A Year in Review

Me vs Pulmonary Hypertension

It has been one year since diagnosis. I really expected things to be different.

When I started this blog another person with Pulmonary Hypertension told me to keep it light- no one wants to hear you complain or cry. While this advice is sound- it is difficult to explain how this process has been without sounding like I am complaining. Truthfully- the past year has sucked. There is no way around it. There is not a nice way to word it. It sucked- and that is okay. Things are allowed to suck. You are allowed to have a bad year- and it is okay to own it. So, if you don't want to hear about my year (which most people would categorize as the physical shits) I would skip this post and continue with your day.

Being diagnosed with Pulmonary Hypertension, especially so suddenly and at such a severe stage proves complex and difficult. It has complicated all of my relationships, and sometimes I carry a lot of guilt. I know this disease isn't just hard on me. It is hard on my parents, my family, my boyfriend, and the few remaining friends I have left.

Someone who cares deeply about me deeply told me the other day that sometimes he thinks that I am faking. I was initially hurt by his words, but then I thought about it. Other than the oxygen, I look completely the same. He has no idea how I feel, and even if I were to describe how I physically feel, he will not truly understand how it feels because he is not in my position. He will not understand that even though I look fine, I am exhausted from working harder than him just to breathe all day. Something like a wheelchair is a visual cue to others that someone is not able to use their legs. When you have a heart/lung disease there is no visual cue.

In the movie Maleficent, the main character has wings because she is a fairy. One day someone she trusted cuts off her wings in her sleep. When she wakes up and discovers her wings are gone she lets out a giant scream and sobs while holding her back where her wings once were. It sounds silly, but I really related to this scene. It felts as if someone, somewhere, decided someday to take away my ability to use my heart and lungs properly. When you are unable to breathe properly- it is hard to do many physical things that you one enjoyed. I can't explain to you how much I miss my independence and exploring the world around me.What hurts more is not being able to do a basic task, like dress myself without supplementary oxygen. Doing so without oxygen leaves me breathless and my lips blue. It is a shitty feeling for anyone. It is something you never expect to happen to you, especially at such a young age. My sense of normalcy has been taken from me, and I don't know why. Sometimes I wonder if I did something wrong to do deserve this. Was it something I ate? Was it karma? Was my name drawn from a hat?

I am in a long-term relationship with a wonderful young man who is in his 20s. He has been as supportive as he knows how, and I am thankful for that. I know it takes a lot of love and faith to stay with someone with a condition with mine- and I know that not even marriage makes people stay in situations like this. I obviously have some guilt about someone so young choosing to stay with me. I don't know what my health will be like in the future. I cannot give him biological children. I really don't know what could happen. I don't know what kind of future I could give to him, or promise him- or anyone.

Not many people have the life experience of dating someone with a serious chronic life-threatening disease. Oddly enough, this is something that I do have experience with. I was in a relationship with someone for nearly 6 years who had childhood cancer. There were a few scares that his cancer returned, this seemed to happen around Christmas (just like my diagnosis.) One year, the year I was supposed to apply to university, the scare seemed more serious than the others. They had to run more tests- and we were obviously very scared and worried. This is a story I have never told anyone else... I shared with a woman at work about the potential cancer scare with my boyfriend at the time, and she shared with me that she too had dated someone with cancer. She said that he was the love of her life- but she couldn't stay with him. She felt it was too much of a gamble to love someone who was more likely to get sick- and perhaps, die. She advised me to do the same and said I should break up with him before things get worse. In a weird way, her advice helped me because I disagreed with it so strongly. Her advice taught me to love everyone as much as I can in all the time that we have together- which is a beautiful lesson that not everyone is brave enough to follow. This woman chose not to spend the rest of her life, with the love her life out of fear. After that I chose to do whatever I could to help. I decided not to apply to university just in case the next round of results were not what we were hoping for. I was prepared to put my life on hold and take care of him, because everything else would be waiting at the end if he did need treatment. Luckily, his cancer did not return. I went to university a year later...I had an early acceptance and was offered multiple scholarships. Everything was waiting for me a year later.

