The thought that I would be so limited for the rest of my life was both stunning and terrifying.
Adjusting to a new reality
The “acute” part of the chronic and acute myopathy was the degree of weakness, which would improve slightly. But they didn’t know by how much, how quickly, or with what permanent impairment.
As for the “chronic” level, it would be substantially worse than it had been six months earlier, when I was already so much weaker it was hard to take care of myself. And that was without any major attacks in 2012. I would have to cope with this myopathy every day for the rest of my life. However long that was going to be. And it didn’t sound like it was going to be very long.
Steroid myopathy
Steroid myopathy primarily, but not exclusively, affects the hips, lower back, thighs, core, shoulders, and upper back and arms — exactly the muscles most needed to get around, move, use your body. To sit or stand erect. To walk or carry anything, to bend or lift, even the lightest objects.
But it also affects my vocal cords and ability to swallow (which is why I have to be careful about choking), my hands, and countless other muscles throughout my body. Even months after my last dose of oral prednisone.
It affects things as disparate as my ability to hold a 4-ounce phone, or control a pen, walk, reach, step up on a curb, pull on clothes, change the sheets on a bed, do laundry or sit and watch a movie. And countless other things.
Regaining my strength
Getting my strength back, or a reasonable amount of it, obviously meant physical therapy. I had gone to PT at GW before for help with my back (I had degenerative disk disease, and a problem with the vertebra in my neck). But this was going to require a whole different level of therapy and for a very long time.
As I gradually recovered from these asthma attacks, we also returned to all the testing to discover what the eosinophils were doing to my body. If nothing else had been underscored in flashing neon letters, it was that we had to stop the asthma attacks.
While those 2013 asthma attacks were the worst I’ve ever had, there were far too many trips to the ER, and a few hospital admissions in the years following. Naturally, much of our attention for the next couple of years was on trying to prevent exacerbations – and anything that could contribute to them. And to get me and my immune system and lungs stronger. Which takes us to my allergies. And back to those pesky eosinophils.
Stopping the asthma attacks
Earlier in 2012, Dr. VL had told me about Xolair, a biologic medication. A biologic is “a substance that is made from a living organism or its products and is used in the prevention, diagnosis, or treatment of disease and other medical conditions. Biological drugs include antibodies, interleukins, and vaccines. Also called biologic agent and biological agent,” according to the definition on Cancer.gov.
Pretty much defines “cutting-edge medicine” to me.
Originally Xolair was developed to help children with anaphylactic peanut allergies. Because it is so effective in reducing IgE loads (the immunoglobulin that is key in allergic asthma), the FDA had also approved it for treatment of those with severe allergic asthma. We got approval for me to begin it, but I actually had to wait until my IgE load dropped before I qualified to start. One of the quirks of biologics is that you can’t be too needy to begin. (Sometime I need to get a researcher to explain that to me.)
But it was the fact that she was having to do monthly blood tests to track those IgE loads that, coincidentally, gave her the details about the eosinophil levels over time. Pure luck.
Life with Xolair
Since 2012, Xolair has had a gradual, but substantial, impact on my allergies and my allergic asthma. I haven’t had an allergy-triggered asthma attack since 2017. It wasn’t a dramatic difference, but each year, I’ve seen a noticeable improvement – something that’s never happened before. I don’t require regular antihistamines anymore, except during peak allergy seasons, which is a huge improvement for me. Just the occasional Zyrtec or Benedryl.
Now I haven’t tested fate by eating any nuts, or by upsetting any bees. But the occasional English pea or whatever that slips past my fork into a bite of something no longer causes me to search quickly through my purse for some Benedryl. And I still carry my Epi-pens everywhere, even in a tiny evening bag.
Xolair’s not a miracle, but I’m certainly happy with it.
What are those damnable cells doing?
Beginning in 2012, Dr. VL regularly monitored my stubbornly high eosinophils. Most high eos levels are due to an allergic reaction, helminth infection or other demonstrable cause, like certain cancers. But mine would increase on a cyclical basis we’ve never understood.
Researchers think high eos levels, over a long period of time and unexplainable cause, usually indicates that the eos are fueling inflammation somewhere. And quite possibly attacking some organ. But the problem is, unless you can do a biopsy, you don’t know what it’s attacking.
One thing is certain, though: They aren’t just pouring out of my bone marrow and flooding into my bloodstream in order to take a tour – they are attacking something!
My various specialists examined me carefully, and continue to, whenever something new crops up, taking a biopsy or sample and checking for eos. And each one so far has turned up with blanks, which was encouraging in many ways. But it also continued to feel like something horrible was waiting around the next corner – what weren’t we able to test? Where were they?
During this period, my doctors were diagnosing a LOT of other medical problems. Some were caused by the steroids, some were of unknown origin. But to me, it’s felt as if something else has been going on, that something has been linking them. Coping with all of them (we’re up to 60+ now), plus asthma and eosinophilia, has been challenging at best, and sometimes completely overwhelming.
An asthma spectrum
Also oddly, during this period, my asthma attacks were continuing, but changing in unusual ways. For example, after a lifetime of asthma characterized by wheezing, it stopped. Still had the feeling of tightness and pressure on my chest, but first less wheezing, then none. And there was less responsiveness to bronchodilators. Which meant, in all likelihood, that I had developed eosinophilic asthma, a relatively new type in the recently expanding category of lung diseases called “asthma.”
Finally in 2016, Dr. VL talked to the NIH’s top researcher on eosinophilic diseases, Dr. Amy Klion. Dr. Klion has been working with an even rarer subgroup of eosinophilic patients, those with HyperEosinophilic Syndrome (HES). Dr. Klion reviewed my case and said I probably have an atypical form of HES.
HES is normally characterized by high levels of eos that never drop. Mine, oddly, would drop, then in a matter of days start to climb again, something I eventually grew to be able to feel my body do. Such a strange sensation. And we have no idea why the roller-coaster. Someday I hope we find out.
A last-ditch cocktail
Dr. Klion recommended that I continue the Xolair, which had been so successful, but then add a new biologic called Nucala, and see if I responded to it.
Biologics are different in some specific ways from more common drugs. Sometimes biologics can trigger viruses that are dormant in your body. I needed a shingles vaccination to reduce the severity of any recurrence before I could begin Nucala. Even though I had already had shingles three times – oh, the joys of crappy immune systems! Then the injections began. And once again, although faster than with Xolair, there was a small, but noticeable improvement in how I felt almost every month.
I started remembering a little of how it used to feel to be me. To go over to a friend’s for dinner – and not worry about their cat. To slowly feel a little more energy, a smidgen more strength, each day.
I have to remind myself, and my family and friends, that there are no guarantees. Nucala, Xolair and the other biologics to come – all can trigger anaphylaxis at any time, with no warning, and fatally. There are also no guarantees that it will continue to work, and won’t cause other serious, possibly permanent, side effects.
But Nucala, in my case, triggered corneal ulcers caused by the zoster virus – and had to be stopped. Although I was ultimately prepared to trade my vision for my lungs. (In the Alves Hierarchy of Need, breathing is more important than sight.)
Biologics are the big leagues of medications. You only qualify if you have no other choice, and if you are willing to gamble with your life. Of course, for most of us, we’ve been gambling with our lives every day since we were born.
