Time to Bust Out

Yesterday was a tough day.

Our appeal to the insurance company went down in flames, refused once again. And our other option of applying to the Ontario Drug Assistance Plan really isn't an option since another ECD patient was recently refused coverage for Kineret, after an appeal as well.

We're hoping that the manufacturer of Kineret will help us out, but I'm not terribly optimistic since they made it very clear at the beginning of my three month trial that it wouldn't be extended.

I have just a few vials of Kineret left, and have started alternating my shots every other day in order to stretch it out. I wonder how soon after that last vial I'll be again hobbling around and in significant pain? The thought scares me silly. Although I still don't have a lot of energy, being able to sleep (mostly) pain free and being able to get up and down the stairs for the last couple of months has been a huge blessing. Just being able to go to another floor of our home has helped me to stay positive.

The sad thing is that if we (myself and the other Ontario ECD patients) had cancer or other prevalent serious illness this wouldn't be an issue. This denial of treatment is down to one thing and one thing only. There is no drug anywhere that lists on its label "for the treatment of Erdheim Chester Disease". A few words keep us from getting treatment coverage.

When I first started this blog almost two years ago I explained why I was using a pseudonym, being self-employed I didn't want to scare away clientele. The reality is that it looks far less likely that I'll ever get back to work and all I'm looking for now is a bit of mobility and pain relief for whatever time I have left.

So we launched one of our big guns yesterday. A letter was sent to a reporter of a major Toronto newspaper who had recently written a series of articles about a young patient refused access to an expensive drug that was provided to another patient elsewhere in Ontario at no charge. We're hoping that International Rare Disease Day falling on Monday will open the door to conversations about treatment coverage for rare illnesses. At the very least shame our insurance company and the government into helping us.

We're well aware that the prognosis for ECD is not good. Kineret and other drugs being prescribed around the world for ECD are not a cure. I'm just looking to spend the rest of my life in a bit more comfort rather than constantly be battling for the opportunity to get some relief.

Let's Play the Good News/Bad News Game

The Good News:

Last week I went back on some heart meds that didn't work out well for me last year, but in combination with Kineret things are looking up! It's taken some experimenting with dosages and the time of day that I take this new drug but I've had two good days in a row. I even felt well enough today to drive for the first time in about a month. Just to the pharmacy (to pick up more drugs mind you) but it felt wonderful to get out of the house.

The Bad News:

On the way back from the pharmacy this morning I picked up the mail. I didn't expect to hear back from the insurance company so soon (the application for Kineret coverage only reached them Thursday evening) - but I could have waited a bit longer for this news. No, no and absolutely no. No coverage for ANYTHING that doesn't specifically state that it's for the treatment of Erdheim Chester Disease. No such thing. Not one drug in existence that states that it's for the treatment of ECD. For the icing on the cake, the letter was unsigned without a printed name of the bottom. An anonymous slap in the face.

So despite the fact that medical teams around the world are investing heavily in research to improve the quality of life, and hopefully the lifespan of ECD patients - their findings are dismissed by our insurance company. And our government also declines to help (a fellow ECD patient in Ontario already had to find this out the hard way, and I've been advised that it's not worth the effort to apply to the government after another patient has been refused coverage. Again because it's "off-label").

I have two and half weeks worth of Kineret left in my fridge. I try really hard not to dwell on this point, but the stats tell us that 60% of ECD patients die within 32 months of diagnosis. I'm at month 25. And I'm in the group (cardiac involvement) that usually lands in the 60%.

So I play that stupid mind game of "what would you do if you were told that you had six months to live?" Fight like hell with our insurance company and government for a drug that might improve those odds? Or save whatever energy I have to spend time with my family? There is no right answer to this one.

I'm not the only ECD patient (or person with a rare and serious illness) out there and many of us are dealing with this issue. Things have to change. Change so that we can stop wishing that we instead had a "common" serious illness for which drugs would be handed out without a blink of an eye because of what is printed on the drug label.

