Despite an improving life expectancy cystic fibrosis is still a life-limiting non-curable illness that requires end of life planning, says Lieven Dupont from Leuven in Belgium.
There were many barriers to having an end-of-life discussion with patients, Lieven told delegates attending the symposium on building a bridge between international guidelines in CF care and CF clinical practice.
For instance the psychological view of the patient, their family and medical team was often one of ‘fighting on’ in the hope of transplantation.
However providing therapies that result in clinical improvement for the patient does not preclude a separate strategy aimed at assessing and alleviating unpleasant symptoms, he told delegates.
A good approach to take was to “hope for the best, aim for the good but prepare for the worst result,” he said.
In essence there were four questions healthcare professionals should ask their patients.
What is important to you and gives your life meaning?
Are there any treatments or types of care that you would or wouldn’t want?
Who would you want to make decisions on your behalf if you weren’t able?
If there was a choice, how and where would you like to spend your last days?
Although therapeutic innovations may continue to enhance survival and quality of life patients, families and physicians will need to confront the premature mortality that accompanies the essentially fatal diagnosis of CF, Lieven concluded.
“Compassionate and skilful end of life care will remain an essential part of a comprehensive care for patients and their families, requiring specialist skills and good cooperation of the CF and palliative care team,” he said.