Achalasia Update: Meeting with surgeon

After two years battling dysphagia, regurgitation, and spitting up foamy messes, I’ve finally gone and met a surgeon. I was reccomennded to Dr. Carmine Simone by my GI specialist, Dr. Sam Friedlander. It took about 3 weeks to get an appointment with Dr. Simone.

Dr. Simone went over the procedure, and basically told me what I already knew. From my tests I had zero peristalsis and a LES pressure slightly above the mean. He recommend that I get a laparoscopic Heller myotomy, but without a fundoplication due to my complete lack of peristalsis.

I’ve booked the surgery for January, as to not interfere too much with the school term. Apparently the doctors do not perform surgery during exam/christmas time, which basically makes them unavailable for most of December.

I also asked Dr. Simone what he though about POEM as a surgical technique. He said that there were POEM trials held by Dr. Marylin at another hospital in Toronto, and that he would not recommend it. He also said that his opinion was biased, gave me Dr. Marylin’s contact information, and suggested I should contact him myself.

I will be continuing reciving treatment from Dr. Chen at AC99 until the surgery. I’ve had noticeable improvements since I’ve started acupuncture. Next week I will be starting a combination of acupuncture and herbal extracts. Dr. Chen seems to think he can restore peristalsis, so I will continue with his treatments.

I also requested a second manometry test to see if the acupuncture has had any unbiased measurable effect. However I believe I was put on the low priority waiting list for this test because I had it done once before.

August 30, 2012August 23, 2012

Everything to know about Esophageal Achalasia: Western Approaches

Western Approaches

Most of the effective western treatments are surgeries. I will list these from least permanent (medicines) to most permanent (surgeries).

Western Treatments

Calcium Channel Blockers (Nitrates) are orally injested drugs such as Nifedipine or Isosorbide Dinitrate. They are taken 15-30 mins before every meal and work by temporarily relax smooth muscle and reduce LES pressure. These drugs have been studied intensely and are effective at lowering the LES pressure (average of 45 mmHg to 15mmHg), but is ineffective at reducing chest pain, and has side effects such as lower blood pressure and cause headaches [Gelfond, 1981] . Nitrates are not seen as a long term solution, and generally only prescribed to patients with early stage Achalasia [Annese, 2006], [Pohl, 2007].

Botulinum Toxin A (Botox) Injections involves endoscopically injecting botox directly into your LES. Botox causes muscles to tighten, and in this case will keep the sphincter open. Success rates are 44-100% 6-12 months after treatment. Success rates are generally better in patients over 50 or for people who cannot risk surgery. Patients tend to develop a resistance to the treatment and eventually it will no longer have any effect.

The procedure sedating the patient, then injecting 100mL of Botox into four quadrants about 1cm above the esophageal spincheter. Patients can return after sedation wears off. Symptomatic improvement is gradual and usually peaks in 1-3 days. [Source]

Botox injections can be effective if the patient is aged, with a high LES pressure [Source].

Pneumatic Balloon Dialation(s) (PBD) involve endoscopically inserting a balloon into your LES, then inflating it to a certain diameter thus stretching and tearing the sphincter muscle. Scar tissue will grow back in its place, which does not hold tension as well as muscle. PBD generally has a 65-85% success rate on a follow up after 6.5 years. This technique brings temporary relief to symptoms of Achalasia, as remission rates are 59% in the first year, and 40% in 5 years, and 36%% remission in 10 years. Symptoms typically recur in up to 50% of patients, with a of dysphagia-free symptoms of 5 years.

PBD is generally more effective in older patients (ages >40), as there is a difference in muscle tone around the esophageal sphincter when compared to younger patients.

Pre-procedure invovles fasting from 12 hours to 2 days, depending on the severity of symptoms. The patient will be sedated, and a 15mm long balloon mounted will be inserted orally on an endoscope into your LES. The balloon is initially inflated to 7-15 psi, and held at pressure for 60 seconds. During this time, there will be discomfort. After the initial inflation, a second inflation at a lower pressure is performed. After the procedure, patients are kept for up to 6 hours to check for perforations. [Source]

If symptoms return after a few weeks, another dilation with a larger balloon is performed. The treatment typically has 3 sizes of balloons (30, 35, 40mm in diamter), and you will start with the smallest size [Source]. The smallest size balloon may not stretch your LES out sufficiently, and therefore the treatment may need to be repeated multiple times with a larger balloon. Although less popular, multiple dilations can be performed in a single treatment, until a target manometry value is achieved. [Source]

There is a 1-2% chance that perforations may occur during this technique. Perforations are tears in the esophagus and can are dangerous as blood can pile up in esophagus. This will need to be repaired immediately, usually by endoscopic surgery.

Although effective at reducing dyphagia, dilations are not effective at reducing chest pains. 2% of post-operatives develop GERD.

Surgeries

Laroscopic Heller Myotomy (LHM) is a safe (no recorded deaths) minimally invasive and procedure (surgery) and considered to be the definitive treatment for Achalasia.

