myotonic muscular dystrophy

[ronnie0a]

My wife of 30 years, Addie Lee Alexander (the former Kay Ann Joncas), was killed in March by the administration of drugs that are not dangerous to most of us. My 911 call had saved Addie's life, as on previous occasions, but this time the Firemen refused to transport Addie to the nearby Trauma Center, where we had prepared the staff to treat myotonic muscular dystrophy (MMD or DM:dystrophia myotonica). Then at the County Emergency Room, the staff refused to follow the protocol established by their parent organization at UCLA. Even though Addie was responsive when taken from our home, her condition deteriorated through the night, as she was repeatedly given strong drugs that cause myotonic paralysis of her vascular system. I was unable to prevent this series of wrongful actions that led to Addie's death, despite all my years of training and caring for this disease.

If others have had similar experiences, please let me know. I will have a battle against Los Angeles County, who claim that they "just treated her like any other coronary patient" - even though Addie did NOT have coronary heart disease!

[becks]

First of all, my heartfelt sympathy is extended to you even though I do not know you nor did I know your wife....I've not had a similar experience either, but I am intrigued since you mentioned your years of experience/training/caring for this disease. My husband was diagnosed just 5 months after we were married (we'll soon celebrate 27 years together) and still we know so little about myotonic dystrophy....how did you learn so much? I'd appreciate any help you can give to shine light on this challenging disease. Thanks

Originally posted by ronnie0a:
My wife of 30 years, Addie Lee Alexander (the former Kay Ann Joncas), was killed in March by the administration of drugs that are not dangerous to most of us. My 911 call had saved Addie's life, as on previous occasions, but this time the Firemen refused to transport Addie to the nearby Trauma Center, where we had prepared the staff to treat myotonic muscular dystrophy (MMD or DM:dystrophia myotonica). Then at the County Emergency Room, the staff refused to follow the protocol established by their parent organization at UCLA. Even though Addie was responsive when taken from our home, her condition deteriorated through the night, as she was repeatedly given strong drugs that cause myotonic paralysis of her vascular system. I was unable to prevent this series of wrongful actions that led to Addie's death, despite all my years of training and caring for this disease.

If others have had similar experiences, please let me know. I will have a battle against Los Angeles County, who claim that they "just treated her like any other coronary patient" - even though Addie did NOT have coronary heart disease!

------------------
B

[ronnie0a]

When Addie and I were first married, I was an organizer of a Conference on dystrophia myotonica at Emanual Lutheran Hospital in Portland, OR. I was serving as Assistant to the Hospital's Audio-Visual Services Department Head.

I realized two things at that Conference: 1) the doctors knew very little about either diagnosis or treatment, and 2) the critical knowledge for a caregiver is advanced first aid and emergency care. I became a Red Cross Instructor, and spent many days in Hospital Medical Libraries. When I joined the Navy, I sought training that would prepare me to work with emergency services operations. I am one of the few evaluators of FEMA Nuclear Power Plant Emergency Exercises, thanks to training at the National Emergency Management Institute sponsored by my employer, FDA. While doing research at the Army Research Labs in Maryland, I provided EMT training to Fire Stations.

Addie and I were lucky to have employer support to let me continue developing my skills to provide immediate care, such as CPR, appropriate to persons who cannot be freely given epinephrine, opiates, or other drugs that are routinely used by EMTs. I was unable to prevent the inappropriate use of these drugs on 3/19-20/2003, but had kept Addie safe for 30 years up until then.

We have a support group in Los Angeles, that meets monthly for a pot luck, and sponsors an Annual Conference on Myotonic Muscular Dystrophy. You don't have to live here to belong. Alice and Ed Gunderson publish a Newsletter with specific information about at least one subject each issue. Since MMD presents such diverse symptoms, there are always new subjects to discuss.

You can email me at kayannjoncas@hotmail.com for contact details. We used to have a web page for the Support Group, but the member who kept it up has had hospitalizations. I'm at work now, and don't have the contact information with me. You may also want to visit Addie's history page at [url="http://www.aiprojects.net/family/hypertext/myotonia.html"]http://www.aiprojects.net/family/hypertext/myotonia.html[/url] - and feel free to brouse our site.

[This message has been edited by ronnie0a (edited 06-18-2003).]