RESPIRATORYMEDICINE(1998) 92, 438-441

Long-term mechanical ventilation in amyotrophic

lateral sclerosis

J. ESCARIWBILL, R. ESTOPA, E. FARRERO, C. MONASTERIO AND F. MANRESA

UT-ES-Respirafhia - Servei de Pneumologia, Ciufaf Sanifhria i Universifhia de Bellvifge,

L’Hospifalef (Barcelona), Spain

Background. Acute respiratory insufficiency (ARl) with alveolar hypoventilation or incapacitating dyspnoea but
without peripheral muscle involvement can be an early manifestation of respiratory involvement in amyotrophic
lateral sclerosis (ALS). Some of these patients benefit from assisted ventilation. The object of this study was to
analyse the results of long-term mechanical ventilation (LTMV) in ten patients with ALS.
Methods. A retrospective analysis of intensive care unit (ICU) or ambulant patients with ALS who underwent
LTMV in a conventional hospital ward was performed. Erect and supine spirometry, blood gas analysis and pulse
oximetry were performed before the start and during the course of ventilation.
Results. Ten patients on LTMV were included. Four from the ICU were ventilated via tracheostomy, and six
ambulant patients had non-invasive (nasal) ventilation. In all cases,ventilation was performed in a conventional
hospital ward. The ambulant patients improved symptomatically during ventilation, confirmed by measurement of
gas exchange and of SaO, by continuous pulse oximetry. Three of the ten patients survive in long-term care - two
with nasal and one with tracheostomy ventilation.
Conclusions. LTMV outside ICU was possible in ten patients, seven of whom returned home. Returning home is
very difficult for patients dependent on a ventilator who lack family support.

RESPIR. MED. (1998) 92, 438-441

Introduction majority of cases, as the patient may become completely

Amyotrophic lateral sclerosisis the most common form of
the so-called motor neurone diseases.It is characterized by
the loss and degeneration of upper and lower motor neu-
rones, presenting initially with spasticity and hyper-reflexia
and later with fasciculations, weakness and loss of muscle
strength. The course of the disease is variable but it is
estimated that survival at 3 years is 50% (l), although 10%
live for up to 10 years (2).
Respiratory insufficiency usually manifests itself late in
the course of ALS. However, caseshave been described in
which the first manifestation of the disease was dyspnoea
with acute respiratory insufficiency (ARI) and alveolar
hypoventilation because of diaphragmatic involvement
(3-5) without involvement of peripheral muscles (6). In
some of these patients with severe symptoms, ventilatory
support has been proposed. Others have been intubated
and ventilated without knowledge of the neurological diag-
nosis. The decision to ventilate a patient with ALS is
complex, given the rapid evolution of the disease in the
dependent on the ventilation, and it does not influence the
underlying course of the disease.
We present our experience of long-term mechanical
ventilation (LTMV) of patients with ALS.
Methods
SUBJECTS
Since 1988, LTMV has been prescribed for ten patients with
ALS. In every case, the neurological diagnosis was estab-
lished by electromyography. The criteria for inclusion were:
(a) patients ventilated in ICU for some time who would not
tolerate disconnection of the ventilator for more than 24 h,
and (b) patients who had moderate involvement of periph-
eral muscles, that is, could walk and maintain an active life,
but had incapacitating dyspnoea, orthopnoea which made
lying down impossible, or diurnal hypercapnia (PaCO,
~45 mmHg.

Received 23 April 1996 and accepted in revised form 22 April PROTOCOL

1997.
Correspondence should be addressed to: Dr Joan Escarrabill,
UFISS- Respiratbria, Servei di Pneumologia, Ciutat Sanitaria i
Universitkia de Bellvitge, C/Feixa Llarga s/n, 08907 L’Hospitalet,
Spain.
In every case ventilation was performed in a conventional
hospital ward. The machines used were home volume-
controlled ventilators (PLV-100, Life Carem), Companion
2801 (Puritan Bennett@) or pressure-support ventilators

