Burden, professional support, and social network in families of children and young adults with muscular dystrophies.

INTRODUCTION: This study explores burden and social and professional support in families of young patients with muscular dystrophies (MDs) in Italy. METHODS: The study was carried out on 502 key relatives of 4- to 25-year-old patients suffering from Duchenne, Becker, or Limb-Girdle MD who were living with at least 1 adult relative. RESULTS: A total of 77.1% of relatives reported feelings of loss, 74.0% had feelings of sadness, and 59.1% had constraints in leisure activities. Burden was higher among relatives of patients with higher disability and who spent more daily hours in caregiving. Practical difficulties were higher among relatives who perceived lower help in patient emergencies and less practical support by their social network. Psychological burden was higher in those relatives who were unemployed, those with poorer support in emergencies, and those with lower social contacts. CONCLUSIONS: Caring for patients with MDs may be demanding for relatives even in the early stages of these disorders, especially when social support is poor and the patient's disability increases.

Psychological and practical difficulties among parents and healthy siblings of children with Duchenne vs. Becker muscular dystrophy: an Italian comparative study.

This study explored the burden in parents and healthy siblings of 4-17 year-old patients with Duchenne (DMD) and Becker (BMD) muscular dystrophies, and whether the burden varied according to clinical aspects and social resources. Data on socio-demographic characteristics, patient's clinical history, parent and healthy children burden, and on parent's social resources were collected using self-reported questionnaires administered to 336 parents of patients with DMD (246) and BMD (90). Parents of patients with DMD reported higher burden than those of patients with BMD, especially concerning feeling of loss (84.3% DMD vs. 57.4% BMD), stigma (44.2% DMD vs. 5.5% BMD) and neglect of hobbies (69.0% DMD vs. 32.5% BMD). Despite the burden, 66% DMD and 62.4% BMD parents stated the caregiving experience had a positive impact on their lives. A minority of parents believed MD has a negative influence on the psychological well-being (31.0% DMD vs. 12.8% BMD), and social life of unaffected children (25.7% vs. 18.4%). In the DMD group, burden correlated with duration of illness and parent age, and burden was higher among parents with lower social contacts and support in emergencies. In DMD, difficulties among healthy children were reported as higher by parents who were older, had higher burden and lower social contacts. In both groups, burden increased in relation to patient disability. These findings underline that the psychological support to be provided to parents of patients with MD, should take into account clinical features of the disease.

Differences in views of schizophrenia during medical education: a comparative study of 1st versus 5th-6th year Italian medical students.

PURPOSE: This study explored medical students' causal explanations and views of schizophrenia, and whether they changed during medical education. METHOD: The survey was carried out on medical students of the Second University of Naples, Italy, who attended their first-year and their fifth- or sixth-year of lessons. The 381 who accepted were asked to read a case-vignette describing a person who met the ICD-10 criteria for schizophrenia and then fill in the Opinions on mental illness Questionnaire. RESULTS: The most frequently cited causes were psychological traumas (60%) and stress (56%), followed by misuse of street drugs (47%), and heredity (42%). 28% of students stated that persons with the disorder could be well again, and 28% that they were unpredictable. Labeling the case as "schizophrenia" and naming heredity among the causes were associated with pessimism about recovery and higher perception of social distance. First-year students more frequently reported psychological traumas among the causes (76 vs. 45%), and less frequently heredity (35 vs. 81%) and stress (42 vs. 69%), and they perceived less social distance from the "schizophrenics" than fifth/sixth-year students. In particular, 18% percent of first-year versus 38% of fifth/sixth-year students believed that these persons were kept at a distance by the other, and 45 versus 57% felt frightened by persons with the condition. CONCLUSIONS: These results indicate a need to include education on stigma and recovery in schizophrenia in the training of medical students.

[Hyperuricaemia and Chronic Kidney Disease].

