General precautions on respiratory care for COVID-19
patients with muscular dystrophies, severe physicaland intellectual disabilities, or neuromusculardisorders

The National Council of Directors of Muscular Dystrophy Facilities
The National Council for Severe Physical and Intellectual Disabilities
The National Hospital Neurology Council

1Preface
When patients with respiratory dysfunction due to neuromuscular disorders such as
muscular dystrophy, severe physical and intellectual disabilities, are suspected to have
COVID-19, the situation is quite different from that of acute respiratory failure among
general COVID-19 patients. In particular, when mechanical ventilation has already been
introduced for chronic respiratory failure in patients with these neuromuscular disorders,special attention is required regarding therapeutic considerations and infection control.
We (the National Council of Directors of Muscular Dystrophy Facilities, the National
Council for Severe Physical and Intellectual Disabilities, the National Hospital
Neurology Council) have developed advice on the treatment of confirmed or suspected
COVID-19 patients with neuromuscular disorders requiring respiratory care. This advice
was based on the opinions and findings of the facilities participating in the councils. The
advice also referred to other international recommendations. We hope that this advice
will prove useful in the preparation of protocols at each facility for facing COVID-19
patients.
June 19th, 2020
Kiyonobu Komai
The President of the National Council of Directors of Muscular Dystrophy
Facilities
Kazuya Goto
The President of the National Council for Severe Physical and Intellectual
Disabilities
Tetsuo Komori
The President of the National Hospital Neurology Council

2

Authors

National Hospital Organization Osaka Toneyama Medical Center: Tsuyoshi
Matsumura, Toshio Saito, Masahide Mori, Miki Kishida, Kenji Tamagaki,
Yoshiaki Yoshida
National Hospital Organization Yakumo Hospital: Yuka Ishikawa
National Hospital Organization Iou Hospital: Kiyonobu Komai (The
President of the National Council of Directors of Muscular Dystrophy
Facilities)
National Hospital Organization Nishi Beppu Hospital: Kazuya Goto (The
President of the National Council for Severe Physical and Intellectual
Disabilities)
National Hospital Organization Suzuka Hospital: Satoshi Kuru
National Hospital Organization Nishiniigata Chuo Hospital: Ryouko Koike
National Hospital Organization Yanai Medical Center: Takashi Miyaji
National Hospital Organization Sizuoka Medical Center: Kouichi Mizoguchi
National Hospital Organization Sendai Nishitaga Hospital: Atsushi Takeda
National Hospital Organization Aomori Hospital: Hiroto Takada
National Hospital Organization Niigata Hospital: Takashi Nakajima
National Hospital Organization Hakone Hospital: Tetsuo Komori (The
President of the National Hospital Neurology Council)

3Introduction
To prevent COVID-19, avoiding the so-called 3 Cs of "closed spaces with poor
ventilation", "crowded places", and "close-contact settings" appears crucial. Manypatients with neuromuscular disorders (NMDs) such as muscular dystrophies (MDs) andsevere physical and intellectual disabilities (SPID), however, need assistance in their
daily lives and close contact is thus unavoidable with these patients. When these patientsrequire medical care, the number and types of caregivers involved will differ dependingon each situation. Among the care and treatment required, respiratory care such asmechanical ventilation (MV) and cough augmentation is essential to maintain patient
health and quality of life (QOL). On the other hand, such treatments are known to scatteraerosols1-4). Dealing with a patient suffering from COVID-19 (including suspectedpatients) raises concerns about the risk of infection not only to the caregiver and/or
medical staff, but also to other people in contact with these personnel. Especially innursing homes and medical care wards, which care for many high-risk patients,
prevention of nosocomial infection is paramount. This document describes the risk ofdroplets and aerosols in respiratory care for patients with NMD, and outlines generalcountermeasures for nursing homes and medical care wards, etc., with reference tostudies and recommendations that have already been issued1,3).

Risk of droplets and aerosols during respiratory care of patients with NMD
1) In patients with NMD, respiratory dysfunction causes type II respiratory failure(alveolar hypoventilation) and reduces airway clearance.

42) Currently, non-invasive ventilation (NIV) is mainly used for type II respiratory
failure. However, NIV involves a non-closed circuit, and thus cannot avoid aerosol
diffusion1,3,4).
3) Even in MV with a tracheotomy (tracheal intermittent positive-pressure ventilation;
TIV), a closed circuit cannot be maintained in patients with severe tracheal deformity,
especially when a speech cannula or loosely adjusted cuff has been used. As a result,leakage will occur and aerosols will be dispersed around the patient.
4) Respiratory physiotherapy such as lung inflation and cough augmentation,mechanically assisted cough (MAC), and suction are extremely important for theprevention of secondary lung injury, but dispersal of droplets and aerosols with thesetreatments cannot be avoided.
5) These procedures are performed by the caregiver in close proximity to the patient, sowhen the patient has developed COVID-19, the caregiver will be exposed to massive
doses of infected droplets/aerosols. Therefore, in respiratory care for confirmed orsuspected COVID-19 patients, we should recognize the risks of droplets and aerosols,and should reduce these risks using appropriate precautions.

