What little I understand about this disease, I have learned from my fellow travellers in the land of Flolan.

It has been over four years since I was diagnosed with pulmonary hypertension. December 3, 1999. I was fortunate in that I had only been symptomatic for about a month, but within that small window, I had lost almost all functionality. I was unable to brush my teeth without pausing for breath. I had to plan my showers, arranging the soap and shampoo and my razor within easy reach, so that I would not have to pause between actions. I gained weight as I had never done before except during pregnancy. My ability to run disappeared almost overnight: from training for a marathon, I became unable to walk even a city block without pausing for breath every few meters.

Every story of PH diagnosis seems fraught with mistaken diagnoses, hurried physicians and ignorance. I was fortunate that my brother was a third-year surgery resident. He knew little, but he also knew what he didn't know. After hearing my symptoms and listening to my stories of my ER visits, he told me to come to Virginia, to his teaching hospital. I had been given dx ranging from depression to asthma to alcoholism, from my three ER visits. Yes--I was depressed from being told that my symptoms were all in my head. Yes, I had symptoms of asthma, since I could not walk up a short flight of stairs without feeling as though I was going to faint. Yes. I could easily have become an alcoholic, since I was being told that all my symptoms were imagined, that I was misusing the ER's services.

So, I drove to Virginia with my mother. Within 24 hours, I had an accurate diagnosis and an inaccurate prognosis from the pulmonologist who diagnosed me. I had never heard of PH except in the context of diet drug usage. Certainly not for healthy female athletes...

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Hello Everyone

My diary responsibilities are making me more aware than previously of my obligations to a world beyond that of my own lived experience of PH.

My husband, Stefan, a mathematician, is as involved with me in treating PH as any of my doctors.  My PH specialist can snap his fingers and have two weeks off, but Stefan is here, by my side for the duration.

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The day began early today: I was woken by the thrashers which are nesting in an enormous cholla cactus just a few feet from the edge of our deck. The babies were born a short few weeks ago, four of them, tiny and fluffy and delicate in their appearance, so frail-looking. The adult thrashers are fierce animals, very territorial and assertive in defending their homestead. They attack any lost packrat or squirrel or smaller bird who strays onto their land. We have seen adult thrashers pursue an unfortunate ringtail through our garden, onto the road, under creosote bushes, until the ringtail found haven under one of our cars. The thrasher half ran, half flew, chasing the ringtail even after it had left the vicinity of the nest, attcking it by swooping down and stabbing the animal with its long sharp beak. The ringtail was only deterred for a few hours, though. Later that same day, the ringtail came back, looking longingly at the food that we place outside for 'our' wild animals. This time, the thrasher was occupied finding food for the baby birds, and ringtail was allowed to eat and run.

It seems that the thrasher babies have left the nest. I went to check on them and to take some photographs (using a long lens so  did not have to approach the nest too cosely) early this afternoon, but it was empty. Much of our evening time, on the deck eating our dinner, is spent observing the animals around us in the desert. The thrashers provide some of our best entertainment. If we are correct, the adults will be back, possibly to have another brood of offspring, but we will not see the young ones very much.

We also have a roadrunner nest in a huge, leafy apple tree, again, just by our deck, only a few feet from where we eat our dinner. The male and the female roadrunners cooperated in the building of the nest--he would bring back many twigs and bits of straw in his beak, then pass them to his partner's beak. She would then add them to the already-existing structure. This process of construction lasted about a week. Now we are waiting for evidence, either seeing or hearing new baby roadrunners. These birds too are very aggressive in defending their homes, swooping down into the midst of our almost-tame doves and finches and cardinals. Unlike the thrashers, they do not pursue those who trespass, but simply frighten them away. This lasts for a few mintues only. The other birds return usually within a few minutes after the roadruner leaves.

PH: this diary seems to be almost an exercise in distracting myself from the immediate reason for writing this journal: PH.

Life is very good. My children and husband are healthy. I am happy, most of the time, though increasingly aware that my health is deteriorating in ways that perhaps are not measurable using echos or other indirect tests. Or is it?

Is It?

I have subtle symptoms: heaviness in my chest, some shortness of breath, some chest pain. None of these is severe, all are transitory. Why then this sense of foreboding? I think perhaps that I am internalising some information given to my husband and me about a year ago, by one of the PH specialists with whom we have consulted. He made the comment, an anecdotal one, that many many patients with PH did very well for about four years on Flolan, but that this four year anniversary was often then marked by a fairly sudden descent into severe PH and death unless one endured a lung transplant...

Last December was my four-year anniversary on Flolan, my four year-year anniversary of being diagnosed.

And yet, this PH specialist was not telling me that this was anything more than his experience, not a published medical opinion even. Just a personal observation, based on his patients' experiences. The emotional stress in our lives, my husband's and mine, recently has been enormous:the death of a close family member, one we loved much and the death of a friend who had PH, but not as severe as mine.

Yes, I know that stress exacerbates the symptoms of PH, but can it actually accelerate the disease process? Do the symptoms caused by stress abate, leaving me much as I was before the episode of stress? My husband and I have lived through some incarnation of hell for the last year and a half, the details are not important, but enough varying stresses that we were literally always waiting for the next disaster. After a series of medical mistakes which were gross malpractice, I nearly died about 15 months ago. Repairing my health has been a slow, incremental process and I am still not back to the health that I experienced on Flolan, until December 2002. I am on Flolan still and will be seeing my PH specialist in about a week, having an echo performed and will talk about lung transplant. What do I expect? A look of amazement that I am still alive? Always.

My PAP has never fallen below about 80mmHg since being diagnosed, even though I have generally felt well since beginning Flolan therapy. I was walking many many miles a day within a few weeks of begining Flolan. I could even envision a life in which I was alive to see all my children grow into adulthood.

I was 42 when diagnosed. Five children. I was in the middle of what was proving to be a vicious divorce. I was in hell, I thought. At least I had my health, I thought.

On a trip to Israel in September of 1999, I was walking in the hills of Jerusalem, with two friends and an Israeli guide. I was looking at the view, from where I stood, of the Wailing Wall, that place of pilgrimage for so many Jews and Christians. I realised that I was breathing heavily after the walk. I thought perhaps I was being affected by the emotions of seeing this magnificent city, with all the historic and cultural and religious significance it has for so many of the world's people. No--I returned home a few days later and tried to run, preparing for a road race. This was not to be. I could not run or even walk the distance from my car to my house without pausing for breath. Brushing my teeth was an exercise in torture, taking me fifteen minutes, because I had to pause so frequently to catch my breath.

I fortunately had health insurance. My mother, an attorney, had told me all the steps to take in order to protect my children and myself during and after the divorce. Her main admontion was that I should purchase a health insurance policy. I became symptomatic  for the first time only a few days after the policy came into effect, a coincidence of timing that I came to appreciate only after reading the stories of so many PH patients who have little or inadequate health insurance policies.

In my next entry, I will write more about my diagnosis, but for now, I want to finish today on a hopeful note, writing about one of my children. The joy that I experience when I even think of any of my children is inexpressible and any words I can find are inadequate. But I want to write about them, because my husband and my children are the defining experiences of my life. PH is not.

My oldest daughter, Kestrel, is in her first year of university. She won a four year academic scholarship and is loving university. When I was first diagnosed, four years ago, I never thought that I would be alive to see her enjoy leaving the nest. Hearing her voice on the phone, when she calls with her weekly updates, is heartbreakingly wonderful. She is attending the same university from which I graduated and has some of the same professors as I had. She is an excellent writer and I am hopeful that she will choose a field of study which will enable her to write and write and write. For her, as for me, the written word provides a therapeutic process that enables us to make some sense of our worlds

Kestrel makes me laugh. She is fiercely intelligent, with a wicked sense of humour which enables us both to laugh at PH and the ridiculous situations in which I have found myself...graveyard humour sometimes, but it helps us to deal with the awfulness of PH.

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In the last year, two of my closest friends with PH have died. Each death was avoidable.

