Two Weeks In

What can I say? I feel better.

Walking has become so much easier, I'm not cringing anymore as I make my way up and down the stairs (and we have lots in our home).

My almost constant fever of the last year has all but disappeared. Just one day last week and even then it was quite mild.

I'm still having cardiac episodes, but they're happening infrequently and don't knock me down as hard as before. Still having shortness of breath, but hey - we're only two weeks into this treatment!

The bone pain is still there, but I'd say that it's becoming less frequent. Sharper pain when it does happen - but I'm being optimistic that it means that the Kineret is doing something. And by something, I mean something good.

As predicted by the nurse at the hospital I've started in the last two days to break out in some unsightly (and fairly itchy!) skin reactions at current and previous injection sites. Tolerable so far, they seem to be under control with oral allergy medication - I'm saving the corticosteroid creams for when the itching is driving me totally crazy! And as I've been told - just hang in there for that first month to six weeks and this phase should pass. So I won't be running around in a bikini anytime in the near future - but who am I kidding, I haven't been scaring people that way in twenty years!

The injections are getting much easier. Just part of my daily routine now, no stressing about them. Yes, they still hurt a bit - but I suspect being relaxed about the shots makes them easier than they might otherwise be. Finding a spot that isn't inflamed might be a challenge for the next while though! We'll make it a party game - help me find a spot that doesn't look like I had a fight with a patch of poison ivy and you win a prize - I'll run up the stairs to fetch anything you need! 'Cause I can now!

I'm sure you can tell that I'm pretty darned pleased with the way the Kineret treatment is going so far. And I'm certain this will only get better as the weeks go on!

Seven Injections and Doing Just Fine

I'm getting used to the shots. Really.

After seven of them I find it easy to quickly poke the needle in, push the syringe and before I know it I'm done. Yes, it does hurt a wee bit for about ten minutes but given the improvement I'm already seeing the small amount of discomfort is well worth it.

It's way too early to say if this experiment is a success (I'm still experiencing some bone pain and my local cardiologist heard the pericarditis still active at yesterday's appointment) but I'm quite impressed for what the Kineret has done so far.

For the last year I'd become increasingly arthritic. I was avoiding stairs whenever possible, and walking even short distances was taking me much longer. Plus I'd be paying for it afterwards with significant joint pain and stiffness, especially the last few months when that first swing of my legs out of bed in the morning told me that I was in for a painful day.

Perhaps I'm exaggerating when I say this - but it does feel like I'm bounding about the last few days. I'm easily tackling the stairs with armloads of laundry, even getting some holiday shopping done with my daughter yesterday - and she didn't have to walk at a snail's pace for me to keep up with her!

I'm feeling "normal-ish". I can't tell you the last time that I felt so able. I'm certainly not up to the pace of a few years ago but not being reminded that I'm ill every time I move has been a tremendous blessing.

I've yet to experience the injection site reaction that is very common with Kineret, the nurse at the hospital told me it's likely to start up within a week or so. Just hang in there for that first month when things can get a bit nasty and I should be in for an easier time after that.

Time to just enjoy what's happening right now. We don't know if this will get rid of the pericarditis or help with the bone pain; only time will tell. But I'm feeling very optimistic! What a tremendous Christmas present that's been given to me. To the three of us.

A Breeze

Yesterday I was at the hospital learning how to give myself the Kineret injection. I'm really not nervous about needles and such, but I'll admit the idea of giving myself a shot in the stomach did give me a slight case of the creeps (the injections should be rotated between a few different areas in the body to lessen the skin irritation, the stomach being one site).

So, let's get that first shot I take into my belly! My nurse was so helpful and calming and before I knew it I'd stuck that sucker into my stomach and pumped my precious Kineret in. Next stop was back to my doctor's clinic upstairs to watch for any reaction. At first there was a wee bit of blotchy redness about 2 inches across, but that was gone within an hour. After that, nada. Two hours later, nada. THREE hours later, nada.

Shortly afterwards my doctor came in to review how I was feeling. Any concerns about an allergic reaction were calmed, I was doing just fine and able to go home.

Honestly, I think my doctor is far more nervous that I am about this. He's leaving today on two weeks vacation and wishes I would have waited until his return to start the Kineret. I'd explained that I really appreciated everyone moving heaven and earth to make this happen THIS week - emotionally I needed to be on more solid ground going into 2011. Feeling more hopeful about the year ahead. The last two holiday seasons were quite difficult, the first was a diagnosis of a leg tumour of (at the time) unknown cause and possibly cancerous, and then last year I was in the middle of weaning off prednisone (which took yet another four months to do successfully) and feeling quite horrible day in and day out.

This year I'm hopeful. I know it's way too early to predict anything after just one injection, but I feel very positive that this drug will improve upon my current quality of life (which to be perfectly blunt has really sucked the last few months).

So, yes - I did push my doctor into starting Kineret before his vacation. He's prepared me well with the pager number for the chief resident at the hospital (who's been prepped on what to do in case I get into trouble), we have antibiotics on hand in case of infection, drugs to help lessen the expected injection site reactions due to come along in a week or so. And I'm good with this. My doctor not so much, but he respects and understands how much I needed to do this now. Before a new year begins. How could I let the opportunity for three months of free access to the drug wait any longer than necessary after we've been hoping for this for so long?

For anyone considering Kineret as a treatment, let me say that the shot itself didn't hurt. It stung a bit for about fifteen minutes afterwards, but I'm pretty sure this was because the syringe was only out of the fridge for about ten minutes before the injection and I've heard that it's much easier if the solution has warmed for an hour or so.

So upstairs I go now for my husband to take his first stab (!) at this in my arm, he practiced this morning with an empty syringe on an orange. He's concerned about hurting me, but I know there's nobody who cares more about whether I feel pain - I know he'll do just fine.

Tomorrow

I have my Kineret.

And tomorrow morning I go to the hospital for my first injection. Quite a few people have worked very hard to have this happen before my doctor and his team go on holidays (they leave tomorrow afternoon). The stars have been shining on us over this last week!

Making my Daughter's Boyfriend Feel at Home

Still waiting on info about starting my Kineret. I'll need to be monitored at the hospital when I take my first injection or two (in case of allergic reaction), but my doctor is leaving on vacation for two weeks on Wednesday (my Kineret arrives that afternoon) - we're hoping to line another doctor who's around over the holiday break but so far no luck. You mean nobody wants to cancel their vacation to babysit me? :-) So it looks like there's a possibility I won't start until the new year.

We can't have many visitors here at home, especially at this time of year when the flu is rampant. I pick up colds and infections too easily (and this will be even more the case when I start Kineret) so anyone coming over has to assure us that they're feeling well - and the first place they head coming in the door is the bathroom to wash up. Just the way it has to be.

Most of my socializing is done over the phone, on Facebook or on email because of these restrictions but there is one visitor who comes by quite often - my daughter's boyfriend (BF). They've been together nine months - he knows the drill and takes the necessary precautions to not expose me to illness. I'm delighted that this doesn't faze him - we love having him around!

He's become quite familar with the odd sense of humour that resides in this household (he's very sweetly told us this is the weirdest family he knows!)

Here's an exchange from a few nights ago.

Me to my daughter and BF : Thanks so much for picking up my prescription at the pharmacy this afternoon after school (she's driving now!). Now that I think about it, I should have had you drive me over there to get it myself. Did the pharmacist explain how to use it? She usually takes me aside for a consultation for new meds.

