Home care models dedicated to COVID-19 patients: the experience of a Local Health District of Veneto Region (Italy).

Background: During COVID-19 pandemic, health professionals have been working in an extreme uncertainty context. Affected patients needed to be cared at home as long as possible to avoid virus spreading and hospital resources saturation. The Veneto Regional Administration (North-east of Italy) released Regional guidelines about it. The Western Healthcare District of the Local Health Authority of the city of Vicenza (180,000 inhabitants) implemented a healthcare pathway following them. Aim of the study is to describe the results and outcomes of such implementation. Methods: In the implemented health care pathway, a new service called "Special Unit of continuity of care" (USCA) with physicians and nurses has been dedicated to the prise en charge at home of patients suffering from Sars-CoV-2. They were referred to the USCA by general practitioners or by hospital specialists, and managed through a daily clinical monitoring by regular home visits and phone calls, specialist consultations and therapy management. In order to prevent hospital admission, an oxygen concentrator when possible has been employed and managed at home by the members of the USCA when the oxygen saturation was below 93%. An observational retrospective study has been conducted using anonymized data from different databases: the USCA activity database (from 12/01/20 to 21/31/21), the hospital and Emergency Department discharge databases, and the "healthcare co-payments exemptions database". The latter database refers to the people excluded - because of their chronicity - from the co-payment of a list of medical exams and services. Descriptive and multivariate logistic regression analyses have been implemented. Results: 1,419 patients suffering from Sars-CoV-2 have been cared and managed by the USCA in the considered period of time (mean 11.4 days), of whom 787 (55.5%) with at least one chronic condition (described in the above quoted "healthcare co-payments exemption database") and 261 provided with oxygen concentrator. 275 (19.4%) needed a hospital admission, 39 (2.8%) in intensive unit; 53 died during hospitalization (3.8%). Out of the 261 patients utilizing oxygen concentrator, 103 have been admitted to hospital (39.5%), 7.3% in intensive unit and 8.0% died. In implemented multivariate analyses, the use of oxygen concentrator, proxy measure of the severity of the condition, is the major determinant for the risk of hospital admission (adj OR: 3.2, CI 2.3-4.3) and of dying within 30 days (adj OR: 2.8 CI 1.5-5.1). Among the 261 patients provided with oxygen concentrator, 158 (60,5%) have been managed at home without any admission to emergency department and/or hospitalization. Conclusions: In an uncertain context such as COVID-19 pandemic, the already-implemented home care model has been modified by integrating the USCA physicians and nurses and specialist care networks to prevent hospitalization and the sense of isolation and abandonment of people as much as possible. Almost 1,500 patients suffering from COVID-19 have been cared for at home over 13 months by such new service with complex and multidisciplinary activities. The risk of hospitalization and death appears determined by the severity of the pathology with high and significant OR 60% of patients with oxygen concentrators who, despite an initial high hyposaturation were not hospitalized, represent, partly, the group of patients who would have been requiring hospital care in the absence of a home care pathway in a standard situation.

High-cost drugs for rare diseases: their expenditure and value based on a regional area-based study.

Background: in the field of rare diseases (RDs) most of the European studies on budget impact analysis of drugs that have been conducted often lay on theoretical assumptions and focus only on Orphan drugs (ODs). Objectives: we aimed to estimate the budget impact of specific drugs for non-oncological RDs, both ODs and non-ODs, using real-world data about patients residing in Veneto Region (Italy) and to describe its expenditure structure and dynamics. Methods: a population-based multi-source observational study was conducted using data from Regional administrative databases; an ad-hoc drugs' list specific for RDs including both ODs and non-ODs and classifying them by ATC codes has been created. Results: In 2019, the total expenditure for drugs specific for RDs was EUR 97.2 million (6.6% of the total Regional budget). The RD drug list included 58 ATC codes, of which 15 ATC had an annual budget impact over EUR 1 million ("blockbuster drugs"). The most expensive treatment was a non-OD drug (Coagulation factor VIII). The two most represented therapeutical areas were the metabolic and the hematological ones. Conclusions: Cost analyses on RD high-cost drugs expenditure should consider any specific RD drug, not only ODs. Expenditure dynamics for RD drugs are peculiar showing "blockbuster drugs". Some therapeutical areas seem to be lacking in the drug research field.

