My latest med SNAFU (not my fault this time)

There’s some background you’ll have to understand to know why this situation is what my father would call a cat’s-ass-trophe. (He had lots of lovely ways of cussing. Another favorite: bass-ackwards, as opposed to ass-backwards.)

So me, my mom, and maybe my grandma all have some form of myasthenia. There are two major forms of myasthenia.  By far the most common is myasthenia gravis, which is the autoimmune form. There is evidence that my mom and I both respond to steroids, which would make it seem as if we have the autoimmune form. There’s also a hereditary, congenital form. This is so rare that often each family with it has its own specific gene causing it. But it runs in our family so that’s a possibility. There’s also new evidence for a hereditary autoimmune form, and while I’m not a doctor, that seems the most likely. 

What is myasthenia?. It’s a disease of the neuromuscular junction. These diseases are very rare, and myasthenia is by far the most common. My mother and I both failed most of the common  tests for myasthenia. The only two we “passed” were the following:

1. We both responded to a drug called pyridostigmine (generic) or Mestinon (trade name).  My mother responded spectacularly.  I responded well: walked better, fell less, etc. 

2. We both got a very positive result on the most sensitive myasthenia test out there: the single fiber EMG (electromyogram). This is the test they give you when all other tests fail, and it picks up myasthenia at an incredibly sensitive rate. 

So we are certain now that we have it. So what is myasthenia? It’s a disease affecting the neuromuscular junction.  

Here’s what Wikipedia says, for lack of a better source: 

The hallmark of myasthenia gravis is fatigability. Muscles become progressively weaker during periods of activity, and improve after periods of rest. Muscles that control eye and eyelid movement, facial expressions, chewing, talking, and swallowing are especially susceptible. The muscles that control breathing and neck and limb movements can also be affected. Often, the physical examination yields results within normal limits.
The onset of the disorder can be sudden. Often, symptoms are intermittent. The diagnosis of myasthenia gravis may be delayed if the symptoms are subtle or variable. In most cases, the first noticeable symptom is weakness of the eye muscles. In others, difficulty in swallowing and slurred speech may be the first signs. The degree of muscle weakness involved in MG varies greatly among patients, ranging from a localized form that is limited to eye muscles (ocular myasthenia), to a severe and generalized form in which many muscles—sometimes including those that control breathing—are affected. Symptoms, which vary in type and severity, may include asymmetrical ptosis (a drooping of one or both eyelids), diplopia (double vision) due to weakness of the muscles that control eye movements, an unstable or waddling gait, weakness in arms, hands, fingers, legs, and neck, a change in facial expression, dysphagia (difficulty in swallowing), shortness of breath, and dysarthria(impaired speech, often nasal due to weakness of the velar muscles).

In myasthenic crisis, a paralysis of the respiratory muscles occurs, necessitating assisted ventilation to sustain life. In patients whose respiratory muscles are already weak, crises may be triggered by infection, fever, an adverse reaction to medication, or emotional stress. Myasthenia does not directly affect the heart muscle.

So to tell you exactly how specific and rare the medication is, I have bottles of liquid Mestinon sitting around my house. There was a substitute staff person training one day and he saw one. “Mestinon…. You have myasthenia gravis?” He asked. When I said yes, he said he had it too. We even have the same neurologist. 

So Mestinon treats myasthenia really well. For those of us who have any degree of lung involvement, it can literally save our lives. It beats having to be on a bipap or other respirator while awake, which used to be my only solution (even encouraged by my pulmonologist, before we had a diagnosis of myasthenia or severe secondary adrenal insufficiency) when my lungs got weak enough that I felt short of breath. And a bipap was even the solution when my mom coded in the ICU a year ago. 

Here’s a longer description of myasthenia gravis from the Mestinon website.

And from the same website, a description of what it does:

WHAT IS MESTINON® (pyridostigmine bromide,USP) AND HOW DOES IT WORK?

MESTINON® is an orally active cholinesterase inhibitor. MESTINON®prevents the breakdown of acetylcholine (pronounced “a cee til coline”) by allowing more acetylcholine to accumulate. Acetylcholine is the chemical that sends nerve impulses to the muscle. With more acetylcholine, there is more control of voluntary functions such as eye movements, limited strength, swallowing and breathing.
WHAT DISEASE DOES MESTINON® TREAT? 
MESTINON® is useful in the treatment of myasthenia gravis. After nearly 50 years of use, MESTINON® continues to be used as the first line of therapy in the treatment of myasthenia gravis.

So Mestinon is crucial for treatment of myasthenia gravis.  Everyone I know with any form of myasthenia is on it. I’ve been on it for years. It’s vital to being able to breathe, walk, and stuff, with minimal difficulty. 

I’m on both Medicare and Medicaid as a result of being in a weird category called Disabled Adult Child. It means I was on SSI, still qualify for SSI, qualified for SSI prior to age 22, but am on my father’s Social Security benefits, first due to his retirement, then after his death.  (RSDI benefits.) This is a strange category that idea seemed equivalent to SSI for Medicaid purposes, meaning you qualify for Medicaid no matter how much money you get. 

And I get a lot more money than I did on SSI. I don’t have enough money to buy my own Mestinon though. 

And insurance just started refusing to cover it. Not because they think I don’t need it.  They know I do. Single fiber EMGs don’t lie. But they have just suddenly stopped covering this drug, period.  My doctor’s office it’s furious and is fighting this. We are going to Legal Aid too. But if we don’t win, I don’t know what I’ll do. Beyond hope that other treatments (CellCept, plasmaphoresis) work well enough to replace it.  But those are slim hopes when they rest on this being autoimmune and the plasmaphoresis could make my stomach bleed worse, meanwhile we know for a fact Mestinon works. 

Every few years, Medicare pulls this bullshit where they say they won’t cover some med that they’ve been covering for years. Or worse, I get an announcement that they’ve never covered something and the pharmacy never told us they were absorbing the costs all this time. Whatever it is, this sucks and makes me freak out and feel powerless.