As previously mentioned, I cannot carry children. Pregnancy increases the risk of mortality in women with PH. Earlier this year I was sent to a gynecologist office to discuss my birth control options. I was sent on a few months after diagnosis. The nurse there asked me how I was dealing with the diagnosis. I responded that it was a "total bummer." She gave me a look and asked "total bummer?" It is probably the worst thing that has ever happened to me, and that extends to those in my immediate circle who love me. Do I want to share this with a stranger? Not really- and I assume on some level a nurse understands the degree of suckage adjusting to that kind of diagnosis is. Being there was a horrible experience. The waiting room wall was plastered with faces of babies the office helped deliver. It dawned on me- this is a place people go for something joyous. I cried in the waiting room facing the pictures of children knowing I was there to prevent having my own because it is too dangerous. I cried speaking to the doctor about my options. It was too soon. (This was the second only time I cried at the doctors.) I imagine it is devastating to find out that pregnancy is too risky for most women of child baring age. I should mention that I have Teaching Preparation Specialization on my degree and worked at various child care centres- I was someone who obviously liked working with children and looked forward to being a parent. There has always been a desire to adopt. I am just worried I won't be healthy, stable or here long enough to be the mother I dreamed of being. However, there have been stories on this blog from other PHers who have adopted and they are 30-40+ diagnosis. How silly would it be if I let opportunities pass me by because I was afraid? What if I reach my goal of living to 87 years old and decided not to pursue anything because I was afraid I may not be here? That would be a life wasted. I am trying to learn that I cannot let fear decide my fate, nor can I let this disease decide my fate for me.

A video posted by serena (@worshipandtribute) on Dec 12, 2014 at 3:03pm PST

I like to end things on a positive note. Although this year has really sucked (like really sucked,)  I haven't accepted defeat. As I mentioned in an earlier post I started to do Wii Fit Yoga as soon as I was able to stand again after diagnosis. I would have to do this at 4 liters of oxygen per minute. Now I walk for 20-40 minutes on a the Wii Fit bored on 2-2.5 litres of oxygen per minute. I can also box using a punching bag and do exercises on a Total Body Gym on the same amount of supplementary oxygen. I never thought I would be on oxygen for an entire year (or more,) but I have clearly made great improvements. I can sit completely without oxygen for the entire day. I have returned to work part-time and only use my oxygen to exit and enter the building. I am starting to do small tasks without oxygen, and my doctor is confident I will get off of it completely once my heart heals as it is. I was in bad shape and I suppose it will take sometime for my body to adapt and heal. I am looking forward to 2015. I am okay with one year out of my life being the physical shits. I plan on having many more, so one year out of 87 isn't bad.

I have also learned that I really really really like having control. Feeling as if a disease has control over me is not an option for me. As such, I have found ways to give me power back against Pulmonary Hypertension. I have done research on the characteristics of Cancer survivors (because no research of this exists for PH.) Oddly enough, a lot of the characteristics of Cancer survivors overlap with the qualities I have found in the PHers who have contributed their story to this blog! I have been trying to embrace those characteristics and incorporating alternative and complementary health care into my life. I have seen a naturopath, Bowen therapist, a CHEK practitioner, hypnotherapist and a healer. It might sound a little crazy- but why not try anything and everything if it is not dangerous? I also regularly get back massages at the fanciest spa in town and I think the world would be a better if everyone could get one. I journal everyday. I do at least 20-40 minutes of cardio everyday. I have altered my diet to suit my new needs. I meditate, I do breathing exercises. I practice self love and do what makes me happy. I say no to things I don't want to do, and use a bathbomb from Lush every Sunday. I do research about Pulmonary Hypertension, medication, so on and so forth and I am my own advocate.

I am not sure what 2015 has in store for me, but I am hopeful that this will be the year I complete my "comeback."

PHighter Friday: Jamie

Post-transplant

I always find it incredibly difficult to explain my PH journey. Even within the small tightknit community of PH, most of our journeys are vastly different. My story starts as a disaster but ends five years later and includes a family, a job, school, and enough energy to walk five kilometers a day.

Let me start from the beginning. Firstly, I was diagnosed late… actually 23 years too late. I was born with a hole in my heart and after countless doctors’ appointments where I was either given antibiotics, or accused of lying to get out of work, I spun my car out on the busiest highway in Toronto, which finally led to my diagnosis.

