Introducing The Players!

Transcription

1 PT and Cystic Fibrosis: A Successful Team from Birth to Healthy Aging! g Part I: Role of PT in the Disease of CF Introducing The Players! Anne Mejia Downs PT, MPH, CCS Rob Dekerlegand PT, MPT, CCS Anne Gould PT Matt Nippins PT, DPT, CCS Paul Ricard PT, DPT, CCS Anne Swisher PT, PhD, CCS Overview of CF and Medical Treatment Combined Sections Meeting

2 Cystic Fibrosis NOT just a lung disease NOT just a childhood disease Prevalence and Genetics Most common lethal inherited disease of Caucasians 1 in 3500 live births will have it 1 in 29 Caucasians will be a carrier Autosomal recessive transmission Genetic defect leads to absence or malfunction of a chloride receptor found in many cells throughout the body (CFTR cystic fibrosis transmembrane receptor) CF Inheritance Combined Sections Meeting

3 Genetics of CF Over 1000 mutations identified 3 F508 is the most common mutation 5 Codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein 3 CFTR gene I I F G ATCATCTTTGGT Types of CFTR problems CFTR Activity: Salt and Water Balance Inside the Airways Non-CF Cell CF Cell 6 Combined Sections Meeting

4 Mechanism of Lung Disease CF Expressions CF: Diagnosis Sweat chloride test Abnormal transporter makes Na & Cl accumulate outside cells Genetic sequencing from blood sample Newborn blood sample screening for protein (IRT) Diagnosis most common in early life, but can be at any point in life Combined Sections Meeting

5 Typical and Atypical Phenotypic Features of CF Chronic sinusitis Severe chronic infection Severe hepatobiliary disease Pancreatic insufficiency Meconium ileus at birth Elevated sweat chloride values Obstructive azoospermia Cystic Fibrosis Foundation. Clinical Practice Guidelines for Cystic Fibrosis Rosenstein BJ, et al. J Pediatr Knowles MR, et al. N Engl J Med CF Lung Disease Increased viscosity of secretions and poor cilia motility leads to retention Frequent respiratory infections Esp. pseudomonas aeruginosa, also MRSA Bronchiectasis results Fibrosis & hyperinflation result CT Scans of Normal and CF Lungs Normal CF Tiddens HA. Pediatr Pulmonol Combined Sections Meeting

6 CF Lung Disease Lung Function Is Not the Whole Story CF Digestive Disease Pancreatic insufficiency Lack of production + blocked ducts Meconium ileus CF related diabetes Autodigestion of pancreas from enzymes destroys Beta cells Patients become insulin deficient Osteoporosis Malabsorption of Vit D & calcium rich fatty foods Combined Sections Meeting

7 CF: Other systems Liver disease Cirrhosis can result Sinus disease Nasal polyps, chronic sinusitis can seed lungs Reproductive disorders Blockages/absence of seminal vesicles or cervix Joint complaints Osteoporosis, CF related arthritis, vertebral fractures, chronic chest wall pain Effect of Nutritional Status on Muscle Size CF: Medical Management Pancreatic enzyme replacement Amylase, lipase Intensive nutritional intervention (150% of normal caloric intake) Airway clearance Early treatment for pulmonary infections Inhaled/oral/IV antibiotics (Tobi) Pulmozyme (DNAse) for thinning secretions Exercise! Combined Sections Meeting

8 CF: Prognosis Median survival is 37 years Nearly half of all CF patients are over age 18 Oldest patient in registry 81 years old! Progressive decline due to respiratory problems Death due to respiratory failure & heart failure Lung transplant an option for few Prognosis for the Future Diagnosis as newborns Goal of NORMAL lung function and growth PREVENT infection and malnutrition Aim for NORMAL exercise capacity Anticipate NORMAL LIFESPAN for baby born today with appropriate care! CF: PT Management (CF 101 for the PT) Airway clearance techniques/teaching Postural intervention Exercise testing & prescription Prevention/management of stress urinary incontinence Prevention/management of musculoskeletal deformities Prevention/management of chronic pain Combined Sections Meeting

9 PT needs of CF Patients Lungs Chest wall & posture Pelvic floor Muscles Bones Adapt for ages 0 90 yrs Adapt for family, stage of illness, pregnancy, aging Overview of Airway Clearance Airway Clearance Techniques Anne Mejia Downs PT, MPH, CCS Combined Sections Meeting

