The burden of common variable immunodeficiency disorders: a retrospective analysis of the European Society for Immunodeficiency (ESID) registry data.

BACKGROUND: Common variable immunodeficiency disorders (CVID) are a group of rare innate disorders characterized by specific antibody deficiency and increased rates of infections, comorbidities and mortality. The burden of CVID in Europe has not been previously estimated. We performed a retrospective analysis of the European Society for Immunodeficiencies (ESID) registry data on the subset of patients classified by their immunologist as CVID and treated between 2004 and 2014. The registered deaths and comorbidities were used to calculate the annual average age-standardized rates of Years of Life Lost to premature death (YLL), Years Lost to Disability (YLD) and Disability Adjusted Life Years (DALY=YLL + YLD). These outcomes were expressed as a rate per 105 of the CVID cohort (the individual disease burden), and of the general population (the societal disease burden). RESULTS: Data of 2700 patients from 23 countries were analysed. Annual comorbidity rates: bronchiectasis, 21.9%; autoimmunity, 23.2%; digestive disorders, 15.6%; solid cancers, 5.5%; lymphoma, 3.8%, exceeded the prevalence in the general population by a factor of 34.0, 7.6, 8.1, 2.4 and 32.6, respectively. The comorbidities of CVID caused 8722 (6069; 12,363) YLD/105 in this cohort, whereas 44% of disability burden was attributable to infections and bronchiectasis. The total individual burden of CVID was 36,785 (33,078, 41,380) DALY/105. With estimated CVID prevalence of ~ 1/ 25,000, the societal burden of CVID ensued 1.5 (1.3, 1.7) DALY/105 of the general population. In exploratory analysis, increased mortality was associated with solid tumor, HR (95% CI): 2.69 (1.10; 6.57) p = 0.030, lymphoma: 5.48 (2.36; 12.71) p < .0001 and granulomatous-lymphocytic interstitial lung disease: 4.85 (1.63; 14.39) p = 0.005. Diagnostic delay (median: 4 years) was associated with a higher risk of death: 1.04 (1.02; 1.06) p = .0003, bronchiectasis: 1.03 (1.01; 1.04) p = .0001, solid tumor: 1.08 (1.04; 1.11) p < .0001 and enteropathy: 1.02 (1.00; 1.05) p = .0447 and stayed unchanged over four decades (p = .228). CONCLUSIONS: While the societal burden of CVID may seem moderate, it is severe to the individual patient. Delay in CVID diagnosis may constitute a modifiable risk factor of serious comorbidities and death but showed no improvement. Tools supporting timely CVID diagnosis should be developed with high priority.

Nurse-led telecoaching of people with type 2 diabetes in primary care: rationale, design and baseline data of a randomized controlled trial.

BACKGROUND: Despite the efforts of the healthcare community to improve the quality of diabetes care, about 50% of people with type 2 diabetes do not reach their treatment targets, increasing the risk of future micro-and macro-vascular complications. Diabetes self-management education has been shown to contribute to better disease control. However, it is not known which strategies involving educational programs are cost-effective. Telehealth applications might support chronic disease management. Transferability of successful distant patient self-management support programs to the Belgian setting needs to be confirmed by studies of a high methodological quality. "The COACH Program" was developed in Australia as target driven educational telephone delivered intervention to support people with different chronic conditions. It proved to be effective in patients with coronary heart disease after hospitalization. Clinical and cost-effectiveness of The COACH Program in people with type 2 diabetes in Belgium needs to be assessed. METHODS/DESIGN: Randomized controlled trial in patients with type 2 diabetes. Patients were selected based on their medication consumption data and were recruited by their sickness fund. They were randomized to receive either usual care plus "The COACH Program" or usual care alone. The study will assess the difference in outcomes between groups. The primary outcome measure is the level of HbA1c. The secondary outcomes are: Total Cholesterol, LDL-Cholesterol, HDL-Cholesterol, Triglycerides, Blood Pressure, body mass index, smoking status; proportion of people at target for HbA1c, LDL-Cholesterol and Blood Pressure; self-perceived health status, diabetes-specific emotional distress and satisfaction with diabetes care. The follow-up period is 18 months. Within-trial and modeled cost-utility analyses, to project effects over life-time horizon beyond the trial duration, will be undertaken from the perspective of the health care system if the intervention is effective. DISCUSSION: The study will enhance our understanding of the potential of telehealth in diabetes management in Belgium. Research on the clinical effectiveness and the cost-effectiveness is essential to support policy makers in future reimbursement and implementation decisions.