A Life Without IX

By Ray Perreault

This is a story about one man’s struggle to be just like everyone else even though he was born different. John is waking in pain. Not just the crippling physical pain that he is use to waking with, but a much deeper and more disturbing kind that eats away at his courage, dragging him deeper into despair. For thirty-two years he has struggled to build a life he felt he had no right to. He is finally giving up now, too tired and beaten to find the courage he needs to pick himself up yet again. Where does the darkness lead, and how can the light of LOVE find him and lead him home?

• Language: English
• Publisher: Ray Perreault
• Release date: Jun 5, 2013
• ISBN: 9781301881048

Ray Perreault

Ray Perreault, author of: A LIFE WITHOUT IX was born in Biddeford, Maine and lived there with his wife Carmen and son Nicholas until 1990. They now resides in Clermont, Florida Ray is the creator and weekly contributor to a blog about Hemophilia and the community of people affected by it. You are welcome to visit his blog at: http:// blog.raymondperreault.com or write to him at: [e-mail]

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A Life Without IX

Published By Ray Perreault at Smashwords

Copyright 2013 Ray Perreault

http://blog.raymondperreault.com

Smashwords Edition, License Notes

This book is licensed for your personal enjoyment only. This book may not be resold or given away to other people. If you would like to share this book with another person, please purchase an additional copy for each recipient. If you’re reading this book and did not purchase it, or it was not purchased for your use only, then please return to Smashword.com and purchase your own copy. Thank you for respecting the hard work of this author.

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This book is dedicated to my wife, Carmen, whose courage, strength, and ability to love unconditionally helped me to realize the wonder of life, living, and how empty a life can be without her in it. To my son, Nicholas, whose innocent smile and child virtues taught me the eternal nature and purity of love, first by his birth, and then by his willingness to share his life with me. To the doctors and nurses who never gave up on me, especially Dr. Connor Moore, a special man who helped me to live a better life and reach for more. And finally, to my Mom and Dad who not only gave me life, but helped me to live it by sheer will.

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John was lying on an examination table in the outpatient room of his local hospital. He was restless and in a lot of pain from an injury to his knee. As he watched the doctor prepare the IV medication that had just finished thawing, his father noticed how tense and helpless he looked struggling with the pain he was in; feeling helpless himself he said:

«John, m'écouter pendant une seconde. Vous avez obtenu d'entendre cela, c'est drôle» :

‘Ti Jean Béjin né dans une belle place de Laurette fut mis à la porte par son père à l’âge de 12 ans.

‘Ti Jean Béjin a parcouru toutes les villes éloignées pour terminer ses études non commencées.

‘Ti Jean Béjin fut pris d’une grande maladie et mourut. Puis on marqua sur son épitaphe « Ici repose le corps de

‘Ti Jean Béjin né dans une belle place de Laurette fut mis à la porte……….»

Auteur inconnu ; Récité à John par son papa.

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Chapter 1: Waking

John wanted to scream till his voice went hoarse. Screaming wouldn’t help though, he knew by now that it never did. He was waking in very bad pain and would soon have a mood to match. No one would argue that he had the right to being in a bad mood, though for John, a bad mood despite his pain was not a usual thing.

He let his mind wander as his mood took hold of him. It took a step down; leading him to think about things you might read in a horror story. He wasn’t fully awake yet, but wished he could just pass out. In his 32 years of living there was more than one occasion when he had thought about passing out from experiencing unbearable pain. Here he was again wondering just how it might happen.

Would he simply close his eyes and fall down into a heap, only to wake a little later in even more pain? Or would his senses slowly be eliminated until sight itself was the last to leave him? He hoped that if the latter were true, pain would be the first sense to go. If that could happen he was sure he would have a smile on his face when he regained consciousness.

John also wondered if this is how people might feel if their eyes cracked, or what it might feel like to take a handsaw and cut off a limb. Sadly, he had thought about doing it before and wanted to many times, even begged his doctor to do it. As terrible as that sounds it did happen. Right now though, he thought he could do it for real. The pain he would feel from cutting off his leg surely couldn’t match the pain he was feeling in his knee. Every time his pain came around again he cursed himself for not having done it--remaining vulnerable to another day of torture. It was a pain he had grown tired of waking to, and every breath he took only made it hurt more.

Every beat of his heart was a sledgehammer connecting with his knee. The blast of pain would radiate up and down his body like an electrical current until it hit his brain, making his eyes water. To say he felt helpless was a huge understatement knowing another heartbeat was coming. There would be another after that; and on it would go--endlessly because his heart would not stop beating.

