Achalasia

Achalasia is a disorder that affects your esophagus. This is the swallowing tube that connects the back of your throat to your stomach. If you have achalasia, your esophagus does not sufficiently push food or liquid into your stomach. In addition, the ring of muscle that circles the lower portion of your esophagus does not relax enough to let food and liquid pass through easily. In fact, achalasia means "failure to relax."

Achalasia usually develops slowly, making it harder for you to swallow food and beverages. It's caused by loss of the nerve cells that control the swallowing muscles in the esophagus. Why these nerve cells degenerate, however, isn’t known. Although achalasia has no cure, symptoms can be controlled with treatment. In rare situations, achalasia results from a tumor. 

Symptoms

Symptoms of achalasia develop gradually. As the esophagus becomes wider and weaker, you may have these symptoms:

• Difficulty swallowing food, a condition called dysphagia

• Food or liquid flowing back up into your throat, or regurgitation

• Waking up at night from coughing or choking because of regurgitation

• Heartburn

• Chest pain or pressure

• Difficulty burping or hiccups

• Weight loss 

Risk factors

Achalasia can develop at any age, but it happens most often between ages 30 and 60. Men and women are equally at risk. Scientists don’t know exactly why this loss of muscle control in the lower esophagus happens, but risk factors may include:

• Genes you are born with

• A disordered immune system that attacks the nerve cells in your esophagus

• The herpes simplex virus or other viral infections 

Diagnosis

If your healthcare provider suspects achalasia from your symptoms, he or she may order three tests to confirm your diagnosis:

• Endoscopy. This is an outpatient procedure during which a flexible telescope is passed through your mouth to examine your esophagus and the valve that opens into your stomach. This valve is called the lower esophageal sphincter (LES).

• Esophogram. This is a special type of X-ray that takes pictures of your esophagus while you swallow a thick contrast material called barium. Signs of achalasia that the radiologist looks for are widening of the esophagus, incomplete emptying, and tightness of your LES.

• Manometry. This is an outpatient procedure during which a pressure-measuring tube is passed through your nose into your esophagus. Pressure measurements are taken as you swallow sips of water. This test may show weak and uncoordinated muscle contractions and pressure buildup at your LES.  

Treatment

No treatment can restore normal esophageal movement. What treatments can do is help relieve your symptoms, open up your LES to improve emptying of your esophagus, and prevent complications. You may also need repeat treatments.

These are common treatments:

• Pneumatic dilation. This is an outpatient procedure done under anesthesia. While your healthcare provider looks into your esophagus through an endoscope, an air-filled balloon is passed through the valve between the esophagus and stomach and then inflated. You may need more than one treatment to get relief.

• Botox injection. Botox is a medicine that can paralyze muscles. Botox can be injected into the muscles that control your LES to relax the valve opening. This procedure is also done during endoscopy. You may be asleep or partially asleep (conscious or "twilight" sedation through an IV). The results usually wear off in 3 months to one year, so the procedure almost always needs to be repeated.

• Surgery. Surgery to open your LES is called myotomy. During myotomy, the muscles of that valve are cut. This type of surgery usually provides long-term relief from achalasia symptoms.

• Medicines. Two commonly used medicines to treat achalasia are calcium channel blockers and long-acting nitrates. These medicines are used if surgery is not a choice and symptoms continue even with Botox injections.

Complications

Although you can’t prevent achalasia, treatment can prevent long-term complications. Possible complications include:

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

• Esophageal perforation. This complication may happen if the walls of your esophagus become weak and distended. Perforation may also happen during treatment. Esophageal perforation may cause a life-threatening infection.

• Esophageal cancer. People with achalasia are at higher risk for esophageal cancer.  

When to call the healthcare provider

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

• Increased difficulty swallowing

• Worsening regurgitation, especially if you are waking up coughing or choking at night

• Symptoms of infection such as chills or fever

• Chest pain or difficulty breathing 

Living with achalasia

Achalasia is a long-term disease, so it's important to learn as much as you can about it and work closely with your medical team. Your healthcare providers will need to follow you on a regular basis, usually once or twice a year, even after symptoms have been controlled. Keep all your appointments. Your healthcare provider may also want to repeat endoscopy and an esophogram.

Here are some lifestyle changes that may help you if you have symptoms of dysphagia or regurgitation:

• Stop smoking.

• Avoid foods or beverages that give you heartburn.

• Drink plenty of fluid when eating and chew your food well.

• Eat smaller meals more often.

• Avoid overeating late at night.

• If you have nighttime symptoms, prop up the head of the bed.