Myotonic Muscular Dystrophy

Beth’s rare, progressive, untreatable form of muscular dystrophy.

Oct 192012
 

My daughters and I have spent the last few days working on Beth’s obituary. This is a task that should not be put off until the death of a loved one. There is so much we would want to tell people about my wife, their mother. But there is also the reality. To begin with, people reading the newspaper and scanning the obituaries are generally not likely to want to read a long story about how much someone meant to you. What’s more, at $10 a line, indulging in excess sentiment could quickly become very expensive–money that should be better used. So when it came right down to it, we realized the important facts of her life trumped the depth of our loss and the breadth of our love. Here is the finished product as it will appear in Sunday’s paper.

Obituary for Elizabeth Shirk

Obituary for Elizabeth Shirk as it will appear Sunday, October 21, 2012.

Oct 142012
 

Beth at Art Reception

Beth at an art reception last year.

Three nights ago I got those terrible phone calls. The first was from my wife’s new assisted-living facility. She was being rushed to the hospital. The second call was from my daughter who was on her way to meet the ambulance and said she would send her husband to get me. When I got inside the emergency room my daughter met me and said “brace yourself, it’s very bad”. Beth, my wife of 38 years was lying on the bed, a ventilation mask over her face, her chest heaving in response to the high amount of pressure they were using to force air and oxygen into her lungs. My daughter explained that without that she could not survive. Although my wife had a Do Not Resuscitate order, my daughter made the decision to allow them to take this extra step to keep her alive until I could be at her bedside and to give us time to bring our two daughters down from Orange County.

So we talked with her as her body struggled. She managed one-syllable answers and at one point opened her eyes and squeezed my hand. Then our daughters arrived and we gathered around and told Beth how much we loved her (although there really were no words that could possibly say how very much that was).

Beth on the beach with our daughters.

Each summer Beth and our daughters would stay at the beach in La Jolla for a couple of glorious weeks.


The doctor gave her pain killer and a sedative, then removed the mask. We held Beth and continued to talk with her as she struggled to breathe on her own. All too soon, she took her last breath and I felt her fingers relax their hold on mine. My wife, my soulmate, the mother of our children, was gone.

As I write this, there is no way to describe the agony. If I could, I would change places with her in an instant, but I can’t. Somehow I have to make sense of a life that doesn’t include Beth and try to use the time I have left to continue to write and help the world know more about the wonderful woman it has lost.

Spring Ahead Watermedia Painting by Beth Shirk

One of Beth's last works of art, Spring Ahead won many awards and now hangs in my room.

Sep 282012
 

Beth on cousin's deck in Englewood, FLA

Beth on the deck of my cousin's apartment on the Gulf coast of Florida, about 39 years ago.

Forty years ago, when Beth and I met, we fell so hopelessly in love we were willing to uproot both of our lives so that we could spend the rest of our years together. We were inseparable, holding hands wherever we went, even on our way to the laundromat where we would sit together and watch our clothes dry. We had two beautiful daughters, now with families of their own. I learned to share her love for art, and we began our retirement intending to spend the rest of our lives painting together. When I became disabled 15 years ago, she helped me cope. Then she became disabled a few years later, as though we needed to share that too.

For the past year or so, Beth has been leaving me. Not out the door or to the arms of another, much farther than that. Her brain is taking her slowly but certainly to a place that I can not visit nor even comprehend. And now this terrible illness, yet to be named despite two weeks of trying by a team of UCSD doctors and psychiatrists, has progressed to the point that the unthinkable is happening. In order for her to receive the kind of care she needs and deserves, she must move to a special facility, and there is no place for me. I can visit as often as I wish, and I will. But when I return to my room, with its ceiling lifts and hospital bed and accessible fixtures, there will be no soft greeting to make this austere environment feel like home. Even worse is knowing that she will also be alone without the hope that I might rescue her from the tigers and lions, snakes and assassins and all the other evil images that come stalking as the light fades. I hope this new place, and new medicine, will make them go away. But even so, I know that the illness has also taken her ability to understand the reasons we are apart. Beth will only know that I am not there, and wonder why the person who once happily spent hours helping her pick curtain fabric has vanished into the murky night.

Mar 112012
 

With an appropriate mobile arm support, I hope to some day return to creating art such as Quiet Harbor now part of the Muscular Dystrophy Association Art Collection.

Since I was diagnosed with Inclusion Body Myositis (IBM) 16 years ago, I have been stubbornly maintaining my independence. I have made use of every technical aid that I could find, beginning with canes and walkers and scooters and ultimately graduating to wheelchairs and hospital beds and overhead ceiling lifts. I started with a swing away lift in the back of van to take my scooter with me wherever I went, then bought a van with a ramp and a transfer seat, and ultimately moved to a van with an ez-lok system in the driver’s position. All these were steps to allow me to independently get around. I adapted my bathroom and my kitchen so that I can could continue to cook et cetera. I adapted my studio, even my workbench in the garage. I designed and sewed special shoes, pants, and leggings. No matter what, this disease was not going to get the better of me.

Today I am reluctantly admitting that this is one battle that ultimately I could not win. IBM is too progressive, too relentless, too untreatable. The final straw came when my right shoulder and arm became so weak that I could no longer raise my arm much above my waist. This meant that it was no longer safe for me to drive. It also meant that I could no longer chop vegetables or stir a skillet. It meant that I could no longer hold a paintbrush and create art. And worst of all, it meant that I was no longer an appropriate caregiver for my wife, whose own battle with Myotonic Muscular Dystrophy was not going well.