An incision is made above the belly button and hollow tube (trocar) is inserted to fill the abdomen with carbon dioxide gas to allow for visualization of the abdominal organs. A camera (esophagoscope) is placed in this tube to allow the surgeon to see during operation. Four more incisions are made in the abdomen and tools are laroscopically used to cut the circular muscle that squeezes the LES. The cut is 1.5cm deep on 6cm of muscle on the esophagus and 2cm on muscle on the stomach are cut. Bleeding from the muscle incisions is minimal so there is no attempt to cauterize. [Source] [Ramacciato, 2005]

Image source: http://www.lapsurg.org/achalasia.html

Cutting this muscle will cause the LES to be stuck permanently open. Now nothing is preventing reflux, so another technique called partial fundoplication can be used to limit reflux. Partial fundoplicaiton involves wrapping a portion of the stomach around the esophagus. Two or three stitches are made to hold the stomach in place. The stomach will be wrapped 180 to 270 degreees around the esophagus. [Ramacciato, 2005]

Standard Heller Myotomies cut 2cm on the stomach, but it has been shown that Extended Myotomies which cut >3cm are more effective at (17% for standard, 9% for extended) reducing reccuring dysphagia. [Wright, 2007]

Average operation time is 150 minutes. Success rates are 75-90%, with failure being defined as requiring another operation. The best candidates have had no other treatments (dilations or Botox) [Source]. These treatments can lead to strictures at the esophageal junction, which need more work to be cut, and thus have a higher rate of perforations. People who fail this operation can undergo another operation with a good success rate. [Source] [Marco et al, 2001]

Most patients are discharged on day 2 or 3, after which a clear liquid diet (ie: apple juice) can be tolerated. Days 4-6 a ‘full’ liquid diet can be tolerated (ie: meal replacement shakes). A week post-op to a month, a soft diet can be started. After a month solid foods can be resumed. Proton pump inhibitors always given to postoperatives. [Discharge Instructions: Heller Myotomy]

About 15% of postoperatives develop GERD [Source]. 93% of postoperatives say they would do the operation again if necessary[Source]. About 30% of postoperatives will need to redo the operation in 12 years [Source].

Fundoplications involve sewing a portion of the stomach around the esophagus. This increased tension prevents reflux. There are three types of fundoplications used: dor, toupet and nissan. Dor-fundoplication is a 180-200 degrees anterior wrap . Toupet-fundoplication 270 posterior wrap. Nissan-fundoplications are the full 360 wrap. Toupet-fundoplication seems to be more effective than Dor-fundoplication in preventing recurrent Achalasia symptoms (3.4% Dor, 17.3% Toupet). Nissan-fundoplications are no longer performed as can cause dysphagia. [Ramacciato, 2005]

Peroral endoscopic myotomy (POEM) is the newest surgery treatment made available with the invention of NOTES (Natural Orifice Translumenal Endoscopic Surgery). This procedure has been shown to significantly reduce dysphagia symptoms and reduce LES pressure (52mmHg to 20mmHg). Development of reflux symptoms appears to be relatively low (1 in 17). Unfortunately as this surgery method is new, there is no long term data available [Inoue,2010]. There are also very few medical centers equipped to perform this surgery; Northwestern Memorial in USA, and Northern Yokohama Hospital in Japan. Clinical trials are being performed, in multiple countries (including Canada!).

This surgery differs from the Heller Myotomy as the tools enter orally, then a 3cm incision is made in your esophageal lining (mucosa). From there the esophageal muscle can be cut. In total 12cm of muscle is cut: 10cm of esophagus, and 2cm of LES [Inoue, 2010]. The incision is then closed with standard endoscopic clips [Zou, 2012].

[Image Source]

This procedure takes less than two hours, after which you will stay in the hospital for a day for observation.

Complications with this procedure include: an 8% chance to develop of Gastroesophageal Reflux Disease (GERD), and 6.9% chance for mucosal perforation during surgery. 25% of patients require additional intervention, with the mean followup time of 5.3 years. [Source with video]

Efficacy of POEM seems to be similar to Larascopic Heller Myotomy [Hungness, 2012]. Advantages of POEM over LHM is that the length of the myotomy is not limited, and there is no need for fundoplication [Miller, 2011].

Esophagectomy is a surgery in which the esophagus is removed. The use of esophagectomy is extremely rare in the treatment of Achalasia. It is a technique more commonly used in esophageal cancer. The stomach is transplanted into the neck or a piece of colon is sewn where the esophagus would be. Mortality rates for this surgery are typically 5-10%. This technique is only used when people have sigmodial esophagus, or have repeated failed myotomies [Glatz, 2007].

My future direction

Personally I am leaning towards the POEM surgery as it has the fastest recovery time and does not require a fundoplication. However there isn’t enough long term data to definitively say it is better than a larascopic heller myotomy. I’m currently looking at enroling in a clinical trial, so that my treatment may take place in a study that may help others in the future. Plus, isn’t it cool to always have the newest technology?