This sledgehammer would bring on other thoughts too, darker thoughts. The kind he would never tell others; the kind you kept hidden in dark places deep inside. They were the kind of thoughts that should be brought into the light and thrown away; or left in a gutter by the side of the road to rot and die. He never really buried them completely or threw them away either. He didn’t know why, but he just couldn’t bring himself to do it.

It was a beautiful spring morning in Maine and John was waking up. You might have been thinking that he was caught in some foreign prison, where torture is just how they communicate. Or that he was on some perilous expedition where he lost his footing and became the unconscious victim of gravity; and upon waking is waiting for the rescue crew that will never come, even though his return was long overdue ... but you would be wrong.

He wasn’t caught by some foreign dictator or militia group. He wasn’t even mountain climbing in some exotic country where he lost his footing, letting nature sink its teeth into him the way it does in so many stories of love and adventure.

John was simply waking up and for him, waking this way wasn’t new or unusual either. He was simply waking in his comfortable bed snuggled with warm blankets, soft pillows, and gentle heat. I know it sounds like it should have been pleasing for him, but his left leg was screaming over all of it; the way it had on countless other days.

He probably should have been in a hospital bed but would have none of that, and things would get worst for him this morning, much worst.

John was the baby of the family and born different than other boys. His body had everything baby boys are born with, except for a teeny tiny protein called factor IX (9).

Simply explained, factor IX is one part of a complex process that helps platelets, which are about 1 or 2 micrometers in size floating around in your blood, releasing chemicals. We’re talking small here. In comparison, a human hair is about forty to fifty micrometers, and a red blood cell is about six to ten. These chemicals allow those platelets to stick together and, as the process continues, they eventually form plugs known as clots.

When these plugs are formed on the outside of the skin after say--a cut on your finger, they dry while exposed to the air and you recognize them as scabs. If you hit your thumb while hammering a nail ouch, smashing the blood vessels under your skin. The blood won’t run out through a break in the skin and form scabs, but will leak out through the broken vessels, and pool under your skin.

As the blood is leaking out of these vessels, the platelets secrete their sticky chemicals allowing them to start sticking together at the break, eventually plugging the hole to stop the flow. You are left with a black and blue bruise which goes away in a couple of days as your body cleans up the mess. John came to understand the whole process simply as IX (9); and that teeny tiny protein became a big bad problem for him.

Just imagine, if you can, that these platelets didn’t stick together. They would never form a scab, to stop the flow out of your body, or a clot to seal a vessel under your skin. Then, 7% of your body weight would just run out of you or pool just under your skin.

One result of this would be the "life giving red juice" ending up where it didn’t belong, starving all that depend on it to live. Another would be that the pooling under your skin begins to stretch it. The extra pressure would cause pain, lots of pain, if allowed to continue; which of course it would, not having a sticky protein there to help form a plug.

Over time IX did cause many problems for John, especially to the parts of his body that moved. When the life giving red juice pooled in one of his joints, enzymes would eat away at the slippery cushion where the bones met. Eventually, the slippery surfaces would become raw bone; raw bone covered in pain receptors sending paralyzing signals directly to his brain. The signals were communications that screamed at him for allowing the slippery cover to be stolen from them. As the years passed it became harder to move his rusty joints. Some of his moving parts even stopped moving, making his body look old long before it should.

Being stuck in the cold New England winters, John took to thinking about sunny tropical islands and the warm breezes that he sometimes read about or saw in movies. He would picture himself on a beach, softly swaying in a hammock supported by palm trees, the sun melting away the pain in his joints; this always made him feel better.

The nights had been cold, as they tend to be this time of year, but the days were warm enough to melt the few remaining remnants of winter. The crocuses had already broken through their winter cover and the robins would soon appear to strengthen the promise of spring.

A spring morning in Maine can be a beautiful thing in the eye of a painter or the mind of a poet. Though John was neither, a painter nor a poet, he had seen and thought about all that was beautiful in his world.

He made a habit of noticing beauty and other things that gave him pleasure. He would store them in the back of his mind, taking them out during times when pain got the upper hand on him; pain got the upper hand a lot with John. Much more than a regular person would experience and certainly more than a man of thirty-two should.

His bed was warm and cozy for sure. But summer hadn’t come yet and there was still a chill in the air. A chill that made him think more of winter than spring. He didn’t think there was much of a difference from winter to spring or spring to summer except that there was less ice and snow to slip on, or drudge through. Waking up this way made John feel that most of his life had been a long cold winter. This spring, he was thinking, wouldn’t be much of a difference.

