ReviewThe impact of co-morbidity in childhood cystic fibrosis
Introduction
Lung disease remains the major cause of morbidity and mortality in Cystic Fibrosis (CF). Its progression can be extremely variable and it is clear that a number of factors, genetic and environmental, play an important role. It is a particular challenge to detect and monitor lung disease in children younger than 6 years of age. A number of risk factors have been identified for rapid decline in respiratory function in newborn screened individuals; including genotype, faltering growth, Pseudomonas aeruginosa colonisation, hospitalization and meconium ileus [1]. Whilst care is directed towards identifying and managing the known complications and risk factors of CF, treatment becomes more difficult when patients have other pathologies. Co-morbid disease can impact significantly on the course of CF; and can be particularly devastating in childhood when lung growth and development is of utmost importance, leading to rapid deterioration and end stage disease at an early age.
Section snippets
Case example
Such progression can be demonstrated by a case of a 6 year old boy with CF, complex heart defects and significant gastro-oesophageal reflux disease (GORD). He was born at 35 weeks gestation with minor respiratory distress at birth. Newborn screening was positive with genotype Phe508del/c.1393-2A>G confirmed with a sweat chloride of 80 mmol/L. He is pancreatic insufficient. At the time of his sweat test he was unwell with tachypnoea and respiratory distress, and therefore admitted for a course of
Discussion
It is difficult to estimate how much congenital heart lesions contribute to the progression of lung disease in CF. A large VSD will lead to left to right shunting, increased pulmonary blood flow and subsequently raised pulmonary vascular resistance [2]. With added inflammation, air trapping and resulting chronic hypoxia associated with repeated infection in CF; vascular remodelling may be accelerated with earlier progression to pulmonary hypertension [3]. In addition to direct effects on the
Conclusion
This review illustrates the challenging issues when faced with co-morbidity in CF and highlights the potential importance of identifying gastroesophageal reflux disease, and its subsequent close surveillance. How aggressively we treat this before and after lung transplantation remains to be clarified, and future studies will guide the optimisation of treatment to protect susceptible lungs from other damaging factors.
Conflict of interest
None declared.
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Cited by (2)
Pulmonary Manifestations of Gastrointestinal, Pancreatic, and Liver Diseases in Children
2021, Pediatric Clinics of North AmericaCitation Excerpt :With advanced lung disease, and after lung transplant, there is a higher incidence of GERD, which in adults is estimated to be around 90%.34 GERD has also been linked to earlier Pseudomonas aeruginosa and Staphylococcus aureus acquisition in young children, as well as poorer lung function.30 Long-term use of PPIs is associated with more frequent lower respiratory tract infections3,35,36 as well as a minimal increase in asthma exacerbations.3