Neurogenic Respiratory Failure



Fig. 17.1
Number of patients in Germany in need of ventilation at the time of hospital discharge





17.2 Epidemiology of Ventilated Patients


In a registry of all German-speaking countries, data on long-term follow-up concerning complications, life expectancy, mortality and quality of life are available for the last 25 years, which allows provision of valid information about patient characteristics, treatments and trends in the population of ventilated SCI patients [4].

In Germany the incidence and the age of ventilated patients have increased dramatically over the last decade [4] (Fig. 17.1). Over the last 13 years, the number of these patients has quadrupled and nearly ten percent of all SCI patients need temporary or permanent ventilation as part of their initial treatment in the hospital [4]. Older patients often have multiple comorbidities, which prolong the time of primary rehabilitation.

In the ventilated patient population, the main causes in case of traumatic SCI are still traffic accidents closely followed by falls. In non-traumatic SCI inflammation, degenerative processes and vessel-related complications are the leading causes (Table 17.1). The most frequent neurological level of lesion is segment C2, while most patients are motor complete (ASIA impairment scale (AIS) A and B in total 87 %) (Tables 17.2 and 17.3). Concerning gender (male = 77.3 %; female = 22.7 %) and mean age at the time of injury (mean age = 43.5 years), the characteristics between non-ventilated and ventilated patients do not differ significantly. Nowadays, patients have a higher age (1997, mean age = 35.4 years; 2014, mean age = 58.2 years) at the time point of injury due to demographic change in industrialised countries.


Table 17.1
Causes of SCI in ventilated patients























































Cause of SCI

Percentage (number of cases)

Traumatic:

84.6 % (n = 93)

 Traffic

 41.8 % (n = 46)

 Fall

 15.5 % (n = 17)

 Work

 8.2 % (n = 9)

 Diving

 5.5 % (n = 6)

 Suicide attempt

 2.7 % (n = 3)

 Leisure/sports

 2.7 % (n = 3)

 Crime

 1.8 % (n = 2)

 Other

 6.4 % (n = 7)

Non-traumatic

15.4 % (n = 17)

 Inflammation

 6.4 % (n = 7)

 Degenerative

 3.6 % (n = 4)

 Vessel related

 2.7 % (n = 3)

 Tumour

 1.8 % (n = 2)

 Other

 0.9 % (n = 1)



Table 17.2
Level of lesion in a subpopulation of N =110

























Neurological level of injury

Percentage (number of cases)

C0

15.5 % (n =17)

C1

0.9 % (n =1)

C2

55.4 % (n =61)

C3

22.7 % (n =25)

C4

5.5 % (n =6)



Table 17.3
Distribution of the lesion severity classified by the ASIA impairment scale (AIS) in a subpopulation of N = 110






















ASIA impairment scale (AIS)

Percentage (number of cases)

A

78.2 % (n = 86)

B

9.1 % (n = 10)

C

10.9 % (n = 12)

D

1.8 % (n = 2)


17.3 Pathophysiology


Different levels of an injury of the spinal cord results in different functional impairment patterns of the diaphragm, the intercostal muscles, the accessory respiratory muscles and the abdominal muscles [5] (Fig. 17.2).

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Fig. 17.2
Overview of the affected inspiratory and expiratory muscles in relation to the neurological level of lesion

Patients with an insufficient capability for coughing often have mucus retention. This increases the risk for atelectasis and pulmonary infections [6] and ultimately leads to a significantly higher mortality [7]. High tetraplegia is usually associated with the severest impairment of the respiratory function.

The paralysis of almost all respiratory muscles including the diaphragm leads to a significant loss of vital capacity and results in the dependency on partial or complete mechanical ventilation. In general, the rule applies that the more cranial the level of lesion is located, the more the respiratory pump is affected. Several factors contribute to this including:


  1. 1.


    The decreased strength of respiratory muscles

     

  2. 2.


    A reduced compliance of lungs and thoracic wall

     

  3. 3.


    A chronic central hypoventilation

     

  4. 4.


    Changes of the patency and reactivity of the airways

     

  5. 5.


    Dyssynergies concerning the muscles of the thorax and abdomen

     


Decreased Strength of Respiratory Muscles

As a result of the weakness of inspiratory muscles, the vital capacity (VC), the tidal volume (TV) and the forced one-second capacity (FVC1) decrease in patients with high cervical SCI [8]. While patients try to maintain a sufficient minute volume, they automatically increase their respiratory rate.

