Cystic Fibrosis: Classic Presentation (Part 2 of 2) From PedsCases

In this post, I link to and excerpt from PedsCases Cystic Fibrosis: Classic Presentation (Part 2 of 2), by Dr. Rose Sun, Oct. 27,2021.

All that follows is from the above resource.

This podcast is the second in a two-part series on cystic fibrosis. In this podcast, the listener will learn about the clinical features and complications of CF, the prognosis of CF, and an approach to prevent, investigate, and manage acute respiratory exacerbations. The podcast was created by Dr. Rose Sun, a second-year pediatrics resident at the Hospital for Sick Children in Toronto, with support from Drs. Tamizan Kherani and Melinda Solomon, pediatric respirologists at the Stollery Children’s Hospital in Edmonton and the Hospital for Sick Children respectively.

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And here is the summary from the script.

Review of Key Learning objectives:

In the second part of podcast we reviewed in detailed the complications of CF. The first being respiratory complications including: bacterial infections, allergic bronchopulmonary aspergillosis (ABPA), pneumothorax and nasal polyps. Then we
discussed the GI complications of pancreatic insufficiency, DIOS, neonatal cholestasis and CF related diabetes. Finally, the complications of the reproductive system leading to infertility. We also looked at the diagnosis and management of an acute pulmonary exacerbation through a case analysis. It is important to remember to start antibiotics early and to continue until eradication. If the infection is chronic, long term antibiotic
therapy may be needed. Pulmonary exacerbations are one of the  leading causes ofmorbidity and mortality in children with CF.

To end this series of podcasts examining CF in the pediatric population, I also want to emphasize the impact of a chronic disease on the day to day lives of patients and their families. For most patients with CF, many hours are spent each day on maintanence therapy including multiple sets of chest physiotherapy, taking inhaled and oral medications, and managing various lifestyle and dietary therapies. This is not only socially and emotionally exhausting on the patients, but also their family members. It is important for us as health care providers to provide family centred care and consider and involve all members of the family in our treatment plan.

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