Seaglass79's Blog

If you’re just tuning into this blog, you should know that this post is long, wordy, detailed, yet oh-so-informative! For a less scientific view of PH, please scroll down and check out the last three paragraphs and then move on to the other posts.

Hello, my name is Colleen and I take Viagra.

To survive Pulmonary Hypertension (PH) you have to take the aforementioned shitload of drugs. (see first post in this blog)

Prior to 1995 there was nothing to be done for a PH patient except tell them to get their affairs in order and possibly get on a list for a double heart and lung transplant (which in and of itself is a kind of disease… with an average 5 year survival rate post-transplant.) Starting with the advent of Flolan in 1995, things slowly began to change.

I’ve sat down to write this installment several times, and I get stuck when I get here. PH is complicated. Treatments, by default, are also complicated. Could I explain it so the average person can understand it? Yeah… if they would stick through reading the pages it would take to do.

I will try not to do that, but bear with me while I try to condense! I think it is important that people understand the treatments and how they fall short. We’re lucky to have many to choose from, but none offer a cure and all come with a high financial and physical cost.

First – the prostanoids (a substance PH patients don’t make enough of, which, according to phassociation.org, is used to “help many PH patients by dilating blood vessels, reducing clotting, slowing down the growth of smooth muscle cells, and improving cardiac output”):

In 1995 we had the advent of Flolan. Flolan is administered via central line in the chest. A tube is hooked to a pump the patient carries and the medicine must be kept on ice. There is an 8 hour version that doesn’t need ice. Side effects are very severe, including: pain, stomach upset, and risk of infection from the line. It can take up to an hour a day just to keep the site clean and dry for regular routine like showering. And at approximately $100,000 a year, it doesn’t come cheap. However, it saves lives and those that I know who are on it say all the risks are worth it.

Then there is Remodulin. This can also be administered via central line, but a pic line in the arm and an under the skin catheter called Sub-Q is also frequently used. The Sub-Q method is extremely painful for many people and they usually have to be on one or more severe pain killers just to tolerate it. Remodulin has a lot of the same fun side effects as Flolan and costs about $75,000 a year.

This past September, Remodulin was released in an inhaled form called Tyvaso -  initial reactions from my friends in the community have been very positive! You take it three times a day and treatment time is only  a few minutes each cycle. I’m excited about this one.

A pill form of Remodulin is also well in the works, with clinical trials going on now.

Next, the other inhaled PH med, Ventavis. Ventavis must be inhaled 6 – 9 times a day, with each treatment lasting up to 10 minutes. New advances with Ventavis are decreasing treatment times. Side effects are mostly things like coughing and headache, but it is generally well tolerated. Patient compliance taking Ventavis is something of an issue because of the pure time involved. I personally would have a pretty hard time finding an hour’s worth of 10 minutes segments in my day and keeping my preschooler quiet while I breath into a machine. (I don’t know the financial cost)

The next category of drugs is the Endothelin Antagonists (PH patients make too much Endothelin, causing the vessels in the lungs to constrict and narrow).

These are Tracleer and Letairis. Both are in pill form and both cost about $50,000 a year. Tracleer is a twice a day pill and Letairis once a day. Side effects usually aren’t too bad – mostly flushing and stuffy noses, headache, and some patients experience swelling in their extremities. However, both run a high risk of liver faliure and so a monthly Liver Function Test (LFT) must be done, as well as a monthly pregnancy test because the medications are highly harmful to a fetus.

(As a side note – pregnancy isn’t really an option for the PH patient anyway. Maternal mortality rates are around 50%.)

Finally, we get to the meds we PHers are famous for – the Phosphodisterase (PDE-5) Inhibitors, known to the common man as Viagra! (and others). These help relax the blood vessels and arteries and improve blood flow. If you know anything about Viagra, then you can see how this makes sense. Interestingly, intense athletes have also begun to use Viagra as a performance enhancer because it helps them breathe more efficiently. The two common forms are: Revatio (Viagra’s cousin) and as of this fall, Adcirca (Cialis’ cousin).

