Decision Advice

[KevinM]

Hello everyone. Apparently, I am the king of rare pathologies, and I would appreciate any thoughts on a decision I must make. Sorry in advance for the long post, but there is a lot to describe.

I was diagnosed 8 months ago with LMN dominant ALS, and thus far still have good function. No bulbar or breathing issues, arms and legs still work. Progression is more systemic weakness and some atrophy in both shoulders, but other than that I’m holding my own.

In 2016 my colonoscopy revealed diverticular disease in sections of the sigmoid colon, and two months ago I developed an uncommon colovesical fistula (a small opening) between my bladder and colon caused by diverticulitis. Essentially, air and small amounts of fecal material leak into my bladder, so I occasionally have air and/or debris with my urine. My symptoms are more annoying than uncomfortable, and I manage them with very little discomfort with daily GasX and a couple of Imodium.

For healthy people, surgery is recommended, but this is very major surgery involving colon resection, a week’s stay in the hospital assuming no complications, significant weight loss, and about a 2 month recovery period to regain strength/weight. It is a much more involved and difficult surgery than a resection for colon cancer, even though it is of benign pathology. The likelihood of accelerating my progression from this type of surgical trauma is high in my opinion.

Alternatively, conservative management through intermittent antibacterial therapy is often used and has been the treatment for me for the past couple of months. Nevertheless, I decided to have a consultation yesterday with one of the more renowned colorectal surgical specialists in the country, Dr. Thomas Read of UF Health (Shands hospital in Gainesville FL) to get his opinion on whether or not I should consider surgery since I’m still healthy enough for intubation, etc. He said that if I didn’t have ALS, he would recommend surgery, but I’m right in that it could advance my ALS progression. But he also said chronic, low grade infection/inflammation, which I will always have as long as the fistula is present, could accelerate progression as well. Bottom line is he doesn’t know enough about ALS to make a recommendation how to proceed. Either way, I run the risk of speeding things up either way.

I’m strongly leaning towards the non-surgical approach, since there are too many unknowns and risks of surgery, but I’d like to get any thoughts from you good folks. Has anyone here had major surgery involving general anesthesia, and if so, did you recover without acceleration of progression? I read one ALS research paper that described significant acceleration of progression in the 3 months after major surgery as compared to the three months prior, but that’s all I could find.

Thanks for any thoughts. Kevin

 

[Nikki J]

I would ask Dr Pulley who knows and sees you.

if I had that issue I would pick surgery unless my neuro strongly opposed. My reasoning is the one big hit vs chronic stress coupled with my progression rate. However if I had a progression like my sister’s I would do the opposite

 

[KevinM]

I would ask Dr Pulley who knows and sees you.

if I had that issue I would pick surgery unless my neuro strongly opposed. My reasoning is the one big hit vs chronic stress coupled with my progression rate. However if I had a progression like my sister’s I would do the opposite

Thanks Nikki. I put a call into Dr. Pulley today. I’ll let you know what he says. There is also the problem of scheduling surgery if I did choose that route. Dr. Read is booked out a couple of months, so there is the probability I will be somewhat weaker than now. So many risks with this surgery...

 

[KarenNWendyn]

Kevin, I agree with Nikki. Your ALS is highly atypical and as I recall you had an EMG which wasn’t exactly diagnostic. Did you ever have a followup EMG confirming the diagnosis? I ask because it’s possible that you were misdiagnosed, or.... you have a MND that is so slowly progressing that the kind of surgery you mention won’t accelerate progression.

I’m also assuming you have been ruled out for inflammatory bowel disease (Crohns or ulcerative colitis) as opposed to a fistula from diverticulitis as Crohns and UC can cause systemic symptoms.

 

[KevinM]

Kevin, I agree with Nikki. Your ALS is highly atypical and as I recall you had an EMG which wasn’t exactly diagnostic. Did you ever have a followup EMG confirming the diagnosis? I ask because it’s possible that you were misdiagnosed, or.... you have a MND that is so slowly progressing that the kind of surgery you mention won’t accelerate progression.

I’m also assuming you have been ruled out for inflammatory bowel disease (Crohns or ulcerative colitis) as opposed to a fistula from diverticulitis as Crohns and UC can cause systemic symptoms.

Thanks for the input, Karen. I’m actually surprised that both you and Nikki would opt for surgery. It is high risk, and I’m already struggling to maintain weight, so I would probably leave the hospital at 130 lbs on a walker. That’s assuming no complications, which are many that can occur with this surgery. I feel okay now, and I’m afraid to risk that.

