Dear Friends:
This page is dedicated to information on the rare and to date, incurable disease called Achalasia. I have been suffering from this disease since it's initial onset around Dec. 04/Jan. 05. There are a LOT of myths and rumors that spread rampant about this disease and more specifically, what is wrong with me, hopefully this information will help inform those who are not in the "know." If you have Achalasia and happened upon my site, first of all, God Bless you, secondly, please know that there are online support groups to help you stay informed about this rare disorder, the best and largest one I have found is a yahoo group. Please feel free to contact me with any questions you may have or if you need someone to talk to, believe me, I can sympathize. I HIGHLY recommend Johns Hopkins, Baltimore, surgeon Dr. Michael Marohn!
If you are like most people, you take your body for granted. As long as all is working well, you seldom think of it. One thing we all take for granted is the simple act of swallowing. You swallow your saliva. You swallow your food. You swallow fluids. Down they all go - while you pay attention to your busy day. You may swallow several times per minute - thousands of times a day, without ever thinking about it. What if every time you swallowed, it was an ordeal? You never knew if your food would go down - or come back up. Every time you ate, you were afraid. Eventually, you might quit eating out in public for fear of an attack. This is what happens to patients with achalasia, an uncommon motility disorder which affects swallowing.
What is Achalasia?
Achalasia is not inherited, the cause and cure are unknown. Achalasia is more common in adults, but affects both sexes equally. Fortunately, it is quite uncommon, affecting only about 1 in 10,000 Americans. Achalasia is a rare disease of the muscle of the esophagus (swallowing tube). The term achalasia means "failure to relax" and refers to the inability of the lower esophageal sphincter (a ring of muscle between the lower esophagus and the stomach) to open and let food pass into the stomach. As a result, patients with achalasia have difficulty swallowing food. In achalasia there is an inability of the lower sphincter to relax and open to let food pass into the stomach. In at least half of the patients, the lower sphincter resting pressure (the pressure in the lower sphincter when the patient is not swallowing) also is abnormally high. In addition to the abnormalities of the lower sphincter, the muscle of the lower half of the esophagus does not contract normally, that is, peristaltic waves do not occur, and, therefore, food and saliva are not propelled down the esophagus and into the stomach. A few patients with achalasia have high-pressure waves in the lower esophageal body following swallows, but these high-pressure waves are not effective in pushing food into the stomach. These patients are referred to as having vigorous achalasia. These abnormalities of the lower sphincter and esophageal body are responsible for food sticking in the esophagus.
What are the symptoms of Achalasia?
First, it is NOT AN EATING DISORDER! The most common symptom of achalasia is difficulty and PAIN swallowing (dysphagia). Patients typically describe food sticking in the chest after it is swallowed. Dysphagia occurs with both solid and liquid food. Moreover, the dysphagia is consistent, meaning that it occurs during virtually every meal. Often the patients have gastro distress including acid reflux, bloating, painful gas and belching. Sometimes, patients will describe only a heavy sensation in their chest after eating that may force them to stop eating. Occasionally, pain may be severe and mimic heart pain. Regurgitation of food that is trapped in the esophagus can occur, especially when the esophagus is dilated. If the regurgitation happens at night while the patient is sleeping, food can enter the throat and cause coughing and choking. If the food enters the trachea (windpipe) and lung, it can lead to pneumonia (aspiration pneumonia). Because of the problem swallowing food, most patients with achalasia lose weight. The inherent weight loss causes further medical problems including malnourishment, emaciation/malice, lethargy, hypotension, anemia, dehydration, muscle deterioration, bone deterioration which causes extreme back pain (Osteopenia & Osteoporosis), vitamin deficiencies (B-12, C, D, K, potassium, calcium, folate, protein and so many others!), low immune system, memory loss, insomnia, panic attacks or post traumatic stress disorder from multiple near death experiences (it is not just a disorder of military folk contrary to what some may believe), clinical depression and general anxiety disorder to name just a few. Due to the lack of peristalsis in late stages of vigorous Achalasia the patient cannot regurgitate anything that falls into the stomach, only what is between the mouth and esophageal sphincter, so very much like a horse, they physically cannot throw up and simply "colic" when they fall ill, this causes all kinds of gastro upsets. Imagine having the stomach flu or food poisoning and not being able to be rid of what ails you, ugh!
How is Achalasia diagnosed?
X-ray studies - a barium swallow performed with a videoesophagram. If the patient has Achalasia the video-esophagram shows that the esophagus is dilated (enlarged or widened), with a characteristic tapered narrowing of the lower end, sometimes likened to a "bird's beak." In addition, the barium stays in the esophagus longer than normal before passing into the stomach.
Manometry - (OUCH) a thin tube that measures the pressure generated by the contracting esophageal muscle is passed through the nose, down the back of the throat and into the esophagus. In a patient with achalasia, no peristaltic waves are seen in the lower half of the esophagus after swallows, and the pressure within the contracted lower esophageal sphincter does not fall with the swallow. An advantage of manometry is that it can diagnose achalasia early in its course at a time at which the video-esophagram may be normal.