On St. Patrick's Day, I lost the friend who had helped me through the darkest hours of living with PPH. She and I correspnded daily, at least once a day, sometimes as frequently as four or five times. She chose to limit the friendship to email and the occasional package through the post. We sent one another books, odds and ends of bizarre clothing. My last package from her contained a wildly electric blue feather boa, six feet in length. A black chiffon full length skirt and a silver top, skin-tight, with black spangles and sequins which were meant to catch the light of the disco ball...we were both in our teens during the 70s...We exchanged books, too. I introduced her to James Lee Bourke, a southern writer whose detective fiction is lush and brilliant and imbued with a sense of the aching beauty of the American south--the terrible clashes of race, the unfathomable richness of the cultures coming together. She forced me to read feminist writers whose prose made my teeth ache with gnashing. 'The Wounded Storyteller' was the one work on which we could agree--how illness changed our self-perception. She had endured in her short life 20 miscarriages. I had five live births.

She died while waiting to overcome kidney failure, at which point she could begin Flolan therapy. She and I fought about Flolan. She felt it to be an unwanted intrusion into her most private life. I knew that without it I would ahve no life, private or not. She did not tell me that she had changed her mind. All I knew was that her emails slowed. She wrote briefly, enigmatically, 'I am fine... I will write more in a few days'. She told me briefly, and without embroidery or explanation, that she was in kidney failure, but that she was fighting it and succeeding.I did not hear from her for a few day. I was annoyed, but not worried. Then, on March 17, I saw her name in my email inbox...

I opened the mail, expecting to read her usual warm greeting, and instead read a line that I could not fathom, from her husband...'I am sorry to tell you that my wife died today, at home, unexpectedly...'. I screamed. I screamed and screamed and screamed. My friend died at home, with her husband in the next room, completely unexpectedly, for no good reason. She had made up her mind to begin Flolan therapy, many months after her PH specialists told her that Flolan was her only chance to live. Those months were crucial. Her kidneys failed, her liver was enlarged and failing. I am so angry I am unable to think rationally about this. My most wonderful friend is gone. Forever. I am not a believer in any supreme being. I do not have the easy comfort of saying that my friend is in a better place. All I know is that she is gone. Her husband is bereft. The world is a lonelier place. The world is diminished without her.

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Before writing any more about my friends' deaths from PH, I want to write about PH treatments, how we are perceived by our PH specialists and the meaning of compliance. Today is hot and sunny here in the desert, the birds are playing happily in the fountain that my husband created for them in one of our gardens, a walled one which gives minimal protection to the more gentle and herbivourous of our birds and other animals--we have seen several tiny rabbits and jackrabbits crunching happily on the bits of bok choy that we left near the fountain. The finches, thrashers, cardinals and sometimes warblers and flycatchers come to visit briefly. The thrashers are bold enough to come to the doorway of our house, though this summer we have not had too many of these welcome visits...they are such wonderful animals to watch. On 'bad' PH days, I can sit on the deck with a book, my laptop and a glass of water and watch the animals for hours. Recently some small squirrels have been coming onto the deck, even when we are sitting outside. They run up the pecan tree, or onto the bird feeders, fill their cheeks to bursting and then zoom across the deck to go home and feed their offspring. I love being part of this little ecosystem, accepted by the birds and mammals...

And this has nothing to do with PH, does it? My mood has shifted now to one which will not be satisfied until I have gone for a walk to check up on 'our' newly hatched thrashers, four of them, the second round of egg-laying for this particular  thrasher couple. More later if my mood allows...being alone in the house with a laptop is great for prompting me to write regularly..in the past I begged many times for extensions of deadlines. My self-discipline is much improved...perhaps a side effect of absolutely having to be rigid in the maintenance of such aspects of PH life as preserving sterility around my central line insertion site and even the routine of mixing Flolan without becoming careless, despite the daily repetition. My children help too--often my youngest daughter,Octavia, watches me and comments...despite her age (she is 13) she is my mixing assistant whose eagle eye catches me whenever I do not spend sufficient time wiping the tops of the bottle of diluent with alcohol...

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Compliance:

This word and its implications in the world of PH were what I had meant to discuss in my last diary entry. I am a coward though.

During the course of being trained in the routines associated with Flolan, the word 'compliance' was used repeatedly as a warning to me. Any indication of lack of compliance would mean possible refusal of my request to be placed on a lung transplant waiting list.

When I was first diagnosed and subsequently told to choose 'Flolan or death', I also chose to be listed for lung transplant (at the University of Virginia). During the course of being trained in the Flolan 'alternative lifestyle' and during the process of being evaluated medically, emotionally and psychologically, for lung transplant, the words 'compliance' and 'compliant' were frequently used. I had to show myself to be compliant with all doctors' instructions in order to be granted a place on the list. So, am I compliant? What are the more subtle implications of that word, those which go beyond simple obedience to the orders of my physicians?

I take all of my pills when I am supposed to. I mix Flolan daily with as much ritualistic rigidity as is possible. My rituals go beyond the ones that I was taught by the nurse who trained me. I am not religious, at all, and perhaps my Flolan rituals are a pale substitute for a religious experience? Perform the rituals perfectly each day and perhaps the Flolan god and the Tracleer goddess will allow me to live a little longer.

So, I am compliant, but not from fear of being thrown off the transplant waiting list. I am living in fear of death from PH, as visceral and as real as any fear of the gods that our ancestors felt when they experienced the wrath of nature. By performing my religious rituals, perhaps Gaia will spare me and the earth will not open up as it did when Persephone was taken into the underworld. PH is not rational. I understand it as little as the ancients understood the floods which would destroy their crops and the lightning that would cause the burning of their villages. My rituals are only as effective as the gods and goddesses of PH allow. Appeasing them is my hope.

Compliance is a measure of my fear.

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More fear

I was diagnosed in December of 1999 after being symptomatic for only a few weeks. My youngest daughter, who is thirteen years old, has only ever known her mother with PH. The implications for Octavia--I cannot cannot cannot comprehend.

The guilt that I feel for having stolen her innocence from her, for introducing her to the adult world of life-threatening disease, sometimes overwhelms me so that I cannot feel anything beyond her pain. Since December 1999, the guilt and the loss are the wallpaper which is in the background of all our family scenes. Most of the time it is unobtrusive. At other moments, it unexpectedly assumes a position of dominance, rather like a photograph in which the background is in sharp focus and the intended subject is blurred.

Octavia is strong. Articulate. Prematurely at ease in the adult world, taking part in all our medical decisions, questioning me about the treatments available. She has decided that she will become a PH specialist so that she can find the cause and develop a cure for PH. How dreadful that my baby girl should live a life so adult.

How grateful I am that she has a mother who responds well to Flolan, despite my PA pressures continuing to be triple digits. How grateful I am that I have few symptoms of PH and am not restricted physically to any significant degree.

I live in fear, for Octavia, of the day that I might have to begin supplemental oxygen therapy . Octavia knows Flolan, knows that my life is dependent upon two AA batteries. She no longer 'sees' the Flolan pump. For sometimes a whole day, we can be together without mentioning PH even once. Oxygen tubing and the cannisters are less easy to misidentify or ignore than an orange silk shoulder strap purse containing my pump. For me, I am happy to begin any therapy which will keep me alive a little longer, make me a little more comfortable. But another confrontation with the ravenous beast that lives in my chest is something I want to spare my baby girl. This is the fear that woke me last night. Today, writing this entry, the fear is less. The sun is brilliant, the desert is beautiful and my oxygen sats are around 95. Octavia is with her father in Dallas, so very far away geographically (and, deo gratias, politically) from where we live.

Tonight Erik and I will have dinner with Russian friends who, like me, are naturalised American citizens. We will sit on the deck and watch the sunset reflected in the mountains, turning them purple and blue and pink. When the heat of the day is beginning to dissipate, we will begin to grill our Black Angus steaks--Porterhouse and NY strip. Katya is bringing a Russian beet and potato salad and heavy rye bread of the kind that makes me salivate to see. I am making taramasalata to start the meal, to eat with delicate, dark and intense Kalamata olives and heavy heavy Greek flatbread. A salad made from whatever is in the fridge and a vinaigrette to dress it...we have an embarrassment of fresh herbs and so perhaps I will make a bearnaise for the steaks. Perhaps not though, being the intensely lazy person I become when the temperature is above 90F. The food we are having tonight originates in the cuisines of Scotland, Greece, Russia and France and yet the combination is so very American. Do you think so?

I love being an American. This will be the first presidential election in which I can vote...and not since I have lived in this country have I ever felt more strongly about the necessity of change. That is another subject--and one which also inspires fear, though of a less emotionally draining nature...

