Heading to UC Davis
 

Hi all. I posted earlier about my 16 year old son. Last Saturday he had an "episode" where he was so weak he couldn't move his legs at all. End up in the hospital and he was admitted. This was actually a good thing...a great thing.

The attending neurologists (not neuromuscular specialists) think it's myasthenia gravis. My son's regular neuro is still convinced it's congenital myasthenic syndrome. The good part of being in the hospital is that they were able to do a CT of his thymus (negative) and insurance will now pay for a bunch of genetic testing that would not have been covered if he hadn't been inpatient.

They also started him on Mestinon (60mg 3x per day) BUT he was sick to his stomach (nauseous and vomitting) before he even started the meds, and then it just got worse because he had an empty stomach when he started the meds. They did make him stronger, though.

Because he was stronger, they sent us home, but we were on a vicious cycle with the nausea so he just stopped taking them (with the doc's ok).

We to into to see the Docs up at UC Davis mid July. They will be doing the specialized muscle biopsy, and then a consult. So I have a bunch of questions:

1) Any suggestions for dealing with stomach upset caused by the mestinon?
2) Have any of you had the specialized muscle biopsy (I guess it's combined with an EMG during the procedure)? If so, and advice about what to expect pain-wise and recovery. We will have a 3 hour flight home and I am trying to prepare to make it as easy as possible)
3) Any experience/recommendations about dealing with the docs at Davis?
4) Any experience/recommendations about staying in the UC Davis Medical Center area?

Any/all advice and/or recommendations are very much appreciated!

Taking Mestinon with food helps. What also can help is starting Mestinon at a lower dose, like 15 mg. Because I'm sensitive to meds, my neuro started me out slow. One dose for the first two days of a LOW dose, then two doses at that same dose amount. The thing about Mestinon is that more isn't necessarily better, meaning more in the dose amount. It's up to your son's doctors but sometimes increasing frequency, like 1 - 4 times a day slowly, may be better at first than increasing the dose amount. BUT don't do anything without approval from the prescribing doctor.

Here's a silly question, how's your son's diet? Does he eat any junk food? Pop? Caffeine? Before an EMG, they recommend that patients be off of Mestinon for 12 hours beforehand. Caffeine 24 hours before. Both can interfere with the test results.

Why was your son sick to his stomach? Sometimes, it can be because kids have to poop. ;) Seriously, it's one of the first questions they're asked in school if they have a sick stomach. Was it awhile since he ate? Did he eat anything in particular that would've caused that? A sick stomach isn't normal either. Oh, and did you know that a person needs acetylcholine to move stools through the GI tract?

The fact that he got stronger and not weaker, probably means that it is indeed a "not enough acetylcholine" situation. Some of the CMS's cause too much acetylcholine. That's unfortunate that he got so weak but at least, like you said, he got care and things were documented. Did he get short of breath at all?

There are two different kinds of muscle biopsies. The first one is a regular one (excisional biopsy), where they slice into the muscle, taking out pieces of it. It's painful because they can only numb the skin and not the muscle. The second one, the one they do for CMS, is usually a "procedure" where someone is under anesthesia. They often take muscle from the intercostal muscles (rib cage area) or from the arm. It's not a simple thing. If they are doing a punch biopsy, I'm not sure what that would reveal, frankly. A "shave" biopsy (a third kind) really wouldn't reveal anything having to do with the muscle. You need to ask them specifically what they are going to do. Some anesthetics can make MG worse.

As far as places to stay:

http://www.ucdmc.ucdavis.edu/aboutus/stay.html

Maps of the area:

http://www.ucdmc.ucdavis.edu/welcome/maps_directions/

As far as dealing with the doctors, how about being yourself? Be courteous, respond to questions, ask questions and just go with the flow. If you are overly "anxious," neurologists tend to put some kind of psycho-babble label on you. Let your son talk too. I think being calm, reasonable and respectful goes a long way to having a good interaction.

This is their leading expert:

http://www.ucdmc.ucdavis.edu/welcome...lli_grant.html

http://www.ucdmc.ucdavis.edu/search/...searchlist=yes

I hope the appointment goes well. If your son gets worse in the meantime, please don't hesitate to take him in. Calling 911 if he can't move like that or can't breathe well or swallow is the best thing to do! You can't always tell how quickly MG/CMS will get or how bad.

Traveling is very hard work for someone with MG or CMS. Make sure he's in a wheelchair or a transport vehicle in the airport. Keep him as still as possible in the airplane. Ask the attendants if they have anything he can sip on right away or bring water on the plane, in case he might choke. One of the worse choking episodes I ever had was on a plane. Keep him cool (not cold, not hot). He also needs as much rest/sleep as possible. Yeah, he may not like being "babied" but it's very risky to travel if he's weak.

Keep asking questions. I believe you guys will get very good care now.

Annie



And this may be too much info, but here you go anyway.

http://neuromuscular.wustl.edu/synmg.html

http://neuromuscular.wustl.edu/lab/mbiopsy.htm

http://www.ncbi.nlm.nih.gov/pubmed/15119478

One thing I do for quick absorption of mestinon is chew it up and hold it under my tongue. I have heard it produces less stomach upset doing it this way too. After it of it has been swallowed, drink a bit of water. It is not lovely to have to taste but it is manageable.

