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Disabil Health J. 2018 Apr;11(2):306-309. doi: 10.1016/j.dhjo.2017.09.003. Epub 2017 Sep 21.

Telemedicine for Facio-Scapulo-Humeral Muscular Dystrophy: A multidisciplinary approach to improve quality of life and reduce hospitalization rate?

Author information

1
IRCCS "Bonino-Pulejo" Research Institute, SS 113, C.da Casazza, Messina, Italy.
2
IRCCS "Bonino-Pulejo" Research Institute, SS 113, C.da Casazza, Messina, Italy. Electronic address: salbro77@tiscali.it.
3
Centro Clinico Nemo, Milan, Italy.
4
Institute of Applied Sciences and Intelligent Systems "Edoardo Caianello" (ISASI), National Research Council of Italy, Messina, Italy.

Abstract

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.

KEYWORDS:

FSHD; Multidisciplinary approach; Neuromuscular disorders; Repisratory failure; Telemedicine; Telerehabilitation

PMID:
28967584
DOI:
10.1016/j.dhjo.2017.09.003
[Indexed for MEDLINE]

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