I have no symptoms associated with Crohns or UC, and Dr. Read felt that since there was no family history and no related symptoms, and since I was confirmed to have diverticular disease, he felt strongly that was the etiology.

I did not do a follow up EMG. Too many tests already. I am getting significantly weaker and have noticeable atrophy in the right shoulder and tricep, and to a lesser degree in the left shoulder, which is what Pulley predicted. I was diagnosed very early in the disease, and it is now starting to move along. Kevin

 

[Nikki J]

Just to be crystal clear- What I said was I would have the surgery for me With that diagnosis and my neuro’s consent. I can not / should not have an opinion for you which is why I said consult Dr Pulley.

Dr Pulley is an excellent neurologist so he must have a reason but I remain surprised that he diagnosed you without an EMG meeting criteria especially as you state you are LMND also given the GI issues

good luck whatever you decide

 

[KarenNWendyn]

Kevin, obviously we on this forum haven’t had the privilege of being able to examine you, have access to all your test results and other records, etc. So by no means are we attempting to step on anyone’s toes. I’m just trying to raise concerns I hope were addressed.

I do recall when you first came here you were also mentioning something about anal sphincter tone. Crohns is worthwhile exploring because it can affect sphincter tone, and can cause fistulae between the colon and bladder, and also fatigue, weight loss and overall wasting.

I do wish you the best and I’m sorry you have to go through all this.

 

[KevinM]

Just to be crystal clear- What I said was I would have the surgery for me With that diagnosis and my neuro’s consent. I can not / should not have an opinion for you which is why I said consult Dr Pulley.

Dr Pulley is an excellent neurologist so he must have a reason but I remain surprised that he diagnosed you without an EMG meeting criteria especially as you state you are LMND also given the GI issues

good luck whatever you decide

I understood that your opinion was for you given similar circumstances, Nikki.

I know of no GI issues related to diverticular disease that causes clinical weakness progression, atrophy, and fasciculations. I would give anything for misdiagnosis, but it is highly unlikely.

 

[Nikki J]

Was celiac ruled out? It is a known mimic

 

[KevinM]

Was celiac ruled out? It is a known mimic

No, but my GI issues are in the colon, not the small intestines. Worth talking to my PCP about, though.

 

[Dee Dee 0617]

Hello, So obviously Nikki and Karen are the science gurus here. I want to clarify a couple of things if you wouldn't mind. Am I correct in understanding you have not been diagnosed with ALS? To date you have shoulder issues? Why wouldn't you have the surgery? If you don't have a confirmed ALS diagnosis. You need the surgery. If you do receive a confirmed ALS diagnosis. It's up to you. Seriously at the end of the day you really can only die from one thing at a time.

 

[Nikki J]

to clarify DeeDee Kevin was told he had ALS by Dr Pulley an ALS specialist

 

[KevinM]

to clarify DeeDee Kevin was told he had ALS by Dr Pulley an ALS specialist

I would add that he provided written confirmation of my diagnosis on June 6, 2019, and I am now on Medicare and SSDI.

 

[lgelb]

Kevin, I would want to know the probability of/likely extent of/implications of change in the fistula's status. Not knowing your height, 130# may be a low weight for you and major abdominal surgery is not a recipe for maintaining it.

Also, if you plan a feeding tube, I would ask the CRS whether/to what extent would the fistula interfere with either a radiological or surgical placement? That would also go to planning for the tube if that's your plan.

 

[KevinM]

Kevin, I would want to know the probability of/likely extent of/implications of change in the fistula's status. Not knowing your height, 130# may be a low weight for you and major abdominal surgery is not a recipe for maintaining it.

Also, if you plan a feeding tube, I would ask the CRS whether/to what extent would the fistula interfere with either a radiological or surgical placement? That would also go to planning for the tube if that's your plan.

Great points, Laurie. Dr. Read couldn’t provide a prediction on whether the fistula might grow larger, stay the same, or even shrink. He did say that spontaneous closing of a bladder fistula was quite rare, and it most probably won’t close on its own with conservative management.

I am 5’9 and currently weigh 147 lbs. I have dropped three pounds over the past month, possibly due to the fistula and the fact I’m modifying my diet away from gluten and dairy to reduce the gas in the colon. Less gas means less fistula symptoms, but it also means I’ve been eating a bit less. I based the 130 lbs. on the fact that colon resections typically involve a 10-15 lb. loss, and I fear I won’t be able to gain it back if I have the surgery.

My advanced directive presently states that I do not wish to have a feeding tube, but since I am still (hopefully) a good ways away from that decision, I may change my mind when the time comes. I’ll make sure to inquire in case I change my mind. Kevin