What is Achalasia? Symptoms, Causes, & Treatment

Achalasia is a rare disorder where your esophagus has trouble moving food down to your stomach. Your esophagus is the tube that connects your mouth to your stomach, and normally it squeezes food downward while a muscle at the bottom relaxes to let food pass through.

In achalasia, this coordinated system breaks down. The esophagus loses its ability to push food down effectively, and the muscle at the bottom stays tight instead of relaxing. This creates a backup of food and liquids in your esophagus, leading to difficulty swallowing and other uncomfortable symptoms.

What are the symptoms of achalasia?

The most common symptom you'll notice is difficulty swallowing, which doctors call dysphagia. This usually starts gradually and gets worse over months or years. You might first notice trouble with solid foods, then later have problems swallowing liquids too.

Here are the main symptoms that people with achalasia experience:

• Difficulty swallowing both solids and liquids
• Food or liquid coming back up (regurgitation), especially when lying down
• Chest pain or pressure, particularly after eating
• Heartburn that doesn't respond well to typical acid reflux medications
• Unintended weight loss due to eating difficulties
• Coughing or choking, especially at night
• Bad breath from food staying in the esophagus

These symptoms often develop slowly, so you might adapt your eating habits without realizing it. Many people start eating more slowly, chewing food more thoroughly, or drinking more liquids with meals.

What are the types of achalasia?

Doctors classify achalasia into three main types based on how your esophagus behaves during special tests. Understanding your type helps determine the best treatment approach for you.

Type I achalasia shows little to no muscle contractions in your esophagus. Your esophagus essentially becomes a passive tube that relies on gravity to move food down. This type often responds well to treatments that widen the muscle at the bottom of your esophagus.

Type II achalasia involves some muscle contractions, but they're not coordinated properly. Instead of the wave-like motion that should push food down, your esophagus contracts in segments. This type typically has the best treatment outcomes.

Type III achalasia features strong, spastic contractions that can actually work against food movement. These powerful but uncoordinated contractions can cause significant chest pain. This type can be more challenging to treat and may require different approaches.

What causes achalasia?

The exact cause of achalasia isn't fully understood, but researchers believe it involves damage to the nerves that control your esophagus. These nerves normally coordinate the muscle contractions that move food toward your stomach.

Most cases are considered primary achalasia, meaning they develop without a clear underlying cause. Scientists think this might involve an autoimmune process where your body's immune system mistakenly attacks the nerve cells in your esophagus, though this theory is still being studied.

Secondary achalasia can occur due to other conditions that damage the esophageal nerves. These include certain cancers, infections like Chagas disease (more common in South America), or previous surgery in the chest area. However, secondary causes are much less common than primary achalasia.

Some research suggests genetic factors might play a role, as very rarely achalasia can run in families. Environmental factors and viral infections have also been proposed as potential triggers, but no definitive cause has been identified.

When to see a doctor for achalasia?

You should see a doctor if you're having ongoing trouble swallowing, especially if it's getting worse over time. Don't wait if swallowing difficulties are affecting your ability to eat normally or if you're losing weight unintentionally.

Seek medical attention promptly if you experience frequent regurgitation, particularly at night, as this can lead to aspiration pneumonia. Chest pain that occurs regularly with eating also warrants evaluation, even if you think it might be heartburn.

Contact your doctor immediately if you can't swallow liquids, if you're vomiting frequently, or if you have severe chest pain. These symptoms might indicate that your condition is progressing or that you're developing complications that need urgent treatment.

What are the risk factors for achalasia?

Age is the most significant risk factor, with achalasia typically developing between ages 30 and 60. However, it can occur at any age, including in children and older adults, though this is less common.

The condition affects men and women equally, and it occurs across all ethnic groups worldwide. Geographic location can influence risk slightly, as certain infections like Chagas disease that can cause secondary achalasia are more common in specific regions.

Having a family history of achalasia slightly increases your risk, though familial cases are extremely rare. Most people who develop achalasia have no family history of the condition. Autoimmune conditions might potentially increase risk, but this connection isn't definitively established.

What are the possible complications of achalasia?

Without treatment, achalasia can lead to several complications that significantly impact your health and quality of life. Understanding these potential issues helps explain why early diagnosis and treatment are important.

Weight loss and malnutrition can develop as eating becomes increasingly difficult. Your body may not get enough calories or nutrients, leading to weakness, fatigue, and other health problems. This is often one of the first serious complications people notice.

Aspiration pneumonia is a concerning complication that occurs when food or liquid from your esophagus enters your lungs. This typically happens at night when you're lying down and regurgitated material goes down the wrong way. Repeated episodes can cause serious lung infections.

Your esophagus may become enlarged over time as food and liquid collect above the tight muscle. This enlargement, called megaesophagus, can make symptoms worse and increase the risk of aspiration. In severe cases, the esophagus can become significantly distorted.

People with long-standing achalasia have a slightly increased risk of developing esophageal cancer, though this risk remains relatively low. Regular monitoring with your doctor helps catch any concerning changes early. The cancer risk appears to be related to chronic inflammation and irritation in the esophagus.

