Lauren Haber CYSTIC FIBROSIS

 A life-threatening genetic disease, which affects the lungs, digestive system and reproductive system  Caused by a defective gene located on chromosome 7  Mutations of gene cause altered production of its respective protein product resulting in a thick, sticky mucus  Mucus clogs the lungs, pancreas and digestive pathways WHAT IS CYSTIC FIBROSIS?

 30,000 children and adults in the United States  70,000 children and adults worldwide  1,000 new cases diagnosed each year  Life expectancy is  Primary causes of mortality are due to lung disease and malnutrition WHO DOES IT AFFECT?

WHAT ARE THE SIGNS AND SYMPTOMS?

 Blood tests  Sweat test  Newborn screening HOW IS IT TESTED?

 There is not yet a cure for cystic fibrosis, but scientists have mad great strides over the years to find one  1989: The CF gene was discovered  Along with gene therapy many developments have been made with drug research  Clinical trials are key to ruling out what does not work and finding a cure. IS THERE A CURE?

 Though there is not yet a cure for Cystic Fibrosis, there are treatments to improve quality and length of life:  Airway Clearance  Medication  Lung transplant  Proper Diet  Exercise HOW IS IT TREATED?

 Improves airway clearness  Encourages deep breathing  Induces coughing  Improves overall quality of life WHAT ARE THE BENEFITS OF EXERCISE?

 Christian is a 22 year old Caucasian male diagnosed with CF (moderate severity) shortly after birth. He has been participating in hockey regularly for most of his life. He is 5’7” (170 cm) and 173lbs (86kg). He has a family history of CF. He is a non-smoker. Has a blood pressure of 102/60 mmHg and a fasting blood glucose of 90 mg/dL. His LDL levels are 105 mg/dL and HDL levels 40 mg/dL. CASE STUDY

FactorsRiskNo Risk Age ✓ Family history ✓ Smoking ✓ Sedentary Lifestyle ✓ Obesity ✓ Hypertension ✓ Dyslipidemia ✓ Diabetes ✓ RISK STRATIFICATION **Only one risk factor but because of pulmonary disease is considered high risk**

 Cardiovascular: Bike or treadmill test (Bruce Protocol)  Muscular strength and endurance: 1RM (grip strength) and push up test  Flexibility: Sit and reach and skinfold assessment  Body Composition: Weight, height and skinfold to assess BMI EXERCISE TESTING

AerobicAnaerobicFlexibility Frequency3-5 days per week 3-7 days per week Intensity70-80% VO2max 3 sets, 8-12 repetitions, 60-80% 1RM second holds to point of stretch without pain Time minutes per day minutes per day minutes per day Type Enjoyable activity: walking, running, sports (hockey) Hockey drills, weight training static and dynamic stretching EXERCISE PROGRAM

 Determine level of pulmonary disease  Wheezing or coughing may be indication of exercise induced bronchoconstriction  Malnutrition must be taken into account  Blood glucose levels must be monitored if CF related diabetes is present, in order to avoid hyper/hypoglycemia EXERCISE AND TESTING PRECAUTIONS

 Cystic Fibrosis does not yet have a cure, but scientists continue to conduct research and make strides in the right direction. Until a cure is found treatments, proper diet and exercise aide in giving people with Cystic Fibrosis a better quality of life. CONCLUSION

 American College of Sports Medicine. (2013). ACSM Guidelines for Exercise Testing and Prescription: Ninth edition. Lippincott Williams & Wilkins  Cystic Fibrosis (2015). American Lung Association. Retrieved March 14, 2015 from website: fibrosis/ fibrosis/  Cystic Fibrosis (May 2014). MedlinePlus. Retrieved March 14, 2015 from website:  Ehrman, J.K., Gordon, P. M., Visich, P.S., Keteyian, S.J. (2013). Clinical Exercise Physiology: 3 rd edition. United States: Human Kinetics, Inc.  Frequently Asked Questions (March 2014). Cystic Fibrosis Foundation. Retrieved March 14, 2015 from website:  Urquhart, D. S. (2011). Exercise testing in cystic fibrosis: why (and how)?. Journal Of The Royal Society Of Medicine (Supplement),104(1), S6-S14. doi: /jrsm.2011.s11102 WORKS CITED