healthmedicinet

This is the first study to report on the evolution of QoL in a LIS population and
is the largest and longest duration examination of a cohort of LIS patients ever conducted.

The LIS persons involved in this study already had chronic LIS statuses for long durations
(the median LIS duration was 7 years in 2007). Several previous studies have reported
the QoL of LIS persons in single assessments 14], 22], 23] through cross-sectional research studies that may not provide definite information
about cause and effect relationships. Longitudinal design provides more valid information
and more robust findings.

The first important finding is that the LIS patients exhibited a rather good QoL maintenance
throughout the 6-year period; nearly ¾ of the patients reported a stable or improved
QoL at 6 years. Regarding the few studies that have used the ACSA score to assess
QoL, LIS patients have reported lower scores (after scoring harmonization) than subjects
who have sustained whiplash injuries 24] and higher scores than patients with facial prostheses 25] and patients with new diagnoses of Alzheimer’s disease 26].

In the same period, the health/handicap (physical and psychological) statuses of some
of the patients changed. We did not observed relationships between the objective parameters
of the health/handicap statuses and level of QoL. Similarly, considering the 11 patients
(of the total of 39) who objectively exhibited deteriorations in their health/handicap
statuses between 2007, their QoL scores were not different from those of the other
patients. It has been previously observed that patients’ subjective QoL is not related
to physical impairments; this observation agrees with previous studies of different
motor neuron disorders 4], 11], 15], 22], 27]–32] and illustrates the “disability paradox” reported by Albrecht and Devlieger 33]. These findings confirm that usual clinical assessments that are based on objective
outcomes do not reflect all of the aspects that patients consider important in to
live. This lack of association between objective health/handicap change and QoL could
also be explained by the presence of the well-known ‘response shift phenomena’ 34]. QoL is self-reported by the patient and might be influenced by this phenomena, which
corresponds to the adaptation to the illness (i.e., adaptation to a bedridden state
and restricted physical/social function in the specific case of LIS). The presence
of a response shift may result in the over- or underestimation of the true changes
and lead to challenges in interpreting QoL measures, especially in longitudinal studies
35]. In this present case, the three classical components of the response shift may have
been incriminated; i.e., reconceptualization defined by as a redefinition of QoL,
reprioritization defined as a change in the importance attributed to the component
domains that constitute QoL, and recalibration defined as a change in a patient’s
internal measurement standard.

Although medical device use declined over time in terms of feeding tubes and tracheotomies,
an important proportion of patients continued to depend on these devices throughout
the study period. Indeed, in 2013, a high proportion of the patients (one-third) had
gastrostomies and tracheotomies primarily due to incomplete swallow recovery, and
10 % had permanent urinary probes. In most studies, swallowing ability and improved
continence have been found to reduce the need for medical devices 3], 4].

Another interesting finding concerns the role of communication. All of the LIS persons
involved in this study could communicate; more than 50 % of the patients used electronic
communication devices, and the other patients communicated only through a yes-no code.
The proportion of patients who preferentially used a yes-no code to communicate tended
to decrease over time, from 77 % in 2007 to and 62 % in 2013. This last restrictive
mode of communication was the single parameter that was associated with a significantly
lower QoL. Communication for LIS people implies the use of alternative communication,
such as eye blinks or eye movements for a yes-no code or communication boards with
letters or symbols that are indicated via eye movements, and both alternatives imply
the avoidance of open-ended and the confirmation of answers with repeated questions
when necessary. Communication is also very limited and requires the help of others.
Electronic communication devices, including patient-computer interfaces such as infrared
eye movement sensors and computer voice prosthetics, have a liberating effect on people
with LIS and enable them to have real dialogues and use the internet instead of passively
responding to the requests of others 36]–38].

The autonomy afforded by an electric wheelchair is recognized as an important element
for an LIS patient. In our study, nearly 60 % of the LIS patients stated that they
were autonomous with a powered electric wheelchair and reported feelings of sufficient
autonomy at home, and such feelings were associated with higher QoL scores, although
this difference was not significant.

