C28 PATHOLOGICAL ASPECTS OF LONG‐TERM SURVIVAL IN ALS PATIENTS ON RESPIRATORS
Takahashi H
Brain Research Institute, Niigata University, Japan
E‐mail address for correspondence: [email protected]
Amyotrophic lateral sclerosis (ALS) is a progressive, in principle sporadic, neurodegenerative disease of unknown cause affecting adults. Pathologically, loss of lower and upper motor neurons, and degeneration of the corticospinal tract are typical features. Most of the affected patients die within a few years due to respiratory failure.
There has long been a question of whether only the motor neuron system undergoes selective degeneration in ALS. The use of assisted ventilation has meant that respiratory failure is no longer necessarily the end‐point of the disease. For example, after the introduction of respiratory support, it has been revealed that in the spinal cord, neurons in Clarke's column and the intermediolateral nucleus are involved primarily in the disease process, and in general tend to disappear with time. The difference in the rate of disappearance between these sensory or autonomic neurons and anterior horn cells represents the rate of degeneration. Therefore, ALS is not simply a motor neuron disease.
What does long‐term survival bring to ALS patients on respirators? It has been reported that the entire manifestation of ALS is represented as a totally locked‐in state with widespread degeneration, including the motor neuron system. It has also been reported that such lesions are not always a feature in long‐surviving ALS patients on respirators. At present, it is certain that at least two pathologic phenotypes (the widespread multisystem form and the non‐widespread, rather classical form) are recognizable.
Is artificial respiratory support (ARS) actually useful for ALS patients? In a previous study, we examined 102 autopsy cases of sporadic ALS that occurred between 1962 and 2000 (Brain Research Institute, University of Niigata, Japan). Among the patients, 28 (20 male, 8 female) were given ARS (duration, 5–156 months), and their disease duration (median, 53.5 months) was significantly longer than that (median, 25.0 months) of patients without ARS (p = 0.0002). With regard to cognitive function, mild dementia was observed in only one patient who received ARS for 114 months; there was no significant correlation between ARS and dementia. In addition, routine pathological examination revealed no apparent expansion of degenerative lesion distribution in the subgroup of cases with ARS. Thus, ARS appears to be a useful choice of conservative treatment when ALS patients wish to live long beyond the point of respiratory failure.
The Japan ALS Association has contributed greatly to creating a positive approach to living with the disease. However, in Japan, there is now an ongoing debate about whether ALS patients on respirators should be allowed to choose for themselves to continue receiving such support. Pathologic studies suggest that the cognitive function of most ALS patients on respirators remains unaffected for a long period, and that such patients are capable of changing their mind about treatment while receiving ARS.
C29 THE LONG‐TERM COMMUNICATION STRATEGY IN PATIENTS WITH TPPV THROUGHOUT THE TOTAL COURSE OF ALS
Hayashi H
Tokyo Metropolitan Neurological Hospital, Tokyo, Japan
E‐mail address for correspondence: [email protected]
This presentation will discuss the long‐term communication strategy based on 100 ALS patients with tracheostomy positive pressure ventilation (TPPV) experienced in Tokyo Metropolitan Neurological Hospital.
In Japan, the number of ALS patients with TPPV is more than 30%, and recently the number of patients at home on ventilation has increased. Although respiratory failure in ALS has long been considered the end‐point in ALS, the recent clinico‐pathological evidence from the patients with TPPV showed that it is not the end‐point, but a part of ALS. In general, the respiratory motor group might certainly be the most vulnerable among the five in the voluntary motor system, i.e. extremity, pontine, medullar, respiratory and external ocular. In addition to the voluntary motor system however, the emotional and autonomic systems also might be involved in ALS, and some patients might develop severely impaired communication, i.e. totally locked‐in state (TLS) and minimal communication state (MCS). The period of the total course of ALS has changed from onset to respiratory failure, which has been known as Charcot's ALS and we have called ‘the previous view of ALS’, to a period from onset to involvement of all efferent motor systems, which we have called ‘the new view of ALS’ and includes the former.
In considering communication strategy throughout the total course of ALS, patients with TPPV should now be included and the physicians should take ‘the new view of ALS’ in caring for them. Traditional verbal communication may be not effective, and different varied communication needs should be developed during the course of increasing impairments of ALS. Two points of view in the communication strategy should be considered.
The first is physical, extrinsic communication using apparatus, i.e. the development of communication‐assistance devices ‘just in time’ for their essential living such as a switch for nurse‐call, etc. The development of these will require good knowledge of the characteristic features and trends of aggravating motor impairments throughout the total course of ALS.
The second is mind‐intrinsic communication not using apparatus, i.e. the allocation of decisional authority in the physician‐patient‐family relationship, and the quality of communication supporting informed decision‐making. In Japanese sociocultural tradition, there remains a tendency that the patient's family might defer to and trust in the professional expertise and role of the physician, and the patient is perceived as outside the decision‐making process, contrary to the individualistic notion at the heart of autonomy in the West. In Japan, the ample opportunity for physician‐patient‐family dialogue in the ‘fiduciary relationship (Shinnrai‐Kankei)’ between the physician and the patient's family based on ‘the new view of ALS’ might have an essential supporting role for patients with TPPV to enhance and foster the enlargement of life, QOL and coping with the inexorable decline in the future.