0954-6111/98/030438+04 $12.00/O 0 1998 W. B. SAUNDERS COMPANY LTD


TABLE 1. Basal characteristics of the ten patients
Age (years) 59.2 i 1.1
Sex (M/F) 812
Months of ALS evolution 17.0 f 12.3
PaO, (mmHg) (Fro,)* 78.3 * 13.1
PaCO, (mmHg) (Fro, 0.2)* 52.5 + 8.1
FVC sitting (ml)* 1830 f 997
FVC sitting (% theoretical) 50.7 f 19.9
FEV, sitting (ml)* 1478 zk918
FEV, sitting (% theoretical) 50 k 12.4
FVC supine (ml)* 1150&627
*Data corresponding to the six ambulatory patients.
(BiPAP S/T, Respironics In@). These were connected to
tracheostomy tubes or nasal masks which were made-to-
measure silicone (7) or commercial masks (Respironics’m).
Wherever possible, the following investigations were per-
formed before commencing ventilation: arterial blood gas
analysis when breathing room air, sitting and supine forced
spirometry (FVC, FEV,, FEC/FEV,%) and nocturnal pulse
oximetry (Pulsox 7, Minolta@) when breathing room air.
Once ventilation was in progress, we checked its efficacy by
carrying out arterial blood gas analysis with the patient
awake, and continuous pulse oximetry during nocturnal
ventilation. We considered that ventilation was effective if it
produced an improvement in symptoms, corrected hyper-
capnia (PaCO, ~45 mmHg when awake or a relative
decrease in PaCO, with improvement in pH up to 7.40),
and if nocturnal pulse oximetry without oxygen showed an
SpO, of greater than 90% for more than 85% of the night.
STATISTICAL ANALYSIS
A chi-squared analysis was used to compare proportions,
and the t-test was used to compare measurements. Values
of PcO.05 were considered significant.
Results
PATIENTS
Table 1 shows the characteristics of the ten patients pre-
scribed LTMV. Arterial blood gasesand pulmonary func-
tion tests were performed in the six ambulatory patients.
The spirometic data showed a restrictive disorder with a
significant fall in FVC (37%) in the supine position,
suggesting diaphragmatic impairment.
CONDITION OF THE PATIENTS WHEN
COMMENCING LTMV
Four patients were in the ICU because of acute respiratory
insufficiency requiring mechanical ventilation via tracheos-
tomy and had failed attempts at weaning. One was receiv-
ing nocturnal ventilation only. Three were being ventilated
almost continuously. The average stay in the ICU was 1.3
AMYOTROPHIC LATERAL SCLEROSIS 439
months (0.5-5.0 months). At the time LTMV was pro-
posed, these four patients were moved to a conventional
hospital ward. The other six were outpatients for whom
LTMV was prescribed during a programmed stay in hospi-
tal. The six had orthopnoea of between 2 and 6 months
duration. One was completely incapacitated when lying
down. Four had alterations of the voice due to shortness of
breath, and one had dysphagia to liquids.
NOCTURNAL BASAL PULSE OXIMETRY AND
SPIROMETRY
This was performed in the five ambulant patients who could
tolerate lying down. The results of pulse oximetry con-
firmed that they had an SpO, of less than 90% for 62.3%
(range 7-97%) of the time, the SpO, falling to between 85
and 90% in all but two caseswho desaturated below 85%.
VENTILATION CHARACTERISTICS
All the patients from the ICU kept their tracheostomy, and
a volume-controlled ventilator was used. The ambulant
patients had nasal masks. Three had a made-to-measure
mask and volume-controlled ventilator, three had a
standard mask and a high-flow, positive-pressure unit
(BiPAP@). Two of the patients using BiPAP later required
a change to a volume-controlled ventilator and a made-to-
measure mask to increase the efficiency of ventilation.
LTMV was given for an average of 12.2 months (1.5 to 47.5
months).
VENTILATION EFFECTIVENESS
The four patients from the ICU continued ventilation with
home units in the conventional hospital ward, demon-
strating that this was as effective as ventilation in the ICU.
A clinical improvement was observed in the ambulant
patients from the start of LTMV; they could better tolerate
lying down, and nocturnal pulse oximetry during ventila-
tion showed an SpO, of less than 90% only 8% (range
0.6-32%) of the time. Comparing these figures with those
of the five patients who had baseline pulse oximetry
measurements shows that the time during which the SpO,
is less than 90% is significantly lower during mechanical
ventilation (P<O.O05). Gas exchange also improved
during ventilation. The PaO, (mean + SD) increased
from 78.3 f 3.1 mmHg when breathing atmospheric air
to 87.2 + 9.0 mmHg during ventilation. The PaCO, fell
from 52.5 f 8.1 mmHg when breathing atmospheric air
to 43.2 f 8.9 mmHg during ventilation.
RETURNING HOME
In seven cases, the patients were able to continue HMV
(home mechanical ventilation). In two casesthere was not
enough family support to allow this, so these patients were
transferred to a non-hospital centre. One patient died in
hospital while adapting to ventilation.
440 J. ESCARRABILL ET AL.
TABLE 2. Causes of death in the ventilated patients
n-IPPV t-IPPV Total
Cause of death (four cases) (three cases) (seven cases)
ALS evolution 214 213 411
Sudden death l/4 l/3 2/l
Suicide l/4 l/7
n-IPPV=nasal mask positive-pressure ventilation.
t-IPPV=tracheostomy positive-pressure ventilation.
FOLLOW UP
Overall survival of our patients with ALS who required
ventilatory support was 60% at 6 months and 30% at 12
months. The causes of death are listed in Table 2. The
patient who committed suicide maintained the mobility of
