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Telemedicine for Facio-Scapulo-Humeral Muscular Dystrophy: A multidisciplinary approach to improve quality of life and reduce hospitalization rate?
Disability and Health Journal xxx (2017) 1e4
Contents lists available at ScienceDirect
Disability and Health Journal journal homepage: www.disabilityandhealthjnl.com
Telemedicine for Facio-Scapulo-Humeral Muscular Dystrophy: A multidisciplinary approach to improve quality of life and reduce hospitalization rate? a, *, Placido Bramanti a, Giuseppe Silvestri a, Simona Portaro a, Rocco Salvatore Calabro a a Michele Torrisi , Valeria Conti-Nibali , Santina Caliri a, Christian Lunetta b, Bernardo Alagna a, Antonino Naro a, Alessia Bramanti c a
IRCCS “Bonino-Pulejo” Research Institute, SS 113, C.da Casazza, Messina, Italy Centro Clinico Nemo, Milan, Italy c Institute of Applied Sciences and Intelligent Systems “Edoardo Caianello” (ISASI), National Research Council of Italy, Messina, Italy b
a r t i c l e i n f o
a b s t r a c t
Article history: Received 12 June 2017 Received in revised form 8 September 2017 Accepted 19 September 2017
Background: Facio-Scapulo-Humeral Muscular Dystrophy (FSHD) is an autosomal dominant inherited disorder characterized by a variable and asymmetric involvement of facial, trunk, upper and lower extremity muscles. Although respiratory weakness is a relatively unknown feature of FSHD, it is not rare. Telemedicine has been used in a variety of health care fields, but only recently, with the advent of sophisticated technology, its interest among health professionals became evident, even in such diseases. Objective: To demonstrate the telemedicine efficacy in FSHD. Methods: Four siblings affected by a severe form of FSHD, living in a rural area far away from the referral center for neuromuscular diseases, who used a wheelchair, suffered from chronic respiratory failure and were provided with long-term non-invasive mechanical ventilation, received a 6-month period of telemedicine support. This consisted of video conferencing (respiratory physiotherapy, psychological support, neurological and pneumological assessment, nurse-coach supervision) and telemonitoring of cardiorespiratory variables (oxygen saturation, blood pressure, and heart rate). Results: We performed 540 video conference sessions per patient, including three daily contacts with short monitoring oximetry measurements, blood pressure, and heart-rate measurements, psychological support, neurological and pneumological assessment, nurse-coach supervision. Conclusions: Our findings indicate that our telemedicine system was user-friendly, efficient for the home treatment of FSHD, and allowed reducing hospital admissions. © 2017 Elsevier Inc. All rights reserved.
Keywords: Telemedicine FSHD Telerehabilitation Neuromuscular disorders Repisratory failure Multidisciplinary approach
Introduction Telemedicine is an open and constantly evolving science, using telecommunication and information technology to provide health care at distance, as well as the transmission of medical, imaging and health informatics data from one site to another.1 The aims of telemedicine are: (i) to provide clinical support; (ii) to overcome geographical barriers; (iii) to use various types of innovative information communication technology; and (iv) to improve health
* Corresponding author. IRCCS Centro Neurolesi “Bonino-Pulejo”, S.S. 113, Contrada Casazza, 98124, Messina, Italy. ). E-mail address: [email protected] (R.S. Calabro
outcomes.2,3 Telemedicine application consists of two basic types: between health professionals and between health professionals and patients.1 Telemedicine can be performed in an asynchronous mode, using recorded data at different times, or in a synchronous mode, involving individuals in real time for the immediate exchange of information.4 In both cases, the transmission of the data can consist of text, audio, video, or images.5,6 Telemedicine has been efficiently applied in different neurological fields, such as cerebrovascular or neurodegenerative disorders.7,8 To date, few data are reported on the application of telemedicine for neuromuscular diseases with chronic respiratory insufficiency, mainly amyotrophic lateral sclerosis. It has been shown that the patients managed with telemedicine received the same quality of care and had similar outcomes to those seen via
https://doi.org/10.1016/j.dhjo.2017.09.003 1936-6574/© 2017 Elsevier Inc. All rights reserved.
