PT and Cystic Fibrosis Part Icardiopt.org/csm2013/PT-and-Cystic-Fibrosis-Part-I-ID-1382196.pdf ·...

1/24/12

Combined Sections Meeting 2013 1

PT and Cystic Fibrosis: A Successful Team from Birth to Healthy Aging! f y g 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

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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

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Genetics of CF

• Over 1000 mutations identified3

• ∆F508 is the most common mutation5

• Codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein3

…I I F G……ATCATCTTTGGT…

——

CFTR gene

Types of CFTR problems

CFTR Activity: Salt and Water Balance Inside the Airways

Non-CF Cell CF Cell6

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Mechanism of Lung Disease

CF Expressions

CF: Diagnosis

• Sweat chloride test– Abnormal transporter makes Na & Cl accumulate outside cells

• Genetic sequencing from blood sampleq g p

• Newborn blood sample screening for protein (IRT)

• Diagnosis most common in early life, but can be at any point in life

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Typical and Atypical Phenotypic Features of CF

Chronic sinusitis

Severe chronic infection

Severe hepatobiliary disease

Cystic Fibrosis Foundation. Clinical Practice Guidelines for Cystic Fibrosis. 1997.Rosenstein BJ, et al. J Pediatr. 1998.

Knowles MR, et al. N Engl J Med. 2002.

Pancreatic insufficiency

Elevated sweat chloride values

Obstructive azoospermia

Severe hepatobiliary disease

Meconium ileus at birth

CF Lung Disease

• Increased viscosity of secretions and poor cilia motility leads to retention

• Frequent respiratory infections

E d i l MRSA– Esp. pseudomonas aeruginosa, also MRSA

– Bronchiectasis results

• Fibrosis & hyperinflation result

CT Scans of Normal and CF Lungs

Normal CF

Tiddens HA. Pediatr Pulmonol. 2002.http://www.rrcc-online.com/paprogram/HISTHTML/RADNORM/CHCT02.HTM.

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CF Lung Disease

Lung Function Is Not the Whole Story

CF Digestive Disease

• Pancreatic insufficiency

– Lack of production + blocked ducts

• Meconium ileus

• CF‐related diabetesCF related diabetes

– Autodigestion of pancreas from enzymes destroys Beta cells

– Patients become insulin deficient

• Osteoporosis

– Malabsorption of Vit D & calcium‐rich fatty foods

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CF: Other systems

• Liver disease

– Cirrhosis can result

• Sinus disease

– Nasal polyps, chronic sinusitis can seed lungsp yp , g

• 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!

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CF: Prognosis

• Median survival is 37 years

– Nearly half of all CF patients are over age 18

– Oldest patient in registry 81 years old!

P i d li d t i t• 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

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Chest wall & posture

Lungs

PT needs of CF Patients

Bones

Pelvic floor Muscles

Adapt for ages 0‐90 yrsAdapt for family, stage of illness, pregnancy, aging…

Overview of Airway ClearanceOverview of Airway Clearance

Airway Clearance Techniques

Anne Mejia‐Downs PT, MPH, CCS

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Airway Clearance—Background

Goals– Improved clearance of pulmonary secretions– Decreased obstruction in the airways– Improved ventilation and gas exchange

ApplicationApplication– 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 NeedsFind 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 that may impact which techniques– Psychosocial information that may impact which techniques are appropriate or how they need to be adapted (home vsschool/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 Drainageg g

• Positive Expiratory Pressure

• High Frequency Chest Wall Oscillation

• Intrapulmonary Percussive Ventilation

• Exercise

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Postural Drainage (PD) and PercussionAlso “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

H I I A li dHow 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

• First technique used with

Disadvantages• Head‐down PD positions are

contraindicated for infants, gastroesophageal reflux, and other conditions

• Requires a caregiver to First technique used with infants—patients are familiar with it

