Chronic Lung Disease in the Patient with Neurodisability ... · with Neurodisability: Concepts and Management Strategies ... tracheostomy and/or airway clearance device ... Sources

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Chronic Lung Disease in the Patient with Neurodisability: Concepts and

Management Strategies

Robert H. Warren, M.D. Adjunct Professor, Department of Pediatrics,

UAMS @ ACH Farrah Jones, BS, RRT

Research Respiratory Therapist Arkansas Children’s Hospital Research Institute


Disclosure Information AACPDM 69th Annual Meeting | October 21-24, 2015

Speaker Name: Robert H. Warren, M.D.

Disclosure of Relevant Financial Relationships

I have no financial relationships to disclose.

Disclosure of Off-Label and/or investigative uses:

I will not discuss off label use and/or investigational use in my presentation


Presentation Objectives

Understand the pathophysiology of airway secretions in the patient with neurodisability and potential for progressive pulmonary dysfunction

Understand the potential clinical application of the Pulmonary Composite

Understand the basic principles of respiratory care medications and devices.

See, through the use of case presentations, the need for individualization of the respiratory therapy management plan

Admission Criteria for the ACRTDC

Child followed by Pulmonary Section

Diagnosis of any type of neuromuscular disease, chronic lung disease and/or syndromes or conditions causing chronic respiratory insufficiency

Requires ventilator support and/or tracheostomy and/or airway clearance device

MISSION STATEMENT

The Arkansas Center for Respiratory

Technology Dependent Children

(ACRTDC) is committed to providing

specialized care to respiratory

technology dependent children and

enhancing quality of life through

caregiver education, research and

advocacy.

CONGENITAL NEUROLOGICAL DIAGNOSES

Becker Muscular Dystrophy Cerebellar Atrophy Cervicothoracic

Syringomyelia/syrinx CHARGE Syndrome Congenital Muscular Dystrophy Congenital Hypomyelination

Syndrome CFC Syndrome Down Syndrome Duchenne Muscular Dystrophy Fredericks Ataxia Jarcho-Levin Syndrome Neuronal Migration Disorder FSH Muscular Dystrophy Cockayne Syndrome Thoracic Dystrophy

Merosin Deficient Muscular Dystrophy Myotonic Dystrophy Neiman Pick Partial Complex IV Mitochondrial

Disease Pompe Disease Soto Syndrome Spina Bifida Spinal Muscular Atrophy Types I & II Undiagnosed Mitochondrial Disorder Undiagnosed Neurodegenerative

Disorder X-linked Myotubular Myopathy Bethlehem Myopathy Marshall Syndrome Metachromatic Leukodystrophy Congenital Scoliosis


Acquired Neurological Diagnoses

NICU

Traumatic Brain Injury Non-accidental

Traumatic Brain Injury/ Spinal Cord Injury Accidental secondary to non-primary pulmonary condition