Please cite this article in press as: Portaro S, et al., Telemedicine for Facio-Scapulo-Humeral Muscular Dystrophy: A multidisciplinary approach to improve quality of life and reduce hospitalization rate?, Disability and Health Journal (2017), https://doi.org/10.1016/j.dhjo.2017.09.003
2
S. Portaro et al. / Disability and Health Journal xxx (2017) 1e4
traditional face-to-face encounters, thus guaranteeing high-quality tertiary ALS care.9e13 No data are available regarding telemedicine application in Facio-Scapulo-Humeral Muscular Dystrophy (FSHD). FSHD is an autosomal dominant inherited disease with an asymmetric involvement of muscles in the facial, trunk, upper and lower extremity regions, with variable severity.14 FSHD is the third most common form of muscular dystrophy. Patients with FSHD may experience respiratory failure because of a progressive weakness of respiratory muscles and scoliosis. Usually, chronic respiratory failure occurs when the patient complains of other signs of severe functional impairment, such as using a wheelchair.15 This study aims at describing a telemedicine system based on video conferencing and telemonitoring of cardiorespiratory variables (oxygen saturation, blood pressure, and heart rate) for the telecare of four siblings affected by FSHD with chronic respiratory failure, living in a rural area far away from the referral center for neuromuscular disorders. Methods The present study involved four siblings affected by a severe form of FSHD with chronic respiratory failure treated with longterm non-invasive mechanical ventilation, who used a wheelchair and lived in a rural area far away from the referral center for neuromuscular diseases. They received a 6-month period of telemedicine support based on video-conferencing (psychological support, neurological and pneumological assessment, nurse-coach monitoring for device use) and telemonitoring of cardiorespiratory variables (oxygen saturation, blood pressure, and heart rate), and telerehabilitation (respiratory physiotherapy). The duration of this experimental telemedicine protocol was set at six months, and a follow-up hospital admission was planned to assess the obtained results. We applied a Telemedicine System that was modified to monitor patients' needs. The patient could interact with the system at any time by accessing a simple menu on the mobile phone. To monitor cardio-respiratory parameters, we used a pulse oximeter (Nonin Onyx® 9500) and an aneroid sphygmomanometer (BOSO). Within the system architecture, the Control Center, the Application Server, and the Database Manager were located at the Hospital Center. The system allowed a Telecare Service Center to perform remote monitoring of biological signals, video conferencing, and to handle emergencies that may arise. An administrator handled database, user privileges, communications, and security from the Hospital. A system firewall protected communications between client and server against unauthorized access. In addition, the system also had a mechanism for user authentication and a password to check the role of the connecting person. The engineers-team visited the patient at home periodically, performing all regular procedures, and checking the equipment by testing all medical and communication devices. Any malfunction was reported to the supervising physician. The local Ethics Committee approved the study and the patients gave their written informed consent to study publication. Telemedicine protocol Depending on the severity of the patient's condition, two different telecare protocols were followed. If the patient's condition was stable, cardio-respiratory parameters were carried out three times a day. The patient sent the data to the telemedicine center, where a skilled operator received these; the neurologist or the pulmonologist (who were on call 24/7) were called for consultation in case of alerts. If the patient's condition worsened, the oxygen saturation was monitored overnight, and reported to the physician the next morning. If the measurements were abnormal, new
instructions were given to the patient's family by the physician. Other videoconferences were carried out, according to the following schedule: psychological consultation one time a week (about 30-min per session); body mass index (BMI) assessment monthly to monitor the assigned diet (about 15-min per session); cardiorespiratory rehabilitation twice a week (about 40-min per session); neurological and pneumological consultations when required or in emergency. For the psychological aspect, we also administered at baseline and after the end of the telemedicine protocol the following scales: 36-Item Short Form Survey (SF-36) to investigate changes in quality of life in eight fields (Physical Functioning, Limitations Due To Physical Problems, Limitations due to Emotional Problems, Pain, General Health, Vitality, Social Functioning, Mental Health); and the Hospital Anxiety and Depression Scale (HADS) to evaluate mood and emotional state. The Psychosocial Impact of Assistive Devices Scale (PIADS) and the Caregiver Burden Inventory (CBI) were administered to verify the effect of telemedicine service on some Psychosocial features (i.e., ability, adaptability and selfesteem) and to evaluate the degree of the perceived caregiver burden, respectively.16e20 The Cardiorespiratory Rehabilitation was performed through a Virtual Reality Rehabilitation System (VRRS) (Khymeia; Padova, Italy), applying biofeedback exercises for monitoring the respiratory rate and the inhalation and exhalation phases. This module requires a spirometer and the Khymeia dedicated cardiorespiratory monitor, in order to perform the exercises. The advantage of the VRRS consisted of the use of a virtual environment that helped the patients to develop knowledge of the results and the performances. Caregivers required only 4 h of training to learn how to use the system.
