At last year's conference on cystic fibrosis (CF) at The Royal Society of Medicine, a mother disclosed that, when her child was started on a new drug, she hoped that it would not work.1 She described immense relief when the new treatment proved ineffective and was stopped. Her reason was simple. She has two children with CF. Both receive two nebulized drugs twice a day, and this together with the twice-daily physiotherapy regimen already occupied 2 hours 20 minutes per day—a considerable proportion of her day and theirs. The thought of another nebulized drug eating further into the family's time for living was simply intolerable.
Probably the only unique feature of this mother was her willingness to admit to such feelings in front of an audience of 200 CF health professionals. The daily grind of physiotherapy, nebulized drugs, exercise, regular medication through the day and oral enzyme therapy with all food, close attention to eating to ensure an adequate dietary intake, visits to doctors for prescriptions and to chemists to obtain the drugs, and regular hospital visits, can invade the lives of all families with a CF child.
The message was rammed home to me a matter of weeks before the conference, when I prescribed two antibiotics to be given intravenously in high dosage in a patient with CF. After discussion with a microbiologist and the pharmacist, it was agreed that the patient needed 3.5 g of one antibiotic eight hourly and 3.6 g of a second antibiotic six hourly. Ordering the drugs seemed simple, until I was confronted by the CF specialist nurse who had calculated the time taken to dissolve the antibiotics in saline, draw up the correct dosage, infuse the drugs through a conventional 'long line'2 (the narrow bore of which prevents rapid bolus injection), flush through with saline, and finally heparinize the line. The total time to perform all these tasks was estimated to be 8 hours for each 24-hour period. It was just as well that this treatment was to be given at home, for I was also told that the ward had insufficient trained nursing staff to devote to this task.
It is all too easy to prescribe a treatment and give advice without thought to the implications for the family. In view of the demands CF doctors make, the marvel, perhaps, is that any CF families manage to follow all the advice. The CF team regard physiotherapy as indispensable. Many families see it as a curse, and most loathe it and the time it takes. The CF team see nebulized and other medications as a major advance, but families are likely to see these as a wretched invasion of family life and yet another source of confrontation with an understandably reluctant child who would prefer to do other things.
Modern treatment offers new hope of survival for children with CF. When my paediatric career started, CF care dwelt largely with the terminal stages, and a defeatist attitude and a poor outcome were inextricably linked. My accumulated knowledge of terminal care is now rarely needed, but the experiences of parents leave no room for complacency. We badly need ways of treating CF that do not eat so heavily into the lives of our patients. Until better forms of physiotherapy become available, and until faster and more efficient nebulizers become affordable, we should stop and think hard before prescribing. The efficacy and cost of a treatment should not be the only considerations.
The topic of coping with CF, as perceived by a mother and as perceived by a professor of health psychology, was just one of the themes covered in the RSM meeting last November. For interested readers the full proceedings of the meeting have now been published as a supplement to the JRSM.3 In addition the papers from the 2001 and 2002 RSM CF conferences are available in electronic form on the JRSM website.