Achalasia is a rare disease that makes it difficult for food and liquids to pass from the esophagus to the stomach. It happens when the nerves and muscles in the esophagus are damaged. Doctors don’t know what causes it and there is no cure.
But achalasia is treatable, said gastroenterologist Michael S. Karasik, MD. Until recently, Karasik was the only doctor in Connecticut to offer what is considered the most effective treatment for achalasia.
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A challenge in treating achalasia is that when the first symptoms appear, it is often mistaken for gastroesophageal reflux disease (GERD). When the esophagus stops working properly, food accumulates in it. With nowhere to go, the patient regurgitates food. In severe cases, patients cannot even swallow liquids. The difference is that with GERD the food comes up from the stomach, and with achalasia the food never goes that far before coming up.
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What to pay attention to
Achalasia often begins sporadically and then becomes more constant. Signs and symptoms may include:
- Inability to swallow (dysphagia), which may feel like food or drink is stuck in your throat
- Regurgitation of food or saliva
- Stomach pains
- Chest pain that comes and goes
- Cough or vomiting, especially at night
- Pneumonia (from aspiration of food into the lungs)
Karasik said he has treated patients ranging from teenagers to adults in their 90s. He said some cases are so severe that patients can vomit seven to 10 times a day and have lost up to 80 pounds. “You are uncomfortable, you cannot sleep, you lose weight and there is a risk of vomit aspiration, which could lead to pneumonia,” he said.
Diagnosis of achalasia
Because the symptoms are similar to other stomach or heart problems, by the time a patient visits Karasik they are often very ill and weak. Common diagnostic methods include:
- Esophageal manometry. This test measures the rhythmic muscle contractions of your esophagus when you swallow, the coordination and force exerted by the muscles of the esophagus, and how well your lower esophageal sphincter relaxes or opens during a swallow. This test is most helpful in determining the type of motility problem you may have.
- X-rays of the upper digestive system (esophagogram). X-rays are taken after drinking a chalky liquid that coats and fills the inside lining of your digestive tract. The liner allows your doctor to see a silhouette of your esophagus, stomach, and upper intestine. You may also need to swallow a barium pill which can help show blockage in the esophagus.
- Upper endoscopy. Your doctor inserts a thin, flexible tube equipped with a light and a camera (endoscope) down your throat to look inside your esophagus and stomach. Endoscopy can help define a partial esophageal blockage if your symptoms or the results of a barium study indicate this possibility. Endoscopy may also be useful to take a sample of tissue (biopsy) to test for complications of reflux such as Barrett’s esophagus.
I have achalasia – what now?
Karasik said there are four options for treating the disease. His preferred approach is peroral endoscopic myotomy (POEM). The newer method is much less invasive than the other surgical option, which means it has faster recovery and fewer complications.
- Peroral endoscopic myotomy (POEM). POEM uses an endoscope – a narrow flexible tube with a camera – that is inserted through the mouth to cut the muscles in the esophagus (a myotomy). Cutting the muscles relaxes them and prevents them from contracting and interfering with swallowing. “I do a lot,” Karasik said. “Patients love it. It provides long-term relief and is approximately 94% effective for all types of achalasia.
- Laparoscopic Heller myotomy (LHM). This method is more invasive, as the surgeon reaches into the esophagus to make the cut through the abdominal wall. This is a laparoscopy, which is less invasive than traditional surgery, but cuts the abdominal muscle.
The non-surgical options are:
- Pneumatic expansion. A balloon is inserted endoscopically into the center of the esophageal sphincter and inflated to effectively tear the opening. Until the introduction of surgical options, this was the most common method, but overall response rates are a maximum of 50% to 75%.
- Botox (botulinum toxin type A). This muscle relaxant can be injected directly into the esophageal sphincter with an endoscopic needle. It paralyzes the muscle so food can pass through. Repeat injections are usually required and the frequency often increases as treatment continues.
The long term
Without a cure, patients who develop achalasia will live with it for the rest of their lives. With the interventions above, this can be manageable. Regardless of the treatment method, Karasik said, “they have to eat differently for the rest of their lives. The esophagus no longer does the job of moving food from the throat into the stomach, and so gravity has to take over.
He advises patients to:
- Always eat slowly.
- Take small bites.
- Drink plenty of fluids.
- Chew their food well.