svb Adult Cystic Fibrosis Committee

of Quebec (ACFCQ)

Interview

Health

Psychology
October 1999

24
 Contents

Editor A Word from the President

Valérie Mouton
2
Researcher
Laval de Launière Editorial
Translators 3
Lucie Ranger, team leader
Mimi Tsang Interview
Stéphanie Wells 4
Revision Committee
Mark Shields
Kim Smiley Reflections
Above All, an Individual 7
Revision Committee, French Issue
Josée Mailhot, Team Leader Ahead of the Game 9
Michèle Côté
Mireille Jean
Health
Columnist Antibiotic Therapy : at the Hospital or at Home 12
Michel Ruel, M.D.
Lung Transplants for Patients with Cystic Fibrosis 14
Graphic Designer
Isabelle Godin Treatment of Cystic Fibrosis in Denmark 18

This newsletter was made possible

thanks to the Adult CF Committee of Research
Quebec (ACFCQ), the Centre d'orienta- Update on Immunocal
TM
Research (HSM
TM
90) 21
tion professionnelle et d'aide
psychologique du Québec enr. and the
generous participation of our sponsors. Psychology
ACFCQ About Love 22
The head office of the Adult CF
Committee of Quebec is located at: Is Suffering Really Necessary? 24

629 Prince Arthur St. West

Montreal, Quebec Colums
H2X 1T9 Health
Telephone: CO2 and Oxygen Flow 26
Montreal Area
(514) 288-3157 Photosensibility and Intraveinous Antibiotics 26
Elsewhere in Quebec
1-800-315-3157
Anti-Viral Vaccines 27
Fax (Montreal Area): A-Zimut
(514) 987-1301
E-mail: Medical Insurance Abroad 28
cpafk@videotron.ca Antibiotics for Multiresistant Organisms 29
Web site:
www.cpafk.qc.ca
Communic-Action
30
 A Word from the President

Dear Friends,

As you know, the Adult CF Committee of Quebec (ACFCQ) is a democratic organization whose mem-
bers enjoy the right to vote in elections every two years. This provides new representatives from the
various regions of Quebec with the opportunity to join the team and breathe new life into existing ideas.
In this way, the ACFCQ will always maintain its vitality.

The April and May 1999 elections brought about changes within the organization. As your new presi-
My mandate dent, I will be continuing the work of great predecessors, such as Marc Fortin, Josée St-Pierre, Élise
d'Anjou, Michel Paquette and Roger Barnard. Over the past 14 years, these individuals allowed the
voices of Quebec’s CF adults to be heard. My mandate is not easy, but it is an interesting challenge. The
is not easy, but professional experience I acquired through training in social services gives me a psychosocial outlook,
which I hope will serve the interests of the ACFCQ.

it is an interesting I have been involved with the ACFCQ for four years. Getting to know other adults with cystic fibrosis has
made me realize the importance of having an association. I believe that the strength of a group lies as
much in the many doors that it opens as in the wealth of opportunities it provides for exchange. Suffering
challenge can be alleviated simply by sharing it with others. The ACFCQ offers just such an opportunity because it
has a wonderful, dynamic team where everyone has a voice.

The ACFCQ’s main objective is to promote the quality of life of every member. In concrete terms, this
means that the organization’s mandate is to defend issues that have a direct impact on the daily
existence of CF adults, ensure visibility and representation before decision-making bodies, listen to its
members’ problems and try to solve them. We are lucky to belong to a committee that is known inter-
nationally as a leader in defending the rights of adults with CF, and it is through us that the ACFCQ exists.

I am very proud to wear the ACFCQ’s colours because I sincerely believe in the value of our association.
Today I would like to assure you that I and the ACFCQ’s administrative team: Christian Auclair, Marcel
Barette, Roger Barnard, Caroline Caron, Dominique Cerutti, Marc-Éric Desmarais, Mireille Dubé, Louis
Dumoulin, Aline Fredette, Murielle Gagné, Sonia Grenier, Sylvain Lavoie, Martin Lemire, Valérie Mouton,
Marie-Hélène Roger, Yves St-Laurent and Laval de Launière, will continue to promote the ideas and opin-
ions of Quebec adults with cystic fibrosis.

Regards,

Anne Savard

2
 Editorial

You probably remember the editorial of the previ-
ous issue of SVB, which, for good reason, was
more like the "Help Wanted" section of the classi-
fied ads. Taking his cue from a relevant Baudelaire
excerpt, Christian Auclair caught my attention
with his questions: "Do you want to chase the
demons? Transcend our ridiculous fates? In a word,
do you have the guts ?" So I asked myself whether
I had the guts, whether I had the audacity and
temerity to take up the position of Editor-in-Chief.
I decided to accept the challenge. And so today, as
new Editor-in-Chief, I deliver this editorial to you.
Quebec’s CF adults may continue to be the best
informed.
As you know, SVB, which was first published in
French, has been available in English since Issue
No. 20. This has given us privileged contact, not
only with the CF adults in Quebec and Canada,
but, thanks to the Internet, with those the world
over. We have a lot to give them, and their expe-
riences can be very valuable to us. The testimony
of a young German adult with cystic fibrosis
appears in this issue.

My name is Valérie, I am an ambitious 20-year-old Before closing, let me reiterate my determination

woman with zest for life, and, like most SVB rea-
ders, I am stuck with cystic fibrosis. My surname,
"Mouton," may be familiar to some of you. I am,
in fact, the "daughter of the other one," Denis
Mouton. I am very proud of what my father
achieved in the Quebec Cystic Fibrosis Association
(QCFA) and the Canadian Cystic Fibrosis
Foundation (CCFF). Now it is my turn to get more
involved with my fellow travellers. Constantly con-
fronted with our reality, that of CF adults, I have a
slightly different slant on the disease than my
father did. My growing awareness of the trials of
CF adults emboldens me to direct my efforts to
improving their quality of life. This is obviously not
an easy task, but because I firmly believe in the
ACFCQ’s actions, I am convinced that by focussing
my energy on its goals, I will achieve my own.
to improve the fate of the CF population. By pool-
ing our energies we can achieve great things.
These lines mark the beginning of my commit-
ment to SVB. Although the position itself repre-
sents a major challenge, succeeding a man like
Christian Auclair makes the task even more
demanding. And I would like to thank Mr. Auclair,
on behalf of us all, for the ten years in which he
generously devoted precious time to the creation
of SVB. In the hope that I may meet the expecta-
tions of all SVB readers, I pick up where he left off
and enthusiastically embrace this new adventure.
Valérie Mouton

I know that improving I decided to join the SVB team because I know that
improving our well-being is largely dependent on
the choices we make. But we must be able to
our well-being is largely make informed choices. And this is SVB’s main
objective: providing the most information possible
so we can weigh our decisions and gain better
dependent on the choices
control over our environment. SVB is directly con-
nected to improving the quality of life of each one
3
we make of us. You will soon realize that my plan consists of
maintaining continuity: like the team, I will do
everything I can to keep improving the quality of
our newsletter—your newsletter—so that