Results Patients were assessed by a neurologist and a pulmonologist before their inclusion in the study. Over a 6-months period, we analysed a series of variables including the use of the system, the satisfaction of the patient, and the clinical impact. Overall, we performed 540 videoconferences per patient, including three daily contacts with short monitoring of oximetry, blood pressure, and heart rate. Additionally, psychological support, neurological and pneumological assessment, respiratory physiotherapist using the VRRS system, and nurse-coach supervision were provided. The system was used on a continual basis. Vital parameters measurements appeared on the TV screen, and were viewed in the Control Center. The hospital alarm was activated through system 45 times, but the events (i.e., low blood/high blood pressure or heart rate or desaturation) were not clinically relevant. The personal information provided by the patients, together with data from oximetry readings, made it possible to identify an acute event managed with the help of the neurologist from the Control Center, which avoided the tracheotomy for patient n.2. Moreover, there were 20 exacerbations associated with infectious disease, requiring pharmacological therapy but not hospital admission. With respect to the clinical impact, after enrolment in the telemedicine program, the total number of hospital admissions for check-up dropped. As illustrated in Fig. 1, we found a mild improvement in mood and emotional status of all the patients. Only one patient showed significant reduction of depression and anxiety level as per HADS, while three patients reported positive improvement in “mental health” sub-item of SF36 after six months (Fig. 1). BMI remained stable. In addition, PIADDS0 scores revealed higher scores in “ability”, indicating better skills to face problems. On the contrary, telemedicine service did not produce any change in CBI.
Please cite this article in press as: Portaro S, et al., Telemedicine for Facio-Scapulo-Humeral Muscular Dystrophy: A multidisciplinary approach to improve quality of life and reduce hospitalization rate?, Disability and Health Journal (2017), https://doi.org/10.1016/j.dhjo.2017.09.003
S. Portaro et al. / Disability and Health Journal xxx (2017) 1e4
3
Fig. 1. Outcome parameters at baseline (T0) and after the end of the telemedicine protocol (T1). Legend: anx: anxiety; dep: depression; HADS Hospital Anxiety and Depression Scale; Mental Health is a SF36 sub-item.
Discussion Home telemonitoring of chronic diseases is a promising patient management approach that provides accurate and reliable data, empowers patients, influences their attitudes and behaviors, and potentially improves their medical conditions.21 In recent years, there is an emerging literature about telemedicine solutions to monitor chronically ill conditions at home, remotely. Telemedicine has been used in a variety of health care fields, but its interest among health professionals has become evident only recently, thanks to the advent of sophisticated technology. Patients with chronic and long-term conditions represent a major health-care problem for public health-care systems. Neuromuscular diseases belong to this group of illness, presenting with progressive muscle wasting, leading to loss of ambulation, difficult swallowing, and respiratory muscle weakness. Neuromuscular diseases associated with respiratory failure have specific characteristics, including
weakness of respiratory muscles, progression towards chronic respiratory failure, and the potential onset of episodes of acute respiratory failure.22 FSHD is the third most common form of muscular dystrophy,14 in which respiratory weakness is not a rare complication.15 Respiratory monitoring is required to identify the real condition of these patients because their low mobility results in low clinical expression of breathlessness. In addition, these patients can experience the insidious development of respiratory failure, so a continuous monitoring of cardiorespiratory variables may be a useful tool to unmask a subtle respiratory involvement, thus providing better care. Oximetry, blood pressure and heart-rate monitoring with our telecare system detected changes in oxygen saturation and facilitated the detection of sub-clinical deteriorations and exacerbations. Thus, we found that exacerbation could be prevented by monitoring respiratory function. Furthermore, our telemedicine protocol empowered patients and clinicians to share decision making and clinical outcomes,
Please cite this article in press as: Portaro S, et al., Telemedicine for Facio-Scapulo-Humeral Muscular Dystrophy: A multidisciplinary approach to improve quality of life and reduce hospitalization rate?, Disability and Health Journal (2017), https://doi.org/10.1016/j.dhjo.2017.09.003
