Accessibility of PAH Therapies in Canada: Part III - Patient Testimonials

Part III: Testimonials From PH Patients Who Are On A Treatment Not Available or Accessible in Canada

This is the third and final part of the series. The first part of the series, Access to Opsumit in Canada can be found here. The second part of the series about Treatments That Are Not Available in Canada Can Be found here.

Below are testimonials from PH patients in the US who are on inhaled PH medications that are not available in Canada. These testimonials are unpaid, and are shared by fellow PH patients in an effort to show the positive effect these medications can have for some PH patients.

"I started Tyvaso on the trial in November of 2004 at UCSD medical center. I was asked to go on the trial due to my high numbers.  I was diagnosed in April of 2004.  My mean pressure in my heart during right heart catheter was 96(normal is between 15 and 25).  I had gone years with a misdiagnosis of severe asthma.  I went in for another right heart cath to start the trial.  My numbers had come down, good news to 56 with the use of a medication called Tracleer.  While on the operating table, I took my first puffs of Tyvaso, I took nine.  I was the first patient ever to take nine puffs of the medication.  My mean pressure dropped from 56 to 46.  It was really a miracle for me.  At the end of the study my pressure was 35.  Darn right near normal.  Sadly though, I had to quit the Tracleer and start on Revatio due to liver problems.  But I have been on Tyvaso since starting.

I started going downhill a little November of 2013, I was needing evening O2, and usually I need only at night time.  At this time I was on Revatio and Tyvaso.  I had another dreaded right heart cath in March of 2014.  My doctor was happy with my pressure of 50.  But I was not.  My husband and I pressured him to add another medicine.  He decided to add opsumit, since I did so well on the tracleer and it is very closely related to tracleer without the liver complications we decided to give it a shot.  Fast forward to June of 2015.  I had an ECHOcardiogram.  I asked my doctor my "estimated" pressure as it is not as thorough as a right heart cath.  He said 29.  Wow...almost normal.  I have been feeling pretty good these days.  I am very reactionary.  I have good days and bad.  Without these medications I would for sure be in pretty bad shape of worse dead.  I have 3 adult children and a husband.  A grandson.  Also, raising my niece and nephew, ages 15 and 12."
- Julie, 48 years old
San Diego, California, USA

"I started the inhaled therapy, Treprostinil (Tyvaso), in 2011 after going back and forth with my doctors about IV therapy since my pressures were high and my physical activity was low.  They decided to try me on Tyvaso just to see how I would respond on it.  At first it was a bit difficult to fall into a routine of breathing on a nebulizer every 4 hours, but I wasn't too concerned after I started to feel better.  Before Tyvaso, I'd have a very hard time doing the simplest daily tasks like making my bed, or even showering, without getting short of breath.  Now after almost 5 years on Tyvaso, I'm able to exercise, walk long distances, go on bike rides, almost anything and everything that I wasn't able to do b efore the medication.  It has also improved my 6-minute walk test results, as well as my echocardiogram results.  Tyvaso, in conjunction with my oral medication, has improved my quality of life tremendously and I'm so grateful to have the therapy available to me."

-Reinee
Bay Area, California, USA

"In 2009 I was diagnosed with Familial Pulmonary Hypertension. At that time I was put on two different treatments. One was a long established drug Tracleer, and the other was an inhaler that had been FDA approved only months prior, Tyvaso. The doctor has switched my pill form of medication, but Tyvaso remains my main treatment. I always credit Tyvaso as saving my life. Before diagnosis I was not able to walk up a flight of stairs without getting out of breath, now I can go hiking and jogging. I can breathe easier with Tyvaso, if I am without it for a day I can feel the difference in my chest and my exercise capacity is diminished. "

- Raeana Rader, 28 years old
USA

"I was diagnosed with pulmonary hypertension after I passed out while walking into work. That
was simply too much exertion for my body. I was placed on I.V. Veletri and two oral medications. They got my symptoms under control but I had terrible side effects from the Velerti. The nausea, vomiting, diarrhea and headaches were the worst. I eventually improved so much that my doctor let me do a conversion from Veletri to Tyvaso. Tyvaso gave me my quality of life back. The only side effect I have with Tyvaso is flushing. I feel like a normal human being again. Because I am no longer on Veletri, I can take my kids swimming and no longer have to sit on the sidelines. I am able to exercise on an elliptical for an hour at a time and I have recently started taking a spin class. Tyvaso is also much cheaper than Veletri and because it is noninvasive, I do not have to worry about line infections which could be costly and detrimental to my health. I feel very blessed and grateful to have Tyvaso available to me."
- Ashley, USA