[Telemonitoring patients with chronic heart failure. Results from a pilot study in the Veneto Region, Italy]. / Il telemonitoraggio territoriale dei pazienti affetti da scompenso cardiaco cronico. I risultati di uno studio pilota nel Veneto.

BACKGROUND: Remote monitoring of patients with chronic heart failure (HF) can prevent acute episodes of HF, optimize treatment, reduce emergency room (ER) visits and hospitalizations, and improve quality of life of patients and caregivers. In current clinical practice, however, the most appropriate model is still under debate. We aimed to evaluate the impact of a new remote telemonitoring (TM) system in the clinical management of HF patients on the reduction of hospitalizations and ER admissions and on possible related-economic benefits. METHODS: A working group participated by primary care and family practitioners, cardiologists, home care nurses of the 8th Local Health Unit of the Veneto Region, Italy, has established a new operating procedure for TM chronic HF patients, identifying the clinical profiles and the inclusion/exclusion criteria of enrollment, the clinical parameters to be monitored, the input/output modalities of the information to/from the call center, the alarm criteria and the response procedures. Patients were profiled into three risk categories based on age, clinical characteristics, prognosis assessment, and social needs and provided with appropriate devices for remote measurement and transmission of blood pressure, heart rate, peripheral oxygen saturation (SpO2) and body weight. Clinical data, self-measured at home according to a predetermined schedule based on the patient's risk profile, were automatically transmitted to the operating center, generating, if above the threshold, a grading of alarms (green/cyan, yellow, red) and the consequent activation of the most appropriate response (returned phone calls to check patient's status, reporting to primary physician/family practitioner for clinical evaluation, notification to the HF outpatient clinic for an early follow-up visit, alerting medical emergency services). The number of hospitalizations and ER visits during the TM period was compared with the pre-TM equivalent for each individual patient. RESULTS: Overall, 22 patients with chronic HF were enrolled (mean age 82.3 ± 8.6 years, 17 males). The average duration of TM was 18.7 ± 8.8 months. Overall, 62 108 home measurements were performed and transmitted. The alarms received by the operating center were 4120 (6.6% of all measurements): 62% cyan, 30% yellow, 8% red. Most of the data above the threshold concerned weight gain (42.0%) and SpO2 variations (28.0%). During the observation period (TM period and pre-TM equivalent interval calculated for each individual patient), a total of 127 hospitalizations and 181 ED visits were recorded. Compared to the pre-TM period, there was a 66% reduction in hospitalizations for all causes (95 vs 32, p<0.001) and a 68% decrease in ED visits (137 vs 44, p<0.001). Similarly, hospitalizations and ED visits due to HF were reduced by 82% (p<0.001) and 66% (p<0.001), respectively, with a decrease in days of HF-related hospitalization from 211 to 48. Despite a higher cost for TM of € 6911.15, a total saving of € 64 103.87 was obtained for the reduction of expenses from € 8665.45 to € 2664.00 for ED visits and from € 71 627.93 to € 13 525.51 for HF hospitalizations. CONCLUSIONS: This pilot experience on remote monitoring HF patients proved to be effective in determining a significant reduction in ED visits and hospitalizations with a consequent significant economic benefit. Larger studies are needed to confirm this favorable outcome.

Exposure to PFAS and small for gestational age new-borns: A birth records study in Veneto Region (Italy).

