Faces of Huntington's

What others

are saying about

Faces of Huntington’s:

In these pages, Carmen has captured the indomitable human spirit. Faces of Huntington's is a must read whether you are in a family affected by HD, a medical professional, or simply someone who cares.

Jim Pollard, Executive Director

Mediplex Rehabilitation and Skilled Nursing Center of Lowell

Faces of Huntington's is a thoughtful, candid, brutally honest, excellent resource book. This thought provoking book is a great resource, and a source of comfort for anyone who is working with or has a chronic, terminal, or disabling disease. Eye opening from the employer perspective, Faces of Huntington's is a library must for an occupational health practitioner.

Terri Janda, RN BSN, Corporate Nurse Coordinator

Occupational Health Nurse Blue Cross Blue Shield of Kansas

The stories and poetry in Faces of Huntington's describe issues and concerns that many families with HD face. This book will provide comfort, humor, and hope for those who care for people with HD. This book will also be useful to student and practicing health professionals who assist families in coping with the long term effects of Huntington's Disease.

Janet K. Williams, PhD, RN, CPNP, CGC

Associate Professor, The University of Iowa

Past-president, International Society of Nurses in Genetics

Faces of Huntington's leads us out of the fray of misunderstanding and stigma of neuropsychiatric disease, and documents in parallel, the journey to hope and consolation in the fellowship of those familiar with and suffering from the disease. It generates the perspective of personal understanding and empathy for its readers, sustained by not a singular style but the determined voices of many. These writings challenge our own struggles and sensitively convey both the measure of humanity which is afflicted, and the courage of those afflicted.

Jeffrey I. Bennett, MD, Director

Neuropsychiatry Clinics and Consultation Psychiatric Services

Assistant Professor of Clinical Psychiatry

University of Chicago Hospital

This book is about ordinary people living extraordinarily heroic lives. It is a book needed by all, especially those of us in medicine, who, for so long, this disease has forced to face our own uncomfortable impotence... forced us to face our own, personal defeat. Bravo to Carmen, author of more than a book, author of so much to so many.

Philip S. Backus, M.D.

Faces of Huntington's is not just about people with Huntington's. It is about nearly all of us, and about chronic, catastrophic, and terminal illness which we have, are, or will encounter in our lifetimes.

Catherine Puhr, ARNP

Faces of Huntington’s

Carmen Leal

Copyright 1998, 2012 by Carmen Leal

Smashwords Edition

All Rights Reserved. No part of this publication may be reproduced, stored in a retrieval system or transmitted in any form or by any means – electronic, mechanical, photocopy, recording or any other – except for brief quotations in printed reviews, without the prior permission of the author.

All Scripture quotations, unless otherwise specified, are from the New International Version of the Bible (Copyright 1973, 1978, 1984 International Bible Society. Used by permission of Zondervan Bible Publishers. All rights reserved.)

ISBN: 978-0-13-041717-6

Cover illustration by Ruth Hargrave

Book Cover Design by Vickie Fraser

I dedicate this book to my husband, Dave, who has Huntington's Disease. Without Dave, this book would never have been conceived of, or written. I also thank my wonderful sons, Nicholas and Justin, for being so special and helping me in ways of which they are not even aware.

This book is also dedicated to my brother Merrill. Though he didn't have Huntington's, Merrill fought his own battle with disease. Merrill’s fight embodied much of what this book is all about. He fought the good fight with hope, faith, joy, love and so much more. I know, when he was done fighting, he was told, Well done, good and faithful servant.

Lastly, this book is dedicated to all of the caring people in my life that helped me get to this point. You know who you are.

Photo: Nicholas Lucien Hahn

For all those heroes of the Huntington’s fight

who are no longer with us.

"I wanted a perfect ending. Now I’ve learned, the hard way, that some poems don’t rhyme, and some stories don’t have a clear beginning, middle, and end. Life is about not knowing, having to change, taking the moment and making the best of it, without knowing what’s going to happen next. Delicious Ambiguity."