his arms and used a firearm. The term ‘ALS evolution’
refers to progression of the muscular weakness with reduc-
tion in free time of ventilation and development of bulbar
impairment. In this situation, mechanical ventilation via a
nasal mask was not sufficiently effective. Although the
tracheostomy was proposed at that moment, and the
patients were informed about the possible improvement of
survival with this procedure, it was refused in all cases.
When symptoms of respiratory insufficiency appeared, the
palliative care with sedative treatments, including mor-
phine, were established. In the patients with tracheostomy
we do not know the exact cause of death because one
moved to another city and the other was admitted to a
hospice. Three patients with ALS are still alive, two on
home ventilation and one in long-term care, having had
ventilation for between 3 and 47.5 months. The two
patients on home ventilation use made-to-measure silicone
nasal masks, and the other is ventilated via a tracheostomy.
Discussion
There is a progressive reduction in vital capacity (VC) as
ALS develops, clearly related to muscular weakness(S), but
symptoms such as resting dyspnoea or orthopnoea usually
appear in very advanced phases of the disease (9). As the
respiratory muscles become involved, they become incapa-
ble of generating the airflow required to mobilize the
secretions, and the cough becomes inefficient. There is
frequent involvement of the upper airway, especially where
there is bulbar disease (10). Gas exchange impairment is a
late finding, usually observed when the VC falls by about
20% or less of the predicted value (2).
In spite of the pre-eminence of respiratory symptoms,
only 5% of patients in Illinois (U.S.A.) with ALS are
chronically ventilated, an incidence of two patients per
million inhabitants. This percentage varies greatly between
centres, from 1.6 to 14.3%, reflecting the different attitudes
of doctors towards LTMV in patients with ALS (11).
Some authors have suggested that the use of intermittent
negative-pressure ventilation using a poncho is efficient and
cheaper (12). However, positive-pressure ventilation using
volumetric ventilators is the most widely used and the safest
for the patient (13,14). Nogueras et al. (1.5)described HMV
in a patient with ALS for the first time in Spain, modifying
a model Mark-7 respiratory to suit the patient. The avail-
ability of small volumetric ventilators allows the use of
HMV in these cases. Our patients needed volume-
controlled ventilators because the pressure-support units do
not allow minute volume increments and do not have
alarms. Two of the three cases who started LTMV using
BiPAP were changed to volume-controlled ventilators,
In every case, LTMV was performed in conventional
hospital wards. In ambulant patients, ventilation corrects
orthopnoea, improves gas exchange and corrects nocturnal
desaturation. In our experience seven of the ten patients
who started LTMV were able to return home on HMV.
Salamand et al. (16) achieved HMV in a third of patients
with ALS and LTMV. Returning home depends .on the
neurological involvement of the patient, family support and
the available medical resources. Our resources are limited.
In our centre we have prescribed HMV to more than 200
patients and we have developed a home support pro-
gramme from the hospital. However, neuromuscular
patients, and especially those affected by ALS, may require
more continuous and specific attention than our pro-
gramme can provide and there are no other specific
resources or centres for these patients. The three patients
who could not return home were immobile (tetraparetic),
had tracheostomies and could not be weaned from the
ventilator. Two were moved to long-term care, and the
third died in a conventional hospital ward, outside the ICU.
The discussion about therapeutic alternatives should not
focus solely on the ICU and the home. The object should be
to avoid long stays in the ICU and find the ideal place
to treat the patient, in a conventional hospital ward, a
long-stay hospital, in long-term care or, where possible, at
home (17).
The survival of patients with ALS on LTMV is very
variable. Salamand et al. (18) describe survival of 1 year in
patients ventilated via a tracheostomy, while Oppenheimer
(19) describes survival of 85% in 29 consecutive patients,
suggesting that success depends largely on available
resources. Our purpose when LTMV is prescribed is not
only to increase survival but to improve the quality of life.
When the patients and their families have received informa-
tion and the treatment has been planned, this aim has been
achieved.
One must remember that patients with ALS on LTMV
will progressively lose mobility and will require help for all
the activities of daily life. This dependency on another
person stops many patients from returning home. More-
over, although non-invasive ventilation with a nasal mask
may be satisfactory initially, dependency on the ventilator
will gradually increase, and we have to consider tracheos-
tomy. None of the patients who started HMV with a nasal
mask later accepted tracheostomy as they were aware of the
evolution and outcome of their illness. In some countries
(20) there is legal support for withdrawing invasive mech-
anical ventilation when desired. The advantages of non-
invasive mechanical ventilation, which requires fewer

