What is Achalasia or Achalasia Stages Causes and Treatment Methods

Many of us may experience swallowing difficulties from time to time, but when it becomes frequent and worsens over time, we need to stop and understand the cause! Achalasia is a rare condition that affects the esophagus, making a person feel like food is stuck in their chest and not going down easily. This happens due to nerve dysfunction that controls the esophagus, causing the valve connecting the esophagus to the stomach to remain closed instead of relaxing as it should.

In this article, we will discuss its causes, symptoms, diagnostic methods, and available treatments, including medications, exercises, and even surgery. If you often struggle with swallowing or experience persistent reflux, this article will help you understand this condition better and learn how to manage it!

What are the long-term consequences of untreated achalasia?
If left untreated, achalasia can lead to complications such as aspiration pneumonia, weight loss, and esophageal dilation. Seeking medical help is crucial for effective management.
Which age group is most affected by achalasia?
While achalasia can affect individuals of any age, it is most commonly seen in people between the ages of 25 and 60.
What complications can arise from achalasia?
Food that accumulates in the esophagus and is later aspirated into the trachea (windpipe), which connects to the lungs, can lead to certain complications, including:
- Pneumonia
- Lung infections

What is Achalasia?

Achalasia is a swallowing disorder that affects the esophagus, the tube that connects the mouth to the stomach. Due to nerve damage, the muscles of the esophagus struggle to push food and liquids into the stomach. As a result, food accumulates in the esophagus, sometimes fermenting and flowing back into the mouth with a bitter taste.

Achalasia is a relatively uncommon disorder and is sometimes misdiagnosed as gastroesophageal reflux disease (GERD). However, in achalasia, food comes from the esophagus, whereas in GERD, the material originates from the stomach.

There is no cure for achalasia symptoms. Once the esophagus is damaged, the muscles can no longer function properly. However, less invasive treatments such as endoscopic procedures or surgery are typically used to manage the symptoms of esophageal dysfunction.

Causes of Achalasia

Nerve Damage or Loss in the Esophagus
The body relies on nerve signals to open the valve between the esophagus and stomach (the lower esophageal sphincter). If these nerves become damaged or weakened, the valve remains closed, preventing food from passing normally.
Immune System Problems (Autoimmune Disorders)
In some cases, the immune system mistakenly attacks its own cells, which can affect the nerves controlling the esophagus. Many researchers believe that achalasia may be triggered by an abnormal immune response.
Previous Viral Infections
Studies suggest that certain viruses, such as the herpes virus or measles virus, may attack and damage the nerves in the esophagus, eventually leading to achalasia.
Genetic (Hereditary) Factors
Some studies indicate that achalasia may have a genetic component, meaning that certain families may have a higher risk of developing the condition.
Rare Syndromes and Diseases
Other medical conditions can lead to achalasia, including:
- Chagas Disease: A parasitic infection transmitted by insect bites that can damage the nerves of the esophagus.
- Neurological Disorders: Conditions like Parkinson’s disease or other nerve-degenerating diseases may contribute to the development of achalasia.

The Three Stages of Achalasia Progression

Achalasia progresses through three main stages, each with different symptoms and effects on swallowing and digestion. Let’s break them down:

???? Stage 1 (Early Stage - Compensatory)

At this stage, the body tries to compensate for the issue, and symptoms are mild or intermittent. The patient may not even notice them clearly.

Symptoms:

Mild difficulty swallowing, especially with solid foods.
Feeling like food takes longer to reach the stomach.
Occasional coughing or choking while eating.
Mild reflux without significant pain.

Stage 2 (Moderate Stage - Decompensatory)

At this point, the condition worsens, and the body can no longer compensate, making the symptoms more noticeable and bothersome.

Symptoms:

Increased difficulty swallowing, even with liquids.
Regurgitation of undigested food.
Feeling of pressure or chest pain after eating.
Weight loss due to difficulty eating.
Sometimes, aspiration pneumonia due to food entering the lungs during regurgitation.

Stage 3 (Late Stage - Complications)

At this stage, the esophagus becomes significantly dilated and weakened, losing its ability to transport food to the stomach.