—Gilda Radner

Table Of Contents

Acknowledgements

Forward by Dr. Richard Dubinsky

Preface

Introduction

Searching for a Cure II BY GABRIELLE HAMILTON

If He’s the One Who’s Sick, Why Do I Feel Like Dying? BY CARMEN LEAL

1 My Story BY CARMEN LEAL

2 Faces of Encouragement BY CARMEN LEAL

This Net BY LEON JOFFE

It’s the Little Things BY SUSAN GARRETT

The City BY KEN SHEARS

Symphony of Unity BY HAROLD

And Then There Were Two BY SHIRLEY PROCELL

Untitled BY GABRIELLE HAMILTON

3 Kym’s Story BY KYM NICHOLSON

4 Faces of Love BY CARMEN LEAL

The Tears Never Dry BY SHIRLEY PROCELL

The Porch BY SHIRLEY PROCELL

I Will Fight BY JEAN ELIZABETH MILLER

Farewell BY KIM SIGNORET

I Really Do Love You, Mom BY SANDY L.

Telling the Children... BY JILL ALAM

Sights and Sounds BY BRENDA PARRIS SIBLEY

The Bracelet Promise BY CARMEN LEAL

Treasured Memories BY JEAN ELIZABETH MILLER

To My Sister ANONYMOUS

To My Child BY GABRIELLE HAMILTON

5 Lou's Story BY LOUISE A. WILKINSON

6 Faces of Humor BY CARMEN LEAL

Laughter BY GABRIELLE HAMILTON

7 Christopher's Story BY CHRISTOPHER CLINE (LEGAL ADOPTED NAME) R.W. HAVENS (BIRTH NAME)

8 Faces of Family BY CARMEN LEAL

Mother BY GABRIELLE HAMILTON

Leo's Story BY LEO WAYNE PIKE

You Are Always There BY KELLY ELIZABETH MILLER

I Will Always Love You BY BRENDA PARRIS SIBLEY

What I Wish BY ANDREA HADDIX

Waiting For the Next Chapter BY DONNA T. DUFFY

A Mother's Love BY SHIRLEY PROCELL

A House Divided BY MONTSE TORRECILLA

Dear Child BY JEANNINE DUTTON

The Wild Plant BY ELTON D. HIGGS

Memories of Dad BY MARY EDWARDS

Stories of Fear, Stories of Love BY CARMEN LEAL

Our Stories BY ALBERT J. CERVI

My Family, My Life BY JIM GILES

My Daughter BY JIM GILES

9 Christine's Story BY CHRISTINE PATERNOSTER

10 Faces of Heroes BY CARMEN LEAL

The Walnut Wall

The Kites BY CARMEN LEAL

Jim Sauer, PhD, pHD BY MARY S. PRICE

My Dad, My Hero BY SHANA MARTIN

A Family of Heroes BY DIANA FISHER

He Made All the Difference BY TOM GILLIHAN

Thank You, Jill BY MARSHA INSTONE

Another Kind of Hero BY GINNY SILVER-KOPOLO

A Mother to Four BY PAT PILLIS

A Chance Encounter BY MURRAY DANIEL THOMPSON

To Pitta BY LEON JOFFE

11 Chuck's Story BY CHUCK YOUNG

12 Faces of Faith BY CARMEN LEAL

Trusting God BY CARMEN LEAL

Dear God BY SHIRLEY PROCELL

With Childlike Faith BY LORETTA DELAUTER

Heart For Any Fate (Psalm) BY BRUCE P., MIAMI, FL

A Prayer BY JEAN E. MILLER

Dr. Huntington, I Presume? BY GREG

Why Me? BY CARMEN LEAL

An Answer to Prayer BY CARMEN LEAL

A Remembrance of Wayne (FOR MY BUDDY, WHOM I NEVER MET) BY HAROLD

13 Trisha's Story BY TRISHA GAUGHAN

14 Faces of Forgiveness BY CARMEN LEAL

Apology BY SHERRY LEE

Till Death Do Us Part BY RUTH HARGRAVE

Always on My Mind BY MARY CHRISTOPHER

This Man Was Me BY SHIRLEY PROCELL

15 Tom's Story BY TOM GILLIHAN

For Helen BY SHIRLEY PROCELL

16 Faces of Hope BY CARMEN LEAL

What Huntington's Cannot Do ANONYMOUS

He Gave Us Today BY SHIRLEY PROCELL

Reflections ANONYMOUS

Where There is Hope BY GARY

Hope for Carolyn BY NOEL CROWSON

With My Feet Beneath Me BY GABRIELLE HAMILTON

...Just For One More Day BY JEAN ELIZABETH MILLER

Huntington's Disease: Friend or Foe BY ROBERTA BRINK

Is There No End? BY KELLY ELIZABETH MILLER

What is Hope? BY CAROL FLESSEL

What a Wonderful World BY BRENDA PARRIS SIBLEY

17 Shana's Story BY SHANA MARTIN

18 Faces of Huntington's BY CARMEN LEAL

HD and ME BY GABRIELLE HAMILTON

To Ease the Pain BY LEON JOFFE

The Search for an Answer FROM WRITINGS BY DOREEN GALANTE

Relieved BY GABRIELLE HAMILTON

Purposeful Ambiguity BY MARY PRICE

Peace of Mind BY JASPER SWART

An End to Secrecy BY SHIRLEY C.

Lies BY GABRIELLE HAMILTON