Transplantation :
A Unique Experience
Stéphanie Wells has cystic fibrosis. On December 3, 1998, she received a double lung transplant
Interview
by at the Notre Dame Campus of the University of Montreal Hospital Centre.
Josée
Mailhot
What made you decide to undergo a
lung transplantation ?
It happened very quickly: the surgery took place
only six and a half months after Dr. Jeanneret
(Director of the Cystic Fibrosis Clinic at Hôtel Dieu
Hospital in Montreal) and I agreed that it was time
to start the process leading to transplantation. I
knew that transplantation would be inevitable one
day, but as long as I did not need to take oxygen,
it remained a remote, somewhat unreal event.
Personally, I consider myself lucky that everything
happened so fast, but I know that some people
would have preferred to have more time to think
it over and get used to the idea of undergoing
such an operation. My state of health had deteri-
orated quite suddenly: in only a few months, my
quality of life had changed dramatically. My respi-
ratory capacity (FEV1) had been stable for about
ten years (about 45%) and in seven months it
dropped to between 16% and 20%! From the
time oxygen became a necessity, in May 1998,
transplantation became essential, and the sooner
the better. So I did not really go through the
periods of doubt that many people with CF expe-
rience, nor did I have time to hesitate. From one
day to the next (literally), I was dependent on an
oxygen tank 24 hours a day, and I had no strength
to do anything at all.
4
What was it like to wait for a com-
patible donor? Were you apprehen-
Interview
sive, did you think about the possi-
bility of dying ?
From the time I was officially registered on the
Quebec transplant list at the end of July 1998, I
waited exactly four months, which is very little
compared to the usual nine to twelve months. I
was optimistic and never became discouraged.
Some days were hard, but in general, I kept my
hopes up at all times. The rareness of my blood
group (AB) was a double-edged sword: the aver-
age waiting period would be shorter because I
was the only one on the list, but there was also the
chance that there would not be a compatible
donor since less than 3% of the population
belongs to the AB blood group. It may seem
strange, but it was only just before the operation
and immediately afterwards that I experienced
fear of dying, despite the fact that all was going
well. Throughout the wait, which was the most
dangerous period, I disregarded the danger and
concentrated on hope. It may seem like foolish-
ness, but this attitude was helpful, allowing me to
go through this period in better spirits. At the risk
of repeating a cliché—but in this case, one that is
so true—the support of my family and friends was
essential. I needed a lot of physical help and psy-
chological support; feeling surrounded and loved
made things so much easier. And I will go as far as
to say that without them, I would not have had
the courage to wait. My immense determination
was matched by their tremendous support.
 What were your motivations ?
I had many different kinds of motivation. Of
course, given that I am only 26 years old, the
greatest one was simply to continue living. But
one thing that helped me considerably was think-
ing about the future and making lots of plans. I
could not wait to finally start working after the
many years I had spent studying. And it is a great
source of satisfaction to be able to do so, part-time
for now, five months after my transplant. I also
wanted to cycle, which I started doing last spring.
I think it is important to think of the future, and for
me and my family, it was a great motivating force.
We often spoke of what we would do together.
Many people are apprehensive about
the operation and the days that fol-
low. Can you tell us about that ?
The most stressful time for me was when the sur-
geon, Dr. Ferraro, called in person: I will never
forget a single detail of that moment. Despite my
determination and my luck at finding a donor, I
became extremely nervous. Everyone reacts differ-
ently, of course, but despite the fact that I was
prepared for this call, the jitters won out. Once at
the hospital, while preparations were being made,
I calmed down. The stress returned a few minutes
before I was anaesthetized. I do not remember
anything about the day of the operation, which
lasted ten hours. I remember the next day.
Intubation, which scared me, was unpleasant but
tolerable. Once the respirator was disconnected,
even though I remained connected in several
places and still had an oxygen mask, I could at
least breathe on my own. I cannot say that my first
“puffs” of air were spectacular: it took a few days
before I could breathe with ease. In fact, I had to
relearn to breathe normally because for many
years my breathing had been quite shallow. After
the transplant, I had to breathe deeply, from the
abdomen, which did not seem natural to me.
What about the pain ?
The first days I felt no pain because of the epidur-
al anaesthesia, which is a local body anaesthesia.
When it was removed, I was in pain for about two
days, and afterwards everything went well. The
pain was kept under control with sedatives. Pain is
different for everybody, but in my case, it was easy
to bear. I recovered quite quickly. Four weeks after
the operation I went home, and if it had not been
for a digestive problem, I could have been released
less than three weeks after the transplant because
my lungs were working very well. A combination
of many factors resulted in a very lazy stomach,
but this has since been corrected.
What are the side effects of anti-
rejection medication ?
There are many, and they vary according to the
individual. I did not experience very many side
effects, but at first, I had headaches, trouble sleep-
ing and my digestion was very sluggish. But this
was nothing in comparison with my previous state
of health; and today, five months after the trans-
plant, I have no side effects and I feel great. I
almost forgot—because it seems like a mere
detail—there are some side effects related to body
image. Prednisone causes a little swelling in the
face, making it rounder (which is something we
are not used to!) and promotes hair growth
(which is easily dealt with). There is also some
scarring, since we are talking about body image,
but this consists of a surprisingly thin horizontal
line and small scars from the drains in the chest
cavity. But this is nothing compared to the gaunt-
ness, the pallor, the need for oxygen that were my
lot before the transplant. I feel much better now!
5
What is rehabilitation like ?
The first step consists of moving as much as possi-
ble while gradually stopping the oxygen. This was
a source of anxiety for me, but the weaning was
 done little by little. I kept the oxygen a little longer
than necessary, I think, because I was so nervous
about it. Then I used it only at night, and in less
than two weeks, I did not need it at all. This was a
wonderful moment for me: I felt truly free! And
what is really encouraging psychologically is the
gradual withdrawal of all the tubes to which I was
connected. When Dr. Ferraro removed the drains,
it was a liberating experience. After being dis-
charged from the hospital, I had to do physiother-
apy for six weeks, two afternoons a week. I
enjoyed it and five months later I am still going.
Doctors are adamant about it because physical
exercise is crucial to the rehabilitation process.
Do you still sometimes worry about
your state of health ?
As I mentioned earlier, I was quite nervous right
after the transplant. I wanted so much for every-
thing to turn out well and I realized that I now had
the chance that I was hoping for and that I had to
do everything in my power to make it work. My
confidence is increasing, everything is going really
well, I have no sign of infection or rejection: I
consider myself very lucky. The lung transplant
team, especially Dr. Ferrero, Dr. Poirier and Karine
Rancourt, the program co-ordinator, are very reas-
suring. Their competence, availability and kindness
have really given me confidence and made my
rehabilitation easier. I want to sincerely thank
them; their support has been invaluable.
and the good times. In this sense, my attitude did
not really change; I am still keenly aware of life’s
day-to-day joys. However, because I have much
more energy than before, I can carry out projects
that I used to only dream about, which makes my
life even more interesting.
Have you any words of encourage-
ment for others who face a similar
situation ?
I would tell all those who feel apprehensive that a
transplant is an unbelievable opportunity; not only
did it save my life, but it gave me a quality of life
that was beyond all my expectations. I can do
everything I want to do now and I feel great. The
transplant enabled me to stay alive and to enjoy
life fully. There are some efforts involved, howev-
er: I had to go to the hospital very often over the
first months for various appointments and I had to
get used to the new medications. But these are
mere details compared to the benefits. I can only
encourage all those who would hesitate!
Thank you, Stéphanie.

Has your attitude to life changed

since the transplant ?

I think that people who suffer from a chronic dis-

ease such as cystic fibrosis learn at a very young
6 age how important it is to make the most of life
Above All, Reflections
an Individual
Almost one year ago, the SVB newsletter staff asked me to submit a written testimony.
After having given it some thought, my first reaction was to ask myself: “What is there to
say about my life? I do not have anything out of the ordinary to tell.” I had been letting
my ideas ripen all this time, when I arrived at the conclusion that, despite the fact that my
existence was in no way extraordinary, it was still a courageous passage from girlhood to
Julie Gagnon
adulthood. I am therefore happy to share my experiences with you and paint a portrait of
Montreal, Quebec,
what I have proudly built for myself over time. Canada

My disease was diagnosed at birth. At that time, I had very little chance of surviving.
However, against all odds, I lived beyond the life expectancy that doctors had predicted for
me, much to my parents’ joy. My health kept improving as the days went by. I was among
the “lucky” ones who only have a mild case. My childhood unfolded smoothly, despite the
sad death of my father when I was only two years old. My mother had to raise me by her-
self. I therefore grew up in a world of adults. My mother pampered me as a child and
ensured that I lacked nothing. She took me with her wherever she went, whether on a
holiday in the Laurentians or a one-week vacation in Mexico, and she signed me up for many
activities, such as classical ballet, swimming and music.

In those days, my mother was an active member of the Quebec Cystic Fibrosis Association.
She organized fund-raising events in our neighbourhood. Because of the ads she placed in
the local papers, people recognized me when we went out. At elementary school, teachers I wanted to be
explained my situation to classmates, and they sometimes organized activities to “raise
money for Julie.” Up until the end of this period, this popularity hardly bothered me at all. recognized for
However, as soon as I started high school, I no longer appreciated it. I wanted to be recog-
nized for who I was and not as “the freak with the fatal disease.” I insisted that my moth-
who I was
er stop all activities that had anything to do with cystic fibrosis.

I was very discouraged to discover that this was not enough. People still came up to me just
as often to question me about my disease. In order to keep this part of my life secret, I devel-
oped the reflex of saying that they had mistaken me for someone else, that I was not the girl
they had seen in the headlines. However, as you can imagine, they knew I was lying.
Denying this aspect of my life was my way of refusing to accept that I was different from
other teenagers. At least this defensive attitude kept people quiet for a while.

High school was a period of rebellion against my disease. I no longer took care of myself; I
7
refused to take my medication and undergo my physiotherapy treatments. I continued to
lie, attributing my absences when hospitalized to bouts of appendicitis, asthma attacks or
mononucleosis. This situation persisted until the day I met a young woman while in hos-
pital, who was four years older than I and who also had cystic fibrosis. I saw myself in her.
 Rather than feel sorry for herself, she forged ahead in life, stopping at nothing to achieve
her goals. In addition, she had an active social life beyond the world of cystic fibrosis. I
learned a lot from this woman. She represented an ideal for me. She helped me accept my
disease by teaching me that we can all be something other than a person with cystic fibro-
sis and that there is nothing wrong with talking about it to our close friends. I kept in We can all be
touch with her until her death. Today, I am still grateful toward life for having put this
angel in my path, because now I know that life is beautiful despite the obstacles we have something other
to overcome each day.

Toward the end of my adolescence, when I was only seventeen and believed that I would than a person with
never be able to have a man as a life partner because it just seemed too complicated, I met
someone who turned things around. At the beginning of our relationship, I was quite wor- cystic fibrosis
ried because I knew that if all went well, I would have to tell him about my state of health.
But he already knew. He had only refrained from mentioning it out of respect. So the day
I decided to talk to him about it, nothing really changed, except for the better. Today, at
twenty-one years of age, I am still with him. We have been engaged for a year and plan to
get married as soon as I graduate from university. We now live together and everything is
wonderful. I take my medication, inhale my aerosols and do my physiotherapy without feel-
ing uncomfortable about it. When I am in the hospital, my boyfriend visits me and
supports me, while respecting my needs and requests. He is discreet and never imposes
himself, and I know I can count on him. Everything is so easy with Stéphane. I adore him
and he makes me happy.

Although I am still young and my life has only begun, I feel it is a success. I was lucky to
have wonderful people around me, who helped make me the woman I am. I can only end
by saying that today, more than ever, I bite into life with gusto, because it is so beautiful!