10 Airway Clearance Background Goals Improved clearance of pulmonary secretions Decreased obstruction in the airways Improved ventilation and gas exchange Application Different techniques are effective for secretion clearance in the CF population; individual efficacy Rates of adherence with airway clearance are not optimal The most effective technique for each patient: The one they are willing to use on a regular basis Assessing ACT Needs Find out: Which ACT(s) does the patient /family have experience with and currently use? Health status and recent changes (less energy consuming techniques are appropriate for an exacerbation or declining health) Psychosocial information thatmay impact which techniques are appropriate or how they need to be adapted (home vs school/work or travel) Examination: Auscultation of lung segments Coughing assessment: inspiration, abdominal strength Sputum: amount, color, consistency Chart Review: PFTs and Chest X Rays Variety of Airway Clearance Techniques (ACT) for CF Postural Drainage and Percussion Active Cycle of Breathing Technique Autogenic Drainage Positive Expiratory Pressure High Frequency Chest Wall Oscillation Intrapulmonary Percussive Ventilation Exercise Combined Sections Meeting

11 Postural Drainage (PD) and Percussion Also conventional airway clearance or Chest PT How It Works: PD positions allow gravity and changes in lung ventilation to move secretions to central airways to be expectorated Percussion assists with loosening secretions from the airway walls How It Is Applied: Patient is positioned to drain the appropriate lung segment, modifying the position as needed Percussion is applied to the segment with cupped hands for 3 5 minutes per position, followed by shaking or vibration during exhalation The patient is encouraged to expectorate loosened secretions before moving to another position PD Positions Percussion & Postural Drainage Advantages Easy to learn to administer Passive takes little effort on the part of the patient Firsttechnique technique used with infants patients are familiar with it Hands on technique allows caregiver to assess pulmonary status Disadvantages Head down PD positions are contraindicated for infants, gastroesophageal reflux, and other conditions Requires a caregiver to perform Increases risk of repetitive motion injury in caregiver Passive patient can choose to disengage from process Combined Sections Meeting

12 Active Cycle of Breathing Technique (ACBT) How It Works: ACBT uses specific patterns of breathing repeated in phases to mobilize secretions How It Is Applied (the cycles include): Breathing control relaxed breathing using tidal volume Thoracic expansion with optional percussion/vibration deep inspiration with passive exhalation Forced expiratory technique (FET) or huffing forced exhalation with glottis open to move loosened secretions Cycle begins with breathing control, with thoracic expansion as needed, and ends with FET Cycles of ACBT Active Cycle of Breathing Technique Advantages Allows active participation of patient Can be easily incorporated dinto conventional P&PD Can be done independently Can be incorporated with nebulizer treatments and PEP devices Disadvantages Caregiver may be required for percussion, if performed during thoracic expansion More effort required than with conventional P& PD Combined Sections Meeting

13 Autogenic Drainage (AD) How It Works: AD uses expiratory airflow to mobilize pulmonary secretions How It Is Applied: Patient sits upright in a quiet room to hear the secretions when exhaling through the mouth with the glottis open AD consists of 3 phases: 1) unsticking the secretions from the peripheral airways 2) collecting the secretions in the larger central airways 3) evacuating the secretions from the airways Autogenic Drainage Autogenic Drainage Advantages May be performed independently The upright position is used (instead of PD positions) Requires no additional equipment Disadvantages Difficult to learn requires much patience and practice Few health care professionals in the US are familiar with AD Requires much effort not suitable for exacerbation or advanced disease Combined Sections Meeting

14 Positive Expiratory Pressure (PEP) How it Works: Creates pressure (10 20 cm H 2 O pressure) to keep the airways open during slightly active exhalation Allows secretions to move without the airways collapsing OscillatoryPEP devices provide concomitant oscillation of the airways How It is Applied: Usually performed in the upright position, but may be used in PD positions Many devices are available, but the most common are the Flutter, TheraPEP, and Acapella Positive Expiratory Pressure Positive Expiratory Pressure Advantages Can be performed independently Technique is easy to learn Devices are inexpensive and portable Can be done concurrently with nebulizer treatments Disadvantages May be contraindicated for patients with sinus problems or ear infections Must be cleaned between treatments Combined Sections Meeting