Sources of Excessive Airway Secretions

Ineffective cough

Airway hyposensitivity

Aspiration

Dysphagia

GER

Airway inflammation

Infection

Mucosal gland proliferation and hypertrophy


Decrease in airway sensitivity – Change in irritant fiber response

Airway Inflammation

Mucosal gland proliferation and hypertrophy

Infection leading to airway and parenchymal damage

Airway Responses


ORIGINATION OF AIRWAY SECRETIONS

INTRODUCED into the airway

PRODUCED in excess in the airway

RETAINED in the airway


Pulmonary Composite Of Child With Neurodisability

State of ambulation

Seizure Disorder

Dysphagia with secondary aspiration

GER with secondary aspiration

Hypopneic Breathing Pattern

Spasticity

Hypotonia/Ineffective Cough

Recurrent acute respiratory illness

Drooling – Excessive oral secretions


THE PULMONARY COMPOSITE

Ambulation Retained/Produced

Seizure Disorder Introduced

Dysphagia Introduced

GER Introduced


The Pulmonary Composite

Hypopnea Retained/Produced

Spasticity Introduced

Hypotonia Retained/Produced

Recurrent Illness Produced

Excessive oral secretions Introduced


Clinical Application of the Pulmonary Composite

Development of a SEVERITY SCORE

Assign “severity score” to each component of the pulmonary composite

Determine ability to decrease the “severity score” for each component


Evolution Of Chronic Lung Disease In Children With Neurodisability

Impaired cough and retained secretions

Aspirated secretions from dysphagia and GER

Respiratory infection / atelectasis

Bronchiectasis / pulmonary dysfunction

Diminished oxygenation

Alveolar hypoventilation and CO2 retention


“Working up” the patient: Clinical Assessment

History – Using the Pulmonary Composite as a Guide Physical Assessment: Visual observation, listen, auscultation Laboratory Date: Resting Tidal Volume End Tidal CO2 Pulse Oximetry Blood Gas Analysis: ABG – VBG – CBG Transcutaneous O2 and CO2 determination Radiology: Chest imaging Sinus imaging


Airway Bacterial Colonization in the Neurodisability Patient

Retrospective study

Total of 93 subjects studied

Charactersitic N (%) unless otherwise noted

Age at time of trach, mean (SD),years 3.0 (4.3)

Length of time with trach, mean (SD) years

6.2 (4.7)

Sex

Male 53 (57.0)

Female 40 (43.0)

Race/Ethnicity

White 58 (62.4)

African American 26 (28.0)

Hispanic 9 (9.7)

Table 1: Demographics of study population, N=93

Table 2: Underlying diagnosis that led to tracheostomy, N=93

Diagnosis Prevalence N (%)

Cerebral Palsy 24 (25.8)

Spina Bifida 5 (5.4)

Moebius Syndrome 3 (3.2)

Laryngomalacia 3 (3.2)

Head Injury 3 (3.2)

Diaphragm Paralysis 2 (2.2)

Mandibular Agenesis 2 (2.2)

Muscular Dystrophy 2 (2.2)

Organism Prevalence N (%)

Pseudomonas aeruginosa 84 (90.3)

Stenotrophomonas maltophilia 72 (77.4)

Serratia marcescens 62 (66.7)

Moraxella catarrhalis 57 (61.3)

MRSA 52 (55.9)

MSSA 43 (46.2)

Haemophilus influenza 41 (44.1)

Group B streptococcus 32 (34.4)

Streptococcus pneumoniae 26 (28.0)

Acinetobacter baumanii 24 (25.8)

Acinetobacter calcoacetuis 13 (14.0)

Serratia liquifacins 9 (9.7)

Haemophilus parainfluenzaw 7 (7.5)

Table 3: Prevalence of pathogens isolated from respiratory cultures of all subjects at any time since trach placement, N=93

Organism N (%) Mean time to isolation, Month (SD)

Mean time to isolation, Range

MSSA 22 5.2 (10.3) 0.2 – 41.7

MRSA 19 6.5 (7.5) 0.2 – 26.4

Pseudomonas aeruginosa

37 7.8 (7.2) 0.2 – 25.1

Stenotrophomonas maltophilia

34 11.0 (10.6) 0.2 – 43.9

Serratia marcescens 22 13.3 (11.7) 0.1 – 38.4

Moraxella catarrhalis

24 16.3 (14.7) 0.1 – 44.3

Haemophilus influenzae

17 16.4 (16.1) 0.1 – 44.4

Table 4: Mean time to isolation after trach for selected organisms, N=45 subjects with respiratory culture after 2006

Figure 1: Organism Prevalence in 2010 based on length of time with tracheostomy (N=90)

Years with tracheostomy

0-1 2-4 5-7 8+

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Identifying the Presence of a Specific Chronic Lung Disease of Neurodisability

IRB study 203740

Study: Cohort of patients with primary neurodisability diagnosis with NO pulmonary disease at the time of initial diagnosis

Hypothesis: Factors in the Pulmonary Composite produce chronic airway secretion overload with resultant progressive chronic lung disease over time.

