Chronic Disease: Cystic Fibrosis Pathophysiology - Essay Example

Introduction Cystic fibrosis pathophysiology Cystic fibrosis is a genetical disorder caused by an inherited trait of autosomal recessive trait known as Cystic Fibrosis Trans-Membrane Regulator (CFTR); affecting body system among the elderly and children. It is characterised by mal-digestion, airways infection and obstruction. CFTR affects mucous viscosity and raises salt secretions Lapin (2002). Mostly affected organ is the intestines and the lungs. CF patients have life expectancy of up to 35 years. However, research and treatment has shown to prolong life with many victims facing their adulthood with more than 35 years Redi, Blizzard, Shugg, Flowers, Cash & Greville (2011). Anthony is 28 years, used to smoke and has osteoporosis. Anthony will be put on a training program not to replace chest physiotherapy, but to reduce the rate at which Anthony will attend the conventional physiotherapy treatment Prasad (2000). Program training Literature Review and Discussion Aerobics Aerobics require unusual extra amount of oxygen to impact on the heart and lung muscles. Aerobics were thought vital in his program so as to reduce risk and progression of heart diseases hence lower mortality rate and improve heart function in Anthony Prasad (2000). However, these exercises consume a lot of energy but the benefits to Anthony supersede the limitation. A study carried by Moorcroft, Dodd & Webb, 1998 showed that sufficient intensity of 2 to 3 times a week is recommended for 30 minutes on aerobic exercises; cool-down of 5 minutes and 5 minutes warm up hence, Anthony’s program was thought helpful to follow the findings. The program will incorporate: treadmill, cycling and running for Anthony’s physical fitness. In addition, Zach, Purrer, & Oberwaldner, (1981) swimming was incorporated because in addition to working the back and the upper body parts it helps in improving ventilation function of the heart and clear mucus within the lungs. However, CF leads to low immunity and hence clean swimming pools were advocated for Anthony. Short-term physiological alteration of pulmonary function on aerobic Celli (1995) Bronchoconstriction is a rare happening among CF victims although may arise following exercises. Significantly, exercise lowers sodium production within the respiratory epithelium of the CF by blocking amiloride sensitive channels of sodium. This effect was found to also impact on sputum viscosity eliminating or reducing airway blockage. Exercise induced bronchospasm may arise. A study conducted shows that 55% of CF victim develop this effect on exercising. The effect is quite unwelcoming since it leads to obstruction of the transient air flow. Nevertheless, although expected it only takes about 5-15 minutes and hence proper preemptive is emphasized. Long-term physiological alteration of pulmonary function on aerobic Moorcroft, Dodd & Webb, 1998 Exercise does not really reduce lung progression in CF, but it is still possible to maintain peak aerobic exercises. However, Redi, et al., (2011) studies have found that there is a correlation between peak VO2 and mortality. Therefore, aerobic exercises that will ensure Anthony achieve VO2 peak level are vital to prolong life. In addition, CF victim who undergo exercise have shown to significantly reduce rate of dyspnoea. Cardiovascular response Indirectly, Lapin (2002) CF lowers the oxygen intake impacting on the ventilation; the hearts function. Hyperinflation in CF victims affects the right ventricles, therefore, lowering stroke volume. Anthony who is a victim, program implementation alteration will be considered to ensure decreased heart rate, increase in stroke volume and increase in maximal cardiac output. Resistance training O’shea, Taylor & Paratz, 2004 found that for patients whose peripheral muscles are weak and have dyspnoea which is pronounced like Anthony the best intervention is resistance training. Although RT indirectly affects lung function, in case of Anthony, weight training is recommended. This is because due to deconditioning there is evident need to strengthen peripheral muscles, improve on myosin heavy chain protein, cytoplasmic