The reduced strength of expiratory muscles leads to a decrease of the end-expiratory reserve volume and, as a consequence, to a rise of the residual capacity. This in turn decreases the vital capacity [9, 10]. Another result of the weakness of expiratory muscles is a limited ability to cough with the associated reduction in peak cough flow [9, 11]. With peak cough flow less than 270 l/min, efficient coughing is not possible [12, 13]. The measurement of peak cough flow with a peak-flow meter represents a simple and inexpensive method to objectively assess the ability for patients for sufficient coughing, which can be easily implemented into the clinical routine.


Reduced Compliance of Lungs and Thoracic Wall

The lung and thorax compliance is worsening immediately after the lesion in tetraplegic patients caused by decrease of the vital capacity and changes of the surfactants due to respiration with low tidal volumes [14, 15]. Additionally, the reduced stability of the thorax due to partially or completely paralysed intercostal muscles leads to a paradoxical inspiration, which means that the thorax flattens during the inspiration [16, 17]. Stiffening of the thorax and potential spasticity of the intercostal muscles contribute to the decrease of compliance [18].


Chronic Central Hypoventilation

The central control of respiration is affected in tetraplegic patients. The underlying mechanisms have not been entirely investigated. As far as we know, breathing effort, as a response to a hypercapnia, is decreased and correlates with the blood pressure fluctuations caused by the spinal lesion. Especially at night, breathing and sleep disorders may become more apparent and, in case of complementary drug therapy, partially enhanced by side effects of centrally acting medications (such as pain and antispastic medications) [19, 20].


Changes in the Patency and Reactivity of the Airways

A bronchial hyperreactivity often occurs after cervical SCI and is significantly associated with decreased airway diameter and patency [21]. Hypoactivity of the disrupted sympathetic airway innervation, in addition to a parasympathetic hyperactivity, is assumed as a cause not only for the hyperreactivity but also for the increased production of bronchial secretions and bronchoconstriction [21, 22].


Dyssynergies of Muscles of the Thorax and Abdomen

The interaction between abdominal and thoracic muscles is also impaired after high cervical SCI. The increased compliance of the abdomen as a consequence of the loss of voluntary innervation of abdominal muscles results in a caudal shift of the diaphragm. The weight of the intra-abdominal organs additionally contributes to a ventral and caudal displacement. Therefore, the vertical diaphragmatic effectiveness decreases, and patient transfers such as mobilisation in a wheelchair lead to a decrease of the tidal volume and accordingly, to a faster occurrence of dyspnoea [23, 24]. Thus, static and dynamic lung volumes are reduced as a direct consequence of the paralysis.

A long-term degradation of lung function is often reported [14, 2528]. Additionally smoking, persistent wheezing, being overweight and the level of the lesion also have a negative impact both on the tidal volume in terms of significant reduction and lung function according to poor oxygenation [25, 26, 29].


17.4 Artificial Ventilation in the Acute Phase



17.4.1 Ventilation Modes


A limited or completely lost respiratory function can be adopted or assisted with an artificial respirator. Modern respirators offer at least two basic modes of operation, which are the mandatory and spontaneous ventilation mode. When in mandatory ventilation mode, the respirator controls and performs the breathing work completely or, in case of minimal residual respiratory function, in complemented mode. While working in complemented mode, important parameters (inspiration pressure, tidal volume and ventilation frequency) are monitored and, if necessary, adjusted by the respirator at any time.

The spontaneous ventilation mode allows the patient to either completely breathe on his own or to be supported by the respirator [30]. The two most important parameters in artificial ventilation are volume and pressure. With adaption of these two parameters, literally every available ventilation mode can be implemented [31]. Just as the discussion over the optimal ventilation mode continues, so does the debate over the optimal control variable. Volume-controlled ventilation (VCV) offers the safety of a preset tidal volume and minute ventilation but requires the clinician to appropriately set the inspiratory flow, flow waveform and inspiratory time. During VCV, airway pressure increases in response to reduced compliance or increased resistance and may increase the risk of ventilator-induced lung injuries. Pressure-controlled ventilation (PCV), by design, limits the maximum airway pressure delivered to the lung but may result in variable tidal and minute volume. According to current recommendations, the target tidal volume should range between 6 and 8 ml/kg body weight in patients with a normal body-mass index (BMI) [32].

