How My Diagnosis is Hurting Me

"What's wrong with me?"

This was a question I found myself asking only a couple of months before being diagnosed with Pulmonary Hypertension. Upon diagnosis an expiration date was stamped on my back like a carton of milk. I was officially diagnosed with Stage 3-4 'Idiopathic Pulmonary Arterial Hypertension.' The word idiopathic is a fancy word meaning that the doctors have no idea why I developed this disease. There was no rhyme or reason.

The word 'idiopathic' attached to my medical files has almost felt like a curse.

Upon diagnosis, blood work was drawn to see if the PH was caused by a secondary condition. But this only weeds out diseases that are detectable through routine blood work. Often times, PH is caused by a secondary disease. If the secondary disease is managed, it can potentially help stabilize the condition of the PH.

I have tried very hard for over two years now to seek the help of specialists outside of the PH scope. I have all these loose ends and puzzle pieces of different symptoms and clues. They all seem to overlap into a grey area, none of which really fit into the diagnosis of PH.

Unfortunately, as soon as another specialist reads the words 'idiopathic' and 'fatal' on my medical chart, the appointment ends. I saw an endocrinologist a few months after diagnosis. She told me that I had probably about 5 years to live, and that there was nothing she could do for me. That was the end of the appointment, without even asking about my symptoms or looking through my files. Since then I have had many failed attempts with other specialists, but I continue to keep trying to find answers.

All my medical files from the first year of my diagnosis start with the same line. "Serena refuses to take her one medication as she believes it makes her worse. Serena has improved since her last visit." I look back at the months leading up to diagnosis and I started taking a medication that can interfere with the autonomic/sympathetic nervous system- although this is extremely rare. (PH- by the way, is also extremely rare.) The autonomic nervous system controls certain functions such breathing and your heart rate.

I explained to every doctor that this medication made me ill. It would make me bedridden within a matter of days after taking it, even after I started making progress. Each doctor told me to continue taking it, but I could feel it killing me. I was prescribed nearly 12 different variations of this medication until I had enough.

I stopped taking it. I switched medical teams. My 6 minute test improved by 100 meters. (This typically only happens in PH 3 months after starting a new medication.) The last time I had started a new medication at this point was a well over a year ago.

There are handfuls of puzzle pieces to my diagnosis that don't add up.

The PH progressed at an alarming rate, which isn't impossible, but very unusual for PH. It takes about 2-3 years for most patients to receive a diagnosis of PH, which unfortunately, allows the disease progress to a later stage. (Sometimes the progression of the disease can be escalated by something like pregnancy.) For me, I developed symptoms in a matter of months after starting this medication and a plane ride. Specialists tried to convince me that I have had it for years, but before June of 2013 I was working at the busiest bakery in town lifting heavy boxes up a rather large stair case- something that would be very difficult for someone to do with PH. At one point in my life I was carrying drum kits and guitars, and loading them into a van. I worked out regularly with no symptoms up until October 2013.

A PH specialist that I was seeing said that I may have POTs because of a few symptoms I show. My regular bloods pressure is 80/50 which is abnormally low. (My blood pressure was this low before starting PH meds, which can lower your blood pressure.) My blood pressure also drops when I stand. My heart rate increases by over 30 beats, and my o2 levels drop. So many of my symptoms overlap with other invisible diseases, making them harder to diagnosis. POTs can also be caused by a nervous system dysfunction.

One of the main causes for PH is a congenital heart defect. Upon diagnosis I learned I had a valve open in the chamber of my heart, and that the hole was rather large. I've heard conflicting opinions over and over again about this hole. It has been so confusing to hear one specialist say I will die quicker without oxygen use, while another one said I am fine because of that hole. A cardiologist said that if I got the hole repaired there is a chance I could be in better shape. I was never given a cardiologist after my diagnosis despite the abnormalities that appeared in various tests. Despite the hole in my heart, I was still given the diagnosis of having an 'idiopathic' disease.

I also had a fistful of other unexplained symptoms that are typically tied to sympathetic nervous system. I remember laying in the hospital bed after my right heart cath telling the doctors that my feet and arms were tingling. They told me that it had nothing to do with PH and that it was fine.

