Update from Michael re: Myotonic Dystrophy

Thanks Michael for this contribution.

    You can ask me what's new and I will usually, without thought, give you the same tired answer, "Not much" or sometimes I'll say, "Not much, what's new with you?"

If I really paused and gave it some thought I would realize that there is plenty new with me. Some of it good, some of it bad. A few things inconsequential, but most of the inconsequential will probably be glossed over or not mentioned at all.

I, of course, have continued to age and with age comes changes, more experiences, which are hopefully accompanied by wisdom and maturity. Wisdom and maturity aren't always a sure thing.

But the big things are as follows: I got a pacemaker and defibrillator (one thing) put in me (not the best thing, but at least I won't die from a faulty heart). I have a tablet that has a text to speech app on it to help with my speech (the tablet has a keyboard attached, so i don't have to individually tap every single letter). I have a benign fatty deposit (a lipoma) on my back that doesn’t need to be addressed unless it hurts. I'm about to get a job coach (if all goes as planned [this is the third or fourth try]).

But the biggest change is my life? I've been having trouble staying awake for the entirety of the day and reading any involved books for, at least, a year. We've been trying medication (primarily Provigil and Ritalin) and they work, to an extent. I still fall asleep earlier in the evenings than I used to. By early I mean 7 or 9 pm depending on the day. I can't even stay awake on some nights, even if we've rented a movie I want to see or we're watching it on Netflix or through DVR (yes, I don’t watch any shows when they are actually airing). Thankfully, I live with my brother so he can wake me up when bedtime rolls around or before (sometimes he needs help waking me, so he calls my mom).

However, why is this happening, I love books and movies, they used to be what I would use to escape from life, with a dash of video games for good measure. We were clueless what was causing this until November 2017, when we went to see the pulmonologist . She used a device to test my CO2 (Carbon Dioxide for the layman) and I had higher levels than the average person is supposed to have (I was at 52, the average is supposed to be 35-45, or even lower at our altitude), apparently my lungs aren't strong enough to expel all of the CO2 in my lungs. Too much CO2 can naturally cause tiredness. I am getting a machine (it's called a Trilogy) to help with my CO2 levels and, while it might take a while to see any change (somewhere between 1-3 months) I am hopeful it will work. I am trying to become more vulnerable and open and I’m hoping the machine will help me be alert enough to accomplish this and many other goals in my life.

Oh yeah, there’s also the small detail that my head, due to poor muscle tone, is hanging low lately and I’m experiencing a loss of balance, but those are closer to being addressed than the rest.

Michael Woodbury

Guest blogger: Jonathan Williams

I met Ann at a writer’s conference in the fall of 2016. When I told her what I did—helping people write their own books and tell their stories—she told me she had a story to tell and wanted to know more. Ann impressed me as someone who was easy to get to know but who was also serious about telling her story and about caring for her family.

Over the next few days at the conference we got to know each other better, and a week or so after the conference ended, I started helping her tell her story. The result is a book the both of us are proud of. It tells the story of living with myotonic dystrophy, and the obvious, and not so obvious, challenges Ann has faced. I’m biased of course because I think Ann’s book is great, but it seems that I am not alone—everyone she has shown it to seems to gobble it up. The reason for this is simple: Ann opens up and tells the truth as she gives us the inside story of her life. Naturally, everyone who reads it is thankful that she has let them peek into her world as she tells her family’s story without holding anything back. Now, Ann didn't write her book to please anyone or to be nice to anyone or to impress anyone. No, she wrote her book to share her joy and her pain. And by doing so, her readers can see that they too can have joy in the midst of pain, whether they care for someone with DM or with some other illness.

