Primary Lateral Sclerosis uncovered

In our monthly newsletter, someone with PLS (the sort of MND I have) sent in this description by a chap called Galen trying to explain the condition. It’s rather good. (I hope he won't mind me quoting it verbatim. As you can tell he has a sense of humour - which is quite helpful in the circumstances.)

If I start telling people about upper and lower neurons I can almost see their eyes glaze over as they decide I must have some need to tediously explain PLS. They then avoid me. If I launch into how PLS may be a "gentler and kinder" form of ALS, or Lou Gehrig’s Disease, people often fixate on the ALS part and have even asked me why I'm not dead yet and how much longer do I expect to live. When I tell them I have a rare condition that causes a loss in communication between the brain and motor functions, most people feel I've been technical enough and sufficiently succinct.

If folks are still curious I can launch into examples. Walking over to a table to set down a glass of water is something that is done almost without thought by most people, however, the communication with my brain and the rest of my body has gotten so that I have to concentrate hard to even walk to the table. Trying to carry a glass of water is likely to overtax the limited capacity I have remaining, with the likely result being that both I and the glass will wind up on the floor. Or it can be more insidious. If I'm about to get out of a chair I may have devote virtually all of my resources into planning and executing exiting the chair, and may actually wind up ignoring anyone trying to talk to me while I try to stand. It isn't because others are boring or anything, rather I have to focus so hard on getting up, it blots out other things.

It's not a problem with the brain, we remain as sharp as ever. It's not our muscles, they work fine. It's communication between the two that is the problem. Walking requires intense communication with our balance sensors, our brain, our legs, feet, even our toes. Our consciousness isn't aware of it, yet it requires constant fine- tuning. Throw a monkey wrench into that communications system and you wind up with someone who can barely stagger anymore, a symptom many of us can relate to. Sometimes one side is affected more than the other, and you get someone like Ronnie, or it can be pretty symmetrical, like Flora, or it can add to an already serious personality disorder, like me.

It can have strange results. You can be at a funeral and your brain can be saying: "Whatever you do, don't make a scene," but because the message gets garbled, your mouth muscles say: "Roger, brain. Commencing uncontrollable laughter immediately." Or you can think you told your mouth to say to the UPS guy: "I'm feeling fine, and you?" only to have him look up at the sky and say: "You're right, it does look like rain."

We are constantly compensating, whether or not we are aware of it. Maybe that is why we get tired all the time "for no reason." We are always having to figure out new ways for our brain to signal our muscles to do stuff. Our brain may be so busy devoting time to keeping essential functions going that it can't worry about things like walking and talking.

This Humpty Dumpty life

We had one of those excellent lunches which occasionally happen on Tuesday. It was with our friends, Peter and Ann. Peter, like me, has PLS. Like me, he was a teacher. He seems to have an endless supply of jokes - which is good medicine. We met in the coffee-shop at Bicester Garden Centre (not up to Cornerstone in Grove, of course, but not at all bad).

We had a merry time comparing notes.We have very similar stories, we realised, although Peter was diagnosed about 5 years before me and has only just stopped driving. He tells me 1 in 5 million have PLS. We certainly agree that our wives are 1 in 5M! He and Ann had a lovely way of describing PLS - which was something like "Not an end of life, but a change of life."

Peter, like his namesake, has a lot more physical courage than me - and so he's had a lot more falls. And he seems to bounce better. Whereas I go rigid when I fall, I think he has mastered the art of relaxing, which, as horseriders know, makes all the difference.

Last week I had an email from my friend in New York who has ALS (as they call MND). She lives in an apartment on her own - and of course there's no free National Health Service in the States. Her rollator (zimmer on wheels) was faulty. So she rang the ALSA repair man, but he failed to return her call. "I was ironically going to my desk to call ALS to see if someone else could set up the exchange, and the rollator was in front of desk - I'd been using it as a tray around my apt..... I'd sat on it before just to see what that's like. I decided to sit on it to make phonecall... and down I go!
"Hit my butt, arm and then my head went back to the wall, a part where two sides come to a point! Shocked and alone, I put my hand to my head, and all blood..., then sat on my bed thinking I could just put some vaseline on it and no biggie, but, exploring the wound, I knew it meant stitches, small but deep.... All day in the ER, CT scan and waiting for neurosurgery to attend!  Actually treatment just two staples, five minutes, and they discharged me OK...." She ended with a three-day stonking headache, and of course the worry over medical insurance - as well as a useless rollator.

Peter told me that one year he fell 255 times. I don't think he was joking.... I imagine it was when he could pick himself up. Otherwise the paramedics would have had him on their blacklist! However, such are the hazards of MND/ALS. As my friend aptly puts it, this Humpty Dumpty life. By the way, apart from stiffness first thing, my back is pretty much as it was. Thanks for your concern.

And also, by the way, my remarkable and courageous friend and fellow-author, Jozanne Moss, celebrated her birthday on Monday - what an achievement!