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04 05 0601
Interview:
Neuromuscular disease
management at home
in Singapore
Interview: Non-invasive
respiratory muscle aids
in the management of
neuromuscular disease
at home
Expert Opinion:
Essential practical
recommendations for home
mechanical ventilation
Insights:
Respiratory physiotherapy for
neuromuscular disease
BRE THING
ISSUE 1 / JULY 2015 / THE MANAGEMENT OF NEUROMUSCULAR DISEASE AT HOME
What are the main respiratory disorders treated at home?
The respiratory disorders treated at home may be categorised as:
1. Respiratory diseases needing pharmacologic treatment
(inhaled or systemic), and/or oxygen supplementation, but not
needing ventilation.
2. Obstructive sleep apnea needing CPAP therapy
3. Ventilatory insufficiency requiring ventilatory assistance
4. Impaired cough that requires assisted coughing
This interview will focus on assisted ventilation and assisted coughing.
What does it mean to be a Ventilator-Assisted
Individual (VAI)?
According to an American College of Chest Physicians consensus
statement, aVAI is someone who requires four or more hours of
ventilation each day for more than one month. In practice, aVAI
is someone with a chronic insufficiency of breathing capacity in
relation to the load of breathing.
Common medical conditions that lead to ventilatory insufficiency
include central hypoventilation, neuromuscular weakness, chest
wall deformities, and obesity hypoventilation. Chronic Obstructive
Pulmonary Disease (COPD) on the other hand is complex, though
we know that acute exacerbations respond well to Non-Invasive
Ventilation (NIV). Indeed, this was evidenced in a 2014 German
study which showed that the addition of NIV to standard treatment
significantly improved survival rates in patients with COPD.1
What criteria do you use to determine if a mechanically
ventilated patient is ready for home management?
The patient should demonstrate stability in the non-respiratory
systems, and sufficient respiratory stability to ensure home
respiratory equipment will be adequate for their needs. In
addition, both patient and caregiver need to be willing and able to
undertake responsibility of care at home in the long-term.
How do you select what type of ventilator to use?
There is now a range of ventilatory devices for home use, and
though the terminology differs, they are essentially classified as:
A. tracheostomy compatible, life-support ventilators;
B. tracheostomy compatible ventilators not suitable for life
support; or
C. NIV (non-life support).
A Singapore perspective:
Home respiratory
care experience
in patients with
neuromuscular
disease
Dr ChanYeow
Senior Consultant,
Department of Anaesthesiology,
Intensive Care and Pain Medicine,
Tan Tock Seng Hospital, Singapore
Organisation/meeting Dates
5th
Conference of the Union AgainstTB & Lung Disease – Asia Pacific Region 2015 31 August – 2 September, 2015
www.aprunion2015.com Sydney, Australia
Chinese Society of Respiratory Diseases Annual Meeting 2015 3–6 September, 2015
www.csrd.org.cn Guiyang City, China
The 17th
International Meeting on Respiratory Care Indonesia 4 – 5 September, 2015
www.respina.org Jakarta, Indonesia
Vietnam National Respiratory Society meeting 2015 6 –7 November, 2015
www.vnrs2015.org Ho Chi Minh City,Vietnam
Indian Chest Society and the National College of Chest Physicians 4 –7 November, 2015
17th
Joint National Conference Jaipur, India
www.napcon2015.org
6th
Asia Pacific Congress of Bronchology and Interventional Pulmonology 26 –28 November, 2015
www.apcb2015.com Bangkok, Thailand
20th
Congress of the APSR 3–6 December, 2015
www.apsr2015.com Kuala Lumpur, Malaysia
Respiratory events in Asia (JULY - DECEMBER 2015)
Reading corner
Letter to the Editor:
Our readers are invited to write to the editor by volunteering
content that is relevant to the Asia-Pacific region, or other
content they feel needs coverage in a publication such as
this.Your input is welcome and valued, particularly with case
studies and hot topics currently debated in the field, as well
as reviews of Asia Pacific congresses and conferences that
you might like to share with the audience.Your letters will
be featured in future issues of Breathing Matters, allowing an
open forum between the experts, to increase the level of
engagement amongst the audience.
Email us on breathingmatters@philips.com with your content.
Copyright © 2015Breathing Matters is supported by
In the next issue of
A book offering viable
management alternatives that
result in prolonged survival
and enhanced quality
of life in patients with
neuromuscular disease.
• Describes how to eliminate the respiratory or cardiac causes
of complications and death for NMD.
• Emphasises the home care/management of severe disabled
individuals with NMDs and those who require ventilator use.
• Describes inexpensive methods of clearing airway secretions and
preventing episodes of respiratory failure that would otherwise
necessitate hospitalisations and expensive, invasive interventions.
The publisher and sponsor have made every reasonable effort to ensure the accuracy of the above weblinks and information provided however any liability or obligation for
loss or damage howsoever arising from the information provided is hereby disclaimed.
For more reading materials visit this website: http://www.doctorbach.com/
M A T T E R S
BRE THING
…the latest in the management of
COPD at home with KOL interviews
and essential updates.
The choice of a ventilator depends upon the:
• patient’s disease progression and requirements with regards
to mode, pressure/volume limits, and alarms.
• performance of the ventilator with regard to synchrony, comfort,
and nuisance alarms.
• ease of use, e.g. controls, screen configuration, weight,
and battery life.
• the cost/reimbursement package – while a rental model
allows a patient to use a lower grade ventilator and upgrade
when needed, when a model is purchased, the patient’s
expected disease progression and future care choices should
be considered and planned for (e.g. tracheostomy versus
no tracheostomy).
• quality of backup service package by vendors.
What types of monitoring systems are required in a
home setting?
This depends on the degree of ventilatory dependence
and also on the ability of the patient to adjust/remove the
interfaces. For example, a patient with mild mitochondrial
myopathy who is still independent with regard to activities of
daily living may only need a simple NIV with S/T modes, with a
minimum of alarms.Temporarily omitting ventilatory treatment
if the ventilator is not delivering normally is an option, and a
pulse oximeter should be used intermittently as an indication
of the need for intensified secretion clearance.
Conversely, a tracheostomized, bulbar, Amyotrophic Lateral
Sclerosis (ALS) patient with no breathing capacity will need
pulse oximetry, high pressure alarms (to detect occlusion),
and low pressure or disconnect alarms (to detect leaks or
disconnections in the system).