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S. Portaro et al. / Disability and Health Journal xxx (2017) 1e4
improving continuity of care and efficiency by remote sensing at a distance, especially for this chronical illness.23,24 In fact, ventilatory failure develops gradually, with hypercapnia during sleep at first, and then during daylight hours25,26; to have the possibility to monitor this aspect may prevent acute events, and therefore our telemedicine system could be considered as a prevention medicine tool. Moreover, the functional limitation associated with muscle weakness of patients with severe neuromuscular diseases makes to travel a crucial factor.12 We found that telemedicine led to positive results, also concerning the psychological aspects. Unfortunately, the experimental protocol was designed to last up to six months, and patients were then assessed during a hospital admission to better evaluate the outcomes. The work was entirely founded by our institute, and this could partly explain the relatively short-term follow up. In keeping with these promising results, it is reasonable to suppose that a further evaluation after a longer period could reveal more beneficial effects on patients and caregivers. Moreover, it could be useful to evaluate the cost-effectiveness ratio of the service, as compared to the traditional care, and extend the protocol to a larger sample so to investigate whether this telemedicine service may be sustained by the national healthcare service. Also, we considered patients affected by a chronic and rare neurological disease for which more time is required to show significant changes. However, patients indicated a reasonable level of satisfaction, as the system was user-friendly for patients and caregivers. It is worthy to note that telemedicine reduced dependence by increasing autonomy and self-monitoring of certain symptoms and prevented hospital admissions to our patients. Indeed, in our study, the combination of videoconference sessions and monitoring oximetry data allowed early detection of respiratory exacerbation and prevented the need for hospital admissions, because patients were treated without having to leave their home.
3.
4. 5.
6.
7. 8.
9.
10. 11.
12.
13.
14. 15. 16. 17.
Conclusion
18.
In conclusion, we may argue that the telemedicine system was effective for the home treatment of four siblings with FSHD and reduced the need for hospital admissions, providing support for the emergency care for local physicians and caregivers, through the presence of an expert team, to better manage the patient's clinical symptoms.
19.
Conflict of interest statement The authors have no conflict of interest to declare. References 1. Craig J, Patterson V. Introduction to the practice of telemedicine. J Telemed Telecare. 2005;11:3e9. 2. WHO Global Observatory for eHealth. WHO Library Cataloguing-in-publication
20. 21. 22.
23. 24. 25. 26.
Data Telemedicine: Opportunities and Developments in Member States: Report on the Second Global Survey on EHealth. 2009 (Global Observatory for eHealth Series, 2). ISBN 978 92 4 156414 4 (NLM classification: W 26.5; ISSN 2220e5462). WHO. In: A Health Telematics Policy in Support of WHO's Health-for-all Strategy for Global Health Development: Report of the WHO Group Consultation on Health Telematics, 11e16 December, Geneva. Geneva: World Health Organization, 1998; 1997. Rao B, Lombardi II A. Telemedicine: current status in developed and developing countries. J Drugs Dermatol. 2009;8(4):371e375. Currell R, Urquhart C, Wainwright P, et al. Telemedicine versus face to face patient care: effects on professional practice and health care outcomes. Nurs Times. 2001;97(35):35. Wootton R, Menzies J, Ferguson P. Follow-up data for patients managed by store and forward telemedicine in developing countries. J Telemed Telecare. 2009;15(2):83e88. Rubin MN, Wellik KE, Channer DD, et al. Systematic review of teleneurology: neurohospitalist neurology. Neurohospitalist. 2013;3(3):120e124. De Luca R, Bramanti A, De Cola MC, et al. Cognitive training for patients with dementia living in a sicilian nursing home: a novel web-based approach. Neurol Sci. 2016;37(10):1685e1691. n C, Morete E, Gonza lez F. Telemedicine system for the care of patients Zamarro with neuromuscular disease and chronic respiratory failure. Arch Med Sci. 2014;10(5):1047e1051. Henderson RD, Hutchinson N, Douglas JA, et al. Telehealth for motor neurone disease. Med J Aust. 2014;201(1):31. n A, Pe rez M, et al. Effectiveness of teleassistance at Martínez O, Jometo improving quality of life in people with neuromuscular diseases. Span J Psychol. 2014;17:E86. Vitacca M, Comini L, Assoni G, et al. Tele-assistance in patients with amyotrophic lateral sclerosis: long term activity and costs. Disabil Rehabil Assist Technol. 