You have one of my spoons...

My new friend--the one with the extremely rare form of Pulmonary Hypertension--introduced me to The Spoon Theory. It is truly a good explanation of what it's like to be limited by a disease and I hope all of my friends will read it. There are those of us who have a short supply of spoons each day and I am learning to pace myself, prioritize, and use my spoons wisely.

Slowing down. Being left out. Not getting stuff done. It is hard. All those little things I used to do each day that were so easy, are monumental tasks for me now. I cannot get enough oxygen. My heart works overtime. I'm on medications. They have side effects. By late morning my body is exhausted because inside... inside it has run a marathon. It's too much for my body to handle. It's breaking down. It's burning up. I cannot change the oil fast enough.

I cannot just do. I cannot just get up and walk and work and create and play. I have to plan ahead for everything. I have to account for every move, how far I need to walk, if there are stairs, and if I can take breaks. I have to plan my outings accordingly. I have to live my life around medicine mixing, ice pack changes (the IV med has to stay cold), and whether or not I will have the energy to carry around an oxygen tank (not to mention the amount of time each tank limits me to). I know I also have to be open to the unexpected and that it comes with consequences. Those are the days spoons are taken from me and I am left with nothing but empty hands, exhaustion, and tears. All I can do is wait and see if morning will bring me a few new spoons.

Today? There are so few spoons today. I must be careful...

Writing out the waves...

And so it continues... this new journey--a journey I never intended to embark on. But as with all journeys, I've met some wonderful and delightful people along the way. Interestingly enough, I would have never met these new friends had it not been for my illness... this new journey.

This week I met a lovely woman who was diagnosed last year with a similar condition - an extremely rare form of Pulmonary Hypertension. Funny thing is, we have a lot more in common than having a rare disease that has scarred our lungs. We are the same age. We have a deep appreciation for nature, gardening, the simple things in life. We are happily married and we have young children. We also share the uncertainty of our future. We have central lines--tubing running through our chests and straight to our hearts. We take some of the same medicines and experience similar symptoms. We have had similar hospital experiences. We worry about our children and their future and wonder how much more time we will have to hold onto their little hands. We watch in awe as our husbands play caregiver roles and help mix our medication. We appreciate them, and our many friends and family who have become our support teams and our protectors.

She has a blog. It's relatively private but she shared it with me. In the last two days I've read through all her posts. I cried, I laughed, I wanted to give her a hug.

There's something to this writing. I explained several posts ago that I was struggling with writing this blog. But, something was telling me I needed to start it and keep family and friends abreast of the latest happening. Not only that, I felt others may be looking for me. After all, I was scouring the internet to find others going through what I was going through. I wanted to know how they were handling things and I wanted to know what they had been through. Do you know what I found? I found that I'm not the only one who feels this way. I'm not the only one fighting a battle. I'm not the only one on a new scary journey that I didn't want to take. I'm not the only one trying to bail water out of this leaky boat...

So, back to this writing. There are actual studies that have been done on patients who write. And it appears that writing about stressful experiences can actually help reduce symptoms in some patients. Some studies reveal that it is expressive writing, rather than general journaling, that demonstrates some beneficial effects in physical and psychological health. Are you thinking what I'm thinking? Indeed, I should be writing more blog posts...

Live it up...

This weekend I had a visit from a childhood friend. It helped. I woke up more confident and focused this morning. She told me I have no choice but to "live it up" from here on out. I promised her I will do so... to the best of my ability.

I know what the statistics say. I know what the odds are. But, I must focus on being on the side with the best outcome and hope the outcome gets better the longer I can hold out. There are always new advancements in medicine and technology and better medications on the horizon. The intravenous epoprostenol I am currently trying wasn't even approved by the FDA until 1995. Since then it has given many patients an opportunity to live longer and for some, has provided the boost needed between diagnosis and a potential lung transplant. And, even though lung transplantation is an extremely complicated surgery with high mortality rates, there are better post-transplant drugs available now.

The weirdest thing is knowing I have a time limit. Sure, anyone can die at any time. Nothing is guaranteed.  But, to know I probably won't make it to see certain milestones is difficult to accept. This morning I read Living with death in the Guardian News, an article about four people sharing their thoughts on living with terminal illness. Holly Webber, who has cancer and was 25 at the time of the article said, "Sometimes I feel like I'm on another planet looking in on this one. I can't relate to people stressing about work.... People are so wound up, but it's such a waste of time and energy. Chill out! I hope that by reading this, someone out there will take a second to think, "I'm glad that's not me. Maybe I should worry less about the things that don't really matter.""

Like Holly, I am finding it hard to relate to the people around me. When I cruise through Facebook I find so many of my friends and family complaining about stuff that is so trivial. Or, things that seem like a crisis are really insignificant in the context of things. For the most part the wrinkles will fall out with a little time, and their life will go on just the same. People complain about their kids or their spouse... but what happens when they no longer have them in their life? People complain about things needing repaired, but what happens when they no longer have it within their capacity to repair things? People complain about traffic, but what happens when they are no longer able to drive or even manage public transportation on their own? People obsess about looks, clothes, and hair; but what happens when their body is ravaged by medication side effects that makes their hair fall out, their face flushed, causes swelling, and/or rashes on their body? People complain when they have a headache or get a common cold, but those too will pass. And of all things, people complain about getting older. What? Getting older is a privilege denied to some. To grow old... they should be thankful.