15 High Frequency Chest Wall Oscillation (HFCWO) How It Works: Air pulse generator supplies a variety of frequencies It increases expiratory airflow to mobilize secretions from the periphery to the central airways to be expectorated It also decreases the viscosity of secretions, making them easier to mobilize How It Is Applied: Patient wears an inflatable vest and sets the desired frequencies 3 different HFCWO systems: The Vest Airway Clearance System, the MedPulse Respiratory Vest System, and the incourage System High Frequency Chest Wall Oscillation High Frequency Chest Wall Oscillation Advantages Can be used independently in upright position Available for younger ages (2 years) and adults Easy to use and maintain Disadvantages Extremely expensive Heavy and not very portable Combined Sections Meeting

16 Intrapulmonary Percussive Ventilation How It Works: Delivers internal intrathoracic percussion and aerosolized solution for bronchodilation simultaneously cm H 20 pressure is generated and mini bursts are delivered into the lungs at rates of cycles per minute How It Is Applied: Internal percussion is delivered through a mouthpiece The device is available for use in the hospital or for home use Intrapulmonary Percussive Ventilation Intrapulmonary Percussive Ventilation Advantages Can be used independently at home Simultaneous use of bronchodilator medication Disadvantages Can be uncomfortable or not well tolerated May not be reimbursed by insurance carrier Combined Sections Meeting

17 Exercise One of the many benefits of exercise is its effect on mobilization of secretions However, current recommendations promote exercise as a supplement to other forms of airway clearance in CF management A variety of exercise programs can be tolerated by patients with CF Oxygen saturation should be monitored Exercise Exercise Advantages Normalizes patients with CF exercise is also performed by people without CF Increased fitness = increased survival Can be tailored to particular patient Disadvantages Recommended to be used with another ACT Not appropriate initially during acute exacerbation Combined Sections Meeting

18 Factors Affecting Selection of ACT Motivation Goals: Patient /family and Health professional /caregiver Effectiveness of the technique being considered Patient s ability to learn and skill of therapist teaching the technique Matching the effort required with the disease severity Need for assistance or equipment Costs Reassess ACTs regularly, including each hospital admission, to ascertain the effectiveness of a given method for the patient s age, clinical status, and preferences During clinic appointments, have the patient demonstrate the technique to gain insight into the need for modifications or a change in method Ventilatory Muscle Issues Ventilatory Muscle Function Strength Ventilatory Muscles Endurance Work Capacity Tension Time Index Netter, F.H. (2003). Atlas of Human Anatomy, 3 rd ed. Icon Learning Systems, Teterboro, NJ Combined Sections Meeting

19 Theoretical Effect of Excess Inspiratory Muscle Work on Exercise Ventilatory Muscle Work Breath Perception Alterations in breathing pattern Alterations in perfusion Dyspnea Gas Exchange Peripheral Fatigue Exercise Intolerance Figure: Parameters of the tension time index in response to graded exercise in children with CF (n=8) compared to healthy controls. *From Keochkerian et al., 2005, p Potential effects of CF on the Ventilatory Muscles Genetics Hyperinflation Nutritional Status Corticosteroids Disease Severity Acute Exacerbations Aging Combined Sections Meeting

20 Ventilatory Muscle Issues in CF??? Strength Endurance Work Capacity Tension Time Index Inspiratory Muscle Training (IMT) in CF Author IMF PFT Dyspnea Exercise QOL Asher (1982) NT NT Sawyer (1993) NT NT DeJong (2001) Enright (2004) NT *IMF: inspiratory muscle function, PFT: pulmonary function tests, QOL: quality of life Home Based IMT in Adults with CF Combined Sections Meeting

21 Home Based IMT in Adults with CF Age FEV 1 (%pred) BMI 6MWD (ft) MIP (cmh 2 O) MIP (%pred) A B C D E Mean: Identifying Potential Candidates for IMT 1. Inspiratory muscle weakness (MIP< 60 cmh 2 O) and/or ventilatory limits to exercise. 2. Moderate to severe pulmonary disease (FEV 1 <70% predicted) 3. Significant hyperinflation (RV/TLC >50%) 4. Nutritional depletion (BMI< 20, IBW <90%, LBM/IBW <70%) 5. Significant complaints of dyspnea 6. Inability to exercise in the traditional manner Inspiratory Muscle Training (IMT) Mode Threshold vs. Flow Intensity At least 30% MIP Frequency 3 7x/week Duration 30 min/session Combined Sections Meeting

