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.

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!