Symptoms:

Swallowing becomes nearly impossible, even with liquids.
Severe weight loss and malnutrition.
Significant esophageal dilation, leading to complications such as infections or ulcers.
Frequent regurgitation, which may cause chronic pneumonia or breathing issues.

Achalasia Symptoms in Detail

Achalasia affects the esophagus, making swallowing progressively harder over time. Symptoms start mildly and worsen gradually, varying in severity depending on the disease stage. Here’s a detailed breakdown:

Difficulty Swallowing (Dysphagia) – The Main Symptom

What Happens?

Initially, swallowing solid foods (like meat or bread) becomes difficult.
Over time, even liquids become hard to swallow.
The sensation of food getting stuck in the chest or coming back up.

Complications:
✔ Food may remain in the esophagus for a long time.
✔ Risk of choking or coughing during meals.

Regurgitation of Undigested Food

What Happens?

Food that doesn’t reach the stomach comes back up into the mouth, especially after eating or while sleeping.
It may have a bitter or acidic taste due to accumulated fluids.

Complications:
✔ Can cause esophagitis (inflammation of the esophagus).
✔ Risk of aspiration pneumonia if food enters the lungs instead of the stomach.

Chest Pain and Pressure

What Happens?

Pain feels like a muscle spasm or strong pressure in the middle of the chest.
It may occur while eating or without an obvious trigger.

Complications:
✔ Can be mistaken for angina (heart-related chest pain), causing concern.
✔ Severe pain may interfere with sleep and daily activities.

Weight Loss & Malnutrition

What Happens?

Due to difficulty eating, the patient consumes less food and starts losing weight.
The body doesn’t get enough nutrients, impacting overall health.

Complications:
✔ General weakness and persistent fatigue.
✔ Vitamin and mineral deficiencies, leading to issues like hair loss and osteoporosis.

Coughing and Choking During Meals or Sleep

What Happens?

Food or liquids may mistakenly enter the airway due to regurgitation.
This can cause frequent coughing, especially at night.

Complications:
✔ Recurrent pneumonia.
✔ Risk of choking during sleep (can be dangerous in severe cases).

Types of Achalasia

Achalasia has different types based on its cause and effect on the esophagus. Scientists have categorized it to make diagnosis and treatment easier. Here’s a breakdown of the types:

Type I - Classic Achalasia

Description:
This is the most common and primary type. It occurs when the esophageal muscles completely lose their ability to move, and the lower esophageal sphincter (LES) remains tightly closed.

Symptoms:

Difficulty swallowing both solid foods and liquids.
Regurgitation of undigested food.
Gradual weight loss.
A sensation of blockage in the chest after eating.

Diagnosis:

Barium swallow X-ray will show a food-filled esophagus that cannot empty into the stomach.
Esophageal manometry will reveal a complete loss of muscle function.

Type II - Achalasia with Panesophageal Pressurization

Description:
In this type, the esophagus attempts to compensate by producing irregular contractions and abnormal pressure to push food down. However, it still fails to transport food effectively to the stomach.

Symptoms:

Chest pain and pressure after eating.
Less regurgitation compared to Type I.
Difficulty swallowing, but not as severe.

Diagnosis:

Barium swallow X-ray shows some esophageal movement, but not strong enough to transport food efficiently.
Esophageal manometry detects high pressure due to uncoordinated contractions.

Note: This type responds better to treatments like balloon dilation or Botox injections.

Type III - Spastic Achalasia

Description:
In this type, the esophageal muscles contract abnormally, creating intense and painful spasms instead of properly moving food downward.

Symptoms:

Severe chest pain, sometimes even without eating.
Extreme difficulty swallowing, especially solid foods.
Less regurgitation than other types, but pain is the most distressing symptom.

Diagnosis:

Esophageal manometry reveals abnormal and spastic contractions.
Barium swallow X-ray may show irregular esophageal movement.

Note: This type is the hardest to treat and often requires surgery if other treatments fail.