Walking on Life's Fence BY PAMELA FOYE

Growing Old Fast BY BRENDA PARRIS SIBLEY

A Caregiver's Bill of Rights ANONYMOUS

A pHD's Bill of Rights BY LOU WILKINSON

Alternative Stages of Huntington's Disease BY JIM POLLARD AND ROSEMARY BEST

A Brand New Day BY SHIRLEY PROCELL

Appendix

Acknowledgements

WRITING the dedication page of your book is a bit like accepting the Oscar for a hard earned Academy Award performance. You have so little space and so very many people to thank. I guess that’s why the acknowledgment page was created – to thank all the people who helped to make the book a reality.

Without my Hunt-Dis family, this book might have been written, but it would not have been the rich, full one that it is. Thank you to this family that has been so much a part of my life for the last two years, and has seen me through each step of this insidious disease. Your openness, your willingness to help in any way you can, and your loving friendship, has often been what has gotten me through.

Again, the book might have been written without my gifted editor, but it would not have been quite as easy or wonderful. Thank you, JoAnn Zarling, for caring enough about the art of writing, myself, and thousands of people you don’t even know, to help me create as fine a book as possible.

I remember when Dave was first diagnosed with Huntington’s Disease. I went to my pastor and cried buckets of tears expecting him to tell me, somehow, that it would all go away and life would be normal. Darryl Keane did not try to make it better, but instead he said something I have thought of many times in the last three years. I know you don’t want to hear what I have to say. But I can see beyond where you are now. I can read the books you will write, hear the songs you will sing, and see the groups to which you will speak.

His statement did nothing to lift my wretchedness and I left the office feeling more bereft than I had ever felt in my life. On top of the sadness and grief, I was filled with anger. I did not want to see beyond where I was. I wanted to go back to where I had been before we learned the devastating news. Thank you, Darryl, for loving me enough to be honest. You knew I had been given the gifts of writing, singing, and speaking, and that at some point all of my life’s experiences would come together for a purpose far larger than mine. They say no one is irreplaceable, but that simply isn’t true. I miss you more than you can ever imagine. Thank you.

I have to thank my son Nick for bullying me into going on to the Internet when I thought it was a waste of time. Thanks to my technologically gifted son, I found information on Huntington’s Disease, and a real family there as well. Nick also taught me how to create a web page and rarely laughed at me when I made mistakes. Nicholas and Justin both had to suffer the loss of their own computer time and the irritating tap, tap, tap of mother hunting and pecking late into the nights.

One evening, as I was chatting with a friend on the computer, we discussed my dream of writing a book for and about people with HD. This lovely man, who has a wife with HD, asked me what it would really take to get this book written. Sensing his sincerity, I told him I needed a new hard drive and a faster modem. Because I had so little space left on the computer, I could not write the book without a larger drive. Now, I won’t mention his name or he would be deluged with requests for computer equipment, but thank you doesn’t even begin to say what I mean. It wasn’t just the modem and hard drive, it was that you believed in me, and were willing to spend money out of your own pocket to see this much-needed book be written.