There'll be days...

Today was one of those days. It hit me. I woke with a headache and one of those migraine aura things. My oxygen was desaturating pretty quickly with the slightest activity so I felt extremely unproductive with my already limited ability to function. I received more paperwork to add to my never ending pile of medical madness, including a new, full schedule for the Cleveland Clinic for the end of March... tests and appointments that will require a stay-over in Cleveland. I wanted to cry.

Of all things, I became obsessed with something so trivial. I wanted corn flakes. But not any ol' corn flakes. I wanted Erewhon organic gluten-free corn flakes. And, since many conventional grocery stores do not carry this particular brand, I assume it would have to be hunted down... perhaps from as far as the co-op, which is a considerable distance from me. And, even though friends have offered to pick up groceries for me, I felt it would be an unreasonable request to ask someone to track down this cereal, possibly available at some specific store, on today--a blustery, cold day. Nope. I just couldn't bring myself to ask.

I told myself it was just too silly to be so upset over these special organic corn flakes. But then I realized it's not about the cereal. It is my pain, my lack of control, my lack of freedom. I'm in mourning. I'm mourning the ability to get up the stairs without feeling light-headed. I'm mourning the loss of several seasons wasted visiting doctor after doctor trying to find out why I was wilting along with the summer flowers. I'm mourning the missed activities with my husband and daughter. I'm mourning what could have been...

Titrating up...

Wednesday was my follow-up at the Cleveland Clinic. It was a long day but fairly simple - a walking test, in which they measure oxygen levels for six minutes of walking; blood-work; and a visit with the doctor. The good news is that I appear no worse. So, we will continue the Flolan therapy as planned and hope for the best.

Thursday, as instructed by my doctor, I raised the Flolan at home. I took Tylenol beforehand, anticipating some pain. I got the usual flushing, headache, and some pain in my calves. But, overall, it was not as bad as when I was in the hospital. That may be because they were upping it about one time each day, where here at home they may up it once a week. The jaw pain while eating (at the beginning of meals) also increased.

Being tethered to an IV pump is awkward. I'm still dependent on the oxygen, too. So, wherever I go I feel like I take up a lot of space, encumbered with tubes and other breathing paraphernalia. I must look a bit like Doctor Octopus and sound like Darth Vader. So, needless to say, between that and my lack of energy, I don't get out much.

My husband and I mix my medication each evening. We timed it the other day and found it takes a little over 32 minutes to mix and change the cartridge on the pump. And, that was on a good day. Once a week I have to change the dressing and one of the connectors that go on the Hickman. And, three times a week I have to change the extension tubing. To shower I have to cover the Hickman site and hang the pump from the towel rod.

Again I cannot express how thankful I am for all the help from family and friends. I hope everyone remembers that this is going to be a long process so please do not hesitate to keep reminding us that you can help.

And for now, I will leave you with a link to an article about Carson Smith, a wonderful lady who is also living with PVOD. The article is six years old and I am happy to report that Carson is still doing well and has been stable on medications.

Flolan

In case anyone wants to see what I have to do every day concerning the medicine visit Flolan Preparation or, How to be More like Rambo at Leela's The Heart-Lung Thing Blog. She does a wonderful job explaining the steps involved in preparing the medicine, complete with photos.

Home sweet home...

Tuesday night I was released from the hospital. I was away from home for a total of ten days and I was horribly home-sick. My mother-in-law was so wonderful, staying several nights camped out in the smallest of chairs and eating the leftover food from my meal trays. My husband was there for a couple nights and was there for the Hickman placement. His voice was one of the first voices I heard when I was coming out of surgery. When I was at the step down floor he made several origami cranes for me and placed them along the windowsill. On the day I was planning to leave, the Accredo nurse, who was training me on the IV pump, turned all the cranes to face home. Through the rest of the day, all the cranes and I looked towards home, anxiously waiting.

Since arriving home I've had daily visits from an Accredo nurse. Each day she has helped us go through the steps of mixing the medication, changing the cartridge on the pump, and starting the meter on the pump. She demonstrated changing the dressing on the Hickman yesterday. I thought it was disgusting seeing the hole in my chest with the tube sewn in. I suppose I will get used to it, but it is so weird. The nurse is extremely sweet and I like her a lot, so when she starts leaving us on our own, like tomorrow, I will miss her visits.

Every day I've been home I've had a gigantic appetite and keep eating. In the hospital I got very little protein. The majority of the food they offered was starches and sugars. What little hunk of meat showed up on my plate, I devoured. The vegetables were canned variety and much of the fruit offered was swimming in artificially flavored syrup. Dessert options included packaged cups of stuff--sherbet, pudding, frozen yogurt--all with artificial or substitute sweeteners, artificial flavors and artificial colors, emulsifiers like headache-causing carrageenan, and other stuff people should never be eating anyway.

Today I started getting my INR monitored, so that I don't take too little or too much blood thinning medication. Being on a thinner means being more careful as the blood will not clot as quickly as normal. I also have to watch certain foods that can alter the function of the medicine or else my INR reading could be thrown off.

I go to the Cleveland Clinic next week for a follow up and more blood-work. I am pretty sure they replaced all of my blood with saline while I was there. So, I assume they want to make sure they drain me again before my body has time to recover.