How we Trick-or-Treated with Muscular Dystrophy

Happy Halloween from the French gang!

Whew, big sigh...we are back from trick- or- treating tonight! Max was diagnosed with RYR1 Congenital Myopathy this past year, so this is the first Halloween we purposely made adaptions for his health. (vs. just saying, "he's getting tired, let's finish up" like in other years). This year we know the "why" behind why he fatigues and we better know what to do. (Thanks Doc for running the big MD genetic test!)

It's 8:20pm though as I write this and everyone is bathed and in pajamas hanging out now:) Trick- or- Treat Lancaster County, PA started at 6pm. We were home by 7:10. But it went great!

To make this year a success, it started with the costume. A big no to the Storm Trooper helmet that weighs a ton and he fell in  wearing two years ago. No to the cool E.T. costume you make with a hoodie and a milk crate...as a crate with an E.T in it is too much to tote around. No to masks in case he trips. No to Ghostbusters as the large amount of equipment is WAY too much. (You should have heard him about that one. "NO. NO. There's SO MUCH with that one. Seriously!" No to Link from Legend of Zelda as that requires carrying a sword. And a shield. Add some pre-teen indecision fickleness and we thought we'd end up as our homemade idea, "Cereal Killer." but that included buying a t-shirt, mini-cereals, and glue, and blood ...well....we just kept looking.

Both kids were managed to be outfitted for $25 at Community Aid Thrift Store (which is like our favorite place in the world). It took a few trips and some patience looking through the costume racks starting in September- but we managed to pull everything together.
 Jessie= $8. Popeye costume= $10
Hat at Party City $7. I made the pipe and a can of Popeye spinach, added a pair of my Birkenstock shoes (Max and I are the same shoe size right now!) and Mimi wore cowboy booties and Max's old cowboy hat.
 
But most importantly: Everyone was comfy and both outfits wore like clothes. (Except I had to cut the arms of the Popeye costume as they came with a strange amount of uncomfortable elastic- I then resewed them to the shirt sleeves.) But that's ok. This can now be a costume we can use again.

Max was unsure how this whole look would go down.  I loved the sailor look of course. LOL
But, not too many 11 year olds out there 'dressin up like vintage Popeye. I figured it would appeal to his classic cinema/cartoon interest.
So how awesome it was to start trick- or- treating and to hear people affectionately say, "Hi Popeye!", "Oh my gosh it's Popeye! I love that!" "Popeye! That's the best costume I've seen!" and "Honey, look, honey, It's Popeye!"

So, How did we do the rest?

1. We walked slowly. We took our time. No rush. We had two hours. We didn't go with friends this year. Although that would have been fun, kids his age move fast, and um. We don't move fast. Both homeschool families we hang out with have very active kids, so we held off on going with them.

2. We carried band-aids and water. (In case.)

3. He did not go up to houses on hills. He let his sister ask for something for her brother and she brought it back for him. My awesome homeschooling neighbor came out to tell me that they would stay down on their driveway for Max since he didn't have his wheelchair. Thank You!

4. We crossed the street only when there were no cars, because he can't hurry especially if he's tired.

5. He tried to avoid stairs on houses. He stayed on the sidewalk of house steps if possible if a homeowner was sitting on the top step.

6. We brought a second empty candy bag and switched him to an empty bag half way through. Then we carried both bags home for him when we were done.

7. We avoided standing and chatting for too long and kept moving.

8. We stopped at the end of the block on the second street when the steps of the row houses on the second block suddenly get longer and steeper.

9. We walked slowly and carefully home.

10. When we got in the door we took a look at the clock and realized we had done it all in just over an hour.  "Whew we got so much candy. We are done now. Yay!"Then we changed out of costumes, inspected the candy, and hung out- waiting on the last hour of trick-or-treaters.

Trick or Treating with Muscular Dystrophy can be Tricky, but just remember to take your time and go slow. We found that the right costume that was easy to wear really helped (but also was the biggest dilemma.) If you will use your wheelchair, comfort will be key too. Make sure you can see where you are driving too and that your costume doesn't get stuck in the wheels!

