Friday, 28 November 2014

PHighter Friday: Randy

My name's Randy Shifflett. I was born with a hole in my heart and had it repaired at age four. Which I found out is what caused me to have pulmonary hypertension later in life. When I was thirteen I ended up having pneumonia in both lungs and it just kept getting worse and worse. It got to the point where whenever I went to the local  hospital, they would immediately take me back because my oxygen level would drop to the seventies. They kept telling me it resolving pneumonia and keep taking the medicine and rest. So, my mom ended up taking me to the University of Virginia (UVA) hospital and it was there when I was diagnosed with pulmonary hypertension.

They admitted me and tried medication after medication but none seemed to work. My mom asked one of the nurses what was going on and he told her that he wasn't supposed to say anything but they honestly didn't know because they were giving me medicine that they used for full grown men. Finally the next day they found a medicine called Tracleer that brought my pulmonary levels down. The medicine worked up to ten years. Then I started going back downhill. The medicine just wasn't working anymore so they ended up changing my medication (I'm sorry I can't remember the name of it). I ended up having an aortic aneurism and had to have another open heart surgery in 2010. It was a long recovery but I finally got through it.

After all that, I told the doctor that I just didn't feel like the medicine was working. So, I ended up on a Veletri pump that I was on for three years. I had headaches, body aches, chest pain, losing my breath, etc. My oxygen level would drop to the eighties during the walk test at UVA. I went through that for three long years after telling the doctors that it made me miserable. Three long years of constant misery and aggravation dealing with having to clean the site, covering it when taking a shower, getting the line tangled up when I went to bed... and they finally switched me to Opsumit and had my central line removed. Now I'm doing good. My oxygen stays in the 90s and improved my distance in the walk test.

I know I was very long winded in this message but I did it for a reason; no matter how hard the struggle, you can make it. It is hard to keep your head up dealing with PH but just take it one day at a time. Don't let life overwhelm you, enjoy it.

Friday, 21 November 2014

PHighter Friday: Elise

When were you diagnosed with PH?
I was diagnosed with PH at the age of 19. Thirteen years ago.  At, the time I was living in Long Island, and was being my usually active self, when I realize the tasks of everyday living including walking were becoming extremely difficult.  I remember being at the gym and doing a leg workout, and having to sit with my head between my legs from being so dizzy.  That same summer, my mom came down to visit me from CT and as we were walking on Jones Beach, I couldn’t take more then 5 steps without having dizziness, ringing in my ears, and shortness of breath.

After finally passing out a few times, it was tine to see a cardiologist.  And after several tests including an EKG, Echo Cardiogram and a right heart Catherization it was determined that I had primary pulmonary hypertension and was in stage 3.  Being so young I didn’t quite fully understand how life threatening this disease was. Growing up as competitive dancer and being a personal trainer and weight lifting, I just wasn’t going to let this get in my way.

After responding to the first oral medication Traceller and being on Procardia XL I was back and able to perform my daily activities, and even went back to the gym to train and workout.    With motivation behind me I decided to compete in a fitness competition, and I successfully did 3 years after being diagnosed.  Along with competing in the Miss CT/Miss USA pageant with the platform of raising awareness for PH.

Daily Activates:
I’ve been a very good responder to the medication and have recently gone off of the Traceleer and now am just taking Norvasc 5mg, (generic brand to Procardia). This year all my tests came out normal showing that my PH is very much under control; I’m able to walk, run, lift weights, and do it all.  I’ve found that keeping my body in shape helps.  The only thing I do realize is that I need a lot of sleep, because my body gets run down and I’m more sucseptable to getting sick when I don’t take care of myself.

The Hardest Part of PH is the medical insurance part. Because I don’t work for a corporation and I am self-employed getting insurance and good insurance to cover all the expenses is so difficult.  When I was younger I was on my parents insurance and I stay in college purposely as a full time student until 26 so that I was covered.  I was also on Cobra for a while and paying over $600 per month on insurances. The struggle has really been financial especially because I’m now on my own.

What does the future hold?
 I’ve been extremely fortunate that I am in the 10 percent who responds well to being on 1 medication, but since going off of the Tracleer most recently, I will have to go for tests (EKG, ECHO, PFT’s) every 3 months to make sure that nothing has changed.
I don’t know what the future holds I could get worse with age, or maybe I could stay the same? I’m not sure. All I know is that I never take life for granite and I appreciate and live life to it’s fullest. 
I’ve accepted the fact that it’s probably not in my best interest to birth my own children but I’m okay with this.  I was always a believer in giving a child a home who needed it.  I’ve come to grips with life, and decided that I am the only one who can really make my dreams happen.

Tuesday, 18 November 2014

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 ( I had discovered the Pulmonary Hypertension Association ( 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.