Paula, who was the better half of him, had seen it in him, felt it too. She often wondered why he didn’t ask for help or complain more. She did see him struggle but never interfered for fear that he would take it wrong. In her eyes, for someone with so many mountains to climb, he certainly was doing well. She believed that he was unique, with an inner strength that made him appealing. He always looked interested which made her feel comfortable; she thought it amazing how he would never show the pain he struggled with or any disappointment he suffered. John hadn’t shared much about his decaying joints or crumbling dreams, still hoping that someday somewhere at some time, he would find a different miracle then the one sitting on the table by his bed. But you know how miracles can be, all light and music hovering out there--just out of reach.

Getting his body to move in the morning was a difficult chore he came to expect. As he slowly moved each joint to waken them, something he spent more time on each year, they would start their chorus of screams. A bleed in one of these joints was just icing on the cake, letting him know how the Hammer was back in control. It wasn’t so much facing a cold slippery winter day, as bad as that was for him, it was more that each swollen joint was an invitation for the Hammer to join the chorus ... the painfully out of tune chorus.

But don’t think for a second that John didn’t find beauty in the cold harsh fist of winter. When he’d look out the window on those cold wind blown days and it would be snowing, not that regular every day snowing, but the few and far between magical snowing ... where the snowflakes were as big as Peace Silver Dollars. They would look so light and airy you had to wonder how they could fall at all. The heavens would be filled with them falling in such numbers that it was like looking at a white nighttime, where the snowflakes lit the sky.

On such a day, John could not stop himself from bundling up in his warmest clothes and heading out to walk. It was one of the few times he enjoyed walking. Even though he knew he would have to pay for his little excursion, he didn’t care. He would pay any price that was demanded of him, walking as long and as far as his body would let him. It was an especially wonderful experience when Paula and Max joined him. They would walk together, John and his little family without as much as two words between them ... lost in the beauty of it all.

John would particularly become lost in the wonder of it. His body would protest the cold, but he would ignore it letting the white nighttime swallow him up. He loved becoming a part of it, never choosing any predetermined direction or path to follow. When he found himself lost in unfamiliar surroundings, he would catch a line post or building corner to point the way home. But mostly, he tried to ignore everything that wasn’t full of wonder.

As he walked, he would raise his head looking up as the flakes fell toward him. He would let them fall on his face and eyes. They would get caught on his eye lashes remaining there for a second before they melted away, letting him see a kaleidoscopic pattern in the sky of flakes. He would gaze at it as he thought to himself, this is what a poet sees in his mind’s eye, and what an artist finds at the end of his brush. Then he would continue walking as if time itself had stopped just for him--and his little family.

Returning home was also wondrous for him after such a walk. As he removed the layers of protection between himself and the deadly cold, huge flakes would fall and melt as they succumbed to the warmth that was lethal to their beauty. Watching the demise of their one of a kind splendor always left him speechless. He would later sit by the largest window he could find, wrapped in as much wool as he could lay his hands on, and while sipping a warm drink watched the show. A wondrous show of nature right here in a little part of the world where just such a thing was possible.

John was born and made his home in Biddeford, a small mill town on the East coast in southern Maine. He thought that most people were connected in small towns like this and speaking to them was like speaking to family.

He was the youngest of four children sharing his parents with two sisters, and a brother. They were born girl, boy, girl, boy. They were a family connected even after his eldest sister married and moved away. She had been the first to leave the nest making him feel sad when she left; but they kept in touch, writing to each other often. It was then that he first came to realize how families do separate, create families of their own, and sometimes move away. He never got over not having her around, especially during family celebrations like birthdays and weekend get-togethers.

John was born into a family culture that might seem foreign in today’s modern world. His mom was first born, and after completing sixth grade she was expected to stay home to help raise the nine children her mother would give birth to. There had been more births but some of the children were stillborn. She wanted to remain in school and protested of course, but children didn’t have a voice back then, especially female children.

She was born in Maine, in fact the very same city John now lived. Her parents were Canadian immigrants from eastern Canada. Though they lived in the United States all of their adult life, they never wanted to give up their Canadian nationality to become American citizens. Their French Canadian heritage was rich in this part of Maine. He could remember how the family would get together for a réveillon; a large family holiday feast and party that would extend long into the night. Of course, the winter holidays always included traditional tourtière: a spicy meat pie.

John always remembered the Saturdays when his mom would bake beans and homemade bread; a tradition in the French speaking homes around this small town. The beans would be set to soak in water the night before, waiting for her to start them early the next day. The house would fill with the fragrance of the all-day recipe, making your mouth water every time you came into the house.

John’s father was born in a small village southeast of Québec, Canada. There were no firm rules about school attendance in his village back then and he never returned after completing the second grade.

After celebrating his tenth birthday, his father brought him to the United States and a work camp where he would learn all about the logging trade. He worked as a lumberjack with his dad until he joined the Army during World War II. It was then that he became a United States citizen and learned to speak