HOME TRACHEOTOMY MECHANICAL VENTILATION IN
AMYOTROPHIC LATERAL SCLEROSIS PATIENTS. CAUSES,
COMPLICATIONS AND ONE YEAR SURVIVAL
(Online data supplement)
Jesús Sancho, MD1,2
Emilio Servera, MD1,2,3
José Luis Díaz, PsyD1,2,3
Pilar Bañuls, MD1,2
Julio Marín, MD2,3
1
Respiratory Care Unit. Respiratory Medicine Department. Hospital Clínico
Universitario. Valencia. Spain.
2
Research Group for Respiratory Problems in Neuromuscular Diseases, Fundación para
la Investigación HCUV-INCLIVA.
3
Universitat Valencia.
Corresponding Author:
Jesús Sancho, MD.
Respiratory Care
Unit.
Respiratory Medicine
Department.
Hospital
Universitario.
Avd Blasco Ibañez 17.46010 Valencia, Spain.
cchinesta@eresmas.com
Keywords: amyotrophic lateral sclerosis, survival, respiratory failure, long-term
mechanical ventilation, tracheotomy.
Clínico
A) RESPIRATORY FUNCTION ASSESSMENT
Spirometry was performed (MS 2000; C. Schatzman, Madrid, Spain) using a
mouthpiece and a nose clip. FVC, FEV1, and FEV1/FVC were recorded in accordance
with European Respiratory Society guidelines and suggested reference values.S1
Maximum inspiratory pressure (PImax) and maximum expiratory pressure (PEmax)
were measured at the mouth (Electrometer 78.905A; Hewlett-Packard, Andover, MA)
while the cheek was held. PImax was performed close to residual volume and PEmax
was performed close to total lung capacity, and the pressures sustained for 1 second
were measured.S2 Three measurements with less than 5% variability were recorded, and
the highest value was used for the data analysis.
To avoid air leaks for those patients with severe bulbofacial weakness, an
oronasal mask (King Mask; King System, Noblesville, IN) was used.
Cough Capacity Assessment
Cough capacity was assessed with a pneumotachograph spirometer (MS 2000;
Schatzman; Madrid, Spain) and a sealed oronasal mask (Martin Vecino, Madrid, Spain)
as described in previous studies.S3 The highest peak cough flow (PCF) measurement
obtained from at least three maximal cough manoeuvres after a deep inspiration with
less than 5% variability was recorded. Maximum insufflation capacity (MIC) by air
stacking was achieved by the patient taking a deep breath, holding it, and then air
stacking consecutively delivered volumes of air from a manual resuscitator (Revivator;
Hersill, Madrid, Spain) through the oronasal mask to the maximum volume that could
be held with a closed glottis. The patient then exhaled the maximally held volume of air
into the pneumotachograph for volume measurement. Manually assisted PCF (PCFMIC)
was measured with a pneumotachograph connected to the mask and the manual
resuscitator in order to achieve MIC; a thoracoabdominal thrust was applied during the
cough effort. Mechanically assisted PCF (PCFMI-E) was measured with a
pneumotachograph connected to the mask and the MI-E device (Cough-Assist; JH
Emerson; Cambridge, MA). It was set at 40 cm H2O of insufflation pressure, -40 cm
H2O of exsufflation pressure with an insufflation/exsufflation ratio of 2/3, and a pause
of 1 s between each cycle. A thoracoabdominal thrust was applied during the
exsufflation cycle.
B) USE OF NONINVASIVE RESPIRATORY MUSCLE AIDS
1) At home
Noninvasive Ventilation
Indications for non-invasive ventilation (NIV) were as follows: signs and
symptoms of hypoventilation (mainly orthopnea, somnolence or headache), PaCO2 >
50 mm Hg, or nocturnal oxygen saturation < 88% during five consecutive minutes.S4
The NIV was delivered via a portable ventilator in volume-cycled assistcontrol mode (PV 501, Breas Medical, Mölndal, Sweden; PV 403, Breas Medical,
Mölndal, Sweden; AiroxHome2, Airox, Pau, France; Legendair, Airox, Pau, France).