8
Ahead Reflections
of the Game
My name is Markus Klingbeil and I am from Germany. I am 22 years old and have cys- Markus Klingbeil
tic fibrosis. I am writing this testimony thanks to the people I met during the 22nd Baden-Württemberg,
Germany
European Cystic Fibrosis Conference and the 8th IACFA Conference, which took place in
Berlin. This was the first time I ever took part in this type of conference and although I
am not a scientist, I learned a lot from the numerous seminars. But what I appreciated
most was meeting other CF adults from different countries, who have the same problems
I have, talking with them and sharing good times in their company. I am very pleased to
tell you a little bit about my life.

I was diagnosed with cystic fibrosis at birth. My parents therefore learned right at the
outset, in light of the scientific knowledge that was available in 1976, how to treat the
disease. We have come a long way since then. But I still remember the dietary restric-
tions imposed at that time, especially all the fatty foods I was not allowed to eat. How I
envied healthy children! I would have given anything for a plate of French fries!
Fortunately, there have been a lot of scientific advances and today I can eat what I please
thanks to pancreatic enzymes.

While growing up, I always wanted to be treated the same as other children. Despite the
limitations imposed by the delicate state of my lungs, I played sports (soccer, basketball)
as best as I could, and in this way, stayed in touch with my friends.

I continued with my education. For the last three years, I have been studying nutrition
at the University of Stuttgart, located in Baden-Württemberg, in the south of Germany.

I am also working on improving treatments for CF adults. We are trying to obtain fund-
ing to open up a hospital for CF adults. At the moment, I have to go to a pediatric hos-
pital when I need treatment. I dislike this situation, where I am surrounded by children
. . . who are often quite noisy!

I prefer getting my I.V. treatments at home. This gives me more space and much more free-
dom. I have to undergo a 14-day antibiotic treatment, twice a year. Because of my studies
and to avoid getting behind in my lab work, I receive my treatments during the holidays.

Despite all the inconveniences caused by cystic fibrosis, it does not rule my life, because
9
I decided I would not let that happen. I try to put aside the depressing aspects of my dis-
 ease and take advantage of life’s pleasures. My goals are to stay in shape and graduate from
university. These positive ideas help me endure my treatments and hospital stays. In
addition, I like to spend my free time with friends and I love to read, go to the movies
and listen to music.

My story is probably not very different from the stories of other adults with cystic fibro-
... [ cystic fibrosis ]
sis. But I am glad to have been able to share some of my experiences with you, who live
across the Atlantic.
does not rule my life,

because I decided I

would not let that

happen

10
 Health
Antibiotic Therapy:
at the Hospital or at Home
Karina Laberge, Intravenous antibiotic therapy, an integral part
of cystic fibrosis, is performed frequently in
Montreal, Quebec, some cases and more occasionally in others, but
is rarely avoidable. When bacterial infection of
Canada the respiratory passages becomes too acute,
treatment is required. Antibiotic therapy may be
unavoidable, but for the last few years, CF
patients have at least had a choice: being treat-
ed at home or in hospital.
Although home offers comfort and privacy, hos-
pitals are reassuring because of the presence of
doctors and nurses. There are advantages and
disadvantages to both; it is therefore important
to be well aware of them in order to make an
informed choice that is suitable to your needs
and state of health. Whatever the decision, the
important thing is to follow the golden rules of
effective antibiotic therapy: plenty of rest and a
strong commitment to treatments.
By the end of the 1980s, antibiotic therapy was
still rarely being administered at home, but
today, with cystic fibrosis clinics, the practice
has become more widely accepted. In 1998, for
example, at the Montreal Chest Institute,
almost two thirds of intravenous treatments
were administered at home. This increase may
be attributed to a trend toward community-
based care, which promotes home services, and
to the development of easy-to-use equipment,
but it is mainly a response to increasing patient
demand.
Medical motives
12
Dr. Alphonse Jeanneret is a physician at the cys-
tic fibrosis clinic at the Hôtel Dieu Campus of
the University of Montreal’s Hospital Centre
(CHUM). More than half of the 200 or so adults
being treated there choose to receive their
antibiotic therapy at home. Doctors respect this
choice, as long as it is possible. “Before sending
patients home,” explains Dr. Jeanneret, “I make
sure that they can be relied on to take all pre-
scribed doses at the appropriate times, get
enough rest and eat well. Most of them do it
properly because they are aware of how impor-
tant the treatment is.”
Hospitalization is also recommended for those
who, because of health problems, are not
strong enough to take care of themselves, or
have other medical problems related to their
bronchial infection. The same goes for those
who live alone and have no one to help them at
home. In these cases, the best option is a short
stay in hospital, where patients do not have to
worry about anything.
According to Dr. Elias Matouk of the Montreal
Chest Institute, many people prefer to spend a
few days in hospital at the beginning of treat-
ment, then complete it at home. This short
hospital stay allows them to get better rest
when the infection is at its most critical point,
and it gives them a chance to become familiar
with the methods for self-administration of
antibiotics under the supervision and advice of
a nurse.
Are the treatments effective? Some scientific
studies have found that patients recover more
slowly at home, the infection in the bronchi
does not resorb as quickly, while others found
no noticeable difference.
Based on their own practices, Dr. Matouk and
Dr. Jeanneret lean more toward the second
study group; according to them, the recovery
period is almost the same at home as it is at the
hospital. As for the long-term effects, there is talized for reasons of safety and practicality. “By Some people prefer
insufficient data to judge whether home antibiot- staying in the hospital, I have all the necessary
ic therapy increases the frequency of treatments. resources at hand. Respiratory therapists, phys-
being treated in
iotherapists and nutritionists are all nearby and
Personal motives I can use the exercise room. It is the best place
to get a good rest.” hospital so they can
Chantal Germain has
Being at been receiving antibiotic A hospital is indeed a good place to meet all
therapy at home for ten kinds of health specialists and become familiar be more closely
home reduces years now, when her state with what they have to offer. It is also a good
of health permits it. She place to make important connections, not only
with medical staff, but also with other CF monitored
has thus avoided about
the boring thirty hospital stays over patients who are going through similar experi-
those years. “Being at ences; these relationships could be very useful
home reduces the boring in difficult times. Another thing to consider is
aspect of
aspect of the disease. It that hospitalization provides a break for the
allows me to lead a more patient’s friends and family, who sometimes
the disease normal life and keep my need it.
spirits up.” She does not
feel that she gets more Although home intravenous antibiotic therapy
rest in hospital, and as far as meals go, there is provides new treatment options and important
no comparison. benefits, the decision remains one of individual
choice. The benefits and inconveniences of hos-
Chantal has certain advantages, however. She pital and home vary according to the needs of
can administer her lung physiotherapy herself, each person. It is up to you to choose.
which gives her more independence, and her
husband helps her. According to Chantal, home
antibiotic therapy is the best choice, especially
since the Intermate system became available,
which is a step toward freedom compared to
traditional intravenous systems. “The miniature
plastic bottles provided by Intermate really simpli-
fy things,” she adds. “They are a lot easier to use;
they are delivered to the home, ready for use, and
they allow greater mobility.” She can even admin-
ister her antibiotics in the car or on the bus.