In the next couple years of Trick- or -Treating we may switch entirely to a development that is flat with very few steps and employ these tactics again. We may also do a Halloween party at our house-which would allow our friends to hang out with us. We shall see!

Happy Halloween 2016 to You and Yours!

The Overly Dramatic Medical Family with the Wheelchair

Here we come into your event or store and low and behold...what's that we are getting out of our car...a wheelchair!

What on earth? Who in the car needs the wheelchair? The boy? What? Why? That family must be over the top or something. He looks fine. He just walked to the wheelchair. He's even laughing and talking! Gosh, well they must just be crazy people or something...He should walk. They are nuts.

This post is for all the parents whose children use a wheelchair and for people who still question why on earth our children are "Gasp!" in a WHEELCHAIR? (you must be kidding!)

My life over the last two years as a Mom is not one that I would ever wish upon you (even though you are really ticking me off....). I am numb at this point frankly. I'm not crazy. I'm actually a marvel of motherhood...my instincts knew that something really wrong was going on with my 9 year old.

Many, many people looked me in the eye during his early childhood and told me that he was just fine. But I had some lingering worries. Why did he drag himself to the car after amusement park days? Why did he moan and grab his head from headaches just after being in Target too long? Why did he limp at the zoo? Why did he walk very very slow out of the mall?

Max at the Brookfield Zoo in Chicago in 2013 with his sister and Dad. He barely made it back to the bus as he couldn't run or walk fast.
Very quickly,  he would  get very hot and sweaty, and would run out of energy. Like at the end of year preschool picnic...

 

At the gardens we visit in the summer...

and after dancing after the MAGIC convention, where we needed to take him back to the room.

We took my son to his doctor when his teacher told him that although bright, he'd never be able to do middle school work, high school work, go to college, or get a job with his attitude (head on desk, rolling his eyes). We took him to the doctor after that because we noticed that his penmanship had changed significantly too. His posture was suddenly poor and he wasn't able to complete his work at school. His doctor looked worried. And so began a year of testing.

It took a long time to get into the specialists we needed to see. But we started to notice that he really turned into a rag doll after about 15-30 minutes of walking around. We didn't know why. People with doctorates didn't know why. Some looked at me with a snicker and said that some kids are just low tone.

But the tests came back, and then more tests were ordered. My son had a rare form of Muscular Dystrophy called RYR1 Congenital Myopathy. He could have been diagnosed when he was younger but of course, MOST children are perfectly fine, and MANY mothers (like me) are Looney Tunes.

So. My son, the one laughing and talking (and walking!) to his wheelchair, is going into a store or into the event seated, instead of walking around or standing. He's in the wheelchair so that he can continue to laugh and talk and not turn into a rag doll and completely lose the contraction of his muscles throughout his body. By sitting, he won't fatigue within half an hour...maybe we can stay there for an hour today. (We started to leave places very very quickly, but with his wheelchair we can stay longer.) By not becoming fatigued today, his muscles may last him longer in life. Literally. If it's hot out, he may not get heat stroke and won't experience exercise intolerance after 15 minutes. (Both part of his condition).

When my son uses his wheelchair, I know he is safer. I don't have a panic feeling, worried that he is running out of batteries. He laughs and talks and almost runs me over.

So, I'm not crazy, and we're not a crazy family. We're excited that our son can hang out with us and stay out with us longer.

Max, first time ever in his wheelchair at Costco on February 13, 2016. About 35 minutes into shopping, he asks me, "Why are we here for so long?" We realized we hadn't taken him to Costco in years!


If you see someone in a wheelchair, believe us...there is probably quite a story behind it.
So you can just be numb to the whole wheelchair thing, and say hello or hold the door for us. That's what we find is nice:).

Restrictive Lung Disease? At Eleven Years?

Max met with his pulmonary doctor for the first time last Friday. It was also Mimi's 6th birthday.
I thought all would be fine so I didn't mind it was her birthday morning. Max's myopathy has a mild to severe lung problem prognosis...so at eleven, we would think he would be fine now.

