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.