Son with ocular MG
 

Hello,

My 17-year-old son was diagnosed with a positive blood antibody AcHR test this past Sept. His symptoms are double vision and droopy eye.

He has been on Mestinon 60mg 3x daily and was started slowly on prednisone 6 weeks ago, now been at 60mg prednisone 1x per day for the past 4 weeks.

The left eye was unable to turn left but once we hit 60mg, after almost 2 weeks, it moves again. The eyes look wonderful, lids perfect, etc. Thank God. But he still is wearing the eye patch as he has double vision all day long.

Saw neuro this week, he is starting to change his pred dosage to create an every other day schedule, to give his adrenals a chance to recover. He wants us to add Imuran soon as he weans him off the prednisone.

I read that Imuran has a slight risk of a deadly form of lymphoma in adolescent males and skin cancer (which runs in the family - he gets checked all the time). I am hoping this eye snaps back into shape before I have to put him on another drug.

Any advice anyone can offer would be great. I am reading everything I can from past posts as well. Do any young men on here take Imuran? CellCept and cyclosporine are options as well for him but I don't really want to start any new drug if this vision issue can be zapped out and controlled without them. I'm sure he will probably have to take one of the steroid-sparing immunosuppressants one day, but hopefully when he is older. I hope this eye works again so I don't have to make this decision now.

Thanks,
Tammy

Tammy,

Welcome to the forum!

I'm sorry that your son has MG, but it is manageable. It might take some time to figure out what the right balance of drugs and lifestyle change will work for him.

I am concerned that your neuro would not START Pred with every other day. Now, after six weeks on Pred, your son's adrenals might already be "in sleep mode" and not be able to handle a drop in 60 mg!

I have known many people whose neuros have made a huge drop such as that in their "every other day" dose after they've been on it for a while and it caused their patient to have an adrenal crisis. If your son has symptoms of extreme fatigue (sleepiness), sweating, increased muscle weakness, or any other odd signs, PLEASE go to the ER. An adrenal crisis can be life-threatening.

http://www.cssassociation.org/patien...an-mesker.html

http://www.mayoclinic.org/prednisone...s/faq-20057923

Also, please talk to the neuro about this, too! The "hormone" field is not their area of expertise. It is that of an endocrinologist. Sometimes doctors can be too cavalier about drugs.

Ocular symptoms are difficult to get rid of except with those strong drugs. Choosing which drugs to use are a very personal and hard choice.

IF he goes on Imuran, his doctor needs to do the TMPT test first.

http://labtestsonline.org/understand...tpmt/tab/test/

http://www.ncbi.nlm.nih.gov/pubmed/19129430

http://emedicine.medscape.com/article/1829596-overview

Are there cancer risks in your family's history? That might impact his choice.

This is an odd side, but did you know that boy's brains do not fully develop until about age 25? Think about what those drugs might do to influence that, too! ;) And I have to wonder what effect they would have on the younger immune system.

Has he read up on MG?

www.myasthenia.org
www.mdausa.org

He should know that heat can make MG exponentially worse. It is one of the causes of an MG crisis, where a MGer's swallowing, breathing, or general strength tank. This can happen quickly and there is no way to know how bad it will get. In that situation, he would need to dial 911. I'm not saying this to scare you, but to make you aware of what can happen. It doesn't mean that it WILL happen! ;)

There have been some younger people on here who have used Pred or other immunosuppression. Again, they do come with side effects and only you guys can figure out what to take or not.

There's a lot to know about MG. I hope you'll take the time to read up on it. He has to know that pushing MG is not a good thing. Alternating an activity with rest, even if he's on drugs, is a smart thing to do. He might need more sleep or a nap during the day.

If MG has not affected his breathing muscles yet, this might be a good time to have baseline breathing tests done. That way, a pulmonologist will know the difference between his normal and what the chest wall muscles are like when they are weaker.

MIP (maximum inspiratory pressure) and MEP (maximum expiratory pressure) are breathing tests that show how well a MGer is doing at breathing air in and out. It's very important to include these tests in the pulmonary function tests that are done.

A pulmonologist is a very important doctor to have for someone with MG. They also work with neurologists in a hospital if a MG patient ever has a MG crisis.

Tweaking the Mestinon dose amount or time in between doses can take time to figure out what is the optimum schedule for him. A larger dose is not necessarily better. Too much acetylcholine is just as bad as not enough.

Myasthenic Crisis: Not enough acetylcholine getting to the muscles due to not enough Mestinon or other drugs.

Cholinergic Crisis: Too much acetylcholine getting to the muscles due to too much Mestinon.

If someone is more active, taking Mestinon every 3 to 4 hours can be better. Do not change a dose unless you speak to the prescribing physician first and set up some parameters of what kind of changes he can make.

What else can we help with?

I hope the taper goes well and that you can figure out what to do next. This can all be very overwhelming, so I hope you will also take some time to let it sink in! It's rough knowing that your son is sick and not knowing exactly how to help. Don't forget to take care of yourself as well. :hug:

Annie

Hi Annie,

Thanks for your reply! You are very informative.