Staying home, however, reduces contact with

medical personnel. Some clinics, like the one at
the Montreal Chest Institute, provide home
visits by specialized nurses. But some people
prefer being treated in hospital so they can be 13
more closely monitored. That is the case for
Frédéric Gauthier, who insists on being hospi-
 Health
Lung Transplants
for Patients
with Cystic Fibrosis
Margaret Hodson1 When cystic fibrosis (CF) was first diagnosed in
1938, approximately 70% of babies died within
MD, MSc, FRCP, DA, DMedEd, one year of birth. Now the average survival in both
North America and Northern Europe is over 30 years
Royal Brompton Hospital, of age. The major cause of mortality and morbidi-
London, England ty is lung disease. Sadly, even with all the advances
in medical care over the last 20 years there are still
patients who develop end-stage respiratory fail-
ure. For these patients lung transplantation can be
life-saving. The first successful transplantation for
CF was performed in the UK in October 1985 and
since then many hundreds of CF patients have had
transplants worldwide.
The patient with CF has severe pulmonary infec-
tion and the most frequently performed transplant
operations are bilateral-single lung transplanta-
tion—when a patient receives two lungs—and
heart-lung transplantation—when a patient
receives two lungs and a heart. In the latter case
the CF patient's heart is given to another patient
needing a heart transplant—the domino procedure.
SPECIAL CHALLENGES FOLLOWING LUNG
TRANSPLANTATION IN CF PATIENTS
The patient with CF is liable to have the same
problems in the post-transplant period as patients
who do not have CF, i.e. infection and rejection,
and a proportion of patients develop obliterative
bronchiolitis. However, there are additional factors
for many CF patients, i.e., poor nutrition, salt loss,
small bowel obstruction, malabsorption of the
drugs used to prevent rejection of the transplant-
ed organs, diabetes mellitus, persistent infection
14
of the upper respiratory tract and liver disease.
This means that it is essential that the CF physi-
cians work closely with the transplant team to
obtain a satisfactory result.
PRE-OPERATIVE ASSESSMENT
Patients should not be considered for transplanta-
tion unless they definitely want a transplant and
they have already had the maximum possible medi-
cal treatment. Patients who want to be considered
for transplantation will need a complete assessment
by a physician with expertise in the care of CF
patients and the transplant team. Some generally
used selection criteria are shown in table below.
Indications
Deteriorating chronic respiratory failure despite
good medical treatment
Limited short—and medium—term survival
(less than 24 months)
Severely impaired quality of life
Patient must want a transplantation
Contraindications
Psychosocial instability
Malignant disease
Active mycobacterial or aspergillus infection
Infection outside the respiratory tract
Pulmonary bacterial pathogens resistant to all
available antibiotics
Methicillin-resistant Staphylococcus aureus not
confined to the respiratory tract
Other end-organ failure (heart, kidney)
Gross malnourishment
Less than 10 years of age or older than 60 years
High-dose corticosteroids
Smoking or addiction to drugs or alcohol
Previous pleurectomy or talc pleurodesis
Pre-operative ventilation
 Increased risk factors
Liver dysfunction
Chemical or abrasion pleurodesis
Previous thoracic surgery
Pre-operative ventilation
A detailed history will be taken including
a social history. The patient is fully exam-
ined and a number of investigations
performed. These investigations include
respiratory function tests, exercise tests,
chest X-ray, CT scan (chest, sinuses) and
detailed haematological, biochemical and
microbiological studies. There will be
investigations of the patient's heart, liver
and kidneys. If any of these initial tests
are abnormal then further investigations
may be necessary.
The patients and their next of kin are fully
informed of the risks and potential benefits
of transplantation. Transplantation is not
the most suitable treatment for every CF
patient nor is it wanted by all patients when
they fully understand what is involved.
TIME ON THE WAITING LIST
Initially, when patients are placed on the
waiting list they are very relieved, but
then as time passes the situation can
become very difficult. Patients may won-
der, "Will an organ become available in
time for me?" During the waiting period
it is important for patients to keep as fit
as possible and inform the CF consultant
immediately if there is any change in their
clinical condition.
A bag must be kept packed and clear
plans made for transportation to the hos-
pital quickly if an organ becomes avail-
able. It is important to understand that
there is only a limited amount of time
between an organ becoming available
and it safely being given to the recipient
(four to six hours). The waiting time for a
transplant can be very stressful and it is
vital that each patient receive adequate
help and support from friends, relatives
and hospital staff.
Major matching criteria of donor and
recipient are blood group, size and spe-
cific immunological features. Sometimes
an organ becomes available quickly, but
at other times there is a long wait. Some
patients have had to wait for as long as 4
years. In our unit the average wait is
between eight and 12 months 2.
THE OPERATION AND IMMEDIATE
POST-OPERATIVE PERIOD
The operation takes six to eight hours
and there is often considerable blood
loss, making blood transfusion essential.
Patients wake up in the intensive care
unit where they may stay for a number of
days. At that time, they will initially be
ventilated and have a number of tubes in
their chest but within a few hours, if all is
well, the patient will be gradually
weaned from the ventilator and be able
to sit up and talk to relatives. The stay in
intensive care is variable, some patients
being fit to return to the ordinary ward in
two or three days, others having to stay
for a number of weeks.
POST-OPERATIVE TREATMENT
When patients are transplanted they
need to take medication for the rest of
their lives to stop them from rejecting the
transplanted organ.
The most commonly used drugs are
cyclosporine, imuran and prednisolone.
Like many effective treatments, these
have some side effects, but it is vital that
patients do not stop or change their
treatments without prior discussion with
their medical advisors. In the immediate
post-operative period many patients get
episodes of rejection which are treated by
intravenous steroids, and some may get
episodes of infection which are treated
by appropriate antibiotics. Many CF cen-
tres maintain transplanted patients on
inhaled colomycin to reduce the risk of
Pseudomonas, which is still present in the
upper airways, contaminating the trans-
planted lungs.
FOLLOW-UP
It is important to remember that lung
transplantation is not a magic cure for CF.
Patients will still have CF bodies but they
will have new lungs. Before leaving hos-
pital, patients will have been taught to
administer their own medications and to
record lung function in a notebook daily.
If there is any change they should contact
the transplant team immediately. Some
patients who live near the transplant cen-
tre can be discharged from the hospital
after about six weeks3 . Initially, however,
they will have to be seen at least once a
week. By the time patients are a year
post-transplantation, they will probably
only need to be seen by a doctor once a
month4 and have a blood test done once a
month to check their immunosuppres-
sant drug levels, renal function and other
haematological values. Patients will need
to continue with their CF care, pancreat-
ic enzymes, vitamin supplements and
other treatments for diabetes or liver
disease if these are appropriate.
LONGER-TERM COMPLICATIONS
Any transplanted patient may at any time
have episodes of infection or rejection,
although after one year these episodes
become less frequent. As time progress- 15
es, however, there is a risk of developing
obliterative bronchiolitis, which may
 Lung Transplants for Patients with CF
occur in up to 50% of patients. There is a gradual
narrowing of the airways and increase in
breathlessness. This needs urgent diagnosis and
treatment. The treatment consists of augmented
immunosuppression, which may mean an alter-
ation in the drugs, such as FK506 or mycophe-
nolate. A small proportion of patients develop other
complications, such as airway stenosis and lympho-
proliferative disease, but these can usually be treat-
ed satisfactorily as long as they are diagnosed early.
RESULT
The St. Louis International Registry5 reported sur-
vival for one, two and three years at 70%, 62%
and 53% respectively for bilateral lung transplan-
tation for CF. Long-term survival has been report-
ed from the Harefield/Brompton series of patients,
who received mainly heart-lung transplantation at
one, five and eight years of 72%, 50% and 35%
respectively. Their longest surviving patient is now
12 years post-transplantation.
CHALLENGES
Lung transplantation for CF is now an established
treatment. It has given new life to many patients.
It has also given good lung function, so the quali-
ty of life has been good, e.g., the FEV1 at one, five
and eight years after transplantation in the
Harefield/Brompton series is 70%, 75% and 77%
predicted. With this kind of lung function, patients
can live a near normal life.
The major challenges, however, are:
Shortage of donor organs. Sadly, approximate-
ly 50% of patients6 die on the waiting list
because suitable organs are not available.
Pioneering work was commenced by Prof.
Starnes, a heart and chest surgeon at UCLA in
the United States, using bilateral lobe trans-
plantation from living donors as a way of
16
increasing the number of patients that can be
successfully transplanted. This technique is
now being used in some specialized centres in
Europe and the United States. Results from
living lobe donors are still very limited but a
one-year survival at 70% has been reported.
Obliterative bronchiolitis. Much research is
currently in progress to find better ways of
diagnosis and treatment.
CONCLUSIONS
Transplantation for patients with CF has now
come of age. There are some patients who were
transplanted more than a decade ago and who are
now living normal lives. The major challenges
consist of shortage of donor organs, which may
partly be met by developing bilateral lobe trans-
plantation from living donors, and obliterative
bronchiolitis. New treatments are being studied
for obliterative bronchiolitis and intensive research
in this area must continue.
1. Dr. Margaret Hodson is a world-renowned cystic
fibrosis lung specialist.
2. The average wait in Quebec is 12 to 18 months.
3. In Quebec, three or four weeks after the operation.
4. In Quebec, every three months.
5. Now the Registry of the International Heart
and Lung transplant Society.
6. In Quebec, 20% of patients die while waiting for
a transplant.
 Sincere Thanks
The ACFCQ would like to thank:
LE SECRÉTARIAT À L’ACTION COMMUNAUTAIRE AUTONOME
Government of Quebec

THE QUEBEC CYSTIC FIBROSIS ASSOCIATION

Canadian Cystic Fibrosis Foundation

L’OFFICE DES PERSONNES HANDICAPÉES DU QUÉBEC

Government of Quebec

The Adult CF Committee of Quebec (ACFCQ) is sincerely grateful to all its supporters and subsidizing agencies.
Thanks to their generosity, the ACFCQ is able to meet its objectives of promoting, defending and
protecting the rights and interests of adults with cystic fibrosis in Quebec.