density, myofibular volume and within the fibre size (Baechle & Earle 2008). In addition, improved muscle mass among the elderly population benefit from increased self-esteem, quick recovery and improved mobility. Frequency of Resistance training Anthony is supposed to undertake inspiratory muscle training as well as complete exercise on chest physiotherapy. Therefore, Yankaskas, Marshall , & Sufian (2004) in regard to RT, muscle group should be trained alternatively; with each group trained at most twice weekly. Anthony will be involved on 2-4 sessions in circuit muscle training (larger-smaller muscles) as it is beneficial than aerobics alone. Osteoporosis Resistance training is vital in Anthony to increase bone tone and strength (Hecker & Aris, 2004) Inspiratory muscle training A study conducted by Brooks, O’brien, Geddes, Crowe & Reid. (2008) on 11 CF adult patients which involved IR 15 min *2*4weeks showed that inspiratory muscle strength and endurance increased as well as improving lung function. Another study showed that; the level of dyspnoea decreases with IMT exercises Lapin (2002). Therefore, in addition to aerobics and strength training IMT was thought important in Anthony’s training program due to its benefits. Medication Bronchodilators Recommended for Anthony to inhale before CPT and exercise relax muscle surrounding the airways and enhance opening up and clearing the mucus Hodson (2000). Acetylene and saline will be used to aid in reducing mucus stickiness in the airways. In addition, saline used to wash nasal passage hence clearing the sinuses. Digestive system Vitamin A, D, E, and K will be supplemented on Anthony to aid in replacing fat soluble vitamins that are not absorbable by the CF victim Hodson (2000). Osteoporosis Vitamin D, K and calcium In addition to micronutrient supplementation, vitamin D, K and calcium will help replenish bone tone which has been affected by osteoporosis Hecker, & Aris, (2004); Sambrook & Eisman, (2000). Although Crimmins (2004), prolonged Vitamin D supplement causes other condition such as diabetes, cataract, and hypertension and weakens bones. Pancreas function Pancreatic enzyme replacement capsule will be given to Anthony to lower pancreas enzyme insufficiency effects caused by CF Hodson (2000). Goals Short term goals on Anthony General infection reduction Decrease dyspnea daily Improve self esteem Improve nutritional status Improved altitude towards exercise Decrease production of sputum within the lungs Long term goals on Anthony Strengthen lung muscles and general cardiovascular muscle structures Prasad (2000) improve and strengthen bone tone Decrease rate of admission following deconditioning Prolong life Improve quality of life Conclusion CF is an inheritable condition basically affecting children from birth. Rarely do people see their adulthood Redi, et al., (2011). However, Hodson (2000) with medication, resistance training, aerobics and breathing exercise quality of life for CF victims has improved and life expectancy increased. Combination of these exercise offer CF patients long and short term health benefit. Aerobic is more beneficial as compared to other exercise this is portrayed by its ability to lower the sputum viscosity. Bronchodilators and acetylene agents and saline promote removal of excess sputum. Research on CF treatment and management is still underway with more sophisticated approaches being invented to curb the challenges accompanying CF sufferers. References Baechle, T., & Earle, R.. (2010). Essentials of Strength training and Condition. National strength and conditioning association. Brooks D., O’brien K. Crowe J. Geddes E., & Reid D. (2008). Effects of inspiratory muscle training in cystic fibrosis: a systematic review. Clin Rehabil, 22 10-11, 1003-1013 Celli B.R. (1995). pulmonary rehabilitation in patients with cystic fibrosis. Am J Respir crit care med 152: 861-864 Crimmins E (2004). Trends in the health of elderly. Annu Rev Public Health 25:79–98 Hecker, T.,& Aris, R. (2004). Management of Osteoporosis in Adults with Cystic Fibrosis. Division of Pulmonary and Critical care medicine, 64(2), 133-147. Hodson M.E. (2000). Treatment of cystic fibrosis in the adult. Respiration 67:595-607 Kuys, S., Hall, K., Peasey, M., Wood, M., Cobb, R. & Bell, S. (2011). Gaming console exercise and cycle or treadmill exercise provide similar cardiovascular demand in adults with cystic fibrosis: a randomised cross-over trial. Journal of physiotherapy, 57, 35-40. Lapin C.D. (2002). Airway physiology, autogenic drainage and active cycle of breathing. Respiratory care. 47:778-785 Moorcroft, A., Dodd, M., Webb, A. (1998). Exercise limitations and training for Patients with cystic fibrosis. Disability and Rehabilitation, 20(6/7), 247- 253. O’Shea S.D., Taylor N.F.