BACKGROUND: Perfluoroalkylated substances (PFAS) in serum are inversely associated with fetal growth. Small for gestational age (SGA) is a measure based on birth weight and gestational age at birth and represents a good indicator of fetal growth but it has been used only in a small number of studies. We examined the association between PFAS exposure and the risk of severe SGA in a PFAS contaminated area in the Veneto Region (North-East of Italy). METHODS: A retrospective cohort study has been developed including all singleton live births reported in the Veneto Region Birth Registry between 2003 and 2018 to mothers living in the contaminated and in a control area. We estimated the association between mothers' area of residence and severe SGA using crude RR (and 95% CI) and stepwise logistic regression, including all the maternal characteristics. RESULTS: The study included 105,114 singleton live births. The occurence of severe SGA was 3.44% in the contaminated area and 2.67% in the control area. The multivariate analysis confirmed that living in the contaminated area significantly increased the odds of severe SGA (adjusted OR 1.27 (95% C.I. 1.16, 1.39)). CONCLUSIONS: The findings suggest that living in a contaminated area by PFAS plays a role in affecting fetal growth and support the hypothesis that PFAS exposure is a risk factor for SGA. Individual data on exposure are needed to confirm the direct association.

The Epidemiology of Transition into Adulthood of Rare Diseases Patients: Results from a Population-Based Registry.

Background: Despite the fact that a considerable number of patients diagnosed with childhood-onset rare diseases (RD) survive into adulthood, limited information is available on the epidemiology of this phenomenon, which has a considerable impact both on patients' care and on the health services. This study describes the epidemiology of transition in a population of RD patients, using data from the Veneto Region Rare Diseases Registry (VRRDR), a web-based registry monitoring since 2002 a consistent number of RD in a defined area (4.9 million inhabitants). Methods: Longitudinal cohorts of patients born in the years 1988 to 1998 and enrolled in the VRRDR in their paediatric age were identified. Data referred to this group of patients, experiencing transition from paediatric to adult age during the years 2006⁻2016, are presented. Results: 2153 RD patients (44.1% females and 55.9% males) passed from childhood to adulthood in the study period, corresponding to a 3-fold increase from 2006 to 2016. The majority of these patients was affected by congenital anomalies (32.0%), by hematologic diseases (15.9%), eye disorders (12.1%) and neoplasms (7.9%). RD patients who experienced transition from paediatric age to adulthood represent the 9.2% of adult patients enrolled in the Registry at 31 December 2016. Conclusions: We described a subset of RD young adults experiencing transition into adulthood. The data reported can be considered as minimum values for estimating the size of this increasing population presenting specific transition needs. These figures are valuable for clinicians, patients and health planners. Public policy interventions are needed in order to promote dedicated care transition pathways in the broader framework of health policies devoted to RD.

Diagnosis of pervasive developmental disorders: when and how? An area-based study about health care providers.

BACKGROUND: Pervasive developmental disorders (PDDs) can be very difficult to diagnose in children and to communicate such a diagnosis to their parents. Families of children with PDD learn of their child's diagnosis long after the first symptoms are noted in the child's behavior. METHODS: An area-based survey was conducted to assess all social and health care providers taking care of patients with PDDs in the Veneto Region (North-East Italy). RESULTS: Only 28% of health care providers arrived at a definite diagnosis when the child was in his/her first year of age, 51% when the child was 2-3 years old and 21% from age of 4 years and up. On average, the latency between the time of the diagnosis and its communication to the family was 6.9 months. However, a number of families did not ever have a diagnosis communicated to them. Sometimes, 68% of the providers did not communicate a PDDs diagnosis to patient's families, and 4% of them quite commonly. CONCLUSION: The well-known delay in making a diagnosis of PDDs has two distinct components: one relating to the difficulty of confirming a diagnosis of PDDs, the other, hitherto unrecognized, relating to the family being notified.

A population-based registry as a source of health indicators for rare diseases: the ten-year experience of the Veneto Region's rare diseases registry.