The neuro started him on 10 mg pred, went up to 60 because his eye was frozen - couldn't turn left. I took him to a neuro opthalmologist and also to an MG expert at Mt. Sinai Hospital in Manhattan and they all agreed on the treatment, so I stuck with it.

His regular neuro said he is slowly moving him to every other day prednisone. He's currently on 60mg per day and the schedule is to be as follows:

This week: 70 mg one day / 50 mg the next day, etc.
Next week: 80 mg one day / 40 mg the next day, etc.
Following week: 80 mg one day / 30 mg the next day, etc.
Following week: 80 mg one day / 20 mg the next day, etc.

This is to go on for weeks and he said the alternating day eventually will be eliminated after time and give the adrenals a chance to "wake up" on that off day.

This slow movement doesn't sound like something that would cause an adrenal crisis, does it? It makes sense.

I worry about him wanting to add Imuran during week 4 to compensate and get into the system before he lowers the prednisone any further. I really don't want him to take Imuran right now if his eye gets better.

Currently he has ocular MG, but I gave emergency info to his teachers and school and I spoke with him about what could happen and what to do if emergency. I hope to God this doesn't happen to him. I would think there could be warning or build up of other symptoms first from going from eye probs to breathing crisis.

I will be asking for help often! I appreciate your guidance. It is scary. Thank God there are drugs for this but now I have to help decide which ones until he can decide for himself one day.

I will be making another visit to Mt. Sinai before deciding on adding another drug. I hope this eye continues to improve and stabilizes so we can see how low we can go with the meds and keep the eye working correctly.

Tammy

It's great that you have good doctors.

The taper of Pred all depends upon what his symptoms are on the alternate days, up until he takes the higher dose. If he notices ANY of the symptoms I described, he/you should contact his doctor immediately. OR go to the hospital for guidance. An adrenal crisis is nothing to mess with.

Some people can do drops like that, others can't.

What bothers me the most about the taper is that they're keeping the 80 mg. steady. That means he'll be on a adrenal roller coaster ride! A taper should include dropping the every other day dose as well. I understand that his eye symptoms were better at the higher dose, and that might be why they are thinking of keeping it high. HOWEVER, that sets him up for disaster down the line when the difference between the two days is higher!

Yes, having MG can be scary, but as long as he is aware of how MG can progress, etc., he should be fine.

I cannot stress enough about hot weather (or being heated up by activity or infection, etc.) making MG worse. If he does get hot, he needs to cool down his entire body right away, with a cool shower or fan or cook packs.

MG rarely stays as ocular MG. Perhaps that's why they did the Pred so soon. Some doctors think that if they stop the immune process before it progresses to generalized, there's a better chance of it not doing so. The studies are mixed on that, though.

There are certain drugs that can make MG worse, too. He should NOT have quinolone antibiotics or Ketek/Telithromycin. Ketek can put a MGer into an MG crisis. Look at the www.myasthenia.org site for more info.

Stress, lack of sleep, surgery, and anything else you can think of that would stress out the immune system (bad food) can make MG worse.

Sometimes MG patients also have other diseases (celiac, thyroid, pernicious anemia, etc.). FYI.

The members here are great! Their experiences, knowledge, and support are invaluable. Ask as many questions as you need answered!

Has anyone mentioned a thymectomy yet? Have they done a scan of his thymus?

Annie

Tammy,

Welcome to the forum. It is a great place for sharing knowledge and support.

Here are two links to helpful resources.

The first is a free PDF document from the Myasthenia Gravis Foundation of America (MGFA). You can download and print a copy.

Myasthenia Gravis: A Manual for the Health Care Provider (PDF)

The second is great primer on Myasthenia Gravis from the Medical News Today Knowledge Center:

http://www.medicalnewstoday.com/articles/179968.php

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Imuran is an immune suppressor. Immune suppressors can take 3 to 6 months before they become effective. Also do a bit a research on Imuran and the other popular choice, Cellcept. Most here favor Cellcept. One of my former Stanford neurologist strongly recommended Imuran. My other former Stanford neurologist strongly recommended Cellcept. What's a patient to do?

Have your neurologists mentioned the thymus? Approximately 15% of MG patients have a tumor in the thymus gland, usually benign. Have your neurologists order a chest CT Scan? If there is an issue with the thymus, it can be removed. The good news is that a thymectomy has greater success rates for the young.

Tapering off of Prednisone is a tricky thing. My first taper failed badly, ending in a major MG crises that required a 6-day stay the hospital, and a 5-day course of IVIG. In short, my first taper dropped too quickly, and without the introduction of an immune suppressor.

This time around I am reducing my Prednisone dose by 5 mg every two weeks. From a high of 60 mg, I am now at 45 mg for another week, then dropping to 40 mg for two more weeks. This time aground I am also taking 1500 mg of Cellcept. I have been taking Cellcept for 3 months now. By the time I get down to 15 mg of Prednisone each day, I will have been on the Cellcept for 6 months.

-Mark-