Friday, 2 August 2013

ALS or PMA or Both

When you first get diagnosed with ALS the medical community, especially the neurologists, are extremely reluctant to get specific about the diagnosis and prognosis. This is, in large part, due to the mysterious nature of this illness. It strikes in so many different ways, moves in such a random pattern, and takes its targets in such a relatively short time. Making any sort of generalization is dangerous. The best they can do is to tell you, based on your current condition, is that you have ALS and most people with ALS die within 36 months of diagnosis or 5 years of symptom onset.

There are a few early signs, elements which help refine that diagnosis and timeline. If you have Bulbar ALS then your prognosis is worse. If you have upper limb onset, then your prognosis is also shortenened. If you have lower limb onset, as has happened in my case, then your prognosis is better by a few months, perhaps a year.

You get your diagnosis and they send you home to deal with your life. They watch and wait, measuring those points of the graph, measuring your FRS and FVC, noting where the signs of atrophy occur next. After about 6 to 10 months of watching, waiting, testing and re-testing the medical community begins to form a better picture of what variant of ALS you might have.

In my case, my doctors are beginning to class me into a type of ALS called "Progressive Muscular Atrophy". This is a rare group, a class of PALS with an unusual progression pattern. With PMA, the primary focus of the disease tends to be in the Lower Motor Neurons. These are the neuron sets that control most of the body's muscular functions. The Upper Motor Neurons control the brain and spinal functions.

PMA is thought to account for as few as 4% of ALS cases. PMA is more prevalent in men whereas Bulbar ALS is more prevalent in women. PALS with PMA tend to live longer however the eventual outcome for the disease is always the same. In addition virtually all PMA patients end up with UMN failure, creating the same ultimate outcomes. Basically PMA is ALS for beginners.

When I was diagnosed the neurologists said I could expect 36 months. Based on my own research, I realized that the first 12 would be reasonable although I would need the chair. The second 12 would be difficult and I would need assistance for daily living. The last 12 would be tough and I would probably require full time home care.

Now those expectations are more difficult to measure. If things go slowly I could live for 5 years, perhaps longer. If things go slowly, I could have as much as 3 years of viable time. If things go really slowly, I could be around for even longer. This is a good thing, it just requires more planning.

1 comment:

  1. "unusual progression pattern" ... so unlike you! :)