Leak points in NIV and countermeasures against them
1) In NIV, leaks can occur from the site of mask fitting, the mouth of nasal mask users,or the exhalation port. When the leak is large, inspiratory air flow from the ventilator
could be increased to maintain the set ventilation. A large amount of aerosol is thenlikely to be dispersed, and the environmental pollution becomes serious1).As acountermeasure, in confirmed or suspected COVID-19 patients, use of a full-facemask (or total face mask) that covers both the nose and mouth and fits the shape of5the face is highly recommended (Fig. 1a,b)1-3). A helmet-type mask that covers theentire head is also available (Fig. 1c), but positive pressure is difficult to maintainwith the air supply capacity of most of the currently used portable types of ventilator,
so a ventilator running off compressed air would be preferable. As ventilation
conditions will differ when the mask or circuit is changed, it is necessary to check thebreathing condition and adjust ventilator settings accordingly.

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Fig. 1. Examples of full-face, total face, and helmet-type masks
a.

b.

c.

a, b. Non-vented disposable masks (Classic Star® Dräger)Full-face mask® (a. left), Total face mask® (a. right)
Suppression of leaks is easily achieved by adjusting the inflation of the air cushion
providing the seal (b)c. Helmet-type mask (StarMed CaStar®; Intersurgical)

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Referring to the homepage of Intersurgical(https://www.intersurgical.com/products/critical-care/starmed-respiratory-hoods-fornon-invasive-mechanical-ventilation-niv)(2020/6/16)

2) In NIV, a single circuit with an expiratory port is widely used. In such cases, aerosols
are constantly scattered from the expiratory port. When a vented mask is used, theexhalate is disseminated into the room air, so a non-vented mask should be used,placing a filter with excellent virus-removal performance (hereinafter, “V filter”)
between the mask and exhalation port (Fig. 2a). This will reduce the amount of virus
diffusion. When a vented mask must be used, it may be possible to cover the portwith a non-woven mask, but care should be taken to avoid blocking exhalation itself.
3) In a single circuit with an exhalation valve, place a V filter between the mask andexhalation valve (Fig. 2b). Avoid using a heating humidifier when using the V filter.

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Fig. 2. Examples of single circuits
a. A single circuit with an expiratory port

b. A single circuit with exhalation valve

Referring to Trilogy platform COVID-19 example circuit configurations (Philips)

94) Where possible, it is advisable to use a double-branch circuit (Fig. 3) with V filters on
both inspiratory and expiratory sides1,3-5). A double-branch circuit is a standard
configuration in critical areas such as intensive care units, and is therefore recommended
for NMD patients as a preventive measure against the infection, although the number of
portable ventilators available at present is limited4,5).

Fig. 3. An example of a double-branch circuit (CleanAir ASTRALTM 150® ResMed)

Referring to the homepage of Fukuda Denshi Co Ltd. (partly modified by the authors)(https://www.fukuda.co.jp/medical/products/inhome_medical/astral.html)(2020/6/16)

105) Use of a heat moisture exchanger (HME) with bacterial filter function is appropriate
for humidification. When using a heating humidifier, use the automatic water supply
module to prevent the risk of aerosol diffusion during water supply4).
6) Additional installation of the V filter and HME increases dead space and circuit
resistance, which may alter ventilation conditions, evoke failure to detect spontaneous
breathing, and result in malfunction of the out-of-circuit alarm. Circuit resistance may
also rise further due to contamination and water retention, as usage time increases. For
these reasons, vital monitoring such as percutaneous O2 saturation (SpO2) as well as
respiratory alarms is recommended in the implementation of these procedures3,4).
7) Most types of portable ventilators take room air, so when a patient is suffering fromCOVID-19 and aerosols containing the virus are dispersed, the inside of the ventilator
will be contaminated. If available, selecting a ventilator that takes compressed air fromthe central piping is ideal. Use of a ventilator model with an air intake filter that has theability to remove viruses is also preferable4), but the number of such models in
distribution is limited. When these measures are not available, the ventilator used for a
confirmed or suspected COVID-19 patient must be solely dedicated to that individual
patient4). The circuit with ventilator must be disposable and should be replaced according
to an appropriate procedure to minimize aerosol diffusion4).

Leak points in TIV and countermeasure against them
1) In TIV, a closed circuit has basically been formed, but many NMD patients use loosely
adjusted cough or speech cannulae to maintain vocal communication, and leakage willthus occur from the mouth, nose and parts of the tracheostomy.

