-

• Damage to the esophageal nerves – The primary region of damage is the esophageal mesenteric plexus and includes autoimmune reactions. This reaction attacks the nerve cells in the muscular layer of the esophageal wall and the lower esophageal sphincter. The nerve cells which control the relaxation and contraction of the muscle fibers start degenerating. Thus, the Lower esophageal sphincter fails to function, and the food and liquids can’t pass through the pipe. 
• Cancer of the esophagus or upper stomach – studies shows that cancer patients can give rise to achalasia-like symptoms. The risk of developing cancer is also a factor in assessing whether there is potential role for surveillance in this disease.
• Family history – people with positive family history of achalasia shows genetic predisposition to the disease.
• Any autoimmune condition – the reaction attacks the nerve cells in the muscular layer of the esophageal wall and the lower esophageal sphincter and they start degenerating.

What are the signs and symptoms commonly seen in this condition?

People with achalasia typically experience dysphagia with other common symptoms like-

• Regurgitation of the undigested food – Damaged tissues of the oesophagus from stomach acid backing up lead to spasms, scarring and narrowing of the food pipe that causes difficulty in swallowing (dysphagia).
• Pain in the chest – chest pain with sensation of food stucked in throat and heaviness could be there.
• Difficulty in swallowing liquids and solids – Dysphagia can occur due to any abnormalities in the oropharynx or oesophagus. Oropharyngeal disorders can cause problems at the beginning of swallowing at the pharynx and upper oesophageal sphincter. 
• Heartburn – it is the main symptom of gastro-esophageal reflux disease. In initial stage of achalasia GERD and heartburn are common.
• Cough triggered when lying in a horizontal position – cough is there because of stomach contents backing up due to failure of Lower esophageal sphincter.
• Aspiration pneumonia – At times food and liquids are retained in the esophagus and may be inhaled through the lungs.

What other diseases look like Achalasia?

Certain diseases that might mimic symptoms of achalasia are-

•  Gastro esophageal reflux disease
•  A hiatus hernia
•  Psychosomatic disorders

GERD: In GERD, the LES doesn’t close after emptying of esophagus and results in reflux of stomach contents back to the esophagus.

A hiatus hernia: In hiatus hernia, the top of your stomach pushes up through an opening in your diaphragm into the chest.

Psychosomatic disorders: It is a physical condition resulting from mental stress and distress. Stress can affect the body in many ways. In psychosomatic disorders, the main line of treatment should be stress management.

What can be the complications of achalasia?

Commonly occurring complications of this condition are-

• Aspiration pneumonia
• GERD
• Esophagitis with fungal infection
• Perforation of the oesophagus
• Oesophageal cancer
• Malnutrition

1. Aspiration pneumonia:

It is the most severe and rare complication of achalasia cardia. Due to the sudden regurgitation process, the gastric contents from the stomach enter the lungs, leading to bronchopneumonia. In this complication, the patient may feel a choking sensation, sudden breathlessness, vomiting, and respiratory distress.  

2. GERD:

The long-term immobility of the oesophagus causes the weakening of the lower oesophageal sphincter that lead to frequent regurgitation of the food contents to the mouth that causes acid reflux. The patient may present with epigastric pain, fullness after meals, water brash and indigestion. 

3. Esophagitis:

There is a constant collection of food and gastric contents in the oesophagus that can irritate the mucosal lining which causes the inflammation of the oesophagus known as esophagitis. There is a fungal infection in the oesophagus. 

4. Perforation of the oesophagus:

Chronic inflammation and irritation in the oesophagus can cause ulceration which leads to the formation of a hole in the oesophagus called an oesophageal perforation. 

5. Oesophageal cancer:

There is not much significant evidence about the medical correlation of oesophageal cancer with achalasia cardia. Though some patients have been diagnosed with adenocarcinoma of the oesophagus have a dilatation procedure for achalasia cardia. 

6. Malnutrition:

There is prolonged weight loss due to the inability to swallow liquids and solids that lead to significant weight loss and weakness which affects the nutritional status. 