& Paratz J. (2004). Peripheral muscle strength training in COPD: A Systematic Review. Chest, 126(3), 903-914 Prasad A. (2000). Physiotherapy in cystic fibriosis. J R Soc Med 93:27-36 Redi D.W., Blizzard C.L., Shugg D.M., Flowers C., Cash C. & Greville H.M. (2011). Changes in cystic fibrosis mortality in Australia, 1979-2005. Medicinal Journal Australia 195:392-395 Sambrook P.N. & Eisman J.A (2000). Osteoarthritis prevention and treatment. Med J Aust; 172:226-229 Yankaskas J.R., Marshall B.C.& Sufian B. (2004). Cystic fibrosis adult care: consensus conference report. Chest 125:1-39 Zach, M, S., Purrer, B. & Oberwaldner, B. (1981). Effect of swimming on forced expiration and sputum clearance in cystic fibrosis. The Lancet, 318 (8257), 1210 – 1203. Client Details Name: Anthony zdrilic Age: 28 years old Gender: Male Assessment Advice planned actions Resources History Smoking Yes (stopped 5 years ago) Quitline: 131 848 Diet/nutrition High Fibre, Fruit and Vegetables Consume balanced diet Heartline: 1300 36 27 87 Alcohol No Directline: 1800 888 236 A 24 hour telephone counselling, information and referral services for drug and alcohol related issues Physical Activity Light Physical Activity 3 times a week Need to perform planned exercise routine with a higher intensity. Heartline: 1300 36 27 87 Go For Your Life info Line: 1300 73 98 99 Depression No Osteoporosis No Examination Bodyweight/BMI weight 71kg, height 172cm, Waist circumference = 86 cm BMI 24 Maintain weight Blood pressure 127/85 Skin cancer None Tests Lipids LDL = 2.9 mmol/L HDL = 1.0 mmol/L Total = 3.9 mmol/L Diabetes Fasting Glucose = 4.1 mmol Cervical Cancer N/A Prostate ND Other as appropriate (e.g. colorectal, cancer, breast cancer) None Medical History For Cystic Fibrosis: Bronchodilators- an enhance opening up and clearing the mucus before exercise. Acetylene and saline will be used to aid in reducing mucus stickiness in the airways and open sinuses Fat soluble vitamin supplementation to replenish body stores Vitamin D and K calcium- prevent or slow osteoporosis Family History Social History Mother: healthy Smoking: No Father: Died at 39 years of age due to Cystic Fibrosis Smoking: No General Recommendations (if appropriate): following Anthony’s CF, condition and is recommended that he performs an exercise program under a professional scientist to improve quality of life, reduce multimorbidity and increase life expectancy. Summary of 4 week Training Program – in order from left to right Initial phase Central phase Final phase Inspiratory muscle training Warm Up (5 mins) Resistance Training (10 mins) Aerobic Training (30 mins) Cooldown (5mins) Inspiratory muscle training Nintendo Wii Sunday Rest day Monday A-C* Wk. 2, 400m walk Wk4, 400m run Wk. 1,3 Star jumps Refer to 'resistance training program' section Treadmill Light jog All exercise Wii Sports Tuesday A-C* Wk. 2, 400m walk Wk4, 400m run Wk. 1,3,: Star jumps No training Swimming Light jog All exercises Wii Sports Wednesday D-G Wk. 2, 400m walk Wk4, 400m run Wk. 1,3 Star jumps Refer to 'resistance training program' section Cycling Light jog A-C* Wii Sports Thursday Rest day Friday All exercises Wk. 2, 400m walk Wk4, 400m run Wk. 1,3 Star jumps Refer to 'resistance training program' section Outdoor Run Light jog D-G Wii Sports Saturday A-C* Wk. 2, 400m walk Wk4, 400m run Wk. 1,3 Star jumps No training skipping Light jog All Exercises Wii Sports Warm Up Weeks 1, 3 Star Jumps for 5 minutes/ motivational music at the background. Week 2 400m walk at own pace Week 4 400m run at own pace Cool down for all weeks 5 minute light jog Inspiratory Muscle Training Exercises A: Stretching the muscles of the chest wall 1. Starting Position: Stand up, hands by your sides, palms facing inwards 2. Rotate palms facing forwards 3. Extend arms, and bring arms above the head so hands touch 4. inhale through the nose when rising the hands 5. turn palms outwards, and exhale through mouth as arms move down to starting position 6. Repeat 10 times B: Pursed-Lip Breathing 1. The exercise is performed when lying down with bent knee or sitting on a firm chair. 