My involvement with Cystic Fibrosis
Cystic fibrosis is a a fascinating and complex condition. My interest in the condition leads back to the time at which I was diagnosed as suffering from CF at the age of 10. Subsequently, I have followed the scientific and medical literature concerning the disease very closely.
My first encounter with CF research came when I was at the University of Surrey, taking my first degree in Microbiology, between 1977 and 1980. For my final year dissertation, I studied the effects of combined antibiotics against Staphylococcus aureus from CF patients, with a view to improving treatment by using synergistic interactions between the drugs.
As I studied Medicine, I became interested in the genetic, biochemical, pathophysiological aspects of the disease, in the pathology of how lung damage occurs in the disease, and in particular, how best to treat it. I became aware that very few older patients with CF existed in the UK, and outside of a few specialist centres, the treatment that they got for the condition was far from the best. This led to my involvement with the Association of CF Adults in the UK. It also became a major goal in my life to ensure that all children and adults with CF in the UK had access to the top quality medical care on which their lives depended.
After I qualified, I was interested in becoming a respiratory physician. In pursuing this aim, I undertook one voluntary attachment at the Brompton Hospital in London, followed by a substantive SHO job. Although I was not working directly with CF patients, I came into contact with the CF patients, and had to look after them out of hours when the regular doctors were off duty. I sometimes got called in to add a personal angle to their treatment. This sometimes had dramatic results. I remember one young lady who was only mildly affected by CF, like myself. However she was simply wasting her life away in hospital. I talked to her about how I managed to cope with my job. I saw her a few years later - she looked fantastic! She looked beautiful, had taken a degree, got a really good job, and a really good man! I cannot attribute this to the talk we had, but if it helped at all, then it was worth it.
As I moved up the career ladder, I got interested in the process by which care for patients was organised in the UK, and found it to be lacking. What kind of care you got was a lottery based on the interests and whims of local doctors and where you happened to live in the UK. At this time, the Conservative Government were introducing reforms to the National Health Service which could potentially be very damaging for people with CF. CF was actually drawing me into the field of health services research and health policy and politics, and ultimately to Public Health Medicine a a specialty.
My first job, on entering Public Health, was to undertake the first of two national surveys of adults with CF. I chose to look at adults, because with the improving prognosis for people with CF, nobody quite knew what to do with large numbers of fit, active and healthy adults with CF, let alone with those who were severely affected. The first was in 1990, and highlighted lots of problems with access to care, and quality of care. The second was in 1994, and demonstrated some improvements, but also some problems introduced since the NHS reforms, including difficulties with general practitioners complaining about the cost of medications now they had to manage their own drug budgets. The third study in 2000 demonstrated further improvements in care for people with CF, but also increasing concerns regarding cross infection in specialist centres.
I was then commissioned by the NHS-R&D to study access and availability to specialist services for CF, and the process by which the internal market worked (or didn't work) in securing access for patients with CF to specialist care. Two surveys were undertaken, one in 1992, the other in 1995. For these studies I undertook two national surveys of parents of children with CF, as well as incorporating the adult survey work. I also surveyed health authorities, general practitioners, and providers of care from those with just a few CF patients, right through to the largest centres in the country. The first study led to some strong recommendations for change, and by the time the second survey was carried out, it seemed that some of these had been successful, although to a variable extent. There were still lots of problems, including patients attending the same hospital getting different treatment depending on where they lived, because different health authorities would pay for different types of treatment.
I have also been involved in developing clinical guidelines for CF care. However, it struck me that although these guidelines were meant to be based on a review of published evidence, in many instances there was no evidence for effectiveness of treatments or management programmes that we were recommending. This stimulated my interest in evidence-based medicine, and led to me becoming involved with the Cochrane Collaboration CF Group as an editor for a period of time, a post which I finally gave up in 2001.
A final interest is the accessibility of good quality information about CF to patients and professionals. In particular a desire to provide good information and also to teach people looking for information on the web about CF to evaluate the information available and to sift out the few pearls from the heap of poor quality information that is available. This has culminated in the ongoing CFStudy.com project.
