04 05 0601Interview: 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 THINGISSUE 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 insuffi ciency 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, a VAI is someone who requires four or more hours of ventilation each day for more than one month. In practice, a VAI is someone with a chronic insuffi ciency of breathing capacity in relation to the load of breathing.

Common medical conditions that lead to ventilatory insuffi ciency 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 signifi cantly 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 suffi cient 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 classifi ed 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 Chan YeowSenior Consultant, Department of Anaesthesiology, Intensive Care and Pain Medicine, Tan Tock Seng Hospital, Singapore

Organisation/meeting Dates

5th Conference of the Union Against TB & Lung Disease – Asia Pacifi c 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 Pacifi c 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)

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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 effor t 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 confi guration, 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 intensifi ed 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 benefi t 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 defl ating 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 benefi t 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 fl ow. 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 humidifi cation necessary?

Absolutely! Many patients fi nd that humidifi cation 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 infl ated, passive systems (Heat Moisture Exchangers [HMEs]) might be adequate. If the cuff is defl ated for speech, the addition of a heated humidifi er is prudent, as the air is exhaled out via the mouth, bypassing the HME. However, it is diffi cult to provide heated humidifi cation 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 fi nancial 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 refi ne the healthcare model. We hope that in a few years, the benefi ts 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 fi nancial 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 fl ow. 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 benefi ts 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 “exsuf f lation” (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.

Cer tain positive pressure ventilators or blowers have the capacity to deliver CPAP. Likewise, cer tain 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 Suppor t (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 diffi culty 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. Ishikawa Y, et al. Neuromuscul Disord. 2011;21:47–51.

Essential clinical updates: 11 practical recommendations for home mechanical ventilationProfessor 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 specifi cally defi ned 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 defi ned before home discharge. Patients and caregivers must be instructed and demonstrate profi ciency 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 fi rst follow-up visit must occur early after prescription (4–8 weeks), and HMV success should be evaluated according to pre-defi ned 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, SingaporeAssistant 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, specifi cally 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 impor tant for patients with NMD with a progressive disease profile. Improved standing, balance, and gait are possible through training and use of assistive devices or or thotics. Teaching fall prevention strategies are impor tant, 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 benefi ts 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 insuffl ation/exsuffl ation 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 defi nitions 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 par ticular 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 identifi cation 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 fl ow (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?

Impor tant 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 impor tant 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 effor t 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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