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Labor Day woes

It has been some time since I last wrote an entry into this diary, for no specific reason, but the nagging guilt I have been feeling over this is now too sigificant to ignore.

Today our weather is overcast and dull, with only a little blue sky visible over the mountains, with thunderstorms predicted for later in the day. This mirrors my mental state--a dull, grey, low-pressure system has been occupying my mind for some weeks now. It seems decided now that we will be moving to eastern Germany for six months.

The funding for my husband's research has definitely been cancelled: his project involves counterterrorism, mathematically modelling ways of identifying potential terrorist recruits, terrorist communications networks, hierarchies within terrorist organisations. etc. Why is this funding being cut now? We have a desperate need to identify these terrorists, don't we? His project is one which is 'knowledge intensive', requiring only the salaries of five or six people and a few computers, little technology at all, nothing expensive. Their results already have proven effective in developing a software prototype which could be further refined over the next months to be applied to a variety of scenarios.

So, the unlikely scenario is this: we have to move to Dresden, a city in a formerly Communist country (Deutsche Demokratisch Republik-DDR) so that my husband may continue this research with the support of the university we will be 'visiting' there. The world has gone mad and 'the centre cannot hold'. I love Germany. Living there was always a possibility for us, since it is the country of Erik's birth and his family is all still there, living in a idyllic village close to the Elbe River, living the life of simplicity and sophistication which Germans do so well. I simply was not ready for such a sudden uprooting--until this last Wednesday, we thought the funding would be saved by the eleventh-hour intervention by fiat of the head of the government agency which was funding their research. This is now no longer a possibility.

My children will be staying in the US, with me flying back a few times during the next months to see them, but the thought of being so far away from them is hell. I try to rationalise: at their age (my youngest, Octavia, is in eighth grade) I and my siblings were at boarding school in Europe, far from our parents, far from home. Somehow that rationale does not convince me. 

For me, going to Germany will give me opportunities for doing some research of my own with colleagues in Europe. My former doctoral supervisor wants to write a paper on the movie 'I, Robot' with me--very flattering and something about which I am very enthusiastic again. I have neglected my academic writing over the last two years,  partly deliberately, but also because of other writing committments, mainly for my husband's newly-established company (a consulting company which uses mathematics to identify security weaknesses and network susceptibilities to attack, etc.). This type of technical writing has been fun. Learning an entirely new vocabulary and style of writing has been a challenge (and sometimes incredibly frustrating), especially when I am required to translate heavily-scientific material into simple English.

Now is time to begin the marathon preparations for leaving. I cannot believe that we are actually going to be living somewhere else, in a city, in a small flat. I have not lived in an urban area for many years, but I am looking forward to being able to find rogan josh and chicken tikka on a whim...one of the ways we schedule my PH checkups in Denver is to make certain that one of the Indian restaurants near our motel is open. Being in a city of such historical significance is exciting, thrilling even, and I am looking forward to the huge challenge of learning more German and  becoming immersed in the culture of that magnificent country.

Enough about that for now--I have to write more frequently here and can continue this from Dresden. In the course of the last hour, while writing this, I have found myself moving away from my intention of making this my last entry. My resistance to composing a diary entry has a lot to do with eliminating what is not important, because one's life, with PH and Flolan, tends to become more complex--does one only write about PH-related topics in these diaries? Does one write about those incidents which are trivial but amusing or which provoke further discussion? How to write about PH, so that newly-diagnosed patients are given a realistic but not depressing or anxiety-provoking picture of life with this beast that lives in our chests?

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Stefan Erik is next to me, both of us on our laptops, he writing his abstracts for a conference later this month to which we are both invited speakers...and as usual I have left my preparations until the last minute. Another avoidance mechanism: I have to write my PH diary.

This afternoon, Stefan and I went to the grocery store together, driving through what counts as a traffic jam in our dusty little New Mexican town . Stefan inadvertantly cut off a man on a Harley Davidson...bad news. As we got out of our car, parked in a 'Disabled' spot, the same man drove up, revved his little engine and confronted Stefan: 'what the **** do you think you are doing?'...Stefan responded...'I am sorry...I did not see you'....man on Harley...'and yeah, you really look disabled'...Stefan Erik is, as his name suggests, a huge German, 6'3" when he slouches, all of it muscle and brain. He has a very powerful appearance and clearly does not look as though he needs that horrid blue card.

Stefan said to the Neanderthal: 'actually no, I am not disabled, but my wife has a rare lung condition which on a bad day makes her unable to walk very far'...(Stefan actually was a little less polite than I have indicated)...I said...'I have pulmonary hypertension. Go home. Google the words and then tell me that I am not disabled...'...I am feeling terribly guilty for saying this. Horribly. I was raised as an agnostic, but with strong ethical standards...and to have been this rude to someone who was simply ignorant is absolutely wrong...although I look healthy, today was one of the few days when I really needed that nasty blue card. I woke this morning with terrible chest pains, after going to bed very late and spending several hours engaged in the most intimate and enjoyable ways with Stefan Erik.

The Harley rider clearly saw that I was not taking advantage of (for example) my great grandmother's arthritis, or anything else, that I was actually ill. He said...'I am sorry for what I said about your disability'...and because I am such a weak, miserable and nasty human being (or facsimile thereof) I said to him...'what you said is unforgiveable...my husband has spent the last four years of his life trying to keep me alive...if he cuts you off in traffic, it is because he is distracted, worrying about my health...'...and this man drove off on his Harley, literally collapsed over the handlebars, as though I had beaten him with a stick...and I am sickened by my bitchiness...this man was a simpleton, clearly, a typical Harley rider: mid 50s, successful professional (probably a doctor, since he clearly was affected by my mention of PH), very articulate, well-groomed, designer jeans (but sort of beaten up, as though he bught them from a REAL motorbike guy). But no helmet. And he complained to Stefan that Stefan's driving could have caused his death.........and now I sit here, over my keyboard wondering how I can contact him to apologise. What has this wretched disease done to me, that I could be this rude to an ignoramus? Please email me if you have any suggestions as to how I can find this man, make my apologies and grovel at his biker-boot-clad feet...

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German PH conference

Hello to all who find this diary, of which I have been so neglectful since moving to Dresden

Writers' block is a terrible thing. It is worse for me now than at any time since becoming symptomatic with PAH. I have re-written just the previous few phrases multiple times, each time with a small variant of tone, an almost negligible change of syntax and with each change I am less pleased. This does not represent me, this writing style. This syntax.

Last Friday, 3 December 2004, was the fifth anniversary of being diagnosed with primary pulmonary hypertension. I have lived beyond the prognosis given to me by several PH specialists, with one exception, the Charlottesville pulmonologist who recommended Flolan, who told my mother that he believed I would 'live for decades'. During these five years, I have always questioned whether this doctor was being optimistic, in order to allow my mother gradually to accept the reality of her eldest daughter's terminal illness. Have I asked him? No. He has not been my PH specialist for the last four years, though we infrequently e-mail one another, making contact occasionally because I like him, very much. But perhaps the last element of denial in my interior turmoil will not allow me to surrender that fantasy?

Living in Germany has been overwhelming in its demands of my psyche. Please do not misunderstand what I am trying to convey. I love living in eastern Germany, in the province of Sachsen, in Dresden. What I will write now will cause some disagreement, but political correctness is more odious than almost any other pseudo-intellectual 'Commandment'. I do not want to offend anyone with my writing, but neither can I censor myself when I think or speak here...

Perhaps you know of the firebombing of Dresden in the waning, dreadful days of the Third Reich, when the Allies took their revenge upon the Fascist aggressors? In a firestrom of bombing, using incendiaries against the women and children and aged and infirm of Saxony, the Allies killed more than we will ever know. So many of these people were refugees from the uglinessof the war, or the atrocities about which we know so much now, annihated, dematerialised as though they had never existed. The bombing of the Frauenkirche, as known for its beauty as is San Marco or Tulum or the Taj Mahal, symbolises the appalling nihilism of that war. Our western civilisation, our culture, in the person of all the victims of that awful war--how could we have come so far, in centuries, from our savage pasts, our tribal mentality of dominance and submission, and yet not learned anything? I am not defending any of the evil of the Third Reich, not at all. The past still lives, in Dresden, which was, after the war, under the control of the Soviets. Totalitarian regimes have controlled Dresden not only under the National Socialists.

