Cystic Fibrosis

My involvement with Cystic Fibrosis

Professional	I am involved in a lot of work for CF at the professional level. Read about my professional interests and research in cystic fibrosis here
Personal	I also have cystic fibrosis myself. My biography, detailing my experiences with CF from the date of diagnosis until the present, at the age of 48 years is also available for you to read. My experiences with sport and exercise are also detailed in an article here.
Voluntary Work	Over the years, I have been involved in raising money for CF, as well as spending it on research. I was also a founder member of the Association of CF Adults UK

To learn more about CF, please visit CFStudy.com

Professional

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

Cochrane CF Group (former editor, reviewer, criticism editor)
Involvement of consumers in setting research agendas (both primary research and systematic reviews)
Member of Research and Medical Advisory Committee, CF Trust
Member of CF Trust sub-groups on Infection Control and Clinical Standards
UK CF Clinical Database - member of steering committee and provision of advice regarding analysis
Screening for CF - population genetic screening, and postnatal screening
Maintaining this CFStudy.com web site for everybody in the CF community
Peer review of articles relating to CF research for several journals
Peer review of research grant applications to the CF Trust and other funding bodies
Needs assessment in respiratory disease for a national project, including cystic fibrosis
Research into the epidemiology of cystic fibrosis by way of national surveys and use of data from the UK CF Clinical Database - this includes particularly looking at ways to measure variations in clinical care and evaluate outcomes of care.

Personal

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

Illness and uncertainty	Coping with CF is all about coping with uncertainty. You never know what tomorrow might bring. It is difficult to plan anything in the short or long term. In the short term, a planned holiday or meeting with friends may be disrupted by serious illness brought on by something as trivial as a cold. In the long term, it is hard to be serious about planning your future or even your retirement, when all the popular conceptions of CF are about a disease that is fatal in childhood.
Employment	All through my working life I have found people who were willing to ignore my CF and allow me to work on my merits, for which I have been profoundly grateful. However, I have also come across people who were far more interested in what I could not do, than what I could do. My sick leave record over the years has been good, although recently, I have experienced two prolonged bouts of illness keeping me away from work for several months. I am fortunate that my employers look at what I have achieved over that period of time, which is equivalent to any other employee, rather than counting the days that I spend at my desk. Employment for people with illness is becoming progressively more difficult as employers take on more financial responsibility for paying them whilst they are sick. Any illness is seen as a liability, rather than a potential asset. I have always worked harder than I need to because I am aware that as one of the first doctors with CF, people are using me as an example. People with CF want to contribute, to work, to be normal individuals in a normal society. It is frightening and degrading when employers will not allow you to employ your skills, just as it is a constant worry that you will be able to manage should your working days be interrupted, or ended by illness
Home, Family and Relationships	Ultimately, all relationships are affected by CF. I am interested in the way in which relationships change as the nature of CF changes. I am also concerned that there is a general assumption among the health care professionals that adults with CF can always live with their parents if they cannot get a mortgage or find a partner. There are a growing number of 40 plus year olds with CF whose parents are elderly and may be infirm. A growing number have no living parents. Parents cannot be assumed to be the carer. Partners may have difficulty if the CF becomes worse and they are suddently expected to become a carer: the beloved partner becomes burden instead of a treasure. The needs of older patients with CF need to be considered: housing needs, financial needs, care needs, and the burden of care they face looking after their own children, and elderly parents and other relatives in addition to their daily treatment regimen.
Ability and Disability	There are times when you need to consider ability to do something, and times when you need to make the most of your disability. I have spent all my life making the best of all those things I CAN do, rather than focusing on those things I cannot do. Disability usually means to the general public that you are in a wheelchair. I am allowed to park in the disabled spaces at work, but never do, because I get so frustrated at having to explain my disability to people who see me walking and think I am fully fit.
Fitness and Exercise	I am convinced that keeping fit and engaging in sport has significantly enhanced my life. From my teens, I have been involved in sport, sometimes at a high level (county cricket level, club athletics and so on). Keeping fit is a challenge for normal people of my age, but becomes much greater if you have spells when you cannot train because of acute respiratory illness. Training benefits are lost very quickly, particularly aerobic fitness. It is frustrating to have to start all over again, three or four times a year, because of bouts of sickness. Fitness is essential to my lifestyle, so I simply have to grin and bear it each time I re-start training. But at the age of 48 I am still fit enough to do a hour of kickboxing, power step aerobics, swim a mile in 45 minutes, cycle 100 kilometres, and do weight training. This underlines the importance of fitness and exercise, and being very determined to keep fitness levels up, come what may. I have written an article about sport and exercise and CF, which can be obtained from the CFStudy.com web site.
Being a Doctor and Patient	It is very difficult being a doctor and a patient. When you are ill, you don't want to take responsibility for your own illness, yet you cannot help it. You cannot help lying in a hospital bed, reacting to colleagues' bleeps, and going through the list of differntial diagnoses from which you might be suffering. You cannot help wondering if your colleagues have forgotten something, however much you trust them. You cannot help wondering if your doctor thinks you are making it all up, using your medical knowledge to fool them. You cannot help noticing the trembling hand and sweaty forehead of the junior doctor sent to take blood from a Consultant. You don't want to ask questions in case your colleagues think you should know the answers. The nurses assume you don't want any help, and are reticent to talk to a Consultant. You don't want to be a bother, so you become a bother by not asking for help. You don't get a shoulder to cry on when you are a doctor. You become a doctor because you care for people, so ifyou are on the open ward, you have to respond to other patients' calls for help. I often end up doing little nursing jobs for people. I even show junior doctors how to put up drips, and nurses how to use the new digital thermometer. You are a patient, but you cannot fraternise or sympathise with patients, because you don't have the energy to answer their questions when they find out you are a doctor. Being a doctor is more than a job, it changes you forever as a person. So you cannot switch it off when you are ill yourself. Colleagues don't really trust you because you are a patient, and might write about your experiences in a patients' newsletter, thus exposing their weaknesses. Fellow patients don't trust you because you have become one of them....you are no longer a proper patient. But being a doctor doesn't stop you hurting, doesn't stop you being breathless, doesn't stop the mental anguish of not knowing whether you will recover, and doesn't stop you being a human being who needs a friend, needs comfort, needs a cuddle and a shoulder to cry on when you are ill. Being a doctor and a patient is very difficult.

Voluntary Work

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.