Presentation 212 d gardner_the pyramid of care for als

The Pyramid of Care for ALS

Patients to Decrease Their

Work of Breathing

De De Gardner MSHP RRT-NPS FAARCChair – Department of Respiratory Care

Stephen Lloyd Barshop Endowed ProfessorUniversity of Texas Health Science Center

at San Antonio

ALS and Airway Clearance

(ALSAC) Is There a Best Therapy

for Airway Clearance in Patients

with ALS?

Disclosures

ALS Association and Will Rogers Institute

Grant : ALS and Airway Clearance (ALSAC) Is

There a Best Therapy for Airway Clearance in

Patients with ALS?

Environmental Protection Agency (EPA):

Faculty as agents of IAQ Change Grant

University of Texas System Grant: WIPE

ASTHMA

University of Texas Health Science Center for

Ethics and Humanities Grants

OBJECTIVES

Describe causes of increased work of

breathing.

Evaluate a patient with ALS using the

pyramid of care™ to address increased work

of breathing.

Describe the role of the airway clearance

therapies for managing patient with ALS.

Compare and contrast therapies used to

manage increased work of breathing in

patients with ALS.

Normal Breathing

Increase Work of Breathing

Inspiratory muscles weaken

Diaphragm weakens = Hypoventilation

Expiratory muscles weaken = Poor cough effort

Bulbar muscles weaken = Dysphagia and dysarthria

Oppenheimer, EA, Guth, D, Fischer, J. Treating Respiratory Problems in ALS patients can improve quality of life.

ALS Functional Rating Scale –

Revised (ALSFRS) Scale of QOL

Disease progression

Assess the respiratory component of the

disease

Dyspnea

Orthopnea

Secretion issues

Compliance with NIPPV

http://www.oxfordmnd.net/information/ALSFRS-

R.pdf

Evaluate Work of Breathing

Vital Capacity

Peak Expiratory Cough Flow

Maximum Inspiratory Pressure (MIP) OR

Sniff Nasal Inspiratory Pressure (SNIP)

Maximum Expiratory Pressure (MEP)

MIP/MEP Standing/sitting AND supine

Evaluate Work of Breathing

Vital Capacity

Peak Expiratory Cough Flow

Maximum Inspiratory Pressure (MIP) OR

Sniff Nasal Inspiratory Pressure (SNIP)

Maximum Expiratory Pressure (MEP)

MIP/MEP Standing/sitting AND supine

Respiratory IssuesGradual deterioration of corticobulbar area of the

brainstem

Facial, head and neck muscles

Weakening of diaphragm and intercostal muscles

Breathing consumes energy & Increased fatigue

Increased Sialorrhea & Thick/sticky mucus

Ineffective cough

Inability to mobilize secretions

Aspiration

Invasive Ventilation

Airway & Secretion

Management

EARLY initiation ofNon Invasive Ventilation

Adjuncts for increased work of breathing

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Meet the patient where there

are

Patient AwarenessMorning Headache

Confusion or foggy mind

Frequent yawning or sighing

Daytime sleepiness

Difficulty sleeping

Shortness of Breath (SOB) with activity

Dyspnea

Power of voice decreases or sense a weak

voice

InvasiveVentilation

Airway & Secretion

Management

EARLY initiation ofNon Invasive

VentilationAdjuncts for

increased work of breathing

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First Steps for First

Signs/SymptomsIncrease head of bed by

45o

Use foam wedge as a

pillow

Incentive Spirometry

Breath Stacking

Conserve energy

Breathing control

Pursed lip breathing

Use NIPPV if ordered

Morning Headache

Confusion or foggy mind

Frequent yawning or sighing

Daytime sleepiness

Difficulty sleeping

Shortness of Breath (SOB) with activity

Dyspnea

Weaker voice

InvasiveVentilation

Airway & Secretion

Management

EARLY initiation of Non Invasive

Ventilation

Adjuncts for increased work of breathing

Wo

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Patient Awareness

Previous signs/symptoms

Spouse complains of snoring or apnea

Claustrophobia

Difficulty speaking

Vivid Colorful or nightmare type dreams

Not able to lie flat in bed

All signs association with

nocturnal hypoventilation

Evaluate Respiratory Function

Medicare criteria for NIPPV

FVC less than 50% (less than 80%)

MIP less than -60 cm H2O

SNIP less than -40 cm H2O

Nocturnal oximetry is less than 88% for

5 minutes

National Institute for Health and Clinical Excellence, Centre for Clinical Practice. The motor

neuron disease: the use of non-invasive ventilation in the management of motor neuron,

disease. London; July 2010:CG 105.