The Baroque beauty of Theaterplatz, where the Frauenkirche has been rebuilt from the original stones not obliterated by the bombing, is one of the places which I love to visit. I can sit and watch as tourists and natives visit the Semperoper, the Zwinger Museum: Baroque splendour, magnificent in its expression of the ideals and the aspirations of western civilisation in architectural form. I can sit at an outdoor cafe and nurse a glass of Saschsen wine, made from grapes grown in vineyards which have been cultivated, according to my husband's sister, since the tenth century. From my favourite cafe, I can hear, faintly, the mournful wails of eastern European folk melodies, played exquisitely by my favourite busker, a saxophonist. Mischa performs underneath the stone arches of one of the alleyways around Theaterplatz, as he has done for years as an itinerant musician. Mischa has a less Bohemian job too, though: in the Restaurant St. Petersburg, an Unzbekistani restaurant where Mischa plays in the evenings with his musical partner, a giant of a man from Russia who plays keyboard and accordion...

And what a disgression this has been, from PAH to the Third Reich to Klezmer and shtetls of the Pale. Are you still reading now? I hope so.

From our flat n Neustadt, north of the Elbe., the number 3 tram reliably, safely takes me south across the River Elbe, and to within only a few hundred meters of this . For about $2, and after a brisk walk through the frigid continental air, I am taken to another century, let us say around the beginning of the nineteenth. Prior to PAH, I would venture further alone, but now, I have to submit to the dragging, terrifying and always-unexpected consequences of living with this demon wrapped inextricably around my throat and chest, inexorably (despite my most optimistic meditations) suffocating me. I write 'suffocating', but I do not mean the physical sort of strangulation, impairing my breathing. I am implying another more insidious form of torture. The impairment to my psyche is here to stay. I can write and analyse and dissect ad nauseam. But I cannot understand and still less can I accept this change in my existence. I will not. And now I cannot write any more about the interior Jane. Enough, this one paragraph of psychojargon. May I go back instead to my tour guide persona?

There is a lack of materialism in Dresden unlike any other place I have ever lived. I feel the same way I did as a child living in northern Canada; living in Dresden is like jumping into a cool lake on a hot and humid summer day, a sublime experience. I miss terribly our home in New Mexico, terribly, terribly. And when we go home in a few days, I will do as the Roman Catholic Pope did when he was well: I will fall to my knees and kiss the ground. New Mexico is home. Hallowed ground for me. Dresden is magnificent and Baroque-ly beautiful, in a way that only an ancient city can be. But I can never be home.  

The writing of this diary now, today, after such a lengthy absence from this 'venue' is to force me to formulate, rationally, the psychological effects of the last few months. Most of the diarists whose entries I read on this web site are humble and self-sacrificing, willing to express themselves without self-censorship, with (seemingly) no thought of anything other than a desire to make the living with PAH less frightening for themselves and, more importantly, for other patients, especially the newly-diagnosed. I read these diaries with wonder, crying and marvelling at the genuine goodness of the authors.  

My desire, with entries to my PHCentral diary, is less noble, probably ignoble actually: I want to present a realistic depiction of my life with PAH, not only the tangible world, but the interior, intangible world of my psyche, using natural language, not the language of natural science or even medical or social sciences to express the changes that I have fought and the changes to which I have succumbed, sometimes willingly, sometimes resisting with my very-diminished emotional strength.

The changes to my body are apparent to anyone who knew me Before the Curse Era (B.C.E.): reduced weight, dry skin which is resistant to all forms of emolient, flushed face from Flolan, reduced muscle mass which makes me appear to have the body of a 70 year-old woman (not a 47 year old), thinning hair from warfarin therapy, broken blood vessels on my face (only a few, but magnified when I see myself in a mirror.

For five years, I have developed an aversion to mirrors, which has little to do with the way I appear. I am afraid that if I begin looking too closely or for too long, I will become unable to stop thinking about my appearance, become obsessive about trying to appear 'non-terminally-ill', trying to look as well as I did five years ago, a waste of time and also so contrary to anything that I have always believed about one's self-perception. By the standards of the day, my life as a woman, a desirable female, was over when I had my thirtieth birthday, perhaps a little later, but certainly no older than 35.

Right--I will end here, for now. My inspiration is finished for today, without my having even broached the subject I meant to explicate. Perhaps this is as well? My psyche is incredibly mundane, not fascinating to anyone outside my own head, but the 'Music of the Spheres', the infinite, those questions that even an early hominid probably had...oh no...I am slipping into a dreadful pseudo-intellectual rhetorical style (actually is there a 'less-than-dreadful' pseudo-intellectual style'?): referring to cultural ikons which I am presuming you know as well as I do. Can you place these references...Ligeti composition, a film from the late 1960s, featuring as its star an IBM supercomputer...?

And my son calls my Flolan pump 'HAL'...

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PAH has slowed my life. Let us not talk about prognosis or life expectancy. I mean the simple pace of my life.

The measure of my days has changed in the last five years. This is simplistic, I know. Every PAH patient has to adjust his or her beliefs and practises and simple routines to a new paradigm.

Waking in the morning with enough energy to get out of bed: something I do not take for granted. Flolan has given me my life again, but in a mutated form. My energy levels are not predictable. Today, while Erik and his doctoral student, Tim, 'did mathematics' at a local pub, I left them to go for a walk. The temperatures outside were raw: below freezing, with a chilly wind which sucked the wind from my lungs. I walked and walked and walked, because I felt normal today. I walked and walked and walked, and thought about this diary. I am a very private person. I do not like to share myself with the rest of the world. If I did not have PAH, I would not consider such a public performance.

Since being diagnosed, I have moved into a different world, one in which most of my closest contacts are with people who have been affected by PAH. This disease is so all-encompassing that it is difficult to be with people who are healthy, who are able to wake in the morning without thinking immediately of their own mortality. Difficult, but not impossible. I love meeting new people, who do not know my diagnosis, who admire my purse, the orange silk embroidered one, a true fashion statement, which holds my Flolan pump...

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Friends: time and again

New friends, those I have made since 'meeting' primary pulmonary hypertension, are few. This is not different from my previous life. My close friends are perhaps three in the course of my life, perhaps four. I am now 47, as you know from previous maunderings. To analyse thse statements arithmetically, I am not a popular woman.

At the time of my diagnosis, I had not seen my oldest friend for several years. She lives in Maine, thousands of miles from me, metaphorically and geographically and our friendship was dormant, not ever in question, but simply  quiescent.

This, the most enduring friendship of my life is with a woman called Adrian, whom I met in between classes, during our first year of high school in a small town in Maine. We were both walking towards the same classroom, for our English class with Mrs. Reynolds. This teacher was a large, formidable woman who dressed almost always in black, in that style of dress called 'shirtwaist', a style that I even now associate with teachers of English. I was miserable, lonely for my mother and my younger siblings, yearning for any sort of approval or acceptance in this new, frightening environment, and desperate for companionship.

Now necessary is a small digression to explain why I was in that corridor, so alone and anxiety-ridden, and why this moment is one of those which defines my life and also gives me a paradigm within which to frame it.

I was 12, a ridiculously young age to be a freshman in an American high school. The previous five years or so of my life were spend in Alberta, Canada, first in a tiny village called Islay and then in a more northern town called Peace River. My father, a British-trained doctor, had been offered a position in Islay upon finishing his medical school education and training in London. At that time, 1965, there were few opportunities in Britain: the NHS was even then under severe strain and working conditions for doctors were not suffieiently well-paid enough to support a family with (at that time) five children). The hours necessary to support our family would require that my father do 'locum tenens' work, in addition to a hospital practice, or that my mother would have to again go back to work, after the birth of my twin brother and sister, who were born in May of 1965, bringing the number of my parents' children to five.

In another entry, I will write about that chapter in the lives of my siblings and me, my parents and their parents. The significance of this heroic journey is difficult to comprehend for anyone not living through that time. My parents moved their growing family to a new continent, in a time when such a journey, even for a holiday, was unthinkable without planning and saving and organising. This was a year in which, for example, my mother bought special outfits for all her children specifically for the journey to western Canada, across the Atlantic ocean, then across that vast expanse of Canada, across the cities and the villages and the magnificen. In those days, one 'dressed-up' for such a rare event: my mother wore a beautiful dress and matching coat, with high heels and a hat. My mother is a beautiful woman, elegantly and fashionably dressed even for a twenty-four-hour journey from London to Amsterdam to Toronto to Edmonton, with five small children in tow behind her.