Both types of patients will benefit from an analysis of
downloaded data; for example, hours of usage, pressures
achieved, leaks, percentage of spontaneous breaths, etc.
How do you determine initial ventilator settings?
Again, this depends on the context. In a ventilator-dependent
patient in the Intensive Care Unit (ICU) awaiting discharge,
there is a need to balance the desire to liberate the patient
from the ventilator, albeit for hours or even minutes, with the
need to provide a generous enough buffer to cater for episodes
of mild infections – I generally set a fairly generous assist control
mode. If I am deflating the tracheostomy cuff to allow speech,
I would need to include a buffer in the pressure or volume
settings to compensate for the leak. Usually such patients benefit
from titration with blood gases or overnight oximetry to ensure
that settings are adequate.
Conversely, in a newly diagnosed ALS patient starting out on
NIV, encouraging compliance is important.We usually use the
lowest settings that allow reasonable improvement in chest
excursions, decreased use of accessory muscles, and most
importantly patient comfort.The settings will be titrated up over
weeks or months as the patient acclimatises to the NIV.
It is also vital to assess cough ability by measuring the peak
cough flow. If the cough ability is poor, airway clearance using
breath stacking (with a manual resuscitator), manually assisted
cough (similar to a Heimlich manoeuvre), or mechanical cough
assistance is necessary.
During ventilator management, is humidification necessary?
Absolutely! Many patients find that humidification improves
tolerance of NIV. For Invasive Ventilation (IV), the upper airway
is bypassed, and dry gas can lead to life-threatening mucus
plugs. If the tracheostomy cuff is inflated, passive systems (Heat
Moisture Exchangers [HMEs]) might be adequate. If the cuff
is deflated for speech, the addition of a heated humidifier is
prudent, as the air is exhaled out via the mouth, bypassing the
HME. However, it is difficult to provide heated humidification
when the patient is travelling around in a wheelchair.
What are the frequent challenges associated with home
ventilator management?
The challenges are many: changes in patient condition;
changes in caregiver arrangements (due to burnout, resignation,
etc.); risk management issues for very dependent patients; and
helping patients make delicate end-of-life decisions without
abandoning support for them.
Is it more cost effective to manage these patients at
home than in the hospital?
From a purely financial point of view, yes! In Singapore,
the cost of care for a VAI in an acute hospital general ward
is approximately SGD$900 per day, compared to SGD$150
at home where family members and informal caregivers
assume care of the patient.The team must always be
vigilant in supporting family and caregivers, as they carry a
substantial burden.
From the qualitative point of view, the patient at home is at
lower risk of cross-infection, has more autonomy in activities,
may participate in more leisure activities, and may even earn
a living.
What are the reimbursement schemes available for
ventilator dependent patients in Singapore?
In Singapore, there is universal health coverage comprising
government subsidies and a pooled insurance scheme to
supplement personal savings and out-of-pocket payments.
As this is still quite new to Singapore, the local Government,
with support from hospital-based charities, is running several
pilot projects with a few large acute hospitals to test cost-
effectiveness and refine the healthcare model.We hope that
in a few years, the benefits of a home ventilation programme
will be widely recognised and a long-term funding model will
be established.
What steps are needed to set-up an effective home
mechanical ventilation program?
This depends on the setting. A simple 4-step program is
suggested below.
In Singapore, the cost of care for a VAI in an acute hospital
general ward is approximately SGD$900 per day, compared
to SGD$150 at home where family members and informal
caregivers assume care of the patient.
Dr Yeow’s recommendation for a multi-disciplinary
home ventilatory care team:
A competent ICU-trained nurse who can function as a
nurse, a physician extender and a “therapist-extender”.
A responsible and responsive technical provider (vendor).
A physician skilled in prescribing and titrating long-
term ventilation and who can direct the nurse and the
technical provider.
A family physician who can help to look after all other
aspects of care.
A respiratory therapist to provide tertiary
equipment expertise.
Other therapists such as physical, occupational
and speech.
A medical social worker for financial and
psychosocial support.
• Learn as much as possible about long-term ventilation and
home care.
• Unlike acute ventilation in the ICU, a VAI at home is usually
awake, not sedated, and should be able to communicate and
control his/her environment.
• A pulmonologist or intensivist would need to familiarise
themselves with long-term care and rehab issues; i.e
synchrony and adequate ventilation, prevent side effects,
and maximise the patient’s daily ablities.
STEP 01
STEP 02
STEP 03
STEP 04
It is vital to assess cough
ability by measuring the peak
cough flow. If the cough ability
is poor, airway clearance using
breath stacking, manually
assisted cough, or mechanical
cough assistance is necessary.
Reference: 1. Köhnlein T et al. Lancet Respir Med. 2014 Sep;2(9):698-705.
We have been very privileged to learn from the experiences of
so many giants in home ventilation from USA, Canada, UK, France,
Netherlands, Germany, Spain, Portugal and Japan. Many of the
home ventilation practitioners are very generous people whose
raison d’être is to improve the lives of VAIs anywhere in the world.
• A team needs to be assembled, comprising, at a minimum,
a core home care practitioner (either an ICU-trained
nurse or a respiratory therapist), a technical provider
(vendor), a family physician and a community nurse.
• It is necessary and helpful to convince local health
authorities of the benefits of home ventilation, and win
their support.This can lead to budgetary changes and
structural changes that will decrease the burden on VAIs
and their families and improve quality of life for all.
• Seek to establish global learning networks.
A multi-disciplinary home
ventilatory care team
consists of an ICU-trained
nurse, a technical provider,
a family physician and a
respiratory therapist.
What are respiratory muscle aids?
Inspiratory and expiratory muscle aids are devices and
techniques that involve the manual or mechanical application
of forces to the body or intermittent pressure changes to the
airway to assist inspiratory or expiratory muscle function.The
devices that act on the body include Negative Pressure Body
Ventilators (NPBVs) and oscillators that create atmospheric
pressure changes around the thorax and abdomen, and body
ventilators and “exsufflation” (forced cough) devices that
apply force directly to the body to mechanically displace
respiratory muscles. Negative pressure applied to the airway
during expiration or coughing assists the expiratory muscles
as forced exsufflation, just as positive pressure applied to the
airway during inhalation (NIV) assists the inspiratory muscles.
