What I've learned about

[Guest guest]

After my son was diagnosed earlier this year, I did lots of

research: reading many studies, figuring out the terminology,

asking questions on this site, and talking with surgeons and

gastroenterologists. This is a summary in plain English of what

I've learned so far. I hope it will be of some help to those who

have been recently diagnosed.

First, what it is. In achalasia, the sphincter from the

esophagus to the stomach doesn't relax properly because the

nerves that tell it to relax in response to a swallow gradually

die off, while the nerves that tell it to contract are just fine

and keep on doing their job. The esophagus gets dilated over

time, stretched out by food that sits there too long. In

addition, the esophagus loses peristalsis, its ability to squeeze

the food downward. Normal people can swallow while standing on

their heads because of peristalsis, but achalasia patients rely

on gravity. It's diagnosed by barium swallow, endoscopy, and

manometry.

Next, how it's treated.

Medicines such as nitrates (Isordil) and calcium channel blockers

(Nifedipine) may relax the sphincter and help some patients

temporarily. Most patients have unpleasant side effects like

headaches. The medicines gradually stop helping, but may be

useful while waiting to get surgery scheduled. Some people have

also reported temporary relief from acupuncture with traditional

Chinese herbal medicine.

Pneumatic dilations are less effective in people under the age of

40, and the younger the patient, the less likely it is that

dilations will help on the long term. Patients who are at least

middle age have a good chance of being helped by dilations if

repeated as needed. Dilations are performed by

gastroenterologists (GI doctors), not surgeons.

Gastroenterologists who specialize in achalasia and have done

many of these forceful balloon dilations have better results and

fewer perforations than inexperienced ones. There's always a

small risk of a perforation which would have to be fixed by

surgery right away.

Botox never helps for long and is only recommended for elderly

patients who cannot risk surgery. Both dilations and Botox

injections cause scarring which makes future surgery more

difficult. Most experts agree that patients under 40 should go

straight to surgery without trying dilations, and certainly

without getting any Botox injections.

Surgery helps most patients, over 90% in many studies. The

myotomy is a lengthwise cut along the esophagus, starting above

the sphincter and extending down onto the stomach a little way.

The esophagus is made of several layers, and the myotomy only

cuts through the outside muscle layers which are squeezing it

shut, leaving the inner muscosal layer intact.

Most surgeons who have treated many achalasia patients and tried

various operations over the years are now using the laparoscopic

approach (keyhole surgery through the abdomen) with anterior

fundoplication (wrapping the fundus of the stomach over the top

of the esophagus).

The thoracoscopic approach (keyhole surgery through the chest)

was used more in the past, but research has shown that patient

outcomes are less likely to be successful with this approach than

the laparoscopic approach. Statistically, patients have less

relief of swallowing problems and more trouble with reflux.

There's also more pain and a longer hospital stay. A few are

still doing it this way, including Dr. Fuller in Los Angeles.

Food can easily pass downward after the myotomy has cut through

the lower esophageal sphincter, but stomach acids can also easily

reflux upward. Many researchers have studied whether it's

necessary to do a fundoplication with a myotomy, and the

consensus is that yes, it's needed in order to prevent excessive

reflux, which can cause serious damage to the esophagus over

time. Part of the stomach is laid over the esophagus and

stitched in place, so whenever the stomach contracts, it also

closes off the esophagus instead of squeezing stomach acids

into it.

Some surgeons do a Toupet (posterior) fundoplication, which wraps

the fundus of the stomach around the back of the esophagus.

However, recent studies have shown that a Dor (anterior)

fundoplication is just as effective at reducing reflux, and some

surgeons who had preferred Toupet are now switching to Dor.

Anterior fundoplication has two advantages: it protects the weak

mucosa of the esophagus which is exposed by the myotomy, and it's

a simpler operation with less cutting around the esophagus than

posterior fundoplication.

A few are starting to use a robotic system for the surgery,

including Horgan (Chicago). They say it provides better ease of

manipulation so there may be fewer perforations (accidentally

poking a hole through the esophagus while doing the myotomy,

something that happens maybe 2% of the time and is easily

repaired with a stitch). However, most top surgeons don't think

there's much point in using the robotic system.

Experience is very important. Surgeons report better outcomes

after their first 50 patients. There are only perhaps a dozen

surgeons in the U.S. who have done enormous numbers of these

operations, including Mayo Clinic in MN (Deschamps etc.), San

Francisco (Patti), Vanderbilt in TN (Torquati etc.), Rochester,

NY (s), Tampa, FL (Rosemurgy), Seattle, WA (Pellegrini),

Cleveland Clinic (Rice), and others. Of course there are very

experienced surgeons elsewhere in the world as well, including

Europe, China, India, and Chile (where many patients get

achalasia from Chagas, a parasitic disease).

This is a delicate operation. It's not easy to get it just

right: extending the myotomy far enough but not too far, and also

getting the fundoplication tight enough but not too tight. You

want the surgery to help you for the rest of your life. Find out

whether your surgeon has published studies on his/her past

experience with a large number of achalasia patients (50+).

It's easy to find phone numbers and even email addresses for

these surgeons on the Internet. Telephone their offices, leave a

message including your phone number, and the surgeon is very

likely to personally return your call within a day or so. Have

your list of questions ready by the phone.

Even if the surgery is done as well as possible, swallowing may

still deteriorate over time. It's important to check every year

or two with a barium swallow, as some may need dilations, a

repeat myotomy, or even (rarely) esophagectomy after 20 or 30

years. It's also a good idea to have pH testing and/or endoscopy

to check for reflux damage, which may lead to cancer of the

esophagus if untreated. There are good medicines to reduce

reflux called proton pump inhibitors. It's also prudent to sleep

with your head elevated by raising the head of the bed or using a

wedge pillow.

At http://scholar.google.com/ you can search for studies by

keywords or a doctor's name. Look up unfamiliar words at

www.onelook.com. Also check out the resources on this group's

website.

Other excellent sources:

http://makeashorterlink.com/?G20623AAC

(understanding swallowing disorders)

http://patients.uptodate.com/topic.asp?file=digestiv/4384

(information for patients with achalasia)

http://makeashorterlink.com/?F516268BD

(guidelines on treatment of achalasia)

http://makeashorterlink.com/?P29D51FBC

(review article on treatment of achalasia)

http://www.clevelandclinic.org/thoracic/phys/swallow/heller2.htm

(how the surgery helps - in pictures)

My 15-year-old son had surgery in Cleveland in July and is doing

very well.

in Lancaster, PA