We headed in, Max playing on his DS. Hanging out in the waiting room. I'm drinking a coffee. We've gone to over 100 appts and therapies in the last year, so we are very used to all this.

The therapist takes Max back and begins the pulmonary testing. Max tries to do what she says but there are a lot of directions, and that's hard for him. (part of his condition). So she tries again and again. At first Max can only breathe out 44% of what of it expected. She tries a few more times...better. With Max trying his absolute best...he scores in the 60's and 70's. As you may imagine, I have no idea what this means. Do you? So we head off into the patient room to meet with the doctor.

The doctor comes in and starts getting a medical history. For the first time really, since the genetic appt over 14 months ago, I go into detail about the odd recurring bouts of pneumonia and bronchitis that Max had in First Grade. He responds saying that Max isn't able to expel all his breath and so he is sure that 4 years ago, Max would get a virus at school. It would then never totally clear, and he would keep getting bronchitis type illnesses. Back then. Four years ago. Because of his muscular dystrophy. (I think back to Max being exhausted and trying to keep up with school work. About the meeting when they thought he may have ADHD, which didn't make sense to me.)  So, in addition to him fighting through his day with weakening muscles...he was fighting ongoing infections in his lungs because his lungs weren't strong AND NO ONE HAD A CLUE!

He said that he wants to quantify the results he had with the therapist, by sending him to Hershey Pulmonary testing and putting him in a booth type room that tests what is happening with the muscles in his chest. He said he could tell from listening to his chest as well, that he was not getting rid of all of his air. 

He said that he is at risk for infections in his lungs. He said he thinks he has Restrictive Lung Disease. He put him on Flovent 2x in the morning and 2x at night. He ordered us a new Nebulizer and more Albuterol. He said that Max may need the cough assist machine and the VEST. (the Vest is what the Cystic Fibrosis patients use.) It vibrates their chest to expel any mucous. 

Max was tired just from the pulmonary testing. My mind started to go into a blank sort of shock. I had interned in my senior year of college at the Cystic Fibrosis Foundation in Harrisburg after two of my friends has died of it. On the doctor's jacket it says, "Cystic Fibrosis." So he's the CF dr... I know about that world. I don't want my son to need anything like that." I think.

I asked him, "So...what do we do if he has a cold...or a bronchitis like cough?" I said, "Should we call his primary doctor because I would think he would be worried for Max." and he replied with a laugh, "You need to call me. You would need to see me." He then told me what days he is at the Lancaster office and what days he is at Hershey.

The therapist came in and brought in an inhaler sample with a spacer. She taught us how to use the spacer. The doctor ordered the nebulizer machine and scheduled his follow up for in 2 months or sooner if needed. He said that Max needs a chest x-ray also.

I came home and looked up the VEST and it's between $4,000 and $8,000. It comes in a duffle bag, so you can take it wherever you go.

We are so thankful for our insurance. I am shocked by all this. I thought this was years and years away.

We have started the Flovent with the spacer. The notes came back too from the doctor. Depending on the results of the next two or three tests, Max may need a BiPap, Vest, and Cough Assist.

We also need to understand that Max goes to Hershey for colds/bronchial symptoms now. 

It's life changing, for sure. And just in time for winter.

I am so glad that he is homeschooling and that we are used to it. I also am relieved that Mimi is homeschooling too. Can you imagine if she came home with every virus from school? I cannot even imagine.

UPDATE November 4, 2016: The pulmonary test is back and confirmed that Max has a mild Restrictive Lung Disease due to his muscle weakness. His test matched what he showed during his office visit.
They are glad that the sleep study is scheduled for early January. May need extra oxygen during sleep they suspect.
Still the same protocol for him right now. Flovent and use Albuterol every two hours with colds. - call the office if he has a cold in case he needs supplemental oxygen/more intensive treatments.

Art with Muscular Dystrophy

We do many art projects at our house. As Max's hands get weaker, we still make art pieces. It is calming for everyone involved:) It is interesting to watch what Max will do to accomplish his task. He will work fast, and boldly as his hands don't have a lot of strength.
I pushed him with the Starry Starry Night above, and he came back later and checked out his work, and couldn't believe he had done that.
He loves pastels because they are very smooth and go on quickly.
Another thing he does is express what we wants to in the shortest way possible. Sometimes that's just with a fast line.