resources and provides the patients with the opportunity of
deciding how to continue mechanical ventilation as ALS
progresses, should be pointed out. Bach (21) reports the
use, in 2.5patients, of non-invasive ventilation which avoids
or delays the use of tracheostomy.
None of the patients who required admission to the ICU
and ventilation via tracheostomy had decided in favour of
ventilation before the AR1 episode. Moss et al. (11) also
report that only a minority of patients with ALS had
decided in favour of ventilation before the onset of respir-
atory insufficiency, and many patients had been intubated
in the emergency department. These decisions in critical
situations should be avoided in ALS patients. Hence, the
patient and family should be carefully informed so that the
treatment can be planned as the disease progresses. The
subjects should understand the benefits and limitations of
invasive and non-invasive mechanical ventilation and also
know the possible choice of palliative care.
Despite the difficulties, LTMV can benefit a minority of
patients with ALS, especially if they have support from
their families. In all cases,we must try to avoid emergency
ventilation, so it is fundamentally important to inform the
patient adequately so that decisions can be made before
AR1 occurs (22). It must be made clear to the patient that
LTMV will not change the natural history of ALS and that,
although they may need the ventilator only during the night
at first, dependency will gradually increase.
There are no firm indications for LTMV in patients with
ALS, but it is more likely that good results will be obtained
in patients who are well motivated, with slowly evolving
disease,with a family which understands the problem and is
willing to help, and where there are the necessaryresources
(23). Likewise, it is reasonable to propose LTMV for
patients whose first manifestation of neurological diseaseis
ARI, persistent hypercapnia or for those with incapacitat-
ing respiratory symptoms with little involvement of pcriph-
era1 muscles. Presently, LTMV is not a treatment for all
patients in very advanced stages of ALS. Finally, it is very
important to focus attention outside the hospital to allow
these patients to return home.
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