• Hands‐on technique allows caregiver to assess pulmonary status

q gperform

• Increases risk of repetitive motion injury in caregiver

• Passive—patient can choose to disengage from process

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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 i t d i t

Disadvantages• Caregiver may be

required for percussion, if performed during thoracic expansionincorporated into

conventional P&PD

• Can be done independently

• Can be incorporated with nebulizer treatments and PEP devices

thoracic expansion

• More effort required than with conventional P& PD

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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– 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

Disadvantages

• Difficult to learn—requires much patience and practice

• Few health careused (instead of PD positions)

• Requires no additional equipment

Few health care professionals in the US are familiar with AD

• Requires much effort—not suitable for exacerbation or advanced disease

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Positive Expiratory Pressure (PEP)

• How it Works:– Creates pressure (10‐20 cm H2O pressure) to keep the airways open during slightly active exhalation

– Allows secretions to move without the airways collapsingOscillatory PEP devices provide concomitant– Oscillatory PEP 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 l

Disadvantages

• May be contraindicated for patients with sinus problems or ear infectionslearn

• Devices are inexpensive and portable

• Can be done concurrently with nebulizer treatments

infections

• Must be cleaned between treatments

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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

Disadvantages

• Extremely expensive

• Heavy and not very portable

• Available for younger ages (2 years) and adults

• Easy to use and maintain

portable

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Intrapulmonary Percussive Ventilation

How It Works:– Delivers internal intrathoracic percussion and aerosolized solution for bronchodilationsimultaneously

– 10‐30 cm H20 pressure is generated and mini‐bursts 2are delivered into the lungs at rates of 100‐240 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



Advantages

• Can be used independently at home

• Simultaneous use of

Disadvantages

• Can be uncomfortable or not well‐tolerated

• May not be reimbursedSimultaneous use of bronchodilator medication

May not be reimbursed by insurance carrier

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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 ppairway 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

Disadvantages

• Recommended to be used with another ACT

• Not appropriate initiallypeople without CF

• Increased fitness = increased survival

• Can be tailored to particular patient

Not appropriate initially during acute exacerbation

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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 Issuesy

Ventilatory Muscles

Ventilatory Muscle Function

• Strength

• Endurance• Endurance

• Work Capacity

• Tension‐Time Index

Netter, F.H. (2003). Atlas of Human Anatomy, 3rd

ed. Icon Learning Systems, Teterboro, NJ

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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. 453.

Potential effects of CF on the Ventilatory Muscles

• Genetics

• Hyperinflation

• Nutritional StatusNutritional Status

• Corticosteroids

• Disease Severity

• Acute Exacerbations

• Aginghttp://www.springerimages.com

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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

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Age FEV1(%pred)

BMI 6MWD(ft)

MIP(cmH2O)

MIP(%pred)

A 28 104 20 1644 93 115

B 22 84 26.1 1926 143 124

Home‐Based IMT in Adults with CF

C 22 79 22.1 1600 108 99

D 36 44 19.1 1270 60 76

E 33 23 21.5 1910 104 102

Mean: 28.2 66.8 21.7 1670 101.6 103.2

Identifying Potential Candidates for IMT

1. Inspiratory muscle weakness (MIP< 60 cmH2O) and/or ventilatory limits to exercise.

2. Moderate to severe pulmonary disease (FEV1 <70% predicted)

3 Significant hyperinflation (RV/TLC >50%)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)

• ModeThreshold vs. Flow

• IntensityAt least 30% MIP

• Frequency3‐7x/week

• Duration30 min/session

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BREAK—5 minBREAK 5 min

Importance of Exercise for People with CF

Why is Physical Activity Importantfor CF?

• Physical reasons– Aerobic capacity

– Airway clearance

– Muscle strength

Weight issues

• Psychological reasons

– Self‐esteem

– Socialization

– Avoids the “sick role”– Weight issues

– Anti‐inflammatory effects

– Posture

– BMD

– Others?