After going to the local walk-in clinic, and having an oximeter reading done, I was diagnosed within the month with stage IV pulmonary hypertension.

My health for the next four years really only went downwards, oral medication did not work, subcutaneous medication did not work, and when I was finally put on IV Remodulin, while it was true that I was feeling much better, a month in a clot broke off the line and caused a minor heart attack.

The final breaking point before my life changed forever was here in Ottawa. I had chest pain so I texted my nurse coordinator who told me to go to emergency immediately. I was pretty busy, so first my wife and I signed my school loan documents before heading over. This was a VERY bad idea, my saturation levels were sitting at under 50 percent, and I was heading into full organ failure.

The decision my PH team came up with was to fly me to Toronto General for a transplant workup. It turned out that I was so far gone, that they put me at the top of the national registry immediately, and I had a double-lung transplant/heart repair within 48 hours.

As I said this was the shift in my life to make everything better. At the end of three months in Toronto, I was jogging, walking up seven flights of stairs, and doing things that I was never able to do.

 I’m not suggesting transplant is for everyone, certainly it has its up’s and down’s, but for me, I’m the healthiest – and happiest – I have ever been.

PHighter Friday: Carolyn

Dorset Tower

My PH symptoms began after the birth of my second child. My son was born February of 1996 my symptoms began in October of 1996 and on April 15th 1997 I was diagnosed with stage 4 PH.

Camel back in Morocco

My PH Journey is not like most, you know the usual, 6 months to 2 years. I was very blessed in receiving the news that I may not survive the night. With given only a few hours to live my family was sent for to say their good byes.

After surviving the night to everyone's surprise I stayed in ICU for a few days and then sent home to “die” in comfort and to make proper arrangements for my demise. After surviving a month I was given 6 months, that is when we decided to take our last family vacation and took our 4 year old daughter and 1 year old son to Disney World. After surviving 6 months I was given a year. Originally I was put on Norsvac until a heart and double lung transplant was necessary. To this day I am still at the same dosage of Norsvac. To my understanding the DR’s do not even suggest Norsvac to new patients as there are so many other meds out there that have show a quicker response to PH patients.

Great Wall of China

When I was 6 months in with PH I am thinking I may survive a little longer. I called the Lung Association to find out where the support group meetings are held. The Lung Association (at that time) had never heard of PH and was told to call The Heart and Stroke Association. They too had never heard of PH. Well then, I will start the first of its kind in Canada. So it began. We met at TGH once a month with a social worker present.

My sister had made pamphlets with pictures of the people that had attended group. Unfortunately, due to the privacy act no one new heard about our group. I just thought that nobody cared. I did get involved with the association in the US (PHA) and attended several of the conferences with my family in tow.  

I had PartyLite, Pampered Chef, Fifth Avenue Jewelry parties, Yuk Yuk fundraiser’s etc etc. With all proceeds going to then, Pulmonary Hypertension Society of Ontario, to support Dr Duncan Stewart efforts for a cure. That was way back in the 90’s.

Snorkeling in Bahamas

In the mean time my Pulmonary pressures are coming down from the original 104 at diagnoses. After a year I was given 2 years,you guessed it then 5 years. Now they do not give me a time of survival as it has been over 17 years. I have always been a traveller, but now I have a bee in my bonnet and want to enjoy as much of the world as I can. I have visited 52 countries to date. This past summer I spent 5 weeks overseas visiting my daughter in Swansea Wales. While there we travelled to Morocco, covered north to south,east to west of Wales. We journeyed to Puglia Italy and I drove through the south of England and Northern Ireland.

Carrick -a-Rede Rope Bridge Northern Ireland

Some of the many things I have accomplished since I have had PH.

While in Oahu Hawaii, I climbed the 175 steps to the top of Diamond Head.

While in Beijing China I climbed the Great Wall.

While in Athens Greece I climbed the Acropolis.

While in Pisa Italy I climbed the 296 steps to the top of the Leaning tower of Pisa.

While in Dorset I climbed the 128 steps up the Dorset Tower.

It is not easy but I am determined to do it.

Tubing in Jamaica

I love to Zip-Line and Snorkel. I walk 5km a day with my dog, Sheldon. In February I will be Para-sailing in the Caribbean. Some much to look forward too.  