Certain positive pressure ventilators or blowers have the
capacity to deliver CPAP. Likewise, certain negative pressure
generators or ventilators used to power NPBVs can create
Continuous Negative Expiratory Pressure (CNEP). CPAP and
CNEP, both first described in the 1870s, act as pneumatic
splints to help maintain airway and alveolar patency and to
increase functional residual capacity.They do not directly
assist respiratory muscle activity, are rarely indicated for
patients with primarily ventilatory muscle weakness, and
should not be considered examples of “NIV”.
Why are they important in the management of
Neuromuscular Disease (NMD)?
Respiratory muscle aids are important because they can
enable patients to avoid respiratory failure, hospitalisation,
intubations, tracheostomies, and a lifetime of needing nursing
care. Also, ventilator un-weanable, intubated patients can be
extubated to respiratory muscle aids so that they never need
tracheostomy tubes.
What types of muscle aids are used in the home setting,
and why?
Continuous Non-invasive Ventilatory Support (CNVS) is
best provided via 15 mm angled mouth pieces (available from
Philips-Respironics) for daytime aid using a Trilogy ventilator
with an active ventilator circuit with the “kiss trigger” on
assist/control mode with preset volumes set at 800 to 1400
mL and a physiological back-up rate. If patients are unable to
use mouthpieces, they can use nasal interfaces day and night
also on assist/control mode, with the same volumes and rate,
unless they develop too much abdominal distension – in which
case we would switch to pressure preset at about 18 to
20 cm H2O.
Do not use Bilevel Positive Airway Pressure (BiPAP) unless
you cannot afford a Trilogy and do not use a Trilogy on BiPAP
settings. In other words, an active circuit is preferable to
a passive circuit. With passive circuits and pressure preset
settings, “air stacking” for deep breaths to speak louder, and
cough with, can be impossible. Air stacking is critical for
healthy lungs so volume preset modes are preferable.
What are the relevant guidelines and/or protocols for
using respiratory muscle aids at home?
When patients develop symptoms of sleep hypoventilation
(fatigue, sleepiness, morning headaches) and show diminished
vital capacity, they are placed on the Trilogy on active circuit
mode at the settings noted above for sleep. If the patient
becomes weaker and has difficulty discontinuing nasal
ventilation in the morning, they are switched from nasal to
mouthpiece ventilation for use when awake during the day.
Polysomnograms are not useful.The goal is not to “titrate
away” apneas and hypopneas but to rest the muscle during
sleep by using full setting CNVS.Update on Non-Invasive Respiratory
Muscle Aids in the management of
neuromuscular disease at home
Professor John R. Bach
Professor and Vice-Chairman
of Physical Medicine and Rehabilitation,
Professor of Neurosciences,
UMDNJ-New Jersey Medical School, USA
Respiratory muscle aids are important because they can enable
patients to avoid respiratory failure, hospitalisation, intubations,
tracheostomies, and a lifetime of needing nursing care.
Three key clinical goals of respiratory muscle aids in the
management of NMD as highlighted by Professor Bach:
1. To maintain normal pCO2 (ventilation) and avoid any oxygen
supplementation (which would “turn off” the drive to breathe
and result in hypercapnia and ultimately in respirator arrest).
2. To clear airway secretions with the CoughAssist to maintain
oxygen saturation greater than 94% when awake.
3. To never agree to a tracheotomy, since it is never needed for
any neuromuscular condition except for ALS after the throat
muscles become spastic and the patient develops stridor.
References: 1. Bach JR, et al. Respir Care. 2015;60(4):477-483. 2. Bach JR, et al. J Rehabilitative Med. 2014;46:1037-1041. 3. IshikawaY, et al. Neuromuscul Disord. 2011;21:47–51.
Essential clinical updates:
11 practical recommendations
for home mechanical ventilation
Professor Nicolino Ambrosino
1. HMV must be prescribed within the setting of an
experienced and authorised centre.The centre should
be responsible for the organisation and maintenance of
the device.1
2. Adaptation to HMV in the ambulatory setting may be as
effective as adaptation in the hospital setting in terms of
therapeutic equivalence in stable patients with CRF due to
COPD, RTD, NMDs, or obesity hypoventilation syndrome.
Out-patient adaptation may represent an important cost
saving for the healthcare system.2
3. The physiological target of HMV must be specifically
defined in different conditions. It has been suggested that
HMV should aim to reduce hypercapnia under mechanical
ventilation and to normalise daytime arterial carbon dioxide
tension (PaCO2) during spontaneous breathing.3
4. In COPD patients with hypercapnia, NIV is able to improve
arterial blood gas levels and unload inspiratory muscles,
independent of whether it has been set clinically on patient
comfort or physiologically tailored to invasively measured
respiratory muscle effort and mechanics.4
5. Overall costs for patients and third-party payers, national
and local reimbursement policies, available human (caregiver)
and material (ventilator, interface, etc.) resources must
be defined before home discharge. Patients and caregivers
must be instructed and demonstrate proficiency with HMV.5
6. Changes of the ventilator or ventilator settings should always
be performed alongside arterial blood gas measurement and
clinical assessment of the patient.6
7. Devices (including identically built machines with
the same settings) should be exchanged within the
prescribing centre.7
8. Long-term NIV requires at least one reserve mask.
Accordingly, the number of masks required each year
should be agreed with the healthcare provider at the
time of prescription.The provider should guarantee 24/7
availability, and ensure a prompt and tailored service
(including back-up ventilators for ventilator dependent
individuals, processes for rapid admission, etc.).8
9. A humidifier is mandatory for invasive ventilation and is
also useful for NIV when symptoms are present.9
10. NMD patients with weak or absent cough, and children,
should be provided with a pulse oximeter and cough-
assist devices.10
11. The first follow-up visit must occur early after prescription
(4–8 weeks), and HMV success should be evaluated
according to pre-defined subjective, clinical, physiologically
measurable, and technically measurable parameters.11
References: 1. Windisch W et al. Pneumologie. 2010;64:640–652. 2. Hazenberg
A et al. Respir Med. 2014;108:1387-1395. 3. Köhnlein T et al. Lancet Respir Med.