Daughter: no Mom, why?

Me: Actually, I'm a little surprised that she didn't insist that I come in personally given what it is.

Daughter: Really? What is it?

Me: Um...medical marijuana.

I couldn't have timed it better. BF was taking a long sip from a water bottle for that last bit, of which most ended up being sprayed across the room. My daughter caught on right away, she know that I've never had an illicit drug in my life, and don't drink alcohol either. It would take some pretty heavy convincing from my doctor to have me smoking up, even for medical reasons!

We may at times be totally inappropriate, sometimes morbid and almost always a bit goofy in this home - but humour is what's getting us through this together. I'm relieved that BF fits right in!

On Cloud Nine!

Kineret. Arriving by Wednesday at the latest.

Just had a conversation with the drug company liaison (am I ever glad I picked up the call coming from an unidentified telephone number at 9 pm on a Friday night, something at times I'd let go to voicemail suspecting a telemarketer).

I can't believe this is actually going to happen after a wait of almost a year. I can't tell you how badly I needed this news, it's been a tough few weeks. I'd made the decision to (hopefully temporarily) shut down my portrait photography business, and had let my clients know about two weeks ago.

Some very thoughtful clients sent wonderfully supportive notes, but I must say - there were a few responses that really soured what was already quite a painful exercise for me. Several individuals to whom I'd donated portrait packages annually to support their favourite local charities tried really hard to make me feel bad for not being able to donate another package this season. Couldn't I commit to mustering the energy to do just one session in the spring? For just a few hours? (my photographer friends would cringe, no session is just a few hours - most of the work comes afterwards).

Oh, and if I couldn't deliver a portrait session - could I donate something of a similar value ($200 - $500) in its place? Maybe you missed the part about me not working anymore, that also means no income for a self-employed photographer without employment insurance.

And the best one of all. After at least three years of promises "we want to book you very soon for a session, we really love your work!" (emailed to me at least three or four times a year), I received an email right after my announcement in my monthly newsletter saying " that's a shame, we're finally ready to book - can you refer me to another photographer?" Ouch!

The latter part of this post was what I was originally going to write tonight to all of you, it had just been too hard to say right away. My photography means everything to me, I hope that has come across in previous posts. It had just become too hard to hold my camera to my eye for any length of time, and a session would wipe me out for days afterwards. And I'd become so unreliable for a booking, resting up for a few days prior just wasn't working anymore. And I detest being unreliable, but that's my story these days.

But there's hope. I really believe that Kineret will do wonders for me. That I'll be able to work again in a few months. That I'll be able to walk further than a half a block again. That I'll start sleeping again without pain waking me up in the wee hours. That I just might feel a little more like my old self again. Because these days I often lose sight of what normal felt like. I know I'm asking for the world, but just maybe I might get a slice of something great coming my way.

Hanging With "My People"

Last night I was able to make it to the hospital in Toronto to attend the dedication ceremony for my doctor with the help of a family friend who drove me to the train station and my husband who "caught" me on the other end.

What an evening it was. Maybe eighty people were there, mostly doctors, a very generous philanthropist who has donated millions over time to the division that takes care of me, and one patient. Me.

I can't tell you what an honour it was to be there. I had a special role - it was the unveiling of a painting/portrait of my doctor for which I had taken the original photograph earlier this year as a special request (it even has my husband and daughter in it too!). The painting will hang in the newly renamed wing; the one I frequent so often that many of the doctors passing through will wave in recognition when we catch sight of each other.


I was among "my people". Certainly not as a peer, this was a gathering of some of the most respected doctors in this city. But I was among people who get what this battle is all about. Who have great respect for this amazing doctor that cares for me, but also respect me for trying to beat this with a smile on my face.


They understood that it was hard to physically get there last night. They get that this disease has a poor prognosis, yet applaud my efforts to keep a brave face.


With a few exceptions, most people outside of the medical community just don't get it. I could go into examples of how little others often understand the challenges of dealing with a serious illness but I'm going to let someone else speak on this frustration - I recently became aware of a blog written from the perspective of a spouse of a person with ECD. She writes frankly about their experiences with "the outside world", and I have to say although not all of their frustrations are what I've experienced, enough of them were similar that she and I connected immediately in emails that have gone back and forth.

If you'd like to read about their lives - the blog can be found here. And I'll post a link on this blog too.


I'd also mentioned in yesterday's blog that I would be going to the dentist today to check on my painful jaw. Not good news I'm afraid, it would appear that ECD has asserted itself there as well. But in need of a "yay", this household is glad that that I've kicked my recent grumpiness aside!

YAYs and BOOs

Just a quick post, I'm on way to the hospital. For once not for me, my doctor is being honoured tonight and we're invited guests (a photography project that I worked on for the hospital is being unveiled tonight). Yay Dr. H!

Yay again to Dr. H. - he managed to get me a three month trial supply of Kineret. A very persuasive man is he, we got the phone call late yesterday. Waiting now for a shipment to arrive from Sweden, and then I start my injections. That's worth at least a quintuple yay!

Boo to my hip. It's been causing me quite a bit of pain, and the results of an x-ray came in yesterday. Doesn't sound like good news, but I'll have a bone scan to confirm exactly what's happening there.

Boo to my jaw. I get so tired of bad news that I neglected to have it checked out when my teeth started not fitting together about six weeks ago. And then it became difficult to eat (which has certainly helped me dump some of this last steroid weight, so that's a yay). While I was on a heavy dose of prednisone for almost a year I was warned that bone problems can develop in the jaw and hip. Am I lucky enough to have gotten both?

Yay to my husband and daughter. I've been a major grump this week. They forgive all, I'm one lucky wife and mom.

Too Much Time, Yet Not Enough

I feel like I'm spending months inside of much shorter time frames. Now that I've written that it doesn't seem to make much sense; but there's too much going on to have logically fit into the last two weeks and that's the only way I can describe it.


Two weeks ago I got a long awaited call from the hospital. The researcher had decided, that no - I was not going to be taking Kineret. For a reason that floored me, he told my doctor that the shots are too painful and patients tend not to comply for that reason. Just one sec here, has a word I've said even been heard? What about short term pain for possible long term gain? Doesn't he know that I'm one tough cookie?


I went to bed that night completely dejected. After all these long months of lobbying, the answer wasn't at all what I had expected. Despite taking my nightly sleeping pill (my leg and chest pain has gotten a bit much at nights lately, and it helps to take the edge off so I can get to sleep) I found myself staring at the clock at 4 a.m. not having slept a wink. I quietly crept downstairs to my office and spent the next two hours composing a letter to my doctors. A rational, unemotional plea to reconsider. What other options do I have right now? So many of the other drugs used for ECD can further complicate my cardiac condition so I really don't have much else to try.


When my husband woke up a few hours later I asked him to read the letter to make sure I was as lucid in the wee hours as I though I had been. He encouraged me in fact to put a bit more emotion in my words, to get across how devastating this decision felt.


A few hours after emailing my letter I got a call from my doctor. He'd spoken to the researcher and Kineret was back on the table. We're still waiting to hear if this is going to happen, but at least we don't have a definite no to deal with. And now it's back to being about funding.


Coincidentally, the Toronto Star published two articles in the last few days that really sum up our struggle to get access to off-label use of expensive drugs.

http://www.healthzone.ca/health/newsfeatures/article/894062
http://www.thestar.com/news/canada/article/894576--8-ontario-patients-in-need-of-500-000-a-year-drug-therapy?bn=1

Should I feel some comfort in knowing that I'm not alone in this battle?