Friday, 14 November 2014

PHighter Friday: Clair

My diagnosis came on much like it does for many other young women.  During high school, I began to notice an increasing amount of chest pain, a racing heartbeat, and shortness of breath.  Characteristic of a developing adolescent, I denied that these symptoms were abnormal.  I attributed my inability to breathe and instances of passing out as being out of shape.  However, after a year of tests, hospital visits, and appointments with doctors and specialists, I was diagnosed with Primary Pulmonary Hypertension. 

As a senior in high school preparing to leave for college, the diagnosis was devastating.  Even though my doctor gave me the green light to go away to college and a positive long-term prognosis, I began to find that the emotional toll of having a progressive disease was more difficult than its physical manifestations.  Even though I attempted to cope with the anxiety that stemmed from my diagnosis with PH, it began to extend to other aspects of my life.  I began to doubt what I could make of and do in my future, and this doubt precipitated new insecurities.

Although there wasn’t a dramatic turning point, the woman with PH I am now is a stark contrast from that newly diagnosed teenager.  I changed the way I took care of myself.  I began to eat healthier by eliminating junk and adding fresh produce to my daily diet.  I also started practicing yoga and working out.  Of course, my workouts are modified.  I’m not running miles upon miles or doing intense cardio, and probably never will. But I keep a consistent schedule of yoga and lead a rock-climbing and yoga club for my students at the school I teach.  I do what I can, and I’m certainly doing much more than I ever thought would be possible after receiving my initial diagnosis

This past summer I spent 6 weeks in Europe.  I climbed Mount Vesuvius, a large volcano in Italy, climbed over 600 stairs to the top of a church in Florence, hiked in the Alps, and visited 6 countries.  The people with me understood that although I said yes to every adventure, I was going to do it at my own pace.  When I climbed Mount Vesuvius, I stopped a little at first, but as the altitude increased, I had to stop every few steps.  In our group, I was the very last person to make it to the crater and I didn’t care. I was only focused on completing my journey to the top no matter how long it took me.

Now a decade into my journey with Pulmonary Hypertension, I’m currently in the process of planning a trip this winter to Zambia, Africa.  Disease or not, everyone has one life.  Dwelling on what will or will not happen will only cause unnecessary worries.  Had I never broken free from my initial doubts or my perceived, grim outlook on my future, I know that I never would have taken the chance to start yoga, or to attempt an ambitious climb to the top of a volcano.  However, I was allowed to do both of these things, and I will continue to experience life, because I am committed to doing the absolute most that I can with my disability.  I will not allow Pulmonary Hypertension – or the psychological toll that comes along with it - to take away my potential and the opportunities that come my way.

Friday, 7 November 2014

PHighter Friday: Anastasia

Slowly but Surely

I wanted to travel the world. I wanted to be a powerful business woman (like Samantha Jones in “Sex and the City”) and have a family. Also, I love sport such as swimming, bicycling, playing basketball, playing badminton and playing tennis.  Most of my childhood was filled with fun activities, travelling, hanging out with friends and breaking the rules. But those dreams were crushed when my doctor told me that I will not be able to do sports that I love, I will not be able to travel, and having children is out of the question….

I was diagnosed with Primary Pulmonary Hypertension when I fifteen years old when I moved to the U.S. Back in Indonesia, I was always chubby and sluggish back in middle school and part of high school. My parents thought it was because lack of exercise and my general doctors in Indonesia did not detect any health issues. When I was about to start high school in U.S., through regular check-up, the family doctor detected irregular heartbeat and I started to notice apparent health issues such as difficulty breathing, chest pain and blue lips. So, I was urged to see a cardiologist and pulmonologist. Sure enough, I had VSD (Ventricular Septal Defect) and PPH (Primary Pulmonary Hypertension).

I was devastated…
For a while, I could not understand why this defect happened to me. Although, overtime I realized that wallowing is not getting me anywhere.

After the diagnosis, I had my open heart surgery on my 16th Birthday to close the hole in my heart. I am taking medications to control my PPH. It is not easy to integrate this disorder into my life at first.
I realize that I am rare. So, as long as that is the case, why not go all the way.  So the key is to accept that I do have this unique condition and I am going to have to live with it for the rest of my life.

So I learn to get to know myself.

I am thirty years old now. I am working full time as a supervisor, I volunteered at convalescent homes, I go to the gym at least three times a week, I went hiking (5 miles up the hills recently) and I am finishing my bachelors in Management and International Business. I do what I can now and everything else will fall into place. I may not go with the phase of normal people, but I will finish what I start slowly but surely.

I changed pulmonologist who is better and gave me support. She advises that I should exercise and keep doing what I do at my own phase.

I believe that our journey gives us strength; the destination is just the icing in the cake. And getting to know myself is the fundamental part of starting the journey and setting up goals. Also, it is important to surround myself with people who are supportive of me and my goals.

Life may not always go as planned, but it does not mean that I cannot make the best out of the one I have now. Like Mother Teresa said ““Yesterday is gone. Tomorrow has not yet come. We have only today. Let us begin.”