There were so many people who sent me stories, quotes, ideas and good wishes, both through regular mail and E-mail. Obviously, there was no way I could use all that I received. Thank you to you who opened your lives to me. Much of this book was really written by those who cared enough to share. I just edited the submissions, and put them all together in what I hope is a readable format. In editing the many quotes, poems, and stories that make up this book, I worked to make only the minimum of changes. These changes in grammar and spelling were made to make what was being expressed more easily understood. The poetry in this book is written from the heart and was chosen to be included because of content, not perfection. I tried to retain the flavor of the pieces and for that reason, when you read this book, you will find different styles of writing.

Forward

by Dr. Richard Dubinsky

HUNTINGTON’S Disease was first described by George Huntington in the late 1800s. He had observed the disease when, as a boy, he made rounds with his father, on horseback, to visit the families that were affected by this illness. In his original report, he vividly described the choreaform (dance-like) movements, the problems with emotional control and thinking, and the inheritance from one generation to the next.

Over the last century, scientific knowledge of Huntington’s disease has increased dramatically. The most striking finding in the brains of people who have died of Huntington’s Disease is the loss of spiney neurons (nerve cells) in the caudate nucleus, a portion of the center of the brain. These neurons provide the connections between other nerve cells in the brain. Without these connections, we have difficulty in making everyday movements such as walking or eating. These connections are no different from our social connections that allow us to function in our society. Without them, we are isolated and clumsy.

We have known for many decades that Huntington’s Disease was usually inherited from a parent who had Huntington’s Disease, yet there were always cases where an affected parent could not be found. When Huntington’s Disease was inherited from the father, the age of onset was earlier in the next generation, yet when inherited from the mother, it was about the same.

To better understand the inheritance of Huntington’s Disease, a large scale study was undertaken in Venezuela, where Huntington’s Disease is exceedingly common in the Lake Maricaibo region. In 1983, the genetic region for Huntington’s Disease was found by this team. Ten years later they sequenced the gene and we could begin to understand the mechanisms of Huntington’s Disease.

All of us have two copies of most of our genes, one inherited from our mother and one inherited from our father. We each have two copies of the INIT 15 gene, the gene that, when abnormal, causes Huntington’s Disease. In Huntington’s Disease, there is a stutter in the genetic code. The sequence CAG is repeated too many times, causing the protein made from the gene to be incorrect. At least three different mechanisms have been found where this abnormal gene product could cause Huntington’s Disease.

The puzzling fact remains that the gene is abnormal from conception, yet the disease occurs only in the last one third of the person’s life. This is also the same for those who contract the juvenile strain of Huntington’s Disease, and those who have abnormally late onset. Though symptoms generally appear between thirty and fifty years of age, they have also appeared as young as two and as old as seventy, and progress over a ten to twenty-five year period. Based on these findings, the first multi-center studies of treatments, designed to slow the progression of Huntington’s Disease, are currently underway.

Another outcome of the genetic research is the ability to tell if someone has the Huntington’s Disease gene. In essence, a person can peer into their future to see if they will develop Huntington’s Disease. The decision to undergo testing is a difficult decision to make and can have far reaching consequences for the person and their family. Until a treatment is available that can slow the disease or delay the onset, pre-symptomatic testing will remain a very individual decision.

Despite all the research that has been completed and the studies still underway, Carmen’s book reminds us that Huntington’s Disease is not an entity in itself. It is a disease that affects people and their families and caregivers. Too often in the practice of medicine, we lose sight of the patient because of the disease. Clinicians and researchers, as well as caregivers and family members, need to be reminded that Huntington’s Disease is an illness that affects people. The poignant and sometimes painful stories in Faces of Huntington’s help us to catch a glimpse of the faces of those affected by this disease. By meeting the people in this book, we can learn lessons about Huntington’s Disease that are as important as the research that has found the cause of Huntington’s Disease.