All patients used NIV via oronasal masks (Mirage NIV, Resmed, Madrid),
lipseals (Tyco-Puritan Bennett, Carlsbad, CA), or nasal interfaces (Healthdyne,
Marietta, GA) during the night-time to optimize comfort and minimize air leaks. During
the daytime, NIV was delivered via a simple mouthpiece, a flexed mouthpiece (flexed
mouthpiece, Respironics, Murrysville, PA), or if ineffective, via a lipseal mouthpiece,
as needed.
Coughing aids
Patients with peak cough flows < 4.25 L/sec were trained in manually assisted
coughing, that is, coughing after air stacking,3 If manually assisted coughing was
ineffective, they were trained in using mechanically assisted coughing (MAC) with a
insufflator-exsufflator
(Cough-Assist
Philips-
Respironics
International,
Inc.,
Murrysville, PA). Sessions consisted of 8–10 cycles with insufflation-exsufflation
pressures + 40 cm H2O together with exsufflation-timed thoracoabdominal thrusts.
The sessions were repeated as scheduled and if needed, until the secretions
were expelled to maintain or return SpO2 to >95%.. (Oximax N-560, Nellcor, Tyco
Healthcare, Pleasanton, CA).S5
2) DURING ACUTE RESPIRATORY INFECTIONS
Noninvasive Ventilation
NIV was delivered via volume-cycled ventilation using the assist/control mode
(the same models as described for at home). If the patient was a vent-user previous to
the episode, changes in ventilation parameters were made as needed to relieve dyspnoea
during the acute illness. If the patient had not been using NIV at home, the ventilator
was initially adjusted in order to attain a tidal volume of about 15 mL/Kg, an
inspiratory/expiratory ratio of 1/1.2 or 1/1.5, a respiratory rate of 14 or 16 bpm and
inspiratory trigger sensitivity of –0.5 cmH2O. The ventilator settings were then
readjusted to a comfortable level for the patient in order to obtain SpO2 greater than
95% and PaCO2 lower than 45 mmHg. Supplemental oxygen was delivered when,
despite adequate ventilation with NIV, SpO2 < 95%.S6
NIV was applied through an oronasal mask (Mirage NV, Resmed, Madrid,
Spain) while sleeping, and while awake with a flexed mouthpiece (flexed mouthpiece,
Respironics, Murrysville, PA); if patients had oral leaks due to bulbar dysfunction, a
lipseal mouthpiece (Puritan Bennet, Carlsbad, CA) was used.
Coughing aids
Respiratory secretions were managed by MAC through an oronasal mask
(Martin Vecino, Madrid, Spain) at least twice every 8 hours and whenever SpO2
decreased below 95%, the peak inspiratory pressure during mechanical ventilation
increased or the patient had an increase in dyspnoea or a sensation of retained
secretions. It was sometimes used as frequently as every 5 – 10 minutes. Higher set
pressures were applied if conventional settings were ineffective due to decreased
pulmonary compliance or increased airway resistance.S7
C) POST TRACHEOTOMY HOME DISCHARGE PLAN
Before home discharge, a bronchoscopy was performed to check the correct
placement of the tracheotomy tube and the absence of associated complications.
All the patients were provided with the home equipment recommended by the
American Association of Respiratory CareS8: an additional ventilator, an Ambu bag, a
Cough-Assist (Cough-Assist Philips-Respironics International, Inc., Murrysville, PA),
conventional portable suction equipment, and a pulsioxymeter.
While the patients were still at the hospital, the family members (or the persons
named by them in their place) were trained in the handling of a Cough-Assist and a
portable pulsioxymeter, conventional aspiration of secretions, the replacement and
cleaning of cannulas, connections to the ventilator, peak inspiratory pressure, the
handling of an Ambu bag, the recognition of the ventilator and pulsioxymeter alarms,
the recognition of changes in the appearance and quantity of the secretions, and the
handling and cleaning of the humidifier. The patients were not transferred to their home
until the nurse supervising the training was satisfied that the carers were able to take on
that responsibility.