2014;2:698-705. 4. Vitacca M et al. Chest. 2000;118:1286–1293. 5. Sunwoo BY
et al. Chest. 2014;145:1134–1140. 6. Farre R et al. Eur Respir J. 2005;26:86–94.
7. Vitacca M et al. Chest. 2002;122:2105–2114. 8. Ambrosino N, Vianello A. Respir
Care Clin North Am. 2002;8:463–478. 9. Nava S et al. Eur Respir J. 2008;32:460–464.
10. Ambrosino N et al. Eur Respir J. 2009;34:444–451. 11. Ambrosino N et al. Respir
Med. 2013;107:1124–1132.
Home physiotherapy for patients
with NMD
Dr Ong Hwee Kuan (DClinPT, Physiotherapy)
Principal Physiotherapist, Physiotherapy Department,
Singapore General Hospital, Singapore
Assistant Professor, Academic Programme, Singapore Institute of Technology
What roles do physiotherapists play in managing
patients with NMD or high spinal cord injuries?
Patients with NMD and spinal cord injury have different
presentations and issues. As a result, the physiotherapy
management approach differs between these two conditions.
However, the overall goals for both patient types are to:
1. prevent secondary complications in the neuromuscular and
cardiopulmonary systems; and
2. optimise function.
A physiotherapist can provide the following consultations,
as outlined by Dr Ong:
Strategies for limb management, specifically in contraction
prevention and spasticity management, through proper
positioning strategies, regular stretching, and joint range-of-
motion exercises.
Strategies for functional optimisation through providing skills
training, teaching coping mechanisms, energy conservation
techniques, gait strategies, and prescribing appropriate
assistive devices.This is especially important for patients
with NMD with a progressive disease profile. Improved
standing, balance, and gait are possible through training and
use of assistive devices or orthotics.Teaching fall prevention
strategies are important, as patients’ strength declines with
disease progression.
Strategies for preventing disuse atrophy or deconditioning
weakness, through appropriate home exercise prescription.
Low intensity strength and aerobic exercise programmes help
to optimise musculoskeletal and cardiorespiratory function;
they may also provide positive benefits of weight control and
a sense of wellbeing.The therapy goal is to maintain existing
strength or to slow progression of weakness, instead of
strengthening the weakened muscles.
Strategies for optimising pulmonary health, through teaching
augmented cough techniques such as; manual cough assist,
breath stacking, or through the use of adjuncts such as
mechanical insufflation/exsufflation and oscillatory devices
(e.g. high frequency chest wall oscillation or intrapulmonary
percussive ventilation). This is especially important for patients
with ineffective cough or frequent episodes of hospitalisation
due to respiratory infections.
Physiotherapists can offer
strategies for optimising
pulmonary health, through
teaching augmented cough
techniques or the use
of adjuncts.
Basics of Mechanical Ventilation
This educational Basics of Mechanical Ventilation app is designed for
healthcare professionals for the management of mechanical ventilation in
patients.The app provides basic, easy-to-understand definitions and diagrams
of various components of ventilation, and suggests normal value ranges. It
also features simple algorithms to guide ventilator management in various
settings and offers guidelines for weaning patients off mechanical ventilation.
This app is jointly developed by the Division of Respiratory Therapy and
Trauma at Lehigh Valley Health Network in Pennsylvania. Available free
from iTunes: https://itunes.apple.com/us/app/basics-mechanical-ventilation/
id671298263?mt=8
The Ventilator Calculator
This calculator is designed by a Respiratory Therapist for Respiratory
Therapists and it provides the practitioner with a fast and easy way to
measure initial ventilator settings, and a quick way to correct settings to
tailor the ventilator to the particular patient’s condition.The Ventilator
Calculator is an excellent tool, and can improve your ability to tailor
the ventilator settings to a patient’s condition. However, your clinical
evaluation of the patient’s condition and the physician’s direction will
always be required for excellent patient care.
This android app is available for USD$13.04 from Google Play:
https://play.google.com/store/apps/details?id=net.cruthu.ventcalc&hl=en
What are some of the basic assessment skills needed by
physiotherapists who manage NMD patients at home?
To provide more effective and holistic care, a physiotherapist
managing NMD patients at home should have the following
basic assessment skills:
• Muscle strength and length testing, for early identification
of new onset of muscle weakness and contracture as the
disease progresses.
• Mobility assessment and movement analysis, to ascertain
the ability and level of assistance required for functional
tasks such as transfer, ambulation, or stair climbing. It is
important to conduct timed-motor performance tasks at
regular intervals to monitor functional changes.
• Monitoring of response to exercise, including levels of
fatigue, changes in weakness and pain. In order to ensure
the right intensity of training, a physiotherapist must be able
to identify signs of overuse syndrome (overwork weakness)
which might exacerbate disease progression.
• Assessment of respiratory function such as breathing
pattern, respiratory rate, inspiratory and cough effort, and
interpreting clinical parameters such as oxygen saturation
from a pulse oximeter, forced vital capacity (supine and
upright), and peak cough flow (unassisted or assisted).
A physiotherapist should be able to identify abnormal
breathing patterns and signs of diaphragmatic weakness.
The home is a good place for caregiver training.Therefore, a
home physiotherapist must be able to assess the caregiver’s
competency in positioning, transfers and performing assisted
cough manoeuvres.
What were some of the important points discussed in the recent workshop organised by Singapore General
Hospital with regard to the respiratory management of NMD patients?
Important points from this 3-day workshop held in April 2014 included the following:
Assessment and management strategies for the 3 main components of respiratory failure in NMD: inability to ventilate,
aspiration risk & inability to cough.
It is important to optimise pulmonary health by ensuring:
1. Good lung expansion by routine manual insufflations when FVC < 1.5L (or 70% predicted). Examples of the techniques
include glossopharyngeal breathing, breath stacking techniques, or mechanical insufflations.1
2. Good cough effort by routine airway secretion clearance manoeuvres when peak cough flow < 270 L/min. This involves
teaching caregivers assisted cough techniques such as breath stacking, cough timed chest squeeze/abdominal thrust, or
mechanical exsufflation.1
Aggressive use of mechanical insufflation/exsufflation may aid extubation success in NMD with acute respiratory failure.2
Oximeter feedback protocol: Patients with peak cough flow (PCF) < 270 L/min should be taught to use oxymetry to guide the
usage of NIV and cough assist.The goal was to use NIV and cough assist as needed to keep oxygen saturation (SpO2) > 95%
in room air. Failing to attain an SpO2 >95% signals the need for quick medical attention.3
References: 1. Bach JR et al. Am J Phys Med Rehabil. 2013 Mar;92(3):267-77 2. Gonçalves MR et al. Crit Care. 2012 Dec 12;16(2):R48. 3. Tzeng AC, Bach JR. Chest. 2000
Nov;118(5):1390-6.