2. Place one hand on your abdomen and the other on your chest. The hand on the abdomen should be seen rising when breathing and that on the chest remain still. 3. Inhale for 2 seconds through the nose and breathe out for 4 seconds through the mouth as though blowing a candle. 4. Repeat this for 10 times. C: Sniffles (good workout for diaphragm) 1. With mouth closed 2. Breath in and out through nose as quickly as possible 3. Repeat for 10 seconds D: Elbow Circles 1. place hands on shoulders, with elbow pointing laterally, fingertips touching shoulders 2. Rotate elbows in backwards circular motion, keeping the fingers on the shoulders 3. Inhale as elbows are brought up 4. Exhale as elbows are brought down E: Shoulder Shrug 1. Place arms by your side, palms facing forwards 2. Inhale, as shoulders are lifted in front of body, moving towards ears 3. Exhale as shoulders roll back down, towards the back of the body 4. Repeat 10 times, and then do 10 repetitions in opposite direction. F: Bicep curls 1. Using dumbbells sit on a chair which is firm. 2. Slowly lift up the weights with elbows facing your body to above your shoulder as you inhale through the nose and exhale through the mouth. 3. Repeat breathing as you bring down the weights. Remember to breathe in as you relax and out when applying effort. 4. Do this 10 times G: Shoulder press 1. Hold the weights still seated on your shoulder. 2. Take a deep breathe and lift the weight above your head and breathe out through the mouth. 3. Repeat 10 times daily. Resistance training Training for Strength Recommendations Load: ≥ 85% Sets: 2-6 Reps: ≤ 6 Rest: 3-5mins Perform a 1RM Submax test RM Testing 1. Select a warm-up load the subject can perform easily for ~8 repetitions without failure Relax for 2-3 minutes 1 Increase the load and let the client perform ~5 repetitions without fail Relax for 2-3 minutes Increase the load and let the client perform ~3 repetitions without fail Relax for 2-3 minutes Repeat with small increments until you are certain that it is a maximal performance. 1. Estimate the subjects 1RM based on the table below Weeks 1-2 Exercise Muscles Load (% 1RM) Sets Reps Rest (mins) Week 1 2 1 2 1 2 Monday Dumbbell Standing Calf Raise Gastrocnemius 85 2 3 4 5 5 5 Dumbbell Reverse Calf Raise Tibialis Anterior 85 2 4 3 5 4 5 Wednesday Lunges Quadriceps 2 3 4 3 5 5 Dumbbell Straight Leg Deadlift Hamstrings 85 2 4 3 4 5 5 Friday Chin ups Biceps 2 3 4 4 5 5 Pull ups Triceps 2 4 3 4 5 5 Weeks 3-4 Exercise Muscles Load (% 1RM) Sets Reps Rest (mins) Week 1 2 3 4 3 4 Monday Inverted Row Back muscles 85 2 3 4 4 5 5 Barbell Shrug Upper Trapzius 85 2 4 3 4 3 5 Wednesday Bench Press Pectoralis Major 85 2 3 4 4 5 5 Sumo deadlift Gluteus Maximus 85 2 4 3 4 5 5 Friday Crunches Rectus Abdominis 2 3 3 4 5 5 Dumbbell One Arm Straight Leg Deadlift Erector Spinea 2 3 3 4 5 5 Aerobic Training For effective heart benefit to Anthony exercise should be increased to 60%-85% heart rate. Maximum heart rate can be estimated using (maximum oxygen use i.e. 220minus age *heart rate of about 60-85%) Table below show Usual Maximum and Target Heart Rates Age (years) Maximum Heart Rate (beats per minute) Target Heart Rate (beats per minute) 20 200 120‒170 30 190 114‒162 40 180 108‒153 50 170 102‒145 60 160 96‒136 70 150 90‒128 However, target heart rate is usually estimated and deconditioned people like Anthony are likely to reach their target heart rate fast or may not even reach the heart rate target. In addition, incase of breathlessness in Anthony he should not strain further. Monday: (30 mins). Week Type Location Intensity 1,2,3& 4 Treadmill Gym 50% - 60% HRmax Tuesday: Swimming (30 mins) Week Distance Rest (s) Stroke 1 200m 60 Freestyle swim. 2 250m 60 Butterfly. 