Case Studies

Case 11: A 14-year-old girl with cystic fibrosis

A 14-year-old girl is seen for routine review in the cystic fibrosis clinic. She was diagnosed in the neonatal period and has been under regular follow-up since infancy. She has been well over the last 2 months with her only current respiratory symptom a persistent mild cough. She has good exercise tolerance and participates in sports at school. She is taking a large number of oral and nebulized medications but manages to adhere to the regimen and her physiotherapy.

• (a) What key features in clinic give a guide to her current health and prognosis?

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  • Growth and weight gain are closely linked to respiratory function.

  • At age 14 she should be progressing through puberty and this is also a sign of good control.

  • Lung function test (spirometry) results.

  • Sputum culture, particularly any more aggressive chronic respiratory infection organisms such as pseudomonas.

  • Chest radiograph changes.

  • Annual screening tests of vitamin levels, liver function, glucose tolerance test.

• (b) What issues are likely to arise over the next 5 years as she reaches adult age?

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  Transition to adulthood is a complex process of increasing independence from parents, self-reliance and change in lifestyle.

  The focus of the consultation moves from discussion with parents and child to more direct discussion with the young person. It is helpful for paediatric and adult clinical teams to work together to achieve gradual transition and continuity of care.

  Specific health issues: ongoing surveillance of respiratory function, respiratory infection, maintaining nutrition and weight. Young adult lifestyle advice relating to contraception and fertility, smoking, drug and alcohol use. Cystic fibrosis related diabetes and liver disease become more common with increasing age.