My Story

By Paul Percival

My Story
by Paul Percival

I was born in Adelaide, South Australia on August 8, 1955. Grew up in a small country town in New South Wales called Tamworth, which is now known as Australia’s Country Music Capital. We moved to Sydney in 1970 and I completed my third year at High School there. In 1971 I started an apprenticeship as a bricklayer and over the next few years applied myself to that.

In 1973 I followed my parents to Darwin, Northern Territory, Australia, and in April of the following year my father died. He’d had a heart attack the preceding October and at the time of his death his heart just stopped beating. We did not then know anything about Primary Pulmonary Hypertension. However, events unfolding later would indicate that he actually had PPH and his heart just wore out. He was 67 when he died and I was 19.

In October 1974 I married my first wife, and in July 1976 our daughter Natasha was born with a hole in her heart. This was not such a big deal, or so we were told, as one in about 200 children is born so, and the hole usually closes itself off by school age. We lived in Darwin and far away from many modern medical facilities, therefore a pediatric cardiologist from Adelaide would fly to Darwin once a year to examine cases like Natasha and make sure things were okay. Our other two children were Rachel and Jarrod, born in 1978 and 1980.

In 1981, on one of those visits from the cardiologist, it was told to us that Natasha’s hole wasn’t getting any better and in fact she would possibly need an operation very soon. We then drove down to Melbourne, Victoria (something like driving from LA to NYC) and had her seen by the best surgeons we had. They performed two cardio catheters and a lung biopsy, which was sent then to London for analysis.

We were then told she had PPH and that there wasn’t anything they could do, but that they would monitor her until she died. Needless to say we were devastated and we searched everywhere for answers, to no avail. Natasha wasted away before our eyes and died in January 1983. One of the hardest things for me to watch was that little white coffin lowered into the ground. I consoled myself with knowing that she literally died in my arms and I knew there wasn’t anything that we could have done more to keep her alive. There was no Flolan then. We were told that this disease was strictly random and that they did not know what caused it at all and that the chance of anyone else in the family getting it was astronomically small. There was no delving into family histories at all.

My answer in dealing with life’s blow was to throw myself into work. The marriage failed in January 1988. I became a single dad of our two children, then aged seven and nine. I actually retired from full time work and did only odd jobs for a few years so that I could always be there for my children. Several years later both children went to live with their mother.

The empire I had built myself had by this time all gone, and I was sort of floating around without a great deal of desire to do or achieve anything. I married a long-time friend in 1991. I had built a multi-million dollar construction company from scratch in my time when I was trying to blot out Natasha’s death. I later sold that company, put the money into real estate, over extended myself and went bust as a recession hit the economy. That’s life. I separated from my second wife after about six weeks and we divorced the following year. I remained single for a long time after that.

In 1994 I decided to get motivated yet again and formed a partnership with a couple of people to go building homes. In 1996 and early 1997 my two partners decided to clean the bank account and take over the business and squeeze me out. This caused a lot of stress, as you can imagine. Now I do not know if the stress at that time triggered the beginning of my disease but over the next few months my condition got worse and worse. In May I got extremely ill. I lost 45 lb. in that month and felt like I had been hit with a freight train.

I finally went to a doctor who did all the blood tests and so forth. I was given anti-depressants. The stress of several court cases I had against my former partners caused me to be hardly able to breathe at every court appearance. I therefore put a hold on the court cases as I could feel they were literally killing me with stress. Eventually the doctor referred me to a specialist. I asked the doctor straight out why and what did he think was wrong. He said, "I think we have to look for tumors or cancer."

I saw the specialist (in Tropic medicine, would you believe.) He was, once he got past the idea that I was a hypochondriac, a very good doctor indeed. He did more blood tests and also did ultra-sounds and a test for which I forget the correct name but it takes thin slices of the brain and creates a picture. During this time I had two strokes that they first thought were migraines and later conceded were strokes.

On December 16, a few days before I was due to fly here to the US to meet a special person, the doctor informed me of what I had. Needless to say I went into shock. I had nursed my daughter through this disease and well knew its prognosis. I arrived at Washington National Airport Christmas morning at 9 AM 1997, as a dying man in shock.

I met that special person there only to have to sit her down and tell her what my situation was. It was a very trying time for me to say the least. I had just turned 42 and it seemed doubtful I would see even 44. Yes the sorrow and self pity came, the ‘why me?’ syndrome, and it took me a long time with much anguish indeed to come to terms with where I was in life.

Thankfully that special person refused to believe that the disease was untreatable as I had told her and when we got to her place she started doing research on the Net. She found among many other things the phassociation page and the discussion board. Well I posted my tale there and I got several replies. One of those replies pointed me eventually to Dr. Sean Gaine who was then at the University of Maryland, Baltimore.

I rang Dr. Gaine’s receptionist and made an appointment. He examined me and sat with me for well over two hours discussing the disease and its treatments and what Flolan may be able to do for me and so forth. FINALLY, I could see light at the end of a very dark tunnel and I was overjoyed.

I reasoned this way -- if Flolan was available here in the US and had been approved by the FDA (1996) then surely it would be available in Australia, as in many areas we lead the world. I did not then know what a fool I was, and the dramas that would befall me when I returned home.

