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Cystic fibrosis pocket guide


of children who have CF allows for immediate initiation of preventative therapies. Although the financial and economic benefits of early diagnosis in CF are evident, the improvement in quality of life for patients with CF and their families resulting from early diagnosis should not be underestimated and are likely to form part of future economic analyses.


Therapeutic monitoring


Patients with CF may be exposed to certain classes of drug in high doses, for a long period of time. Monitoring serum levels of these drugs aims to both maximise their efficacy and minimise sub-therapeutic doses and the risk of adverse side effects. This is of particular importance in cases where the clinical response to the drug may be poor and toxicity is expected. Serum levels of tobramycin, amikacin and the conazoles should be routinely determined and adjusted accordingly.5


Adherence CF patients endure a complex


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regimen of treatment, involving exercise and physiotherapy, management of nutrition and administration of medications in a variety of formats (for example, oral, inhaled or IV), often requiring administration at various times throughout the day.7


Adherence to this time- consuming and demanding regimen is understandably low in people with CF, and poor adherence is linked to an increased likelihood of pulmonary exacerbation.13 With disease progression comes an increased therapeutic burden. However, with an ever-increasing drive towards preventative therapies in early-stage disease, even patients with mild disease, with perhaps few or no clinically obvious symptoms, are likely to experience a high therapeutic burden. In these cases, where there is no directly perceived benefit of therapy, adherence to treatment regimes can prove challenging. This becomes increasingly apparent as the patient moves from childhood into adolescence.14 Other barriers to adherence


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