Study Group: 136 patients age range x to x and with multiple congenital and acquired neurodisability diagnoses



IRB study 203740

Methodology:

Identify factors at time of initial presentation

to the pulmonary clinic

Follow factor persistence and accumulation

over time.

End points: Progression to pulmonary

insufficiency and mechanical ventilation or

end of study period whichever comes first



IRB study 203740

• Clinical Questions:

Role of pulmonary factors individually and

collectively

Individual contribution in order of

prominence


Blucker, R., Elliott, T.R., Warren, R.H., Warren, A.M. (2011). Psychological adjustment of family caregivers of children who have severe neurodisabilites that require chronic respiratory management. Family, Systems & Health, September 2011, Vol 29, No. 3, p.215


Trait Resilience and Distress Among Family Caregivers of Children with Severe Disabilities Receiving Pulmonary Services

Courtney Francis1, Ryan T. Blucker1, Robert Warren2, Ann Marie Warren3, Timothy R. Elliott1, and Jack W. Berry4

1Texas A&M University, Department of Educational Psychology, 2Arkansas Children’s Hospital, 3Baylor Institute for Rehabilitation, 4Samford University

Method

Introduction Results

This study is a cross-sectional study of 60 caregivers of children with severe

disabilities who were being followed for chronic pulmonary management.

Caregivers provided informed consent and completed the Big 5 Questionnaire

(BFQ). Participants were family caregivers of children with severe disabilities

who are seen in the Arkansas Center for Respiratory Technology Dependent

Children (ACRTDC) outpatient clinic. The caregivers of these children, and the

focus of this study, are typically parents (either coupled or single), grandparents,

older siblings, or an extended family member. Participants were self- identified

as a primary caregiver of a patient followed in the ACRTDC program. Measures

given include the PHQ (to assess depression and anxiety), the SF-12 (to assess

physical and mental health), and a measure of social problem solving abilities.

First hierarchical clustering with Ward's to get the personality clusters, then we

used a k-means clustering using the results of Ward's method as a starting point.

Differences between the personality prototypes on the adjustment variables were

then examined.

*all p’s < .05

The purpose of this study was to examine personality prototypes among family

caregivers of children with severe disabilities, and the relation of trait resilience

to their distress. Research indicates that as with many caregiver roles, burnout

and psychological distress are commonplace. Furthermore, the literature

suggests that the Big Five personality factors (John & Srivastava, 1999), can be

reliable predictors of functioning in important domains of life such as

psychological adjustment and coping (McCrae, 1991; McCrae & Costa, 1986),

overall happiness and satisfaction with life (Costa & McCrae, 1980; Myers &

Diener, 1995), and general physical health and wellness behaviors (Booth-

Kewley & Vickers, 1994). More recently, the Big Five personality can be used

to determine personality prototypes, including trait resilience, that may be

associated with adjustment and coping in times of stress (Berry, Elliott &

Rivera, 2007). Trait resilience has yet to examined among family caregivers,

generally, and specifically among caregivers of children with severe

neurodisabilities who require chronic pulmonary management.

A series of one-way ANOVAs revealed that the resilient caregivers

reported significantly lower levels of anxiety (p=.035) and greater

mental health (p=.001) than caregivers characterized as

uncontrolled or overcontrolled (See Table 1).

Overcontrolled caregivers reported significantly greater tendencies

to avoid problems, and a more negative problem-solving orientation

than other caregivers. Resilient caregivers reported more rational

problem-solving abilities and a more positive orientation than

others. These findings are consistent with the Berry et al study of

personality prototypes. As the literature suggests the characteristics

found in resilient prototypes do not uniquely predispose an

individual to better health and adjustment nor do the characteristics

found in overcontrolled and undercontrolled types predispose one to

distress.