I've had night sweats, muscle weakness, the inability to regulate my temperature, intolerance to the heat and cold, nerve pain in my thighs and head, along with having my blood pressure drop upon standing. My blood work has also came back irregular for  markers for various things, but again, the word 'idiopathic' stops any specialists from trying to put together these puzzle pieces. I sometimes feel like they see me like a lost cause.

In March of this year I finally saw an rheumatologist. She took a look at the blood work done from the very day I was hospitalized and diagnosed, nearly two and half years ago. She laughed a little and pointed something out to the student doctor. She then told me that I wasn't crazy, that something was definitely abnormal. I had a high amount of white blood cells. She told me that she had a gut feeling that I had Lupus, and I was immediately put on some heavy duty imunno-suppressants. I received a phone call three weeks later informing me that I didn't have Lupus, or any other rheumatoid diseases. I am still on the Lupus medication, just to see what happens.

I have come into my appointments with research papers, along with written timelines and events leading up to my diagnosis. I bring up my strange reaction to do different medications. They are a little bread crumbs leading me to somewhere. Unfortunately, my desire to be proactive in my treatment has not been with an open mind from the specialists I have seen. Several times I have heard "you have idiopathic PH, sweetheart" as if that is a concrete diagnosis.

The word idiopathic relates to any disease that arises spontaneously with an unknown cause. To have it treated as if it a concrete diagnosis that answers to all of my symptoms, and has been diagnosed through blood work, is beyond frustrating.

Thankfully, my family doctor is a wonderful and patient man. From the very start of my diagnosis he has said that he would do whatever he could to help me. I recently went to him with my concerns, and he is happily referring me to several different specialists. He even suggested that I research different specialists to see what one would have the most interest in my case. I have been referred to other specialists before, I think they feel scared to over step over any other specialist's work and diagnosis.

It is discouraging, but I will keep trying until...

Truthfully, a large part of me is frightened that my PH will not be managed in an optimum way if my other issues are not addressed. It is clear to me that they over lap, and it is terrifying to think I could get sicker because of this. I hate thinking that I might get sicker, or even worse, all because no one will listen to me. All because they see the word 'idiopathic.' That giant question mark has made it impossible to get a real second opinion, or another diagnosis from another specialist.

I have already had one specialist tell me that I am "not crazy." I try to stay hopeful that someday my puzzle pieces will make sense to a specialist, and that I can get better treatment to help me stay here and healthy for as long as possible.

PHighter Friday: Astrid

 Hi everyone!

Hi everyone! My name is Astrid and I am 22 years old. I’m a newly diagnosed IPAH patient. I was diagnosed about 3.5 months ago and I live in The Netherlands.

It all started in 2014. I was passing out while I was doing sports. I did not think about it much until the

beginning of January 2015. I started to get extremely tired and I could not walk for 100 meters without gasping for air or getting dizzy. I also had palpitations. I could not work (I work as a registered nurse at a local hospital) and I was too tired to follow my classes (I was doing a bachelor in anesthetic and ambulance studies). I had no idea what was happening to me. The only thing I knew was that I needed to see the family doctor as soon as possible to get it checked. She thought I had some viral infection and I went home.

My symptoms were worsening and I felt so bad that I went to the ER on April the 17th. After some tests at the ER they were thinking about Pulmonary Hypertension. I knew what it was because of my medical background and I was so shocked! That evening I had an emergency RHC and my mean pressures were in the 120’s. My heart was exhausted and I was in NYHA (WHO) class 4. I went immediately to ICU and started with Veletri. I was there for 8 days. My brother returned immediately from his travels in Venezuela, I knew this was serious.

They were increasing the dosage of Veletri day by day. It was a pain in the butt because of the side effects. However, the nurses and doctors at the ICU were very nice and attentive, which made everything a bit better. While I was laying over there I always stayed positive. When I heard I would be attached to a pump 24/7 for the next years or even my whole life it was hard but I was thinking that if I could live a normal life with this pump I couldn´t care less. They told me they did not know if the medication would work out for me because of my high mean pressure, so there were some serious doubts about my recovery. However, I never lost hope. In the back of my mind, I knew my story was going to be a different one. After 2.5 weeks I was discharged from the hospital and went home to my parents house.

First time riding my bike again since diagnosis!