Rare Types of Achalasia

Secondary Achalasia
Occurs due to other conditions such as Chagas disease, which affects the nerves, or esophageal tumors that prevent the lower esophageal sphincter (LES) from opening properly.

Achalasia in Children
Extremely rare but can occur due to genetic disorders or nerve dysfunction from birth.

Diagnosis of Achalasia

Achalasia is diagnosed in multiple steps using several tests to confirm the issue, identify its type, and determine its severity. Let’s go through the diagnostic process in detail:

Medical History & Physical Examination

The first step involves the doctor asking about symptoms such as:
✔ Difficulty swallowing? Is it worsening over time?
✔ Regurgitation of undigested food?
✔ Chest pain?
✔ Weight loss?

A physical examination follows to check for visible weight loss or signs of malnutrition.

Barium Swallow Test (Esophagogram)

How it works:

You drink a liquid called barium, which appears on X-rays.
The doctor takes X-ray images to observe how the liquid moves through your esophagus into your stomach.

What does it show in achalasia?
The esophagus appears dilated (enlarged) and filled with food, while the lower esophageal sphincter (LES) remains closed.
A "bird’s beak" sign appears in the X-ray, indicating narrowing at the lower esophagus.

Importance:
This is the first key test that suggests achalasia, but it doesn’t specify the exact type.

Esophageal Manometry (Pressure Measurement Test)

How it works:

A thin tube with pressure sensors is inserted through the nose into the esophagus.
You swallow small amounts of water while the device records esophageal muscle pressure and LES function.

What does it show in achalasia?
The esophagus has no normal contractions to push food down.
The LES does not relax properly when swallowing.
Helps distinguish between classic, pressurized, and spastic achalasia types.

Importance:
This is the most accurate test for diagnosing achalasia and identifying its type.

Endoscopy (Esophagogastroduodenoscopy - EGD)

How it works:

A doctor inserts a thin tube with a camera through the mouth into the esophagus and stomach.
A biopsy (tissue sample) may be taken if a tumor or inflammation is suspected.

What does it show in achalasia?
The esophagus is enlarged and filled with undigested food.
The LES appears tight and does not open properly.
Rules out esophageal tumors or gastric inflammation, which may cause similar symptoms.

Importance:
Endoscopy is used when the doctor suspects another underlying cause, such as a tumor or esophagitis.

Additional Tests (If Needed)

Gastric Emptying Test:

If the patient has severe regurgitation, this test evaluates how quickly food leaves the stomach.
Helps rule out gastroparesis (delayed stomach emptying).

CT Scan or MRI:

If the doctor suspects a tumor compressing the esophagus, imaging scans may be ordered to confirm the diagnosis.

Risks and Complications of Achalasia

If left untreated, achalasia can lead to serious health problems, as food fails to reach the stomach properly, affecting both digestion and nutrition. Let’s go through the complications in detail:

Megaesophagus (Esophageal Dilation)

What happens?

Over time, the esophagus expands due to food accumulation that cannot pass into the stomach.
The muscles weaken further, causing the esophagus to lose its ability to push food down.

Complications:
✔ Food and liquids remain stagnant in the esophagus for long periods.
✔ Severe swallowing difficulties, even with water.
✔ In advanced cases, surgical removal of part of the esophagus may be required.

Esophagitis & Aspiration Pneumonia

What happens?

Food regurgitating from the esophagus can cause severe inflammation of the esophageal lining.
Sometimes, regurgitated food enters the lungs instead of the stomach, leading to serious respiratory infections.

Complications:
✔ Persistent coughing, especially during sleep.
✔ Chronic lung infections and difficulty breathing.
✔ Repeated aspiration can cause long-term respiratory issues.

Weight Loss & Malnutrition

What happens?

Due to difficulty eating, patients consume less food than needed, leading to weight loss and malnutrition.

Complications:
✔ General weakness and constant fatigue.
✔ Vitamin and mineral deficiencies, causing hair loss and osteoporosis.
✔ Weakened immune system, increasing susceptibility to infections.

Esophageal Ulcers & Increased Risk of Esophageal Cancer

What happens?

