‘Strong Fighters,’ ALS Patients with Locked-in Syndrome Report Decent Quality of Life in Small Study

‘Strong Fighters,’ ALS Patients with Locked-in Syndrome Report Decent Quality of Life in Small Study

Amyotrophic lateral sclerosis (ALS) patients with locked-in syndrome reported having a satisfactory quality of life and low levels of depression, in spite of their near-complete disability, in a small but recent study.

The results of the questionaire-based study were presented by its lead author, Dorothée Lulé, at the 28th International Symposium on MND/ALS, held in Boston from Dec. 7–10. She described the plurality of 25 locked-in ALS patients surveyed as “strong fighters” who “mostly have a strong wish for life.”

A rare neurological disorder associated with some progressive cases of ALS, locked-in syndrome refers to patients who no longer have any voluntary control of their body’s muscles, with the exception of eye movement. As a result, independence and communication are severely impaired.

While locked-in syndrome is often associated with a complete lack of quality of life, researchers in the study, “ALS patients with locked-in syndrome: quality of life, depression and medical decision making,” sought to poll ALS patients with the disorder directly and compile measurable results on quality of life, depression, well-being, and preferences to end life.

They aimed to better understand the effects of locked-in syndrome on health management and life enhancing/sustaining treatments.

Although there are major unmet needs in treating ALS, therapeutic options such Riluzole (rilutek) — which helps to prolong survival in ALS patients – as well as life-sustaining treatments, such an invasive and non-invasive ventilation, do exist. This study aimed to better understand whether these treatment options would be deemed worthwhile by patients with severe disability.

Researchers interviewed 25 locked-in syndrome patients using eye control technology that allowed them to respond to an in-depth questionnaire on quality of life.

Interview sessions, which took three to four hours, included a range of standard quality of life (QoL), depressiveness, and physical function tests, such as the ALSFRS-R, giving the researchers an opportunity to determine attitudes toward hastened death (SAHD) and preferences regarding a hypothetical end to therapeutic interventions in the future.

Results contradicted prevailing beliefs regarding ALS patients with locked-in syndrome’s views on quality of and the will to live, researchers reported.

In a QoL graph that spanned +5 to -5 to quantify overall quality of life, the median score was 1, indicating a net positive perspective on  life quality of life in these ALS patients.

On the issues of depression and the wish for a hastened death, participants again defied conventional thinking: only five were found to have “clinically relevant depression,” making depressiveness rare among this group of patients. And in those five people, depression was clinically diagnosed prior to disease onset and not associated with ALS or locked-in syndrome.

The well-being and relative satisfaction with quality of life was not found to be associated with physical function. Lulé explained that the progressive nature of the disease may lead patients to be far more adaptable to their disability, and that “caregivers and outsiders may underestimate psychological adaptation,” describing the study’s patients as “strong fighters.”

Moreover, as noted in the study’s abstract, “preferences for termination of therapeutic devices in the future were reported in a subset of patients only. Wish for hastened death was low and in the range of patients in early stages of the disease as previously reported, indicating a constantly low level throughout the disease.”

Lulé admitted that quality of life is a highly subjective issue, and that there is a wide range of cultural impacts on the perspectives explored in the study that cannot be fully accounted for in its results.

The researchers concluded that, given participants’ reports of high levels of social support, overall well-being in ALS patients with locked-in syndrome may be directly a result of the social support provided them.

One comment

  1. William says:

    The desire to live and fight for life is strongest when the end is perilously close. No one living can really say how they would feel or how they wouldn’t want to live that way – until you’ve actually been in a life-ending situation. We all think it is easy to put ourselves in someone else’s shoes, but it’s not. That said, the findings in this study are not surprising to me at all. It seems an abnormal result to us, but, we’ve never been there and despite what we think we can’t honestly say what we would do or how we would feel in the same situation.

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