Non Invasive Positive

Pressure Ventilation Acronyms & Synonyms

NIV

NIPPV

Bi-PAP

Decreases the work of breathing

Increases depth of breathing

Increases mucous mobility

NIPPVBegin with IPAP (12 cm H2O) and EPAP (5 cm H2O)

Depending on patient and interface

Start with short time frames

Wear it during the day

Work up to all night

Start immediately at night and all night

Only daytime

Only night time

24 hours a day x 7 days a week

Need to provide patient 2 interfaces

Decrease chance of skin break down Alsa.org

Masks (Interfaces)

Masks (Interfaces)

Early NIPPVOptimal timing is difficult

Symptomatic

Early intervention

Prolong survival time ~ 1 week – 6 months

Reduces the decline of Vital Capacity

Better compliance

Cost effective

Miller RG, Jackson CE. Practice Parameter Update: Neurology. 2009

Chio A, Logroscino G and et. Al Prognostic Factors in ALS: A Critical Review. 2009. 10; 5-6: 310-323

Gruis KL, Chernew ME, Brown DL. The cost effectiveness of early noninvasive ventilation for ALS patients. BMC Health Services Research. 2005; 5:58.

Evaluate Respiratory Function

Medicare criteria for NIPPVFVC less than 50% MIP less than 60 cm H2OSNIP less than 40 cm H20Nocturnal oximetry is less than 88% for 5 minutes

National Institute for Health and Clinical Excellence, Centre for Clinical Practice. The motor neuron disease: the use of non-invasive ventilation in

the management of motor neuron, disease. London; July 2010:CG 105.

BREATHING, SNIFFING,

& COUGHING

InvasiveVentilation

Airway & Secretion

Management

EARLY initiation of Non Invasive Ventilation

Adjuncts for increased work of breathing

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Patient Awareness

Difficult to clear airways

Sense of smell decreases

Power behind the cough is gone

Inability to sniff or blow nose

“Just cant get enough air in”

Inflammation

Mucus production

Mucus plugging

Mucus retention

Infection

Increased work

of breathing

Shortness of breath

Decreased ability to

cough

Secretion Management

Reduce salivary gland issues

Mucolytics

Increase hydration

Oral Guaifenesin (Mucinex, Robitussin)

AnticholinergicsSuction devices

Mechanical Insufflator Exsufflator (MIE)

High Frequency Chest Wall Oscillation (HFCWO)

Biphasic Curiass

Fan Therapy

Box fan

Blowing on low, medium or high

Towards the face

Decrease perception of increased

WOB

Stimulates receptors in the trigeminal

nerve in the cheek and nasopharynx

Patient AwarenessIncrease saliva production

Choke on saliva

Drooling

Keep tissues at hand

Swallowing difficulty

Spasms in the airway (laryngospasm)

Notice change in consistency in saliva

Sialorrhea ManagementAnticholinergic Agents (Amitriptyline)

Glycopyrolate (Robinul)

Hyocyamine (Levsin)

Atropine (Saltropine)

Clonazepam (Klonopin)

Transderm – scopolamine

Botox

Parotid or submandibular gland radiation therapy

Increase fluid intake

Oral suction device

PATIENT AWARENESS

Coughing /choking when eating OR drinking

Change food content or consistency

Increase time to eat more than 30 -45 minutes

Tired when eating

Losing weight or not able to maintain weight

Dehydrated

VC drops by 20% or is at 50%

Percutaneous Endoscopic

Gastrostomy (PEG) tube

Allows control over feeding

Less tired

Avoids large meals that increase abdominal pressure

Allows small meals

Maintain hydration

Decrease risk for aspiration

Maintain weight

Reserve energy

Invasive Ventilation

Airway & Secretion

Management

EARLY initiation of Non Invasive Ventilation

Adjuncts for increased work of breathing

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PATIENT AWARENESS

Increased SOB with NIPPV

MIE/HFCWO demonstrates some

relief

Resources in place to do so

Personal choice

Invasive Mechanical

VentilationEnd stage ALS

NIPPV not as effective

Resources available

Elective tracheostomy

Passy-Muir Valve

Long Term Invasive Mechanical

Ventilation

Palliative Care

Invasive Ventilation

Airway & Secretion Management

EARLY initiation of Non Invasive Ventilation

Adjuncts for increased work of breathing

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PALLIATIVE CAREPatient does not opt for invasive ventilation

Hospice

VC less than 30%

NIPPV

Morphine

Acts on nerves

Decrease response to hypoxia and

hypercapnia

Alters perception of breathlessness

AAN Practice Parameter Update

Riluzole should be offered to slow disease progression

PEG considered to stabilize weight and prolong survival

NIPPV

considered to treat respiratory insufficiency to

lengthen survival

consider to slow the decline in FVC

improve QOL

Early NIPPV may increase compliance

MIE may be considered to help clear secretions

HFCWO is considered as well

Miller RG, Jackson CE. Practice Parameter Update: Neurology. 2009

ALS AIRWAY

CLEARANCE

(ALSAC) STUDY

Investigators De De Gardner, MSHP, RRT, FAARC

Carlayne Jackson, MD

Carolyn Walden

Pam Kittrel, RN

ALSAC STUDY ALS patients develop progressive expiratory respiratory muscle

weakness which leads to an ineffective cough.