Richard M. Dubinsky, M.D.

Department of Neurology

University of Kansas Medical Center

Preface

FACES of Huntington’s is in no way a medical or scientific book. It is important to remember that there are no two people with this disease who have exactly the same symptoms in the same order. For instance, some people suffer with a tremendous amount of chorea, others barely have any. Additional symptoms may be equally different. The stories shared in this book are personal, and no one should project themselves or a loved one into any of the scenarios. This book is simply an honest look, through many eyes, of people who have and are living with HD.

In 1993, my husband was diagnosed with Huntington’s Disease. We had never heard of HD, and when I went to the library in search of information, there was very little available. My first Huntington’s convention in 1994 helped to a degree, but I still felt isolated by the lack of people who had HD in my area. I was also still frustrated that there was so little written for and about the people who daily fight the HD battle. In late 1994, my son introduced me to the Internet, and there I found more information than I had found in a year of searching bookshelves.

In this book, there are some terms that relate to the Internet. I use them almost without thinking now, because the Internet has become such a part of my life. Knowing there are many of you who are unfamiliar with this medium, several common internet terms are listed below.

For those who would like to know more about the available information on Huntington’s Disease on the Internet, there is a reference section at the end of this book. I also recommend an excellent book called, The Beginner’s Illustrated Internet Directory, by Betty Shulman. This easy to understand book will help you avoid becoming roadkill on the information superhighway. The book includes a wealth of basic knowledge about the Internet and should answer many of your questions. If you are interested in ordering this guide, call 1-800-444-2524.

Hunt-Dis is an on-line discussion group. This is a mailing list that allows individuals who are interested in all aspects of HD to communicate with each other. All you need in order to participate in a mailing list is E-mail access. E-mail stands for electronic mail. There are currently over three hundred people around the world on the Hunt-Dis.

The Internet is a vast network linking thousands of computers all over the world. These computers are physically connected to each other using special phone lines.

To explore all the Internet has to offer, you need to be on-line. When your computer establishes a connection through the modem, you are on-line and may begin using the various services on the Internet.

One last term used in a number of places in the book is pHD. This is a phrase that was coined on the Hunt-Dis as a form of short hand. pHD stands for person with Huntington’s Disease.

Carmen Leal

Introduction

Searching for a Cure II

BY GABRIELLE HAMILTON

Searching for a cure

to save myself

from myself

or inevitable betrayal

by the slow mutiny of

my arms, legs and mind.

Searching for a cure

while others hope

because I know what lays ahead

and I have faith

in the dreams we’ve planned

for tomorrow and ever after.

Searching for a cure

so that your love for me

will not be spoiled

by the monster

that is Huntington’s Disease

whose hold on me tightens

each year

despite our valiant attempts

to remain at large.

Searching for a cure

for others like me

who sometimes wish

they’d never been born

to suffer

this weighty burden.

January 12, 1996

If He’s the One Who’s Sick, Why Do I Feel Like Dying?

BY CARMEN LEAL

It was our third wedding anniversary. The day flew by at whirlwind speed as I went to work, shuttled the kids to and from soccer, cooked dinner and went about the daily routine. Having come out of a previous marriage fraught with anger, erratic schedules and a myriad of other problems, I focused on the wonderful solidity of my David. He did not have a charismatic personality nor would he ever earn substantial sums of money. But he adored me, was a hard worker, a romantic, and a strong Christian. I spent some of that anniversary day contemplating a long, secure marriage.

An hour after I thought David would be home from the doctor, he dragged himself into the house. The drawn look on his ghostly-pale face was only the first indication that something was extremely wrong. I tried to discover what had caused him to appear so much older now than he had just that morning. Without knowing what was wrong, I somehow understood the severity of the moment.

Instead of the joyous celebration, plans and gifts were abandoned as we began to discuss what we were facing. David seemed unable to articulate the diagnosis or even the thoughts churning in his mind. I was stunned by the news. My first response was to enfold my best friend in my arms as our separate thoughts became entwined. As the tears coursed down my face, everything within me silently screamed. Our anniversary was to be a time to celebrate our love, a time to plan for our future. The timing seemed a cruel hoax that I could not understand.

