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.” 

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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.