When patients were medically stable and the caregivers trained, they were
transferred to their homes. We had two kinds of resources for the provision of home
care: 1) the Unidades de Hospitalización Domiciliaria (Home Hospitalization Units, the
usual method in the public healthcare system of the Comunidad Valenciana in Spain)
which undertake treatment in the home (with a visit from a doctor once a day, two daily
visits from a nurse and an emergency service), but with the drawback that this care is
limited in time with regard to the solution of a particular problem; and also 2) care
funded by means of a grant from the Fundación para la Investigación de Hospital
Clínico de Valencia, with a nurse visiting the patients at their homes regularly (once a
fortnight) and a pulmonologist visiting every three months, in accordance with the
recommendations by the American College of Chest Physicians.S9 Moreover, the
caregivers could contact our Unit by telephone when necessary – the calls were taken by
the Unit’s medical staff on work days and the pulmonologist on duty at night and during
public holidays.
The Spanish national health system provides patients with equipment, medical
disposables and emergency home medical care free of charge. The local ALS patients’
association (ADELA Valencia) provides physical therapy treatment and equipment for
alternative communication.
The HTMV was delivered using the same portable ventilators and modes as for
NIV (see above) and ventilator parameters were adjusted for comfort, to maintain
PaCO2 near to 45 mm Hg, and to maintain SpO2 < 95%,
While using uncuffed tubes (Shiley Tracheotomy Tubes, Tyco Healthcare,
Pleasanton, CA, USA), ventilator volume was increased to compensate for any
increasing upper airway leaks which appeared. When ineffective to maintain normal
SpO2 and PaCO2, uncuffed tubes were changed to cuffed ones and the ventilator
parameters were set initially with the cuff deflated. When adequate ventilation was not
achieved in this manner, the cuff was inflated to a pressure of < 30 cm H2O (Hi-Lo,
Mallinckrodt, Hennef, Germany) and then ventilator parameters were adjusted.
Respiratory secretions were expelled using MAC through the tracheotomy
tube.S10 Eight cycles with set pressures of + 40 cm H2O, followed by superficial airway
suctioning with a conventional catheter, were applied when SpO2 decreased below 95%,
when the patient felt retained secretions, and when the peak inspiratory pressure during
mechanical ventilation increased.
REFERENCES
S1.- Quanjer PH, Tammeling GJ, Cotes JE, Pedersen OF, Peslin R, Yernault JC. Lung
volumes and forced ventilatory flows: report of Working Party “Standardization of
Lung Function Test.” Eur Respir J 1993;6(Suppl 16):5–40.
S2.- Black LF, Hyatt RE. Maximal respiratory pressures: normal values and relationship
to age and sex. Am Rev Respir Dis 1969;99:696–702.
S3.- Sancho J, Servera E, Díaz, Marín J. Predictors of ineffective cough during a chest
infection in patients with stable amyotrophic lateral sclerosis. Am J Respir Crit Care
Med 2007;175:1266-71.
S4.-Clinical indications for noninvasive positive pressure ventilation in chronic
respiratory failure due to restrictive lung disease, COPD and nocturnal
hypoventilation –A Consensus Conference Report. Chest 1999;116:521-34.
S5.-Bach JR, Bianchi C, Auifero E. Oximetry and indications fot tracheostomy for
amyotrophic lateral sclerosis. Chest 2004;126:1502-7.
S6.- Servera E, Sancho J, Zafra MJ, Catala A, Vergara P, Marín J. Alternatives to
endotracheal intubation in patients with neuromuscular diseases. Am J Phys Med
Rehabil 2005;84:851-7.
S7.- Sancho J, Servera E, Marín J, Vergara P, Belda FJ, Bach JR. Effect of lung
mechanics on mechanically assisted flows and volumes. Am J Phys Med Rehabil
2004;83:698-703.
S8.- Kohorts J, Blakely P, Dockter C, Pruit W. AARC Clinical Practice guideline:
Long-term invasive mechanical ventilation in home-2007 revision & update. Respir
Care 2007;52:1056-62.
S9.-Make BJ, Hill NS, Goldberg AI, Bach JR, Criner GJ, Dunne PE, et al. Mechanical
Ventilation beyond the intensive care unit: Report of a Consensus Conference of the
American College Chest Physicians. Chest 1998;113:289s-344s.
S10.- Sancho J, Servera E, Vergara P, Marín J. Mechanical Insufflation-Exsufflation vs
suctioning via tracheostomy tube for patients with amyotrophic lateral sclerosis.A
pilot study. Am J Phys Med Rehabil 2003;82:750-3.