UC Davis is THE place for a muscle biopsy so rest assured you are going to the right place for your son.

Good luck and best wishes, debra

Thanks for all the info and the good wishes. We just got back from the hospital...second go around. When we left the first time (against my better judgement), my son couldn't eat or drink...and he didn't get any better at home. Two days later he was dehydrated, and cramping, so back we went. Since it wasn't a neuro problem this time, he was treated by the GI team, and finally got the whole thing under control. I'm just a mom, so what do I know, but I think he got a stomach bug, it caused a "crisis", then they gave him Mestinon on an empty, irritated stomach, that never got a chance to get better. At the hospital, they took him off the Mestinon, and decided to keep him off of it since we should be getting answers at Davis this week. He lost 12 pounds, but feels really good now, and is eating everything in sight.

Is there really no one on this board who has been treated/diagnosed at Davis?

Not very many people go to UC Davis, unless they live around there or have a CMS. Sorry you can't get feedback from people with actual experience.

What happens when anyone has GI issues, whether it's vomiting or diarrhea, is that you lose fluid AND electrolytes. Any imbalance in things like magnesium, potassium and calcium will have an adverse effect on MG or CMS. Did they do a metabolic panel on your son? It's a simple blood test that is often standard for someone with MG who is doing worse. If you don't know what's wrong, how can you fix it? ;) And you can't assume a deficiency of an electrolyte and throw a banana (potassium), chocolate (magnesium) and milk (calcium/magnesium) at it.

I hope the appt. goes well.

Annie

Two things: First of all, when your son's doctor runs blood tests, PLEASE have him add a Celiac screening. If a person has one auto-immune disease, they are more likely to develop more. Celiac Disease is more common than most doctors realize (although the CD groups have done a great job of getting information out there), and 97% of people don't present with "typical" symptoms.

A lot of people with MG say that they have benefitted from a gluten-free diet, even if they don't test positive for CD. It's best to know if he has it, though - a person with CD has to stay on a GF diet for life. It's not that hard these days!

Secondly, it's my understanding that the DOSAGE of Mestinon can affect how one feels. When I was first getting diagnosed (and all of the "standard" tests came back negative), my neuro did a medication trial. If a person doesn't have MG, they won't be able to tolerate even the lowest dosage of Mestinon. (I was diagnosed based on the fact that I could tolerate quite a bit of it at one time!)

Most neuros will start a person out at 30 mg, then work up to 60, 90, 120, etc. If a dosage is too high, the patient will know it within an hour or two, because they will feel absolutely AWFUL.

I couldn't see any difference at 30 or 60 mg; at 90 mg I felt a lot better; at 120 mg, I was on the couch feeling like I was going to die.....

Once you figure out what the tolerance level is, then you start figuring out the timespan that works best. Mestinon changes the rate at which Acetylcholine is absorbed by the body, and the body's absorption of AChE is a very delicate balance.

Most people find that rather than increasing the dosage of Mestinon, they shorten the interval at which they take it if they feel the need to take "more". Increasing the dosage makes them feel bad, and too much Mestinon will affect the AChE levels in the body to the point that a cholinergic crisis might occur.

Fortunately, the effects of Mestinon only last 4-6 hours, so if a person gets too much when figuring out their tolerance level, they will only feel bad for a few hours. It will work itself out of the system pretty quickly.

Good luck with your son's diagnosis - I'm sure it's a relief to have an answer!:hug:

They ran a genetic CD test during the hospitalization, and it came back negative, so that is definitive. Other than whatever is causing the muscle weakness, my son seemes to be remarkably healthy. Hopefully that will make everything else a little easier.

The more I read up on the Mestinon, the greater mystery it is why they would start my son on 60 mg before he was over the stomach bug and could at least try it with food in his stomach. His breathing was not compromised (the RT's all commented at how it was off the charts good) so they could have waited. When/if we start him back up, we'll go with the 30, maybe even as low as 15.

Today is the biopsy and EMG. Hopefully tomorrow we'll know more.

Thanks for the support.

Hope tests all go well and you are able to get some answers. Please let us know.

FYI...15 mg 4x/day is the way I started Mestinon....and had no troubles.

Good point, Teresa! It may be worth the antibody tests anyway. And you can have gluten intolerance, which some doctors do not acknowledge, and not have the major genetic markers. So keep an eye out for any symptoms after eating gluten.

I hope the rest of the testing goes well. And that you get answers. Hang in there, I know the waiting is very hard.

Annie

the biopsy went very well but we were surprised it was done with just a local (no sedation) and in the vascular clinic (not the hospital). We are still in Sacramento because my son's leg hurt too much to fly. It hurts to bend it (because he has to use the muscle they cut into). The docs were super pleased with the sample but it will still take a few weeks to get definitive answers. I have to say, the neuro's here have been kind, caring, communicative, patient and accommodating. I feel like I've been transported to a different planet and very fortunate to have been able to get in here, and so quickly.