Lots more to share about the last two weeks, but I'll save that for another day. But on the drug funding front, we're still waiting.

We're Still Giggling!

Despite the somewhat more somber mood in our household, we're still trying to hang on to our sense of humour.

A few days ago, my daughter got a call from her Dad's house (she splits her time between two households), asking if there was any particular reason why her school uniform was strewn across the front lawn. Turns out she had stuffed her clothes into her school bag as she ran out to the driveway where her driving instructor was waiting (he was going to drop her off at our house afterward and she needed her uniform for the next school day). They'd fallen out as she swung her bag over her shoulder. Easily solved, my husband picked up the clothes that evening. No worse for their public airing.

The next morning I felt well enough to drive my daughter to school (she otherwise takes two buses which don't always connect as scheduled due to lots of local road construction). We leave pretty early and I didn't feel like showering and dressing for the half hour round trip. The sweatpants I'd been wearing the previous evening were handy so I pulled them over my pj bottoms so the neighbours wouldn't be able to see that I hadn't bothered to dress properly.

My daughter got in the drivers seat, and I climbed in beside her (btw - she's getting to be quite a good driver, keeping our fingers crossed that she passes her next exam to allow her to drive on her own).

An uneventful trip, and I was soon back on our street. What the heck is in the driveway? Can't be...

I hurried over to the side of the drive where I'd climbed into the car 30 minutes earlier. A wee bit embarrassing - but it was my panties. In plain view.

They must have slipped out of the leg of the sweatpants, I hadn't separated my laundry in my exhausted state the night before.

How many of my neighbours saw, and how many are wondering what kind of drugs I'm on these days? And why can't the women in our family keep track of their clothes?

We truly are still finding lots to laugh about. And my latest comedic indulgence? "Modern Family". Did you catch the episode about Gloria's accent and mispronunciations? That sure brought back memories of growing up with a mother with a strong German accent. I can't tell you how many times my friends would look at me with alarm, clearing misunderstanding what my Mom had just said.

I don't think my husband and daughter can understand just how much it helps when they get me laughing. And they do, every single day (especially when I keep inadvertently providing the fodder!)

Just Don't Get Me Started

This afternoon I had an annual appointment with my local GP, I don't think I've mentioned him before in a post. One of these female check-up type thingies, not my favourite things to do but gotta suck it up and get it done. Or do I?

I'm at that age when a few extra tests are requisitioned at the annual check-up. None are terribly pleasant; parts get squished, prodded, palpated and swabbed. But lately I wonder why I'm getting them done; if I'm being honest with myself I believe that it's highly unlikely that if cancer was detected that I'd do anything about it at this stage. I realize I might raise an eyebrow or two out there, but really - I'm already dealing with a serious illness that has no cure. Would I subject myself to more surgery/treatments than what I'm already prepared (or not prepared) to do to deal with the ECD? And really, could anyone have that much bad luck with their health?

Dr. A. has been getting updates on my condition from the hospital in Toronto. As he started our conversation he already had tears in his eyes, confirming that what he'd read was not good news. I'm not sure how I feel about his reaction. On one hand I recognize that he's a kind, compassionate man; isn't that what we'd all want in a doctor? On the other hand, his reaction brings out something in me that I don't recognize. It's not self-pity; after all it was me reassuring him at the end of my appointment that I felt quite optimistic about the next drug trial.

I'm used to emotional outpourings when sharing details of my illness with others. It's not something I spend a lot of time talking about, but I try to be forthright when people care enough to ask about the progression of my illness.

Today's appointment? I think I can best describe my reaction as fear. I try so hard not to be overwhelmed by my illness but his tears make me feel like I ought to be more frightened.

I know my GP's heart is in the right place, but it makes me need my husband to hurry home for one of his wonderful hugs that make the rest of the world disappear for just a little while.

The View from the Bottom Step

I'm raising my white flag.

I sit at the bottom step looking up at where I came from. I didn't arrive here with a thud. Nor did I arrive at the bottom. as I would have expected with a slow, careful descension; step by step.

It's taken a few weeks to get here, feeling as if I've been tripping my way down the stairs clawing to grab onto a rail, a step or by some miracle a tremendous gust of wind to nudge me back up a step or two.

The quick decline in my health has been horrifying to me. I've tried to keep some normalcy; cooking a couple of meals a week, keeping up with the laundry, even driving my daughter to school once in a while. At the very best I've even been able to do two photo sessions with clients.

At its worst, I realize that this is all slipping away from me very quickly right now. Most days are spent in bed lately, trying to keep my mind off of the pain that seems to be settling into other bones with a burn that at times is nearly intolerable. Trying to save up enough energy to do a few simple tasks each day. Trying to ignore that my pacemaker kicks in all day long to correct a heartbeat that refuses to keep steady. Trying to keep the relentless fever under control. Watching so much slip away from me.

October was especially hard to deal with. It's the month that as a photographer I would have been out in nearly every kind of weather either photographing families for their holiday pictures, or capturing the fall colours in their glory for the galleries that sell my work. Although most of them have dropped me in the last year because I've not been able to honour my obligations to regularly provide new images.

Last night my doctor called (yes, a Saturday night) to check on me. We're back in to see him tomorrow and by the sounds of it he's ready to try to help me pull out the big guns. Enough of this being stoic and trying to handle the pain and discomfort with a brave face. It's time to admit that I need more help than perhaps I've been letting on.

Within minutes after the doctor's call, I received some very sad news. One of the members of our ECD support group passed on this week. Only 33, leaving behind a young daughter. My heart goes out to her loving, and beloved family.

This really is an evil disease.

The Mirror is Not My Friend

However, my husband and daughter most definitely are.

I'm looking pretty haggard these days. Lack of sleep and everything else going nuts in my body have me hardly recognizing myself these days. I've lost the steroid chipmunk cheeks and about half of the steroid weight so far - but all I see are sunken eyes and a rather unhealthy looking tint to my skin. Yesterday I came across a photo of myself taken about two years ago and I seem to have aged at least ten years since then.

But every single day, both of my sweeties tell me that I look beautiful. I'm grateful that they can see past what I'm seeing in the mirror. Or they've both become great fibbers :-)

One upside - every year I dress up as a witch at Halloween to dish out the treats; this year I won't need much make-up to look convincing!

Not a No

Yesterday was hospital day. It was the longest I'd gone between appointments in two years, and I'd had high hopes that there would have been a significant move forward towards the next treatment.

No such luck, but in all it was still a pretty good meeting with my lead doctor. We're in agreement that my health is declining and that we have to come up with some sort of treatment plan. My wish to try Kineret hasn't been shot down, it's still being considered - but we're going to take a different path. I'll be meeting with the doctor/researcher who obtained the Enbrel donation for me - and have him assess me in person and decide on the next step together.

He is however, renowned in his field and lectures around the world frequently - so getting in to see him might take awhile. That's the hard part to swallow - as I get weaker and the "good days" become less frequent it's sometimes difficult to be optimistic about the road ahead. I can't help but hope that the Kineret (or something else that is prescribed) just might be the miracle treatment that improves my quality of life. So I'm not wallowing, right? Still looking forward. Most days.

Yesterday's appointment felt far less clinical than usual. It was an honest discussion about how frustrating it is to treat an illness about which so little is known, and how treatment options have so far been hit and miss with the small number of patients around the world.

