The World of Sixty Five Roses

By Kairen Griffiths and Emily Griffiths

Kairen Griffiths has recently retired from working as a Cystic Fibrosis Nurse in the north-east of Scotland for the last 26 years. The patients, families and colleagues with whom she worked alongside over those years left a significant and lasting impression on her. The stories recounted in this book are both heart-warming and heart-rending and will appeal to anyone with an interest in this complex and challenging condition.

• Language: English
• Publisher: Lulu.com
• Release date: Mar 21, 2021
• ISBN: 9781716309946

Book preview

The World of Sixty Five Roses

~ An insight into living with

Cystic Fibrosis ~

by Kairen Griffiths

Cover illustration by Emily Griffiths

First edition

© Kairen Griffiths 2020

ISBN: 978-1-71686-358-5

This book is dedicated to all the patients with Cystic Fibrosis whom I

have cared for over the years.

The 65 Roses Story

The 65 Roses story dates back to 1965 when an observant 4-year-old, hearing the name of his disease for the first time, pronounced cystic fibrosis as 65 Roses. Today, 65 Roses is a term often used by young children with cystic fibrosis to pronounce the name of their disease.

65 Roses® is a registered trademark of the Cystic Fibrosis Foundation.

Disclaimer

This book is semi-autobiographical.  Most of the characters in the book are based on patients whom I have known over the twenty-six years that I worked in the field of Cystic Fibrosis.  Some of the stories told comprise a composite character, based upon several people.  All patient names and identities have been changed in order to protect confidentiality.  However, patients included in this book have given their express permission for their stories to be told.  For those patients who have died, their families have generously given me their blessing to allow the experiences of their loved ones to be recounted in this book.  I am truly grateful to them all and hope that I do justice to their incredible stories.  I have used the real names of some of the professional colleagues that I mention in the book as it would have been difficult to disguise their identities.  Each of these colleagues are aware of this and have allowed me to do this.

All medical and clinical facts quoted in this book are believed to be correct at the time of printing.

Nothing in this book should be taken as an allegation against a real and living person or against a real institution, unless it is true.  All untrue ‘facts’ herein that could be construed as allegations should be regarded as part of the fictional side of this book.

~ Introduction ~

Imagine the following scenario.  You have recently given birth to a beautiful baby.  Everyone is surrounding you with congratulatory messages and gifts full of happiness and delight.  Your child is the apple of your eye, your new little bundle of joy who looks just perfect to you and to everybody else.

And then, the bombshell. 

‘Your baby has cystic fibrosis’.

Suddenly, your world is turned upside down.  What does this mean?  Am I dreaming?  Tell me this is not happening.  What do we do now?  Will my baby die?

The shock and disbelief that must hit a new parent when they hear those words is unimaginable.  Since the 2000’s, all babies born in the UK are tested at birth for cystic fibrosis.  Many, many parents have heard these words since then and they will all have felt the same emotions. 

Years ago, the outlook was bleak for those with cystic fibrosis - today, there is much hope and optimism for those with the condition, for those who look after for people with it and for the health professionals and scientists involved in caring and treating patients.  There is hope on the horizon, but for those living with cystic fibrosis every day of their lives, it remains a complicated and difficult condition to manage.

I have been carrying this book around in my head for a long time.  How to write it, where to write it, who to include in it and will I do these people and their stories justice?

I have recently retired as a nurse, with forty years’ experience mainly in the NHS and with twenty-six of those years working as a Cystic Fibrosis Nurse Specialist in a major teaching hospital in the North East of Scotland. 

What a job it was.  One that was full of the most incredible experiences, a chance to meet the most inspirational of people and such a roller coaster of a ride - joyous to heart-rending. 

Cystic fibrosis is a complex and difficult condition, one that I will expand upon more fully in later chapters.  It is an inherited, recessive disorder (meaning that both parents need to carry and pass on a copy of the faulty gene) and it currently affects around 10,500 people in the UK today.

I do believe there is no other job quite like that of the Cystic Fibrosis Nurse Specialist - we get the chance to get to know patients and their families incredibly well and we accompany them through the best and worst of times.  I had known some of the patients for twenty-six years when I came to retire and you really do become part of the family, whether you choose this or not.  It is an extremely privileged position to be in; a chance to be a part of other people’s lives when they are going through the most traumatic or uplifting of times.  I have been to many weddings, twenty-first birthday parties and fundraising events, but I have also been to countless funerals and been alongside patients as they reach the end of their lives.  This is a great privilege but it can also be an unbelievably tragic and poignant situation for all involved.

CF affects many of the body’s major life systems.  As a result of this, people with CF are cared for by a multitude of health professionals, collectively known as a multidisciplinary team.  Multidisciplinary teams in NHS hospitals are now commonplace for a whole variety of diseases and illnesses, but people with CF were one of the first groups to be looked after in this way.  Working within a multidisciplinary team can bring its own challenges but can also be very rewarding, especially when different disciplines work together, bringing their own expertise, ideas and respect for each other. 