The first week of being home I was walking the dog again. THANKGOD! I was not out of breath anymore or neither feeling my heart pounding out of my chest. Day by day it got better. The second week after being discharged I was even riding my bike again. I live in The Netherlands so we do everything by bike over here. It was so weird and above all expectations. Every time I was riding my bike again I had tears of joy. I went back to my own apartment and started living on my own again.

In July I started working again at the local hospital. Because of the things I went through I started to realize even more what being a nurse is all about. The negative thing was that I decided to quit my studies because of some serious delays. My life is pretty normal right now besides of having infusion therapy 24/7. A couple of weeks ago I had my first echo since the diagnosis. My pressures downsized enormously and my heart was in pretty good condition again. It does not seem to suffer from the pressure anymore. My pressures are still above the normal range though. I went from class 4 to 1+.  I hope to get more better in time. I also started to eat cleaner and do (basic) work-outs. It is still impossible for me to do running but that is fine with me.

This week I had some great news. My PH specialist wants me to switch from the infusion therapy to oral medication. This process takes a couple of months and I just started. I need to have a lot of patience and faith. I hope it all goes well. I am a little nervous, but even more excited to have a look at my future results.

3 months after diagnosis

What I want to say to all (new) PH patients: have hope and faith! There are better days to come. On some days life is all about dreams, hopes and visions for the future. But there are some days where life is just about putting one foot in front of the other and that is okay. We need to remember that researchers are doing the best they can for us day by day. I have noticed that new medications are being introduced every year. Hopefully these new coming medications will keep us stable.

At last I want to thank my parents, my brother, my boyfriend, my friends, my PH nurse Nicole and my PH specialist Dr. Heijdra. Without them I would not feel as good as I am doing today.

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: Karen

My journey with IPAH started nearly 27 years ago. I was about 4 months pregnant and went to the doctors as I was feeling breathless. This was my third baby. I never had breathlessness with my other two pregnancies.

I was given two inhalers for asthma.I started taking them regularly. I was then at Antenatal Clinic, and the lady I was seeing there told me that I was very breathless. I said "yes, I have asthma." She asked me when was I told that I had asthma. I said about three weeks ago..she asked if I would mind going for an ecg. At this time I was also going through a messy divorce, and my husband at the time had the girls. I was living with a new partner. I had the ecg and went home.

I shortly recieved a phone call telling me to pack a bag and get straight to hospital. I went to the hospital so scared out of my mind. I was then seen by a doctor who said he thought that I had blood clots on the lungs, but would test me in the morning. I was admitted for two weeks to get stabilized. The following morning I was seen by two doctors and had lung function tests. I couldn't have X ray due to being pregnant. They had a meeting and then came to see me in the afternoon. They suspected blood clots on the lungs and told me to consider a termination. I couldn't go through with that because I had lost a baby at 6 months prior between my two girls. I went ahead with the pregnancy. I had to give my self three injections of Heperin daily. The baby was born with no complications.

This is me and my husband , daughter Sarah is the middle daughter her hubby and my grandsons.

It was not until two days after that the vicar came in to read me the last rites. My blood pressure had plummeted through the floor. I was on constant oxygen and bed rest. Some how I must of had someone looking over me because I made it. Once my little baby was three months old I went to a chest hospital in London, England. I had numerous tests and was told that I had PH. I was put on Warfarin, Propafenone, and Nicardipine. No real PH drugs were available at the time. I was seen by this hospital every three months. During this time with PH I had many bad days, and with a new baby and two daughters it was very hard work. I had alot of support from my friends and family, which is good if you have PH. I got very depressed and shut my self away for three months of not going out etc. One day I got up and said to my self "Come on Karen, get out of this rut" and to this day I havent looked back, now only forward.

I was sent to Hammersmith hospital 14 years ago and that was the best thing ever. I was admitted for a week for full lots of tests and met some wonderful people. All the doctors and nurses in the Hammersmith PH team are fantastic. With the right medications like Bosentan, Sildenafil, Digoxin, and 8 liters of oxygen over night I have been good . I have recently been put on Macitentan and its brilliant so far. I had previously suffered with severe SVT's which is where I was taken to the local A&E and given Adenisone to stop them. Once the drug never worked and I had to have a cardio version. At hammersmith they sorted this problem out with having a heart ablation twice.

This is my daughter Leanne of 26 years old. 