So, yes, I take Viagra. Lots and lots and lots of Viagra. Side effects are generally mild and well tolerated and include things such as: headaches, flushing (I turn quite rosy sometimes!), nasal congestion, and really interesting dinnertime conversations when someone notices you popping a pill. You have to take it three times a day and it costs around $10,000 a year. Adcirica is only once a day and costs a little less, but so far I haven’t been thrilled with the side effects I am hearing about (mostly severe headache).

Let me rant for a moment. For the sake of example: Drugstore.com charges $149 for 10 50 mg pills of Viagra (a total of 500 mg). Revatio (the same exact stuff as Viagra, but packaged and marketed for PH) spread out in the 20 mg doses would end up costing about $231 for the same 500 mg. Why is it that the cost of my drug is $82 more dollars to help me stay alive for eight days (taking the prescribed 60 mg a day) while the old guy who wants to just get it on 10 times pays so much less????

I digress…

So, what you may have noticed, is that there are three primary categories of medication. Each targets a known pathway of PH. Many American patients are on more than one therapy (which is not an option in some countries), with a brave few on one from each category. There aren’t many studies about the efficacy of more than one therapy, but in theory it makes perfect sense since each category of drug has a different job in the body. However, what we don’t know is the potential impact of so many high-caliber medications in the system at once. As a friend pointed out though, we do know the impact of no drugs in the system (death) and even with two the outcome is still sub-standard in both life expectancy and life quality.

Survival rates of PH without treatment is at 2.8 years. With treatment, well, they just don’t know. Five years seems about average, seven to eight is considered long-term

One PH medication that doesn’t get a great deal of attention is Calcium Channel Blockers (CCB). CCBs work really well on only about 6 – 10% of the PH population. Side effects are again fairly mild and the medication is often very effective for a long time for the lucky few who respond to it.

Many patients are also on Digoxin for heart support and Coumadin because Phers blood tends to clot more easily – raising the risk of stroke, etc. For those of you not in the know, Coumadin also has a common name – rat poison. Yup, same stuff. I haven’t been in a hurry to get on it, although I do understand the benefits and risks of not. I’m taking my chances.

Finally, many people are on oxygen therapy. Some wear it 24/7, while others (like me) need it only at night. Phers tend to have oxygen levels desaturate in their sleep more than most.

For those of you keeping count at home, I take: Letairis, Revatio, and Digoxin. Tyvaso seems to perhaps be on my horizon. I wear oxygen at night. I am very grateful my insurance company covers all but $42/month of the $7,000/month it is costing to keep me alive.

What I really want to impress is that medications are incredibly costly and many have debilitating or at least life-changing side effects. We’ve made phenomenal gains since 1995, but are nowhere near close enough to where we need to be.

We need medications that don’t make us as disabled (or more disabled) than the disease itself. We need ones that don’t put us in the poor house or wreck havoc on other organs.

Most of all, we need a cure.

(Post sources: www.phassociation.org, www.phcentral.org)

So today is PH Blogging Day – a day set aside by the Pulmonary Hypertension Association to encourage as many people as possible to take to the highways and byways of cyberland and spread the word about PH.

If you’ve followed this blog through its short life, you already know what it feels like to have PH. You know how it is diagnosed and what it does. You know one way to not just survive, but to thrive in spite of PH.

Do you e-mail? Do you have a facebook or myspace page? Twitter? If you do, I would love it if you could take a minute to tell all of your friends and family about Pulmonary Hypertension.

Tell them my story, if you want. You can also hand out my contact information and I can share more if they’re curious.

Send them a link to www.phassociation.org.

Or, send them a link to PHAs Blogspot: http://phawareness.blogspot.com/

Or, send them here to this blog: http://seaglass79.wordpress.com/

Whatever you do, spread the word. With awareness comes the saving of lives. Hopefully, funding and research dollars will also continue to be allocated our way so that the cure that has to be just on the horizon shows up.

Thank you for your support.

I knew I was in trouble the day a cardiologist told me the best case scenario he was hoping for was to find a hole in my heart that we could repair. He told me there was a worst case scenario too… but we weren’t going to talk about it unless we got there. If the best thing is a hole in the heart, what’s the worst? Well, as it turned out, Pulmonary Hypertension of course. We got there.