112) Open-type suction involves a further problem, in that aerosols are scattered during theprocedure. As a countermeasure, the respiratory circuit should be arranged in the same
way as NIV. Properly manage the cuff pressure to maintain a closed circuit6). Use aclosed tracheal suction system to prevent diffusion of aerosols during suction, or use adouble suction tube to reduce the frequency of suctions.

Precautions for oxygen therapy1) Oxygen monotherapy without MV should be avoided in patients with NMD. Whenrequired, it is preferable to use a face mask rather than a nasal cannula and to cover itwith a surgical mask, in order to prevent aerosol diffusion3), but the risk of CO2 retention
becomes markedly higher.
2) When oxygen is used, monitor the ventilation status by transcutaneous CO2
monitoring or regular blood gas tests, and when any concern about CO2 narcosis arises,
immediately consider introducing MV.

Precautions for MAC
1) Currently, several models are used as sputum-assisting devices. Their main functions
are mechanical insufflation-exsufflation (MI-E), intrapulmonary percussive ventilation(IPV), and high-frequency chest wall oscillation (HFCWO). MAC is extremely
important for maintaining airway clearance in NMD patients, but careful judgement of
the necessity and urgency is warranted for confirmed or suspected COVID-19 patientsand the maximum protective measures should be taken, since MAC produces anddisseminates large amounts of aerosols.

122) MI-E induces coughing by applying positive pressure to the airways for a few
seconds, then shifting to negative pressure to promote sputum production. In cases using
a mask, when adhesion is insufficient, leakage may occur from the mounting portion in
the positive phase. Even in cases of tracheotomy, leakage may occur if cuff pressure isinsufficient. Further, contamination of the inside of the device becomes a problem, since
the exhalate of the patient is sucked into the device during the negative phase. Keep themask suitably tight and properly adjust the cuff pressure to minimize leaks and place a V
filter between the mask/tracheal cannula and circuit.

3) For IPV, place a V filter between the expiratory port and mask/tracheal cannula andport.

4) In HFCWO, cover the percussion wrap attached to the chest wall with vinyl so that it
does not come into direct contact with the patient5). Covering the patient’s head with avinyl tent, etc., is expected to suppress aerosol diffusion to some extent, but evidence iscurrently lacking. The sputum support device has a risk of contaminating the inside ofthe device, because it takes room air regardless of the mode. Thus, limiting its use to
individual COVID-19 patients is necessary.

Precautions for respiratory physiotherapy
1) For patients with NMD, respiratory physiotherapy such as lung inflation withresuscitation bags and cough augmentation are performed. Air leaks from masks anddroplets/aerosols dissemination associated with cough also occur during these treatments,
so the necessity of physiotherapy is carefully judged in confirmed or suspected COVID19 patients. When using a resuscitation bag, attach a V filter between the mask/cannula

13and the resuscitation bag and exclusively use the resuscitation bag for the COVID-19
patients4).

2) Protective measures should be taken during lung inflation and cough augmentation,such as having the patient wear a mask and directing the face to the side opposite thecaregiver during exhalation, if possible. Adequate ventilation is important during andafter respiratory physiotherapy.

3) In NMD patients, respiratory physiotherapy is extremely important, even whensuffering from COVID-19, but treatment should be minimized in consideration of theinfection state and sputum status, and alternative treatments such as postural drainage
should be carefully considered3,7,8).

Consideration of the environment surrounding patient rooms and methods ofpreventing caregiver infection
1) Active ventilation, isolation of confirmed or suspected COVID-19 patients from noninfected patients, appropriate use of personal protective equipment (PPE), andenvironmental cleaning should be carefully considered.

2) The first step is to perform clear zoning. The rooms of COVID-19 patients must be
designated as infected areas, and a semi-clean area should be placed between infectedareas and clean areas. Clearly divide each area with a tape or partition to prevent viruses
from being brought into the clean area. This is particularly important when COVID-19
and non-infected patients are together within the same ward or facility, and care should
be taken not to cross the infected and clean areas.

143) For air ventilation, isolation in a negative-pressure compartment is ideal, in
consideration of the risk of constant aerosol diffusion in patients with NIV or
tracheostomy with a semi-closed circuit. Even if this is difficult, the confirmed orsuspected COVID-19 patient should be isolated in a private room as much as possible,
and adequate air ventilation should always be performed. This entails a certain
unavoidable cost, but it is preferable to create a negative-pressure space using a simple
negative-pressure device. If air flow from the infected area to semi-clean or clean areas isdifficult to control, consider blocking the air flow with a vinyl curtain.