My daughter is nearly 27 and a beautiful young lady I must say.I have two grandsons who keep me going, and a wonderful husband who gives me such a lot of support and does a lot for me. I had a nasty partner before him.


I am quoted at being class 3 IPAH. I was told 20 years ago that I had 5 years left to live. No one can tell you how long you got to live. I have learnt that having IPAH you have to pace your self in life. This may make people laugh but at times I get very scared, especially at night time. I think my heart is not going. So I sit up and get my stethescope out and listen to my heart. My husband asks me "what you doing?" I say I am making sure my heart is going lol. I know its mad, but I like to hear it. I also check my oxygen sats too. You have to remain positive and be strong during your PH journey.

PHighter Friday: Susie Alvarez

Susie Alvarez

23 year Pulmonary Hypertension long term survivor

Diagnosed idiopathic and familial, Stage 3

Support group co leader, PH support group of Greater L.A.

Board of Directors, Help Mates 4 Hope, Los Angeles

State of California employee, 31 years

I was born and raised in Pomona, California. I am the oldest of three sisters and the aunt to two nephews. As a child, I was also a champion baton twirler, winning numerous awards.  I graduated college and moved to Los Angeles in 1983 and started a 30 year career with the State of California located in Culver City.  My life was full with family events, work projects, vacations, and much fun and promise.  

While attending additional college and working full time, I began to notice I was short of breath during my busy day.  I continued to keep up with my daily work routine but had to drop my college track and dance classes as I could just not keep up with the physical activity the classes required.  I continued to experience shortness of breath, fatigue, and generally feel out of shape.   I made a mental note to visit my Doctor after the upcoming Fourth of July holiday which I was looking forward to away from work.

On June 30, 1992, my life was forever changed. I cashed my paycheck after work and began the drive home.  Without warning, I had a crushing pain in my chest as though someone was sitting on it.  I pulled over on a very busy city street during heavy afternoon traffic and parked right under the “no parking anytime” sign which caught the attention of a parking officer. She approached my car and I suddenly grabbed her by the chest and gasped “I can’t breathe!”   I saw the urgent look on her face as she called paramedics, who arrived and took me to Cedars Sinai hospital in Los Angeles.   During the week I was there, I had several tests done every day to find out why I was so sick.  I remember coming in and out of consciousness and not knowing what day or time it was.

On the morning of Sat July 4th 1992, the pulmonologist visited my room. He quickly sat down and said

“We found out what’s wrong with you. You have Pulmonary Hypertension which is a fatal lung condition for which there is no cure.

Go home, get your affairs in order, and the best we can tell you is that you have 5 years to live. We will send you home with a prescription to help you breathe but your illness will progressively get worse. Eventually you will need a lung transplant.” 

Then he left the room. 

My mind went into a fog, I felt so confused and very scared. I was hyperventilating through my tears.  My family was located miles away and I did not have the strength to pick up the phone and call them.  I was alone in a cold hospital room and had just received the most devastating news someone could ever hear.    I remember grabbing the nurse by the arm, crying all night, and the only question in my head was

********************WHAT IS PULMONARY HYPERTENSION AND HOW DID I GET IT??**********************

At the time of my diagnosis, the internet and cell phones were not widely available to search for resources. I had no written information or source of support to find out more information about this medical disease that was to end my life.  For 3 years, I did not know anyone who had PH or who knew what PH was. I continued to work but always the question stayed in my mind. My Doctor’s words were correct.  My symptoms of PH progressively worsened, I was short of breath, fatigued, dizzy most of the day and was told I was being referred to a PH Dr. Center for further treatment.

My life was forever changed again when I visited the PH center in Torrance, Ca.   I met a cardiologist who knew what PH was and how to treat it.   He explained I immediately needed an IV line placed in my chest with medicine attached to a pump that would open up the arteries in my lungs to help me breathe.  I would have to wear this pump and medication 24 hours a day which also required care of the IV site and mixing medication that would be attached to the pump.

In March 1996, I was placed on Flolan (epoprostenol), the only FDA approved therapy available to patients at that time, and remained on this therapy for 11 1/2 years. With continued improvement, I participated in several clinical trials for oral therapies to treat PH while I remained on the IV line therapy. I began attending support groups, PHA’s International Conference, and local events in my area for people who lived with PH where I learned more about this medical condition.