Accessibility of PAH Therapies in Canada: Part II

Part II: Treatments for PAH That Are Not Available In Canada

As someone with Pulmonary Hypertension, I was very disappointed to learn that a couple of potentially life altering medications that have been available for many years in the United States of America are not available here in Canada. I had assumed that because Canada offers free “universal” health care, that it also had the best interest of its patients in mind. Needless to say, I was completely shocked to learn that some prostanoid-family medications like Tyvaso (inhaled treprostinil) and Ventavis (inhaled iloprost) and Orenitram (oral tablet form of treprostinil) are not available here in Canada. These medications are a less invasive form of intravenous prostanoids such as epoprostenol (Flolan, Caripul) and subcutaneous treprostinil (Remodulin). I asked PH specialist Dr. Sanjay Mehta to answer some questions I have regarding the accessibility of of PH therapies in Canada.

Serena: Could you explain the benefits of inhaled and oral versions of treprostinil over the intravenous and subcutaneous prostanoid treatments? Do you believe that medications like Tyvaso and Orenitram should be available to Canadians with PH? Could you briefly explain why these medications are not currently available here in Canada?  Is there anything that we can do as a community to have these medications available here?



Dr. Mehta:  Treprostinil is a very effective treatment for PH. It is currently approved and available in Canada both for intravenous and subcutaneous administration as Remodulin.  Both of these approaches require an external pump and a cassette or syringe to be filled regularly, usually every 1 or 2 days. 

Treprostinil has been modified for patients to be able to take it by either breathing it in (inhaled Tyvaso in the US) or by taking a pill (oral Orenitram in the US). Both of these approaches have been shown to be effective in treating PH. Moreover, the inhaled and oral routes of drug administration clearly simplify the treatment compared with much more complex intravenous/subcutaneous treprostinil administration.  However, inhaled and/or oral treprostinil are not the best treatments for all PH patients. Inhaled treprostinil may not be as effective as intravenous/subcutaneous treprostinil, and is typically only used as an addition to other oral PH therapies in the US. Oral treprostinil is effective, but side-effects of nausea, abdominal pain, and diarrhea can be difficult for some patients. 

Ultimately, both inhaled and oral treprostinil are not available to Canadian PH patients because the pharmaceutical manufacturer never submitted an application for Health Canada approval. This is largely because of business reasons, as the PH market in Canada is much smaller than in the US, and it is expensive to launch a new medication in Canada.  Moreover, many PH medications cost less in Canada than in the US, because of government regulations. As such, the cheaper price for a new PH medication in Canada can lead to pressure on a company to reduce the price in the US, which would mean they make less money in the much bigger American market. 


Serena:  Are you able to speak to a trial for and the benefits of having Remodulin administered through an implantable pump as opposed to subcutaneous or intravenous methods? Do you foresee this implatable pump being available in Canada?

Dr. Mehta:  There was an exciting trial in the US that studied whether Remodulin could be administrated via an implantable pump, rather than an external pump.  Such an implanted pump has a reservoir of medication that could last for a prolonged period, such as a month. This reservoir could be refilled regularly, such as every month, in the PH clinic.  As a result, patients would not have to look after preparing medication every day or every second day at home, or changing tubing / cassettes / needles, which would clearly improve their quality of life.  This implantable pump for Remodulin may become available in the US in 2016, and then hopefully one day in Canada.

Serena: This concludes our two-part interview. Are you able to share any insight on any exciting and promising treatments on the horizon in Canada?

Dr. Mehta: Despite all the advances we have seen in the last years in regard to PH therapies, PAH is still a progressive, often fatal illness for which we have no cure.  Tremendous research both in Canada and around the world continues to better understand the disease and what exactly is happening to the blood vessels of the lungs to cause PAH.  There are many ideas for new treatment approaches, including both pharmaceuticals and potentially gene-therapies.  This is a very hopeful time that PAH patients will continue to benefit from this research and new therapies, until such a time when we can say to a patient, “You have PAH, but we can treat you so that it will not affect you in everyday life and will not shorten your life.”

The PHight or Flight Project would like to thank Dr. Sanjay Mehta and The Pulmonary Hypertension Association of Canada for their assistance on the Accessibility of PAH Therapies in Canada Series.

