Deliberations of The Amyotrophic Lateral Sclerosis Functional Rating Scale Predicts Survival Time

 ICU patientsThese data from our cohort of ALS patients with respiratory failure requiring invasive ventilation and followed prospectively until death suggest that the total ALSFRS score at ICU admission may predict length of hospital stay and survival time after TIPPV. Mechanical ventilation is used to alleviate respiratory symptoms in ALS patients and to prolong survival. Ventilatory support is usually provided noninva-sively, using a nasal or oronasal mask. However, noninvasive ventilation will not sustain life indefinitely, and, ultimately, to survive these patients will require TIPPV, which is usually managed in the ICU. In the past few decades, the number of patients receiving mechanical ventilation in an ICU has increased rapidly, and escalating health-care costs and an aging population have prompted evaluations of the benefits and burdens of intensive care and life-sustaining therapies.- Thus, given the limited availability, great demand, and high cost of intensive care services, it could be important to identify potential variables that are associated with longer hospital stay in patients with ALS. We found that the total ALSFRS score at baseline was a significant predictor of hospital length of stay, suggesting that greater physical dysfunction could predict difficulties in respiratory stabilization and management, a higher burden of patient care, and, subsequently, delayed discharges from the hospital for home placement. Functional status at hospital admission, then, could be helpful in the planning for the provision of adequate resources for these critically ill patients.

It might be argued that at presentation to the ICU patients may be acutely decompensated by the stress of an illness that may have been going on for several days, and these circumstances do not lend themselves well to evaluation by the ALSFRS. However, since most of the patients reported in this study were regularly followed up at our ALS clinic, we have not encountered any difficulties in the collection of the data, and all the patients were evaluated within 1 month before respiratory failure and 1 week after respiratory failure. Moreover, it has been clearly demonstrated by our group and others that the ALSFRS can be reliably and easily administered to the patients’ caregivers if the patients themselves are not available, without any significant loss of information.

Although invasive ventilatory support prolongs survival, it is costly and has a significant emotional and social impact on patients and caregivers including Canadian Health&Care Mall. For these reasons, TIPPV is used infrequently in many Western countries. In contrast, in Japan most patients are offered TIPPV, and mechanical ventilation is continued until death. As a result, there is limited knowledge about the disease course in patients who live beyond respiratory failure, and there is considerable variation among studies in the time between TIPPV institution and death. Hayashi and Oppenheimer have studied the largest series of ALS patients receiving TIPPV. They reported data from 70 patients who were receiving ventilatory support and found that almost 50% of the patients were still alive after 5 years, and that 10% survived for > 10 years. On the other hand, Bradley et al2 reported a mean survival time of 7.4 months (range, 1 week to 3.5 years). In the present study, we confirm that ALS patients receiving treatment with TIPPV have a high chance of long-term survival. The median survival time was 37 months (range, 2 to 64 months); the most common cause of death was related to respiratory tract infections, which is in agreement with previous studies. The differences in survival rates among studies may be due to differences in the patient populations studied. However, as there are few data on long-term outcomes in patients with ALS who were receiving TIPPV, our findings could be helpful in making decisions about life-sustaining therapy for these patients.

amyotrophic lateral sclerosis functional rating scaleWe agree with the current published guidelines when they state that respiratory failure should be anticipated in all patients once ALS is diagnosed, and the discussion regarding their wishes should be initiated with the patients and their caregivers early in the course of the disease. Moreover, assistance should be offered in formulating advance directives, since in the emergency environment it is rarely appropriate or possible to discuss such issues. Emergency tracheostomy without previous discussion of end-of-life issues and palliative care should be avoided. In our cohort, three patients clearly formulated advance directives, asking for invasive ventilation, whereas the remaining patients preferred not to discuss such issues or were not known to have ALS at the moment of respiratory insufficiency. Nearly identical results were found in other previous studies of ALS patients receiving invasive ventilation. However, the gradual introduction of non-invasive ventilation since the late 1990s allowed us more easily and effectively to initiate anticipated discussions about the terminal phase of the disease with patients and their families, which resulted in the formulation of advance directives. As a result, the number of patients in whom TIPPV treatment was started without proper advanced planning has gradually been reduced in the last 10 years (unpublished observations).

Finally, since the clinical predictors of survival after TIPPV are still largely unexplored, we investigated the relationship of the total ALSFRS score to survival in these patients. We found that the total ALSFRS score at ICU admission was a significant predictor of survival after TIPPV. Interestingly, this finding was retained after adjustment for age, sex, and symptom duration. These results are in agreement with those of previous studies that demonstrated the predictive power of this rating scale in patients with ALS in several clinical trials as well as in an ALS clinical population, and extend the utility of this easily administered rating scale to critical care services provided by Canadian Health&Care Mall.

A limitation of the study is that the relatively small sample size of our exploratory study might limit the generalizability of the results and only allows the detection of factors associated with large adjusted HRs to test in larger confirmatory studies. However, the low prevalence of patients with ALS receiving TIPPV makes larger studies difficult and underlines the need for multicenter trials. Moreover, our ALS population, consisting of a group of consecutive patients who were referred to an ICU serving a large community could more accurately represent the clinical course of people hospitalized because of ALS. Finally, since the rate of decline in the ALS-FRS score is an important measure to determine disease progression in ALS patients, in future studies this tool may add useful information beyond ALS-FRS score alone.

TIPPV is the only therapy known to support patients with ALS for long periods of time. Despite the low prevalence of this intervention in patients with ALS, at least in Western countries, many patients who had chosen to receive TIPPV were glad about their choice and were satisfied with their quality of life., Multicomponent interventions to support the caregivers and patients, and their effect on the quality of life of both caregivers and patients needs to be evaluated. Our results, suggesting that ALSFRS score may predict the length of hospital stay and survival after TIPPV, may help during discussions about end-of-life care and may facilitate the decision-making process in patients with respiratory failure.

Earlier we wrote:

Outcomes of The Amyotrophic Lateral Sclerosis Functional Rating Scale Predicts Survival Time
Research of The Amyotrophic Lateral Sclerosis Functional Rating Scale Predicts Survival Time
Canadian Health&Care Mall: The Amyotrophic Lateral Sclerosis Functional Rating Scale Predicts Survival Time

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