Tag Archives: CMT

Try to Behave

21 Aug

A few weeks ago The Squire asked our GP about the arthritis in his hands. That good man suggested the problem might be my husband’s CMT* attacking his upper extremities.

A trip to a neurologist, Dr. Thomas, who agreed that this was the problem. He was surprised that The Squire had not even gotten a diagnosis until he was in his mid-30s, and astounded that he was in as “great shape” as he is. Most people with CMT are in a wheelchair – or worse – by their 70s. They discussed the three-times-a-week trips to the gym; keep on keeping on, but try not to overdo it. While The Squire can prevent too much more deterioration, he can’t build muscle, because there really isn’t anything there to build on. The doctor didn’t think the hearing loss was related, as there are no muscles in the ear.

“Now, let’s take a look at those hands.” A nerve conduction test was scheduled and performed, and there is, not surprisingly, a good deal of weakness caused by the nerves dying. One thing the doctor pointed out was that The Squire should be very careful of where his hands are and what they are doing. “You may think you have an item in your hand, but drop it because you don’t have a firm grip on it. Also, when you reach for something, your hand may not go as far as your brain says it has, so be careful of that.” Twice, The Squire has grabbed for the egg turner and touched the side of the frying pan instead, and now it makes sense.

So – he came home after getting the results of the nerve conduction test, and relayed all of this good stuff to me. He wandered around a bit, and then came into the kitchen to clean a bag of fresh string beans I’d gotten from a friend in our knitting group. “I have to find a way to keep busy without messing up my hands and feet any worse than they are.”

Today, he went outside and took rocks out of the stream, tossed them into the cart, and took them away to toss into another spot. So much for not messing up has hands and feet.

Try to behave yourself.

*CMT –  Charcot-Marie-Tooth syndrome is named after the three doctors, Jean-Martin Charcot, Pierre Marie, and Howard Henry Tooth, who first clinically identified it. It is an hereditary condition which causes the nerves to die and the muscles atrophy. If it “kicks in” when you are a child, the bones “warp” to a certain extent to accommodate this, but if it begins in later years, the pain of the tendons pulling against the bones is so severe that some patients have a leg amputated, rather than endure the torment. There is no cure, only palliative care. The Squire has donated his body to the anatomy board.


16 Oct

For several weeks, The Squire has been complaining about a pain in his belly. And, for several weeks, I have been telling him to call and make an appointment with the doctor.

This morning, at 0-dark ugly, he slammed into the bedroom to tell me the pain was so bad he needed to go to the ER.

We got there at 6:45, and when I left at 9:30 the only thing they had done was to take his vitals. He called me at 10:30 to say they had drawn blood, and again at 11:15 to say he was in a cubbyhole and had seen a doctor, who had also marveled over his poor CMT feet* and ordered an IV, because he was dehydrated. Last time he got dehydrated, they kept him for four days.

At 2:00 he called to say they had done a sonogram, and were going to take him down for a CAT scan.

In between, I got a call from Eldest Daughter and the Rector’s Warden. We never miss church, and today, of all days, I was scheduled to be chalicist, so this caused a certain amount of consternation.

The Squire called again at 4PM to say he was free to go. When I picked him up he said they had wanted to keep him for IV therapy, but he refused. Gee, thanks, Boss. So, he has two prescriptions, which he can’t fill until tomorrow morning, and he will call the doctor, by gum. And don’t tell me if you get seriously dehydrated; I’m not buying it.

It’s a good thing I love that man, that’s all I can say.

— – – – – –

*This is pretty much SOP. We can’t begin to count the doctors (other than neurologists) who have told us “I studied CMT* in medical school, but I’ve never actually seen a patient.”  This is why we have both donated our bodies to science.

*Charcot-Marie-Tooth is a genetic degenerative demyelination of the peroneal nerves. (And doesn’t that sound impressive?)  As the nerves die, the muscles wither. If you develop it as a child, the bones “warp”, but if it kicks in when you are an adult, the pain is so unbearable that sufferers often have a limb amputated to release the tension of the muscles pulling against the tendons and bones. It was named for the three doctors who first described it: Jean-Martin Charcot, Pierre Marie, and Henry Howard Tooth.

Good News and Bad News

8 Dec

The good news is that I have felt better for the last three weeks than I can ever remember. Oh, I still have trouble with my thumb, and always will, I’m afraid, but other than that, both physically and emotionally, I can not remember feeling this healthy for the last twenty years or so. A few tweaks of my meds, and a shot in the back, and I am ready to party.

The bad news is that The Squire is having major trouble with his feet. He was in dreadful pain yesterday, and this morning I called his podiatrist, and the doctor had him come in right away.

The bones in his right foot are collapsing.

He has Charcot-Marie-Tooth syndrome (CMT), which is a degenerative nerve condition. As the nerves die, the muscles atrophy, and if it hits when you are young, the bones “warp” as the muscles pull away. (If it “kicks in” when you are an adult, the pain can be so severe that the patients {usually men} opt to have their foot amputated, so in that sense we are quite fortunate.) The condition is osteoclastic – it leeches the calcium from the bones – and this is what is causing his foot to simply “melt”. He has already lost the bones in several toes, and now it seems to have begun working on this foot.

The doctor says a new pair of shoes, built to support the foot and force it into position, rather than rolling to the outside as it is now, will help “for a while”. Ultimately, the doctor suggests surgery to fuse what bones there are.

Everything that has ever been done to his feet has made the problem worse, rather than better, so he has already ruled out surgery on one level, but he is looking to get a second opinion.

And through it all, he is the sweetest, most even-tempered person I know.