*This is the second part of a three part series. The first part of the series, Access to Opsumit in Canada can be found here. The third and final part of the series will share testimonials from PH patients in the US who are on the various medications for PH that are not available in Canada. 

Accessibility of PAH Therapies in Canada: Part I - Access to Opsumit

Part I: Access to Opsumit in Canada

In March 2015, the Canadian Agency for Drugs and Technologies in Health (CADTH) suggested that all newly diagnosed people with Pulmonary Arterial Hypertension (PAH) in functional class II or III receive the same mono-therapy approach for their treatment. This approach would take the decisions for treatment out of the hands of PH experts and place it in the hands of government bureaucrats. I asked PH specialist Dr. Sanjay Mehta* to answer some questions I have regarding the accessibility of PH therapies in Canada.



Serena: I know that you are very involved and passionate about the PH community, and that you have been working very hard to ensure that all newly diagnosed people have access to the therapy that is best suited to their needs.Could you briefly explain why it is so important that PH specialists, like yourself, are able to treat PH patients on an individual basis and recommend a therapy suited to their needs instead of the stepwise, rigidtherapy approach suggested by the CADTH? 



Dr. Mehta:  It is one of the most basic principles of medicine: that each patient is an individual, and needs to be understood, respected, and treated as an individual. This includes doctors making decisions about the best medical treatment for a patient’s illness.  Although many patients may have the same illness, as in the case of PH, they are all still unique individuals. As such, there is no reason to expect that they respond similarly to PH medications, or that the same medication is the best one for each patient. Expert PH physicians need to consider many factors in deciding on the best initial and subsequent medical treatment for each PH patient. The decision is based on having expertise and experience in PH, understanding the patient and their other conditions, and the specific risks of each treatment.  Even in clinical studies of PH medications, all patient participants don’t respond similarly and, while some may respond well, others may not.  Importantly, in dealing with a serious, progressive illness like PH, a patient can’t afford to waste precious time “trying” anything other than the most effective, best treatment for them.

Serena:  The newest Health Canada-approved oral medication for PAH, macitentan (Opsumit) is not available to most Canadian PAH patients, as per the recent pan-Canadian Pharmaceutical Alliance (pCPA) decision to close negotiations with the manufacturer, with the impact that this drug is not approved for funding in most of Canada. 

I understand that Opsumit is generally well tolerated, and that it was tested in one of the largest and longest clinical studies of any approved PH treatment. Can you discuss some of the benefits of Opsumit? If the government continues to deny funding for Opsumit, what is the future for this medication in Canada?



Dr. Mehta:  In 2013, the Seraphin study reported on the benefits of macitentan (Opsumit) in what was at the time the largest, longest study ever in PAH patients. It showed that macitentan reduced the morbidity (severity of illness) in PAH patients, specifically reducing the risk of progressive worsening of PAH by 45% and reducing the risk of hospitalization by 50% over 3 years.  Moreover, treatment with macitentan significantly improved symptoms, quality of life, and exercise capacity in PAH patients. This is a unique study that showed for the first time the long-term benefits of treatment with a PAH medication, compared to all other PAH studies which only looked at benefits over 3-6 months. Clearly, PAH patients don’t want to just improve over the short-term, but hopefully remain well for many years!

As a result, treatment with macitentan has been strongly recommended by the most recent 2014 PH Clinical Practice Guidelines jointly published by the European Society of Cardiology and the European Respiratory Society.  Moreover, macitentan has been approved for funding in the US, and in many European countries, and many PAH patients are currently being treated with macitentan.  Notably in Canada, Quebec approved public funding of macitentan in October of 2013, such that Quebec PAH patients have complete access to macitentan as a treatment for their PAH, should their expert PH physician decide it is the best treatment for them.  Similarly, several large private insurance companies across Canada have approved macitentan coverage for their clients.  However, the pCPA recently denied funding for macitentan for the rest of Canadians (PAH patients living in all the other provinces and territories who do not have access to private health care), and indeed, has broken off negotiations with the pharmaceutical manufacturer, Actelion.