The airway clearance devices are the Mechanical

Insufflation/Exsufflation (MIE) otherwise known as the CoughAssist™

and the High Frequency Chest Wall Oscillation device (HFCWO)

otherwise known to ALS patients as “the Vest”

The broad objectives of our program are to evaluate the combined

use of the MIE and HFCWO to provide full respiratory airway

clearance and cough assistance among ALS patients.

The Specific Aims of the Proposed

Pilot Project are:

To evaluate the effectiveness of the

MIE and HFCWO used in

combination compared to each used

alone

To compare compliance, tolerability

and quality of life among the three

groups.

Primary Hypothesis

MIE and HFCWO in combination will be more

effective than MIE or HFCWO alone.

Determined by evaluating the Respiratory

Complications Severity Scale

Capture events that would indicate the

severity of such complications from best

(no complications) to worst (death due to

respiratory complications).

Inclusion Criteria:

Adults diagnosed with ALS AND their caregivers

Age 21 or above

Peak cough flow less than 160 Liters per minute

Complaints of problems clearing airway secretions

Exclusion Criteria include:

Current use of SmartVest or CoughAssist

Tracheostomy

Congestive heart failure

All contraindications for the SmartVest

Head and/or neck injury that has not been stabilized;

Active hemorrhage with hemodynamic instability;

Uncontrolled hypertension;

Active or recent gross hemoptysis; and

All contraindications for the Cough Assist

History of bullous emphysema

Known susceptibility to pneumothorax

Pneumomediastinum

Recent barotrauma

Participant Commitments

Participants randomized to 1 of 3 groups

Participant and caregiver attend 3 clinic visits 3

months apart

Daily use of devices as prescribed

Record use of the device and secretion amount

daily

Home care respiratory therapist visit monthly

between clinic visits

Participant and caregiver complete

questionnaires

Visual Analog Patient’s/Caregiver’s Perception of patient’s

ability to move secretions

Scale

Poor ability to move airway secretions

Excellent ability to move airway secretions

Patient’s/Caregiver’s Perception of patient’s

ability to move secretions

Scale

No Problem

Serious Problem

Global Impression of Change

Patient and Caregiver

Since you have been in the study, do you feel

secretion removal is:

Markedly worse

Slightly worse

Not any different

Slightly better

Markedly better

Visit One/Two/Three

for the Patient and CaregiverPatient

Pulmonary Function Testing

Physical Exam

ALSFRS – R Survey Online

Impression of Change

Caregiver

Patient Caregiver Form

Impression of Change

Descriptive Results

N=28 (16 Males, 12 Females)

Diagnosed with ALS

Between the ages of 36 - 75

10 completed study

2 are active

13 have dropped

3 are deceased

Group 1 - MIE

n= 9 (5 males, 4 females)

5 dropped, 3 completed, 1 deceased

Group 2 MIE +HFCWO

n= 10 (7 males, 3 females)

4 dropped, 4 completed, 1 active, 1

deceased

Group 3 - HFCWO

n= 9 (4 males, 5 females)

4 dropped, 3 completed, 1 active, 1

deceased

Discussion Difficult to conduct research with ALS patients and caregivers

Complexities of the disease

Psychosocial confounders

Guidelines and Practice parameters demonstrate using the devices early to have a positive effect

Patients may not be psychological ready

Patients who presented to the clinic were further along in the disease process

It is unclear the patient and family were slow to seek care or that PCP are not familiar with the signs of symptoms of ALS.

Insurance companies can be a barrier to covering cost of equipment

Copays are a difficult issue for patients.

Conclusion

Increased work of breathing is scary

Involves respiratory weakness that leads to

respiratory failure

Respiratory parameters determine

respiratory insufficiency

Initiate PEG early on as well

Initiate NIPPV early and more aggressive

airway clearance therapies

Respiratory Therapist

Make a Difference

De De Gardner, MSHP, RRT-NPS, FAARC

210-567-7960

[email protected]

ALS Association Certified Center of Excellence

Medical Arts & Research Center

8300 Floyd Curl Drive, 4th Floor, MC 7883

San Antonio, TX 78229

Medical Director - Carlayne E. Jackson, M.D.

Phone: 210-450-9700

Fax: 210-450-6041