Sincere Thanks

The

invites you to visit its Website

www.cpafk.qc.ca
sponsored by Solvay Pharma Inc.
 Health

Treatment
of Cystic Fibrosis
in Denmark

Niels Høiby, Department Introduction function is also tested to detect signs of cirrhosis.
2 Treatment begins as soon
of Clinical Microbiology Denmark (43,000 km , population 5.1 million) is a Life expectancy
as cystic fibrosis is
country consisting of many islands and one penin-
at Rigshospitalet, diagnosed. The improved
sula. The Faroe Islands (about 50,000 inhabitants)
prognosis correlates to for patients at the
and Greenland (about 50,000 inhabitants) are also
University of early and intensive treat-
part of Denmark. The Danish CF Association was
ment of bacterial colo-
Copenhagen, founded 30 years ago; its executive officer is Danish CF Clinic in
nization and infection of
Hanne Wendel Tybkjœr, who is the mother of a CF
Christian Koch and the lungs, and monthly
patient. Co-operation between the Danish CF
follow-up in CF centres Copenhagen is
Association and the CF centres has always been
Birgitte Frederiksen, is therefore necessary.
very good and some doctors from the centres are
Life expectancy for pa-
Danish Cystic Fibrosis members of the Association’s executive commit- over 40 years
tients at the Danish
tee. The incidence of CF in Denmark is 1:4700 and
Centre, Copenhagen, Cystic Fibrosis Centre in
delta F508 accounts for 90% of the CF mutations.
Copenhagen is over 40 of age
Approximately 350 CF patients are registered in
Denmark years of age.
Denmark, and treatment is centralized in two clin-
ics: Copenhagen (founded in 1968, 260 CF
Prevention and treatment of bacterial
patients, chairman, Christian Koch) and Arhus
infections
(founded in 1990, 100 CF patients, chairman,
Peter Oluf Schiøtz). Shared care is avoided, so near- The congenital defect associated with cystic fibro-
ly all patients are treated in these two clinics, which sis causes major inflammation of patients’ lungs.
are located in pediatric wards, although adult CF This inflammatory response is mostly a side effect
patients are also treated there. There are no plans for of the presence of polymorphonuclear leucocytes
the establishment of CF centres for adults. and antibodies. Proteases and oxygen radicals
released by the polymorphonuclear leucocytes
Medicine is socialized in Denmark, which means
gradually destroy the lung tissue. The inflamma-
that the counties pay for the centralized treatment
tion is therefore already present in CF infants, so it
of their CF patients in the two clinics. Screening
is important to treat any bacterial lung coloniza-
for CF is not yet automatically carried out in
tion and infection aggressively in patients. We
Denmark but the median age at diagnosis is 7.4
have found that acute episodes of chronic respira-
months. All CF patients are seen every month in
tory disease in CF patients were caused by a
the out-patient clinics. The visits include physical
bacterium (63%), a bacterium and a virus (13%)
examinations, lung function tests, examination of
and a virus (6%), whereas no etiology could be
the microbiology of respiratory secretions and
detected in 18% of cases. Early treatment of
18 immunological tests. At least once a year,
bacterial infections based on precise microbiologi-
endocrine pancreatic function is investigated in
cal diagnosis is therefore one of the cornerstones
patients over 10 years of age by the oral glucose
of CF care in Denmark. When respiratory tract
tolerance test to diagnose diabetes mellitus. Liver
infections persist, most of the viscosity of
sputum is due to DNA from the neu-
trophil granulocytes, which are present in
large quantities in infected patients and
lead to chronic inflammation. Inhalation
with recombinant DNAse (Pulmozyme) is
an efficient way to reduce the viscosity of
secretions. This treatment is given to all
Danish CF patients with chronic lung
infections. They also receive anti-inflam-
matory therapy, which consists of daily
inhalation of steroids. Lung physiothera-
py is performed by means of PEP masks.
Because of the insidious nature of some
of the respiratory tract infections in CF
patients, frequent (monthly) bacteriologi-
cal examination of sputum and bronchial
secretions is necessary. The technique
used to evaluate respiratory tract flora in
patients who do not produce large
amounts of sputum is endolaryngeal suc-
tion. Measurement of the antibody
response to Pseudomonas aeruginosa
and other bacteria to detect chronic
infection that was not diagnosed in the
sputum culture is carried out at least
once every year, and more often if clinical
deterioration occurs.
A number of reports have shown that
cross-infection with Pseudomonas aerug-
inosa occurred in the Danish CF Centre in
Copenhagen. By improving hygienic
measures and treating infected and non-
infected patients in different wards and
on different days in the out-patient clinic,
it was possible to prevent such cross-
TABLE
Principles of antibiotic therapy in Danish
CF patients.
1 Staphyloccocus aureus, Hemophilus influenzae, 2 Significant antibody response against
intermittent Pseudomonas aeruginosa are Pseudomonas aeruginosa indicates chronic
treated every time they are detected in the infection, and two-week courses of anti-
lower airways, whether or not there are clinical Pseudomonas treatment is given every three
symptoms. months for the rest of the patient's life
Straphyloccocus aureus • tobramycin + piperacillin,
Two-week courses of dicloxacillin + fusidic acid • or + ceftazidime,
orally; rifampicin or clindamycin may replace • or + aztreonam,
one of these drugs. In case of chronic infection • or + imipenem-cilistatin, or + meropenem
or ≥ three recurrent infections/year, a one- • and colistin inhalation, and/or + ciprofloxacin
month treatment with two of these drugs is orally.
given followed by three months of dicloxacillin
3 Other rarely occurring bacteria are treated
as monotherapy.
along similar guidelines according to their
Hemophilus influenzae antibiotic sensitivity.
Two-week courses of pivampicillin or amoxicillin
orally (clavulanic acid is added in case of beta-
lactamase producing bacteria), or alternatively
cefuroxime axetil, or combined with rifampicin.
Pseudomonas aeruginosa
Ciprofloxacin orally + colistin inhalation for
three months.
infection at the Centre. The yearly inci- As a consequence of these preventive
dence of new chronic Pseudomonas measures, the mean age for contracting
aeruginosa infection was thus reduced to chronic Pseudomonas aeruginosa infec-
a "normal" level that is unrelated to the tion has increased from six to 15 years
treatment centre, which is about one to during the last decade.
two percent per year. The water used in
dentists' instruments is often contaminat- Aggressive antibiotic treatment and the
ed with Pseudomonas aeruginosa and drugs used at the Danish CF clinics are
other bacteria, and this has been shown shown in the table. The survival of Danish
to be a risk for CF patients. The Danish CF patients has improved very much, as can 19
CF Centre is now focussing on disinfec- be seen in the figure, and the most impor-
tion of the water used in such equipment.
 Treatment of Cystic Fibrosis

100%
90%
Cumulative survival proportion

80%
70%
Median age
60% Legend of diagnosis

50% 1989 - 1993 (1200 PY) 7.4 months

1984 - 1988 (1096 PY) 8.4 months

40% 1979 - 1983 ( 926 PY) 11.3 months

30% 1974 - 1978 ( 735 PY) 12.0 months

PY = persons years
20%
10%
0%
0 5 10 15 20 25 30 35 40 45 50
Years after diagnosis

Legend of the figure

Modified life tables at five-year intervals using five-year age bands for CF patients treated at the
Danish CF Centre in Copenhagen. Analysis by years after diagnostic (from Frederiksen et al. Pediatric
Pulmonology 1996; 21: 153-158).
PY = person years.

tant reason for this is thought to be the Danish Cystic Fibrosis Patients. Acta Pædiat
aggressive treatment of lung infections. Scand 1989;78:395-404.

Jensen ET, Giwercman B, Ojeniyi B, et al.

References Epidemiology of Pseudomonas aeruginosa in
Frederiksen B, Lanng S, Koch C, Høiby N. Cystic Fibrosis and the Possible Role of
Improved Survival in the Danish Cystic Fibrosis Contamination by Dental Equipment. J Hosp
Centre : Results of Aggressive Treatment. Infect 1997;36:117-122.
Pediatric Pulmonology 1996;21:153-158.
Koch C, Høiby N. Pathogenesis of Cystic
Frederiksen B, Koch C, Høiby N. Antibiotic Fibrosis. Lancet 1993;341:1065-1069.
Treatment of Initial Colonization with
Pedersen SS, Koch C, Høiby N, Rosendal K. An
Pseudomonas aeruginosa Postpones Chronic
Epidemic Spread of Multiresistant Pseudomonas
Infection and Prevents Deterioration of
aeruginosa in a Cystic Fibrosis Centre.
Pulmonary Function in Cystic Fibrosis. Pediatric
J Antimicrob Chemother 1986;17:505-516.
Pulmonology 1997;23:330-335.
20
Høiby N, Pedersen SS. Estimated Risk of Cross-
Infection with Pseudomonas aeruginosa in
 Research

Update on Immunocal TM
Research (HSM 90 TM
):
Larry C. Lands, In the Autumn 1998 issue of SVB, we discussed Immunotec Research Inc. A summary of this work
the important contribution of inflammation to was presented at the 1999 International
M.D., Ph.D.
lung disease in Cystic Fibrosis (CF) and the poten- Conference of the American Thoracic Society.
Director, CF Clinic, tial of antioxidants to help control this inflamma- The full report is in press in the Journal of Applied
tion. The antioxidant network is complex, but at Physiology.
McGill University
its core is a small peptide called glutathione.
We believe that supplementation with Immu-
Medical Centre— Glutathione is made up of three amino acids, of
nocal™ may be even more beneficial to both lung
which cysteine is the most critical. If glutathione
Montreal Children's function and muscular performance in patients
levels can be raised, then antioxidant protection
with CF. We have now commenced a trial in young
Hospital Montreal, will be increased.
adults with CF. If you would like further informa-
Quebec, Canada The major difficulty in boosting glutathione levels tion, please contact me at (514) 934-4442 or via
is providing cysteine to the cells. This is best e-mail at larry.lands@muhc.mcgill.ca.
achieved by supplying cysteine in the form of a
dipeptide (two amino acids linked together). The
cysteine in Immunocal™, made from cow's milk
whey protein, comes in such a dipeptide form.
We proposed that using Immunocal™ would
result in increased glutathione levels. As a func-
tional consequence of augmented gluthatione
levels and antioxidant protection, we anticipated
that muscular performance would be enhanced.