Now I have always worked outside, even when running the construction company, and was incredibly fit before I became ill. This was to stand me in good stead and may have given me enough stamina to endure what I did.

I had organized before I knew what it was that ailed me that on this trip to the US I would also take the train right across Canada. This I duly did, much to the dismay of that special person who had by that stage become a sort of fiancé. She was to come out to Australia later in 1998 and we were to marry once I had things sorted out medically there.

I caught a train to NYC and spent six days there with another net friend and did the tourist bit in late February 1998. At this stage I was surviving on sheer will power as I was getting worse by the day with the extra strain I was putting on myself. From NYC I went to St. Johns, New Brunswick and right across Canada on the train. The one highlight of that whole trip was meeting three long-time net friends in Saskatoon and spending time with them. These wonderful people still host my homepage and I love them dearly.

By the time I got down to Seattle, Washington I had burst some blood vessels in my lungs and was coughing up some serious blood. I flew back to Virginia and rested at my future wife’s place so as to be able to fly home to Australia.

I flew back to Darwin at the end of March 1998 and was referred to Dr. MacDonald at St. Vincent’s Hospital in Sydney. I arrived there early April and they did more scans, x-rays and a right heart catheter. They tested me for nitric oxide and Flolan challenge, as they called it. I was told that my cardiac output was less than 3 and that I had no noticeable response to Flolan and that 100 people out of 100 people with my symptoms would be dead within two years. I was once again devastated.

Dr. Gaine had given me his email address and asked me to keep in contact with him. I sent him an email explaining the tests and the results and he wrote straight back and told me that Flolan is a long-term treatment and the fact that I had shown no response to it initially was nothing and that I should demand the drug. This I did and was able to present the staff there with clinical trial results that Dr. Gaine supplied to me to back-up the request. The doctors there started me on a small dose of Flolan. I almost immediately improved that much that my sister and brother-in-law went from discussing funeral arrangements with me to how well I was looking.

Australian Government policy makes each hospital pay for the drugs, and the Australian Government has never approved Flolan as a registered drug. Every case needs to be approved by the health minister of that state and paid for out of the hospital’s operating capital. They told me they would give me the drug only as a bridge to a heart and lung transplant. Many clinical studies which Dr. Gaine forwarded to me to back-up the claim for treatment solely with Flolan (and not have it tied to a transplant) fell on deaf ears. The doctors would agree with me personally, but simple economics made the hospital’s policy. Finally I came to understand the only way I could get treated as I should be treated was to return to the US.

I was able at this time to contact Olsten here in the US and they were extremely helpful. They gave me the criteria they had for helping in cases like mine where no insurance was available. That I was already on Flolan when I returned to the US swayed the scales in my favor.

I had to have a proper hickman catheter fitted before they would supply me any drug. This was to replace the temporary line that had been put in back in Australia. This I duly had done at MCV in December 1998. At that stage Olsten began shipping the drug and all ancillaries to me at whatever dose my doctors prescribed. I am indeed indebted to Olsten for this and their ongoing support. The requirement is that I must remain in the US. They will not ship the drug outside the US for anyone. Those are their rules.

When I climbed onto that plane out of Sydney back in September 1998 I knew I would never see my homeland again and very likely would never see any of my family again, as costs would prohibit them from visiting me in the US. Now I love Australia with a passion, but I love life far more. I have lived here in the US since then with net friends who have helped me survive here. That special person and I were finally married on October 30, 1999 at Lake Tahoe. Nevada.

This story has huge gaps in it but I wanted to convey just some of what my position is and was and to show that with some determination we can overcome almost anything and beat this disease. During my lifetime so far I have led a blessed life, really. I have been able to do all that I wanted to do and everything basically that I have set my mind to. I have lived and worked in some of the most beautiful parts of Australia. I have seen the sun rise over Ayers Rock. I have worked at the ocean’s edge on the eighth wonder of the world, Australia's Great Barrier Reef.

I really am blessed to have lived as I have done. I have two wonderful children who are growing up to be quite reasonable adults, and had the love of a special child whom I lost when she was very young. I have met many wonderful and interesting people both in Australia and in my travels overseas and amongst them have found some true "gems", people who have become very special friends to me. Now as I continue to fight my illness and win on foreign shores I still search and reach and strive to be the very best that I can be for me and to be a useful and productive member of society.

Thank you for reading my story, folks. May we all try and may we all succeed in overcoming this wretched disease. My best wishes are with you all.

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We feel strongly that free and open discussion regarding PH will assist in the generation and the refinement of new and existing ideas. To that end, we provide the opportunity for anyone from the medical and patient communities to submit editorials, letters to the editor or short articles on a PH-related topic. We will be encouraging opposing points of view. Areas may include treatments, causes, advocacy, disability, etc.

PHCentral is committed to the expression of diverse opinions from members of the PH Community.

We feel strongly that free and open discussion regarding PH will assist in the generation and the refinement of new and existing ideas. To that end, we provide the opportunity for anyone from the medical and patient communities to submit editorials, letters to the editor or short articles on a PH-related topic. We will be encouraging opposing points of view. Areas may include treatments, causes, advocacy, disability, etc.