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PHRCS0837 Respiratory Care Newsletter_V10_FA

  • 1. 04 05 0601 Interview: Neuromuscular disease management at home in Singapore Interview: Non-invasive respiratory muscle aids in the management of neuromuscular disease at home Expert Opinion: Essential practical recommendations for home mechanical ventilation Insights: Respiratory physiotherapy for neuromuscular disease BRE THING ISSUE 1 / JULY 2015 / THE MANAGEMENT OF NEUROMUSCULAR DISEASE AT HOME What are the main respiratory disorders treated at home? The respiratory disorders treated at home may be categorised as: 1. Respiratory diseases needing pharmacologic treatment (inhaled or systemic), and/or oxygen supplementation, but not needing ventilation. 2. Obstructive sleep apnea needing CPAP therapy 3. Ventilatory insufficiency requiring ventilatory assistance 4. Impaired cough that requires assisted coughing This interview will focus on assisted ventilation and assisted coughing. What does it mean to be a Ventilator-Assisted Individual (VAI)? According to an American College of Chest Physicians consensus statement, aVAI is someone who requires four or more hours of ventilation each day for more than one month. In practice, aVAI is someone with a chronic insufficiency of breathing capacity in relation to the load of breathing. Common medical conditions that lead to ventilatory insufficiency include central hypoventilation, neuromuscular weakness, chest wall deformities, and obesity hypoventilation. Chronic Obstructive Pulmonary Disease (COPD) on the other hand is complex, though we know that acute exacerbations respond well to Non-Invasive Ventilation (NIV). Indeed, this was evidenced in a 2014 German study which showed that the addition of NIV to standard treatment significantly improved survival rates in patients with COPD.1 What criteria do you use to determine if a mechanically ventilated patient is ready for home management? The patient should demonstrate stability in the non-respiratory systems, and sufficient respiratory stability to ensure home respiratory equipment will be adequate for their needs. In addition, both patient and caregiver need to be willing and able to undertake responsibility of care at home in the long-term. How do you select what type of ventilator to use? There is now a range of ventilatory devices for home use, and though the terminology differs, they are essentially classified as: A. tracheostomy compatible, life-support ventilators; B. tracheostomy compatible ventilators not suitable for life support; or C. NIV (non-life support). A Singapore perspective: Home respiratory care experience in patients with neuromuscular disease Dr ChanYeow Senior Consultant, Department of Anaesthesiology, Intensive Care and Pain Medicine, Tan Tock Seng Hospital, Singapore Organisation/meeting Dates 5th Conference of the Union AgainstTB & Lung Disease – Asia Pacific Region 2015 31 August – 2 September, 2015 www.aprunion2015.com Sydney, Australia Chinese Society of Respiratory Diseases Annual Meeting 2015 3–6 September, 2015 www.csrd.org.cn Guiyang City, China The 17th International Meeting on Respiratory Care Indonesia 4 – 5 September, 2015 www.respina.org Jakarta, Indonesia Vietnam National Respiratory Society meeting 2015 6 –7 November, 2015 www.vnrs2015.org Ho Chi Minh City,Vietnam Indian Chest Society and the National College of Chest Physicians 4 –7 November, 2015 17th Joint National Conference Jaipur, India www.napcon2015.org 6th Asia Pacific Congress of Bronchology and Interventional Pulmonology 26 –28 November, 2015 www.apcb2015.com Bangkok, Thailand 20th Congress of the APSR 3–6 December, 2015 www.apsr2015.com Kuala Lumpur, Malaysia Respiratory events in Asia (JULY - DECEMBER 2015) Reading corner Letter to the Editor: Our readers are invited to write to the editor by volunteering content that is relevant to the Asia-Pacific region, or other content they feel needs coverage in a publication such as this.Your input is welcome and valued, particularly with case studies and hot topics currently debated in the field, as well as reviews of Asia Pacific congresses and conferences that you might like to share with the audience.Your letters will be featured in future issues of Breathing Matters, allowing an open forum between the experts, to increase the level of engagement amongst the audience. Email us on breathingmatters@philips.com with your content. Copyright © 2015Breathing Matters is supported by In the next issue of A book offering viable management alternatives that result in prolonged survival and enhanced quality of life in patients with neuromuscular disease. • Describes how to eliminate the respiratory or cardiac causes of complications and death for NMD. • Emphasises the home care/management of severe disabled individuals with NMDs and those who require ventilator use. • Describes inexpensive methods of clearing airway secretions and preventing episodes of respiratory failure that would otherwise necessitate hospitalisations and expensive, invasive interventions. The publisher and sponsor have made every reasonable effort to ensure the accuracy of the above weblinks and information provided however any liability or obligation for loss or damage howsoever arising from the information provided is hereby disclaimed. For more reading materials visit this website: http://www.doctorbach.com/ M A T T E R S BRE THING …the latest in the management of COPD at home with KOL interviews and essential updates.