It is difficult to describe to someone who hasn’t “Been There” what it is like to lay in a hospital bed, have a doctor look you in the eye, and rattle off the name of some life-altering and life-threatening disease you have never heard of and apply it to you.

Where do you go from there? I imagine people have a huge range of responses. I only know my own. I freaked. Looking back on the months after diagnosis, it was pretty much me in a dark cave. Scouring the internet for any information I could find (a stupid idea – most of it is woefully outdated and false and will scare you senseless), finding sources that finally explained something updated and accurate, pacing the house late into the middle of the night just trying to wrap my mind around the whole damn thing. Crying. A lot.

During the day you probably wouldn’t have seen much of this from me. Fake it until you make it. I was still working, I still had a child to care for, a family and friends to respond to. People who say “As long as you have your health you have everything” haven’t actually lived through the blow of loosing your health and coming through on the other side still intact. I am very grateful there is more to life than just health, or I’d be screwed.

I have a wonderful support system, but for a long time none of it helped. I just had to deal on my terms in my own way. But, it really comes to a point where you must make a choice for yourself. Either keep living your life in a state of shock and grief, or pick yourself up, brush off, and start living again. There is no in between in my case. It was live, truly live, or face death head on feeling like I was… well, dieing. That doesn’t seem like such a good option, certainly not the mindset that would keep me strong for the fight of my life.

I’ve tried to explain to people how once you have a disease like this you never ever for a single second get to forget it. They often look confused – if it such a part of your everyday don’t you just get used to it? Not really. I remember every time I pop a pill – five for the PH and a huge handful of vitamins and supplements for a bunch of other reasons – all PH related. I remember every time I look in the back of the car at a load of groceries and try to gauge how heavy each bag is and which I should carry together so I don’t arrive in the house breathless and dizzy. I remember every night as I shove a tube of oxygen up my nose, and many times as I fall asleep and feel my heart palpitate with each breath. You never get to forget.

But, you can’t stop living either. And you have to find ways to do that. Some days you need more ways to focus on living over the disease than others. So, I’ve developed an arsenal.

Some are serious – like totally giving this whole thing up to God. I believe I will be healed. I don’t know how. I don’t know when. But I WILL be. It is actually that simple. In the meantime though, I still have to get by. I have to live the human side while I wait for the divine. This fight could get a hell of a lot harder before it gets better.

As much as I believe in the power of prayer, I also believe in the power of positive thinking. If I believe I will be healed, if I truly believe this is going to end up okay, my attitude is going to be better. And if the mind/body connection is half as strong as I suspect it to be, just keeping a positive mindset will keep me stronger.

The support system of friends and family I have gets me really far. I have lots of phriends in the PH Community who get the struggle first hand, who offer advice and encouragement on treatments, and sometimes a “Yeah, I know that sucks” is enough too. I have healthy friends and family who are amazing. Always checking in, lending a hand when needed. I don’t know how many people are praying for me – but I do know that over 450 people have read this blog and that’s a lot of positive vibes and well wishes. I believe in the power of that too!

Then there’s the more light hearted side. I only go to appointments wearing cute shoes. Cute shoes make me happy. If I don’t have to have an exercise test that day, you can bet they’re going to be cute and hot and with a minimum of three inch heels. If I do have an exercise test, even my sneakers are ones that make me smile.

I only take my meds by washing them down with water from a wine glass. I don’t know why – but nice stemware makes me happy too. So if I use it when I’m doing something I don’t want to do – like take $7,000 a month in drugs – it makes it a little easier.

Lately I’ve been moving to a more altruistic lifeline – reaching out and helping other patients. I am thrilled to have been asked to sit on a Patient Advisory Board for the Pulmonary Hypertension Association (PHA). We’re going to work together to reach other young people who have so much of their lives ahead of them as they also navigate PH. I’ve also joined “Generation Hope” – another PHA organization that consists of a private e-mail group where we can support other young patients. How great is the name of that group? I hope to do some writing for the quarterly newsletter that PHA puts out nationally. I want to turn all this PH knowledge I have bumping around in my head into something that helps others.