22 BREAK 5 min Importance of Exercise for People with CF Why is Physical Activity Important for CF? Physical reasons Aerobic capacity Airway clearance Muscle strength Weight issues Anti inflammatory effects Posture BMD Others? Psychological reasons Self esteem Socialization Avoids the sick role Body image Combats depression Combats fatigue Others? Combined Sections Meeting

23 Why Are People (without CF) Physically Active? Physical reasons Disease prevention Weight management Increase strength Increase fitness Psychological reasons Body image Stress management Socialization Why Are People With CF NOT Physically Active? Time Expense Effort/discomfort Insecurity Inability Body image Lack of access to facilities Disease factors Dyspnea Need for supplemental oxygen CFRD Joint issues Exclusion from participation by others Stress urinary incontinence Fear? Exercise Predicts Status VO 2 max is more strongly related to lifespan, quality of life and functional capacity than FEV 1 Annual exercise test should be done Annual exercise test should be done All patients should view exercise as treatment of CF (not just a nice thing to do) Exercise is Medicine Combined Sections Meeting

24 Factors that May Affect Peripheral Muscle in CF Genetic mutations Mutations associated with more severe disease had lower peak aerobic capacity Selvadurai et al 2002 Malabsorption Decreased lean body mass (LBM) Altered function of muscle? (CFTR found in skeletal muscle) CF Related Diabetes (CFRD) effects? Aerobic Training Effects in CF Adults with mild to moderate lung disease who participated in aerobic exercise maintained peak oxygen consumption even while pulmonary function declined Moorecroft et al 1997 Aerobic Training Effects in CF 1 year home biking program in children improved leg muscle strength, VO 2 /kg LBM (Gulmans et al 1999) 3 year home aerobic exercise program slowed decline in lung function compared to nonexercising subjects with CF (Schneiderman Walker et al 2000) Combined Sections Meeting

25 Anaerobic Training in CF Children with mild lung disease, randomized to training or control group Training: second duration activities for 45 min twice weekly for 12 weeks Training i improved anaerobic performance (peak power, mean power), VO 2 peak, peak workload and Quality of Life Control group declined in VO 2 peak Klijn et al 2004 Resistance Training in CF Large trial (n=67) of resistance vs. aerobic training in children with CF Both groups exercised 3 times weekly for 1 year Stepping machine vs. upper body resistance training Both groups improved peak work and strength Orenstein et al 2004 Bone Health & CF Poor bone accrual Poor bone maintenance Risk for fractures Can have serious functional consequences on posture, airway clearance ability, lung transplantation access Combined Sections Meeting

26 Double Lung Transplantation 50 70% of patients with CF have osteoporosis (Hind et al J CF 2008), which may preclude this treatment. Photos courtesy of Woo MS. Balance of Factors for Bone Density Mechanical stresses Inflammation Inactivity Deposition Resorption The Good News About Exercise and Inflammation Combined Sections Meeting

27 Bone Mineral Density in Cystic Fibrosis: Benefit of Exercise Capacity* 25 patients, aged underwent BMD assessment and maximal exercise test VO 2 strongest independent predictor of BMD (R 2 = 0.86) Suggests that exercise is important determinant of BMD in proximal femur, less so in lumbar spine sites *Dodd, Barry, Cawood, McKenna and Gallagher J Clinical Densitometry 11(4): , 2008 Bone Health, Daily Physical Activity and Exercise Tolerance in Cystic Fibrosis Patients* 50 patients with CF older than 16 years Wore armband physical activity monitor Positive correlation of daily physical activity with: Lumbar spine (r = 0.36) Femoral neck (r = 0.51) Total hip (r = 0.54) *Garcia, Giraldez Sanchez, Ramos et al. Chest 140(2): , Evidence that gaining BMD is possible in children and adolescents Hind & Burrows review (Bone 2006) of 22 trials Activities included jumping, games, dance, and resistance exercises Meanincreasesafter after 6 months: % pre pubertal % early pubertal % pubertal age NOTE: delayed puberty in CF opens window for training longer Combined Sections Meeting

28 Frozen Soda Can Theory: Mary Massery PT, DPT, DSc Thoracic kyphosis present in up to 77% of girls and 36% of boys (Hind et al JCF 2008) Think about effect of fracture/pain on airway clearance ability. Spinal Fracture Symptoms Loss of height need to keep measuring adults! Back pain May be sudden onset with cough/sneeze OR Chronic pain in thoracic area due to increased work of thoracic muscles and altered posture Low back pain increased with exaggerated lordosis, relieved with flexion Back or rib pain, esp. with cough Role of Exercise Bone building: Impact stimulates ground reaction forces Mechanical strain on tendon attachment sites Prevent tfracture: Avoid high risk activities? Suppress inflammation Do something, there may be a risk. Don t do anything, we know it will get worse! Combined Sections Meeting