My sons bounded into the house, unaware of the tragic news, and we somehow went through a charade of our nightly routine. With the homework done and the chores completed, Nicholas and Justin prepared for bed. I silently begged for strength to get through prayer time with each son.

The nightmare of our anniversary finally ended and we sought refuge in each other’s arms. Lying in bed, I knew one thing could not change. As angry as I was with this turn of events, I knew we had to continue to pray to God. I searched my mind for some praise to offer God as a way of starting off our nightly prayers. I thanked Him for three wonderful years of marriage, took a deep breath and somehow continued. After what seemed an eternity, I fell into an uneasy sleep.

I had never heard of Huntington’s Disease, nor was I particularly knowledgeable about the brain in general. I began to search for information, thinking somehow that with knowledge there is power. I just knew that if I could talk to the professionals I could get a handle on this thing. We would spend every penny, make every sacrifice, if only Dave could be spared.

But no matter how much I wanted it to be different, there was no treatment and no cure. It seemed David had lived since birth with the time bomb of the gene which causes Huntington’s Disease. Unknown to any of us, David’s mother had been afflicted with Huntington’s. As the facts became real, I learned this would be a long-term disease. I contacted the Huntington’s group in New York and was soon inundated with information. I began to learn far more than I ever wanted to know.

The pieces of the puzzle that was Huntington’s Disease slowly shifted into place, unasked questions were answered, and the completed picture became painfully clear. The constant movement wasn’t nerves like I thought, it was HD. So was the forgetfulness, the indecipherable handwriting, the drunken walk, the slurred speech and innumerable other physical signs. The irrational anger that exploded unbidden, the numerous falls, and the poor way of handling finances were just three other signs. The list continued to grow until I was consumed with guilt that I had not forced him to see a doctor sooner. The deadly progression of HD had set up camp in his brain and nothing would persuade it to break ranks.

A series of bad falls had been the catalyst to seeing the doctor and to the ending of David’s working days. Looking back, HD was probably the reason for his lack of assertiveness and, consequently, his halt midway in the climb up the corporate ladder. HD probably helped to end his previous twenty-one-year marriage. I know it affected his disciplinary skills along with his ability to get a new job.

At forty-three years of age, David shuffles through his days like an old man of eighty. As his appetite and ability to swallow continue to decrease, so does his weight. I am never sure who lives in his body. Is it my best friend? Or has the obstinate child who replaces David come to visit? His childlike tantrums coupled with his helplessness bring me to tears. Sometimes there is a violent person in his body, and at other times, it is an old man who sits and stares into space. Each of these people throws me into a turmoil that ends with a cry to God to please give me back my husband.

Every family who experiences the shock of HD reacts in a different way. HD is not only a disease of the afflicted; the entire family suffers. For my family, the challenges of step-parenting were thrown into disarray as David slowly lost control of his authority. I increased my work load at the office and at home. Ensuring that the car is in working condition is my total responsibility now that David no longer drives. That in itself is a problem because I have to run every errand and take the children to each school or sports event. The uncertainty of the future in terms of finances and how I will care for David is a panic which never leaves my mind.

The worse part of HD, for me, is losing my best friend in bits and pieces. I find myself erecting barriers around my heart to steel myself against the inevitable loss. Just when I persuade myself that the changes in David are permanent, he’ll have a good day when he is close to being his old self. I fall in love with him all over again; then I watch him slip away. The grief that washes over me brings me to my knees to cry out my anguish to God once more.

Throughout this time, I have learned that as much as I love my husband, God loves Him even more. I know that there is a reason why we are being allowed the privilege to walk through this together, though daily seeing our suffering as a privilege is no easy task.

I have often asked, Why must I suffer? My suffering does a marvelous job of keeping me from pride. My suffering also is a constant reminder of just how much Christ suffered for me. What I am feeling is minuscule by comparison.

I am learning to understand that my suffering is only for a brief time when compared to eternity. There is a better time beyond earth. How I