4) Caregivers should wear N95 masks when entering infected areas. Wear PPE such as
gloves, face guard, vinyl apron (arm cover) and cap when performing care1,4,9). To
prevent contact infection, thoroughly clean the hands and perform hand sterilization at
the time of entry/exit and care, regularly perform environmental cleaning (disinfection)of the patient rooms and infected areas. Items in the infected area should not be taken
out, in principle. When such items must be taken out, thoroughly disinfect them. Timeand effort is required to remove PPE, and the caregiver is likely to be infected when
undressing. It is important that two members form a pair and check each other to ensure
appropriate removal.

5) Consider using telecommunications such as intercoms and videophones to avoid
unnecessary entry and stay in the infected room. In addition, individual caregivers should
avoid caring for both COVID-19 and non-infected patients within the same shift.

Things to be aware of when implementing infection control

151) To date, we have described general measures, but actual implementation of thesemeasures involves many difficulties and issues to be considered.

2) The first is the serious lack of supplies caused by the global pandemic of COVID-19.
In a questionnaire survey of 12 facilities in the Japanese National Hospital Organization,
which has special wards for patients with MD and SPID, serious concerns were raised
regarding the supply of ventilators, tracheal cannulae, ventilator circuits, HMEs and
HEPA filters. As a result, most facilities have taken or have been considering measures
such as postponing the replacement cycle or using alternatives (Table 1). A serious
supply shortage of PPE was also seen. N95 masks are indispensable in the care ofpatients at high aerosol risk1,4,9), but securing a stable supply has been difficult.

Table 1. Impact of COVID on supply of ventilators and related products (Urgent
investigation of 12 facilities related to supply of ventilators and related products during
pandemic condition of COVID-19)

Facility

Ventilator

Circuit, HME

TrachealcannulaEnteral
tube

○

×

×

△

○

○

×

×

○

△

×

×

×

×

×

△

△

1
2
3
○
4
5
○
6

16

○

×

○

○

△

×

×

×

○

×

○

×

×

×

×

×

×

×

○

7
8
○

9
10
11
12
○, As usual; △, considering measures (postponing the replacement cycle or using
alternatives, etc.); ×, lack of supplies or taking measures; Blank, no answer.
3) The second is a structural problem with facilities. Most hospitals and institutions thattreat patients with MD and SPID do not have a negative-pressure room, etc. Furthermore,
many facilities are obliged to treat COVID-19 and non-infected patients within the sameward, and proper zoning seems difficult in some facilities.
4) In addition, many problems are seen on the side of patients and also in terms of ethicalissues. In NMD patients, where respiratory care is essential, daily treatment and careshould be continued even under pandemic conditions of COVID-192,6,10). In the case ofconfirmed or suspected COVID-19 patients, a comprehensive judgment will be madeconsidering the necessity of treatment, the medical condition and the environment. Somepatients have developmental disorders, mental retardation, or dementia, and many
patients thus cannot accept changes even if it is necessary to change the ventilator systemor device in terms of infection control and treatment of infectious diseases. Concerns
have also been raised regarding mental stress associated with isolation and restrictions on
visits by family members6).

175) Infection control always involves an ethical dilemma, because of the constraints
placed on individual freedom and QOL. In particular, how much treatment is appropriate
when a positive contact is suspected due to onset in a family member or caregiver is anannoying problem. How best to adjust the many contradictory issues such as the life and
QOL of the patient, the safety of caregivers and other patients, and the maintenance of
medical functions may differ depending on the situation, the condition of the patient, theenvironment, etc. In actual application, it is important to take the best possible measuresaccording to individual circumstances, taking into consideration the infection status, thecondition of the general population, the necessity of respiratory care, the environment,
and collective immunity status.
6) On the other hand, COVID-19 is likely to become serious in NMD patients with
reduced respiratory function and swallowing dysfunction2,10). With COVID-19, therespiratory status may rapidly deteriorate even in the general population in a short time,
so changes in respiratory management methods and endotracheal intubation are likely tobe necessary. Endotracheal intubation is often difficult in patients with MD/SPID due tospinal/thoracic deformity and difficulty opening the mouth, so emergency intubation
should be avoided. If limits to NIV support exist, intubation should be performed during
a time when sufficient human resources can be secured3).
7) Consideration should be given to the use of a video laryngoscope, as intubation
procedures carry the risk of aerosol dissemination. To prevent droplets/aerosols fromdirectly hitting the operator, consider a device such as covering the head with an aerosolbox (Fig. 4).
Fig. 4. Example of an aerosol box

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Referring to the blog of the intensive care unit at Kameda Medical Center(http://www.kameda.com/pr/intensive_care_medicine/post_59.html) (2020/5/18)

Closing remarks
Clusters of COVID-19 have been formed in medical hospitals/institutions and nursing
homes. In medical wards and nursing facilities that treat many NMD patients requiring
respiratory care, precautions against infection within the facility are extremely important.
We hope you find this helpful in taking appropriate precautions while maintaining daily
care.

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