One thing that I always wonder about is how far away is stem cell technology? What if I get the surgery but then stem cell technology allow me to regain control of my esophagus? Could it repair the scars from the surgery as well? Or would the surgery exempt me from regaining control. Well I’m guessing we are probably about 30 years away from widespread use, so I might as well take the engineering approach do what you can with the time you are given.

August 27, 2012August 23, 2012

Everything to know about Esophageal Achalasia: Western Diagnostic Techniques

Western Diagnostic Techniques

There are three tests used to determine if the symptoms are Achalasia: barium swallow, manometry, and endoscopy. These tests are used to determine if the difficulty swallowing (Dyphagia) is due to Achalasia or from another possible disease (ie: esophagal cancer).

Severity of Achalasia can be scored objectively using the Eckardt Score, the Vantrappen Classification, and the Adams’s Stages. The Edkart score is the most useful for clinical evaluation The Edkart score ranges from 0 to 12, 12 being the worst symptoms [Gockel, 2007].

Barium swallow involves swallowing a chalky-white barium slurry in front of an X-Ray recording. Since barium is opaque to X-rays, the movement of the esophagus can be seen in real time. The barium slurry does not taste bad, but may make your poo chalky white. When I did the test a year and a half ago, the technician recognised there was something wrong immediately as the barium was not easily passing through the LES into my stomach. Only until I had drank a large amount, my LES would open and let the liquid into my stomach. I believe at that time I still had some parastalsis in my upper esophagus.

Manometry involves pushing a pressure sensitive tube about 2mm in diameter through your nose into your esophagus. They will ask you to swallow small sips of water, and the tube will measure the pressure along your esophagus. When I had my test, they lubed up the tube and put it in my nose. It is an extremely strange sensation as you continue to push it down. At certain point my gag reflex kicked in and I started dry heaving, but funny enough as it continues to go down the gagging will stop. The results were that I had no pressure change in my esophagus while swallowing; no parastalsis. My LES activated at a pressure of 25mmHg, which is pretty normal, however was incomplete relaxation (it only opened to 36%).

Endoscopy involves sedation and sticking a camera-tube down your throat. An IV needle was placed in my hand and I was told to count to 10. I think I go to about 3 before I don’t remember anything until waking up. This camera is used mainly to check that your esophagus doesn’t have any tumors which would obstruct food while swallowing. Mine was clear.

Personal experience

I “failed” all three tests and was thus diagnosed with classic Achalasia. This was over a year ago and I believe my symptoms are being managed with herbal chinese medicine and acupuncture. Before undergoing surgery, I would like to another manometry to see if my symptoms have further degenerated.

August 25, 2012August 29, 2012

Everything to know about Esophageal Achalasia: Introduction to Achalasia

Yesterday I went to see my GI specialist about my condition; Achalasia. This would be the second visit. The first visit was approximately a year ago, although I seem to have had symptoms for a little over two years.

I’ve done about a years worth of internet reading about Achalasia, as well as had firsthand experience. This post will be a summary of my knowledge and experience, and I hope this will help others who suffer from this affliction.

This will be the first part of a series.

What is Achalasia?

Esophageal Achalasia (aka Achalasia cardia) is a degenerative nerve disease which causes loss of the involuntary esophageal movement when swallowing (this is called parastalsis) and increased pressure on the lower esophageal sphincter (LES).

My Personal Experience

From my experience, this result primary symptoms such as: difficulty while swallowing, regurgitation, chest pains, waking up choking on saliva in the middle of the night, unintentional weight loss, and spitting up large amounts of saliva. This is a relatively rare disease, affecting 1 in 100000 people.

One of the secondary symtoms I’ve encountered is an inflamed esophagus (esophagitis) due to excess stress which made regurgitation much more frequent. I believe the freqent regurgitation also caused some reflux problems, and this caused stomach acid to irritate my esophagus. Symptoms of esophagitis included nausea, vomiting, a burning sensation in the esophagus. The nausea was so bad that I couldn’t keep any solids or liquids down, and my body weight dropped from 140lbs to 124 in two weeks.

I first noticed symptoms about two years ago while I on my PEY internship. I woke up one day with a burning in my chest which hurt so much that I thought I was going to die. I later found out that it was just heartburn and I could have taken an antiacid for that. Afterwards I would get heartburn whenever I was walking around the shop.

Over the next year I started developing problems when swallowing. I used to eat extremely quickly, but things started to get stuck while eating. I’ve developed techniques like eating with a straight back, or drinking warm or carbonated liquids to push the food down. I’ve managed to eat normally without losing any weight. The biggest difference is that I’ve gone from eating a meal in 10 minutes to about an hour, and I need to drink somewhere between 4-8 cups of water with each meal.

Chugging a cup of water at the end of every meal is extremely important because it washes whatever is left down to the stomach. I’ve had times where I’ve spit up breakfast food during the evening. Also, I find that if there is solid blockage, salivia will accumulate on top of it, cause discomfort and I will have to spit it out.