I felt that we were skating just above the admission that we seem to dip towards at some appointments. Unless I'm careless crossing a busy street, this illness is what will end my life. My doctor has a way of asking questions of the two of us (my husband is almost always with me at these appointments) to see where I'm at mentally with all of this. Actually where all three of us are at; my husband and daughter suffer through this just as much as I do in their own way.

Yesterday I felt emotionally strong and I hoped it came across that way to my doctor. I sometimes think about the seven stages of grief - and believe that I tend to bounce quiet a bit between them. Up a step, down two and back up again on the ladder. Yet I think that he could read between the lines; the only change to my prescriptions was to make sure that I get outside for ten minutes a day. Drive (or get driven as is the case most days now) to a park, sit on a bench, or walk if I'm up to it - but get outside of the house.

I've learned much about myself in the last two years. I'm as human as everyone else. And I have to forgive myself for not always being Miss Optimistic and Strong. Bad things happen and I'm not weak for sometimes thinking that I got a raw deal in all of this.

After Ten Long, Long Days

Sometimes I think I should have called this blog "Up the Creek Without a Paddle".

It's been a trying time over the last while. Today is the first day in a week and a half that I've not had a fever or significant chest pain. I'm not sure what my body is trying to tell me but I'm really not getting the message. Well, maybe that's not entirely true.

Eleven days ago I felt well enough to go to my hospital appointment on my own on the train. My husband dropped me off at the station so that I didn't have to deal with that long walk from the end of the parking lot. A pleasant ride, lots of families going into the city for the day to visit the CNE (the annual end of summer Canadian National Exhibition). From there, the streetcar to the hospital. Lots of Torontonians detest the streetcar; they're slow, noisy and can be unbearably hot in the summer if you happen to land on an older un-airconditioned car. But I love it. I've always loved this city, and the streetcar shows me a slice of this city that many who rely solely on their cars miss altogether.

My doctor and I discussed our revised plan of action after the disappointing Enbrel trial. We decided to let me have a break from the "more serious" drugs and let me stay on maintenance meds for a month to let my system calm down. I had sent him some info on Kineret (a drug I mentioned in a post early in the year); a study has produced some promising results. The clinical trial documented the use of Kineret for two ECD patients in Europe. Yes, I said two. That's what happens with extremely rare diseases, but I'll take whatever hope I can grab onto. Kineret was initially dismissed by my team of doctors but I'm keeping my fingers crossed that we can start this whole approval/insurance application/beg for a drug donation circus all over again.

After my appointment I still felt fairly energized and decided to again tackle the streetcar, this time with a pit stop for some people watching on Yonge Street. A visit to a favourite store, and then feeling still pretty awake (rare for me!) I decided to walk down to the train station.

I hadn't had that much fun in a long time. I felt normal. Years ago I worked in the financial district, facing these crowds everyday. Although I love my new career, at that moment I missed the normalcy of that life so far away. Busy, productive, somewhat predictable. Travelling a lot to some fascinating cities (and some really not so fascinating!). I regularly updated my five year plan, and it all seemed feasible - and for a few years afterwards it was.

I came home with a big smile. No one who'd seen my that day in the city would have guessed that I'm ill, maybe noticed that I walk a bit awkwardly and slower than I used to. Maybe she has a twisted ankle? Sore from an extra tough workout at the gym?

The next morning I awoke with a fever and excruciating chest pain. And I don't use that word lightly. And for the next ten days it didn't let up except for a few brief hours every few days. No appetite, the most I could stomach was a few crackers to take with my pills. After a week I called my doctor who asked me to double all my meds, and add one more to try to get on top of this flare of pericarditis. He suggested that I might have picked up a virus on my trip to the hospital/downtown - after all my immune system isn't very strong right now. Maybe I just exerted myself far too much on my downtown trip and my heart was rebelling.

I've been feeling defeated, that I will pay each time for a small bit of freedom. It just seems too coincidental that every time I do something a bit more strenuous that a virus is the cause.

And I'm simply amazed that despite the daily gymnastics going on my chest, that my heart still keeps going. One truly amazing example of the resiliency of the human body. For how long?

Today I'm eating again, working a bit in my office and even managed a quick trip to the grocery store. These better days are essential to my mental health and I treasure them.

Being First Is Not Always a Good Thing

You haven't heard from me in awhile, and I'll tell you that this isn't an easy post to write.

Our experiment with Enbrel has failed. Miserably.

There will be people who Google "Enbrel" and will be led to this blog, so I want to quickly say that I understand that this drug has been a godsend for many patients who have conditions that Enbrel is meant to treat. My use was "off-label", and we had no prior use of this drug for Erdheim Chester Disease to use as a reference. And maybe it might even help others with ECD, but it just wasn't a good match for my particular set of cardiac ailments.

The side effects of the first injection were unpleasant but tolerable. The second shot was another story altogether. I won't bore you with all the details but it's been quite the nightmare. Two weeks later I'm still dealing with a number of issues that weren't present before starting Enbrel.

My doctor wanted me to keep going, to have the next dose administered during a hospital stay of a few days in cardiac ward. And I was told that if I went into cardiac arrest, they could be there right away for me (okay, that statement was a wee bit intimidating just on its own).

I mulled over this option for a few days but my heart continued (and continues!) to act up. Every ounce of me is saying this just isn't right and I've chosen to trust my gut instinct.

Despite some very dear and wise friends gently and lovingly telling me that I shouldn't feel this way, I'm feeling like I've failed. That I'd done something to cause such a violent reaction to the drug, that I hadn't been tough enough or not wanted this to succeed enough. People ask me if I was afraid of injecting myself (no, it really didn't bother me at all).

I have 10 weeks worth of this drug safely stored in my fridge at home and it taunts me everytime open it. I think about how hard everyone worked to get me this drug and how long I waited for that lucky break of a donation. I feel like I've failed my medical team and that I've disappointed them.

Deep down I know I have to do what I think is right. But these feelings of guilt are difficult to shake. I worry that my doctors won't be willing to try other treatments on me. On the other hand I worry that this experiment may have shortened my time. My heart is doing backflips and just doesn't seem to want to fall into a regular pattern. I get two minute breaks whenever the pacemaker kicks in, the rest of the time it's annoying the heck out of me with the pain, weird sensations and strange rhythms.

And sometimes I'm just not sure I have it in me to keep fighting this battle as hard as I have been.

Day Six on Enbrel

I've been meaning to blog for days about my first injection, but just figured there would be more to tell as the days passed.

First of all, it didn't hurt much. That's where Googling can get you into trouble! I'd seen so many other patients write on-line about how painful these shots are, but it wasn't bad at all. Burned for a minute or so and then it was over. And I injected myself, pretty darned easy with the Sureclick injector.

So much happened in a few short hours at the hospital, I won't go into detail except to tell you that the nurse who instructed and supervised me was wonderful (as are all of the team members I met that morning) - and to say that the facilities were something else! Beautifully appointed with real wood panelling, lovely lighting and comfortable furnishings. It felt more a visit to an estate than to a hospital (thanks to a very generous donor who wanted to make the patients going through clinical trials feel more comfortable - and it works!)

My first (almost) week on Enbrel has been up and down. At the end of first day, I was completely without any pain. Something that hasn't happened in a very long time!

Second day was unexpected. I'm usually upbeat and optimistic, and I was a depressed, weepy, glop of girl who couldn't shut up about all things sad and grim. I have no idea where that came from! I'm blaming the drugs, but sure hope that it doesn't happen again.