I was also referred to UCLA Medical Center, Los Angeles, evaluated and placed on the transplant list for a double lung transplant. I remained on the transplant list for 13 months.  During that time I moved to the top of the list and one evening I was called in for a transplant. The surgeon informed me the donated lungs were not viable and I was sent home. Shortly thereafter, I was removed from the transplant list by my PH doctor who informed me the IV medicine was improving my quality of life.  

Finally, on Oct. 31^st 2007, I was taken off the epoprostenol pump therapy and walked out of the hospital – PUMP FREE!

Before this date, I transitioned to oral therapies Letaris (abrisentan), Adcirca (tadalafil)  and Coumadin and it felt so good to be free of the pump.   

During my journey and with the improvement in my quality of life, I continued to work full time and began traveling both in and out of the U.S. for work and vacations.   In 2011, I volunteered to serve as the co-leader for the PH Support Group of Greater Los Angeles

In June 2013 our group suffered the tragic loss of our co-leader and PH patient, Angel Alcala.   With the addition of a new and energetic co-leader, Joannie Yuille, the Greater L.A. PH support group began an annual fundraising event, A Message of Hope”, in Angel’s honor.

We both enjoy engaging the PH community and provide a comfortable place for hope and support. We hold six meeting a year, one special event each May, and our first annual PHA Chapter sponsored o2 Breathe Walk in the fall.   Our support group has enjoyed speakers from Pulmonary Rehabilitation, the Red Cross, nutritionists, creative writing, networking for caregivers, occupational therapists, musicians, and much more.

I also serve on the Board of Directors for Help Mates for Hope, a non-profit organization based in Los Angeles and committed to filling in the gaps in foster children’s lives.  Our group has been awarded grants from the Ronald McDonald house charities, local businesses, the Los Angeles Police Department, The California Highway Patrol, and many others’ who sponsor our events to provide foster children education, clothing, and a place to learn and grow. Visit us at www.helpmates4hope.org or on our Facebook page.  

I started attending Zumba dance classes in 2012 after consulting with my doctor for a safe and fun form of exercise.  I feel a

“shot of happy” when I feel the music and dance.  I also enjoy spending time with my canine kid, Mimi, a Pomeranian-Chihuahua

who takes walks with me several times a week for exercise.

In 2012, I attended PHA’s Congressional luncheon at the Capitol in Washington D.C. to ask my senators; to co-sponsor the Pulmonary Hypertension Research and Diagnosis Act (H.R. 2073).  We also taught them the importance of early diagnosis with the campaign, Sometimes It’s PH.  

After being married and divorced in the 1980’s, I have lived alone the entire time I have been diagnosed and felt this was my destiny to live alone with this rare disease.     In 2012 I volunteered to assist at the registration desk at the PHA International Conference.  I met another co-leader, Perry Mamigonian, from Fresno CA. We chatted about living with PH and the invaluable resources the PH Association and Conference provides to patients today.   Perry was also a part of the group of patients who attended the 2012 Congressional Luncheon.     In 2011, Perry initiated a campaign in Ca. among support group leaders in his state for a state sponsored proclamation recognizing November as PH Awareness month which he received in 2011.   We attended several PH events

including the 1^st annual PHA Chapter o2 Breathe Gala in San Francisco, Ca.  As PHriends, we discovered we had many ideals and life goals in common and began dating in 2013.  This has been the most significant event of my PH journey to find a partner who loves me!

I’m often asked how? and why I do so much for PHA?  My answer is that I was once a newly diagnosed patient, living alone, and so afraid of dying without knowing what PH is.  I’ve survived beyond the five year timeline I was give over 20 years ago.  My journey was not an easy one by any means.  As a long term survivor, every day is a blessing for me.  The loss of close PHriends to this disease

and the hope I bring to newly diagnosed patients who I meet keep me motivated to work towards awareness, support, and hope for a cure. 

Please reach out to me at:

Ph:  310-938-7930

Email:  batterygrl@yahoo.com

Pinterest:  PH-Greater L.A. Support Group  

Twitter: @batterygrl

Facebook: Greater L.A. PH Support Group

PHighter Friday: Kimberlee

I was diagnosed in 2009 with idiopathic pulmonary hypertension at the age of 30. I believe I started experiencing symptoms six months to a year prior: extreme shortness of breath, syncope, chest pain, dizziness, fatigue, and cough. So, I scheduled an appointment with my primary care physician who then referred me to a pulmonologist. The specialist did electrocardiograms and pulmonary function tests, and concluded that I had exercise-induced asthma.