Oh… and I’m getting a tattoo… something to remind me of my battle, but also of the victory that will come. Stay tuned on that one! I just put it in writing, on the Internet no less, so I’d better follow through!

Okay boys and girls, all you faithful blog readers, get ready for a lesson in PH…

First of all, with the heart there is left side and right side involvement. This is about right side involvement – the kind I am most familiar with.

PH is known as “the other high blood pressure” – a totally misleading term. It is indeed high blood pressure , but treating it is no where as simple as popping a pill, watching your sodium, and loosing some weight. The blood pressure problem is within the pulmonary artery of the lungs. Simply stated, the heart has to work really hard against this extra pressure. It tends to enlarge (as mine has) and eventually it can lose its ability to pump (which I’m hoping mine won’t!).

PH knows no age, race, or gender. ANYONE can get it. However, it primarily strikes women in their childbearing years. It is not unusual for symptoms to emerge with or just after pregnancy, as was the case with me. Pregnancy doesn’t cause it, but if you’re going to have it anyway, something about pregnancy seems to kickstart it a bit.

Symptoms of PH include quite a list. I have experienced the following common symptoms:

Chest pain, shortness of breath on “easy” exertion like climbing a flight of stairs or carrying groceries, low energy/fatigue, dizziness and fainting, and heart palpitations.

In addition, a patient might have: swollen ankles and legs and bluish lips and skin.

This list, unfortunately, looks like a whole lot of other symptoms lists for other diseases. That is, in part, why so many people go through many doctor visits, misdiagnosis, and even years of suffering before a correct diagnosis is given. At that point, for many, the disease is at an advanced state and treatment has to be swift and intense. (More on that later)

There are some preliminary screenings for PH. It might be suspected from a chest x-ray showing the enlarged heart and/or an abnormal EKG (that’s me again!). An echo cardiogram gives estimates of pressures and you can measure cardiac output and heart size as well… but diagnosis is best done via a Right Heart Catheter (RHC).  Oh yeah, those are as fun as they sound. A thin tube is inserted into the body via the groin or the neck and then they move it around and take a look at your pulmonary system. The best part – the patient is awake for the whole thing. I have seen the inside of my heart up on a screen while laying on a gurney in a freeeeeeezzzzziinnnnnggg room, being worked on by a tech who resembled Harry Connick Jr. (That wasn’t so bad!). Valium is a beautiful thing in these situations, but not always offered. After two such procedures, one drugged and one not, I assure you, offered or not, it will be politely requested demanded for the next round!

Once there is a definitive diagnosis, the fun really begins. First, you have to just wrap your head around the fact that you have an “incurrable and often fatal disease”. I think that is another post for another time…

You have to look for and/or rule out all possible causes, such as: autoimmune disorders (such as Scleroderma/CREST or Lupus – two common culpirts), an Atrial Septal Defect (an ASD, a birth defect of the heart – a “hole”), and blood clots in the lungs (Pulmonary Embolism). It can also be caused by other underlying health issues like liver disease and drug use – specifically diet drugs like Phen-phen and “recreational drugs” like cocain. (Yes, they really call them recreational – it took me a while to figure out what I was being asked when they screened me. And the answer was “NO!”).

These tests are not a lot of fun. You give up vials and vials of blood. Screening for blood clots in the lungs involves a procedure where you are actually radioactive for a while after (a fact not mentioned to me before the test was just about in progress!) This also means that while driving home, you may be pulled over for suspected terrorist activity since you are emmitting radioactive waves for a while. That didn’t happen to me, but I kind of wish it had. It would have been fun to explain!

“Oh, no, officer, I’m not carrying illegal chemical explosives… that’s me your nifty little machine caught.  I’m radioactive, you see…”

Back to the topic at hand, I have NONE of the underlying causes. This makes me a lucky one or two in a million who have what is often referred to as Primary or Idiopathic Pulmonary Arterial Hypertension. Yup, one in a million. How do you like those odds? I knew I should have played the lottery more…

After diagnosis, comes the treatment regime, but if you’ve stuck with me this far, you’ve probably read enough for one day! Thank you.