2012;7(6):494e500. Selkirk SM, Washington MO, McClellan F, et al. Delivering tertiary centre specialty care to ALS patients via telemedicine: a retrospective cohort analysis. Amyotroph Lateral Scler Front Degener. 2017;21:1e9. Tawil R. Facioscapulohumeral muscular dystrophy. Neurotherapeutics. 2008;5(4):601e606. Wohlgemuth M, van der Kooi EL, van Kesteren RG, et al. Ventilatory support in facioscapulohumeral muscular dystrophy. Neurology. 2004;63(1):176e178. G, Sicotte C. Home telemonitoring for respiratory conditions: a Jaana M, Pare systematic review. Am J Manag Care. 2009;15(5):313e320. Cooper CB. Respiratory applications of telemedicine. Thorax. 2009;64(3): 189e191. Koizumi T, Takizawa M, Nakai K, et al. Trial of remote telemedicine support for patients with chronic respiratory failure at home through a multistation communication system. Telemed J E Health. 2005;11(4):481e486. Raza T, Joshi M, Schapira RM, et al. Pulmonary telemedicineea model to access the subspecialist services in underserved rural areas. Int J Med Inf. 2009;78(1): 53e59. Smith SM, Elkin SL, Partridge MR. Technology and its role in respiratory care. Prim Care Respir J. 2009;18(3):159e164. G, Jaana M, Sicotte C. Systematic review of home telemonitoring for Pare chronic diseases: the evidence base. J Am Med Inf Assoc. 2007;14(3):269e277. Perrin C, Unterborn JN, Ambrosio CD, et al. Pulmonary complications of chronic neuromuscular diseases and their management. Muscle Nerve. 2004;29(1): 5e27. Roine R, Ohinmaa A, Hailey D. Assessing telemedicine: a systematic review of the literature. CMAJ. 2001;165(6):765e771. Hailey D, Roine R, Ohinmaa A. Systematic review of evidence for the benefits of telemedicine. J Telemed Telecare. 2002;8(Suppl 1):1e30. MacDuff A, Grant IS. Critical care management of neuromuscular disease, including long-term ventilation. Curr Opin Crit Care. 2003;9(2):106e112. Sancho J, Servera E. Noninvasive ventilation for patients with neuromuscular disease and acute respiratory failure. Chest. 2008;133(1):314e315. author reply 315.
Please cite this article in press as: Portaro S, et al., Telemedicine for Facio-Scapulo-Humeral Muscular Dystrophy: A multidisciplinary approach to improve quality of life and reduce hospitalization rate?, Disability and Health Journal (2017), https://doi.org/10.1016/j.dhjo.2017.09.003
Contents lists available at ScienceDirect
Disability and Health Journal journal homepage: www.disabilityandhealthjnl.com
Telemedicine for Facio-Scapulo-Humeral Muscular Dystrophy: A multidisciplinary approach to improve quality of life and reduce hospitalization rate? a, *, Placido Bramanti a, Giuseppe Silvestri a, Simona Portaro a, Rocco Salvatore Calabro a a Michele Torrisi , Valeria Conti-Nibali , Santina Caliri a, Christian Lunetta b, Bernardo Alagna a, Antonino Naro a, Alessia Bramanti c a
IRCCS “Bonino-Pulejo” Research Institute, SS 113, C.da Casazza, Messina, Italy Centro Clinico Nemo, Milan, Italy c Institute of Applied Sciences and Intelligent Systems “Edoardo Caianello” (ISASI), National Research Council of Italy, Messina, Italy b
a r t i c l e i n f o
a b s t r a c t
Article history: Received 12 June 2017 Received in revised form 8 September 2017 Accepted 19 September 2017
Background: Facio-Scapulo-Humeral Muscular Dystrophy (FSHD) is an autosomal dominant inherited disorder characterized by a variable and asymmetric involvement of facial, trunk, upper and lower extremity muscles. Although respiratory weakness is a relatively unknown feature of FSHD, it is not rare. Telemedicine has been used in a variety of health care fields, but only recently, with the advent of sophisticated technology, its interest among health professionals became evident, even in such diseases. Objective: To demonstrate the telemedicine efficacy in FSHD. Methods: Four siblings affected by a severe form of FSHD, living in a rural area far away from the referral center for neuromuscular diseases, who used a wheelchair, suffered from chronic respiratory failure and were provided with long-term non-invasive mechanical ventilation, received a 6-month period of telemedicine support. This consisted of video conferencing (respiratory physiotherapy, psychological support, neurological and pneumological assessment, nurse-coach supervision) and telemonitoring of cardiorespiratory variables (oxygen saturation, blood pressure, and heart rate). Results: We performed 540 video conference sessions per patient, including three daily contacts with short monitoring oximetry measurements, blood pressure, and heart-rate measurements, psychological support, neurological and pneumological assessment, nurse-coach supervision. Conclusions: Our findings indicate that our telemedicine system was user-friendly, efficient for the home treatment of FSHD, and allowed reducing hospital admissions. © 2017 Elsevier Inc. All rights reserved.
