Cystic fibrosis and survival to 40 years: a case-control study

Abstract

The clinical course of patients with cystic fibrosis (CF) is variable and probably determined by many interacting factors. We aimed to examine the influence of early social and clinical factors on long-term survival.A case-control study of adult CF patients was used to compare long-term survivors (aged o40 yrs) with patients who died before reaching 30 yrs of age. Each case (n578) was matched by birth date with at least one control (n5152), after exclusion of ''late diagnosis'' patients. Probability-weighted logistic regression models were used to identify influences on survival.Factors resulting in increased probabilities of survival included high body mass index (OR 1.76, 95% CI 1.40-2.22), forced expiratory volume in 1 s (OR per 5% increase 1.54, 95% CI 1.32-1.80), and forced vital capacity (OR per 5% increase 1.54, 95% CI 1.33-1.78) at transfer to the adult clinic and the exclusive use of oral antibiotics (OR 8.31,. Factors resulting in decreased probabilities of survival were Pseudomonas aeruginosa acquisition (OR 0.18, 95% 0.05-0.65) or pneumothorax before transfer to the adult clinic (OR 0.02, 95% CI 0.004-0.08) and referral from a paediatric clinic in a deprived area (OR 0.13, 95% CI 0.04-0.38).Long-term survival is associated with the clinical features present by the time of referral to an adult clinic. Even ''early-diagnosis'' disease appears to have different phenotypes, possibly independent of CF gene function, that have different survival patterns.

Report of the European Respiratory Society/European Cystic Fibrosis Society task force on the care of adults with cystic fibrosis

“…The metabolic consequences of being overweight or obese with CF are unclear; however, as adults live longer and potentiator and corrector therapy increases body mass index, problems with obesity may become an issue [19]. Hyperlipidaemia in the adult CF population has recently been recognised and may add to risk in the future [3,9]. Long term systemic inflammation may be associated with cardiovascular complications and early evidence of increased arterial stiffness has been reported in children with CF [20].…”

“…Long term systemic inflammation may be associated with cardiovascular complications and early evidence of increased arterial stiffness has been reported in children with CF [20]. The range and frequency of complications are likely to expand in the coming years [9].…”

“…The multi-organ challenges of CF also requires access to specialists from a wide range of disciplines including, for example, hepatology, nephrology, endocrinology, particularly diabetes and metabolic bone disease, and ear, nose and throat [3,9]. Experts in reproduction, genetic counselling and obstetrics and gynaecology are also needed to deal with infertility and the management of pregnancy in CF [8].…”

“…While this emphasis on prevention of disease progression and complications remains in adult care, the morbidity associated with recurrent pulmonary exacerbations often requiring hospitalisation, under-nutrition and the many systemic complications occurring in CF are more prominent. Therefore, there is a requirement to deliver expert and responsive care for adults with CF for all aspects of disease [3,9].…”

Prolonged Oxygen Kinetics During Constant Workload Submaximal Exercise Is Associated With Disease Severity in Adult Subjects With Cystic Fibrosis

“…3,4 The prognosis of the disease is worse in women, and it is dependent on nutritional status, respiratory involvement, colonization by Pseudomonas aeruginosa, and existence of complications such as pneumothorax and right ventricular failure. 5 Advances in the diagnosis and the therapeutic management of CF have gradually led to a significant increase in life expectancy, accompanied by a corresponding increase in the survival rate of CF patients. 6 Lung function parameters such as FEV 1 , 7 peak oxygen uptake (V O 2peak ) during incremental cardiopulmonary exercise testing (CPET) 8 and the clinical evaluation system known as the Schwachman score 9 are common prognostic indices used to assess functional capacity and disease severity in CF patients.…”