This is a very concerning development for Canadian PAH patients, most of whom have been denied access to public funding for treatment with macitentan. Moreover, this establishes a dangerous precedent whereby any and all future new PAH therapies (for example, the new oral **selexipag or Uptravi, which was just approved by the FDA in the US) may similarly not get approval for funding for Canadian PAH patients.  It would appear that Canadian and provincial governments are saying that PAH patients are doing just fine with the therapies they have available today!  Clearly, PAH patients and their physicians know otherwise;  despite treatment with the many PAH medications we have, many PAH patients remain seriously ill, limited in everyday life, and their disease continues to progress until it takes their lives, on average 7-10 years after diagnosis.  Is it reasonable to accept that?  Should we not try to further improve the health and lives of PAH patients?  Most definitely, all Canadian PAH patients and their caregivers would want us to continue to develop, test, approve and make available newer and better PAH treatments. Lack of government understanding of this critical issue is already leading to less than optimal treatment of Canadian PAH patients, as currently demonstrated by lack of access to macitentan, and likely to reoccur with lack of availability of future new PAH treatments. 

Serena:  Thank you for sharing your thoughts on this complex issue. Is there anything that you would like to share going forward?

Dr. Mehta:  All Canadian PAH patients and their caregivers should be heartened by the incredible progress we’ve made in the treatment of PAH, since the 1st medication, intravenous epoprostenol (Flolan) became available in 1997. Currently, 9 different medications are approved and generally available for the treatment of PAH in Canada. As a result, the quality of life and survival of most PAH patients have significantly improved. This is important to keep in mind and we should remain hopeful that, thanks to the joint efforts of dedicated advocates, members of the medical community, and PHA Canada, new treatments such as Opsumit will be made available to all PH patients in Canada. This is what we must continue to strive for.

** selexipag/Uptravi received approval by Health Canada on Tuesday, January 26th, 2016 for the treatment of PH.

The PHight or Flight Project would like to thank Dr. Sanjay Mehta and The Pulmonary Hypertension Association of Canada for their assistance on the Accessibility of PAH Therapies in Canada Series.

PHighter Friday: Karen S

I was diagnosed with IPAH 8 years ago at the age of 52, after years of palpitations and breathlessness on excretion. Prior to diagnosis I presented to my GP on various occasions with my concerns about these symptoms. Each time I was told that it was anxiety/depression and finally prescribed anti depressants. Despite the advice from my GP my concerns and symptoms remained.

I would become breathless during my household chores, especially vacuuming and climbing the stairs. My palpitations would start, or as I referred to them, my flutterings. I even called it my 'jelly heart'. I would joke to my family ''you will remember this when I've gone''. I never dreamed it would soon be so serious.
The breathlessness and palpitations continued and were now accompanied by dizziness. I would become dizzy just walking from work to my car. I knew it was time to return to my GP.

At that visit my usual GP was unavailable and I was seen by a locum doctor. I was not expecting much but to my surprise I truly believe that this doctor saved my life! He listened to me, examined me, and decided to send me for an ECG. There then followed a battery of tests at my local hospital which lead to a diagnosis of IPAH and a referral to Papworth Hospital. I had no idea what IPAH was. When I inevitably consulted Dr Google I felt like I had been hit with a sledgehammer. I was in turmoil, I panicked I didn't know what to do with myself. I wanted answers, why me? I was a healthy woman, I had never smoked or abused my body how could I have something wrong with my heart and lungs? The words 'incurable' and 'fatal' kept going round in my head.

After attending Papworth for a 5 day stay, I felt more hopeful as I was now on a drug - bosentan and had now been given a prognosis of about five years.

I was doing well on Bosentan but it suddenly began affecting my liver and so I had to stop taking it. I was then put onto sildenafil. A few months later I agreed to take part in a trial for a pill form of treprostinil.
I was feeling so much better, I could climb stairs, I had more energy, I would go on walks and bicycle rides. Life was good again. I continued like this for about five years 'the deadline'. Then very, very worryingly, I started to feel ill again. The breathlessness and palpitations started again. I was in panic mode again and denial, I even lied to the team at Papworth telling them I felt ok, but they knew differently. I was then prescribed ambrisentan along with the other drugs that I was taking I was elated, another ray of hope. I just got better and better. My 6mw improved and my lung function tests were better. I was back to my normal life.

Then a few months ago I was asked to take part in another trial. This involved having iron infusions. I felt fantastic! My family noticed a difference in me and my daughter claimed she had her 'mom back'. My exercise tolerance and general wellbeing were like they were before I ever had PH.