Cluster analysis of BFQ yielded 3 cluster prototypes: 22

caregivers were characterized as resilient, 17 were

uncontrolled types, and 17 were overcontrolled types.

As referenced in Figure1, the resilient caregiver group is

a perfect reflection of what is typically found in trait

resilience: high extraversion, low neuroticism, and

above average on openness, conscientiousness, and

agreeableness. The undercontrolled group is high on

extraversion, but also high in neuroticism, and below

average in agreeableness and conscientiousness. The

overcontrolled group is low on extraversion and high

neuroticism (the signature feature of this type).

Discussion Family caregivers who have resilient personality styles may be less likely to have anxiety issues. Caregivers who are more overcontrolled may be particularly predisposed to distress and lack effective problem-

solving skills. Although the study is limited by its cross-sectional nature and its reliance on self-report measures, the results indicate that trait resilience may be identified among family caregivers, and caregivers

have characteristics associated with an overcontrolled personality style may be prone to distress and adjustment problems. This information may be useful in developing strategic programs for caregivers who may be

at risk for distress.

Figure 1. Table 1.


Discussion

Results indicate that resilient characteristics may derive from common and normative

personality traits. Resilient caregivers defined in this manner appear to have

significantly lower levels of depression and anxiety than other caregivers. Resilient

caregivers are also more likely to endorse resilient tendencies than caregivers who

tend to have overcontrolled personality styles. Resilient caregivers are also more

likely than overcontrolled caregivers to have supportive social relationships that help

them cope and that maintain their self esteem. However, resilient caregivers may not

differ from other caregivers in other coping behaviors, indicating that their ongoing

and enduring personality style may have a greater influence on their ability to adjust

than any single coping activity.

Conclusions

Although the study is limited by its cross-sectional nature and its

reliance on self-report measures, the results indicate that trait resilience

may be identified among family caregivers, and caregivers have

characteristics associated with an overcontrolled personality style may

be prone to distress and adjustment problems. This information may be

useful in identifying family caregivers at risk for distress and for

developing strategic programs for them.

Results

We conducted a cluster analysis of the Big Five personality measure to classify caregivers as a resilient

prototype (N = 22), an overcontrolled prototype (N = 22) or as an undercontrolled prototype (N = 19), based on an a

priori model of trait resilience. As depicted below, resilient caregivers were characterized by higher z-score

averages on Agreeableness and Conscientiousness, and low z-score averages on Neuroticism. Undercontrolled

caregivers had the highest z-score averages on Extraversion. Overcontrolled caregivers had the highest average

z-scores on Neuroticism.

A series of comparisons revealed that the Resilient caregivers differed from the other prototypes on several

variables.

Resilient caregivers were significantly less

Depressed on the PHQ2 screen (M = .05, SD = .22)

than overcontrolled caregivers (M = 1.48, SD = 2.20). Resilient caregivers also had a significantly

lower score on the PHQ item to screen for anxiety

than undercontrolled caregivers (M = .38, SD = .59;

M = 1.00, SD = .87; respectively). Resilient

caregivers had a significantly higher score on the

Conner-Davidson measure of resilience (M = 88.16,

SD = 7.23) than Overcontrolled caregivers (M =

75.02, SD = .13.79).Resilient caregivers were significantly more likely to cope by maintaining

social support, self esteem and psychological

stability (as measured by the CHIPS; M = 32.14,

SD = 7.73) than overcontrolled caregivers (M =

25.14, SD = 8.12). No other differences were found

between the caregiver personality prototypes on the other coping measures.