Prolonged food retention in the esophagus can cause chronic inflammation and ulcers in the esophageal lining.
Over time, chronic inflammation increases the risk of squamous cell carcinoma of the esophagus.

Complications:
✔ Internal bleeding due to ulcers.
✔ Severe swallowing difficulties if a tumor develops.
✔ Higher likelihood of major surgery (esophagectomy) if cancer is diagnosed at an advanced stage.

Medication Treatment for Achalasia

Currently, there is no definitive cure for achalasia with medications, but certain drugs can help reduce symptoms and improve swallowing, especially in early-stage cases or for patients who are not eligible for surgery or balloon dilation.

Muscle Relaxants

How do they help?

These medications help reduce the contraction of the lower esophageal sphincter (LES), making it easier for food to pass into the stomach.

Common Medications:
✔ Nifedipine – A blood pressure medication that helps relax the esophageal muscles.
✔ Verapamil – Works similarly but has a slightly weaker effect.

Side Effects:
Dizziness and low blood pressure.
Headache and swelling in the feet.
Temporary effect – must be taken shortly before meals.

Nitrate Medications (Vasodilators)

How do they help?

These drugs help relax the LES, making it easier for food to enter the stomach.

Common Medications:
✔ Nitroglycerin – Taken under the tongue before meals.
✔ Isosorbide dinitrate – Available as chewable tablets or sublingual tablets.

Side Effects:
Dizziness and headaches due to blood vessel dilation.
Low blood pressure, which may cause fatigue and weakness.
Temporary effect, and effectiveness may decrease with prolonged use.

Botox Injections (Botulinum Toxin)

How do they help?

Injected directly into the LES to partially paralyze the muscles, reducing spasms and allowing the sphincter to open more easily.
Effects last 3 to 6 months, and repeat injections may be required.

Disadvantages:
Temporary effect, with decreasing efficacy over time.
In some cases, inflammation or scarring may develop at the injection site.

Medications for Digestion & Reducing Reflux

How do they help?

These drugs improve gastrointestinal motility and reduce reflux and bloating caused by food remaining in the esophagus.

Common Medications:
✔ Metoclopramide (Primperan) – Slightly improves esophageal motility.
✔ Domperidone (Motilium) – Helps reduce bloating and feeling of fullness.

Side Effects:
May cause fatigue and dizziness.
Some may have neurological side effects with long-term use.

Herbal Treatment for Achalasia – Is It Possible?

Achalasia is a chronic condition caused by a neuromuscular dysfunction of the esophagus. Herbs cannot cure the disease itself, but they may help relieve symptoms such as difficulty swallowing, acid reflux, and bloating.

If you choose to use herbs, they should be combined with proper medical treatment and taken under medical supervision, as they are not a replacement for medication, dilation, or surgery.

Peppermint

How does it help?

Contains menthol, which helps relax the esophageal muscles and the lower esophageal sphincter (LES).
May help reduce spasms and discomfort while eating.

☕ How to Use:
Drink a warm cup of peppermint tea 15-20 minutes before meals.
Add two drops of peppermint oil to a cup of water and drink it.

Warning:
If you have severe acid reflux, peppermint may worsen symptoms.

Ginger

How does it help?

Stimulates digestive movement, helping food pass more quickly into the stomach.
Reduces bloating and fullness caused by food remaining in the esophagus.

☕ How to Use:
Drink ginger tea one hour after meals.
Add a slice of fresh ginger to warm water and drink it.

Warning:
Overuse may cause heartburn in some people.

Chamomile

How does it help?

Reduces inflammation and soothes the digestive system.
Helps relax esophageal muscles and reduce stress-related symptoms.

☕ How to Use:
Drink a warm cup of chamomile tea before bedtime to aid digestion and relaxation.

Licorice Root

How does it help?

Protects the esophageal lining from irritation caused by acid reflux.
Has a calming effect on the digestive system.

☕ How to Use:
Boil licorice root and drink it after meals.
Avoid consuming large amounts, as it may increase blood pressure.

Warning:
Not recommended for people with high blood pressure or heart conditions.

Fenugreek

How does it help?