We studied healthy individuals before and after 3

months supplementation with either casein,
which is low in cysteine, or Immunocal™. We
found that glutathione levels rose on average
35%, and muscle performance increased by 13%
in the group treated with Immunocal™. This is
the first demonstration that prolonged supple-
mentation with a product designed to provide
readily absorbable glutathione precursors could
increase glutathione levels and improve perfor- 21
mance. This work was funded by a grant from
the Canadian CF Foundation, with support from
 Psychology
About Love
Writing about love is quite a difficult task consid-
ering that the great diversity of meanings attached
to this concept is both a source of confusion and
of many disappointments. A man may love a
woman, but it will be a different kind of love for a
wife, a mother, a fling or the Virgin Mary. The
young girl he fell in love with in his teens will be
loved differently thirty years later, if he still loves
her at all. We love sunny days, but we can also
love rainy days; they are different kinds of love, as
are all cases of love, be it for something, for some-
one, or for the feeling itself. Some people love
fruit, liquor, the pain of physical exercise, acrid
cigars or sweet chocolate; others love to suffer or
inflict pain, demean themselves or dominate
others. There is the love of money, power, solitude
and even death, and the matter of loving Coke
more than Pepsi.
We are talking about a protean emotion with
many expressions and, what is more important,
with many objects. When we reflect on this seem-
ingly extrinsic thing called love or its transitional
attributes, we quickly realize we are going in
circles. The thing we are trying to define does not
exist independently, and maybe it does not exist at
all. Nevertheless, throughout our entire lives, we
search for this "thing" that slips away as soon as
we try to pinpoint it. What a strange endeavour it
is to name that which is beyond our understand-
ing, to give it a concrete form, to project it onto
life, people or objects.
It simply is not enough to say that something
inside us loves, that we feel good whenever the
flavour of a particular dish stimulates our taste
buds, or that our imagination is sparked by the
22 way a girl arches her back, smiling as she fixes her
hair. This takes much of the mystery away from
the things that move us. Thus we carry on in life,
often blind to ourselves and always believing that
objects of love are realities of the surrounding
world, real and not pure fabrication. This is a
source of sweet illusions...
In this century, many misguided philosophers tried
to reduce love—all kinds of love—to mere sexual
desire. This sounded interesting at a time when
the sight of a young woman's ankle glimpsed in
the shadows would unleash torrid fantasies.
Today, however, now that sexuality has become
commonplace, those ideas appear folkloric, and
poor Freud's theories have been reduced to the
playing out of his own frustrations and countless
biases. But long before Freud, people attempted
to reduce love to so many other silly things that
this example is not so ridiculous. What comes to
mind is love for the gods, love for humanity, the
influence of the stars or the devil, the aesthetic
impulse of absolute Beauty, not to mention love of
the homeland, the tribe, the spirits of the dead
and archetypes (whatever meaning or lack thereof
can be attributed to this bizarre concept). These
reductions are a great source of security for those
who feel anxiety when faced with the enormity of
individual freedom.
Ironically, this thing we call love always feeds off
some form of absence. We know that people who
are satiated or filled with passion are not very like-
ly to fall in love, neither are people who are in a
hurry. Once Cupid strikes, all the other women in
the world cease to exist, and even the features of
the woman we just fell in love with tend to disap-
pear. Moreover, when we are not in love or when
we feel deprived of our usual essential wholeness,
the need for love overcomes us, and at that
moment, external objects seem invested with the
power to fulfill us.
This absence of self, which pushes us outwards, is
the most interesting and most vital characteristic
of human existence. This is what keeps us alive,
inspiring us to want to appropriate things or
persons, drawing us to deeds and actions in an
endless forward fall, a fall that can only be com-
 Sergio Kokis
Montreal, Quebec,
Canada

pared to that of a child starting to walk. This vital es them by every day, and they try to protect exact- Living... is the
appetite for what is outside us is probably the ly that which they fail to achieve: forward motion.
unnameable and complex thing that we generical-
ly call love. I love oranges for the fresh effect on Love and life; needs, imperfection, and the hope overwhelming
my palate of the slightly acid water when my to correct them, new battles and new challenges.
mouth is parched. That girl attracts me by her very A small child gets up, falls forward, gets back up
and falls again. He savours the things within his desire to want
absence, and it distresses me that her gentle
manner is not addressed to me. However, the girl reach, but he does not stop at the inert sense of
beside her does nothing for me, as she does not security found in possessions and achievements. to complete
fulfill any of my needs. It hurts me when my coun- Lovers would not be happy to receive all at once
try is in danger or when I am far away amongst each other's eternal love; their future would be
strangers who do not captivate me at all. Politicians very dull, just like the image of heaven. Love has ourselves
lacking support know very well how to threaten the to be constantly renewed; there have to be new
country in their speeches in order to appear like a needs and absences. Otherwise it would resemble
saviour in the eyes of an indifferent nation. the bitter misplaced vanities of youth that paralyse
existence in a state of eternal having-been. "The
Each human being, on top of being unique, is also quest is worth more," said the Knight of La
completely alone. Alone, and fortunately, incom- Mancha, who, more than anyone, knew as much
plete. As everyone can imagine, heavenly perfec- about love as he knew about illusions. And if
tion and eternity must be utterly boring, and that Dulcinea had not constantly been re-created in his
is why all religions teach us more about hell than imagination, would he have gained anything by
paradise. If they tried to describe the latter in worshipping her?
detail, the faithful would quickly lose their faith.
Perfection is only attained through death, immo-
bility and the brutality of inert objects. Living, on
the other hand, is the overwhelming desire to
want to complete ourselves, to taste the next
orange, to think about the book we will write or
read tomorrow, to wish for joys never before felt,
be it seeing our children reach adulthood, buying
a long-desired sports car, paying off a mortgage or
awaiting the coming spring. There is a reason why
many people die soon after retirement, and why
the fight against certain deadly diseases can some-
times keep people alive. Something essential
about the nature of love can undoubtedly be
found in these examples.
23
Unfortunately, quite a few people's lives are ani-
mated mainly by the fear of dying. This is a kind of
negative love, which instead of making them fall
forward, freezes time for them in the here and
yesterday out of a simple desire for static perma-
nency. These people love life solely because it pass-
 Psychology
Is Suffering
Really Necessary?
In traditional milieus that are strongly influenced
by religion, suffering has long been perceived as
both a punishment for one’s sins and a way to
redeem oneself to obtain eternal salvation. Not so
long ago, these ideas were blindly accepted, with-
out being examined or criticized, since they were
imposed by an authority that no one dared call
into question. Times have changed, and the
Church has seen its claims swept aside by freedom
of thought. Yet today, even though very few peo-
ple still entertain this kind of belief, deep down,
many feel uncomfortable with the idea of suffering.
Some tend to believe it is both inevitable and
unfair. You still hear comments like this: “It’s
always the same people who have to suffer!”; and
this type of question: “If God is good, why does He
allow suffering?”; or remarks like the following:
“The rich and powerful don’t suffer as much as the
poor.” “I can understand adults suffering, but why
should children have to?” It makes you think. . . .
First, let us make a distinction between pain and
suffering. I believe that pain is inevitable and that
it has nothing to do with will. It is more of a
biological response. Pain can be a reaction to for-
eign bodies invading the organism, such as germs,
viruses and bacteria. It can also stem from defor-
mity, infection or hereditary disease, among other
things. In some cases, it can even be useful, a kind
of alarm signal. And even though medicine has, in
24 large part, managed to eliminate or relieve physi-
cal pain, it still exists in other forms that, unfortu-
nately, are impossible to control or neutralize.
But suffering, in my opinion, is different. First of
all, it is inside, in the conscience, the seat of
thoughts and emotions, and not in the body. It is
not a biological reaction to unpleasant or hostile
phenomena, but a psychological reaction to every-
thing that is felt to be unpleasant, repulsive, vio-
lent, undesirable, restricting or simply unaccept-
able. It is clear that the emotional reaction that
creates suffering does not necessarily depend on
real or tangible events. Despite everything, individ-
uals may react just as violently and profoundly to
imaginary, or merely virtual, situations as to con-
crete ones. The way I see it, emotional suffering
does not differentiate between the imaginary and
the real: it is not the thing that hurts us, but our
reaction to it.
Obviously, real events can
Emotional suffer-
trigger an emotional reac-
tion in an individual and
ing does not dif- cause suffering; think of
the terrible feelings that
ferentiate between death can provoke. The
suffering that a person
feels as a result of this is
the imaginary and
well founded and justified,
while the suffering caused
the real by imaginary events is not.
Usually, in the case of real events, people react
with great sorrow, which may be short-lived or last
a long time and may be more or less deeply felt.
They still may suffer greatly afterwards, but it is
 Placide Gaboury
Montreal, Quebec,
Canada

necessary to confront this suffering, to live
through it, and not try to escape it. In fact, if it is
properly experienced, and if people often talk
about it with their friends to alleviate the sorrow,
it eventually loses its impact. However, if we pro-
long suffering through the imagination or through
self-pity, the drama is maintained and, at that
point, our reaction is the same as to an imaginary
event: we make ourselves suffer. In a case like this,
emotional suffering is deliberately provoked and
maintained. Consequently, it seems that it is large-
ly up to us to stop the suffering, and that no one
can do anything about it as long as we fail to take
responsibility for it.
sive: we overreact because instead of looking at
the facts, we consider the possibilities. We may
even imagine the worst, even though the reality is
non-threatening; this attitude is a form of escap-
ing reality. Unfortunately, many people spend their
time in an imaginary world, in a soap opera or
melodrama. Yet reality, although it sometimes can
be painful, is the only thing that can heal us and
restore inner harmony. So seeing things as they are
is healthy, beneficial and liberating. Life can then
turn around, take on new meaning. . . .

In the case of a real event, such as the death of a

loved one, suffering occurs spontaneously: we do
not provoke it. We then react in a normal, natural
way, which is a sign of good mental health and
humanity. However, were we to continue mourn-
ing for 20 years, it would no longer be a sign of
health, but a reaction to a product of the imagi-
nation, which is being pathologically maintained
and cultivated. Grieving over someone’s death is
healthy and natural; failure to do so would be
unhealthy. But life must follow its course, and we
have to go with the current.