Most studies comparing the effects of VCV and PCV were not well controlled or designed and offer little to our understanding of when and how to use each control variable in invasive ventilation [31]. Nevertheless, in SCI patients PCV seems to be more advantageous for prevention of atelectasis and for potential compensation of volume loss (e.g. phonation while ventilated with an unblocked cannula).

When choosing the ventilation mode for an individual patient, the clinicians have to keep in mind that a spinal cord-injured patient suffers from an impairment of the respiratory pump without a primary pulmonary disease. Patients with SCI and associated severe pulmonary diseases have to be treated additionally according to pneumological respiratory guidelines [31] including carefully selected medications and adapted ventilation modes.


17.4.2 Tidal Volumes


The aims of artificially assisted ventilation in persons with SCI are:



  • Sufficient oxygenation with subjective well-being


  • Prevention of forming atelectasis


  • Enabling of phonation during ventilation

There are a few reports published about tidal volumes between 900 and 1000 ml (sometimes even higher) applied in patients with tetraplegia requiring invasive ventilation [33]. In case of a normal BMI, 10–15 ml/kg ideal body weight is recommended during the acute phase [34]. In the presence of atelectasis, a slow increase of 20 ml/kg ideal body weight with a maximal pressure of 30 cmH2O is described in order to minimise the risk of a barotrauma [32, 35]. It was shown in a 10-year observational study that the risk for developing an atelectasis increases with lower tidal volumes [33]. As a consequence, it is recommended to apply higher tidal volumes for successful treatment of atelectasis while reducing the breathing frequency to avoid chronical hyperventilation [33].

On the basis of clinical experience with long-term ventilated tetraplegic patients with inserted unblocked tracheal cannula, six main advantages of using higher tidal volumes were stated [36]:



  • Improvement of ability to speak


  • Prevention of atelectasis


  • Enablement of alternating ventilation volumes without developing hypoxemia


  • Maintenance of pulmonary compliance


  • Suppression of residual respiratory muscles activity due to low paCO2 values


  • Prevention of subjective dyspnoea during ventilation by achieving normal blood gas values

General observations of long-term ventilation with high tidal volumes show that the respiratory alkalosis associated with the hypocapnia can be completely renally compensated without pathological pH values. Theoretically possible negative effects of hypocapnia, e.g. reduced cerebral blood flow caused by vasoconstriction and thereby increased susceptibility for seizures, were not observed in the long-term course [37].

In principle, high tidal volumes with hyperventilation also offer the risk of potassium loss and increased osteoporosis by chronic hypocapnia [38]. The former has to be verified regularly and, if necessary, applied. Concerning osteoporosis it should be noted that tetraplegia by itself is leading to an increased osteoporosis in the long-term course, to which many factors beside the hypocapnia contribute.

In conclusion, according to the literature, the following recommendations can be given with regard to invasive long-term ventilation of lung-healthy persons with tetraplegia:


  1. 1.


    Use of pressure-controlled ventilation modes with relatively high tidal volumes and reduced breathing frequency starting with 10–12 ml/kg ideal body weight in normal BMI depending on the clinical course (e.g. if atelectasis develop) can be applied. A maximal inspiratory ventilation pressure of 30 cmH2O should not be exceeded. If necessary, tidal volumes of 15–20 ml/kg ideal body weight may be used.

     

  2. 2.


    Although general recommendations for parameters of ventilation such as inspiratory pressure or ventilation frequency exist, there is still the need to individually adapt these parameters to every patient. These adaptations may arise from thermal and circulatory dysregulations or due to chances in muscular or bronchial spasticity. They should be based on the results of a volumetric and – also in the non-clinical follow-up – capnometric assessment.

     

  3. 3.


    Using a non-blocked or non-cuffed tracheal cannula on an individual basis for as long as possible to improves phonation and prevents of tracheal ulcers.

     


17.5 Rehabilitation of Ventilated Patients During the Acute Phase



17.5.1 Communication and Mobilisation


Invasively ventilated patients with SCI should be provided with the possibility to speak during the very acute phase of injury during the stay in the intensive care (ICU) and intermediate care (IMCU) units. Apart from the advantage of better communication and the associated higher quality of life, the opportunity to speak creates a higher level of laryngeal awareness. This may help to improve oral ingestion and to prevent episodes of aspiration [39].

