Growth failure in cystic fibrosis

Objective: The aim of this study was to describe and compare the nutritiona l status of children aged 0-18 years attending the cystic fibrosis (CF) cli nic at the Royal Children's Hospital, Brisbane, Australia, as outpatients i n 1986 and 1996. Methodology: The heights, weights and pulmonary function of children attend ing the CF clinic as outpatients in 1986 (n = 97) and 1996 (n = 227) were r etrospectively analysed using a computerized database maintained by the CF clinic. The heights and weights were analysed in terms of z scores for heig ht for age (HAZ), weight for age (WAZ) and weight for height (WHZ). Pulmona ry function data is not available for all children. Forced expiratory volum e in one second (FEV1), forced vital capacity (FVC) and forced mid expirato ry flows (FEF) were expressed as a percentage of predicted and are presente d here. Results: The 1986 sample consisted of 41 males (age range 0.18-14.59 years, mean age 6.52 (4.33)) and 56 females (age range 0.15-14.99 years, mean age 7.75 (3.70)). The 1996 sample consisted of 111 males (age range 0.09-17.97 years, mean age 8.80 (5.49)) and 114 females (age range 0.12-17.98 years, mean age 8.49 (5.26)). In 1986, males were shorter than females (P = 0.0096 ) and females had a lower mean FVC than males (P = 0.0438). In 1996, males were shorter. lighter and more wasted than females (P = 0.0357, P = 0.0034 and P = 0.0273, respectively) and females had a lower mean FEV1 and mean FV C than males (P = 0.0176 and P = 0.0079, respectively). Males in 1996 were lighter and more wasted than males in 1986 (P = 0.0023 and P = 0.0139, resp ectively) and had a lower mean FEV1, mean FVC and mean FEF (P < 0.0001, P = 0.0012 and P = 0.0069, respectively). Females in 1996 were shorter and lig hter than females in 1986 (P = 0.0273 and P = 0.0405, respectively) and had a lower mean FEV1, mean NC and mean FEF (P < 0.0001, P < 0.0001 and P < 0. 0001, respectively). When subjects were classified according to FEV1 (FEV1 greater than or equal to 75% or FEV1 < 75%), there were no significant diff erences in z scores between the 1986 group and 1996 group, Similarly, when the 1986 group were matched for gender and FEV1 with the 1996 group, there were no significant differences in z scores for males or females. Conclusions: It is suggested that the apparent worsening of nutritional sta tus among the 1996 group of CF patients is in fact due to an effect of incr eased survival of patients with more severe clinical symptoms. The findings from this study highlight the continuing, and in fact, worsening problem o f growth failure in children with CF.