– Body image

– Combats depression

– Combats “fatigue”

– Others?

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Why Are People (without CF)Physically Active?

• Physical reasons

– Disease prevention

– Weight management

• Psychological reasons

– Body image

– Stress management

– Increase strength

– Increase fitness

– Socialization

Why Are People With CF NOT Physically Active?

• Time

• Expense

• Effort/discomfort

• Insecurity

• Disease factors– Dyspnea

– Need for supplemental oxygen

– CFRD– Inability

– Body image

• Lack of access to facilities

CFRD

– Joint issues

– Exclusion from participation by others

– Stress urinary incontinence

– Fear?

Exercise Predicts Status

• VO2max is more strongly related to lifespan, quality of life and functional capacity than FEV1

• Annual exercise test should be done• Annual exercise test should be done

• All patients should view exercise as treatmentof CF (not just a nice thing to do)

– “Exercise is Medicine”™

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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

bi iaerobic 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, VO2/kg LBM (Gulmans et al 1999)

• 3‐year home aerobic exercise program slowed3 year home aerobic exercise program slowed decline in lung function compared to non‐exercising subjects with CF (Schneiderman‐Walker et

al 2000)

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Anaerobic Training in CF

• Children with mild lung disease, randomized to training or control group

• Training: 20‐30 second duration activities for 45 min twice weekly for 12 weeks

T i i i d bi f ( k• Training improved anaerobic performance (peak power, mean power), VO2peak, peak workload and Quality of Life

• Control group declined in VO2peak• 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 yearyear– 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

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Double‐Lung Transplantation

Photos courtesy of Woo MS.

50‐70% of patients with CF have osteoporosis (Hind et al J CF 2008), which may preclude this treatment.

Balance of Factors for Bone Density

Mechanical stresses

InactivityInflammation

Deposition Resorption

The Good News About Exercise and Inflammation…

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Bone Mineral Density in Cystic Fibrosis: Benefit of Exercise Capacity*

• 25 patients, aged 17‐52 underwent BMD assessment and maximal exercise test

• VO2 strongest independent predictor of BMD (R2= 0 86)(R2= 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):537‐542, 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):475‐481, 2011.

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

• Mean increases after 6 months:Mean increases after 6 months:– 0.9‐4.5% pre‐pubertal– 1.1‐5.5% early pubertal– 0.3‐1.9% pubertal age

• NOTE: delayed puberty in CF opens window for training longer

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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 ORMay 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

P t f t• Prevent fracture:

– Avoid high‐risk activities

– ? Suppress inflammation

• “Do something, there may be a risk. Don’t do anything, we know it will get worse!”

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Pelvic Floor Muscle Issue

• Chronic cough increases intra‐abdominal pressure

• If pelvic floor cannot hold, leakage happens

d b i• 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 askedp– 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 Centerin Your Local CF Center

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CF Care Centers

• Definition

– Specialized centers supported by the CF Foundation to provide multidisciplinary care for patients with CF

• Locationsocat o s

– 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 centerstheir own centers

• Guided by “coaches” and structured processes of quality improvement

Brigham & Women's Hospital and Children’s Hospital of Boston Example

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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)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)

New Patients:Appropriate GXT completed on last

For Readmissions:Same GXT as used in past is completed (see

treatment session (see J-drive for

protocols

GXT results & individualized

HEP are emailed to CHB Clinic

Team

J-drive for past results &

prescriptions

An individualized HEP (aerobic &

interval) is developed &

given in paper form to the patient

GXT results & individualized

HEP are saved on the J-drive

BWH/BCH Incremental Treadmill Test

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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 2 METS 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 (insertworking 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 20‐30 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 2 METS 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 bothspeed and intensity during recovery phasesspeed 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 10‐15 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

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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!)

PT and Cystic Fibrosis Part Icardiopt.org/csm2013/PT-and-Cystic-Fibrosis-Part-I-ID-1382196.pdf ·...

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