When I was first asked to write about the work that I have done for the last 26 years, I thought it might not be too difficult, but when I finally settled down to write I found it hard to know where to start. I have been incredibly lucky in my job and my choice of specialisation, having more or less ‘fallen’ into this post in 1993. I moved to Aberdeen from London in 1991 because my husband's job as a geologist had taken us to the North East of Scotland, which was quite a culture shock for me, having lived for 15 years in London's East End. I had completed a District Nursing qualification and I was settling into life in Scotland, making new friends, and had also just had a baby.
I did not apply for the post when it was first advertised, as I felt I had no knowledge or experience in looking after people with cystic fibrosis (CF). However, I did apply the second time and I was fortunate enough to be given the job. The CF specialist job was part time and involved setting up a new adult service in Aberdeen Royal Infirmary.
CF is an inherited, recessive condition affecting around 10 500 people in the UK today. Around 1 in 2000 births will be a baby affected with CF and in Scotland it has been routinely tested for as part of the newborn blood spot screening (Guthrie test) since 2003. It mainly affects the lungs but the gastrointestinal, reproductive and hepatobiliary systems may also be involved. CF is an incredibly complex disorder. Patients are often required to take vast quantities of regular medication, and have physiotherapy in order to keep themselves as well as possible. Many of those patients are in the adolescent age range and it is difficult to imagine how hard this must be for them, when they are trying to become independent adults and fit in with their peer group at the same time. It is a tricky period for any teenager, let alone when CF is involved as well.
I started my new job as a CF nurse specialist in August 1993 and loved it from the start. The service was led by two inspirational respiratory consultants and I will never forget their devotion to the patients or their expertise in the field of CF. There was a small CF multidisciplinary team when I started, comprising the consultants, a physiotherapist, a dietitian and me. We were a great team and it was a privilege to work with such dedicated and motivated people.
‘This has been a job of tremendous ups and downs, highs and lows. We go through good times with the patients and their families, but also extremely difficult periods that can be heartrending for all involved’
When I started working in CF, there were 24 adult patients attending the clinic. The average life expectancy at that time was about 28 years of age and the main treatments for the condition were antibiotics and physiotherapy. New treatments were being developed all the time and over the years life expectancy has become better and better. In 2000, the adult clinic became larger than the children's clinic for the first time, which I felt was a significant milestone. Now, as I am about to retire, our numbers have reached 70 adult patients and treatment options are much more varied—the latest drugs being produced are incredibly exciting. I feel that after all these years, a potential ‘cure’ for CF is in sight.
Over the years, as our patient numbers grew, so did the size of the CF team. Team members came and went but the majority of the team remained stable. I think that once people start to work in the field of CF, it draws you in and makes it hard to leave! It is a unique area to work in, as you get to know the patients and their families incredibly well. You are with them throughout their journey, through the good times and the bad times—I do not know of many other nursing roles where this is the case. We see most patients on average every 2-3 months in clinic; some we will see much more frequently, others just once a year. I have known some of the patients since they were 13 or 14 years old, and they may now be in their 40s. As you can imagine, you become very involved in their lives over the years. This has been a job of tremendous ups and downs, highs and lows. We go through good times with the patients and their families, but also extremely difficult periods that can be heartrending for all involved.
The majority of our patients are young adults and CF can be a tough condition to live with. As it progresses, the possibility of lung transplant is often considered and patients know this is a last resort. When lung transplantation is successful, it is an absolute miracle, a transformation of a patient's quality of life. However, there will be times when a patient will not reach the point of transplant. This can be for many reasons, including the patient's own choice. We have had several patients who felt transplant was not the right route for them, that they would rather die with good palliative care, in a place of their choice and with people they wish to have surrounding them. This can be hard for families (and sometimes for us) to accept but we always need to respect the decision of the patients and, in fact, I think it can show immense bravery on their part. Even when a person decides to go down the transplant route, there are always more people waiting for new lungs than there will ever be organs available, so inevitably there will be some people who die on the waiting list. This scenario is heartbreaking for everyone.
Over the years, I have been closely involved with many of our patient's deaths, and although this can be traumatic and difficult it can also be a huge privilege. The death of a young person is always very hard to come to terms with and it can be heartbreaking to witness a family's grief and distress at this time. All we can do is be there for them and alongside them, and we offer as much support to families afterwards as they wish to have.
Conversely, when a successful transplant happens, it can be the most joyful thing. This does not mean that life is plain sailing afterwards—a great amount of hard work and diligence is still required in order to keep well—but it is incredible to see the transformation in a patient's quality of life and wellbeing. I want to mention a recent example of a patient who I have known for more than 20 years. Her condition had been declining for many months. She was finally admitted to our respiratory ward on a long-term basis on nasal ventilation with high-flow oxygen and was too breathless to walk or eat properly. She had been on the lung transplant waiting list for 2 years and time was running out. Due to her deteriorating condition, she was finally put onto the urgent national transplant list and she got her new lungs just a few weeks ago. We have been in regular contact with her and her husband, and the latest communication was a photo of her eating a huge cheeseburger. Her lung function has gone from a predicted value of 14% to 81%—what a miracle. And, as I've told her, an amazing ‘retirement present’ for me.
So, although much of my job over the last 26 years has involved end-of-life and palliative care in CF, the future is bright. New drugs that are coming along the pipeline look very promising and these are exciting times in the world of CF. I have been so fortunate to have had a job in this field and to have been involved with such an inspirational group of patients.
The icing on the cake was to have been nominated for the British Journal of Nursing Awards 2019 in the ‘Nurse of the Year’ category. It was an incredible evening of celebration and I was joined by family and friends. For me, it was a perfect end to a very enjoyable and worthwhile career.