Discussion
For children affected by type 1 and 2 SMA, advanced telemedicine
platforms seem to be a feasible and accurate solution that represents an
unanticipated positive experience to overcome old and new limitations in
time of Covid.
Our study showed that lung auscultation in type 1 and 2 SMA children can
be remotely performed even by layman parents. The feasibility of the
tool in patients with SMA revealed that the tool can always be used to
obtain reliable auscultation but adaptations to find optimal landmarks
are often needed in cases with asymmetrical or rotated chest and trunk.
In our experience, remote auscultation resulted almost straightforward
in children with symmetric chest or mild-moderate scoliosis. In
contrast, in patients with severe scoliosis optimal landmarks for
digital lung auscultation could still be identified but required a
preliminary assessment by experienced physicians, with traditional
stethoscopes that allowed a tailor-made mapping of the landmarks for
future reference to be used at home by the carers. In this study we also
used lung ultrasound but it did not provide additional information on
the landmarks compared to the traditional stethoscope auscultation. Once
the landmarks were identified, after adequate 30 minutes in-person
training, all carers were able to perform the lung examination following
the step-by-step procedure as displayed by the device. The carers of
children with severe scoliosis (Cobb angle>50°),
roto-scoliosis, kyphosis or chest deformities, reported that extra care
was required to firmly hold in place the digital device.
Our results would therefore suggest, that following some preliminary
work for landmarks identification, the device can be easily used by
carers. As several factors, such as growth, increasing scoliosis or
chest deformities due to the progression of the disease may interfere
with the position of the landmarks, we expect that the landmarks should
be reassessed at each hospital visit
In our study, recording good quality sounds did not represent a
challenge for most carers as confirmed by the remote assessment of their
recordings by the expert medical team. Obtaining good results was
probably facilitated by the device displaying several alert messages
when poor quality sounds were recorded, inviting the examiner to record
again until acceptable quality was obtained. There were very few
disturbed recordings that had minor issues rated by examiner as due to
interfering noises such as friction (unsteady holding in place the
device) or human voices heard in the background.
Interestingly, in the 5 patients who were ventilated, the sound of
mechanical ventilation did not interfere with auscultation and was
correctly recognized in 100% of cases, probably due to its
characteristic noise and unchanged rhythm. Surprisingly, it did not even
trigger an alert message from the device which would have prevented
remote recording in these children.
When asked to comment on their experience in video, all carers reported
an overall enthusiastic experience, with “surprising” “unexpected”
and “easy” being the 3 more frequently used adjectives. When asked
about the potential future implementation “hope” was the most common
comment followed by “I wish I’ll not make mistakes” apparently
revealing the mixed feelings between excitement and anxiety. Some carers
revealed some auscultation-related-stress which waned over time, with
exercise, and with remote assistance by our trained physicians and by
the ICT group. In all cases a carer’s personal motivation contributed to
the fast learning and to the high number of successful lung evaluations,
regardless of previous technical skills, age, gender or scientific
background.
As expected, having their carers performing the assessment was very well
accepted and perceived as a low-stress procedure for children. The
application of colorful stickers was perceived as a fun game, according
to their carers opinion. We believe that such “hands-on” training
could be scheduled as part of the routine clinical evaluation in
neuromuscular centers.
One of the limitations of this study, that was meant to assess the
suitability of the new devices, was that while we were able to establish
the feasibility of the tool to obtain reliable recordings and to
establish the adaptations needed and the level of training, we could not
assess the sensitivity of the device to detect pathological findings,
probably due to Covid-related restrictions and parental extra
precautions, all the patients enrolled in this study were all in good
health and were evaluated in the absence of lung infections. Further
studies are therefore needed to evaluate the sensitivity of the device
and the concordance of responses in comparison with traditional
auscultation in neuromuscular patients affected by respiratory
infections. In conclusion the results of our study, that is part of a
larger Advance Telemedicine project , suggest, for the first time, that
remote devices may be used to address the un-meet need reported by
patients with chronic disabilities (such SMA) and their carers regarding
the possibility to assess patients in the presence of a possible acute
event. Although the use of the new device has been driven by the needs
at the time of pandemic, their use could be postulated also in the
future as they may facilitate some aspects of care, reduce the number of
visits to the hospital and ease direct costs (i.e. transport, overnight
stays) and indirect costs (work and school permits or organizational
expenses) for families.