Integrated care of muscular dystrophies in Italy. Part 1. Pharmacological treatment and rehabilitative interventions

Luisa Politano1 ¹, Marianna Scutifero ¹, Melania Patalano ², Alessandra Sagliocchi ², Antonella Zaccaro ², Federica Civati ³, Erika Brighina ³, Gianluca Vita ⁴, Sonia Messina ⁴, Maria Sframeli ⁴, Maria Elena Lombardo ⁵, Roberta Scalise ⁵, Giulia Colia ⁶, Maria Catteruccia ⁶, Angela Berardinelli ⁷, Maria Chiara Motta ⁷, Alessandra Gaiani ⁸, Claudio Semplicini ⁸, Luca Bello ⁸, Guja Astrea ⁹, Giulia Ricci ⁹, Maria Grazia D’Angelo ³, Giuseppe Vita ⁴, Marika Pane ⁵, Adele D’Amico ⁶, Umberto Balottin ⁷, Corrado Angelini ⁸, Roberta Battini ⁹ and Lorenza Magliano ²

¹ Cardiomyology and Medical Genetics, Department of Experimental Medicine, Campania University “Luigi Vanvitelli” (former denomination: Second University of Naples), Italy; ² Department of Psychology, Campania University “Luigi Vanvitelli”, Italy; ³ NeuroMuscular Unit, Department of NeuroRehabilitation, IRCCS “E. Medea”, Bosisio Parini (LC), Italy; ⁴ Department of Neurosciences, University of Messina; ⁵ Department of Paediatric Neurology, Catholic University, Rome, Italy; ⁶ Unit of Neuromuscular and Neurodegenerative Diseases, Bambin Gesù Children’s Hospital, Rome, Italy; ⁷ Child Neuropsychiatry Unit, Department of Brain and Behavioral Sciences, University of Pavia, Pavia, Italy; ⁸ Department of Neurosciences, University of Padova, Italy; ⁹ Developmental Neuroscience, IRCCS Stella Maris, Pisa Italy; ¹⁰ Department of Brain and Behavioural Sciences, Child Neuropsychiatry Unit, University of Pavia, Italy

This paper describes the pharmacological therapies and rehabilitative interventions received by 502 patients with Muscular Dystrophies, evaluated in relation to patient’s socio-demographic and clinical variables, and geographical areas.

Data were collected by the MD-Socio-Demographic and Clinical Schedule (MD-SC-CS) and by the Family Problems Questionnaire (FPQ).

The most part of the enrolled patients were in drug treatment. The number of the medications increased in relation to patient’s age, disability degree and duration of illness and was higher among patients with Duchenne Muscular Dystrophy (DMD) compared with Becker (BMD) or Limb-Girdle Muscular Dystrophies (LGMD). Steroids (deflazacort or prednisone) were the drug most frequently used, followed by cardiologic and bone metabolism drugs. In general, patients using steroids were younger and had a shorter duration of illness; patients using cardiac drugs and dietary supplements were older and had a longer duration of illness.

Rehabilitative interventions were provided to about 70% (351/502) of patients, mainly DMD. Of these, physiotherapy was the more frequent treatment (96.6%) and was prevalently performed in rehabilitative centres (about 70% of patients) and at home in only 30%. Hydrokinetic-therapy was practiced by 6.8% of patients. Respiratory rehabilitation was provided to 47.0% of patients (165/351) and assisted mechanical ventilation to 13.1% (46). The amount of rehabilitative interventions increased in relation to the patient’s age, level of disability and duration of illness.

Compared to Central and Northern Italy, in Southern Italy there was a higher attention to cardiological impairment as shown by a higher number of patients receiving heart drugs.

No statistically significant differences concerning the possibility to have access to rehabilitative interventions were noted among the three geographical areas. However, patient living in Southern Italy tend to receive rehabilitation more often at home.

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