A Pharmaceutical Guinea Pig...

That's me.

My back goes through periods of more and less intense pain. It does not, any longer, have periods (however brief) of no pain whatsoever. It simply doesn't happen anymore. Some days are better than others, but no day is without its pain.

Sounds like a life anyone would want, right? Where can I sign up for this? Oh... wait, I've been signed up without giving permission. How'd that happen?

It is unlikely that this is related to the muscular dystrophy. I am sure that the accident in 2001 is at partially culpable in this. Hmmmm. If you look at my special version of MD, called DM 2, which is described below:

"Classification

Myotonic dystrophy is the most common form of muscular dystrophy allowing adult survival and the second most common form of any skeletal muscle disease after Duchenne muscular dystrophy. There are currently two known types of adult onset DM, both identifiable by DNA analysis:

--Myotonic dystrophy type 1 (DM1), also known as Steinert's disease. DM1 has a congenital form that can severely affect babies and a childhood onset form.

--Myotonic dystrophy type 2 (DM2), commonly referred to as PROMM or proximal myotonic myopathy. This is what I have.

Type 1 is by far the most common form, accounting for 98% of all myotonic dystrophy cases, however DM2 can be more difficult to be diagnosed because of unusual phenotypes and is believed to be underdiagnosed.

Differences in DM

While both diseases are considered slow degenerative conditions, DM2 is considered to be generally milder than DM1.

--The severe congenital form that affects babies in DM1 has not been found in DM2 and the early onset of symptoms is rarely noted to appear in younger patients in the medical literature.
--The repeat expansion for DM2 is considerably larger than for DM1, ranging from 75 to over 11,000.
--Unlike DM1, the size of the repeated DNA expansion does not appear to make a difference in the age of onset or disease severity in DM2.
--Anticipation is a common feature of DM1. It appears to be less significant in type 2 and most current reviews only report mild anticipation as a feature of DM2.

Symptoms

Presentation of symptoms varies considerably by form (DM1/DM2), severity and even unusual DM2 phenotypes. DM1 patients often present with myotonia, disabling distal weakness and severe cognitive problems. DM2 patients commonly present with muscle pain, stiffness, fatigue, or the development of proximal lower extremity weakness (Day & al, 2003). The characteristic pattern of weakness is different for DM1 and DM2: In DM1, it is noted in face and jaw muscles, the drooping of the eyelids (ptosis), weakness of the neck muscles, hands and lower legs. In DM2, the weakness is more evident in proximal muscles, those closer to the trunk of the body: neck, shoulders, hip flexors and upper legs.

Cognitive manifestations for DM2 include problems with executive function (i.e. organization, concentration, word-finding etc) and hypersomnia. Conduction abnormalities are more common in DM1 than DM2, but all patients are advised to have an annual ECG. Insulin resistance is a significant risk factor in both forms of the disease for diabetes, cholesterol, heart, stroke, lipids, fatty liver, etc.

Diabetes type 2 is suspected of being more common in DM2 than in DM1. Generally far fewer DM2 patients require assistive devices (canes, walkers, wheelchairs, scooters) than in DM1, though they experience increasing difficulties climbing stairs as the disease progresses, and falling or stumbling may sometimes be reported.

Genetics

DM is a genetic condition which is inherited in an autosomal dominant pattern, meaning that inheriting a mutant gene from one parent will result in the condition.

DM2
DM2 is similarly caused by a defect of the ZNF9 gene on chromosome 3q21.

The repeat expansion for DM2 is much larger than for DM1, ranging from 75 to over 11000 repeats. Unlike DM1, the size of the repeated DNA expansion does not appear to make a difference in the age of onset or disease severity in DM2. Anticipation appears to be less significant in DM2 and most current reviews only report mild anticipation as a feature of DM2.

Management

There is currently no cure for or treatment specific to myotonic dystrophy. Heart problems, cataracts, and other abnormalities associated with the condition can be treated but not cured. However there are medical interventions and medications that may relieve some of the symptoms such as myotonia, pain and excessive sleepiness. Some treatments have been subject to systematic review for safety and efficacy through the Cochrane Reviews for symptoms such as hypersomnia (excessive daytime sleepiness), myotonia, strength training and aerobic exercise training and foot drop."

OK, that is a considerably watered down version of my world. Maybe... maybe the back problems are directly related to the DM2. Great. So now I know. But you saw what is under "Management"... nothing. So I guess that limits my options.

Which brings me to being the Guinea Pig of the western world. The western pharmaceutical world! I have been using Skellaxin (useless) and Oxycodone (harmful) to control the issues. When it is really severe, I use Soma but there is no functioning on it at all. To date, the doctors all ask about physical therapy, which mirrors yoga exactly. It's tough to do yoga with this, but I do it and the cost is considerably less than going to a facility to do the same things over for $90 a week.

I have $90 a week, but I can think of 100 ways - and likely more - better and much more fun ways to spend it. Much better.

I won't die from this directly, which is nice; and my heart checks out - I'm one of the fortunate carriers in that I don't have the likely heart problems. I do have a family history of diabetes, but so far cutting back on sugar has helped me dramatically. So far, I have dodged the bullet. I don't know if I can dodge it forever, but I certainly plan to try.

However, the whole muscle relaxants in tandem with oxycodone isn't working for me. I have little to no threat of liver disease but if I keep taking oxycodone I will. How is that helpful? Aside from that, it only dulls the pain, not really combats it.

So I went to my doctor again. I like her but I wish she had a more developed sense of humour. All of my sarcastic humour that makes the most stoic person laugh is completely ineffective. But that is not her fault. Anyway, she gave me samples of a new drug called Amrix. It is an all-day muscle relaxant that is okay to work with and doesn't seem to be too harmful. It does work... but it is not without its issues. The first night the Ambien was unable to put me to sleep and I was wide awake at 2200; not good.

But it works during the day, and that is good. I don't know what I will do at night...

There is one small caveate... the recommended dose is 15mg once a day for no more than three weeks. What do I do then?