Another comment should be made concerning the life conditions of the patients. In
2013, 82 % (32/39) of the patients lived in their own homes. Among these patients,
81 % were already at home in 2007, but 19 % moved from an institutional setting to
their personal home, which implies that both the health/handicap status and the family
circle allowed for such a move. Surprisingly, the patients living in institutional
settings reported systematically higher scores than those living at home (albeit this
difference was non-significant), which could be explained by the soothing role of
a medical environment. Marital status did not affect the QoL score, but single persons
generally reported lower scores than the individuals who were part of a couple. Financial
income was not related to overall well-being, which is probably reflective of the
French health care system, specifically the universal health-care insurance and existence
of resource allocations to help maintain very dependent persons at home via the financing
of human and technical aid.

The mental/psychological conditions are important to consider. We did not observe
a link between mood disorders and QoL levels, although a high proportion of our patients
reported having mood disorders and/or feelings of depression. Previous studies that
have assessed patients with severe diseases (e.g., amyotrophic lateral sclerosis)
have reported contradictory relationships between depression and QoL 39]. Nevertheless, more than a quarter of our cohort reporting having suicidal thoughts
as previously described,40] and such thoughts unsurprisingly influence their QoL. Lastly, family members, careers
and medical professionals frequently assume that LIS persons would choose to die,
but this was not true. Our results indicate that the demand for euthanasia was almost
non-existent in our group of patients, and a great number of the patients expressed
a desire for resuscitation if necessary.

Strengths and limitations

The sample size was arguably too small. When we tried to identify linked factors using
the multivariate approach, associations may have been missed due to low statistical
power. Larger samples would allow for the confirmation of these findings despite the
rarity of this condition. However, the present report is the only study that has followed
patients for a rather long period.

The representativeness and the size of our sample should be discussed. Our patients
appeared to be relatively similar to the populations of other studies in terms of
age, gender, and LIS etiology 3], 4], 41]. As described in previous studies, 4], 5] the mortality rate was very low; two patients died in 6 years after initial medical
stabilization (more than a year). According to Doble et al., 4] the 10-year survival of LIS persons is 83 %, and the 20-year survival is 40 %. The
limited data preclude an appreciation of the true prevalence/incidence of this syndrome,
38]. While it can be assumed that a significant proportion of individuals with LIS are
in contact with the French Association of Locked-In Syndrome, we can hypothesize that
these individuals have a better social support compared to the individuals who are
not in contact with patients’ associations. Future studies should better apprehend
the exhaustiveness.

The proportion of patients lost to follow-up appears high and troublesome to the significance
of findings. It could be hypothesized that the non-respondents included patients with
more severe physical and/or mental and/or social conditions, more severe communication
limitations, and more important cognitive impairment. This can lead to an overestimation
of the QoL level. However, we can assume that the respondents did not differ from
the non-respondents in terms of gender and age, which ensures the relative validity
of our findings.

We were unable to confirm the impact of other potential QoL determinants. Cognitive
impairment, social support and social bonding, and satisfaction with these supports
were not collected in our study. Future studies should explore these parameters.

Finally, the communication limitations of LIS persons make patients’ assessments particularly
difficult. As we did not collect the information whether the patient him/herself responded
or if the caregiver filled out the questionnaire on behalf of the patient, we can
provide an inaccurate estimation of the patient’s QoL. The QoL scale had to be selected
based on the ease of use with this population and consisted mostly of the use of eye
blinking and vertical eye movements to communicate. The use of the self-report Anamnestic
Comparative Self-Assessment to determine the QoL level should be discussed. Indeed
this questionnaire provides a single global measure restricting the QoL concept. Although
we appreciate this tool for its capacity for self-administration and its short time
of completion, we recognize that a more specific QoL questionnaire or a multidimensional
questionnaire could provide a more satisfactory picture of the self-perceived lives
of these patients.