How is achalasia diagnosed?

Diagnosing achalasia typically involves several tests that help your doctor see how your esophagus is working. The process usually starts with discussing your symptoms and medical history in detail.

A barium swallow is often the first test your doctor will order. You'll drink a chalky liquid containing barium, then get X-rays as the liquid moves through your esophagus. This test can show if food or liquid is getting stuck and reveal the characteristic "bird's beak" appearance of the esophagus in achalasia.

Upper endoscopy involves passing a thin, flexible tube with a camera down your throat to directly examine your esophagus and stomach. This test helps rule out other conditions and can show food retention in the esophagus. Your doctor can also take tissue samples if needed.

Esophageal manometry is considered the gold standard test for diagnosing achalasia. A thin tube with pressure sensors is passed through your nose into your esophagus to measure muscle contractions and pressure. This test definitively diagnoses achalasia and determines which type you have.

High-resolution manometry provides even more detailed information about esophageal function and has become the preferred method at many medical centers. CT scans might be used to rule out other conditions or evaluate for complications.

What is the treatment for achalasia?

Treatment for achalasia focuses on reducing the pressure at the bottom of your esophagus to help food pass through more easily. While there's no cure that restores normal esophageal function, several effective treatments can significantly improve your symptoms and quality of life.

Pneumatic dilation is a procedure where your doctor uses a balloon to stretch the tight muscle at the bottom of your esophagus. The balloon is inflated with controlled pressure to partially tear the muscle fibers, allowing food to pass through more easily. This treatment is effective for many people, though symptoms may return over time.

Laparoscopic Heller myotomy is a minimally invasive surgery that involves cutting the muscle fibers that won't relax properly. This procedure is often combined with a fundoplication, where part of your stomach is wrapped around the esophagus to prevent acid reflux. The surgery typically provides long-lasting relief.

Peroral endoscopic myotomy (POEM) is a newer technique where the surgeon accesses the muscle through your mouth using an endoscope. This approach avoids external incisions and can be very effective, though long-term data is still being collected. It may cause more acid reflux than traditional surgery.

Botulinum toxin injections can temporarily weaken the tight muscle and provide symptom relief for several months. This treatment is often used for people who aren't good candidates for surgery or dilation, or as a temporary measure while planning other treatments.

Medications like calcium channel blockers or nitrates can help relax the esophageal muscle, though they're generally less effective than other treatments. They might be used as initial therapy or in combination with other approaches.

How to manage achalasia at home?

While medical treatment is essential, several strategies can help you manage symptoms and eat more comfortably at home. These approaches work best when combined with proper medical care from your healthcare team.

Eating smaller, more frequent meals can make swallowing easier and reduce the feeling of food getting stuck. Take your time when eating and chew food thoroughly. Drinking warm liquids with meals can help food move through your esophagus more easily.

Sleeping with your head elevated can reduce nighttime regurgitation and lower your risk of aspiration. Use extra pillows or raise the head of your bed by 6 to 8 inches. Avoid eating large meals close to bedtime, as this increases the likelihood of regurgitation while lying down.

Staying well-hydrated is important, especially since you might eat less due to swallowing difficulties. Room temperature or warm liquids are often easier to swallow than very cold drinks. Avoid carbonated beverages, as they can increase pressure in your esophagus.

Keep track of which foods are easier or harder for you to swallow, and adjust your diet accordingly. Many people find that softer foods and liquids are more manageable. Don't hesitate to modify food textures or use a blender if it helps you maintain good nutrition.

How should you prepare for your doctor appointment?

Preparing for your appointment can help ensure you get the most out of your visit and provide your doctor with the information needed for accurate diagnosis and treatment planning.

Keep a detailed symptom diary for at least a week before your appointment. Note when symptoms occur, what you were eating or drinking, and how severe the symptoms were. Include information about any weight loss, how your symptoms affect daily activities, and any patterns you've noticed.

Make a list of all medications, supplements, and vitamins you're taking, including dosages. Bring information about any previous treatments you've tried for your symptoms, including over-the-counter antacids or other remedies.

Write down questions you want to ask your doctor. Consider asking about treatment options, expected outcomes, lifestyle modifications, and follow-up care. Don't hesitate to ask for clarification if medical terms or concepts aren't clear to you.

Bring a family member or friend if possible, as they can help you remember important information discussed during the appointment. Having support can also be helpful if you're feeling anxious about your symptoms or potential treatments.

What's the key takeaway about achalasia?

Achalasia is a manageable condition, even though it can significantly impact your daily life initially. The key is getting proper diagnosis and treatment, which can dramatically improve your symptoms and help you return to enjoying meals and normal activities.

While achalasia is a chronic condition that requires ongoing management, most people achieve good symptom control with appropriate treatment. The various treatment options available mean that if one approach doesn't work well for you, there are other effective alternatives to try.

Working closely with your healthcare team and following their recommendations gives you the best chance of managing your symptoms successfully. With proper treatment, many people with achalasia can maintain a good quality of life and continue to enjoy eating, even if some adjustments to eating habits are needed.