Eating and Myotonic Dystrophy

This is a picture of the dinner that I made last night. It's called "Cheesy Chicken Instant Mac With Veggies" It's made in an instant pot. What's great about it is that it has chicken (extra protein) and lots of carrots and spinach but it tastes like an incredibly creamy mac and cheese. When feeding my myotonic family I have to worry about swallowing issues. Lately it's hard for Kent to eat a green salad. I was pleased with this dish because I was able to get the greens in our meal without serving a green salad. 

Sometimes people with Myotonic Dystrophy have difficulty eating and swallowing. Of all my children, Michael has had the most trouble eating and swallowing, and has had multiple episodes of aspiration pneumonia. Eventually, the only way to help him was to get him fitted with a feeding tube.

When Michael got his feeding tube, every one of his siblings said, “Oh wow, I’m so jealous.” So why did they say they were jealous? It’s because eating is so much work for my family with this disease. So when they said they were jealous, Michael said, “Well, I know where you can get one!” But despite some jealousy, the others didn’t really want a feeding tube, even though they could see an advantage to having one. Now Michael only uses his feeding tube for clear liquids but by keeping his muscles hydrated, he doesn't have choking issues any more.

The vast majority of us rarely have trouble eating or swallowing. But for most individuals who have Myotonic Dystrophy eating and swallowing isn’t something they can take for granted. Growing up, Michael could still eat and drink, but it was always difficult for him. He survived a good portion of his life without a feeding tube, and despite the difficulties that have come with having a tube permanently inserted into his body, the feeding tube has improved Michael’s quality of life. Michael’s feeding tube helps him function on so many levels that it has become a valuable trade off. We use Michael’s gravity bag and feeding tube to administer him his fluids. In our family we now have a little joke that Michael gets watered twice a day. For a long time Michael was putting just 16 ounces of fluids/water into his tube twice a day, but now he's up to 24 ounces. 

The swallowing issues are because the sphincter muscles in a DM person’s throat don’t work well and this often allows clear liquids to slip through these muscles and end up in their lungs. If those liquids are water or saliva, those are both pretty much benign, and it’s not a big deal, but having that happen is obviously quite uncomfortable. As a result, myotonic individuals often have trouble staying hydrated because they avoid these clear liquids that so often end up in their lungs. Food purees are hard for them to swallow as well, and when Michael has had to endure taking a lot of barium milkshakes for some of the swallowing studies he’s done, it’s always been difficult for him to swallow. The feeding tube then becomes a great way for DM patients to get all the liquids they need without having to try to swallow them.

Many people think that once you get a feeding tube you can never eat again. And there is something to that because if your swallowing is so bad that you need a feeding tube then maybe you shouldn’t be eating. But for many people a feeding tube doesn’t always preclude still being able to eat.

Originally, we gave Michael all of his nutrition via his feeding tube, thinking that that’s what we were supposed to do. Somehow we missed hearing that that wasn’t necessary.As long as he was hydrated, he could still eat and get all of his nutrition that way. This meant no more supplements via his feeding tube. 

Michael’s tube is technically called a flush mount non-balloon feeding tube. Michael’s even written a poem about his feeding tube and he has named it “My first feeding tube.” 

Follow Ann's Myotonic Dystrophy blog

I am SO EXCITED to announce that you can now register to follow my blog. Please go to annswoodbury.com and at the bottom of the website page you can register.

The purpose of my blog is to help educate individuals as to the complexity of Myotonic Dystrophy. Even those of us who live with it tend to forget how multi-systemic this disorder is.

I was just talking to a dear friend about the loss of her myotonic daughter due to excessive levels of CO2. Her daughters levels were over 300. It's heartbreaking that we are still losing family members because this disease is still not entirely understood.

When Michael and Chad saw the new pulmonologist who monitored their CO2 levels  I thought that their levels of 51 were excessive. Normal is 35-45. Michael and Chad have been provided with a new machine instead of bi-pap to help them with their sleep apnea. The machine is a Respironics Trilogy Active with PAP (Positive Air Pressure) Exhalation assistance. Excessive CO2 is toxic and can cause confusion, lethargy, sleepiness, and if not treated, death.

4 a.m. E.R. visits

                                          University of Utah E.R. at 4 a.m.

Last December I went up at the U of U E.R. for 2 nights, Dec. 1 and Dec. 5 with Chad and Michael respectively. We think that we are almost due for a frequent user discount :)

First Chad got the stomach/diarrhea virus and then he gave it to Michael. They likely got it from a caregiver since no one else in our family has had this bug. Neither one could stop throwing up even when we gave them Zofran (Rx anti-emetic), it was also coming out the other end. In both cases when I called Instacare, they asked a few questions and told me to take them to the E.R. Once they were hooked up to an I.V. and given intravenous Zofran, they finally stopped throwing up but by then they were so dehydrated that they had to be given 3+ liters of I.V. fluid. They are both now home and both are gradually improving. However, I'm exhausted.

Chad was scheduled for a pacemaker tomorrow but because he hasn't fully recovered they have had to postpone it to later this month.

Constant medical situations are unavoidable when you have a family with myotonic dystrophy.