And even with hand weakness, we still managed to introduce and practice cursive. He proudly "signs" his name now whenever asked.:)

Medic Alert

It arrived today- Max's bracelet.
He should wear this everyday forever. It's orange because it's his favorite color. It's thin because it's the lightest weight. Hopefully, it won't bother him.
It says on the back, "Malignant Hyperthermia. Muscular Dystrophy."
Now we need to order Mimi's and mine.
A new chapter...

Homeschooling Muscular Dystrophy

We have been homeschooling Max for just about two years now. Are you considering homeschooling your child with Muscular Dystrophy?

The first fantastic thing about starting to homeschool was realizing my son doesn't need to wear a school uniform. And he won't lose his coat anymore! LOL We started on December 15, so he was able to get quite comfy in athletic pants and long sleeve pull over shirts. We don't homeschool in our PJ's. Maybe if we're sick, we might do that...but we always get ourselves dressed and have a nice breakfast before starting.

How my son sat in little classroom chairs with slippery seats and no arms rests all day with significant proximal weakness, I have no idea. We use vintage Ethan Allen chairs for Max and I that have nice arms to them. We have seat cushions on them, and sometimes use back cushions too.

We only eat at the table if there is a special homeschool treat, and we use covered cups. We keep all the books we are using in an IKEA storage container on the table. If a drink spills, it won't ruin our lovely curriculum. We use the green Trofast bins like on the top shelf of this picture from IKEA.

Max usually starts his morning with about a 6-10 page story from a reader for his grade level. It is usually a historical, moral, or cultural story. Sometimes it is a fable. He reads that on the couch.
We then go to the table. 
We use pencil grippers or jumbo pens that might have a built in gripper. We do a short cursive exercise while we listen to an audio story.

We do not do cyber school because Max had fatigue issues from public school and we knew we needed to customize his learning and his day. We shop in the spring for his curriculum for the next year. It averages between $200 and $500, depending on what we can buy used. Our style is more traditional learning with hands on activities and field trips. We prefer curriculum designed for homeschoolers.

This year Max started using Teaching Textbooks for Math. It is so awesome. He is really learning and is able to do most of his math work for the program on his computer. Some he does in the oversized workbook that has large spaces for him to write. Due to his hand weakness, he has a lot of trouble writing small.

We then work on Grammar. We go over 2 pages of grammar together with him answering verbally, or using a highlighter to select his answers instead of a pen.

He also reads from his science book daily, and his social studies book daily. He has been studying Egypt since school started. Last year, we did a ton of lapbooking for both subjects, but his OT said it was just too much. We are still doing bits of lapbooking for social studies, but no where near what we used to do. We've just moved onto projects and they are more exciting for him I think. A couple weeks ago we made Canopic jars from paper mache. (of course he happens not to be sitting in the arm chair here, because he moved over for the picture. LOL)

 

We also do spelling weekly. We do the exercises verbally if at all possible each day to become more familiar with the words. He takes a test on Friday, and he writes the words for that.

For writing exercises, we have begun to use Dragon Voice Recognition Technology. Don't forget you need a headset if you buy the program.

Art we often do mid-week, or on late Sunday afternoons (this year anyway so far) with my Mother - in Law. She has bought us some paints, brushes and canvases recently. I also take the kids to historic sites and we do pastel work open air. Both have been lots of fun. We have also done art classes at the library which are free. Max love the pastels as they glide so nicely. We tried foam grippers on the paint brushes but it didn't work out, so Max just uses regular paint brushes.

For music Max has been taking online piano lessons. We also attend free concerts with our homeschool friends when we can. We also learn new songs each year. Last year we enjoyed learning pioneer tunes.

Gym for this year so far is Horseback Riding therapy. Last year, it was PT and OT which both included yoga. Recess is playing with his sister outside.

He ends his day with reading at least 20 pages from a good book:)

Afternoons are free, or else we're at the library, therapy, a field trip, at our Co-op, with other homeschool friends, or at the doctor.