A diagnosis of PH is frightening and devastating, but I am testament that with a positive mental attitude, support from a wonderful family and friends, a wonderful team at Papworth ( and mustn't forget the drugs ) life can go on and there is so much help available. I feel very hopeful for the future.

PHighter Friday: Shannon

Confessions of a Young Adult PH Patient

Hi everyone, my name is Shannon O’Donnell and I am twenty years old, and I have primary pulmonary hypertension. I am on IV Remodulin, Revatio, blood thinners, high blood pressure medication, and oxygen at night.  I was diagnosed when I was six years old. I was told that I wouldn’t live pass a year without a double lung transplant and now it’s been fourteen years since then. And now, I’m doing great, I’m off the transplant list, and talking to my doctor about getting off IV Remodulin and going to the oral form of it.

My story begins on May 31^st, 2001, I was six years old and  had no idea what was going on due to being a young child not really understanding why I’m different from my friends. My doctor wanted to admit me right away but my parents took me home in order to graduate from kindergarten with my friends, my parents wanted good things to remember of me before we started treatment. A few days later, I graduated kindergarten and had huge party with my family. The next day, we went back to Boston children’s started this rollercoaster called fighting PH. I started out on nitrous oxide then that stopped working and I had no choice but to go on Flolan, which was only approved for adults since there were no medicines approved for children at the time. That’s what I remember of the start of this uphill battle.

In second grade, I went on a Make-A-Wish to Orlando; my wish was to go to SeaWorld and swim with the dolphins and feed Shamu (a killer whale or an orca whichever species name you prefer.) I got to Disney too, and see my family mom’s side as well. Then six years later I was put on IV Remodulin due my school system threatening to take away my rights to education due having to be on ice packs 24/7 unless I switched to medicine that did not involve being on ice. Then being on the cad pump got in the way of being a teenager and I switched to the crono-5 pump. About three days after going on the pump, it decided to go off in the middle of class. I raised my hand and calmly told my teacher I had to go the nurses office and my headmaster (principal) saw me asked me to hand over my phone (he thought the pump was a phone.) I flipped over my pump and he escorted me to the nurse when saw he look of fear on my face.  Then a year later came prom, and I had no idea what to do with my pump, so my sewing teacher transformed a spandex skourt into a spandex skourt with a pump pocket. I still use it till use it today whenever I wear a skirt or a dress. Then a year later came senior year. Senior year ah the worries of college acceptance letters, SATs, and graduating. Not for me. I was in and out of the hospital and was barely passing all my classes. Well, I graduated and moved on to a community college where teachers don’t understand zero below temperatures aren't good for PH patients to be waiting for a crowded train.

I was told I couldn’t go to school full time or do sports. Now I go to college part time at a local community college, and take karate at a local dojo. I volunteer at The Hole In The Wall Gang Camp founded by Paul Newman (I was a camper there as well.) I also teach karate to mentally challenged children, and teach second grade religious education at my church.  Life at diagnosis was hard because you can’t tell a six year old not to run around. I don’t really remember much expect that. Back then, I coped with everything by playing my dolls, coloring, reading, listening to music, and playing video games. Today, I still cope by drawing, reading, listening to music, playing video games, and watching TV and watching movies. If I have learned anything from watching many Disney movies is that even if you’re different you will always have good friends by your side no matter where you go.

The advice I would have given myself when I was younger is that you got to hold your head up high and smile because you are the greatest star. My advice to anyone that reads this is that "Your imperfections make you beautiful; they make you who you are.” –Demi Lovato.

Guest Post: Living with CTEPH

Living with CTEPH By Guest Blogger Marilyn

In June 2011 I almost died from multiple small blood clots in my lungs. I spent a total of 22 days in my local hospital before my pulmonologists figured out why I was still clotting even though I was on both heparin and warfarin. In the afternoon of my last hospital day, one of the pulmonologists walked into my room smiling broadly.

“I’ve figured out what’s wrong!” he said. “You have factor V (5) Leiden!” 

Looking at him a little blankly I asked, “What’s that?” 

“It’s a clotting disorder,” he said.

My mind racing, I asked “Does that run in families?”

“Who in your family had a stroke?” was his answer.

I then filled him in on my mother and my grandparents: My mother had Parkinson’s Disease and started having frequent TIA’s (mini-strokes) in the last year of her life. Her father had many TIA’s over about 18 months and died from a pulmonary embolus (PE). My mother’s mother died of a stroke. My father’s mother also had a stroke which contributed to her death.  As I finished my list the doctor told me that factor V Leiden (fVL) is a genetic clotting defect that is fairly common, but is frequently only found in cases like mine of unexplained clotting problems. I went home the next day intending to do some in depth research on the internet.  Somehow the fVL drove the fact that I have pulmonary hypertension out of my mind.