SP15 RESILIENCE AMONG FAMILY CAREGIVERS OF CHILDREN WITH SEVERE NEURODISABILITIES REQUIRING CHRONIC RESPIRATORY MANAGEMENT

Timothy R. Elliott1, Robert H. Warren2, Ryan Blucker3, Jack W. Berry4, Jessica Chang1, Ann Marie Warren5

1Educational Psychology, Texas A&M University, College Station, TX; 2Pediatrics, University of Arkansas for Medical Sciences, Little Rock, Arkansas, AR; 3Pediatrics, University of Oklahoma Health Sciences Center, Oklahoma City, OK; 4Psychology, Samford University, Homewood, AL; 5Trauma, Baylor University

Medical Center, Dallas, TX

Objectives

The study was conducted to identify the characteristics of

resilient caregivers of children with severe neurodisabilities who

require chronic respiratory management. Differences in the

psychological adjustment and coping styles of caregivers

classified as resilient, overcontrolled and undercontrolled were

also examined.

Methods

Design: Cross-sectional, correlational study.

Participants and Setting: 68 family caregivers of children

receiving care at the Arkansas Center for Respiratory

Technology Dependent Children outpatient clinic. All children had chronic pulmonary symptoms that required daily respiratory

plans and all had either acquired or congenital neurodisabilities.

Sixty-eight caregivers consented to participate in the study. All

but one were women, and the sample ranged in age from 24 to

60. Over 75% of the participants were Caucasian; the rest were

African-American.

Material and Methods: Consenting caregivers were given a packet containing the study questionnaires and these were

returned to clinic staff. Study instruments including a

personality measure of the Big Five Inventory of personality

traits; the Connor-Davidson measure of resilience; the two-item

depression screen on the Patient Health Questionnaire; and two

measures of coping: the Family Crisis Oriented Personal

Evaluation Scales (FACES) and the Coping Health Inventory for Parents (CHIPS).

Contact information: Timothy R. Elliott, PhD.,[email protected] Robert Warren, MD, [email protected]

http://www.archildrens.org/

http://www.uams.edu/


Disclosure Information AACPDM 69th Annual Meeting | October 21-24, 2015

Speaker Name: Farrah Jones, BS, RRT

Disclosure of Relevant Financial Relationships

I have no financial relationships to disclose.

Disclosure of Off-Label and/or investigative uses:

I will not discuss off label use and/or investigational use in my presentation


The Individual Respiratory Care Plan

Step 1: Patient Assessment

Step 2: Medication/Device selection

Step 3: Determine Frequency of Therapy

Step 4: Ongoing Evaluation


Creating The Respiratory Care Plan: Individualization Is The Key

1. Selection of aerosol medications: Bronchodilators, decongestants, anti-inflammatories, mucolytics, antibiotics

2. Delivery systems: MDI & spacer or updraft nebulizer possibly including use of hyperinflation delivery

3. Assessment for airway clearance devices: In-exsufflator, high frequency oscillation, intrapulmonary percussionator

4. Ventilator support


Bronchodilators

Albuterol (Proventil, Ventolin, Proair) Dosage: MDI – 2 puffs QID Nebulizer – 0.03 mL / kg QID Levalbuterol (Xopenex) Dosage: MDI – 2-4 puffs Q 6 hours

Nebulizer - 0.63 mg every 6 hours Ipratropium Bromide (Atrovent) Dosage: MDI - 2 to 4 puffs QID Nebulizer – 250 to 500 mcg QID


Anti-inflammatory

Fluticasone Propionate (Flovent)

Dosage: 88mcg to 880mcg BID

Budesonide (Pulmicort Respules)

Dosage: 0.25mg to 0.5mg BID nebulized

Beclamethasone Dipropionate (Qvar)

Dosage: 80mcg to 160mcg BID


Mucolytics

Acetylcysteine (Mucomyst) Dosage: 2 mL of 10% or 20% in 3mL of saline. Always give bronchodilator concurrently Dornase alfa (Pulmozyme) Dosage: 2.5 mg/unit dose Sodium Bicarbonate (Na HCO3 8.4%) Dosage: 1 mL in 3 mL normal saline given up to 4 times a day


Antibiotics

Tobramycin Inhalation (Tobi) Trade name: TOBI Dosage: 300 mg nebulized BID Tobramycin for injection Dosage: 80 mg nebulized BID (albuterol given prior) Ceftazadime (Fortaz) Dosage: 1 or 2 grams nebulized BID


