Cystic fibrosis

Learning from illness

Dr Nicola Herron reflects on how losing two siblings to cystic fibrosis has influenced her as a person ­ and as a GP

The music festival was fantastic. The day culminated in a concert featuring the Hothouse Flowers and Juliet Turner. We brought teenagers from four local schools together for a day of music workshops and discussions about transplantation.

The kids also met a little girl who had had a liver transplant the year before and were mesmerised by her.

The festival was in aid of Live-On, the charity we set up after my younger sister Rachel died of cystic fibrosis in 2003, aged 36.

I'd also lost my brother Michael to CF in 1996, aged just 21. Our experience with CF and transplantation drive us to raise awareness and some money for local patients who are travelling to England for a transplant.

Rachel's story

Rachel was born in 1966, less than a year

after me. It was obvious quite early on that she had a problem with malabsorption. A tentative diagnosis of coeliac disease had been made and she was on a high-calorie, gluten-free diet.

But it wasn't until our younger brother Michael was born eight years later that CF came on to the horizon.

We all had a sweat test. Rachel and Michael tested positive while my two older brothers, Paul and John, and myself were given the all-clear. By this stage Rachel was starting to have some respiratory symptoms and the gluten-free diet wasn't controlling her malabsorption.

Though it was good to have an accurate diagnosis and for her to be started on the

CF treatment regime, the prognosis for the condition at that time was very poor and the diagnosis brought little comfort.

Rachel's symptoms responded well to her new treatment regime. She had chest physio twice-daily and took an enzyme supplement with meals. She had regular antibiotic treatment as an inpatient. This was the gold standard treatment at the time.

Rachel became a nursery school teacher, but after several admissions for chest infections she eventually admitted classroom teaching was seriously damaging her health and switched to working as a home tutor.

By the time Rachel was 36 her restricted lung function was limiting her activities. What none of us had realised was that Rachel's compensatory mechanisms had been working increasingly to maintain a viable lung function.

One day, out of the blue, she found she couldn't breathe. She waited for things to settle and when they didn't she dialled 999.

After a lifetime of gradually increasing respiratory symptoms this came as a shock. It became apparent no amount of anti-

biotics or physio were going to improve things this time.

Rachel's team in the unit in Belfast City Hospital discussed the possibility of transplantation. Rachel didn't hesitate. She had seen how a lung transplant had transformed Michael's life so she agreed to be assessed immediately.

The team from Newcastle do a regular outreach clinic in Belfast. They met Rachel and recommended that she got a PEG tube inserted.

They also asked for bone densitometry and other investigations to be completed before Rachel would be accepted for full assessment in Newcastle.

All these investigations and procedures took their toll and Rachel became very

unwell after her PEG insertion and it took another few weeks before she would be fit to make the journey to England.

The average wait is currently 14 months in most centres. Unfortunately for Rachel the wait proved too long. Her lung function continued to deteriorate and she died in

September 2003.

Michael's story

Michael was diagnosed with CF when he was just a couple of weeks old. When he was 12 we asked about transplantation, but were told the transplant and the post-treatments would make life very difficult for him. But Michael's CF was causing rapid lung deterioration, making his quality of life nearly non-existent anyway.

We had also watched other children on the ward become sicker and die and it

was only a matter of time before Michael would succumb. Eventually we persuaded Michael's medical team to refer him to Great Ormond Street for transplant assessment.

The transplant team there could not have been more different in their approach. They didn't gloss over the problems with transplantation, but they did seem to understand how hard it is for a child to live with CF. They were also very honest about the fact that there were no guarantees of Michael getting a suitable set of organs in time.

They did, however, also tell us the positive side of transplantation and they were so enthusiastic about it that we began to feel hopeful about Michael's future for the first time in months.

This was the start of a very long journey for Michael and the rest of the family. He had his heart-lung transplant in January 1989 when he was 13. He had a second transplant in Harefield hospital in 1994 ­ he needed a single lung this time.

The transformation in his life after his first transplant was miraculous. He returned to school and joined in all normal activities for the first time in his life. He formed a rock band and even opened a computer games shop.

And because CF patients already take regular medications and avoid people with infections they are good patients for transplants. Michael lived until September 1996, when he died of renal failure.

The influence of CF

My experiences in hospital influenced my decision to go into medicine. I was impressed by the team work in hospital

wards, and the positive effect the staff have on people's lives.

As a GP, the experience has given me a powerful awareness what it is like to be 'on the other side'.

Even as a qualified doctor I often felt unable to express how I truly felt, or to ask all the questions I wanted to ask.

My experience on the other side of the fence has illuminated the potential pitfalls of the all-powerful clinical team. Patients who want to have a say in their care can

be seen as interfering and awkward. I now deliberately try to see their side of a debate when there is a difference of opinion.

Being the 'expert' member of the family was difficult for me. Both Rachel and Michael placed great trust in my opinion and ability to fix things when they were

going wrong. I was really glad that my husband Joe is also a GP. I had to appear confident, and I often had to explain and excuse some of the inadequacies in the NHS.

I haven't mentioned my family history to any CF patients since Rachel and Michael died. I'm not sure if it would be wise or helpful to them.

I now feel deeply saddened when I meet people who have lost their love of being alive. I never once heard Rachel or Michael say they wanted to die.

Nicola Herron is a GP in Derry,

Northern Ireland

What I've learned

· Always give a little hope ­ even if it's only about symptom control. Be honest but kind when discussing a patient's condition.

· Watch the teenagers with CF ­ compliance is a real problem at this age.

· Discuss transplantation early ­ remember it is 18 months at least from referral to transplant.

· Parents of CF children will probably be exhausted, anxious and scared, so treat them with kid gloves. Let them know that you are there for them.

· When a patient with CF dies the relatives' grief will be mixed with an element of relief that the patient's suffering is over and that they no longer have to worry about what the next day is going to bring.

Let the relative know it is okay to feel that mix of emotions. They may be feeling guilty that they are feeling that way.

· People with CF are living longer than ever before. Life expectancy of a person with CF has risen from only a few months in the 1930s to about 31 years now.