Where to Lay Blame

I feel as if I've been hit by a bus the last few weeks. What did I do to myself to set off this latest round? That shovelling from a few weeks back? One of my meds? Something I've been eating?

It's natural to search for a reason when things change. I have a difficult time admitting that it might simply be the natural progression of my illness; that I have to be more accepting of how I'm feeling and the limitations that my health places upon me.

Not quite ready to blame Kineret however. I'm nine weeks into this trial and it's still helping greatly with my mobility and to some degree with my bone pain. My heart on the other hand has been misbehaving. Badly.

It's been rounds of scans and other tests to see what might be going on besides the pericarditis. We're waiting for results of the latest CAT scans to see if my lungs and brain might have developed any problems. Nothing seems to be working right from the weird tremors that shake my hands, the numbness in my foot, and the continued breathing difficulties.

And I discovered yet one more medication that I'm allergic to - Pulmicort. I was put on this steroid inhaler to see if it would help with my breathing but all I got was a lovely rash from neck to knees for a few days.

It's hard not to feel beaten down and trodden upon by ECD when it's hard to do anything more than lay on the couch, occasionally getting up to do something useful around the house that doesn't require a lot of exertion. Every action is measured, is it worthy of how it's likely to make me feel afterwards?

Yes, I'm feeling sorry for myself right now. This just isn't me. It's me with ECD tugging at me every single moment until I want to kick it clear across Lake Ontario. If only I could muster enough energy to at least get it to the curb for a few days.

Self Admissions

I detest admitting that I'm sick. But ECD has slapped me around a bit the last few weeks, and just maybe it's time to realize that I shouldn't push myself so hard. I think I've said this before but maybe it's high time I had a serious chat with myself. And actually followed through.

I'm eight weeks into my twelve week Kineret trial, and decided today that I definitely want to keep taking it beyond the trial period. Now comes the funding nightmare, but the upside is that the drug company is helping me this time. I suspect that their voice is much louder than mine!

Kineret has helped greatly with my mobility, and now with the bone pain (I've hardly had any the past week!) The cardiac issues are something else, but maybe Kineret needs more time. Or I need to stop pushing those boundaries so hard.

Although it felt great at the time, shovelling the snow a few weeks back seems to have set off weeks of misery. What the heck did I do to myself? I'd been doing so well in the first few weeks of Kineret, did I undo all that in just one hour?

I've made a promise to my family. No more lifting, no more "let me do it!", and most definitely no more shovelling. No more of anything that feels like it just might be more than I should be doing.

Give the inflammation in my heart a chance to settle down, to let Kineret do its thing. To hopefully breathe more normally again (talking on most days this past week sets off a round of coughing that leaves me gasping for air). Not to mention the tremors that have kicked in too. What an adventure one night last week trying to get food on my fork, and then fork to mouth. I came way too close to asking someone else at the table to feed me so I didn't leave the table hungry.

My doctor called this morning (on his day off no less) to let me know I'd be at the hospital longer than usual tomorrow. He didn't want to say just yet what tests were being lined up, but I'm ready to know more about the extent of the ECD in my body.

Not only ready, I need to know.

New Friends

Bad things happen. And not that we want bad things to happen to others but there is some comfort to be found in knowing that you're not the only person facing a particular challenge.

Finding the Erdheim Chester Global Alliance has allowed me to connect with other ECD patients around the world, and for this I've been grateful. And I'm so very thankful for the efforts of the Alliance volunteers who maintain the website , raise funds for further studies, provide us with a forum to communicate and much more. Especially the "much more" part - a special thanks to Kathy who is always there to listen and offer suggestions as we navigate the maze of dealing with a rare illness.

Here comes what could be a very long story, but I'll keep this short. In the last week I've connected with two ECD families, one about a five hour drive away and one about a ten minute drive away. The first diagnosed in the last couple of months, the latter in the last two weeks.

I was floored. Here are two families that face the same Ontario health system, in some cases the same hospitals (however not the same doctors, but we'll be working on making some connections between our medical teams!)

Of course I wouldn't wish this illness on anyone, but there was elation in finding nearby "teammates" in this challenge. Last weekend I even met the wife of the patient who lives very close by and we hope to get our families together soon (and the one further away promises to be in this area later this year).

