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.