Keywords: Telemedicine FSHD Telerehabilitation Neuromuscular disorders Repisratory failure Multidisciplinary approach
Introduction Telemedicine is an open and constantly evolving science, using telecommunication and information technology to provide health care at distance, as well as the transmission of medical, imaging and health informatics data from one site to another.1 The aims of telemedicine are: (i) to provide clinical support; (ii) to overcome geographical barriers; (iii) to use various types of innovative information communication technology; and (iv) to improve health
* Corresponding author. IRCCS Centro Neurolesi “Bonino-Pulejo”, S.S. 113, Contrada Casazza, 98124, Messina, Italy. ). E-mail address: [email protected] (R.S. Calabro
outcomes.2,3 Telemedicine application consists of two basic types: between health professionals and between health professionals and patients.1 Telemedicine can be performed in an asynchronous mode, using recorded data at different times, or in a synchronous mode, involving individuals in real time for the immediate exchange of information.4 In both cases, the transmission of the data can consist of text, audio, video, or images.5,6 Telemedicine has been efficiently applied in different neurological fields, such as cerebrovascular or neurodegenerative disorders.7,8 To date, few data are reported on the application of telemedicine for neuromuscular diseases with chronic respiratory insufficiency, mainly amyotrophic lateral sclerosis. It has been shown that the patients managed with telemedicine received the same quality of care and had similar outcomes to those seen via
https://doi.org/10.1016/j.dhjo.2017.09.003 1936-6574/© 2017 Elsevier Inc. All rights reserved.
Please cite this article in press as: Portaro S, et al., Telemedicine for Facio-Scapulo-Humeral Muscular Dystrophy: A multidisciplinary approach to improve quality of life and reduce hospitalization rate?, Disability and Health Journal (2017), https://doi.org/10.1016/j.dhjo.2017.09.003
2
S. Portaro et al. / Disability and Health Journal xxx (2017) 1e4
traditional face-to-face encounters, thus guaranteeing high-quality tertiary ALS care.9e13 No data are available regarding telemedicine application in Facio-Scapulo-Humeral Muscular Dystrophy (FSHD). FSHD is an autosomal dominant inherited disease with an asymmetric involvement of muscles in the facial, trunk, upper and lower extremity regions, with variable severity.14 FSHD is the third most common form of muscular dystrophy. Patients with FSHD may experience respiratory failure because of a progressive weakness of respiratory muscles and scoliosis. Usually, chronic respiratory failure occurs when the patient complains of other signs of severe functional impairment, such as using a wheelchair.15 This study aims at describing a telemedicine system based on video conferencing and telemonitoring of cardiorespiratory variables (oxygen saturation, blood pressure, and heart rate) for the telecare of four siblings affected by FSHD with chronic respiratory failure, living in a rural area far away from the referral center for neuromuscular disorders. Methods The present study involved four siblings affected by a severe form of FSHD with chronic respiratory failure treated with longterm non-invasive mechanical ventilation, who used a wheelchair and lived in a rural area far away from the referral center for neuromuscular diseases. They received a 6-month period of telemedicine support based on video-conferencing (psychological support, neurological and pneumological assessment, nurse-coach monitoring for device use) and telemonitoring of cardiorespiratory variables (oxygen saturation, blood pressure, and heart rate), and telerehabilitation (respiratory physiotherapy). The duration of this experimental telemedicine protocol was set at six months, and a follow-up hospital admission was planned to assess the obtained results. We applied a Telemedicine System that was modified to monitor patients' needs. The patient could interact with the system at any time by accessing a simple menu on the mobile phone. To monitor cardio-respiratory parameters, we used a pulse oximeter (Nonin Onyx® 9500) and an aneroid sphygmomanometer (BOSO). Within the system architecture, the Control Center, the Application Server, and the Database Manager were located at the Hospital Center. The system allowed a Telecare Service Center to perform remote monitoring of biological signals, video conferencing, and to handle emergencies that may arise. An administrator handled database, user privileges, communications, and security from the Hospital. A system firewall protected communications between client and server against unauthorized access. In addition, the system also had a mechanism for user authentication and a password to check the role of the connecting person. The engineers-team visited the patient at home periodically, performing all regular procedures, and checking the equipment by testing all medical and communication devices. Any malfunction was reported to the supervising physician. The local Ethics Committee approved the study and the patients gave their written informed consent to study publication. Telemedicine protocol Depending on the severity of the patient's condition, two different telecare protocols were followed. If the patient's condition was stable, cardio-respiratory parameters were carried out three times a day. The patient sent the data to the telemedicine center, where a skilled operator received these; the neurologist or the pulmonologist (who were on call 24/7) were called for consultation in case of alerts. If the patient's condition worsened, the oxygen saturation was monitored overnight, and reported to the physician the next morning. If the measurements were abnormal, new