BACKGROUND: Although rare diseases have become a major public health issue, there is a paucity of population-based data on rare diseases. The aim of this epidemiological study was to provide descriptive figures referring to a sizable group of unrelated rare diseases. METHODS: Data from the rare diseases registry established in the Veneto Region of north-east Italy (population 4,900,000), referring to the years from 2002 to 2012, were analyzed. The registry is based on a web-based system accessed by different users. Cases are enrolled by two different sources: clinicians working at Centers of expertise officially designated to diagnose and care patients with rare diseases and health professionals working in the local health districts. Deaths of patients are monitored by Death Registry. RESULTS: So far, 19,547 patients with rare diseases have been registered, and 23% of them are pediatric cases. The overall raw prevalence of the rare diseases monitored in the population under study is 33.09 per 10,000 inhabitants (95% CI 32.56-33.62), whilst the overall incidence is 3.85 per 10,000 inhabitants (95% CI 3.67-4.03). The most commonly-recorded diagnoses belong to the following nosological groups: congenital malformations (Prevalence: 5.45/10,000), hematological diseases (4.83/10,000), ocular disorders (4.47/10,000), diseases of the nervous system (3.51/10,000), and metabolic disorders (2,95/10,000). Most of the deaths in the study population occur among pediatric patients with congenital malformations, and among adult cases with neurological diseases. Rare diseases of the central nervous system carry the highest fatality rate (71.36/1,000). Rare diseases explain 4.2% of general population Years of Life Lost (YLLs), comparing to 1.2% attributable to infectious diseases and 2.6% to diabetes mellitus. CONCLUSIONS: Our estimates of the burden of rare diseases at population level confirm that these conditions are a relevant public health issue. Our snapshot of their epidemiology is important for public health planning purposes, going to show that population-based registries are useful tools for generating health indicators relating to a considerable number of rare diseases, rather than to specific conditions.

Visual, motor, and psychomotor development in small-for-gestational-age preterm infants.

PURPOSE: To study causal links between the visual, cognitive, and psychomotor outcomes of premature babies who are small for gestational age (SGA). METHODS: A cohort study of 17 SGA cases and 34 controls who were appropriate for gestational age (AGA) was carried out. The cases were all premature babies without any other pathology. All subjects underwent a visual, mental, and psychomotor evaluation at 1 year of age. RESULTS: Of the SGA cases, 41% had a "below normal" visual acuity versus 17.7% of the AGA controls. At 1 year of age the SGA babies showed an odds ratio of 18.73 for low visual acuity, 9.09 for low mental performance, and no significant risk for a decreased psychomotor performance. CONCLUSIONS: In this small cohort of premature infants, the SGA babies were more prone to developing low visual performance and abnormal cognitive development.

[Rehabilitation in the Veneto Region: towards a logic of clinical governance]. / L'offerta assistenziale riabilitativa nella Regione Veneto: verso una logica di governo clinico.

OBJECTIVES: to describe the rehabilitation services provided by the health system in the Veneto Region (4.7 million population) in terms of their organizational setting, the number of patients rehabilitated, the activities involved, the human resources and the costs to the healthcare system. DESIGN AND SETTING: a cross-sectional study based on several approaches. Rehabilitation activities for inpatients were explored using hospital discharge records, while territorial activities were assessed from specific data covering accredited centers and an ad hoc questionnaire completed by each rehabilitation center. Indexes of the rehabilitation services provided were calculated for each Local Health Agency and Region. The rehabilitation activities, the number of patients involved and the service provided by each center were explored by multiple correspondence analysis. MAIN OUTCOME MEASURES: the organizational setting and workload of the rehabilitation centers, the services delivered and activities involved, the human resources employed and the estimated costs to the healthcare system. RESULTS: in 2002, 320 rehabilitation centers (80.6% of them for outpatients) and 2,500 full-time equivalent personnel took care of about 300,000 patients delivering more than 5.5 million treatment sessions. Patients were under 17 years old in 19.3% of cases. The service varied considerably within and between Local Health Agencies. Rehabilitation mainly concerned physical (58.7%) and psychological treatments (19.1%) and speech therapy (3.7%). The rehabilitation facilities dedicated to one particular activity tended to take care of a large number of patients with a low complexity profile, e.g. those needing physical exercises, while facilities providing services that demand a multidisciplinary approach dealt with fewer patients, who were mostly children. The total cost of rehabilitation was estimated at 659 million Euro. CONCLUSION: rehabilitation activities have a great impact on health service users and providers, with related costs that were estimated to be about one third of the amount normally allocated to hospital services. There was no homogeneity of the facilities provided for outpatients in terms of type of rehabilitation, number of patients, services provided, and resources used Although activities involving outpatients represented the vast majority of the service provided, no systematic data are currently available on this area. A data flow on outpatient rehabilitation would afford a unique opportunity for clinical governance and cost containment.