We also love, love, love field trips, but of course with RYR1 Myopathy we battle fatigue. Last year, Max went on something like 27 field trips! I always feel so thankful when we are on our adventures that Max isn't stuck inside, but gets to do so many exciting things.

 

When homeschooling Max, I have to always be ready to adapt his work. Sometimes he is absolutely exhausted. I can tell if he slumped more than usual, or if his writing is shakier than ever.

We love that it is very easy to go to Dr. appts. We don't have to report to anyone. We just plan our week accordingly. I absolutely cannot imagine managing his endless Dr. visits with him at regular school.

Max used to get sick quite often during the school year and had endless bouts of bronchitis. He has had VERY few illnesses since homeschooling.

If our area is expecting a snowstorm, we run around and have fun the day before the storm and we don't do much school. We may even go out to eat, out shopping, whatever. When the storm starts, we hunker down and gets loads of work done. 

I also find that I get a lot of use out of our vacuum:). I am messy with all the projects I have the kids do. And Muscular Dsytrophy is messy too, especially with limited hand function. I always give the area under and around the table a quick sweep at the end of the day.

xoxoxoxoxo

Updates are us!

Oh my, it's been so long since I've written on my blog. We have many updates for you!

The genetic test for Max came back in July and his official diagnosis is RYR1 Congenital Myopathy. Now we know a little more what we could expect. He has a medic alert bracelet on the way for Malignant Hyperthermia. He has an appt with pulmonary to test his lung function again, and also a sleep study scheduled for January. He also has an MDA full clinic appt early in the year- which will be several hours long. His heart should be spared...his lungs maybe not (hence the lung appts). RYR1 Myopathy comes with a major fatigue issue. Hmmm. Mom IS able to recognize fatigue in her child! And, RYR1 Myopathy alters the calcium release in your muscles, pouring out too much...and messing with muscle contraction. Hmmm...Max turns into a rag doll in 12 minutes on the playground!

The National Institutes of Health (NIH) in Bethesda is currently working on a trial testing an antioxidant on RYR1 patients and we have applied for that and are waiting to hear back. They asked that my husband and I be tested for the variant. Our genetic tests came back a few weeks ago, and I have the same variant as Max. I am his carrier. But there is also a little catch. I have to wear a Malignant Hyperthermia bracelet too, And, having the variant is probably the reason why I'm so bad in the heat. And another thing...my back and chest are both going crazy pushing Max's manual chair. To the point where I really can't push him anymore. I'm having muscle spasms that are very intense and last for hours. I have 2 appts in one day scheduled for November with Max's Neurologist.

Max and Mimi have been busy homeschooling. Our favorite subject of 5th grade right now is...wait for it....Math! Thank you to Teaching Textbooks for teaching my son! It is awesome. I am so happy we ordered it for him. Mimi is still five and is reading very well. I think she is at a mid-1st grade level. She completed kindergarten at home last year and is well into her first grade work now.

Max has also started therapeutic horseback riding at Greystone Manor here in Lancaster County. Another win-win! Max could have hip contractures in his future...soooo we hope riding helps keep that at bay ..plus it's wonderful for many other aspects of therapy.

We have also become involved with the Muscle Movement Foundation which pairs people with muscle disease with a sports team. Max became an Honorary Captain and Muscle Champion of the St. Elizabeth High School Viking's Football team this season! Rob, the founder is about the nicest guy in the world and we are so happy to know him.

Max has also enjoyed volunteering with our local MDA chapter and  participated in Lock-Up this week in Lancaster. He was able to give about 5 short speeches to area business people in attendance. He also met his new gang...the Lancaster City Firefighters:) We hope to get together with them very soon and they gave Max an open invitation to the station.

As a family, we are adjusting to the new normal. We are flying by the seat of our pants as we learn to adapt things for Max. We hope that if you are healthy, you continue to realize that you really are walking on air. We are challenged every week with things like our dishes being too heavy for Max, or needing all cups with lids, or easy to wear clothing. We are enjoying though, making new associations and being the best we can be, despite these ongoing challenges.