Sunday, November 14, 2010

A Lesson In Medications

November 21, 2009
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, 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: 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.
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