Misure di qualità di vita (QoL) specifiche per la SLA. Validazione dell’adattamento italiano di ALSAQ-40 e ALSAQ-5

摘要

Evaluation of quality of life (QoL) has acquired increasing importance in degenerative diseases with poor prognosis such as amyotrophic lateral sclerosis (ALS). The lack of treatments for ALS patients and the disease progression imply the need for palliative therapies that have to be evaluated according to international guidelines. ALSAQ-40 and its shortened form, ALSAQ-5, are widely used ALS patient-focused disease questionnairies [5,6]. Our purpose is to validate the Italian version of ALSAQ-40 and ALSAQ-5 [10] in a large cohort of Italian ALS patients and to further characterize QoL relation with muscle strength, motor disability and respiratory failure. We recruited 76 ALS patients to complete the Italian version of ALSAQ-40 and ALSAQ-5. To verify test-retest reliability, 30 patients were revaluated after three months. The internal reliability of the translated ALSAQ-40 scales and the test-retest reliability were assessed by means of statistical index as Cronbach‘s alpha. The SF-36 questionnaire and Revised ALS Functional Rating Scale (ALSFR-R) [2] scale were used to assess Italian ALSAQ-40 validity. The limb muscles Medical Research Council (MRC) and Forced Vital Capacity (FVC) scores were used to measure, respectively, the degree of patient loss of strength and respiratory failure. Italian adaptation of ALSAQ-40 and ALSAQ-5 psychometric properties are reliable and similar to those of the original English version. The Italian adaptation showed a very good internal consistency (all subscales Cronbach’s alpha > 0.86) and a good construct validity as shown by the patterns of correlation between the subscales and SF-36 scores. ALSAQ-5 showed a positive correlation with the corresponding total and subscale scores of the ALSAQ-40 (Spearman’s correlation > 0.73). Moreover, we found a strong correlation between Italian ALSAQ-40 and ALSFRS-R, and between MRC and FVC scores and Physical Mobility and ADL/Independence ALSAQ-40 subscales scores. ALSAQ-40 Emotional Functioning subscale and either muscle strength or functional ability are not correlated. Emotive connotations patients assign to their life can remain positive when their health is severely impaired. In conclusion, we found the Italian adaptation of ALSAQ-40 and ALSAQ-5 questionnaires as valid, reliable and useful in terms of disease-specific QoL instruments for Italian ALS patients. Our study suggests also that quality of life can be maintained as physical function declines. y