Special Considerations

Delivery Devices

Order of medications

Financial/insurance


Delivery Devices


Order of Medications

• Bronchodilators

• Mucolytics

• Anti-inflammatories

• Antibiotics


Mucus Mobilization Airway Secretion

Clearance

Manual physical therapy techniques

Mechanical devices - Vibrators - HFCWO - IPV - M/I-E



Special considerations

Order of therapies

Tracheostomy considerations

Financial / insurance barriers

Caregiver limitations

Patient tolerance

Ability to clear secretions after secretion mobilization techniques


Skills Validation Quality Improvement Project

Presently ongoing in caregivers of children with a primary neurodisability who have secondary acute and chronic pulmonary symptoms


Skills Validation Quality Improvement Project

Notification of the caregiver prior to clinic visit

The caregiver must bring their own equipment

Perform skills validation at the end of the clinic visit

Remedial education will be provided if needed

Certificate of completion

Pre and post surveys for caregiver feedback


Skills Validation Quality Improvement Project Preliminary Results

81% of participants had deficiencies in some area of home equipment use (set up, settings, technique, etc.)

81% would like to see SV for all respiratory equipment

45% reported an increase in confidence and decrease in anxiety when using equipment


Home Care

Establish daily respiratory care plan

Interaction with DME-Home Nursing

Develop stable lines of communication



Goals for the Child with Neurodisability

Provide clinical stability

Prevent acute exacerbations of illness

Reduce or halt the progression of pulmonary disease

Reduce/prevent emergency room visits

Reduce/prevent hospitalizations for acute pulmonary illness

Allow the patient to be integrated into the family unit


Case Study: 9 month old Caucasian male Diagnosis: Neurodisability secondary to Kernicterus

Pulmonary Composite Factors: Seizures, Dysphagia, Drooling, GER, Infective cough, extreme spasticity

Pulmonary Symptoms: cough, airway secretion accumulation (audible airway noise)

Challenges to the Respiratory Care Management Plan: resistance to all devices to deliver medications and airway clearance devices


What is your plan for this patient?

Medications?

Devices?

Frequency?


Case study: AF 17 year old Caucasian female Diagnosis: Merosin Deficient Muscular Dystrophy

• Pulmonary Composite Factors: GER, dysphagia, non-ambulatory, hypopneic breathing pattern

• Symptoms: chronic hypoventilation, but no other respiratory symptoms

• Challenges to respiratory care management plan: significant (100 degree) scoliosis with lung restriction, continuous NG tube, no tracheostomy



Medications?

Devices?

Frequency?

Ventilation?


Case study: 19 year old Caucasian female Diagnosis: Lissencephaly

• Pulmonary Composite Factors: non-ambulatory, GER, dysphagia, seizures, hypotonia, hypopneic breathing pattern

• Pulmonary Symptoms: chronic hypoventilation, copious amount of thick secretions, colonization with pseudomonas aeruginosa, occasional wheeze

• Challenges to Respiratory Care Management Plan: copious amount of secretions, insurance/financial barriers, large patient size, chronic bacteria colonization



Medications?

Devices?

Frequency?

Ventilation?


Case Study: 16 year old Caucasian male Diagnosis: Ulrich’s Muscular Dystrophy

Pulmonary Composite Factors: non-ambulatory, hypotonia, hypopneic breathing pattern

Pulmonary Symptoms: the need to create an aggressive pre-op respiratory care plan for spinal surgery to improve VC

Challenges to the Respiratory Management Plan: Currently on non-invasive ventilation for 8 hrs/night, albuterol MDI followed by cough assist BID



Medications?

Devices?

Frequency?

Ventilation?

Documents

Chronic Lung Disease in the Patient with Neurodisability ... · with Neurodisability: Concepts and Management Strategies ... tracheostomy and/or airway clearance device ... Sources