Current work-related CF interests include
Although I am now a successful doctor, it was not easy to achieve this whilst having CF. I was diagnosed as having CF at the age of 10. Although I am relatively mildly affected by CF, it still pervades every aspect of my life. Here are some of the things that particularly interest me on a personal level about CF
My involvement in voluntary work for CF began very early in life, knitting woolly hats to sell at coffee mornings to raise money for CF research.
The next stage came whilst at Medical School, when I became involved as an inaugural member of the Association of CF Adults (UK). The inaugural committee was a talented and determined group of individuals. We were all determined to something to improve the lot of the adults with CF in the UK. Adults at that time (nearly 20 years ago) were treated as a curiosity. The medical teams were not always sure how to deal with us. Having laboured for years to keep children with CF alive, they were suddenly faced with armies of young people wanting jobs, their own homes, mortgages, cars, life insurance, their own children. It was difficult to know how to deal with adults wanting to be doctors, lawyers, priests, professional sportsmen and women. Young people with CF were suddenly taking off, becoming independent, and exploring the World. Medical teams were faced with the problems of getting nebulisers up the Himalayas or the Amazon jungle. These were problems simply outside their experience, and ACFA felt that a forum was needed where adult patients could share their own information, experience and practical advice.
At the same time, adults with CF were facing considerable problems with things that to others are mundane: getting places in higher education, obtaining employment, obtaining driving licenses, life insurance, mortgages, private pension plans and so on. Much of the difficulty was due to the existing prejudicial thinking that CF was a childrens' disease, and that adults were an exception who would eventually become a liability. I do not think this set of prejudices has been fully overcome 20 years on, although progress has been made.
Finally, those adults who were disabled by their illness were having inordinate difficulties in obtaining social security benefits, since the rules on disability were inflexible, and geared towards those in wheelchairs, ignoring other types of difficulty such as breathlessness.
ACFA set about developing information to distribute to adults, thus empowering them to change things. We also collected information through surveys, and compiled a book based on our own experiences to help those to come. Local group meetings, national and international conferences were organised, newsletters were produced, and the organisation grew in influence, stature and membership. Unfortunately problems due to cross-infection with Burkholderia cepacia severely curtailed activities. At the same time, I was moving on and up in my career, and simply could not spare the time involved to organise activities. So I remained a member, but the older generation has given over to the young, and ACFA has moved on - indeed so much so that because the majority of patients with CF are now adults, its role has been taken over by the CF Trust, and adults with CF have moved into the mainstream.
This does not mean that I do not support the causes of adults with CF. I am frequently invited to speak at meetings on behalf of the CF Trust. These are usually meetings to which adults, parents, doctors and health care professionals, and other interested parties are invited. When I was a teenager, there were very few older patients who could pass on their experiences. The prejudice about CF being a childrens' disease, and the prognosis being poor still remains among the general public. It is hard work to travel and prepare for these meetings, but the look of hope on the faces of young people with CF, parents whose babies have just been diagnosed, and even the health care professionals themselves makes it all worthwhile.
In 1996, I undertook a major challenge, when I did the Three Peaks Walk for CF. This involves walking up the highest mountains in Wales (Snowdon), England (Sca Fell) and Scotland (Ben Nevis) in one weekend. I trained for months for this event, although I was already quite fit. I managed Snowdon well enough, and got to the top of Sca Fell, climbing mostly at night. On the way down from Sca Fell, I fell and twisted my right knee, damaging the cartilage. I managed to walk down, and climb part of the way up Ben Nevis, but the knee was too painful. Despite being 37 years old at the time, and having CF, it was not my lungs that failed me in the end. And despite the injury I raised over 1000 UK pounds for CF research.
I continue to support fundraising for CF whenever I can. Recently Quick Quacks motorcycle club - a club for doctors who ride motorbikes of which I was a founding member - made a donation to the CF Trust.