Third day was the toughest so far. I woke during the night with bad stomach cramps, nausea, fever and wondering why I had done this to myself. I called into the Enbrel hotline around lunchtime (they want to hear about pre-specified side effects, fever being one of them) and they suggested going to a walk-in clinic to see if I'd picked up an infection at the hospital (Enbrel greatly reduces my immunity). I decided to wait it out, and sure enough by evening I felt a bit better. Even ate a little, but that day sure helped with dropping off a bit of the steroid weight!

Since then it's been easier, but I can't say that my ECD symptoms have eased at all. The bone and chest pain returned after that wonderful first day, but I do realize it can take a few weeks for any benefit of the drug to make itself known. I don't think that I've been having any side effects from the drugs since Saturday except maybe being a wee bit sleepier than my already frequently napping self!

I'm so used to taking an assortment of pills daily that it feels a little odd to be waiting for my next injection on Thursday. Every night I dream that I've gone to the fridge for the next dose of my liquid gold, and awaken quite relieved that I've not jumped the gun accidentally!

Doing a (modified) Happy Dance!

It was two long weeks of knowing that my donated Enbrel was waiting for me at the hospital, numerous unreturned phone calls to the coordinator (and I missed the one call that did come in last week while I was in the shower) - and finally today I got the call that I'll be starting my clinical trial for Enbrel - tomorrow! (thanks to my husband who lit a fire under someone's behind this morning, he's great at doing that in a most respectful manner).

Thank goodness this is finally happening (I'm very optimistic that this stuff will help!) - I hit a low spot last night that I was unprepared for. My leg pain rose to a level that was close to intolerable - yet I haven't been able to take any stronger pain meds as to keep my body "clean" for the trial.

My poor neighbour came over to our porch last night to check on me (as she often does, I can't imagine a lovelier person to live next to!) and the tears let loose. I'd come on the porch to take my mind off the pain; watching the little kids play in the street always cheers me up. Something about her gentle smile opened the floodgates and she sat for almost an hour with me, encouraging me to vent all this pent-up frustration with the long delays, my pain and my general sense of being pretty useless to my family of late.

With this good news, I'm doing my Happy Dance - modified of course because I'm not supposed to jar, twist, jump on, run with or otherwise compromise my fragile thigh bone - but a Happy Dance all the same!

Nothing New

I'm grateful that many of my readers have been checking in to the blog since my last post. I've been quiet on here because there's really nothing to tell!

Last week I met with my regular doctor in Toronto. He let me know that the supply of Enbrel arrived and was in the hands of the doc who through some pretty deft negotiations obtained the trial dose for me. Had to repeat a bunch of the baseline tests since a few months had passed since we first set out on this path. Normally I'm fairly patient in the long waits for blood tests, x-rays etc. but that day I seemed to have little tolerance - maybe all this waiting, testing and more waiting is getting to me.

Now we wait once again. That drug is sitting in a refrigerator somewhere in the bowels of Mt. Sinai hospital and I await the call that the doctor, my injection instructor, the paperwork etc. are all lined up and I can head into the hospital for my first dose.

Ring you darned phone, ring.

Update - 15 minutes after sending this post out, a call from the hospital came in! Playing telephone tag at the moment, but it's the coordinator of the clinical trial who would like to speak with me. Let's hope we can get things lined up for next week!

Small victories

In a world where it's mostly one step forward, two steps back I have to revel in every success, no matter how small and seemingly insignificant to most. My health continues to deteriorate slowly but I still to try to keep my chin up and remain optimistic. Tomorrow I'll find out when I can begin my three month trial with Enbrel (might be as early as this week!) and we have high hopes in this household that it will help with the pain, ease the pericarditis and let me feel somewhat normal more often.

It's been three months since I stopped taking steroids (huge success on its own there!) and I'm noticing that its effects are starting to wear off. I'm not as fatigued, my muscles ache less, have lost the "moonface" and have started to lose a bit of the twenty pounds I'd gained (down six, hurray!)

However, the leg and cardiac pain unfortunately continue to worsen. Weird thoughts enter my mind when I wake from the pain during the night. If I went into the bathroom, couldn't I just cut my leg off with the nail scissors? What if I just willed my heart to stop for a few hours, wouldn't that give it a much needed rest for a few hours? Nights are sometimes just a bit too frightening.

Back to the days - although I'm not allowed to do any aerobic exercises (and me being me, I've had to test that several times - and it does indeed send my heart pattern into a tailspin every time) I've been able in the last few weeks to get back to doing Wii Fit a few times a week. The gentle stuff; balance, strength, and yoga. Not only do I enjoy it, it does seem to be helping to tone up all the areas on me that seemed to have gone out of control while on steroids. I don't look six months pregnant anymore - those steroids seem to love settling into the belly!

I'll keep you updated on the Enbrel. Although we're thrilled that I've been given the opportunity to try it - there's a small part of me that jumps ahead to the end of the three month trial. What if this doesn't help, or god forbid - makes things even worse? What next? And I then have to bring my mind back to positive thoughts - this WILL help and I'll be able to get back to doing some of the things I love to do. Keeping everything crossed that is cross-able!

Sharing Fantastic News!

Thanks to the considerable and determined efforts of my doctors, the drug company has agreed to donate a three month supply of Enbrel. This news came a few hours ago with a phone call from my specialist (who interrupted his vacation to follow up on the progress of the appeal).

Since hearing the news I've had a huge grin on my face - along with more than a few happy tears. Although I've been trying hard not to think about it, thoughts about how to finance this drug have dominated my thoughts of late and it's such a huge relief to hear the decision. And of course a much bigger relief that the answer was yes!

We don't know if Enbrel will help, I'll be the very first Erdheim Chester Disease patient to try it. However, I'm beyond grateful for the opportunity to see if it offers any improvement. If it works to slow down the progression of my illness, we'll need to look at avenues for financing of the drug beyond the next three months. But we can't worry about that right now, we just have to hope that this stuff helps!

I'll hear more next Monday when my doctor is back from vacation, I'll keep you updated. I know that many of you have offered your good wishes and prayers for good news. I know that they all helped, and I thank you!

No excuses, well maybe just one!

Although the prognosis for ECD can be a rathering depressing prospect, I'm trying to stay optimistic about being around for quite awhile. Mind you, when I first learned of my diagnosis my husband I did make some preparations with our lawyer and bank, it seemed like the rational thing to do.

But beyond that, I continue to try to grow my business (I can work infrequently, but it's a joy when I can!) , to keep learning, to make plans (although it's like trying to nail jello to a wall at times). I detest being a burden on anyone but the reality is that I can't do many activities that I once could. So, we hired a cleaning lady to come in every two weeks and from time to time I have to pay someone to take care of chores that I can no longer manage. But I try. I really do try to do what I can - cooking, laundry etc. No excuses.

A big crossword fan, I love spending the last half hour of the day doing a puzzle or two. The really hard ones. The ones with the five stars, the ones marked as challenging - I love making my brain work (or more accurately my memory!). I used to persevere until every last square was done, and I'll admit to cheating now and again with a peek to back of the book. Shame on me, but I just couldn't start a new puzzle until the old was done!

Things are different now. If I'm not enjoying the puzzle theme, or just don't feel like finishing - I don't. Move on to the next puzzle (I work from the books with hundreds to choose from), leave squares open and blank. I think I might be driving my husband a little crazy with this, he's a die-hard "finisher"!

But I tell myself, I'm sick - I can do what I want. For only this one little thing. Puzzles. Everything else? Keep plugging away...