Factor V Leiden is a genetic clotting disorder that can be either homozygous (one defective gene from each parent) or heterozygous (only one defective gene). I am heterozygous. Many people have this defect but never know it because it seems to require a trigger or secondary condition to cause clotting. Even then the person may not re-clot after an acute episode. Somehow that piece of information didn’t stick in my brain – I just knew I had already had “too numerous to count” pulmonary emboli. I didn’t want it to happen again. I might not be as lucky next time and I still have a lot of living to do.

In November 2009 my husband and I retired, closed up our house, moved ourselves, our Golden Retriever, Bonnie, and our Ragdoll cat, Zoey onto our boat and set out to realize the dream we had nurtured for many years. Our boat is a 36 foot motor sailor, which means it has a full complement of sails, but also has a hefty 90 horsepower inboard diesel engine. She was built in Finland for sailing in Northern Europe.  She is solidly built to handle storms with a pilot house and a diesel heater.  We had moved the boat to Baltimore in late October, so in November we returned to Baltimore and pointed our bow south down the Atlantic Intracoastal Waterway. The day we left, the temperature was 27 degrees and the harbor had a skin of ice on it.  We were glad to be heading to warmer climes – we thought.  Unfortunately we picked one of the coldest winters on record to make our initial foray into the world of full-time cruising.  We had friends in St. Mary’s, Georgia who regaled us with stories of this quaint Southern Georgia town.  St. Mary’s was supposed to be our first stop, until it got warmer and we could resume our trek further south.  Once we were here, though, we fell in love. This was 2010, when the housing market had finally just about hit bottom, and there were some nice homes to be had in this area. My husband decided to buy one of them, thinking that it is much easier to sail out of southern Georgia than northern New Hampshire.

We took the boat back to Maine for the summer and prepared to move our “stuff” south. We spent the rest of that year traveling up and down the eastern seaboard by car, truck, U-Haul, and boat. In November we left the boat in Elizabeth City, NC and went to Vermont, to my brother’s inn, for Thanksgiving. He was battling colon cancer, but it never occurred to us that this might be his last holiday.  He passed away on December 23, 2010.

Because my brother and sister-in-law didn’t like funerals, it was his wish not to have one. Instead, as many of his friends and family as were able to attend assembled at the Mad River Glen ski area to remember him and celebrate his life.  My husband doesn’t deal well with this type of thing, so he stayed in St. Mary’s with the animals and I drove north alone. For two days I drove the familiar Interstates and thought of my brother and what he meant to me.  The trip was two days up, two days back and two days at the inn.

So, why have I told you all this?  Because it was all that traveling, especially the last trip, that likely caused the DVT that was found in my right calf.  Part of the reason I got so sick before seeking medical care, was that I had absolutely no symptoms from the clot in my leg. Normally a DVT causes redness, swelling, heat and pain in the affected area. I had none of those.  When I started having trouble with coughing and shortness of breath, I thought it was my asthma, which I’ve had for 25+ years.  Only when I finally couldn’t walk more than a few feet without having severe respiratory distress did I give in and go to the local ER. They found the clots, and my life changed forever.

Once I was home from the hospital I started searching the Internet for more information. In my research I found out that there are a number of genetic clotting disorders, the most common being fVL and protein G20210A deficiency. Rarer are antithrombin III deficiency, protein C and protein S deficiencies and MTHFR, among others. MTHFR stands for methylenetetrahydrofolate. Basically it alters the body’s ability to properly process folate, leading to abnormal clotting.  There are also acquired or secondary clotting abnormalities stemming from diseases like Systemic Lupus and Scleroderma. These clotting disorders involve resistance to parts of the normal clotting cascade and are considered auto-immune disorders.   Now that I have you totally cross-eyed and confused, what does all this mean?

If you have a clotting disorder, and throw pulmonary emboli which then do not resolve as is normal with PE’s, the clots block the free flow of blood to the lungs and cause increased pressures in the pulmonary arteries and back pressure into the right side of the heart. Over time, these higher pressures lead to right-sided heart failure.  This is called Chronic ThromboEmbolic Pulmonary Hypertension or CTEPH, now given its own designation by the World Health Organization.  I have CTEPH. I am now a “lifer” on Coumadin (or warfarin), whose level is checked by a blood test – INR.