My father had preceeded my mother to Islay, by  few months, to begin his new job, as the only doctor for many miles in any direction. His letters to me from that time are poignant: so full of hope and excitement, the thrill of the unknown and the trepidation of a new beginning. I missed him terribly during that brief time of his absence and the moment in which I saw him as we crossed the tarmac of Edmonton International Airport (no jet bridges in 1965!) was perfect.

This brief disgression again has expanded into another chapter altogether from it original purpose, again. Again. No discipline, and a wanton freedom that comes from writing into the vacuum of cyberspace, a sucking black hole into which I can throw all my history and my thoughts and not even hear an echo of them.

May I now try to write about meeting my friend Adrian?

My father and mother decided that they wished to leave Canada, for several reasons, in 1970, after the end of my eigth grade year in a small Catholic school called Glenmary, in Peace River. One significant reason for this move was to be closer to England, after the death of my mother's father and her mother's decining health. My father, after many interviews and rejections, was offered a position in a small town in Maine, as I mentioned above (many digressive thoughts ago).

For this latest upheaval, it was decided that my father should travel again before the rest of our family, in order to begin his latest new job. My sister Helen and I would fly to Boston a few weeks later, in order to start school, while my mother remained in Alberta with our other sister and three brothers. Our mother would arrange the details of packing up the household and join us in the USA as soon as possible. I was to begin high school, my sister to begin fourth grade, in a new school, new town, new country.

The new school year would begin before all the details of this latest international move could be organised and this time, I said goodbye to my mother in Edmonton International Airport, traveling with my sister through Toronto, to Logan International Airport in Boston. I was 12. Helen was 9. Again, we were dressed in specially bought clothes for this occasion, though my mother did relax her sense of style suficiently to allow us to wear trousers, though not 'bell bottoms', those Hippie-esque trousers which carried with them a certain immoral quality which was not apparent to me.I thought they were 'cool', an expression of belonging to a certain avante garde. My outfit constisted of black tapered trousers, the kind with an elastic band which ran under one's foot, to keep the trousers anchored, the material (some combination of stretchy polyester-type fabric, I think) unwrinkled. Helen's trousers were similar, black like mine. We wore red cotton blouses and matching head bands. Helen was a pretty girl, very pretty, who had a strong resemblance to our father--dark brown hair, dark brown eyes, a tiny little nose, generous mouth with, even at this young age, full lips. Helen's skin tone was very pale and, in combination with her hair and mouth, she was the very ideal of Irish beauty. She still is today (she is in her early forties).

The final elegant touch to our outfits was our shoes.

Helen and I were allowed to choose and wear black patent leather pumps...the Mary Jane style with the band across the instep. Perfection. Because I had passed one of those significant milestones of age (I think that 12 was my mother's cutoff), I was allowed even to choose shoes which had a 'high heel'...all of 1 1/2 inches.

The details of our journey together across Canada to our new home was eventful. Another time for that.

Thus, my father, Helen and I were housed in an apartment in the nurses' quarters of the hospital of this small community, waiting until the rest of our family arrived before we moved into a 'real' house. I had my books. I was reading 'Jane Eyre' and my father had introduced me to Agatha Christie's detective stories.

Those days were lonely. Desperately so. I read excessively. Helen and I walked around the town together, along the river, the Kennebec, which had once been a navigable river, along which much commercial traffic flowed. I missed my mother and my siblings and my only substitute was the family with which the novel 'Jane Eyre' ends. My father was working long hours, attempting to do well in his new position, so tired at night that he had little energy for his daughters. We understood. We ate all of our meals in the hospital cafeteria. Shared our table with nurses and doctors and occasionally patients. Because of PAH,I have once again become educated about hospital cafeterias. The universal smells of these institutions: watery boiled cabbage (does anyone really eat this, or is it a spray scent to disguise less pleasant odors?) and meatloaf and soggy whitebread toast, jello and canned 'fruit salad', weak de-caffeinated coffee and wilted lettuce. None of these has changed in the inervening 30 years. Arriving in Charlottesville after being ambulanced there from Roanoke in December of 1999, being wheeled in through the emergency room entrance, my sense of smell immediately took me back to 1970, to that cafeteria in which I ate my breakfast and dinner. The solitude of that time has really been a metonymy for all subsequent experiences, for me.

My birthday of that year came a few weeks after arriving in Maine. My father forgot the date. So did my sister. So did I. After school that day, Helen and I were walking on the town green, quietly wasting time until our father finished work for the day. I had not noticed the date, probably, in retrospect, because of my state of melancholy. But: I had just begun to read 'The Secret Garden', with its story of the solitary life of a young girl who has been orphaned and lives a life of aloneness which I understood too well. I was not unhappy, as strange as that may seem. Melancholy is both more and less than unhappiness, a personality type as much as a mood.

As we were walking, I realised the date. Before thinking, I said 'today is my birthday' to Helen, whose reaction made me more miserable, causing her embarrassment. I asked her not to tell our father. Helen, being a worried younger sister, did tell him. He apologised, many times, making me feel worse. My mother, when she called later that evening to wish me a happy birthday, wanted to know how we were celebrating the day. It was not a good birthday. Worst was my father's guilt.

I was lonely then in ways that I cannot adequately address even today. I was friendless in a new town, completely intimidated by the enormous school, the high school in which I was the youngest person, I think. My optimism about the future, that my mother and siblings would soon be arriving from Canada, was present always, but I sometimes could not see my way through the intervening days or weeks before their arrival. I love Helen and my father, but my refuge from the world had always been my large family, where I was always needed, where there was always a sibling to play with and hug and kiss and read to.

And then one day, I took an Agatha Christie book with me to school, because I liked to read during my lunchbreak, too shy to approach any table where other students were sitting. I would buy my lunch (that institutional food again), find an empty table and read Agatha's latest, reading for the 20 or so minutes before my afternoon classes began. While reading, I also listened to and watched the other students, who seemed to be living the 'high school experience' that I had anticipated since the first term that I attended school in Islay, where my school had four classrooms, each with two grades being taught there. The exception was the classroom in which the seventh through ninth grades were taught...after that, one was bussed to a regional high school, a place as remote to my experience as that of an illiterate peasant of the Middle Ages. Stories of the subjects studied (Latin and Typing and Woodwork) and the social life involved made the prospect of spending three or four years in such an environment an absolutely fantastic dream. The few high school students I met were the sons and daughters of my parent's friends in Islay, among them the twins, Danny and Donna, who used to babysit my siblings and me occasionally. They seemed so adult, so 'cool'...I wanted to be a part of their world, to be an almost adult, to use language which was not encouraged in our house, to watch TV together, have a pizza and Ginger Ale and listen to pop music...

But instead, because of my shyness and the difficult circumstances of my life, I was reading Agatha alone in the cafeteria.

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Friends: time and again

New friends, those I have made since 'meeting' primary pulmonary hypertension, are few. This is not different from my previous life. My close friends are perhaps three in the course of my life, perhaps four. I am now 47, as you know from previous maunderings. To analyse thse statements arithmetically, I am not a popular woman.

At the time of my diagnosis, I had not seen my oldest friend for several years. She lives in Maine, thousands of miles from me, metaphorically and geographically and our friendship was dormant, not ever in question, but simply  quiescent.

This, the most enduring friendship of my life is with a woman called Adrian, whom I met in between classes, during our first year of high school in a small town in Maine. We were both walking towards the same classroom, for our English class with Mrs. Reynolds. This teacher was a large, formidable woman who dressed almost always in black, in that style of dress called 'shirtwaist', a style that I even now associate with teachers of English. I was miserable, lonely for my mother and my younger siblings, yearning for any sort of approval or acceptance in this new, frightening environment, and desperate for companionship.

Now necessary is a small digression to explain why I was in that corridor, so alone and anxiety-ridden, and why this moment is one of those which defines my life and also gives me a paradigm within which to frame it.