One of those days

It's a rainy Saturday, and I could really use a bit of Fizzy Lifting Drink. Just sayin'.

I'm not sad, not discouraged - just indifferent today and I'd welcome a Willy Wonka Magic Ticket to add some excitement to my day. I'm allowed to live in a fantasy world now and again. :-)

Maybe I'll just settle for my mid-day vitamin fix (maybe with a bit of chocolate, because you never know)!

Still waiting

I'd mentioned a couple of posts back that our insurance company had refused coverage for an experimental drug. My husband (not one to give up easily) launched an appeal to have them reconsider their decision. Still a bit fat no despite his rather compelling plea to let me give this a try. They've made it very clear that their company will not fund any treatments for Erdheim Chester Disease since nothing has been proven to help. Same position coming from our government health agency.

My doctor hasn't given up hope, he's approaching the drug company again with results of the research conducted by my medical team, as well as seeking out alternate potential donors. I won't be giving up hope either until he raises the white flag.

It makes me wonder however how anyone who isn't wealthy can invest hope in a new treatment. I realize this isn't the case with all diseases, but it comes back again to not having enough ECD patients to make it worth anyone's while. While I say my prayers for anyone who is ill, I'm wishing extra hard for anyone unlucky enough to be dealt a very rare condition. This certainly has been a learning experience in so many ways.

Speaking of learning, I've found it quite difficult to explain exactly what ECD is to people who've not heard of it - which is basically everybody. I dread going to any practitioner who asks if there have been any changes in my health status since my last appointment. Yesterday I came across a description of Erdheim Chester Disease that is succinct and I believe, fairly clear for the lay person to understand. I think I'm going to take a suggestion from the ECD support group, print this one out and give it to anyone who needs to understand what's happening to my body.

http://www.mayoclinic.org/medical-edge-newspaper-2010/jun-18a.html

Thanks to Dr. Castle at the Mayo Clinic for this posting.

fyi - a couple of small corrections. To my knowledge, there are closer to 300 known cases (a very select club I belong to), and ECD is as yet unclassified. In the future it may be designated a cancer, auto-immune disease (or just as a plain old weird aberration of nature?)

This made my smile just a little wider tonight....

My husband and I were catching up on the events of the day today, when he stopped me (and he rarely interrupts) and said "I just noticed that your face is getting thinner honey, sorry - go on". Those few words elated me more than they ought to have - but really, after having that moonface for almost a year it was music to my ears!

The day started off well enough...

Insurance application denied.

The reason given is that ECD is not on the list of illnesses that Enbrel has been proven to help. I don't know why they had us (and my medical team) jump through hoops to make a case for this drug when all along they knew that they wouldn't approve it.

Maybe this isn't completely rational (and I'll admit that I'm not feeling terribly sensible at this moment) - I'm feeling like a complete stranger has made a decision as to whether my life is worth investing in. They don't care that I have a teenage daughter I haven't finished raising, that I've only been married just a few short years to a man who has brought such happiness and security to my life, and that I'm getting worse as time goes on and desperate for a chance to be in less pain.

I'm a victim of a set of hard and fast rules that don't make allowances for a very rare illness that doesn't have a set treatment plan. No room to bend for something that falls away from the ordinary (not that other serious illnesses are to be taken lightly, just that having more people suffer a particular affliction means a much better opportunity to have outrageously expensive drugs financed).

I need a good cry, and will get ready to look at this with fresh eyes in the morning. But tonight, I need to let myself feel sad.

Ending Up Happy

Still waiting on that insurance letter, but in the meantime...

For those of you who know me personally, you're aware that most of my 45 years have not been easy ones. You'd wonder how so many bad things could happen in one person's life, in fact - a dear friend for years urged me to write a book about my experiences. Childhood traumas that no one should have to endure, leaving home at a very young age to fend for myself, a first marriage that should never have happened (except for the tremendous gift of a daughter whom I treasure), and health issues that never seem to let up.

A frightening path is ahead. If I let myself think about it I could put myself into a right state, but I try hard not to. I'd like to share with you the thoughts that keep me sane.

I am loved by two people who mean everything to me. Both came to me in the later years of my life, and I truly don't know what I'd do without my husband and daughter. Someone upstairs has been looking out for me in sending me the gift of unconditional love.

I believe in karma. I've tried hard to do the right thing, take the high road and trust that I will get the odd break. And I do.

I'm getting much better at letting go, not sweating the small stuff. That's all I'll say on that subject right now, but it's been a huge transition for me!

Finally being able to pursue my passion. As I child I was discouraged from following my heart, but almost ten years ago after yet another health crisis I decided that I could no longer keep plugging away at a career that didn't make my heart sing. My income tanked significantly, and I had numerous setbacks - but I've been able wake up each morning excited about what I'm doing. Even though for the last year and half it's been impossible to photograph as much as I would have wanted to - I can still create images in my head whenever I want. Really, I live and breathe this stuff!

I choose to be happy. Most days. It's really unusual for me not to be happy. I choose to be thankful for the blessings in my life - primarily for those two very special people, and for the chance to follow my heart. I should add that had it not been for giving up my corporate career, I wouldn't have met my husband. It was just meant to be!

Believing that a happy ending is possible. Understanding that a happy ending doesn't have to include a cure for my illness, or living to a ripe old age. I choose to see my happy ending accommodating these challenges.

On a note of following a passion; having been pretty much housebound for almost a year and half, I've come to enjoy downloadable podcasts. Granted, the majority of my subscriptions are photography related and likely of little interest to most of you but I do come across the occasional podcast that resonates with me on another level, and might with you too.

This one is by Sir Ken Robinson. The theme is education reform, but I found myself smiling as I heard him speak about following ones' passion.

http://www.ted.com/talks/sir_ken_robinson_bring_on_the_revolution.html

Do let me know if you pull a gem or two out of this one as well!

In a Holding Pattern

I love getting mail. Yup, even the bills - crazy me. And there's a letter I'm anxiously awaiting, a decision from our insurance company letting us know whether they'll cover a portion of the cost of Enbrel.

It's been a busy time. First I met with a new doctor, a rheumatologist who was the one to give the final approval for me to try this drug. Lots of tests afterwards to make sure that I didn't have additional pre-existing conditions that would take me out of the running for this experiment.

Next - a slew of paperwork for the insurance company. The application itself, with supporting letters from my doctors to explain their reasoning for wanting to try this drug on me. The letters were forwarded to me first, and then in turn sent to the insurance company. Not gonna lie, those letters were hard to digest. It's one thing to be told you have a serious illness, it's another to see it all spelled out on paper. And I do mean all spelled out - the extent of my illness along with the prognosis. Ouch. Plus we had to attach a list of all the drugs prescribed by my pharmacy over the last year - it was disconcerting to see just how long that list was.

Next, discussions with the pharmaceutical company. They have quite an extensive support program for patients taking Enbrel. Unfortunately I'm not eligible for many services because I'll be using Enbrel "off-label" (for a condition it's not intended to treat). But, I can speak to a nurse 24/7 if I have any questions or concerns and that's reassuring. Plus, they sent me a pretty neat welcome kit with a travel cooler bag (Enbrel needs to be kept refrigerated), alcohol wipes and a sharps container for used syringes. And an instructional DVD - but I refuse to watch it until I know for sure that this is a go.

Then today I received a call from a nurse at my hospital who'll be teaching me how to self-inject, and will supervise my first few shots to ensure that I'm doing it right.