I'm excited about connecting with these two families, and we've committed to working together towards getting drug funding (we're all facing the challenge of getting help paying for Kineret) and other issues that might be unique to furthering the cause for ECD for patients in Canada.

On the subject of Kineret, I'm well used to the shots now. A nurse from Biovitrum (the maker of Kineret) called this week to check on me and gave me some very useful suggestions for making the injections a little less painful.

Here's the dirt.
After six weeks of treatment my joint pain has greatly diminished to the point that I'm walking quite normally again.
Bone pain is still there, but less frequent. When it does come it seems sharper, but I'm concentrating on the "less frequent" part!
The cardiac pain has still been a bit of a challenge. Today is the first day in about two weeks where I've not felt it (I hope I haven't jinxed myself, still about six hours to go!) The pericardial rub is still going strong, although my doctors feel that it's less widespread across the heart and concentrated more in one area.

Still very optimistic about this trial!

Too Stubborn For My Own Good

In my last post, I was boasting about how I had shoveled the driveway twice over the weekend when we got a good dumping of snow. I tell you, sometimes I really ought to be more careful - I'm often pushing my limits a bit more than I ought to. Despite my husband asking me to take it easy and offering to do these more vigorous chores instead - there I go and try and do it myself.

After having a great week I think I undid my progress; I ended up in bed for a few days trying not to get my heart too agitated - it really let me know that I had overdone it.

Lesson learned. For now. :-)

Next obstacle was my flu shot. Yes, I should have gotten it a couple of months ago but there never seemed to be a clinic date that worked. At an appointment with our new GP a couple of days ago (we had to find a new local doctor, it's a long and boring story) Dr. M suggested that we get our shots, especially given that Kineret plays havoc with the immune system. She had the vaccine on hand and my daughter and I got our shots at long last.

Another setback. I've gotten my flu shot annually since my daughter was born (I'd been a single mom much of that time and wouldn't have been much use to her if I'd been sick) and never had more of a reaction that a bit of soreness in the arm and a slight fever the next day. This time was quite different (and I can't blame the Kineret, my daughter had a similar reaction and wasn't able to go to school yesterday because of it) - this time we both were lightheaded (to the point of having difficulty standing), nauseated, and generally feeling pretty icky. We're both starting to feel better today, and appreciate this is still much better than having the flu for a week or more.

So after a difficult week, I say the same thing that I say to my husband at the end of every challenging day.
Tomorrow will be better.

Going Strong!

What an amazing week.

A few days ago I had my first check-up with my doctor since starting Kineret (I should say doctors, there was a quite a parade of them coming in to see how I was doing!)

Although my pericarditis can still be heard through the stethoscope, it was generally agreed that it has become quieter. One cardiologist who's listened several times was sure that it was even undetectable every few heartbeats.

Less bone pain every day. I've even had some completely pain-free days, and I've dropped my pain meds by half - and intend to drop down further over the next week.

The rashes are tolerable, and I've stopped my oral antihistamines. I was given a stronger topical ointment and that seems to be enough.

I can't tell you just how much better I'm feeling as each day goes on. The odd day I've had a setback; it seems that increased sodium intake really doesn't agree with me especially since I've been on Kineret. It throws my heart into an arrhythmia for about twenty four hours but that's fairly easily avoided (but I really had to have that steak wrap last week when we went out for lunch with friends, it's been so long since we'd been out and I'd been craving one since I last visited that restaurant over two years ago!)

This is all great stuff but the real measure of how well I'm doing is what activities I can do now that weren't possible before. Let me sum it up. I shovelled the driveway. Twice this week. And this morning was a pretty heavy snowfall! And not one single twinge from my heart. This may not seem like a huge deal, but for someone who most days had quite a bit of trouble getting up two flights of stairs at once this is of enormous relevance.

Although my doctor is very cautiously optimistic about what Kineret will do, I can't help but be pretty darned excited about how I'm feeling. And I'm feeling hope.