I was 12, a ridiculously young age to be a freshman in an American high school. The previous five years or so of my life were spend in Alberta, Canada, first in a tiny village called Islay and then in a more northern town called Peace River. My father, a British-trained doctor, had been offered a position in Islay upon finishing his medical school education and training in London. At that time, 1965, there were few opportunities in Britain: the NHS was even then under severe strain and working conditions for doctors were not sufficiently well-paid to support a family with (at that time) five children). The hours necessary to support our family would require that my father do 'locum tenens' work, in addition to a hospital practice, or that my mother would have to again go back to work, after the birth of my twin brother and sister. With their births, in May of 1965, my parents' children totalled five.

In another entry, I will write about that chapter in the lives of my siblings and me, my parents and their parents. The significance of this heroic journey is difficult to comprehend for anyone not living through that time. My parents moved their growing family to a new continent, in a time when such a journey, even for a holiday, was unthinkable without planning and saving and organising. This was a year in which, for example, my mother bought special outfits for all her children specifically for the journey to western Canada, across the Atlantic ocean, then across that vast expanse of Canada, across the cities and the villages and the magnificent prairies. In those days, one 'dressed-up' for such a rare event: my mother wore a beautiful dress and matching coat, with high heels and a hat. My mother is a beautiful woman, elegantly and fashionably dressed even for a twenty-four-hour journey from London to Amsterdam to Toronto to Edmonton, with five small children in tow behind her, clinging to her or being carried by her.

My father had preceeded my mother to Islay, by  few months, to begin his new job, as the only doctor for many miles in any direction. His letters to me from that time are poignant: so full of hope and excitement, the thrill of the unknown and the trepidation of a new beginning. I missed him terribly during that brief time of his absence.  The moment in which I saw him again, as we crossed the tarmac of Edmonton International Airport (no jet bridges in 1965!) was beyond-words perfect.

This brief disgression again has expanded into another chapter altogether from it original purpose, again. Again. No discipline, and a wanton freedom that comes from writing into the vacuum of cyberspace, a sucking black hole into which I can throw all my history and my thoughts and not even hear an echo of them.

May I now try to write about meeting my friend Adrian?

My father and mother decided that they wished to leave Canada, for several reasons, in 1970, after the end of my eighth grade year in a small Catholic school called Glenmary, in Peace River. One significant reason for this move was to be closer to England, after the death of my mother's father and her mother's decining health. My father, after many interviews and rejections, was offered a position in a small town in Maine, as I mentioned above (many digressive thoughts ago).

For this latest upheaval, it was decided that my father should travel again before the rest of our family, in order to begin his latest new job. My sister Helen and I would fly to Boston a few weeks later, in order to start school, while my mother remained in Alberta with our other sister and three brothers. If you are doing the arithmetic, you will see that my siblings had now increased in number. My youngest brother, Cyril, was born in 1968, the first of our family to be a Canadian by birth!

Our mother would arrange the details of packing up the household and join us in the USA as soon as possible. I was to begin high school, my sister to begin fourth grade, in a new school, new town, new country.

The new school year would begin before all the details of this latest international move could be organised and this time, I said goodbye to my mother in Edmonton International Airport, traveling with my sister through Toronto, to Logan International Airport in Boston. I was 12. Helen was 9. Again, we were dressed in specially bought clothes for this occasion, though my mother did relax her sense of style suficiently to allow us to wear trousers, though not 'bell bottoms', those Hippie-esque trousers which carried with them a certain immoral quality which was not apparent to me.I thought they were 'cool', an expression of belonging to a certain avante garde. My outfit constisted of black tapered trousers, the kind with an elastic band which ran under one's foot, to keep the trousers anchored, the material (some combination of stretchy polyester-type fabric, I think) unwrinkled. Helen's trousers were similar, black like mine. We wore red cotton blouses and matching head bands. Helen was a pretty girl, very pretty, who had a strong resemblance to our father--dark brown hair, dark brown eyes, a tiny little nose, generous mouth with, even at this young age, full lips. Helen's skin tone was very pale and, in combination with her hair and mouth, she was the very ideal of Irish beauty. She still is today (she is in her early forties).

The final elegant touch to our outfits was our shoes.

Helen and I were allowed to choose and wear black patent leather pumps...the Mary Jane style with the band across the instep. Perfection. Because I had passed one of those significant milestones of age (I think that 12 was my mother's cutoff), I was allowed even to choose shoes which had a 'high heel'...all of 1 1/2 inches.

The details of our journey together across Canada to our new home was eventful. Another time for that.

Thus, my father, Helen and I were housed in an apartment in the nurses' quarters of the hospital of this small community, waiting until the rest of our family arrived before we moved into a 'real' house. I had my books. I was reading 'Jane Eyre' and my father had introduced me to Agatha Christie's detective stories.

Those days were lonely. Desperately so. I read excessively. Helen and I walked around the town together, along the river, the Kennebec, which had once been a navigable river, along which much commercial traffic flowed. I missed my mother and my siblings and my only substitute was the family with which the novel 'Jane Eyre' ends. My father was working long hours, attempting to do well in his new position, so tired at night that he had little energy for his daughters. We understood. We ate all of our meals in the hospital cafeteria. Shared our table with nurses and doctors and occasionally patients. Because of PAH,I have once again become educated about hospital cafeterias. The universal smells of these institutions: watery boiled cabbage (does anyone really eat this, or is it a spray scent to disguise less pleasant odors?) and meatloaf and soggy whitebread toast, jello and canned 'fruit salad', weak de-caffeinated coffee and wilted lettuce. None of these has changed in the inervening 30 years. Arriving in Charlottesville after being ambulanced there from Roanoke in December of 1999, being wheeled in through the emergency room entrance, my sense of smell immediately took me back to 1970, to that cafeteria in which I ate my breakfast and dinner. The solitude of that time has really been a metonymy for all subsequent experiences, for me.

My birthday of that year came a few weeks after arriving in Maine. My father forgot the date. So did my sister. So did I. After school that day, Helen and I were walking on the town green, quietly wasting time until our father finished work for the day. I had not noticed the date, probably, in retrospect, because of my state of melancholy. But: I had just begun to read 'The Secret Garden', with its story of the solitary life of a young girl who has been orphaned and lives a life of aloneness which I understood too well. I was not unhappy, as strange as that may seem. Melancholy is both more and less than unhappiness, a personality type as much as a mood.

As we were walking, I realised the date. Before thinking, I said 'today is my birthday' to Helen, whose reaction made me more miserable, causing her embarrassment. I asked her not to tell our father. Helen, being a worried younger sister, did tell him. He apologised, many times, making me feel worse. My mother, when she called later that evening to wish me a happy birthday, wanted to know how we were celebrating the day. It was not a good birthday. Worst was my father's guilt.

I was lonely then in ways that I cannot adequately address even today. I was friendless in a new town, completely intimidated by the enormous school, the high school in which I was the youngest person, I think. My optimism about the future, that my mother and siblings would soon be arriving from Canada, was present always, but I sometimes could not see my way through the intervening days or weeks before their arrival. I love Helen and my father, but my refuge from the world had always been my large family, where I was always needed, where there was always a sibling to play with and hug and kiss and read to.

And then one day, I took an Agatha Christie book with me to school, because I liked to read during my lunchbreak, too shy to approach any table where other students were sitting. I would buy my lunch (that institutional food again), find an empty table and read Agatha's latest, reading for the 20 or so minutes before my afternoon classes began. While reading, I also listened to and watched the other students, who seemed to be living the 'high school experience' that I had anticipated since the first term that I attended school in Islay, where my school had four classrooms, each with two grades being taught there. The exception was the classroom in which the seventh through ninth grades were taught...after that, one was bussed to a regional high school, a place as remote to my experience as that of an illiterate peasant of the Middle Ages. Stories of the subjects studied (Latin and Typing and Woodwork) and the social life involved made the prospect of spending three or four years in such an environment an absolutely fantastic dream. The few high school students I met were the sons and daughters of my parent's friends in Islay, among them the twins, Danny and Donna, who used to babysit my siblings and me occasionally. They seemed so adult, so 'cool'...I wanted to be a part of their world, to be an almost adult, to use language which was not encouraged in our house, to watch TV together, have a pizza and Ginger Ale and listen to pop music...