I knew it had to be something else because my mother is a severe asthmatic and commented, “I do not experience what you do on my worst days.” I continued to seek medical attention from hospitals and physicians, but was always told it was asthma, so use inhalers and lose weight - later it was noted the weight gain was from fluid retention. I was also told that I was fine, and what I was experiencing was all in my mind. And, finally, I was told that I must be using illegal drugs because of the symptoms (I never used illegal drugs or smoked.). I thought I was going crazy because I knew I was sick, but the medical community was of no help to me — I was dumbfounded. I continued to pray and hope whatever was wrong would be revealed soon.

Each day I went to work, I had a rough time with my patients because I could not perform my duties due to my limited mobility and declining health.  On one particular day I was very symptomatic and briefly fainted, so my best friend who was also working and another nurse took my vital signs, which were normal with the exception of my oxygen saturation and heart rate. I determined I had to go home because I was very weak, so we immediately notified the Charge Nurse, and my friend took me to my car; she offered to take me home, but I insisted I could do it.

I drove to my parents’ house because I knew I could not make it up the stairs to my apartment and I wanted my mom. As soon as I arrived I immediately told my parents what happened, so they called 911. I was taken to the emergency room by ambulance and tests were periodically done, but doctors did not believe I had a serious condition, and the hospital wanted to release me. My mother immediately spoke on my behalf for them to transfer me to the hospital of her choosing and they complied with my mother’s demand. Upon arrival I became unresponsive and tests were urgently performed, including various x-rays, echocardiogram and right heart catherization, which lead to the diagnosis of pulmonary hypertension by two physicians who are also pulmonary hypertension specialists in separate facilities.

My family was told that if the first hospital had released me, my chances of survival were low because I was at stage 4 pulmonary hypertension. Phenomenally, I survived, and once I became responsive, I was told the news that ultimately changed my life forever. “You have pulmonary hypertension.”  I was clueless on what this meant for my future.

I am now living a “new normal.” I currently am on combination therapy: treprostinil (continuously through a central venous catheter and a portable infusion pump), ambrisentan, riociguat, and calcium channel blocker. I also use several other medications to help ease the daily side effects of the PH medications. I used supplemental oxygen 24/7 at the beginning; then after doing pulmonary rehabilitation for three months, I was able to switch to during exertion or as needed. After two more separate admissions to the pulmonary rehabilitation program, I am fortunate that my activity tolerance is increasing.

Most importantly, I am determined not to allow PH to stop me from pursing my educational goal.  If I give PH the power, then I lose respect for myself. I must be my own advocate and cheerleader.

Also, I lead four support groups because I believe no one should face this journey alone. In addition, I participate in PHA events: I have spoken at PHA’s Congressional Luncheon and two of PHA’s International PH Conferences. I also advocate for those with medical disabilities, speak at community events and at my church.

Additionally, I am a member of the Social Media Advisory Board for PHA, and part of PHA’s Generation Hope, and a mentor for United Therapeutics’ PH peer network program. I am also the co-founder of a very active patient-led PH group on Facebook, PH Family, a growing global forum since 2010 for patients and caregivers to voice questions and concerns about this life threatening disease and to support each other.    

Pulmonary hypertension has taught me to live life; I am more appreciative of my quality of life than the quantity. I am blessed to have loving parents because without them and my faith, I would not be able to overcome the daily challenges I face. This is my journey and I must be a testimony, so that others will see that there is life after being diagnosed with a chronic illness.

As a PH community we must stand together and be “empowered by hope.” Every day I want there to be a medical cure for this rare disease, but until there is, I must cherish my moments with my family, friends, and church family because they are a wonderful support system. Pulmonary hypertension has changed my life drastically, but I am determined to ensure it does not negatively impact my life entirely.  I know what is important to me; therefore, I will not allow PH to have power over my destiny.

My next goal is to return to the working world and have the most important titles: “Mrs.” and “Mommy.” My journey is not yet complete — to be continued!