Italian experience of pediatric liver transplantation.

The SIGENP Group has created an Italian Liver Transplantation database. The study considers all patients under 18 yr of age on the waiting list or transplanted between 1984 and 2005. Demographic and clinical data were collected and a descriptive analysis was conducted. Kaplan-Meier survival curves were calculated and Cox's proportional-hazards regression analysis were performed to identify predictors of death after transplantation. Twenty-two Italian centers took part and data were collected on 622 cases: only 53.8% of the transplants performed up until 1998 were carried out in Italy, while this was true of 97.7% of the operations performed between 1999 and 2005. Recipient survival curve analysis revealed one-, two- and five-yr survival rates of 88, 87 and 84%, respectively, and a significant improvement in survival after 1998 (p = 0.0322). Cox's analysis identified the following risk factors for death after liver transplantation, i.e. transplantation before 1998, neoplasms or fulminant hepatic failure as indications, being in intensive care at the time of transplantation and retransplantation. The center where the transplant is performed also revealed an influence on patient survival. Thanks to a better patient follow-up and more cooperation between specialists, the mean survival after liver transplantation is improving and Italian children can be transplanted in Italy.

[How current data can guide mother-infant health planning. An insight into data sources and methodologies]. / Può l'informazione corrente orientare la programmazione per l'area materno-infantile? Fonti e metodi a confronto.

OBJECTIVES: To evaluate how Hospital Discharge Records (SDO) and Certificates of Delivery Care (CEDAP) can be utilized to determine the number of deliveries and births; to calculate indicators for monitoring mother-infant health status, the exposure to risk factors during pregnancy and the health care provided. DESIGN AND SETTING: CEDAP and SDO of all patients admitted to any hospital in the Veneto Region (4.7 million inhabitants, about 44,000 births) during the year 2003 were considered. An area-based study on deliveries and births, a retrospective cohort study on pregnancies, and a prospective cohort study on newborns up to the first year of age were performed to calculate health indicators. These indicators were compared to two official data sources: National Institute of Statistics (ISTAT) and Italian National Institute of Health (ISS). MAIN OUTCOME MEASURES: Descriptive indicators concerning mother-infant health status (conception, pregnancy, delivery, newborns and events during the first year of age). RESULTS: SDO provide highly accurate data on pregnancies, births and stillborns, which overlap with values reported by ISTAT Combining data from SDO and CEDAP with findings fom the process of tracking mothers and infants'cohorts, allows the calculation of many indicators on conception (n. 9), pregnancy (n. 8), delivery (n. 8), newborn (n. 15), and events during the first year of age (n. 6). In the Veneto Region the general fertility rate is 40.95% per hundred, with a relatively late mean age at delivery (32 years). Pregnant women undergoing prenatal invasive procedures are 23.5% and the caesarean section rate is 29%. Infants born to foreign mothers are 16%, whereas 2.5% are conceived through assisted reproduction techniques, 0.9% are very low birth weight, and 0.3% are extremely low birth weight; neonatal and infant mortality rates are 2.2% per hundred and 2.9% per hundred respectively. CONCLUSION: The proposed system is immediately feasible also at a local level, making reliable and informative data available to guide health policies.