During my career in CF, I was also fortunate enough to work with nurses and other health professionals throughout the UK and further afield.  I learnt so much from these other experts and I relished the opportunities to attend meetings and conferences all over Europe.  To meet others from such diverse backgrounds but all with a common goal - to care for those with CF - has been without any doubt one of the highlights of my nursing career.  I will never forget many of the CF colleagues and friends I have met over the last twenty-six years and consider myself very lucky to have been in this position.

This book is dedicated to my family, friends and work colleagues, all of whom have given me support and encouragement over the years - I am truly grateful to you all.  But most of all, this book is dedicated to all the patients and families I have met; an amazing and inspirational group of people - you have all taught me so much about how to live life and enjoy every moment - thank you all.

1

~ What is Cystic Fibrosis? ~

Cystic fibrosis (CF) is the UK’s most commonly inherited genetic recessive disorder.  By this, I mean that both parents need to carry and pass on one copy of the faulty CF gene in order to have a baby with CF.

I once worked with a wise respiratory professor who always described the faulty CF gene as being two letters that have been printed the wrong way around in one word of one page in a dictionary.  All the other thousands of words in the dictionary have been printed correctly and on skimming through the book, no one would notice this misprint.  But in fact, this one misprint can have a major and devastating effect on a person.  This is CF; everything else can be working perfectly but due to this one small misprint, a person’s life can be changed in an incredible way.

One in every twenty-five people in the general population in the UK will be a carrier of the CF gene.  It is extremely common.  In a class of school children, at least one child will be a carrier of the CF gene; in a large business meeting, there is a chance that at least one person will be carrying the gene; in a rugby match it is likely that one player will be a carrier.  But you do not know if you are a carrier of the CF gene unless you are tested for it, or if you produce a child who has CF.  Many of us may be CF gene carriers without ever realising it.  So, two CF gene carriers need to meet each other and then there is a one in four chance that their child will be born with CF. 

The chances are the same (one in four) for each pregnancy and the condition affects boys and girls equally.

There are many different manifestations of CF from the very mild (many people walking around today with mild CF will be totally unaware) to moderate and severe effects of the condition. The severity of the illness may depend on the genetic mutation of the CF gene inherited from the parents.

The mechanics of CF are complex and scientific, so this book will not go into them in any great detail, but to put it simply, the salt and chloride channels in every cell in the body are affected to varying degrees, thus not allowing these substances to move freely.  As a result, any mucus-producing area of the body will be affected by CF, mainly the lungs but also the gastrointestinal system and the reproductive system.  In other words, three of the main bodily systems conducive to life - breathing, eating and reproducing - are affected.  The heart and brain are usually completely normal. Again, this is a complex area to explain, but it is an important one to understand especially with the new classes of medications that are emerging to treat the condition.

The CF gene has over 1000 different mutations and more are being discovered all the time. 

¹

The most common CF genetic mutation in the UK and Europe is one called Delta F508 (or F508del as it is now more commonly known).  Approximately seventy percent of those with CF in the UK will have one inherited F508del mutation and around forty-eight percent will have F508del mutations for both of their inherited genes.  There are many other commonly recognised mutations in CF and many will dictate the course of the disease, whether it be a mild or more severe manifestation of the condition.

New disease modifying precision drugs have been developed to treat CF over the last few years and it is now vital for patients and health professionals to be aware and informed of their specific mutations, in order for the appropriate medication to be prescribed.

Symptoms of CF can be many and varied for each person.  No two people with CF is the same and they can never be compared with each other.  There are definite trends and patterns in the illness, and as health professionals, we can take a good guess as to the course of the condition for each of our patients, but this will depend on many things including medication adherence, current age and stage of the patient, their genetic mutation and personality. Many of these things will vary depending on general life events and external stressors, meaning that it is usually impossible to accurately predict the future for each patient.  This can be frustrating for patients and their families, especially in this day and age when people like to know what the future holds and want to have things mapped out in front of them.

Impossible to do in CF!

CF mainly affects the lungs, although a baby with CF is usually born with perfectly normal organs.  The constant and chronic infections, inflammation and bacterial colonisation causes lung damage over the years which is why all CF teams try to prevent this from occurring from an early age.  All bacteria thrive in the warm, moist and inviting atmosphere in the lungs - a perfect environment for them to flourish.  All patients are experts in coughing up sputum and providing constant samples for the hospital labs to analyse and report on. 

Throughout my nursing career, sputum has been one of the few things I have found difficult to come to terms with - thick, yellow or green slime, sometimes with all sorts of unidentifiable bits in it - even writing this makes me feel squeamish and nauseous.  Yes, strange that I ended up in the field of CF, where expertise in sputum evaluation and identification is vital.  Patients would often have their sputum pots on their bedside table next to their trays of lunch or supper and would take great pleasure in taking off the lids and showing the contents to me just after I had eaten my own lunch.  But I always tried never to show my true reactions to this - to this day I hope none of the patients realised how horrified I was when the dreaded open pot was pointed in my direction.

However, for people with CF, sputum production and coughing are a daily part of their lives and they have often not known anything different.  Bacteria, with scary sounding names such as staphylococcus and pseudomonas will often become colonised in the airways and lungs, resulting in thick, infected looking sputum and a chronic deep and painful sounding cough.  Patients often tell stories of being on a train or in a classroom and receiving disapproving looks from other people as they try to contain their coughing, to make it sound