But instead, because of my shyness and the difficult circumstances of my life, I was reading Agatha alone in the cafeteria.

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Adrian was walking beside me in the hall. She looked at my books and said: 'You read Agatha Christie too?'...and she looked at me as though I were a fellow-traveller in the land of literature. And she pronounced 'Agatha' in a way which I knew to be correct, stress on the first syllable, not the way that I had first presumed it was pronounced, 'aGAtha', and which my father corrected with just the right balance of humour and disdain, a mixture which my father employed often in his life amongst the heathen (his children and the unchurched, in this case all non-Europeans, all non-Roman Catholics, all non-intellectuals and all non-readers of Agatha Christie, Graham Greene, Evelyn Waugh, Ovid, St. Augustine and Plato).

I, being as shy as I was, spluttered 'yes...you know who she is too?'...a gauche and silly comment. Of course she knows who Agatha Christie is, you twit. You absolute bird dropping, Jane. This girl is the first person to address you as a human being in this machine of a thousand-pupil high school. She has seen that you are beyond-salvation socially now. You cannot even answer a simple yes or no question, without blushing the blush of ten thousand virginal brides. This is not a test question. The answer to which will decide whether the lions will feast on you today. She asked only, in a friendly tone, with a huge smile, whether you also read Agatha...My blood pressure soared, my autonomus nervous system went into high gear, causing chaos in my vagal nerve, making my stomach roil and threaten to embarrass me in ways too horrible to survive socially.

'Yes, cool. You are the only person I have ever seen of our age who reads her novels. This is great'...

...and my school year suddenly ceased being the morass of social annihilation that it had threatened to become, that I had foreseen from the first time I saw the building: two stories, a thousand classrooms, millions of students, all of them cooler than I, long hippie hair, bell-bottom trousers, Janis Joplin hair (on the boys) and Beatles haircuts (on the girls). Waving 'peace' to one another between classses. Talking about sex. Drugs. 'Nights in White Satin', smoking pot and driving the MG that Daddy gave him to celebrate his 18th birthday, being ticketed by the police, but Daddy is a doctor, so the ticket will not be paid. Torn up and thrown out of the car window...

...these conversations which had previously been ones to which I was only an eavesdropper...during class...walking to the school bus...suddenly, I was having a conversation with a real High School Student. A girl who seemed to be interested in me...possibly a girl who might be a friend. Elation!

At home, talk about Vietnam was omnipresent. My parents friends, most of whom were doctors, some with sons of draft-eligible age: should we encourage our sons to go to Canada, join the National Guard? Friends of my parents: a surgeon just back from doing trauma surgery on the battlefields of Vietnam, depressed, drinking too much, but thrilled with his new skills. Learned while cracking chests and amputating legs of young men. High school students who were involved. Because their brothers were being sent over there...but also because it was a cause. Ethically these students were knowledgeable, brave in their outspokenness. My knowledge of politics was miniscule.

But I learned. Maine,usually the most tranquil of states, in those years became for me a place of political education. At school, there were bomb threats called in, the presumed reason being to make a statement against the war. Gardiner Area High School has never, before or since, I think, been so politically engaged...

My parents were not in favour of the war. After a brief period of indecision, my father saw the devastation of the poor from the underclass of America, (or so I see it now, idealised for 'public' viewing, writing this, about 35 years later). My father's political opinions were terribly conservative in some areas, but radially 'left' in others. My mother had lived through the horror of her home country being bombed, in World War 2 in Britain. Neither of my parents thought that Vietnam was anything more than a dreadful imperialist incursion (and I am sorry for the cliche, but it is so very appropriate in mentioning this era, yes?) but their horror was re-directed, towards the plight of the returning soldiers. They taught me that these men had done what their country asked them to, but upon returning to their home country, they were spat upon and shoutd at by the very people who advocated non-violent solutions. This influenced my thinking about the war, very very much.

My mother's political opinions, at that time, were conservative, though never as 'right' as my father. My mother always wants those around her to be happy, well-fed, cared for and loved. Her life has been spent taking care of people and her political opinions have always been directed to optimising the happiness of those around her. My mother is unique. She is a woman of strength. She has used her strength to do huge good in this world, and continues to do so. My mother's political opinions have evolved greatly over the last 35 years and she would now describe herself, I think, as a 'lefty-liberal'

My parents' political education of me began, in a conscious manner, around this time of my childhood, my freshman year of high school in the USA. Previously, their political talk to me was limited to discussions of local elections in Canada, in which we perhaps had met te candidates.  The 'Progressive Conservative' party, a strange confluence of opposing philosophies,  was the political party in Alberta which my parents discussed most frequently while we were living there, the time in which Pierre Elliot Trudeau was elected Prime Minister for the first time. My parents did not like Trudeau or approve of his leftish leanings, of course, but to me he was a person of fascination. He had a wife who was very young, a former Miss Canada or something similar, a woman who was most photogenic, always beside her husband when he was in public.

My parents' political education of me was sound--I was not ever excluded from their discussions of the war, for example. At one point, about a year after the day on which Adrian and I met for the first time, from boarding school in England, I wrote an anti-war letter to the editor of the Gardiner area newspaper, explaining about the dreadful circumstances to which our servicemen were returning after serving their country, while rich white boys spent the time in the National Guard, one weekend a month or. The letter was published, and  received a few replies, most of the encouraging me to keep writing, a few telling me that they thought the letter had been ghost-written by my father or mother. Not at all.

So, Adrian and I talked briefly that day, before class started. I floated out of the class when it was over. We had agreed to share our books, since Adrian had more ACs and so did I...my parents would do anything to create peace in our house (six children under the age of 13 was a little noisy at times. One could even be forgiven making one's bed, if one were quiet and good and reading something 'worthwhile'. Comics and 'Nancy Drew' and 'Seventeen' magazine did not count.

From that day onwards, Adrian and I were friends. I seemed to think that she was looking for a friend too, not as desperately as I, since she had already a large group of girls who liked her, with whom she seemed content. I was an outcast, the weird one, the one who read Ovid. Adrian understood. She invited me to come to her house after school one day, taking the school bus, a different one from the one I took. This was really a test. I had only spoken to Adrian in class, until now. Could I maintain a real conversation wth her? For the two hours until my mother came to pick me up? I was sweating and trying to think of excuses not to go (e.g. migraine, cholera, sleeping sickness, lice) because I knew I would not please this person whom I so wished to be my friend. She spoke English. Her verbs and nouns agreed, which meant that she would understand what I was trying to say...my Canadian friends had to teach me their dialect, because they could not understand my standard, 'received BBC' English...so now I hoped that I would be understood by her.

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How is it that I can be writing this at the end of 2004, and yet feel as though I am living through my young adolescence? I have a few diaries from the early 70s, those which were not thrown into the rubbish by my former husband, and when I read them, I feel that my mind has not changed since then, that I am still the same person. All the experiences of my life have not changed me. Does this make me insensitive? Grotesquely unfeeling? Stupid? I could be writing the same thoughts now, 'live' on the internet, as I wrote over 30 years ago.

Are we not supposed to learn, to change our psyches as we live, to learn from our experience of the mundane-ness of lived experience? I have lived through hell, in many forms, and none of them has changed me. The constants in my life have been: my love of my children, my friendship with Adrian, the love of my mother and father, my sense of wonder and optimism, that the world is somehow perfectable, if only I could find the right formula.

And yet, this world around me does not change. Still the sun sets in the west rises in the east, and in the interim, we all flail about, attempting to do the most good we can, the least evil. Why is it that we are all given an essential knowledge of good and evil? We comprehend, all of us, the absolute existence of Good and Evil, some sense of the Numinous, something transcendant, something truly good, and yet to find such goodness within our own lives is so rare.

But perhaps not--perhaps we are to take the good where we find it, in whatever diluted form it occurs in our brief existences on this planet.Perhaps, when I receive a witty email from Adrian, or when I have a brief chat with my precous daughter Martha, I am living the most perfect existence that one can, as a fallible Homo Sapiens sapiens. I was raised in the Roman Catholic Church, though my parents had far more belief in the intellectual aspects of that church's history than in its lived reality. My parents, I think now, were good agnostics. They could believe in the goodness of the philosophy of the Roman Catholic Church, but the belief in a trascendant, all-powerful, God was beyond their ability to comprehend.