The inability to speak poses a considerable restriction on participating in everyday life for patients which are in need of permanent or partial ventilation [40]. If only minor or moderate swallowing disorders are present, loud and clear phonation during invasive ventilation is feasible and can be learned [4143]. Phonation always depends on airflow through the glottis enabling the vocal cords to vibrate and produce tones. Unrestricted patients normally phonate during expiration [44]. In contrast, phonation in ventilated patients mainly occurs during inspiration, because the required airflow from the ventilator is generated during the inspiration, while expiration mainly proceeds passively [45].

Acute and long-term ventilated patients have a high risk of developing pneumonia [46], decubital ulcers [47] and deep vein thromboses [48]. Therefore, mobilisation of those patients is recommended as early as possible, even in IC and IMC units [4951]. To achieve this early mobilisation, appropriate equipment and personal resources must be present in the form of:



  • Physicians and therapists experienced in SCI and ventilation


  • ICU/IMCU monitoring including capno- and spirometry


  • Provision of patient-adapted technical assistive devices (e.g. respirators, wheelchairs, lifter systems)


17.5.2 Tracheostomy


In the context of acute care of tetraplegic patients, a tracheotomy is often required to enable sufficient respiration and secretion management. Tracheostomy also may be needed in case of prolonged ventilator dependency if typical problems associated with the use of oropharyngeal or nasopharyngeal tubes appear. A tracheotomy can be done either by dilatation percutaneously (PT) or surgically as an open tracheostomy (OT). Further advantages of a tracheostomy are the early reduction or suspension of analgosedation resulting in an alert and compliant patient who is able to participate in a SCI-specific therapy programme, consisting of mobilisation into the wheelchair, phonation, food intake during ventilation and weaning.

In general, a tracheotomy is recommended in tetraplegic patients with:



  • Motor complete tetraplegia (AIS A and B) [52]


  • Vital capacity ≤500 ml


  • Injury Severity Score (ISS) >32


  • PaO2/FIO2-ratio <300 for 3 days after initiation of ventilation [53]

Studies also demonstrated that an early tracheotomy (<10 days after the onset of paralysis) shortens both the duration of stay in an intensive care unit and the overall ventilation period (assuming that weaning was successful) [54]. With regard to complication rates of both techniques (PT and OT), there is inconsistent evidence [55, 56]. In the case of a permanently invasively ventilated patient, the current German Respiratory Society guideline “non-invasive and invasive ventilation” recommends stable medical care for outpatient ventilation and therefore the placement of an epithelial open tracheotomy [57]. Regardless of the used technique, PT can only be accepted for a short and successful weaning process in the ICU/IMCU or exceptionally in home mechanical ventilation due to the tendency to shrink and the risk of malposition of the cannula [57].


17.6 Weaning


Even for specialised centres, weaning of ventilated tetraplegic patients is a challenge for many reasons especially because of recurrent pulmonary infections [58]. Besides this, vegetative dysfunctions such as hypotonia, bradycardia, autonomic dysreflexia or hypothermia represent additional complications during the weaning process [59, 60]. Weaning in SCI patients is often prolonged and interrupted [61, 62]; therefore, the majority of the patients are ventilated via tracheotomy. The length of this process ranges between 40 and 232 days. The rate of weaning failure is consistently reported to be in the range of 30 % [62].


17.6.1 Pathophysiology


The innervation and strength of the diaphragm muscle determines the vital capacity of the lungs [63, 64]. The higher the vital capacity, the better the weaning prognosis [65]. Therefore the weaning process should not be initiated when the vital capacity of a lung-healthy patient is below 1000 ml [66]. The aim during the weaning process is to systematically train the diaphragm muscle while avoiding excessive fatigue of the muscle. Disregard may lead to an extension of the weaning period or failure of the weaning process [67].

The diaphragm muscle is prone to a fast conversion from slow-fatiguing type I to fast-fatiguing type IIb fibres after paralysis. The training during the weaning reverts this paralysis-induced fibre transformation and leads to more slow-fatiguing muscle fibres [6871].


17.6.2 Confounding Factors


From the clinical experience, weaning should not be started or must be interrupted when at least one of the following confounding factors is present:



  • Pneumonia


  • Septicaemia


  • Constant fever >38.5 °C


  • Complete paralysis of the diaphragm muscle


  • Vital capacity <1000 ml


  • Relevant autonomic dysreflexia


  • Severe spasticity of relevant respiratory muscles


  • Serious pressure sores


  • Constant heart rate >140 bpm


  • Constant breathing rate >35/min


  • Metabolic acidosis


  • Inadequate mental status


  • Anaesthesia


17.6.3 Execution of the Weaning Process


Weaning should be started in a lying or reclined position (bed or wheelchair) during daytime (e.g. 8 a.m.–8 p.m.) in the ICU under the control of certain vital parameters. This includes breathing frequency, breathing (tidal) volume and values of carbon dioxide (capnography). During night-time recovery of all respiratory muscles should be ensured by correct settings of mandatory ventilation modes of the external respirator [66].