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.

Myotonic Dystrophy: Doing What Needs to be Done

I can only imagine that when you have a child that has Down’s syndrome, Batten disease, or many of the other disorders that affect both mind and body, that you do what you have to do. Of course, it would be a very intensive labor of love to help them and keep them thriving. I imagine that people would think that you were awesome because of all of your efforts, and you are awesome, but you are also only doing what needs to be done.

Well, what do you do when you have a disorder like childhood or teen-onset myotonic dystrophy where your children look normal except for some male pattern baldness and muscle wasting? As far as everything that you read, it is a muscle disease.

I can tell you from my experience that the mental/cognitive effects are as devastating as the physical effects (and the physical effects are extensive from swallowing, pneumonia, weak hand movement, unstable walking, to the heart quitting). The point that I am trying to make is that many people compliment me and tell me what a wonderful mother I am. The reason that I share my experiences isn’t to brag about myself, I am trying to make a point that those with myotonic dystrophy are affected in both body muscles and their mind. So, I am only doing what needs to be done.

Because myotonic individuals look almost normal, most of us don’t realize the effects on their mind, including us parents. I may sound like a broken record about what I do but hopefully as I become a more skilled writer, I will be able to make the point that I do what I do because my family would not only cease to thrive, they would probably not be alive if I didn’t adapt my life in whatever ways are necessary to be there for my family. 

Absent Caregiver

When I got up and looked at my text messages I saw one from Rily (today's caregiver), that he had sent at midnight. It said "I'm so sorry to text you so late but I fell off a roof a few hours ago. I got a little hurt so I won't be there tomorrow. I'm so sorry." Rily is the best caregiver for Michael and Chad and they love him. Rily usually arrives at 9 a.m. I erroneously assumed that he had told his company and that they would send over a replacement. When no one had contacted me by 9:40 a.m. to say that they had arrived I called the company, A Caring Hand. They were unaware of  Rily's injury but stated that they would do their best to get someone over to "The Brother's" house (they call Michael and Chad "The Brothers" - which is more appropriate than "the boys"). They did inform me that it would take awhile.

Therefore, it fell on me to wake both Michael and Chad or let them sleep for who knows how long. It is a long process to wake them but I have learned how to do it without having to run over to their place. I call Michael's cell phone first, then Chad's. But who knows if their cell phones are in their rooms with them. So, then I call the house phone which has an extension in Michael's room. I just keep rotating between calling each phone until someone answers. I started calling them at 9:50 a.m. It only took until 10:15 (tongue in cheek) for them to answer.

Should I pull my hair out now?

Photo by Brooke Cagle on Unsplash

Stimulants are so important in their life. Chad takes ritalin and provigil upon waking, after I talk him through getting out of bed and getting it out of the safe (previous caregivers have stolen these street value drugs so they have purchased a safe). Michael takes extended release ritalin and provigil, again I walk him through waking up and getting it out of the safe. 

After they get these medications into their bodies and I walk them through the process of getting into the front room they use their airway clearance vest. This is a device made by Hill-Rom that shakes the bedoogle out of them for 20 minutes. It's really incredible because they can cough better and breathe better if they use it 2 times a day. But one of the percs of it is that it shakes them so much that they can't fall back asleep (well, truth be told, Michael has fallen asleep with it on once). 

Once they have taken their stimulants and been shaken for 20 minutes they can usually stay up to get breakfast and get on with their day. I have taught them to look out for each other because invariably they will fall asleep even after taking their stimulants. So, I keep in touch with them for the first half hour to make sure that they stay up.  But after that half hour I am able to get on with my day. 

Normally, when the cargivers show up at 9 a.m. and get them going then I don't have to do all of this. You can see why I worship the caregivers :)

Myotonic dystrophy job coach

I am just jumping in here midstream. If you want the backstory, I do have my story written in manuscript form. Let me know if you are interested in a copy.

Michael has worked with Vocational Rehabilitation (Voc. Rehab.) since 2002. Voc. Rehab. hung in there putting him through college and he graduated in 2015 (13 years of college) with a Bachelor's degree in Mass Communication. Except for a 4 month job at a local high school he has not had any employment since graduation. Since Voc Rehab's purpose is to get those with disabilities employed their work with Michael isn't finished yet.

Due to Michael's Myotonic Dystrophy and the effects on his brain (Acquired Brain Injury) Michael does not have any follow through or motivation to pursue employment. Voc. Rehab. has finally realized that Michael needs a job coach. Of course, they only realized this because of my advocacy. After trying and failing for over a year to get a job coaching company to work with Michael, I got involved.

It is such a complicated dance that has to happen. Voc. rehab. wants Michael to do the research on job coaches, expecting him to call all of them and choose the one that he likes the best. Of course, he won't follow through. If I make it happen I would have to  get him on the phone with me and insist that he make the call. Then I would have to interpret for him and I can't even understand him, much less interpret for him. It is just easier for me to do it.

I went online and the second place that I went to was http://www.gochrysalis.com/

The picture on their home page was this:


If you know Michael, you know that he is a big superman fan.
The decision is made. How do you like my research?

Now the journey starts with Michael's job coach. :)