In the course of helping Ann with her book, I got to know a couple members of her family. At the conference where I first met her, Ann had her son Michael with her. Of her four children, Michael suffers the most from myotonic dystrophy. As is typical with this disease, his immune system is compromised and his muscle tone is diminished, which leads to a host of problems, including digestive issues, problems swallowing and eating, and (for Michael in particular) problems speaking. You see, when Michael speaks, he has difficulty forming plosives (sounds like b, t, d, etc.), and Ann had to translate whatever he said for me. But Michael’s speech issues don’t hide his happy and vibrant spirit and personality. Having myotonic dystrophy hasn’t prevented him from smiling or cracking jokes, luckily, so he does both a lot. Later on, I also took the opportunity to attend a doctor’s appointment with Ann, Michael, and Chad at the University of Utah Clinic, and I got to hear about the extensive list of medications Chad and Michael have to take daily. I saw firsthand a simple test performed on Michael by their doctor, which demonstrated that he had this form of muscular dystrophy. It was a fascinating and a sobering moment to see how this profound disease could be diagnosed so simply.

If you want to know more about the physical and mental effects of DM, Ann’s book is as good a place as any. I’ve mentioned a few of Michael’s physical effects, so I won’t repeat those here, but I do want to mention the one mental effect that disables people with DM more than you might realize, and that is their impaired executive functions. Most of us, who don’t suffer from DM, take our ability to make decisions and prioritize our daily tasks for granted. But for most people with DM, these are often monumental tasks. In her book, Ann tells about a time when she took her oldest son grocery shopping after he had moved out on his own. When they got to the frozen food section and she started to ask him what vegetables he wanted to get, his brain overloaded, and he sat down in the middle of the grocery store and couldn’t move. Ann had to end up just choosing some veggies for him and finally got him to get up and keep going. There’s more experiences like this in her book, so please read it to find out more, but that one is typical and illustrates one of the biggest challenges people with DM face.

Being as familiar as I now am with Ann and her family’s story, I know well that raising and caring for her husband and her four children has been anything but easy. Still, through writing her book and through serving in her community and at home, Ann has kept the focus in her life on serving others.

Ann’s story is still with me. Even though you may not know anyone with this debilitating disease, her story should still speak to you. With all of the suffering and uncertainty in our world today, the time is right for a story like Ann’s.

Jonathan is a co-author, writer, and ghostwriter. His email is ghostwriter@jwghostwriter.com and his website is www.jwghostwriter.com.

Kent and Andrea update

We have had illness at our house. Kent (who has cancer along with his myotonic muscular dystrophy got real sick and ended up at Huntsman Acute Care (Huntsman ER). They gave him a LOT of fluids and ran a full panel of bloodwork to determine what he had. It was determined that he didn't have the flu but that he had a virus called Parainfluenza type 1. This is typically an upper respiratory virus but it hits those with comprimised immune systems harder than most. Huntsman sent Kent home with a cocktail of virus medicine, including an incredible pill called Tesla Pearls that numbs the reflex in the lungs that causes the urge to cough. If Kent doesn't have these pills he coughs so much that he throws up and/or can't get any rest. Kent missed a few days of work and if you know Kent, that means that he was very sick. He never misses work for anything.

Today we are up at Huntsman and Kent is having an infusion of IVIG (Intravenous Immunoglobulin). Kent's Immunoglobulin G numbers are very low and that affects his ability to fight off infection. The IVIG is a human blood product containing the pooled immunoglobulin G from the plasma of approximately a thousand blood donors. This infusion causes quite a few reactions so it will be an all day visit while they watch him and then he will go home with me to have me watch him. It will take a couple of days for him to get his strength back.

I got Kent's virus but it hasn't hit me as hard as it hit Kent and I am almost recovered.

Last week I went with Andrea to see the cardiologist, Dr. Daniel Cox. Andrea had to have a cardiac MRI the week before. These are expensive and we'll need to help her with her deductible and co-pay. At the visit they told Andrea that her heart has a few issues but not severe enough yet to warrant a pace maker. They did put on a 2 week holter monitor so that they can double check the other test results. Assuming that the holter monitor doesn't show anything new, she won't need to be seen for another year. Because her brother's have pacemakers she is genetically extra vulnerable so they will watch her extra close.