  • 2. The choice of a ventilator depends upon the: • patient’s disease progression and requirements with regards to mode, pressure/volume limits, and alarms. • performance of the ventilator with regard to synchrony, comfort, and nuisance alarms. • ease of use, e.g. controls, screen configuration, weight, and battery life. • the cost/reimbursement package – while a rental model allows a patient to use a lower grade ventilator and upgrade when needed, when a model is purchased, the patient’s expected disease progression and future care choices should be considered and planned for (e.g. tracheostomy versus no tracheostomy). • quality of backup service package by vendors. What types of monitoring systems are required in a home setting? This depends on the degree of ventilatory dependence and also on the ability of the patient to adjust/remove the interfaces. For example, a patient with mild mitochondrial myopathy who is still independent with regard to activities of daily living may only need a simple NIV with S/T modes, with a minimum of alarms.Temporarily omitting ventilatory treatment if the ventilator is not delivering normally is an option, and a pulse oximeter should be used intermittently as an indication of the need for intensified secretion clearance. Conversely, a tracheostomized, bulbar, Amyotrophic Lateral Sclerosis (ALS) patient with no breathing capacity will need pulse oximetry, high pressure alarms (to detect occlusion), and low pressure or disconnect alarms (to detect leaks or disconnections in the system). Both types of patients will benefit from an analysis of downloaded data; for example, hours of usage, pressures achieved, leaks, percentage of spontaneous breaths, etc. How do you determine initial ventilator settings? Again, this depends on the context. In a ventilator-dependent patient in the Intensive Care Unit (ICU) awaiting discharge, there is a need to balance the desire to liberate the patient from the ventilator, albeit for hours or even minutes, with the need to provide a generous enough buffer to cater for episodes of mild infections – I generally set a fairly generous assist control mode. If I am deflating the tracheostomy cuff to allow speech, I would need to include a buffer in the pressure or volume settings to compensate for the leak. Usually such patients benefit from titration with blood gases or overnight oximetry to ensure that settings are adequate. Conversely, in a newly diagnosed ALS patient starting out on NIV, encouraging compliance is important.We usually use the lowest settings that allow reasonable improvement in chest excursions, decreased use of accessory muscles, and most importantly patient comfort.The settings will be titrated up over weeks or months as the patient acclimatises to the NIV. It is also vital to assess cough ability by measuring the peak cough flow. If the cough ability is poor, airway clearance using breath stacking (with a manual resuscitator), manually assisted cough (similar to a Heimlich manoeuvre), or mechanical cough assistance is necessary. During ventilator management, is humidification necessary? Absolutely! Many patients find that humidification improves tolerance of NIV. For Invasive Ventilation (IV), the upper airway is bypassed, and dry gas can lead to life-threatening mucus plugs. If the tracheostomy cuff is inflated, passive systems (Heat Moisture Exchangers [HMEs]) might be adequate. If the cuff is deflated for speech, the addition of a heated humidifier is prudent, as the air is exhaled out via the mouth, bypassing the HME. However, it is difficult to provide heated humidification when the patient is travelling around in a wheelchair. What are the frequent challenges associated with home ventilator management? The challenges are many: changes in patient condition; changes in caregiver arrangements (due to burnout, resignation, etc.); risk management issues for very dependent patients; and helping patients make delicate end-of-life decisions without abandoning support for them. Is it more cost effective to manage these patients at home than in the hospital? From a purely financial point of view, yes! In Singapore, the cost of care for a VAI in an acute hospital general ward is approximately SGD$900 per day, compared to SGD$150 at home where family members and informal caregivers assume care of the patient.The team must always be vigilant in supporting family and caregivers, as they carry a substantial burden. From the qualitative point of view, the patient at home is at lower risk of cross-infection, has more autonomy in activities, may participate in more leisure activities, and may even earn a living. What are the reimbursement schemes available for ventilator dependent patients in Singapore? In Singapore, there is universal health coverage comprising government subsidies and a pooled insurance scheme to supplement personal savings and out-of-pocket payments. As this is still quite new to Singapore, the local Government, with support from hospital-based charities, is running several pilot projects with a few large acute hospitals to test cost- effectiveness and refine the healthcare model.We hope that in a few years, the benefits of a home ventilation programme will be widely recognised and a long-term funding model will be established. What steps are needed to set-up an effective home mechanical ventilation program? This depends on the setting. A simple 4-step program is suggested below. In Singapore, the cost of care for a VAI in an acute hospital general ward is approximately SGD$900 per day, compared to SGD$150 at home where family members and informal caregivers assume care of the patient. Dr Yeow’s recommendation for a multi-disciplinary home ventilatory care team: A competent ICU-trained nurse who can function as a nurse, a physician extender and a “therapist-extender”. A responsible and responsive technical provider (vendor). A physician skilled in prescribing and titrating long- term ventilation and who can direct the nurse and the technical provider. A family physician who can help to look after all other aspects of care. A respiratory therapist to provide tertiary equipment expertise. Other therapists such as physical, occupational and speech. A medical social worker for financial and psychosocial support. • Learn as much as possible about long-term ventilation and home care. • Unlike acute ventilation in the ICU, a VAI at home is usually awake, not sedated, and should be able to communicate and control his/her environment. • A pulmonologist or intensivist would need to familiarise themselves with long-term care and rehab issues; i.e synchrony and adequate ventilation, prevent side effects, and maximise the patient’s daily ablities. STEP 01 STEP 02 STEP 03 STEP 04 It is vital to assess cough ability by measuring the peak cough flow. If the cough ability is poor, airway clearance using breath stacking, manually assisted cough, or mechanical cough assistance is necessary. Reference: 1. Köhnlein T et al. Lancet Respir Med. 2014 Sep;2(9):698-705. We have been very privileged to learn from the experiences of so many giants in home ventilation from USA, Canada, UK, France, Netherlands, Germany, Spain, Portugal and Japan. Many of the home ventilation practitioners are very generous people whose raison d’être is to improve the lives of VAIs anywhere in the world. • A team needs to be assembled, comprising, at a minimum, a core home care practitioner (either an ICU-trained nurse or a respiratory therapist), a technical provider (vendor), a family physician and a community nurse. • It is necessary and helpful to convince local health authorities of the benefits of home ventilation, and win their support.This can lead to budgetary changes and structural changes that will decrease the burden on VAIs and their families and improve quality of life for all. • Seek to establish global learning networks. A multi-disciplinary home ventilatory care team consists of an ICU-trained nurse, a technical provider, a family physician and a respiratory therapist.