instructions were given to the patient's family by the physician. Other videoconferences were carried out, according to the following schedule: psychological consultation one time a week (about 30-min per session); body mass index (BMI) assessment monthly to monitor the assigned diet (about 15-min per session); cardiorespiratory rehabilitation twice a week (about 40-min per session); neurological and pneumological consultations when required or in emergency. For the psychological aspect, we also administered at baseline and after the end of the telemedicine protocol the following scales: 36-Item Short Form Survey (SF-36) to investigate changes in quality of life in eight fields (Physical Functioning, Limitations Due To Physical Problems, Limitations due to Emotional Problems, Pain, General Health, Vitality, Social Functioning, Mental Health); and the Hospital Anxiety and Depression Scale (HADS) to evaluate mood and emotional state. The Psychosocial Impact of Assistive Devices Scale (PIADS) and the Caregiver Burden Inventory (CBI) were administered to verify the effect of telemedicine service on some Psychosocial features (i.e., ability, adaptability and selfesteem) and to evaluate the degree of the perceived caregiver burden, respectively.16e20 The Cardiorespiratory Rehabilitation was performed through a Virtual Reality Rehabilitation System (VRRS) (Khymeia; Padova, Italy), applying biofeedback exercises for monitoring the respiratory rate and the inhalation and exhalation phases. This module requires a spirometer and the Khymeia dedicated cardiorespiratory monitor, in order to perform the exercises. The advantage of the VRRS consisted of the use of a virtual environment that helped the patients to develop knowledge of the results and the performances. Caregivers required only 4 h of training to learn how to use the system.
Results Patients were assessed by a neurologist and a pulmonologist before their inclusion in the study. Over a 6-months period, we analysed a series of variables including the use of the system, the satisfaction of the patient, and the clinical impact. Overall, we performed 540 videoconferences per patient, including three daily contacts with short monitoring of oximetry, blood pressure, and heart rate. Additionally, psychological support, neurological and pneumological assessment, respiratory physiotherapist using the VRRS system, and nurse-coach supervision were provided. The system was used on a continual basis. Vital parameters measurements appeared on the TV screen, and were viewed in the Control Center. The hospital alarm was activated through system 45 times, but the events (i.e., low blood/high blood pressure or heart rate or desaturation) were not clinically relevant. The personal information provided by the patients, together with data from oximetry readings, made it possible to identify an acute event managed with the help of the neurologist from the Control Center, which avoided the tracheotomy for patient n.2. Moreover, there were 20 exacerbations associated with infectious disease, requiring pharmacological therapy but not hospital admission. With respect to the clinical impact, after enrolment in the telemedicine program, the total number of hospital admissions for check-up dropped. As illustrated in Fig. 1, we found a mild improvement in mood and emotional status of all the patients. Only one patient showed significant reduction of depression and anxiety level as per HADS, while three patients reported positive improvement in “mental health” sub-item of SF36 after six months (Fig. 1). BMI remained stable. In addition, PIADDS0 scores revealed higher scores in “ability”, indicating better skills to face problems. On the contrary, telemedicine service did not produce any change in CBI.
Please cite this article in press as: Portaro S, et al., Telemedicine for Facio-Scapulo-Humeral Muscular Dystrophy: A multidisciplinary approach to improve quality of life and reduce hospitalization rate?, Disability and Health Journal (2017), https://doi.org/10.1016/j.dhjo.2017.09.003
S. Portaro et al. / Disability and Health Journal xxx (2017) 1e4
3
Fig. 1. Outcome parameters at baseline (T0) and after the end of the telemedicine protocol (T1). Legend: anx: anxiety; dep: depression; HADS Hospital Anxiety and Depression Scale; Mental Health is a SF36 sub-item.