Reduces acid reflux and protects the esophagus from irritation.
Improves digestion and helps relieve bloating.

☕ How to Use:
Boil one teaspoon of fenugreek seeds in water and drink after meals.

Warning:
May cause bloating in some individuals, so start with a small amount.

Exercises for Achalasia to Improve Swallowing

Swallowing exercises strengthen esophageal muscles and help food pass more smoothly. While they do not cure achalasia, they may be beneficial for mild cases or during recovery after surgery or dilation.

Tongue Press Exercise

Purpose:

Activates esophageal and pharyngeal muscles to improve food movement.

How to Do It:
Place the tip of your tongue against the roof of your mouth.
Press firmly as if trying to push the roof of your mouth upward.
Hold for 5-10 seconds.
Repeat 10 times daily.

Dry Swallow Exercise

Purpose:

Strengthens swallowing reflexes and stimulates the vagus nerve.

How to Do It:
Take a deep breath and swallow your saliva without drinking water.
2️⃣Focus on feeling the movement in your esophagus.
Repeat 10 times daily, especially before meals.

Shaker Exercise

Purpose:

Strengthens neck and esophageal muscles and helps open the LES.

How to Do It:
Lie flat on your back on a stable surface.
Lift your head toward your chest as if trying to look at your feet, but keep your shoulders down.
Hold the position for 10 seconds, then relax.
Repeat 5 times daily.

Straw Blowing Exercise

Purpose:

Strengthens throat and esophageal muscles for better swallowing control.

How to Do It:
Take a deep breath and hold a cup of water.
Use a straw to blow into the water forcefully for 5 seconds.
Relax and repeat 10 times daily.

Gargling Exercise

Purpose:

Improves swallowing coordination and strengthens laryngeal muscles.

How to Do It:
Take a sip of warm water and gargle for 5-10 seconds.
Focus on feeling the throat movement while gargling.
Repeat 5 times daily.

High-Risk Groups for Achalasia

Not everyone is at risk of developing Achalasia, but certain groups are more susceptible due to genetic, neurological, or other medical factors affecting the esophagus. Here’s a detailed look at the most at-risk groups:

Middle-aged and Older Adults

Age is a significant factor, as achalasia typically appears between ages 25 and 60, with the highest occurrence in people in their 40s and 50s.
As people age, the nerves controlling esophageal movement may deteriorate, increasing the risk of achalasia.

Individuals with a Family History of Achalasia

While achalasia is not directly inherited, some studies suggest a genetic link in certain families.
If there are family members with achalasia, the risk of developing the condition may be higher.
Some genetic mutations might contribute to the development of the disease.

People with Autoimmune Disorders

Autoimmune diseases, where the immune system attacks nerves and muscles, may increase the risk.
The most common autoimmune diseases linked to achalasia include:
✔ Lupus (Systemic Lupus Erythematosus)
✔ Multiple Sclerosis (MS)
✔ Dermatomyositis (a condition affecting muscles and skin)

In these cases, the progressive nerve damage affects esophageal function, leading to swallowing difficulties.

People with Chagas Disease

This is a rare parasitic disease but is more common in South America. It is caused by the Trypanosoma cruzi parasite.
This parasite damages nerves, especially those controlling esophageal movement, leading to achalasia-like symptoms.
While rare in Egypt and the Arab world, it is a major cause of achalasia in Latin America.

People with Diabetes

People with Type 2 Diabetes may develop autonomic neuropathy, a condition that affects the nerves controlling involuntary functions.
If the nerves controlling the esophagus become damaged, the esophagus may lose its ability to push food down, leading to symptoms similar to achalasia.

Individuals with Esophageal or Chest Tumors

Certain benign or cancerous tumors can compress the vagus nerve or esophageal nerve plexus, leading to achalasia-like symptoms.
These tumors may be located in:
✔ The esophagus itself.
✔ The lungs or nearby lymph nodes.

People Who Have Undergone Radiation Therapy to the Chest

Radiation therapy for lung or esophageal cancer can damage the nerves that control esophageal muscles.
This nerve damage can lead to symptoms of achalasia over time.