As there are a significant number of patients with clotting disorders who have clotted even while taking an anticoagulant, I tended to be paranoid about my INR being less than 2.5 for the first two years after my diagnosis.  It is only in the last year, really, that I have been relatively happy as long as my INR is above 2. In the back of my mind always lurks the possibility of clotting again and the thought that I might not be so lucky again.  For many years, my main concern when looking at my family health history has been that a stroke which didn’t kill me might steal away my ability to think, reason, and direct my own care. This new threat of stroke has led my husband and I to put the boat up for sale and give up our dream. I am thankful for the one year we had on the boat and heartbroken that I must give it up. I simply cannot take the risk of being offshore and having a PE or a stroke.

I said above that I am on warfarin.  I follow a couple of PE pages on Facebook, and a question that frequently comes up for discussion is whether or not the newer anticoagulants are a good choice. This is something that the doctors and researchers are still struggling with.  Warfarin has a few drawbacks, as do the others. Warfarin acts by blocking Vitamin K, important in the clotting cascade. Therefore, intake of Vitamin K in foods has to be closely monitored. That means that most green vegetables have to be omitted or limited.  Also, warfarin levels are measured by the INR, which is a blood test.  Many people find that a major disadvantage.  The drug is also notoriously difficult to regulate in some people (like me).  Luckily, I don’t mind having blood taken so it doesn’t bother me.  One of the major pluses of warfarin is that it has an antidote. If someone on warfarin arrives at the ER or MD’s office with bleeding, the INR can be checked quickly and Vitamin K administered. This counteracts the warfarin, allowing normal clotting and diminished bleeding.

The three newer anticoagulants – Pradaxa, Eliquis, and Xarelto - work by a different mechanism, so there are none of the food restrictions seen with warfarin.  They do, however, have their own concerns. There is no definitive test for whether they are working or not. I find the automatic assumption that you are taking the pill so your level of anticoagulation must be adequate less than reassuring. There is also no antidote to these drugs, although several are currently in development. The average half-life of the drugs is 48 hours. That may not sound bad to most people, but imagine yourself with a hemorrhage. That means not just a little nosebleed or a cut that won’t clot.  Not having an antidote can be a life or death situation. The third disadvantage is cost. Any new drug is very expensive, and sometimes will not be covered by insurance.  Warfarin has been around for many, many years, is generic and cheap.  Having said all that, please be aware that this is my opinion only.  I feel much safer on warfarin than I would on a newer drug.

Until last October, CTEPH was treated with the same drugs as other types of PH. Last October, however, a new drug was approved by the FDA to specifically treat CTEPH. It is called Adempas.  I started on Adcirca (which I got free from the manufacturer) in 2012, then switched to Revatio when I went on Medicare, because it was cheaper.  I wasn’t as pleased with the Revatio though, so when my pulmonologist suggested I try Adempas, I said yes.  I, like many others I have talked to, have had great results with it. My level of endurance has increased and the pressures in my pulmonary arteries went down by 20 points in six months. I am now freed from the restriction of being on oxygen 15 hours a day to mostly using it when I exercise or when I go to bed. I was doing so well, I decided to attend the 2014 International PH Conference and Scientific Sessions in June in Indianapolis.  It was a totally incredible experience. I drove myself there and back, breaking each trip into two days. I returned home ecstatic but exhausted.  It took me three weeks to fully recover, but it was worth every minute.

2014 has been a banner year for me. Last December I started a web page/blog called Of Bad Lungs and Blood Clots (www.ofbadlungsandbloodclots.com). I had discovered the Pulmonary Hypertension Association (www.phassociation.org) website in November and requested permission to use their logo with a link to their site. That email led to a phone call from PHA’s Patient and Caregiver Services Manager and my beginning close involvement with PHA.  I attended Conference, met a lot of new PHriends, participated in the fashion show, and did some writing. I am a member of the CTEPH Advisory Board and recently worked on setting up the personal stories on the CTEPH web page.  In late August I decided to start a new support group here in southeast Georgia.  I am very busy and doing things I never would have believed I would do.  But the best thing is the writing I have been doing.  I feel as though my 40 years as an RN were only leading me to this point in my life, where I get to do what I love and help others at the same time.