During the daytime, every hour should consist of a spontaneous breathing (training) part and a ventilator (recovery) part. In clearly defined steps of 5–10 min, the training sessions (up to 12) are slowly increased day by day until the patient is breathing spontaneously 12 h without any ventilator support. If the patient is stable during daytime, the night-time weaning can be initiated with increasing periods of spontaneous breathing, e.g. 1 hour per night.

The mentioned vital parameters including spirometry should be monitored and the weaning process interrupted, if confounding factors evolve. The application of a standardised protocol is recommended [15, 72, 73].

The supervision and adaption of the weaning process requires a highly trained staff to achieve a successful outcome. This knowledge is normally only present in specialised SCI centres. Therefore, the rapid transfer of patients in need of artificial ventilation from the ICU to SCI centres is recommended by SCI societies worldwide to ensure an adequate weaning procedure.


17.7 Mucus and Secretion Management


The liquefaction and loosening of bronchial secretions improves effective ventilation and the weaning regime. It avoids atelectasis and pneumonia in temporary as well as in long-term ventilation [7476].

Efficient mucus and secretion management is achieved by:



  • Hyperinflation of the lungs up to the maximum capacity before the evacuation of mucus


  • Specific positioning of the upper body (e.g. 135° or kneeling position; Figs. 17.3 and 17.4)


  • Assisted coughing (Figs. 17.5, 17.6 and 17.7)

    A323639_1_En_17_Fig3_HTML.gif


    Fig. 17.3
    Manual secretolysis bedside by using a vibrax massage device


    A323639_1_En_17_Fig4_HTML.jpg


    Fig. 17.4
    Manual secretolysis during physiotherapy by using a vibrax massage device


    A323639_1_En_17_Fig5_HTML.gif


    Fig. 17.5
    Chest compression made by one person


  • Deep inspiration by means of air stacking or glossopharyngeal respiration


  • Insufflation with artificial respiration bag, an intermittent positive pressure volume device (IPPV) or with in-exsufflators (e.g. Cough assist®, Philips GmbH Respironics, Herrsching, Germany; Pegaso®, Dima Italia S.r.l. Bologna, Italy; Nippy-Clearway®, B&D Electromedical, Warwickshire, England), followed by manual cough assistance by means of dorso-cranial compression on the upper abdomen


  • Inhalation with hyperosmolar saline


  • Extra- or intracorporal chest vibration (e.g. Vibrax®, HEBRU-THERAPIEGERÄTE GmbH, Igersheim, Germany; Acapella®, MPV MEDICAL GmbH, Putzbrunn, Germany; Cornett®, R. Cegla GmbH & Co. KG, Montabaur, Germany; water bottle) (Figs. 17.3, 17.4, and 17.6)

    A323639_1_En_17_Fig6_HTML.gif


    Fig. 17.6
    Chest compression made by two persons in combination with an intermittent positive pressure-supported inspiration


    A323639_1_En_17_Fig7_HTML.gif


    Fig. 17.7
    Chest compression made by two persons during exhalation

Before initiating outpatient ventilation, the patient and the caregivers must be instructed and trained in the procedures of efficient secretion management. Knowledge about the value of maximum inspiratory capacity and maximum peak cough flow is obligatory. Manually assisted coughing in combination with hyperinflation of the lungs should be performed in all patients with peak cough flow <270 l/min [7779]. Cough assisting machines should be used in addition when hyperinflation and supported coughing fails [8082]. Air-warming and air-moistening should be used regularly in invasively ventilated patients. Active (breathing gas humidifier) and passive (heat and moisture exchange filter) procedures are both reliable and convenient ways for dissolving mucus.


17.8 Long-Term Ventilation in the Chronic Phase


Objective information about the pattern of use of artificial ventilation in the chronic stage after SCI is rare. The following data from Germany may serve as a reference for western industrial countries [4] (Table 17.4):
Aug 17, 2017 | Posted by in PHYSICAL MEDICINE & REHABILITATION | Comments Off on Neurogenic Respiratory Failure

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