Achalasia

What is achalasia?

Achalasia is a swallowing disorder that affects the esophagus. The esophagus is a tube that connects the back of your throat to your stomach. Food and liquid go down the tube when you swallow. If you have achalasia, the muscles of the esophagus don’t work well at pushing food or liquid down into your stomach. The valve at the lower end of your esophagus called the lower esophageal sphincter (LES) also has a problem. The ring of muscle in the valve does not relax enough to let food and liquid pass easily from the esophagus into the stomach. Achalasia means “failure to relax.” 

Achalasia often develops slowly. It becomes harder to swallow food and drinks over time. Achalasia has no cure. But symptoms can be controlled with treatment.

What causes achalasia?

In most cases, it's caused by loss of the nerve cells that control the swallowing muscles in the esophagus. Healthcare providers don’t yet know why these nerve cells are lost. In rare cases, achalasia is caused by a tumor.

Who is at risk for achalasia?

Achalasia can occur at any age. But it happens most often between ages 30 and 60. Men and women are equally at risk. Healthcare providers don’t know why achalasia happens. But risk factors may include:

• Having certain genes

• Having a problem with your immune system that causes it to attack nerve cells in your esophagus

• Having herpes simplex virus or other viral infections

• Having Chagas disease. This is an infection caused by a parasite. The parasite is passed to people through the bite of an insect. Chagas disease is mainly found in poor rural areas of Mexico and Central and South America.

What are the symptoms of achalasia?

Symptoms can occur a bit differently in each person. They develop over time as the esophagus becomes wider and weaker. Symptoms can include:

• Trouble swallowing food (dysphagia)

• Food or liquid flowing back up into your throat (regurgitation)

• Waking up at night coughing or choking due to regurgitation

• Heartburn

• Chest pain or pressure

• Trouble burping

• Hiccups

• Weight loss

The symptoms of achalasia may be like other health conditions. Always see your healthcare provider for a diagnosis.

How is achalasia diagnosed?

Your healthcare provider will ask about your symptoms and health history. Your provider will give you a physical exam. You may also have tests, such as:

• Endoscopy. A flexible lighted tube is passed through your mouth to look at your esophagus and the lower esophageal sphincter (LES).

• Esophogram. This is a type of X-ray that takes pictures of your esophagus while you swallow a thick contrast material called barium. You may swallow a barium tablet as well. The radiologist looks for signs of achalasia. These include widening of the esophagus, incomplete emptying, and tightness of your LES.

• Manometry. A thin tube that measures pressure is passed through your nose down into your esophagus. Pressure measurements are taken as you swallow sips of water. This test can show if the muscles in your esophagus are weak and don’t work well. The test can also show pressure buildup at your LES. This test confirms a diagnosis of achalasia.

How is achalasia treated?

There's no known cure for achalasia. But treatments can manage your symptoms. Talk with your healthcare provider about the risks, benefits, and possible side effects of all treatments. Treatment can:

• Ease your symptoms

• Open up your LES so your esophagus empties more easily

• Help prevent complications

You may also need repeat treatments. Treatment may include the following.

Pneumatic dilation

This is an outpatient procedure done under anesthesia. Your healthcare provider looks into your esophagus through an endoscope. Your provider passes a small balloon through the LES. The balloon is then inflated using an X-ray to guide it. You may need more than 1 treatment to get relief.

Botulinum toxin injection

Botulinum toxin is a medicine that can paralyze muscles. It can be injected into the muscles that control your LES. This helps to relax the valve opening. This procedure is done during endoscopy. You may be asleep or partly asleep with sedation through an IV (intravenous line). The benefits often go away in 3 to 12 months. So the procedure needs to be repeated.