Hyperuricemia is defined as serum uric acid values greater than 6 mg/dl and could occur either due to hyperproduction or as a result of reduced renal excretion, which exceeds gut compensation. In Italy, prevalence is around 12% of the general population and increases in renal disease up to 60%. Recent experimental studies demonstrated a role of uric acid in the development of arterial hypertension and systemic arteriosclerosis, with an increase in cardiovascular risk. It also appears from observational studies that high uric acid is an independent risk factor associated with de novo onset of chronic kidney disease after adjustment of main confounding variables. Hyperuricemic subjects treated with febuxostat, a selective inhibitor of xantino-oxidase, showed in RCTs a better control of hyperuricaemia in comparison with those receiving allopurinol. Moreover, observational studies indicate that urate lowering treatment could be helpful in reducing cardiovascular events as well as in slowing the progression of chronic kidney disease; randomized controlled studies, designed to assess as primary outcome the nephroprotective effect of urate lowering treatment, are in progress.

Integrated care of muscular dystrophies in Italy. Part 2. Psychological treatments, social and welfare support, and financial costs.

This paper describes the psycho-social treatments received by 502 patients with MDs and their relatives, and the costs for care sustained by the families in the previous six month period. Data were collected by the MD-Care Schedule (MD-CS) and the Family Problems Questionnaire (FPQ). Psycho-educational interventions were provided to 72 patients (14.3%), and social/welfare support to 331 patients (65.9%). Social/welfare support was higher in patients with DMD or LGMD, in those showing more severe disability, and in patients who were in contact with centres located in Northern Italy. Psycho-educational interventions were received by 156 (31%) relatives, and social/welfare support by 55 (10.9%) and mainly provided by Family/Patients Associations (83.6%). Relatives with higher educational levels, who spent more daily hours in the assistance of patients with DMD, and in contact with centres in Central Italy more frequently benefited from psycho-educational interventions. In the previous year, costs for care were sustained by 314 (63.9%) relatives. Financial difficulties related to patient's condition, were higher in families of patients who needed more intensive rehabilitation and daily hours of caregiving, and in families who lived further away from the reference's centre. These results showed that psycho-social aspects of MDs care are only partially met in Italy, and that ad hoc supportive interventions for these patients and their families should be potentiated.

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

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 ventilaventilation 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.

[Clinical experience with ferric carboxymaltose in non-dialysis chronic kidney disease]. / Ferro carbossimaltosio nella malattia renale cronica non dialitica: una iniziale esperienza clinica.

BACKGROUND: Patients with non-dialysis-dependent chronic kidney disease (ND-CKD) often show anemia and iron deficiency despite oral iron supplementation caused by poor iron absorption, intolerance and non-compliance. METHODS: We prospectively followed seven adult patients with ND-CKD (eGFR <60 ml/min/1.73m2), anemia (Hb<11 g/dl or treatment with ESA), iron deficiency (TSAT<20% and/or ferritin<100 ng/mL) and intolerant or non-responders to oral iron supplementation. Patients received ferric carboxymaltose (FCM) (single dose of 500 mg iv) eventually followed by further doses if iron deficiency persisted. Hemoglobin, ferritin, TSAT and ESA doses were recorded at baseline and after 2, 4, 8, 12, 16, 20 and 24 weeks. RESULTS: After 2 weeks of FCM, ferritin increased from 5348 to 222154 ng/mL (P<0.05) and remained steady thereafter. The increase of TSAT from baseline (115%) was more gradual being significant from week 4 (198%) up to week 24 (2412%). During the study, patients received on average 2.31.0 injections of FCM, to the amount of 1143440 mg. Hb levels remained stable throughout the study, despite a significant reduction of ESA dosage (from 3426 g/week at baseline to 1116 and 1710 g/week, after 4 and 24 weeks, respectively). On average, the ESA dose saving was 2024 g/week. Even considering the higher cost of FCM, ESA dose reduction allowed shortening overall costs by 673/patient during the 24 weeks of study. CONCLUSION: In ND-CKD patients, FCM is effective in correcting iron deficiency and associated with stable Hb levels and significant decrease of ESA dosage. This allows a marked reduction of costs for anemia correction.

"Social dangerousness and incurability in schizophrenia": results of an educational intervention for medical and psychology students.

This study explored the influence of an educational intervention addressing common prejudices and scientific evidence about schizophrenia on medical and psychology students' views of this disorder. The intervention--consisting in two three-hour lessons with an interval of a week between--was run at first for medical students and then for psychology students. Participants' views of schizophrenia were assessed at baseline vs. at post intervention by matched questionnaires. At medical school, participation was voluntary and also included a six-month online re-assessment, while at psychology school, participation was mandatory. A total of 211 students attended the educational initiative. At post intervention assessment, students more frequently mentioned psychosocial causes of schizophrenia, and more firmly believed that recovery in schizophrenia is possible and that persons with this disorder are not unpredictable and dangerous vs. their baseline assessment. The online six-month assessment confirmed favourable changes in medical students' views found at post intervention. These results confirm that an educational intervention including personal experiences and scientific evidence can be successful in reducing students' prejudices toward persons with schizophrenia.