How to diagnose Achalasia?

Diagnosis of achalasia is multifactorial. First, a physical examination might be performed. 

However, physical examination is not enough. Few tests should be performed to understand and diagnose this condition.

It can be done by

• X-ray imaging: To evaluate the structure of the esophagus.

• Barium swallow: Barium solution is swallowed, and its path is evaluated along the esophagus using X-rays.

• Esophageal manometry – This test measures the force, timing, and efficiency of muscular contractions of the esophagus and the lower esophagus sphincter. The absence of these contractions is taken as a positive sign of achalasia cardia. 

     – Lower oesophageal sphincter (LES) fails to relax upon wet swallow (<75% relaxation)

     – Pressure of LES <26 mm Hg is normal,>100 is considered achalasia, > 200 is nutcracker achalasia.

     – Aperistalsis in the oesophageal body.

     – Relative increase in intra-esophageal pressure as compared with intra-gastric pressure.

• Upper gastrointestinal endoscopy: This test gives a clear view of the inner esophageal wall. In this test, a narrow tube with a camera called an endoscope, is inserted down the esophagus, and the images are seen on the screen.
• Biopsy: A small sample of tissue may be acquired from the lower part of the oesophagus to look for cancer cells. 
• Wireless pH testing or 24-hour pH impedance testing: To evaluate the acidity in the oesophagus during an extended period and rule out other conditions such as GERD.

Can achalasia lead to malnutrition?

Achalasia can be a serious health issue if not treated and may lead to severe weight loss and malnutrition in later stages.

It can also cause chest pain, fatigue, and compromised immune symptoms leading to infections like pneumonia.

Which precautions can be helpful in Achalasia?

Some precautions that patients with achalasia can take to improve the prognosis of their condition are-

• Eat the food slowly and chew very well.
• Drink plenty of fluids when eating.
• Avoid eating solid food for three to four hours before going to bed.
• Raise the bed head or sleep with the wedge pillow to avoid flare-up of symptoms at night.
• Food that aggravates the reflux needs to be avoided.
• If there is a symptom of dysphagia or regurgitation, then stop smoking. Smoking can weaken the lower esophageal sphincter. 

Can the cases return after surgery? 

Recurrence is common in cases of achalasia cardia after surgery. There are multiple causes for recurrence but the primary cause in early return is the incomplete myotomy and in late recurrence is the fibrosis due to myotomy. 

What happens if it is left untreated? 

It is serious if left untreated. Patients with achalasia cardia have trouble eating food and drinking fluids. It can result in weight loss and malnutrition. This condition can also result in esophageal cancer if left untreated for a long period of time.  

It can also be represented as a dilated and tortuous esophagus, which may occur in patients who have already been treated surgically yet no improvement in dysphagia, is seen. In such cases, esophagectomy is the only option.  

What is the difference between achalasia cardia and GERD? 

What are the foods to avoid if you have Achalasia Cardia?

Alcohol 

Patients with achalasia cardia should avoid alcohol intake. Chronic ethanol intake can cause permanent damage to the liver, kidneys, pancreas and even the throat. Achalasia cardia includes difficulty swallowing and chest pain do  aggravate after drinking alcohol. Drinking alcohol always makes the condition worse. Alcohol causes inflammation in the lining of the oesophagus by relaxing the lower oesophageal sphincter. 

Caffeine 

Patients with achalasia cardia mainly suffering from acid reflux have a negative effect of caffeine on this condition. 

Caffeinated beverages like coffee, soft drinks and teas can aggravate acid reflux. 

Caffeine lowers the pressure of the oesophageal sphincter. Caffeine has the capacity to loosen the lower oesophageal sphincter which leads to acid reflux and results in damage to the oesophageal lining. 

Citrus fruits 

All the citrus fruits like lemons, oranges, pineapple, lime, grapefruits etc. are more likely to cause acid reflux. Because the acidic content present in citrus fruits triggers acid reflux and relaxes the lower oesophageal sphincter which may worsen the condition. 