3 300m 60 backstroke 4 350m 60 any stroke you want Wednesday: Cycling (30 mins) Week Type Location Intensity 1 Outdoor Bike Riding local park 50% - 60% HRmax 2 Indoor Bike Gym 50% - 60% HRmax 3 Outdoor Bike Riding Near shopping centre 50% - 60% HRmax 4 Indoor Bike Gym 50% - 60% HRmax Friday: Running (30 mins) week Type Venue Intensity 1 Outdoor Run shore of the beach 50% - 60% HRmax 2 Outdoor Run local park 50% - 60% HRmax 3 Outdoor Run To the near shopping centre 60% - 70% HRmax 4 Outdoor Run To the near college/ or high school 60% - 70% HRmax Saturday: Skipping (30 mins). 15 minutes each variation Week 1: Standard Skip Week 2: Standard + feet together Week 3: Running Jumps+ Feet apart Week 4: Double jumps + High knee jumps Nintendo Wii Play 'Wii: Sports' video game according to client’s wish Dr ……………. Accredited Exercise Physiologist (ESSA) PhD (ACU) Email: drad.aarons@medical-practice.com Phone: 0473 691325 Medicare provider number: 8275903 B Date: 26/03/2013 Appointment: 23/04/2013 Referring Doctor: ………………. Name: Anthony zdrilic DOB: (04/03/1985) Age 28 years Gender: M Dear Dr. ……………………., Thank you for referring Mr. Zdrilic to me for exercise prescription to help him improve quality of life, airway capacity and prolong life in relation cystic fibrosis disorder. MR. zdrilic smokes, is osteoporosis sufferer, father died at 39 years with CF, live on vegetarian diet, is on bronchodilator, acetylene and saline drugs and micronutrient supplementation: vitamin D, K and calcium. To him these drugs help him in exercise ability and improve bone health a fact supported by my research. Mr Zdrilic is active 3 times a week on light intensities, usually walking. At the date of 26/03/13 he is: Height- 172cm Weight- 71kg BMI- 24kg/m2 Waist circumference- 86cm Resting HR- 65bpm Resting BP- 127/85 Mr zdrilic VO2 peak after treadmill VO2 peak test is 34.2mL/kg/min and a maximum heart rate of 185 where test was stopped because of dyspnoea. Mr. zdrilic only reported mild breathlessness during the test which increased at the final stage. His body measurements lie within norm. Therefore, the program aims to improve quality of life. Physiology Intervention exercises aim to achieve the following: Improve inspiratory muscle strength as result of inspiratory muscle training Muscle mass increment via progressive resistance training Bone health improvement via progressive resistance training severity and infection frequency reduction via enhanced immune system lowered dyspnoea rate via aerobic training general quality of life improvement accrued from the whole intervention I have sufficiently offered appropriate guidelines for the exercise intervention more so regarding adequate hydration, especially in regards to salt intake for sweat replacement. The need to exercise in immediate access to oxygen supplementation and within gym has been explained to Mr. Zdrilic A follow up session has been made for the 23/04/2013 to address any issues arising from the training program on Mr. Zdrilic side. Exercise program consists of different varieties of moderate intensity aerobic training, high intensity resistance training and high intensity inspiration muscle training. Attached is Mr.Zdrilic training program copy for your review. In case of any question you’re welcomed to contact me. Signed …………………………. Dr …………………… Accredited Exercise Physiologist (ESSA) PhD (ACU) Email: drad.aarons@medical-practice.com Phone: 0473 691325 Medicare provider number: 8275903 B Date: 26/03/2013 Appointment: 23/04/2013 Referring Doctor: ………………. Name: Anthony zdrilic DOB: (04/03/1985) Age 28 years Gender: M Dear Dr. ……………………., Dear Mr Zdrilic, I appreciate for taking initiative and attending consultation and fitness level evaluation with me. The information aims and your exercise plan will be based on the measurements, gathered from you. This will help in your immune boosting, functional capacity and quality of life improvement. Below is your body composition versus the normal values. Body Composition Measurement Anthony zdrilic Healthy Range Height height 172cm n/a Weight weight 71kg, 55-73kg BMI 24kg/m2 18.5-25kg/m2 Waist Circumference 86cm Read More