I keep myself busy daily. I have not worked since my daughter was born, 21 ago. When my kids were in grade school I ran the milkshake and pizza days the school choir and the reading program. I was a Brownie, Girl Guide and Pathfinder leader for seven years. I run a ladies book group every Tuesday morning at my home. Every Thursday I go into Toronto to visit friends. I organize a yearly ladies cruise getaway in February. I host a monthly Quiz Night in Brooklin.

PHighter Friday: Becca

 At one month old, when my parents adopted they were told, "I has a 13% chance of survival to the age of 5."When she was 3 months old they were told by the doctors there was nothing more they could do... they felt that I had 6 months or less to live. Hospice started coming to our house to help my family through the darkness. When I was a year old we were told that "things" had changed with her heart. There was a surgical procedure that could be performed that would enable me to grow up!

Miracle #1.On November 19th 1993 when I was 13 months old I had my second open heart surgery. The operation went well but I would not come off of the heart and lung machine. I was put on ECMO, a portable heart lung machine considered a "heroic measure" and taken to the Intensive Care Unit. Four days later she was taken off of ECMO and a month later she left the hospital.

Miracle #2.When I was 26 months old we traveled to UCLA for my 3rd surgery. My family had been told it was very high risk and I would have a very "rocky" recovery period. I surprised everyone, did wonderfully and was home in 10 days.

Miracle # 3.When I was 5years old she had her 7th heart catherization. In this cath massive ballooning and the placement of 2 stents were performed. I began bleeding from her lungs... they felt it would be very difficult to stop the bleeding, and if the bleeding did not stop she would die.

Miracle # 4.I had surgery at Lucile Packard Children's Hospital (LPCH), part of Stanford, when she was 12 years old. She did well but 2 days after we returned home she was back in a local hospital’s Intensive Care Unit fighting desperately for her life. During surgery she had contracted a deadly infection Methicillin Resistant Stafyloccus Arus (MRSA) that raged through my body. After 5 weeks in intensive care I went home very weak, in a wheel chair, with a PIC line and 6 more weeks of IV medicine to finish.

Miracle # 5.In my 8th grade year I started having arrhythmias. After trying for 4 months to control my irregular heart beats my condition turned life threatening and I was life-flighted to LPCH. I spent 2 weeks in the intensive care unit as they increased a strong anti-arrhythmia medicine and placed into me an experimental pacemaker/defibrillator.

Miracle #6 Just this past summer I had to go all the way to Pittsburgh for a heart-lung transplant evaluation. After being turned down by 3 other centers, Pittsburgh was our last hope. Thankfully the surgeon has decided that he thinks he can successfully do the transplant, but that at this time I am too healthy.

Looking back at my life, there were more than a few times when I wasn’t expecting to make it. Now I just celebrated my 22nd birthday with family and friends. Going to college was something my family didn’t know I’d be able to do, but I’m in my 3rd year at a community college and I love it.

I was lucky in the sense that I was born with my PH (secondary to my heart defect) because I have had my whole life to learn how to cope with this and I don’t have an old life to look back on and miss. But there have been times when I think about having a normal life or the things I wish I was able to do (go on roller coasters, fly without oxygen and go into the mountains) and miss the thought of those experiences.

In all honesty though, I’m not sure I’d change the fact that I’m sick if I was given a chance to. If I hadn’t been born sick I wouldn’t have been adopted by the amazing family I have now, wouldn’t have gone to the wonderful heart camp where I made so many friends, might not have picked up my two favorite hobbies; writing and performing ASL. Having PH has impacted my life negatively and I know I will more than likely die from it, but I’ve learned to look at what it has given me as well.

They say "It takes a village to raise a child." For me the saying would need to be changed to "It has taken five hospitals (in 3 different states), 4 pediatric cardiothoracic surgeons, several pediatric cardiologists, intensivists, pulmonologists, pulmonary hypertension specialists, infectious specialists, nurse practioners, nurses, respiratory therapists, a wonderful pediatrician, a special child psychologist, a loving family, caring friends and understanding teachers and schools to raise me and allow me to thrive as much as I have.

http://lifeasachronicallyillteen.blogspot.ca/

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.

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.

Medication:

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.

Elise Firestone
www.elisefirestone.com
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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.