Helping cystic fibrosis sufferers take control of their disease

One in 20 of us in Northern Ireland carries the defective gene for cystic fibrosis. Valerie Hall, nurse consultant in the field, tells Jane Bell about advances in treatment for the disease

Nurse Consultant Valerie Hall says care for people with CF is continuing to evolve

Cystic fibrosis (CF) is a common inherited disease mainly affecting the white European population. The gene mutation in CF causes thick sticky mucus to accumulate in organs such as the lungs and pancreas. People with CF have to undertake an intensive daily treatment regime with inhaled drugs, oral medications and physiotherapy. Most adjust to this regime throughout their lifetime and live as full and normal a life as possible. Our philosophy is to enable sufferers to gain control of their CF and not have CF control them.

With 1 in 20 of us in NI carrying the defective gene for CF, what are the implications?

In order to have CF a child must inherit a defective gene from both parents. The likelihood of this happening is very low. We are fortunate here in NI that neonatal screening of all babies has been available since 1989. Mothers will be familiar with the heel prick test and CF is one of the illnesses which are screened for on the Guthrie card. On average 10 babies are born each year with CF in NI. Some people are still being diagnosed in adulthood. These generally are people who may have been born before screening was available or who have mild or atypical CF.

There are two regional Cystic Fibrosis Centres in NI. Who do they cater for?

The Regional Paediatric Centre is located in the Royal Belfast Hospital for Sick Children and provides care for 190 children. The Regional Adult Centre is located on the Belfast City Hospital site with 220 adults. A seamless service is provided between the two centres with transition from paediatric to adult care taking place around 18 years of age.

What have been the main advances in treatment in, say, the past 10-15 years? And what has it meant for sufferers?

There has been a dramatic improvement in quality of life and survival. This is due to a number of factors including aggressive nutritional support, more effective formulations of pancreatic enzymes and antibiotics, airway clearance techniques and drug delivery and the establishment of specialist units with multi-professional teams expert in CF management.

The ultimate treatment is lung transplantation. What are the issues here?

People with severe lung disease will be given the option of being referred for lung transplantation. The main issue surrounding transplantation continues to be the lack of donor organs. The average waiting time for lung transplantation is around 2 years. Unfortunately not everyone needing a lung transplant will receive one in time. For those who do, this completely transforms their lives. We continue to desperately need more people to join the organ donor register.

What drew you into this area of nursing?

My background is respiratory nursing. When the opportunity arose in 1995 to develop what was then a newly commissioned CF centre for adults in Northern Ireland, I decided that challenge was for me. Some 12 years later I cannot imagine doing anything else.

What are the satisfactions and frustrations of the job?

It is a privilege to provide care for and to work in partnership with this remarkable group of people. Each day brings new and exiting challenges. Care for people with CF continues to evolve and seeing the real difference that this makes to people's lives is extremely rewarding. It is an honour to work with such a dedicated team including doctors, nurses, physiotherapists, social workers, dietitians, pharmacists and many others who all make an enormous contribution.

One frustration of the job is watching those waiting for lung transplant.

Another is obviously never having enough hours in the day.

You were among the finalists in the RCN Nurse of the Year event. What do the scope and standard of entries in the Awards tell us about nursing today?

This event provides an opportunity to celebrate the excellence in nursing today and to recognise special achievements. Nurses in Northern Ireland can be proud of the standard of care and services they provide throughout the health service. When I listened to the presentations and standard of entries I was humbled to have been chosen as a finalist. This was not simply however about those of us honoured at the event but recognition of the contribution of many nurses out there whose work often goes unrecognised. I would like to take this opportunity to thank the RCN for hosting such a wonderful event.