Failed botox for Crycopharyngeal achalasia. HELP!?!?!?!

Achalasia
#1

My husband has crycopharyngeal achalasia and it has progressed to the point that he now has a gastrostomy feeding tube because he can no longer swallow.

We have seen every type of specialist and to no avail. Finally, we found a surgical ENT who recognized the disorder (instead of basically calling my husband a psycho who is imagining this whole thing… “globus hystericus.”)

He proceeded with the botox injections a few weeks ago and, horribly enough, my husband had no response. Matter of fact, in my opinion, he is worse.

More choking, more coughing, and increased hoarseness. We are so frustrated and disheartened because the ENT said if he fails botox he will most likely fail a surgical myotomy.

Please, I’m begging ANYONE… is anyone able to provide us any hope? My husband was very active, a retired Marine, and full of life. He’s down to 130 lbs despite bolus tubefeedings daily. He continues to work and really is trying to stay as positive as he can… but I can tell this is taking it’s toll.

It surely is on me as well. I’m an RN with an MSN who works in clinical research. There have been few retrospective trials that evaluate crycopharyngeal myotomy post failed botox. One study indicated a 70% success rate, but there were fo few patients evaluated that the evidence was not statistically compelling. We have yet to call the ENT because we are hoping for a delayed onset of the botox being possible… however it’s not looking good. The ENT has us on the schedule for the myotomy on March 15th, but he isn’t aware that the botox has failed (we haven’t been exactly forthcoming about it yet because we’re hoping the surgery could help… again… praying it’ll help.)

Does anyone have any input or ideas for us??

#2

Your post broke my heart. You need to seek advice of a GI doctor that has experience with this rare disease or a thoracic surgeon who specializes in esphogus issues. I have both I had end stage achalasia they opted not to do Botox because they didn’t think it would work and it would create too much scar tissue which would hamper surgery when needed I have had a heller myotomy with wrap a redo heller myotomy and am 4 years post transhiatal esphogectomy. Doing well and enjoying life now. No feeding tube no spasms no medication. You must put together the right team of doctors it is a lot of work but I am glad I did it I feel so confident in my doctors that I never worry about or question my care. The other doctors I had told me it was all in my head. Doesn’t sound like it to me or my new doctors. I live in Boston MA so it was not that difficult to find a few doctors who knew about achalasia. I went to Brigham and Women’s hospital. But I know Mass General specializes in it as well as Dr Rice at the Cleveland Clinic. Best of luck hope this helps you out. You and your husband will be in my thoughts and prayers


Tricia
#3

You do not indicate where you live. I am in the Seattle area and go to Carlos Pelligrini at the UW Medical Center. He is the only doctor in this area who is a specialist in achalasia. I’ve heard others who say that Dr. Rice at the Cleveland Clinic back east is the specialist there. They are few and far between.

#4

We live in Amarillo, Texas. The ENT I finally found is in Lubbock, about 2 hrs south of us. Wonderful man, very helpful and concerned, but does not think surgery will be of benefit if the botox fails.

Tricia: this achalasia is a bit more rare and affects the upper esophageal sphincter. I've not had problems finding surgeons who can do the lower esophagus... it's the ENT's who handle the UES. We have none here.

I've pulled tons of research articles and have yet to find ANY that discourage the use of crycopharyngeal myotomy in severe cases like my husbands. Matter of fact, it seems to be the definitive treatment option when all other interventions fail.

I guess my frustration is that it has taken years to get anyone to even listen to my husband. Now that we have, he may not proceed with the surgery. My husband is 52 yrs old... he's not ready to be a tubefeeder for the rest of his life if there are still other options. :(