  • 3. What are respiratory muscle aids? Inspiratory and expiratory muscle aids are devices and techniques that involve the manual or mechanical application of forces to the body or intermittent pressure changes to the airway to assist inspiratory or expiratory muscle function.The devices that act on the body include Negative Pressure Body Ventilators (NPBVs) and oscillators that create atmospheric pressure changes around the thorax and abdomen, and body ventilators and “exsufflation” (forced cough) devices that apply force directly to the body to mechanically displace respiratory muscles. Negative pressure applied to the airway during expiration or coughing assists the expiratory muscles as forced exsufflation, just as positive pressure applied to the airway during inhalation (NIV) assists the inspiratory muscles. Certain positive pressure ventilators or blowers have the capacity to deliver CPAP. Likewise, certain negative pressure generators or ventilators used to power NPBVs can create Continuous Negative Expiratory Pressure (CNEP). CPAP and CNEP, both first described in the 1870s, act as pneumatic splints to help maintain airway and alveolar patency and to increase functional residual capacity.They do not directly assist respiratory muscle activity, are rarely indicated for patients with primarily ventilatory muscle weakness, and should not be considered examples of “NIV”. Why are they important in the management of Neuromuscular Disease (NMD)? Respiratory muscle aids are important because they can enable patients to avoid respiratory failure, hospitalisation, intubations, tracheostomies, and a lifetime of needing nursing care. Also, ventilator un-weanable, intubated patients can be extubated to respiratory muscle aids so that they never need tracheostomy tubes. What types of muscle aids are used in the home setting, and why? Continuous Non-invasive Ventilatory Support (CNVS) is best provided via 15 mm angled mouth pieces (available from Philips-Respironics) for daytime aid using a Trilogy ventilator with an active ventilator circuit with the “kiss trigger” on assist/control mode with preset volumes set at 800 to 1400 mL and a physiological back-up rate. If patients are unable to use mouthpieces, they can use nasal interfaces day and night also on assist/control mode, with the same volumes and rate, unless they develop too much abdominal distension – in which case we would switch to pressure preset at about 18 to 20 cm H2O. Do not use Bilevel Positive Airway Pressure (BiPAP) unless you cannot afford a Trilogy and do not use a Trilogy on BiPAP settings. In other words, an active circuit is preferable to a passive circuit. With passive circuits and pressure preset settings, “air stacking” for deep breaths to speak louder, and cough with, can be impossible. Air stacking is critical for healthy lungs so volume preset modes are preferable. What are the relevant guidelines and/or protocols for using respiratory muscle aids at home? When patients develop symptoms of sleep hypoventilation (fatigue, sleepiness, morning headaches) and show diminished vital capacity, they are placed on the Trilogy on active circuit mode at the settings noted above for sleep. If the patient becomes weaker and has difficulty discontinuing nasal ventilation in the morning, they are switched from nasal to mouthpiece ventilation for use when awake during the day. Polysomnograms are not useful.The goal is not to “titrate away” apneas and hypopneas but to rest the muscle during sleep by using full setting CNVS.Update on Non-Invasive Respiratory Muscle Aids in the management of neuromuscular disease at home Professor John R. Bach Professor and Vice-Chairman of Physical Medicine and Rehabilitation, Professor of Neurosciences, UMDNJ-New Jersey Medical School, USA Respiratory muscle aids are important because they can enable patients to avoid respiratory failure, hospitalisation, intubations, tracheostomies, and a lifetime of needing nursing care. Three key clinical goals of respiratory muscle aids in the management of NMD as highlighted by Professor Bach: 1. To maintain normal pCO2 (ventilation) and avoid any oxygen supplementation (which would “turn off” the drive to breathe and result in hypercapnia and ultimately in respirator arrest). 2. To clear airway secretions with the CoughAssist to maintain oxygen saturation greater than 94% when awake. 3. To never agree to a tracheotomy, since it is never needed for any neuromuscular condition except for ALS after the throat muscles become spastic and the patient develops stridor. References: 1. Bach JR, et al. Respir Care. 2015;60(4):477-483. 2. Bach JR, et al. J Rehabilitative Med. 2014;46:1037-1041. 3. IshikawaY, et al. Neuromuscul Disord. 2011;21:47–51. Essential clinical updates: 11 practical recommendations for home mechanical ventilation Professor Nicolino Ambrosino 1. HMV must be prescribed within the setting of an experienced and authorised centre.The centre should be responsible for the organisation and maintenance of the device.1 2. Adaptation to HMV in the ambulatory setting may be as effective as adaptation in the hospital setting in terms of therapeutic equivalence in stable patients with CRF due to COPD, RTD, NMDs, or obesity hypoventilation syndrome. Out-patient adaptation may represent an important cost saving for the healthcare system.2 3. The physiological target of HMV must be specifically defined in different conditions. It has been suggested that HMV should aim to reduce hypercapnia under mechanical ventilation and to normalise daytime arterial carbon dioxide tension (PaCO2) during spontaneous breathing.3 4. In COPD patients with hypercapnia, NIV is able to improve arterial blood gas levels and unload inspiratory muscles, independent of whether it has been set clinically on patient comfort or physiologically tailored to invasively measured respiratory muscle effort and mechanics.4 5. Overall costs for patients and third-party payers, national and local reimbursement policies, available human (caregiver) and material (ventilator, interface, etc.) resources must be defined before home discharge. Patients and caregivers must be instructed and demonstrate proficiency with HMV.5 6. Changes of the ventilator or ventilator settings should always be performed alongside arterial blood gas measurement and clinical assessment of the patient.6 7. Devices (including identically built machines with the same settings) should be exchanged within the prescribing centre.7 8. Long-term NIV requires at least one reserve mask. Accordingly, the number of masks required each year should be agreed with the healthcare provider at the time of prescription.The provider should guarantee 24/7 availability, and ensure a prompt and tailored service (including back-up ventilators for ventilator dependent individuals, processes for rapid admission, etc.).8 9. A humidifier is mandatory for invasive ventilation and is also useful for NIV when symptoms are present.9 10. NMD patients with weak or absent cough, and children, should be provided with a pulse oximeter and cough- assist devices.10 11. The first follow-up visit must occur early after prescription (4–8 weeks), and HMV success should be evaluated according to pre-defined subjective, clinical, physiologically measurable, and technically measurable parameters.11 References: 1. Windisch W et al. Pneumologie. 2010;64:640–652. 2. Hazenberg A et al. Respir Med. 2014;108:1387-1395. 3. Köhnlein T et al. Lancet Respir Med. 2014;2:698-705. 4. Vitacca M et al. Chest. 2000;118:1286–1293. 5. Sunwoo BY et al. Chest. 2014;145:1134–1140. 6. Farre R et al. Eur Respir J. 2005;26:86–94. 7. Vitacca M et al. Chest. 2002;122:2105–2114. 8. Ambrosino N, Vianello A. Respir Care Clin North Am. 2002;8:463–478. 9. Nava S et al. Eur Respir J. 2008;32:460–464. 10. Ambrosino N et al. Eur Respir J. 2009;34:444–451. 11. Ambrosino N et al. Respir Med. 2013;107:1124–1132.