And everything now rides on whether my insurance company will help us out. So everyday I wait to see what the mail brings. And I have a strong feeling that this drug will do good things. Fingers crossed, toes crossed. I'd even cross my wonky white blood cells if I knew how; that's the least those little devils could do for all the trouble they're causing me!

Finally!

Thank you dear readers for your words of encouragement, and for hanging in there with me. Today I finally received the phonecall that I've been waiting months for - a decision on which treatment I can try next.

The drug of choice is Enbrel, a biologic medication. Related to Kineret, but according to my medical team it's a safer alternative. Not without risks, but my doctors have thoroughly reviewed the pros and cons of several potential meds to find the one that is the best fit for me.

It's likely to be a month or more before I can begin treatment. Appointments with specialists, discussions with our insurance company (this stuff is REALLY expensive!). Maybe even the chance that the pharmaceutical company can give us a break too. If I ask very nicely?

I'm off to start the paperwork!

Anxious for a decision

I've been quiet for the last few weeks, at least as far as posting goes. Truth is, I'm feeling pretty rotten and it's an effort to sit in front of my computer. And I haven't felt like sharing. I'm thrilled to finally be off the steroids (about 2.5 weeks and counting) but as my doctor predicted, the pericarditis is not only acting up - it's screeching at the top of its lungs at me.

Last night was pretty scary. I'd say that it was the worst chest pain so far in the more than two years since the pericarditis was detected. And not just my chest, the pain wraps itself around to my back, my left arm and up into my neck. I feel like I'm being strangled by a boa constrictor that just won't loosen its grip. And of course it was worst at 2 a.m. - that wicked hour when all is much too quiet, leaving imaginations to run wild. I'd not yet slept though I'd gone to bed three hours earlier. And then the tears started. And I don't cry easily from pain. Or maybe it was sheer desperation that nothing has eased the pain and the difficulty of breathing. All these drugs, and nothing good has come of them.

I have my next hospital visit tomorrow. I spoke with my doctor earlier today to let him know of my discomfort, to see if he wanted to schedule any additional tests. He let me know that the review of meds continues, he's not yet ready to offer the next treatment. His team is leaning towards chemo, and I'm hoping to try one of the new biologic meds (Kineret that I mentioned in my last post). In the meantime I've doubled up on my current meds to try to ease the pain (and I'm very happy to report that I'm feeling better this evening than I did last night).

This waiting is nearly intolerable. I'm on-line reading up on the results of drug trials of both drugs being reviewed, asking myself which side effects I'm willing to bear - on one hand I'm grateful that the internet can offer us so much information, but then again maybe it would be better if I had less to digest at the moment. And I wait.

Too much excitement for one week

To some degree I measure my health status by the frequency of my visits to the emergency room. I made it to over 10 months, but last Friday broke my streak.

It's been a tough time with pain and breathing problems, but Friday night was different. I'd had a very quiet day, nothing strenuous (my usual trigger). Just hanging out on the couch watching Coronation Street (the only show that I hate to miss! Blame my first roommate Laura for getting me hooked as a teenager).

Suddenly it felt like my chest was freezing up. No air in or out for a few seconds. It passed. This has happened before, alarming for a few seconds but then it's over. But this time it happened again a few minutes later. Then again, with less time in between and more forcefully. Until I could hardly stand up. When I have an arrythmia it often helps if I make myself cough, but this wasn't working. Nothing seemed to help, and it felt like I was suffocating. Is this what drowning would feel like?

My husband called 911, and a fabulous group of paramedics and EMTs showed up at our front door within a few minutes. Thankfully after oxygen, several doses of nitroglycerin and a few hours in the ER, my breathing was back to usual. Still a bit difficult but nowhere near as frightening as it had been hours earlier.

This episode scared the heck out of us. I don't want to think negatively, but I suspect this might not be the only time this will happen. Having Erdheim Chester Disease firmly entrenched in my heart is going to cause problems. Lately it's been a wild ride of low to very high heart rates, low to high blood pressure - my heart just can't seem to decide what to do. Can't blame it for having a hissy fit last Friday I suppose.

I talked to my specialist today, he told me that I probably won't be prescribed the drug I'd been hoping to try (Kineret), that the team would rather I try a chemotherapy drug that has been effective for slowing down the progression of lung cancer (I don't have lung cancer, but then again ECD treatment is hit and miss, maybe it might help to reduce my symptoms).

I'm just not happy about that prospect. I'm digging up all the info I can find on both drugs so that I can make an informed decision about the chemo option. But I REALLY don't want chemo. Really, really don't. Shall I say it again?

Wish me luck over the coming weeks, my doc today instructed me to go cold turkey on the prednisone. 3 mg to nothing. One one hand, I'm thrilled at the prospect of getting off of this stuff, more side effects than I can count on all fingers and toes. On the other hand, dropping one mg at a time had been difficult, the withdrawal process has been rough. But looking at the big picture, I think I'd rather take a big hit of withdrawal symptoms for a few weeks rather than keep dragging this out over the next few months. Plus vanity plays into this a bit - I'm tired of carrying this extra weight - and the last thing I want to do is buy warm weather clothing for this larger size! So here I go.... three, two, one - deflate!

Time Flies. Or Not.

Today is the first anniversary of getting my diagnosis of Erdheim Chester Disease. How clearly I remember our concern of being called back to the orthopaedic oncologist's office after having had an initial all-clear on cancer just a week or so earlier. The relief of the previous week turned to fear - who gets called back after being told that a tumour isn't cancerous? Had the pathologist made an error?

I'm really not sure where my mind is at today. Today is one of my tough days, and even lifting my arm above my head seems like a monumental effort. What will the next year bring? Will I see any improvement - will I have access to the drug that just might improve my quality of life?

The waiting is doing my head in right now.

Patience my dear, patience...

Last post I wrote about my hope to be able to try a new drug. An experimental one. I visited the hospital yesterday for one of my very frequent check-ups, and to discuss the possibility of giving Kineret a try. There's more to this process that I had thought!

In theory, my doctors believe that Kineret might be of help with not only reducing my bone pain, but might also slow down the progression of my illness. However, there are some serious risks with this drug that need to be further investigated. Kineret (and bear with me here, this is all jumble to me!) is an "interleukin-1 receptor antagonist" - part of the "biologic response modifier" family of treatments. This drug increases the risk of infection, and also carries a small risk of causing lymphoma among other side effects.

This drug is intended to treat moderate to severe rheumatoid arthritis - and that's where the complication comes in. Researchers don't know what the implications are for Erdheim-Chester Disease; white blood cells already don't know how to behave properly so nobody really knows how they'd react to this treatment. Just a handful of ECD patients have tried this, and only for a short time - far too soon to determine any long term implications.

My medical team will be doing further research, including consulting with an oncologist at a cancer centre in the US who has been doing advanced research on interleukin treatments. If that's a go - then seek gov't approval to use this drug for an illness it was otherwise intended for. Then, funding - this isn't cheap!

Am I still on board? Yes. Do I understand that this could go either way? Yes. I know that there's no cure, but I'm desperate for a better quality of life for at least awhile. I need to hope for something good, because some nights when I'm feeling quite awful it can be hard to envision a better day tomorrow.

Yesterday was a pretty good day. So is today. I'm feeling greedy, I want more days like these.

More Druggies - One I'm Actually Hoping For!

I find that I'm fighting an overall sense of dismay over my illness. How dare it not respond to all the meds I've taken so far? What blinkin' nerve!