Discussion Home telemonitoring of chronic diseases is a promising patient management approach that provides accurate and reliable data, empowers patients, influences their attitudes and behaviors, and potentially improves their medical conditions.21 In recent years, there is an emerging literature about telemedicine solutions to monitor chronically ill conditions at home, remotely. Telemedicine has been used in a variety of health care fields, but its interest among health professionals has become evident only recently, thanks to the advent of sophisticated technology. Patients with chronic and long-term conditions represent a major health-care problem for public health-care systems. Neuromuscular diseases belong to this group of illness, presenting with progressive muscle wasting, leading to loss of ambulation, difficult swallowing, and respiratory muscle weakness. Neuromuscular diseases associated with respiratory failure have specific characteristics, including
weakness of respiratory muscles, progression towards chronic respiratory failure, and the potential onset of episodes of acute respiratory failure.22 FSHD is the third most common form of muscular dystrophy,14 in which respiratory weakness is not a rare complication.15 Respiratory monitoring is required to identify the real condition of these patients because their low mobility results in low clinical expression of breathlessness. In addition, these patients can experience the insidious development of respiratory failure, so a continuous monitoring of cardiorespiratory variables may be a useful tool to unmask a subtle respiratory involvement, thus providing better care. Oximetry, blood pressure and heart-rate monitoring with our telecare system detected changes in oxygen saturation and facilitated the detection of sub-clinical deteriorations and exacerbations. Thus, we found that exacerbation could be prevented by monitoring respiratory function. Furthermore, our telemedicine protocol empowered patients and clinicians to share decision making and clinical outcomes,
Please cite this article in press as: Portaro S, et al., Telemedicine for Facio-Scapulo-Humeral Muscular Dystrophy: A multidisciplinary approach to improve quality of life and reduce hospitalization rate?, Disability and Health Journal (2017), https://doi.org/10.1016/j.dhjo.2017.09.003
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improving continuity of care and efficiency by remote sensing at a distance, especially for this chronical illness.23,24 In fact, ventilatory failure develops gradually, with hypercapnia during sleep at first, and then during daylight hours25,26; to have the possibility to monitor this aspect may prevent acute events, and therefore our telemedicine system could be considered as a prevention medicine tool. Moreover, the functional limitation associated with muscle weakness of patients with severe neuromuscular diseases makes to travel a crucial factor.12 We found that telemedicine led to positive results, also concerning the psychological aspects. Unfortunately, the experimental protocol was designed to last up to six months, and patients were then assessed during a hospital admission to better evaluate the outcomes. The work was entirely founded by our institute, and this could partly explain the relatively short-term follow up. In keeping with these promising results, it is reasonable to suppose that a further evaluation after a longer period could reveal more beneficial effects on patients and caregivers. Moreover, it could be useful to evaluate the cost-effectiveness ratio of the service, as compared to the traditional care, and extend the protocol to a larger sample so to investigate whether this telemedicine service may be sustained by the national healthcare service. Also, we considered patients affected by a chronic and rare neurological disease for which more time is required to show significant changes. However, patients indicated a reasonable level of satisfaction, as the system was user-friendly for patients and caregivers. It is worthy to note that telemedicine reduced dependence by increasing autonomy and self-monitoring of certain symptoms and prevented hospital admissions to our patients. Indeed, in our study, the combination of videoconference sessions and monitoring oximetry data allowed early detection of respiratory exacerbation and prevented the need for hospital admissions, because patients were treated without having to leave their home.
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Conclusion
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In conclusion, we may argue that the telemedicine system was effective for the home treatment of four siblings with FSHD and reduced the need for hospital admissions, providing support for the emergency care for local physicians and caregivers, through the presence of an expert team, to better manage the patient's clinical symptoms.
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Conflict of interest statement The authors have no conflict of interest to declare. References 1. Craig J, Patterson V. Introduction to the practice of telemedicine. J Telemed Telecare. 2005;11:3e9. 2. WHO Global Observatory for eHealth. WHO Library Cataloguing-in-publication
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Please cite this article in press as: Portaro S, et al., Telemedicine for Facio-Scapulo-Humeral Muscular Dystrophy: A multidisciplinary approach to improve quality of life and reduce hospitalization rate?, Disability and Health Journal (2017), https://doi.org/10.1016/j.dhjo.2017.09.003