I sit here, on the couch in my little A-frame house, in the high desert of New Mexico, looking out over the desert (actually, I cannot see anything, since it is after dark, but I am writing from memory, from my vision of the mountains three miles away across the open desert). Can I possibly be more content than I am now? I feel the presence of something more powerful than I. I have not been given the gift of a belief in a transcendant deity, as much as I have wished for such certitude. I so wish that I could go to a church on Sunday, or a synagogue on Friday, and feel that I was worshipping the entity which created this magnificent universe. I can't. I simply cannot believe. Why? Why is it that my friends are so able to believe? Why does the God in whom I do not believe give some of us faith, but leave the rest of us abandoned? Why can I sit here, desperate for some certainty, but feeling abandoned, because I have no innate belief. If one has to try to believe, then the belief is not valid.

Duress, the 'need' to believe, invalidates the belief, I know...but all of this is just intelectualisation, wasted, bombastic words, nothing but sturm und drang. Signifying nothing. I love my husband, Stefan Erik. I love my children. I love my friend Adrian. For them I would do anything, as pathetically heroic as that reads. My favourite authors, beyond Agatha Christie (for whom I am thankful for her role in the formation of the most significant friendship of my life) are the European High Romantics: all of whom believe in the capacity of humans to both create and destroy, in whose writings the power of emotion is all-consuming.

Life is not necessarily good. It is, though, all that we have. All that we have. And so, it is to be valued and cherished and preserved. Each moment we have is pristine. Each moment we have can never be replicated. Each moment we have is never to be repeated, never to be dismissed, always to be lived with complete abandon and complete devotion. Waiting for the next act or the next scene in our lives, we can die while the scenery is being re-arranged.

And so, life goes on. Thanks to the All Powerfu Deity about whom I have no knowledge, but complete belief. Does this contradict what I wrote earlier today? I do not think so.

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This diary business is difficult to maintain-this evening I read an entry from a woman whose diary entries I read as soon as they are posted. She wrote (and this is a paraphrase of a high degree) that she feels a little disappointed when diarists do not contribute as frequently as she would like.

And when I read her words, I realised that she and I were thinking the same way. I read these dairies religiously, and always think 'why does this woman not write more, she is so talented and gives away so much wisdom'...and then I realise that we are all too busy to write more frequently, a commentary upon the treatments available to us today...and aren't we fortunate? Yes, we are, but I know that we also have a higher duty to those who are newly-diagnosed, to tell these women about our experiences.

I hate PAH.

I hate PAH. With a passion that is almost impossible to convey. Through my experience of this disease, I have met the most brave women ever to live on this earth. I have seen and talked to women whose lives have been taken from them (and please no platitudes about the quality of life versus the length of it), who are completely debilitated because they took diet drugs for as little as two weeks a few decades ago. My PAH is idiopathic, unexplainable, but already I feel as much guilt (for the sake of my children) as if I had deliberately taken some sort of dreadful poison. Talking to my friends who took Phen-Fen, I am in the presence of some sort of meta-history, some sort of fable about how we should trust our own selves, our own bodies. My friends who took these drugs are women with enormous intelligence, who were perceived by their environments as being somehow less worthy because their bodies did not conform to some middle-class, mid-20th century ideal of womanhood.

When I first became a patient of the Badesch group in Denver, about three years after being dx with PAH, I had a meeting, an unexpected one, with the head of clinical research there.  In the course of our talk together, he asked me whether I had ever taken diet drugs. This was not asked in any sort of judgmental way. He simply was curious in a scientific manner. I was incensed. I weigh about 105 lbs when I am naked (yuck, what a horrible image). His reaction to my disbelief: when the poisonous diet drugs were at their most popular, women even as small as I were being prescribed them.

My quality of life: until last Thursday, was very good. Superlative almost. I am on Flolan, as I have written about previously. For reasons beyond my control, I have had 6 previous central lines. I have had infections which were caused by a lack of awareness of hygiene on the part of ER personnel, my Groshongs and my Hickmans have failed mechanically. None of my central lines has failed due to any lack of care on my part--I think.

Last Thursday, while talking to my husband about whether we should travel together to Germany or not--if I stayed in the US for longer, I could spend more time with my children-- I looked down at my legs, at my Gap jeans, to see a rapidly spreading red stain...on my right thigh, where my central line extension tubing was sitting. A large red stain. A very large and rapidly growing red stain.

Some right-brain function took over, and I followed, with my hands and my eyes, the tubing, from my pump to my extension tubing, to the site where the Hickman entered my chest. At each juncture, there was blood leaking out. I felt cold, as though I had come in to the heated house after I had been walking in the snow for an hour or so..and while this contrast in temperature can be very very energising, on this occasion it was terrifying. And then not so--my mind was alternating between extremes of sensation.

I was not certain whether my subjective feeling of coldness was due to a reaction of fear, that I was going to die from interruption of my Flolan. Or perhaps I was actually suffering from a lack of oxygen to my tissues. I felt so cold, so very very cold, from deep within me, some sort of radiation of cold, some preturnatural chill which I thought could not be changed, could not be warmed. It was all-encompassing in its power. Some part of it was seductive. I could simply lose myself in this chill. Relish it. I remember reading about dying from PAH, how it could be not traumatic at all. This always seemed to me to be grotesque: death is the end of all we know. How can we not fight against it? I am not religious, though I wish I had that faith. But, even so, or perhaps because of my lack of religious faith, I do not want to die. I love life. I love my family and my friends. Having PAH has only intensified that feeling.

I wish sometimes that I had the expectation of another, next life, but I do not. That is a gift which I wish I had, but no amount of prayer has given me that faith. Any ideas? I do not mean to be flippant. I love my religious friends and envy them.And I ask them to pray for me, despite the lack of logic in that, a non-believer asking for the power of prayer to 'be'.

So, the inevitable ER visit ensued. Incompetence all around, more than ever before. One small but crucial event: an RN called Rachel was there. Rachel has seen me on several occasions. She knows about Flolan. Hallelujah! She started two IVs, one for Flolan, one for everything else. I reminded her that Flolan could not be piggy-backed with any other drug, nor could the line be used for taking blood. Rachel is some sort of angel: she is always followed by a nursing student, teaching them the intricacies and delicate aspects of nursing. Because of her, PAH treatment will be taught to many new nurses in my home town.

I have posted the details of my central line replacement on the PHA message board. It was horrible. Horrible. Details: I talked to the self-described 'vascular surgeon', about what he would do. We agreed: he would replace the Hickman with another Hickman, using the same site. If he had to move the line, he would be certain to make the new entrance site in a place which I described to him, not near my bra strap area etc...the entire process, he said, would take only about half an hour...

and two hours later, I woke up with a Groshong in the other side of my chest, with the entrance site very very close to my armpit. No chance of maintaining sterile technique. No chance at all. And this is where I am now. Sitting here on the couch, with three Primapore dressings overlapping on my new central line site, trying to prevent infection, simply waiting for infection to strike. I have taken Levaquin since the day afer my latest episode, not due to the prescience of the surgeon, but because I am so terrified of an infection. I always keep a 10day supply of L at home...and it may have saved my life this time too, although I am still waiting for the next week or so to find out whether this is so...

Life with PAH. I was dx on December 3, 1999. Had anyone told me, before that date, that I would live connected to a pump, which was driven by 2 AA batteries, pumping a drug into my heart , a drug which was so short in its half life that the pump could not be interrupted, ever, for more than five minutes, I would have dismissed that person's vision of my future with a laugh and a thought that he or she was suffering from some sort of bizarre hallucinations. No such purgatory could exist.

And here we are, five years on.

Another universe.

I am fortunate, I have had children. Had I been told at the age of 23 or so that I could not bear children, I would have been suicidal. I have had seven pregnancies, resulting in six live births. My one miscarriage was horrific, and filled me with absolute sadness: I could not imagine being told that I could never bear a child. My husband and I attended the German PH conference last October, and during the course of this, I met two young women who had just been diagnosed with PAH. One was 17, the other 24. They both have been told that they cannot bear a child, because pregnancy would place intolerable stress on their hearts and lungs. What a cruel fate. Our bodies, our female hips and uteri and hearts are prepared to procreate. We are fortunate, I know to live in an era when women are not forced (at least in our western European societies) to become pregnan