What is important to remember is that where pain

is physiological in origin and can be relieved, for
the most part, with medication, suffering is an
emotional reaction, and therefore of psychological 25
origin. This reaction can sometimes become exces-
 Health
Column
Dr. Michel Ruel
Centre hospitalier Would you like to know more about a
universitaire specific aspect of the disease? The
de Québec,
CHUL Pavilion Health Column, written by Dr. Michel
Ruel, a specialist in cystic fibrosis, will
open the door to all kinds of informa-
tion. Take advantage of this opportuni-
ty. All inquiries are confidential. Write
to the ACFCQ (Health Column).
CO2 and Oxygen Flow
Why does my body retain more C02 when
I increase the oxygen flow from my oxygen
concentrator?
Hervé Gagnon
Carbon dioxide (C02) is one of the organism’s
waste products and blood levels reflect ventilation
(gaseous exchanges) produced by the lungs.
However, the brain (most of the time in an invol-
untary, automatic manner) orders the respiratory
muscles to perform ventilation. There are three
kinds of signals that alert the brain when ventila-
tion is needed: a decrease in blood oxygen (02)
levels and an increase in C02 and acidity in the
blood. For some who suffer from chronic respira-
tory insufficiency, there may be loss of sensitivity in
the brain to the stimulus provided by increased
C02 levels; ventilation is therefore not increased.
These people generally have to leave it up to the
26 decrease of 02 in the blood to stimulate respira-
tion. But if there is too great an increase of 02
resulting from increased oxygen flow from the
concentrator, the brain loses its main stimulus for
triggering ventilation, so C02 levels rise, causing
headaches, drowsiness and even confusion. In
these cases, 02 deficiency should not be corrected
too aggressively: blood oxygen levels should
be slightly lower than the norm. You should be
aware that there may be other reasons for increased
C02 levels, especially when respiratory muscles
are exhausted.
Photosensitivity and Intravenous
Antibiotics
Are there any contraindications to sunbathing
during intravenous antibiotic treatments? If
so, what are the risks?
Sylvia Subara
There are in fact a number of medications that
make skin photosensitive, resulting in inflamma-
tion (swelling, redness and heat) after exposure to
the sun. The absorbed medication ends up in the
skin and with sufficient exposure to sunlight there
is a physicochemical reaction causing inflamma-
tion, as in sunburns. Antibiotics are one of these
medications. However, it is not the way the antibi-
otics are administered (orally or intravenously), but
rather the type of antibiotic which causes a pho-
tosensitive reaction. Antibiotics known as photo-
sensitizers are tetracyclines (including doxycycline
and minocycline) and sulfamides (found in
Bactrim). The family of quinolones, which includes
ciprofloxacin, may also bring about photosensitive
reactions. Intravenous antibiotics used to treat res-
piratory infections in people with cystic fibrosis are
usually not implicated, with the exception of
Bactrim (generic name: trimethoprim-sul-
famethoxazole), which is usually taken orally, and
sometimes intravenously. Among other drugs that
cause photosensitivity are certain diuretics, some
oral hypoglycemiants (used in the treatment of
diabetes) and some anti-inflamatories, to name
only medications occasionally used to treat cystic
fibrosis. Some drugs used for psychological disorders
(depression, psychosis) are also known photosensi-
tizers. To sum up, whenever you take medication
that is a potential photosensitizer, protect yourself
against the sun (appropriate clothing and sunblock).
Anti-Viral Vaccines
Last year, I had a lung transplant. Since I am in
great shape, I want to fulfil a lifelong dream: a
trip to Central Africa. Would anti-viral vaccines
be risky for me? If so, what should I do to
guard against infections?
prevent intestinal infections that cause fever and
diarrhea, take the usual precautions (drink only
bottled water, peel fruits and vegetables before
eating them) and use ciprofloxacin if you do
contract an infection. So before travelling to any
“exotic” country, it is important to consult some-
one who works in a specialized travellers’ clinic in
order to get the best preventive measures adapted
to your destination and state of health.

André Boisclair

You did not choose the safest destination for a

lung transplant recipient, even a healthy one. The
risk of infection in Central Africa is quite high,
unless you stay in the bigger hotels. Moreover, the
quality of health care in these countries is usually
far below North American standards. This being
said, I contacted an infectious disease specialist to
answer your question. He believes that anti-viral
vaccines pose a theoretical risk: they stimulate the
immune system, which could, in theory, result in
rejection of the transplanted lungs. Given this pos-
sibility, it would be better to abstain. However, you
may use other effective preventive measures: for
hepatitis A, instead of a vaccine, you can receive
gamma globulin injections (antibodies), which
provide good temporary protection. Malaria and
intestinal infections occur frequently in these
countries. To guard against malaria, you need to
apply bug repellant and take oral anti-malarial
medication before, during and after your trip. To
27
 A-z imut
Column
This is your column! You can ask us any
question on any subject. Remember that
your concerns may be of interest to oth-
ers. Once again, we would like to remind
you that all letters remain anonymous.
Write to the ACFCQ (A-Zimuth Column).
Medical Insurance Abroad
I have two serious problems: cystic fibrosis and
an insatiable desire to travel. Every year, or
almost, I fly off into the wild blue yonder. My
question is the following: Does the Blue Cross
basic travel insurance plan give me enough
coverage? If not, is there an insurance policy
that would reduce the risk of financial ruin
should I become ill in a foreign land?
Vincent Tardif
When travelling outside Canada, obtaining
health insurance is an essential precaution. Our
global health plan only covers a small part of
expenses incurred in another country. The
refund allowed by the Régie de l'assurance-mal-
adie du Québec for hospitalization is only $100
a day, while in the United States, for example,
daily costs can easily run up to $3,000.
Like all types of insurance, travel insurance is
28 based on risk assessment. Age and medical
precedents are the two main variables consid-
ered during this assessment. Quebec Blue Cross
identifies pre-existing conditions through the
following questions: Have you consulted a
physician? Have you taken or been prescribed
medication? Have you been hospitalized? Have
you been treated or were you advised to get
treatment for one of the following medical con-
ditions?
cardiovascular conditions
chronic obstructive lung conditions
neurological conditions
insulin-dependent diabetes
kidney failure, kidney transplantation
gastrointestinal conditions
cancer or malignant tumours
For persons under the age of 55 years with a
pre-existing condition, a three-month period of
stability is required before insurance coverage
can be obtained; this means that during this
period, no new diseases were diagnosed, no
new medication or change in the dosage was
prescribed, there was no hospitalization or new
treatments, and no changes in the medical con-
dition were observed.
If there is a pre-existing condition, i.e. there was
instability during the three-month period, the
traveller has to fill out a medical questionnaire.
If it is accepted, the pre-existing condition will
be covered at a higher rate, if not, there is no
coverage.
Since cystic fibrosis is a lung disease with fre-
quent periods of instability, the conditions may
not be covered by travel insurance. It is there-
fore essential to contact one’s insurance agent
to clearly determine what can be done,
depending on the individual situation.
All medical conditions that are unrelated to this
disease, such as accidents, would be covered by
the insurance policy. In addition, services that
provide assistance are always useful to an
insured party, whether the condition in question