29 Pelvic Floor Muscle Issue Chronic cough increases intra abdominal pressure If pelvic floor cannot hold, leakage happens Documented to be very common in women and present in males as well (Brenda Button & colleagues) Impacts quality of life and participation Pelvic Floor Muscle Training Issues Posture affects muscle function Consider posture during airway clearance Airway clearance can lead to high pressure coughing and cough attacks All patients should be asked Typical response No!...but well.er.sometimes... All patients should be taught basics Empty bladder before ACT Good posture when coughing Controlled coughing/huff technique the knack pre emptive pelvic floor muscle contraction How to Get Involved in Your Local CF Center Combined Sections Meeting

30 CF Care Centers Definition Specialized centers supported by the CF Foundation to provide multidisciplinary care for patients with CF Locations o 110 centers nationwide (260 clinics) (CFF.org for map) Staffing Physicians, nurses, social workers, dietitians, physical therapists, respiratory therapists, psychologists, pharmacists, researchers Quality Improvement Example: Learning & Leadership Collaborative CF Foundation sponsored initiative Goal is to have care centers develop ideas for quality improvement and measure them at their own centers Guided by coaches and structured processes of quality improvement Brigham & Women's Hospital and Children s Hospital of Boston Example Combined Sections Meeting

31 Critical Milestones Exercise is chosen as a quality improvement project for the Dartmouth Institute's Adult QI (AQI2) Project (May 2011) Exercise testing and prescription guidelines developed (June 2011) BWH/CHB staff present project at first AQI2 group meeting in Baltimore, MD (July 2011) BWH/CHB staff present project to at second AQI2 group meeting in Anaheim, CA (Oct 2011) BWH PT CF staff are given in service for GXT and exercise prescription (November 2011) BWH Standard of Care for Patients with CF is updated (February 2012) Steven Verticcio Memorial Fund donation allowed purchase of a treadmill for CHB clinic (February 2012) and BWH inpatient (April 2012) Patient is admitted to BWH & PT is consulted PT consult is ranked to be seen in 24hrs PT examination completed (see BWH SOC) GXT results & individualized HEP are ed to CHB Clinic Team GXT results & individualized HEP are saved on the J-drive New Patients: Appropriate GXT completed on last treatment session (see J-drive for protocols An individualized HEP (aerobic & interval) is developed & given in paper form to the patient For Readmissions: Same GXT as used in past is completed (see J-drive for past results & prescriptions BWH/BCH Incremental Treadmill Test Combined Sections Meeting

32 Home Exercise Template Aerobic Home Exercise Program Warm up by progressively increasing the speed on the equipment or walking faster for 5 10 minutes. RPE should be 0.5 to 1/10 or 1.5 to 2METS if using equipment with this data. Increase your walking speed until you feel like you are working at a RPE of 3 4/10 or stay within the range of (insert 40 60% of peak METS achieved here) METS if using a treadmill or other equipment. Continue walking at this speed for minutes Cool down by progressively slowing your walking or equipment speed for 5 10 minutes. RPE should slowly decrease to 0.5 to 1/10 Home Exercise Template Interval Program Warm up by progressively increasing the speed on the equipment or walking faster for 5 10 minutes. RPE should be 0.5 to 1/10 or 1.5 to 2METS if using equipment with this data. Intervals: Ideally recovery intervals are twice or three times as long as the work intervals. You may need to decrease both speed and intensity during recovery phases Work phase: At least (insert 60% of peak METs here) METs or RPE 6 7/10 (30 seconds to 2 minutes) Recovery phase: 1.5 to 3 METS or RPE 2 3/10 (2 3 minutes) Repeat intervals for minutes Cool down by progressively slowing your walking or equipment speed for 5 10 minutes. RPE should slowly decrease to 0.5 to 1/10 Data From Poster Presentation Combined Sections Meeting

33 Other Ways/Settings Outpatient CF clinic School system Home health Outpatient PT clinic Specialty clinics (e.g. women s health) End of Part I (break time!) Combined Sections Meeting