Surgery

Surgery may be done to open your LES. This is called myotomy. During myotomy, the muscles of the LES are cut. This procedure can be done using a minimally-invasive approach called laparoscopy. A few small cuts (incisions) are made. A thin, lighted tube called a laparoscope is used. This scope lets the healthcare provider see inside your body and work through the small incisions. At the same time, your surgeon may do a procedure called a fundoplication to help reduce acid reflux (gastroesophageal reflux disease (GERD) after the procedure. This is done by wrapping the very top of the stomach around the lower part of the esophagus. This type of surgery often gives long-term relief from achalasia symptoms.

Peroral endoscopic myotomy (POEM)

Your healthcare provider inserts an endoscope through your mouth and down your esophagus. An incision is made in the inner lining of the esophagus. The endoscope then enters between the layers of the esophagus to the muscle. Part of the muscle layer is removed in the lower part of the esophagus, the LES, and the upper part of the stomach. This decreases the tightness from the LES. It allows food and liquid to pass from the esophagus into the stomach more easily. This procedure is available at certain healthcare centers. It is not a option for everyone. POEM is considered less invasive and recovery is rapid. But it results in frequent acid reflux and you most likely will need treatment for GERD after the procedure.

Medicines

There are 2 medicines often used to treat achalasia. They are calcium channel blockers and long-acting nitrates. These medicines are used if surgery is not a choice or if your symptoms continue after getting botulinum toxin injections.

What are possible complications of achalasia?

Treatment can help prevent long-term complications, such as:

• Aspiration pneumonia. This is caused when food or liquids in your esophagus back up into your throat and you breathe them into your lungs.

• Esophageal perforation. This is a hole in the esophagus. It may happen if the walls of your esophagus become weak and bulge. It may also happen during treatment. Esophageal perforation may cause a life-threatening infection.

• Esophageal cancer. People with achalasia are at higher risk for this type of cancer.

How can I prevent achalasia?

Researchers don’t know how to prevent achalasia.

Living with achalasia

Achalasia is a chronic condition. But you can manage it by working with your healthcare provider to create a treatment plan. Your healthcare team will need to see you 1 or 2 times a year, even after your symptoms have lessened. You may need repeat endoscopy and esophogram procedures.

If you have symptoms of dysphagia or regurgitation:

• Stop smoking.

• Don’t eat foods or have drinks that give you heartburn.

• Drink plenty of fluids when eating. Chew your food well.

• Eat smaller meals more often.

• Don’t overeat late at night.

• If you have symptoms at night, prop up the head of your bed.

When should I call my healthcare provider?

Call your healthcare provider if you have any questions about your medicines or treatment. Tell your healthcare provider right away if you have any of these:

• Trouble swallowing that gets worse

• Regurgitation that gets worse

• Waking up coughing or choking at night

• Symptoms of infection, such as chills or fever

• Chest pain or trouble breathing ( Call 911)

Key points about achalasia

• Achalasia is a swallowing disorder that affects the esophagus. The muscles of the esophagus don’t work well at pushing food or liquid into your stomach.

• Achalasia usually develops slowly. It becomes harder to swallow food and drinks over time.

• Symptoms can include trouble swallowing food and food or liquid flowing back up into your throat.

• You may have tests, such as an endoscopy or esophogram.

• There is no known cure. But treatments can manage your symptoms. Treatment may include pneumatic dilation, botulinum toxin injections, surgery, or medicines.

Next steps

Tips to help you get the most from a visit to your healthcare provider:

• Know the reason for your visit and what you want to happen.

• Before your visit, write down questions you want answered.

• Bring someone with you to help you ask questions and remember what your provider tells you.

• At the visit, write down the name of a new diagnosis and any new medicines, treatments, or tests. Also write down any new directions your provider gives you.

• Know why a new medicine or treatment is prescribed and how it will help you. Also know what the side effects are.

• Ask if your condition can be treated in other ways.

• Know why a test or procedure is recommended and what the results could mean.

• Know what to expect if you do not take the medicine or have the test or procedure.

• If you have a follow-up appointment, write down the date, time, and purpose for that visit.

• Know how you can contact your healthcare provider if you have questions, especially after office hours or on weekends.