Treatments will come some other time in the near future… next post will be a little bit more about what it is really like living with PH. I hope you stay tuned.

(In the interest of proper citations practice, I have to give credit to www.phassociation.org for the fact in this post. This stuff is all in my head now, but most of it came at the beginning from the really good folks there)

Well, here I am blogging. I’ve kicked around the idea of doing this now and then. Kind of wondered what the point was – sure, my thoughts are interesting to me (or I wouldn’t be thinking them, right?) but to others?? I haven’t quite caught on to the whole “my life is an open book on facebook/youtube/myspace/twitter” thing… Although I am, of course, a facebook addict, I mostly share pictures of my insanely cute kid and keep up with my friends (and many of their insanely cute kids).

My dad has bugged me to be a writer for as long as I can remember (Hi Dad!). Okay, so maybe I’m finally stepping up. Or, maybe, I finally have something that is so important to say that I think the world needs to hear it. Yeah… it is probably more of the latter (No offense, Dad).

But here we are – November 2009. It is Pulmonary Hypertension (PH) Awareness Month. My second such month in a line of what is looking like many to come.

I started “going public” with my disease last year, so there’s a good chance many of you even taking the time to read this (thank you, by the way) know my original story and know a little about PH. I’m actually not going to dwell on that part much, at least for now.

In short, PH is “a simplified name for a complex health problem” (www.phassociation.org). It is progressive and currently incurable. It causes chronic high blood pressure in my lungs and it effects my heart in a big way. So big, in fact, that the Grinch and his heart have nothing on me – mine grew a storybook three sizes a while ago, and it doesn’t like it! Average survival rate, with treatment, is 50% at five years. I don’t know about you, but those odds (while improved from even a few years ago) sound pretty crappy to me. Thank God I’m not a statistic.

So, what does PH mean to a real person, not as a medical diagnosis?

In the day-to-day this means I take a shit load of drugs (Pardon the profanity -I decided this is my blog, my voice, and I say “shit” sometimes!). It means I have to sleep with an oxygen tube up my nose. I also think this is shitty. It means I spend more time at doctors than I care to think about. It means that I can’t carry the groceries, prod a toddler along, and block the dog from escaping all at once because if I do I will probably pass out. It means I used to kick box and dance my heart out and I can’t anymore. And that breaks my heart. It means I look at my son and pray in a way I didn’t think possible that I will live to see him grow up. It means I can’t talk about that part any more because as brave as I am (almost) every day, thinking about him like that makes me start to cry immediately.

PH means I look at my phriends (others in the PH community) and stand in awe of their fights. I know I have it easy compared to some. Many of them are attached 24/7 to pumps that deposit medication directly into their bodies through a tube in their chest. Mere minutes off these medications could be life threatening. The side effects of the medication can be incredibly debilitating – but I’m told even intense physical pain, nausea/diarrhea , flushing, rashes, and line infections beat feeling like you can’t take another breath or get out of bed. And I believe them. It means waiting desperately for a phone call that donor heart and lungs have been found, but knowing that after transplant a whole different fight begins. I also can’t help but wonder if this is my future too. PH is, after all, a progressive disease, and I have no idea how I would handle some of that. Some of them are parents of PH patients – I can’t even imagine what that must be like, but they are some incredibly strong people.

PH means some good things too. It means I’ve found out I’m stronger than I knew. It means I used to believe in miracles, but now I’ve lived them. It means I’ve found a network of friends who fight the same fight I do and I am so deeply grateful for them. It means my friends and family I had around before I was sick  have rallied around me more than ever, and I feel their support and love daily. It means I’ve renewed and deepened my faith in God, and also made a few concessions towards modern medicine that I might not have, had I not gotten sick. It means I look towards the future with a sense of insecurity I hate, but that is balanced with unshakable faith. It means that cliche of “when you get sick every moment becomes more precious” is actually true.

I’m not really sure what else I’m going to write about. I think I’ll take it as it comes. If anyone reading this wants to hear more about something, I’m open to inspiration. Also, I would love it if you would leave a “I was here!” comment so I know, well, that you were here!

Take Care