is covered or not. This is why it is important to
obtain travel insurance when taking a trip out-
side the country.
Dominique Fortin
Quebec Blue Cross
Montreal, Quebec, Canada
Antibiotics for Multiresistant Organisms
According to my physician, my bacteria are
multiresistant to antibiotics. Is it true that
there is a laboratory in Ottawa where, thanks
to a specific technique, antibiotic combinations
capable of foiling recalcitrant bacteria are
being developed ? If so, I would greatly
appreciate information on the lab procedures
used to carry out these analyses, on the
dependability of the "winning" combinations
and on the costs associated with this type
of operation.
Louis Dumoulin
To answer your question, we have indeed devel-
oped a technique for performing laboratory
studies on antibiotic combinations that can kill
multiresistant organisms from individual CF
patients. The laboratory procedure involves test-
ing 298 different double and triple antibiotic
combinations for Burkholderia cepacia and 361
different double and triple antibiotic combina-
tions for Pseudomonas aeruginosa. These tests
are done in 96 well microtitre plates using a
broth for growing the organism. One, two or
three different antibiotics are added individually
to each well. We then examine the plates at 24
and 48 hours to determine if the well is turbid
(i.e. the organism has grown) or clear. If the well
is clear, we then subculture fluid from that well
A-Zimut
to determine if growth of the organism was just
inhibited or whether the organism has truly
been killed. We then provide the referring
physician with all the antibiotic combinations
that work for the particular isolate that was
sent in from a specific patient. However, one
cannot generalize: what works in one patient
may not work in another. In addition, probably
one of the most important outcomes of this
kind of testing is determining antibiotic combi-
nations that do not work for a patient, in other
words, antibiotic combinations that actually
allow the organism to grow easily. We have
only anecdotal case reports where people have
utilized these antibiotic combinations for a spe-
cific patient with positive results. However, this
testing has not been studied thoroughly and
carefully for patients. We are in the process of
developing such a study and hope to begin it
sometime next year. It is a very labour-intensive
process requiring a very high skill level. These tests
cost us approximately $300 per isolate tested,
which is what our hospital charges to test speci-
mens referred in from outside our institution.
We perform these tests at cost and hope that
the results are helpful to the physician who
sends in the specimen for testing. We are cur-
rently looking only at multiresistant organisms.
In other words, isolates for which routine labo-
ratory tests show that the organism is multire-
sistant to antibiotics. Further information on
how to refer an isolate in for testing can be
obtained by faxing a request to the Division of
Infectious Disease at (613) 738-4832, to the
attention of Wendy Ferris.
29
Noni E. MacDonald, M.D., FRCPC
Chief, Divison of Infectious Disease
Director, Cystic Fibrosis Service,
Children’s Hospital of Eastern Ontario
Ottawa, Ontario, Canada
 communic- action
A Change in the Prescription Drug
Insurance Plan
As of October 1, 1999, income security recipients
who have serious employment problems do not
have to pay for prescription drugs and neither do
their spouses. This change in the Prescription Drug
Insurance Plan concerns all CF adults and their
spouses who are income security beneficiaries.
Income Security/Prescription Drug
Insurance
If you are a low-income employee (full-time or
part-time) and you are registered with the RAMQ’s
Prescription Drug Insurance Plan, this message is
for you. Just as before the implementation of the
Régie de l’assurance-maladie’s Prescription Drug
Insurance Plan, you may, upon request, obtain a
Carnet de réclamation from Quebec’s welfare
department. This Carnet exempts the user from
the $176 premium and reduces the maximum
annual contribution from $750 to $200.
Before you apply to the welfare department for a
Carnet, you should be aware that there is a maze
of bureaucratic procedures involved. The officer
responsible for your file must gather a consider-
able amount of information to assess your eligibil-
ity. Expect to shuffle a lot of paperwork (medical
certificate, financial statement, etc.) and be pre-
pared to answer very personal questions.
For further information, contact an officer from
the welfare department in your area or ACFCQ
headquarters at (514) 288-3157 or 1-800-315-
3157.
30
Work Integration Program
The Work Integration Program is sponsored by the
Office des personnes handicapées du Québec, and
guarantees the income of salaried cystic-fibrosis
individuals while they are hospitalized (for a maxi-
mum of six weeks every year).
In your area, the Services externes de la main-
d’oeuvre (SEMO), an external employment service,
is responsible for administering various facets of
the Work Integration Program. To obtain this ser-
vice, you have to register at your SEMO immedi-
ately if you are currently employed or as soon as
you start a new job.
If you don’t know how to find your local SEMO,
call us at (514) 288-3157 or toll free at 1-800-315-
3157 if you are outside the Montreal Area.
Defending the Rights and Interests of
Quebec’s CF Adults
Are you the victim of discrimination or are you
being treated poorly? If you want to know your
rights as a recipient of health or social welfare ser-
vices, get in touch with ACFCQ headquarters. We
can help you.
Contamination and Hygiene
The ACFCQ feels it is important to remind
Quebec’s CF adults of the principles of hygiene for
reducing the risk of contamination from
Burkholderia cepacia.
Always cover your mouth when you cough.
Avoid intimate contact with other persons with
cystic fibrosis.
 Avoid handshakes.
Avoid contact with other people with cystic
fibrosis in small or poorly ventilated spaces (e.g.
car or bedroom).
Wear a mask when you know you have been
infected with Burkholderia cepacia and are in
the presence of persons with cystic fibrosis.
Never put your mouth to a container (glass,
bottle, etc.) or cutlery used by someone who
has cystic fibrosis.
Get into the habit of washing your hands regular-
ly, especially if you handle objects used by anyone
with cystic fibrosis.
Never share medical equipment (nebulizers,
syringes, tubes, etc.) that belongs to other people
with cystic fibrosis.
The ACFCQ adamantly believes that Burkholderia
cepacia contamination is everybody’s concern.
There is no reason to feel ashamed or embarrassed
about reminding your CF friends of the basic rules
of hygiene to follow for your safety as well as
theirs.
Nomination/COPHAN
The ACFCQ is pleased to announce that Aline
Fredette, the representative of CF adults in the
Estrie region, was elected for a second consecutive
term to the Board of Directors of the
Confédération des organismes de personnes
handicapées du Québec—COPHAN—(the Quebec
Confederation of Organizations for Handicapped
Persons) during its last annual meeting held in
Montreal on June 19, 1999. We congratulate her
and wish her well in the exercise of her duties.
Parking Stickers for Persons with Physical
Disabilities
Would you like to avoid local parking problems? If
you would like a parking sticker for persons with
physical disabilities, simply fill out a request form
from the Société de l’assurance automobile du
Québec (SAAQ). The cost is $15 per sticker.
You may obtain the form directly from the SAAQ
by calling (418) 643-7620 (Quebec City and area),
(514) 873-7620 (Montreal and area) or 1-800-
361-7620 (elsewhere in Quebec). You may also
get in touch with ACFCQ headquarters.
Thanks/SACA
The ACFCQ would like to thank the Secrétariat à
l’action communautaire autonome—SACA—
(Secretariat for Independent Community Action)
for the financial support it has provided over the
last two years. Its contributions have given ACFCQ
headquarters a greater sense of permanence and
have enabled the organization to more effectively
meet its objectives, which are to promote, defend
and protect the rights and interests of Quebec
adults with cystic fibrosis.
CPAFK Express
Quebec’s CF adults now have access to another
information tool: the CPAFK Express (in French
only for the moment). This newsletter will enable
the ACFCQ to maintain a closer relationship with
its members and provide them with more infor-
31
mation.
 We expect to publish four issues a year, but
demand will ultimately dictate the number of
issues as well as the publishing dates.
Please share any comments you have about this
newsletter so that we can improve the form and
content.
Genetic Screening
Do you have cystic fibrosis? Would your close rel-
atives (brothers, sisters, cousins) like to have chil-
dren? Let them know that there are effective
screening techniques for identifying carriers of the
CF gene.
Before they commit themselves to the noble
adventure of parenthood, inform them that there
is an inexpensive, safe and painless way to set
their minds at rest as to the probability of bearing
children with cystic fibrosis. The procedure is sim-
ple: all they have to do is contact their family doc-
tor and ask for a genetic test. Their doctor will put
them in touch with a genetic team specializing in
this type of diagnosis.
Feel free to ask the doctor or nurse in charge of
your cystic fibrosis clinic for more information on
the subject. They are well acquainted with refer-
ence procedures.
IACFA Newsletter
IACFA (International Association of Cystic Fibrosis
Adults) is an international organization of adults
with cystic fibrosis, which is administered by vol-
unteers who have the disease. IACFA publishes a
newsletter four times a year. If you would like to
32
receive it, write to: Barbara Palys, 82 Ayer Road,
Harvard, Mass., 01451, USA. Or you can e-mail
her at: bpalys@genesis.tiac.net.
Council of Canadians with Disabilities
(CCD) Award
The Adult CF Committee of Quebec extends warm
congratulations to Aline Fredette, Estrie represen-
tative of CF Adults, for winning the CCD Award
for 1998-99 for her exceptional contribution to
the cause, her promotion of the interests and
defense of the rights of people with disabilities.
The Board of Directors of the Confédération des
organismes de personnes handicapées du Québec
(COPHAN) nominated Aline Fredette, who distin-
guished herself by her great dedication to voicing
the needs of adults with cystic fibrosis as well as
backing them in the numerous issues being han-
dled by COPHAN. COPHAN’s Board of Directors
also want to highlight Aline Fredette’s involvement
in the Coalition of People With Disabilities. Every
year, this award is conferred on a person with dis-
abilities in each province. The award was present-
ed to her during COPHAN’s Annual Meeting of
June 19, 1999. Aline Fredette represents the
ACFCQ in COPHAN.
Correspondence Courses
Would you like to take correspondence courses
accredited by the Quebec department of educa-
tion? Nothing could be simpler. For high school
courses dial (514) 353-3355 (Montreal and area)
or 1-800-249-2848 (elsewhere in Quebec). For
college courses, dial (514) 864-6464 (Montreal
and area) or 1-800-665-6400 (elsewhere in
Quebec).
Minutes of Meetings Change of Address
Copies of the minutes of the Adult CF Committee If you have a change of address, please get in
of Quebec’s Board of Director and Executive touch with us by calling (514) 288-3157
Committee meetings are available on request from (Montreal and area) or 1-800-315-3157 (else-
ACFCQ headquarters. where in Quebec). You can also write to the
ACFCQ, 629 Prince Arthur Street West, Montreal,
Web Site Quebec H2X 1T9, or e-mail us at:
The ACFCQ has been working very hard on the cpafk@videotron.ca.
construction of a new Web site, which should be
available sometime before the beginning of win-
ter. The future Web site will be more complete,
informative and dynamic and will enable the
ACFCQ to better meet the educational goals that
are part of its social mission.

We apologize to our Web surfers who, for good

reason, are frustrated by the continuous post-
ponement of the new site’s operation.

Employee
The ACFCQ now has the technical and profession-
al support of a new employee. Renata Isajlovic has
been working since the beginning of August as
assistant co-ordinator, project manager and office
automation technician. We were able to hire this
valuable colleague, whom many of you already
know, thanks to the generosity of the organiza-
tions that subsidize us: the Quebec Cystic Fibrosis
Association, the Secrétariat à l’action communau-
taire autonome and the Office des personnes
handicapées du Québec.

Renata Isajlovic’s presence at ACFCQ headquarters

will enable us to provide you with direct service
from Monday to Friday, 9:00 a.m. to 4:30 p.m. So
take advantage of the situation! 33
www.cpafk.qc.ca

www.cpafk.qc.ca
www.cpafk.qc.ca
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