"I have got something positive out of this situation": psychological benefits of caregiving in relatives of young people with muscular dystrophy.

This paper focuses on the psychological benefits of caregiving in key relatives of patients with muscular dystrophies (MD), a group of rare diseases characterized by progressive weakness and restriction of the patient's functional abilities. We describe whether relatives perceived caregiving to be a positive experience and test whether relatives' perceptions vary in relation to their view of the patient as a valued person, the degree of involvement in care, and the level of support provided by social network and professionals. The study sample included 502 key relatives of patients aged 4-25 years, suffering from Duchenne, Becker, or limb-girdle MD, in treatment for at least 6 months to one of the eight participating centers, living with at least one relative aged 18-80 years. Of key relatives, 88 % stated that they had gotten something positive out of the situation, 96 % considered their patients to be sensitive, and 94 % viewed their patients as talented. Positive aspects of caregiving were more recognized by key relatives who were more convinced that the patient was sensitive and who perceived that they received higher level of professional help and psychological social support. These results suggest that most key relatives consider that their caregiving experience has had a positive impact on their lives, despite the practical difficulties of caring for patients with MD. Professionals should help relatives to identify the benefits of caregiving without denying its difficulties. Clinicians themselves should develop positive attitudes towards family involvement in the care of patients with long-term diseases.

Effect of diagnostic labeling and causal explanations on medical students' views about treatments for psychosis and the need to share information with service users.

This study examines whether medical students' views of treatments for 'schizophrenia' and of patients' rights to be informed about their condition and their medication were influenced by diagnostic labeling and causal explanations and whether they differed over medical training. Three hundred and eighty-one Italian students attending their first or fifth/sixth year of medical studies read a vignette portraying someone who met diagnostic criteria for 'schizophrenia' and completed a self-report questionnaire. The study found that labeling the case as 'schizophrenia' and naming heredity among its causes were associated with confidence in psychiatrists and psychiatric drugs. Naming psychological traumas among the causes was associated with confidence in psychologists and greater acknowledgment of users' right to be informed about drugs. Compared to first year students, those at their fifth/sixth-year of studies more strongly endorsed drugs, had less confidence in psychologists and family support, and were less keen to share information on drugs with patients. These findings highlight that students' beliefs vary during training and are significantly related to diagnostic labeling and belief in a biogenetic causal model. Psychiatric curricula for medical students should include greater integration of psychological and medical aspects in clinical management of 'schizophrenia'; more information on the psychosocial causes of mental health problems.

The influence of causal explanations and diagnostic labeling on medical students' views of schizophrenia.

PURPOSE: Negative attitudes toward mental illness among medical professionals can influence the quality of medical care they provide. The authors examined the impact of causal explanations and diagnostic labeling on medical students' views of schizophrenia. METHOD: Medical students in their fifth and sixth years at the Second University of Naples (Italy) who attended lectures from April through June 2010 completed a self-report questionnaire regarding their beliefs about the mental disorder described (but not named) in a case vignette depicting a person who meets the International Classification of Diseases-10 criteria for schizophrenia. RESULTS: Of the 232 students invited, 194 (84%) completed the questionnaire. Students most frequently cited heredity as the cause (81%), followed by stress (69%), psychological traumas (45%), and misuse of street drugs (44%). Most students (82%) labeled the case "schizophrenia"; a minority (24%) believed that persons with the case vignette disorder could be well again. Both labeling the case as "schizophrenia" and naming heredity as the cause were independently associated with pessimism about the possibility of recovery and with the perception that "others" keep their distance from persons with this diagnosis. Heredity was more frequently cited by respondents who labeled the case schizophrenia and was significantly associated with students' perception that people with this diagnosis are unpredictable. CONCLUSIONS: These findings confirm that, in a sample of medical students, biogenetic causal explanations and diagnostic labeling have negative effects on beliefs about schizophrenia. They highlight the need to educate medical students about recovery from and stigma related to schizophrenia.