The first thing I did was have my van converted once again, only this time in the opposite direction. I had the passenger seat moved over into the driver’s seat position and put the ez-lok on the passenger side. This meant that I could pull into the van and lock myself in on the passenger side — provided I had found a willing driver to take me where I needed to go. Fortunately, my friends and family and neighbor have pitched in and I still have been able to get around when absolutely necessary. Perhaps more importantly, my wife, who also can’t drive and who has more medical challenges than I, could get to her various doctor appointments. When drivers aren’t available we are able to get to appointments using the accessible bus transportation called MTS access. It gets the job done, but it is certainly not a convenience. I plan an article on the general subject of bus transportation (and lack there of) soon.

But the really big change I have made is to hire caregivers for the two of us morning and night. The loss of arm strength meant that I was at great risk of being stranded when trying to use my ceiling lift to get into or out of bed or onto and off the toilet. After several close calls and more than a few minutes of hanging suspended in a very painful and awkward position, I realized I simply couldn’t go it alone anymore. It is an expensive adaptation and one that we will not be able to afford indefinitely. But for now it is getting us through each day. In future articles I will talk more about the good points of having caregivers.

I am also searching for a “mobile arm support.” The right one might restore some of the functions of my right hand and could possibly let me try to paint again.

Dec 222010
 

Rainbow north of our Rancho Bernardo home.

Rainbow north of our Rancho Bernardo home.

The New Year is often a time for reflection and introspection. So in 2010 I took this opportunity to review the highlights (ho ho) of my journey with inclusion body myositis, along with my wife’s struggle with myotonic dystrophy. This is my second annual update. Sadly, my wife’s struggle ended October 11, 2012.

1985-1995 (pre-diagnosis) stumbling while jogging, golf club flying out of hands, difficulty rising with backpack, jogging speed declining

1996 – diagnosed at UCSD Medical Center

1997 – started using walking sticks to help avoid falls

1998 – purchased scooter with elevating seat for distances more than 100 yards

purchased van with lift in rear for taking scooter along

1999 – fitted for full leg braces (KAFO) and forearm crutches

purchased Jazzy wheelchair with elevating seat

had condo modified with ramps at curb, entrances, sunken living room

replaced roman tub with roll-in shower, added roll-up counter in kitchen

bought fiberglass portable rampp

2000 – purchased raised toilet seat

purchased hand controls for van

retired on disability

gave up piano, golf, tennis, took up watercolor

2001 – became coordinator for paint out group of San Diego Watercolor Society

2002 – became Membership Director of San Diego Watercolor Society

2004 – became International Exhibition Director of San Diego Watercolor Society

purchased van with ramp and transfer seat

2005 – purchased Pride lift chair

became Technology Director of San Diego Watercolor Society

began making hooks and dressing sticks

purchased grabbers (six)

Beth erroneously diagnosed with ALS by local Centre for Healthcare neurologist

(two months later) Beth correctly diagnosed with myotonic muscular dystrophy by MDA neurologist

acquired Permobil C500 (Medicare) for Mike

purchased Jazzy 1103 wheelchair for Beth

2006 – purchased Biobidet

became President San Diego Watercolor Society

purchased single story home in Rancho Bernardo (San Dego)

added 200 feet of outside sidewalks for wheelchair access to yard

added ADA bathroom (elevated toilet with bidet, low-lip shower, roll-under sink, room for wheelchair)

acquired hospital bed (Medicare)

2007 – began using shelf liners to lift legs

began purchasing wheelchair pants from USA Jeans

2008 – purchased ceiling lifts for bedroom and bathroom

2009 – purchased rechargeable wine opener

2010 – purchased iPad for drawing, games, voice recognition, etc.

purchased automatic can opener, jar opener

purchased computerized sewing machine (no foot pedal)

made belly bag, art apron, cooking apron, robe, work table, sliding pad

2011 – Lost ability to make horizontal transfers and began using overhead Waverly Glen lift.

Sewed pants that velcroed around me after being lowered onto them.

Hired part time caregiver for showers ( no longer safe to do on my own).

Lost ability to drive, began relying on the bus.

Hired second caregiver and greatly expanded their hours to daily as Beth also needed help.

2012 – Beth began hallucinating.

Moved both of us to assisted living and put home up for rent.

Beth died of respiratory failure due to myotonic muscular dystrophy.

Lost most strength in arms, began using mobile arm support.

Oct 062010
 

My wife recently underwent surgery. The surgery went well, but getting the medical team to understand the implications of myotonic muscular dystrophy on her care was very difficult. The situation was made even more difficult because the health care providers involved are still keeping the records in longhand. This meant we had to repeat all the information every time we saw a new provider–and there were several. Even though we warned everyone that myotonic muscular dystrophy has serious implications for anesthesia, no one seemed to understand, not even the surgeon.

Then, on the night of surgery, we received a phone call from the anesthesiologist wanting to know more about myotonic muscular dystrophy as he had never dealt with it before. Fortunately, after I gave him a brief rundown of the risks involved, he went onto the internet and learned more about it. After that, he became so concerned that he advised canceling the surgery until he could learn more and perhaps do more tests. However after discussing it with the surgeon, and since this was cancer surgery, they decided to go ahead, but use what precautions they had learned about such as using as little paralyzing agent as possible. They also decided to admit my wife, instead of discharging her the day of surgery. Although she needed oxygen that night, by the next day she was strong enough to go home. Ironically, when signing the discharge papers, we discovered the hospital thought she had “mascular dystrophy.”

Hospital form reads mascular dystrophy

Archaic handwritten records resulted in this fictional illness being listed as my wife's medical condition.

The morals of this story: 1. If you have a rare disease, you must be your own care coordinator – and be an educator for the medical profession. 2. Every healthcare system needs to convert to electronic patient records now!

For more information on the issues involved with anesthesia and myotonic dystrophy, follow this link:
http://www.mdausa.org/publications/quest/q73anesthesia2.html