A few meds that I know I'll be adding soon:

- a biphosphonate to help with the bone density loss. I'm hoping this next one goes better than the Fosamax last summer. This time we'll be trying the intravenous route for delivery.

- a beta blocker. My heart rate has been out of control of late, and yesterday's data download from my pacemaker confirmed it. Over 8,000 episodes of a heart rate over 140 beats per minute over the last six months, most have no correlation to exercise, stressful situations etc. A good number in the middle of the night - I know my dreams are a bit wacky but that's a bit much! A bit ironic since I had the pacemaker implanted almost six years ago to deal with bradycardia - a very slow heart rate. That's still happening too, dropping suddenly to below 50 bpm on average about 15-20 times a day. My heart is the queen of cardiac roller coaster rides! It's time to exit that amusement park.

- and here's the drug I'm excited about. Kineret. Keeping everything crossed that I can cross that I'm given the chance to try it. Several other ECD patients have reported that Kineret has greatly reduced their bone pain (which has been getting significantly worse over the last months). An exciting prospect for this alone, but what really got me hyped was reading about a study whereby three patients who had been dealing with pericarditis has almost immediate resolution after the first dose! After having tried pretty much the same course of treatment that I've tried! (colchicine, steroids, other anti-inflammatory drugs). I immediately sent off the article to my doctor, who is now discussing the drug with his team. I'm on pins and needles waiting for my appt next Tuesday to find out if we can go down this path. It would need to be approved for use (it's meant for moderate to severe rheumatoid arthritis), and if approved then we'd need to find funding (I've read that the cost is between $15-20K per year in Canada).

Kineret is injected daily, both by husband and daughter are on board to give me an injection when it's time to rotate to a location that I can't reach. I'd be able to do the other spots myself. I'm getting ahead of myself though. It's hard not to, I'm feeling rather beaten down by the pain, fatigue and general unwellness of late. Just when I'm feeling better for a few days the pericarditis acts up again, letting me know who's boss. Time for a mutiny, don't you think?

Catching Up...And Down Again...

A few posts ago I mentioned that I'd had a bone density test, and I'm not at all pleased about the result. The scan indicated that I'd had significant bone density loss since my last test - thank you again steroids! You're the gift that just keeps on giving.

I dropped a few days ago to 4 mg and it's pretty hard to stay awake. I was supposed to go into Toronto to have a check-up on my pacemaker this morning, but there was no way that I would have been capable of manoevering myself through the train and subway system today - just that dopey. That's been rescheduled a few weeks out when I hope to be far more coherent again. I'm sure I'll be ready for another nap before this post is finished!

I'm getting frustrated with my lack of progression on a number of work and personal projects, I don't understand why I can't just will myself to stay awake, will myself to feel better, will myself to feel less pain. I'm working very hard on my "mind over matter" approach to my illness but there are times I just have to admit that wanting to feel better isn't enough.

As long as it's not the "C" word

Not quite as tired as I have been the last week, and getting quite a bit of work done today (not the "running after small children taking their picture" kind of work that I would prefer to be doing, it's image retouching on my computer but work nonetheless). Hurray!

I had an appt to have a bone density scan done at a local hospital a few weeks ago, and a comment that the technician offhandedly offered continues to bother me a bit. I was having the scan done to see if the steroids have caused bone density loss, and she asked if my medical status had changed since my last scan two years ago. I briefly updated her on Erdheim-Chester Disease, and mentioned that she might see a bone lesion on my femur on the scan - and not to be alarmed, I'm aware of it.

The technician asked if it was cancer. I said no, ECD isn't officially classified as cancer - it's usually described as an auto-immune illness yet often treated by oncologists. She told me that I should consider myself very lucky that it wasn't cancer, and thank goodness I could look forward to a long life unlike many cancer patients. Hello? Were you listening to me?

I've gotten this reaction a few times. As long as it isn't cancer. How fortunate for you!

Frankly, a fair number of cancers have a much better prognosis than ECD. And most have a course of treatment to follow. ECD doesn't have this yet. Some positive development with some drugs, but it appears to be mostly trials and investigative attempts at this time. Nothing close to a cure (and I'll be happy to correct this should one of the many researchers working on this illness let me know about any new developments!) :-)

When my orthopaedic oncologist first saw my scans last year, his guess was that the lesion was 1st stage bone cancer. He felt confident that if this were the case it could be successfully and quickly treated. I can't tell you how often in the last year I've wished that this had been the diagnosis.

Lethargic...

Fatigued.
Spent.
Dog Tired.
Exhausted.
Weary.
Sapped.
Weakened.
Beat.
Done for.
Petered out.
Drained.
Languid.
Conked out.
Shot.
Depleted.

And much too tired to alphabetize this list. You know I wanted to.

Down to 5 mg of Prednisone, and getting a whole lotta nuthin' done these days.

Awesomeness...Is that a Word?

With being housebound so often this last year, I've been spending a lot more time on the Internet. There are some amazing websites and blogs out there - I'm in full-out learning mode these days! Just recently I discovered a blog that always put a smile on my face -"1000 Awesome Things".

The author of the blog has been doing a countdown from 1000 - 1, and is today sitting at Awesome Thing #589. Most people wouldn't be at all surprised that my favourites are food related, but there are so many posts that bring back lovely memories or give me a smile when I realize that others also find delight in some rather quirky things that can happen in daily life!

If you have a few minutes to spare, check it out...but don't be surprised to find that an hour has passed when you finally take a break from reading all that Awesomeness!

Which ones are your favourites?

Click here to visit the blog.

And What Is Next?

Thank you faithful readers for spurring me on to write another entry, I guess I let things slide for awhile! No excuses, just took a little break over the holidays. Speaking of which they were very quiet around here, just what we needed after an eventful year.

We got back a few hours ago from a visit to the hospital. Nothing much to report, I'm continuing on the prednisone taper (my third attempt at dropping from 8 to 7 mg actually went quite well over the last few weeks!) and I'm planning another drop tomorrow to 6 mg. Wish me luck, we're at that crucial stage over the next while to see if my adrenal glands can be cajoled back into action after being suppressed by the steroids.

My doc said the next visit will include a serious discussion about what comes next in my treatment plan. Likely medication candidates will be Gleevac (chemo) or Interferon Alpha (not chemo, but somewhat similar impact on the system/side effects). I don't remember if I'd mentioned it in previous posts but I'm struggling with this decision. Whether to take any of the "hard" drugs at all. It's been a difficult time trying a myriad of treatments over the last nine months, and I'm "side-effected out" - it's just felt like too much at times and I need a break. We anticipate another 3 - 4 months of steroid tapering - which should end just about the time that the weather starts to get nice again. More than anything I'd like to feel the sun on my face, take long walks with my family (long being a relative term, I'll start with treks around the block - steroid therapy has kicked my behind and I need to build up some strength again), and photograph again! I missed last spring/summer entirely (but apparently so did most of Ontario, it was the summer that wasn't) with staying indoors not feeling great and I feel that I have lots of catching up to do.

Last post I wrote about wondering what was next, what could I look forward to? Still working on that, but it's become clear to me -first priority is to enjoy some time with my family (hopefully we can take that New York City trip that we had to pass on last August). Second is one (or several) photography projects. A few ideas are brewing, I'll share once I've decided on what I'd like to do. I miss holding my camera in my hands and can't wait to get out there again.

I wish you all a happy and healthy 2010!