My contact: livingwithph@gmail.com

PHighter Friday: Carole

Like most people with PH my journey began without my noticing it and it gradually built until my old life was no more, a new one was to begin.

After going for a walk with my husband I noticed that my feet did not want to climb the steps, they kept hitting the edges, it was just too hard to pull them up all the way to the top.  I began to find it difficult to walk up the incline to our house.  I felt dizzy and sick and my legs were heavy.

One day after walking through the woods it just got too hard walk the the incline back to my house.  Time for specialist advice.  I was fortunate enough to meet with a doctor who had heard of, though not seen, PH.  He advised my husband to take me home, back to the UK, we were living in Spain at that time. He said I had maybe six weeks to live!!!!

The despair hit me like a brick wall, and, of course, we Googled PH.  Not a good plan at all.  On the journey back home I cried because I could not dress myself!,  looking back I can't believe the despair and hopelessness that hit me.

I was fortunate enough to be met at the Royal Hallamshire  by my A Team, my angels.  I was told not to get out of bed, they feared a heart attack as the right side of my heart was five times the norm.  I was told I had many tests to get through but would be pushed to them all. I must not walk! I was immediately given a cannula in my hand and attached to a drip pumping  iloprost into my body at two minute intervals.  Then the tears began, too many to mention but I remember the professor sitting at the side of my bed and telling me I would need a Hickman line inserted into my body that would enable me to get out of bed and carry on with life as best I could.

Line inserted I went home after five weeks to begin a new phase of my life.  It was hard, the things I could do before I could not now do.  My husband  took over the roll of housekeeper,  he also took over the roll of nurse.  He changed my Hickman pump daily and made sure it was kept clean.  My fingers didn't seem to want to work any more and my brain had turned to mush! I remember one day thinking I would vac the house before my husband came back from the shops and the despair I felt when I realized I could not even push the vac around.  Then came a year of adjustment and yes, I will admit of self pity.  Why me, what life did I now have!  It was hard, so hard but after a year I was given more tests after one of my six weekly visits to my hospital and how can I ever forget the jubilation that I was to see that day.

My bed was crowded around with all my PH team. Beams were to be seen by all.  My specialist doctor was all but jumping with excitement when I was told that the iloprost had worked!  My heart was how the size of a normal persons and my right heart catheter showed it was now 38!  Now a new journey began, one without the Hickman line and one with oral medicines, ambrisenten and sildenifil.

It took three months for the change over to be completed and for my new life to begin.  Slowly my body adjusted to the new medication and slowly I began to climb out of the pit of despair I had been in.  Small steps, baby steps at first.  I was scared but began a regime of walking further each day, always with someone at my side as the fear of dizziness of  collapsing was with me but I persevered,  gradually I began to feel stronger, was able to do more, was able to help in the house, able to shop on my own, able to be my own person again.

Recently I walked my personal best on my six minute walk test of  720 metres.  Some days I can walk eight miles, though in fairness five is the norm, some days I can't do much, hence the roller coaster ride we are on.  At last I had joined the people who were responding, who were living with ph and not letting it beat them.

I remember at the beginning of this PH journey looking out of the window and seeing people walking, just walking and giving it no thought.  How envious I was of them and thought it would never again be me, now though it is.  I am not saying I don't get bad days, we all do but the good far outweigh the bad.  My mobility scooter has not been used very much in the last two years, it sits in the garage and sometimes, though rarely now it gets used.  Most times my legs do the walking!

My advice would be to push yourself a little more each day.  Don't set out to do a marathon, take small steps and find what makes you comfortable.  Listen to your body, if it says slow down then do it, if it says " I can do a little more" then do a little more.

Remember PH medications are changing all the time, research is going strong.  There will be a cure  for this and I believe it will be in my lifetime.  I trial anything I am asked to trial.  I want to be part of the team that finds this cure and beats it!

Stay strong in heart and spirit.  When the bad days hit remember it's a roller coaster, the good days will follow.  Remember we are a PH family and we have friends here who can relate to everything we are going through,  my journey began in a wheelchair but I have fought to use my body again.  Fight, fight , fight and keep a good spirit within  you, believe in yourself.  Most of all remember you are not alone, there are people here who KNOW exactly how you are feeling. 

You walk with friends. Xxxxxx