Chocolate 

Dark chocolates contain Flavanols, which induce nitric oxide synthesis and may result in the relaxation of the lower oesophageal sphincter. When these muscles relax the stomach content can rise up and can cause burning in the oesophagus. Chocolate also aggravates persistent symptoms like hyperacidity, belching etc. 

Tomatoes 

Tomatoes consist of two types of acid citric acid and malice acid which sometimes cause heartburn due to the hyper secretion of gastric juices. Hyper secretion of gastric juices can cause acid reflux that may lead to esophagitis. 

What food intake can a patient with Achalasia cardia do?   

• Food to include-  

Adopt a soft-textured diet. In case of more severity, a pureed or liquid diet may be needed. Incorporate soft-cooked, mashed, or pureed foods like soups, smoothies, and crock-pot meals. 

1. Include foods rich in protein like chicken, fish, and soy, and eat more high-fiber food.
2. Take a softer and pureed diet like soups, porridge, and mashed vegetables as this will help in swallowing food better without any discomfort.
3.  Dairy products like yogurt or milk work very well in easing symptoms of the digestive system.
4. Ginger plays an excellent role in helping digestion in our body and preventing heartburn.
5. Fluids like broth, stew, and vegetables blended well or juices can be consumed. 
6. Drink at least 10 to 12 glasses of water daily to stay hydrated.   
7. Peppermint as an ingredient in teas and yogurt dips is known to help reduce gastric acid secretion and soothe the body.    
8. Fruit smoothies.
9. Protein shakes and smoothies are helpful when appetite or intake is low. 
10. Sip a small number of liquids with meals to ease swallowing. 
11. Eat applesauce-consistency fruits and squeeze pouches for snacks or you can stir them into yogurt.  
12. Add sauces and gravies to moisten the food.
13. Take small bites, chew food thoroughly, and limit stressful distractions at mealtimes. 
14. Do not go to bed immediately after a meal. Wait at least three hours after eating before lying down to prevent regurgitation and heartburn. 

What are different ways to eat a healthy diet with achalasia- 

Ways to increase vegetable intake-

• Vegetable-based soups will contain small pieces of tender cooked vegetables 
• Frozen vegetables can be easily cooked or steamed until fork tender. 
• Backed sweet potato or squash with butter can be mashed with a fork as a side dish. 
• Great vegetables such as zucchini or carrots into scrambled eggs, sauces, and pasta. 
• Add vegetables to smoothies, along with protein sauces and fruits. 

Ways to Increase Fruit Intake-

• Peel and bake fruit (i.e., baked apples with cinnamon, berry crisp) for dessert.
• Fruits smoothies.
• Include a canned fruit cup at the meal.

What are the commonly indicated Homeopathic medications for Achalasia?

• Phosphorus- It is an effective homoeopathic remedy for achalasia in cases with regurgitation of food. The food comes up again in mouthfuls. There is a contraction of the cardiac opening, which is too narrow, and the food is hardly swallowed. 
•  Carbo veg- Carbo veg is an effective homoeopathic medicine for achalasia cardia for managing symptoms. It is suitable to manage heartburn in cases of achalasia along with putrid belching and sour water brash after eating and drinking. Pain feels in the epigastrium region.
• Asafetida- It is usually an indicated remedy for spasmodic contraction of the esophagus and stomach with impaired peristalsis. It also works well in cases with a feeling of globus hystericus which is a sensation of a ball rising in the throat.
• Lachesis mutus- Suited for patients experiencing difficulty in swallowing along with a constant sensation of a lump in the throat with regurgitation of food. There is no difficulty in swallowing solid foods.
• Alumina – Alumina is a well-suited homoeopathic remedy that helps in managing the symptom of difficulty in swallowing. There is a sensation of lump present in the throat. It can swallow but small morsels at a time. It helps in managing the symptoms of constriction of the oesophagus