Endoscopy - is a procedure in which a flexible fiber-optic tube with a light and camera on the end is swallowed. The camera provides direct visualization of the inside of the esophagus. One of the earliest endoscopic findings in achalasia is resistance as the endoscope is passed from the esophagus and into the stomach due to the high pressure in the lower esophageal sphincter. Later, endoscopy may reveal a dilated esophagus and a lack of peristaltic waves. Endoscopy also is important because it excludes the presence of esophageal cancer. Patients with Achalasia are at higher risk for esophageal cancer and should be tested annually.
Since there is no cure, are there treatments?
Yes, if you are lucky. Treatments for achalasia include PRAYER, oral medications, dilation or stretching of the lower esophageal sphincter (dilation), surgery to cut the sphincter (esophagomyotomy) as the best way to permanently weaken a overly tight LES valve is to cut it, has over a 90% success rate in alleviating the symptoms of achalasia. Surgery is not as successful in cases of vigorous achalasia. Unfortunately I was one of the 10%, making me even more rare. The injection of botulinum toxin (Botox) into the sphincter is another option and lastly, the most risky, the partial or entire removal of the esophagus and replacement of part of the jejunum (esophagectomy). All four treatments reduce the pressure within the lower esophageal sphincter to allow easier passage of food from the esophagus into the stomach. I have tried all the oral medications, endured dozens of dilations and Botox injections and three Heller Myotomies, the initial with a Toupet Fundoplication (three = two failed and one repair, repair thanks to Surgeon Michael Marohn at Johns Hopkins Hospital, Baltimore, MD). Due to the previous two failed myotomies, Dr. Marohn could only do so much with the mess I brought to him. There are only a few options left for me since all else has failed, I can choose to get a feeding tube and/or an eventual esophagectomy (I will not take this risk due to the mortality rate of the surgery). So...I am going with prayer, it is God's will, I am very blessed and extremely thankful to be alive, God never gives you more than you can handle, regardless of how hard things may seem at the time! Attitude is EVERYTHING, I AM A FIGHTER, I AM A SURVIVOR. I thank God everyday for blessing me with the most wonderful family and most loving and loyal friends.
Well, here I go again my next endoscopy and dilatation is 10/19, praying it helps! Turns out I need them every six months, FOREVER to keep things open and know what is going on in there. Dialations actually strecth and mildly tear the restriction, the shorter time in between dialations the less risk for a full perforation of the esophagus.
1/21/09 - It took awhile but it did help some, I finally made it to 100lb.s! Thanks to McDonald's Sweet Tea and Kit Kat bars - ha! Healthy combo. huh?! I will take it however I can get it. All the other issues are still kicking me hard, I cannot stay well, I have been sick with one type of flu or another since November. This week I had chest x-rays, blood work, ultrasounds, etc.. trying to figure out why I am STILL sick and the blood work came back with high billirubin levels (liver issue). I do have massive scar tissue which has attached my liver to my stomach so I hope it hasn't gotten so bad that it isn't causing my liver to work hard to expel bile. Will keep updating.
3/17/09 - Here we go again, can't swallow well and it hurts trying, all the bad stuff is back except I have held my weight steady, and I will take any positives I can! So an ceroscopy with dilation is scheduled in next couple of weeks before it goes too far, ugh - I didn't make it six months this time :(!
8/09 - Well I almost made it, getting an appt. a.s.a.p. as I am having quite a lot of pain both swallowing and in my chest where the esophagus ends (my trouble spot). I have gained some weight, very pleased with that recent development! Will keep you posted on the latest. Dilation done 9/09.
2/10 - Still had a rough time even after 9/09 dilation, lost more weight, sick and miserable, sometimes entire days would pass without being able to eat anything at all. This time we went for a pneumatic dilation with a much larger dilator in the hospital (too risky to do in same day surgery center). That was ROUGH, have to say the most painful one I have had to date... BUT it worked, I can swallow the foods I have been missing now, that is the FIRST time since 2004. I know it is temporary, it is the nature of the beast, but I will take it and run with it. First stop...steak and hamburgers, rolls...glorious bread, ahhh it is amazing! I am ransacked with lots of other digestive issues of course, other pain, etc... but I am eating like a real person almost, for as long as it will last!
Since at least 2006, I was officially diagnosed by my friends with CRS, among a million other scripts, they have me on a prescription vitamin "food" pill that is for older folks with Dementia, so if I forget who you are, don't be alarmed....just remind me. If you have seen Disney's Finding Nemo, I am truly DORY!
Hope you found this page informative or enlightening! Keep the faith AND a sense of humor!!!!
Sincerely,
Stephanie Fisher Snyder
Information collected from personal knowledge/experience and various sources, including:
http://www.gihealth.com/html/education/achalasia.html
http://www.medicinenet.com/achalasia/article.htm
http://www.hopkinsmedicine.org/health_information_library/index.html?ArticleID=75145
http://www.uptodate.com/patients/content/topic.do?topicKey=digestiv/4384
http://www.chestjournal.org/cgi/content/abstract/52/4/508
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