  • 4. Home physiotherapy for patients with NMD Dr Ong Hwee Kuan (DClinPT, Physiotherapy) Principal Physiotherapist, Physiotherapy Department, Singapore General Hospital, Singapore Assistant Professor, Academic Programme, Singapore Institute of Technology What roles do physiotherapists play in managing patients with NMD or high spinal cord injuries? Patients with NMD and spinal cord injury have different presentations and issues. As a result, the physiotherapy management approach differs between these two conditions. However, the overall goals for both patient types are to: 1. prevent secondary complications in the neuromuscular and cardiopulmonary systems; and 2. optimise function. A physiotherapist can provide the following consultations, as outlined by Dr Ong: Strategies for limb management, specifically in contraction prevention and spasticity management, through proper positioning strategies, regular stretching, and joint range-of- motion exercises. Strategies for functional optimisation through providing skills training, teaching coping mechanisms, energy conservation techniques, gait strategies, and prescribing appropriate assistive devices.This is especially important for patients with NMD with a progressive disease profile. Improved standing, balance, and gait are possible through training and use of assistive devices or orthotics.Teaching fall prevention strategies are important, as patients’ strength declines with disease progression. Strategies for preventing disuse atrophy or deconditioning weakness, through appropriate home exercise prescription. Low intensity strength and aerobic exercise programmes help to optimise musculoskeletal and cardiorespiratory function; they may also provide positive benefits of weight control and a sense of wellbeing.The therapy goal is to maintain existing strength or to slow progression of weakness, instead of strengthening the weakened muscles. Strategies for optimising pulmonary health, through teaching augmented cough techniques such as; manual cough assist, breath stacking, or through the use of adjuncts such as mechanical insufflation/exsufflation and oscillatory devices (e.g. high frequency chest wall oscillation or intrapulmonary percussive ventilation). This is especially important for patients with ineffective cough or frequent episodes of hospitalisation due to respiratory infections. Physiotherapists can offer strategies for optimising pulmonary health, through teaching augmented cough techniques or the use of adjuncts. Basics of Mechanical Ventilation This educational Basics of Mechanical Ventilation app is designed for healthcare professionals for the management of mechanical ventilation in patients.The app provides basic, easy-to-understand definitions and diagrams of various components of ventilation, and suggests normal value ranges. It also features simple algorithms to guide ventilator management in various settings and offers guidelines for weaning patients off mechanical ventilation. This app is jointly developed by the Division of Respiratory Therapy and Trauma at Lehigh Valley Health Network in Pennsylvania. Available free from iTunes: https://itunes.apple.com/us/app/basics-mechanical-ventilation/ id671298263?mt=8 The Ventilator Calculator This calculator is designed by a Respiratory Therapist for Respiratory Therapists and it provides the practitioner with a fast and easy way to measure initial ventilator settings, and a quick way to correct settings to tailor the ventilator to the particular patient’s condition.The Ventilator Calculator is an excellent tool, and can improve your ability to tailor the ventilator settings to a patient’s condition. However, your clinical evaluation of the patient’s condition and the physician’s direction will always be required for excellent patient care. This android app is available for USD$13.04 from Google Play: https://play.google.com/store/apps/details?id=net.cruthu.ventcalc&hl=en What are some of the basic assessment skills needed by physiotherapists who manage NMD patients at home? To provide more effective and holistic care, a physiotherapist managing NMD patients at home should have the following basic assessment skills: • Muscle strength and length testing, for early identification of new onset of muscle weakness and contracture as the disease progresses. • Mobility assessment and movement analysis, to ascertain the ability and level of assistance required for functional tasks such as transfer, ambulation, or stair climbing. It is important to conduct timed-motor performance tasks at regular intervals to monitor functional changes. • Monitoring of response to exercise, including levels of fatigue, changes in weakness and pain. In order to ensure the right intensity of training, a physiotherapist must be able to identify signs of overuse syndrome (overwork weakness) which might exacerbate disease progression. • Assessment of respiratory function such as breathing pattern, respiratory rate, inspiratory and cough effort, and interpreting clinical parameters such as oxygen saturation from a pulse oximeter, forced vital capacity (supine and upright), and peak cough flow (unassisted or assisted). A physiotherapist should be able to identify abnormal breathing patterns and signs of diaphragmatic weakness. The home is a good place for caregiver training.Therefore, a home physiotherapist must be able to assess the caregiver’s competency in positioning, transfers and performing assisted cough manoeuvres. What were some of the important points discussed in the recent workshop organised by Singapore General Hospital with regard to the respiratory management of NMD patients? Important points from this 3-day workshop held in April 2014 included the following: Assessment and management strategies for the 3 main components of respiratory failure in NMD: inability to ventilate, aspiration risk & inability to cough. It is important to optimise pulmonary health by ensuring: 1. Good lung expansion by routine manual insufflations when FVC < 1.5L (or 70% predicted). Examples of the techniques include glossopharyngeal breathing, breath stacking techniques, or mechanical insufflations.1 2. Good cough effort by routine airway secretion clearance manoeuvres when peak cough flow < 270 L/min. This involves teaching caregivers assisted cough techniques such as breath stacking, cough timed chest squeeze/abdominal thrust, or mechanical exsufflation.1 Aggressive use of mechanical insufflation/exsufflation may aid extubation success in NMD with acute respiratory failure.2 Oximeter feedback protocol: Patients with peak cough flow (PCF) < 270 L/min should be taught to use oxymetry to guide the usage of NIV and cough assist.The goal was to use NIV and cough assist as needed to keep oxygen saturation (SpO2) > 95% in room air. Failing to attain an SpO2 >95% signals the need for quick medical attention.3 References: 1. Bach JR et al. Am J Phys Med Rehabil. 2013 Mar;92(3):267-77 2. Gonçalves MR et al. Crit Care. 2012 Dec 12;16(2):R48. 3. Tzeng AC, Bach JR. Chest. 2000 Nov;118(5):1390-6. Connect