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Neuromuscular and rare diseases

Malalties neuromusculars i malalties rares a Vall d'Hebron

Neuromuscular disease is a chronic illness that results in serious disability, loss of independence, and with significant psychosocial consequences. Respiratory alterations are the main cause of morbidity and mortality in patients with neuromuscular diseases. They are significantly affected by the evolution of the disease and are the reason for multiple hospital admissions where the patient’s life is seriously endangered.

Description

The main causes of respiratory impairment are hypoventilation due to weak inspiratory muscles and a lack of ability to cough due to weak expiratory muscles. Ventilatory support via non-invasive mechanical ventilation or tracheotomy can prevent or reverse ventilatory failure in these patients.

The loss of expiratory strength means that patients are unable to expel bronchial secretions. If the bulbar muscles are also affected and patients run the risk of inhaling saliva, the contents of the mouth or food, this can induce multiple respiratory infections, pneumonia and atelectasis which results in obstruction of the airway and seriously endangers the patient's life.

The combination of non-invasive mechanical ventilation to assist coughing decreases morbidity and hospital admissions for these patients.

 

Symptoms

  • Respiratory and ventilatory failure
  • Unable to expel secretions and ineffective cough
  • Dysphagia and dysarthria due to impairment of the bulbar muscles  
  • Impairment of peripheral muscles limits function and basic daily activities

 There are currently around 60,000 people with the condition in Spain.

 

Diagnosis

In the Cardiorespiratory Rehabilitation Unit, we monitor maximal inspiratory and expiratory pressure (MIP and MEP) and peak expiratory flow (PEF), also known as peak cough flow (PCF) and carry out spirometry.

 

Treatment 

Treatment goals are focused on controlling the evolution of the ventilatory failure and avoiding or improving episodes of respiratory failure. To achieve these objectives, manual techniques or equipment have to be used. These are techniques to encourage pulmonary expansion, manually assist coughing, and others.

 

Prevention 

One very important objective is to train the main carer in physiotherapy techniques in order to avoid possible complications in the respiratory system.

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General Hospital

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Cross-departmental services

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Healthcare specialisations
    
Related professionals
Dr. Jaume Joan
Ferrer Sancho
Head of Department
Pneumology
Dr. José
Álvarez Sabín
Head of Department
Neurology
Dr. Josep
Gámez
Head of Section
Neurology
Lead Researcher
Peripheral Nervous System
Dr. Maria Luiza
De Souza Galvão
Medical Coordinator
Pneumology
Dra. Maria
Salvadó
Researcher
Peripheral Nervous System
Dra. Marta
Torres Ferrús
Researcher
Headaches and Neurological Pain
Dr. Oriol
de Fàbregues
Lead Researcher
Neurodegenerative Diseases
Lead Researcher
Neurology
Dr. David
Gómez Andrés
Doctor
Paediatric Neurology
Dr. Mario
Culebras Amigo
Sra. Elisenda
Conde Rodriguez
Nurse
Peripheral Nervous System
Sra. Laia
Vilalta Amblàs
 
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