RESUMEN
BACKGROUND: Acute hepatic porphyria (AHP) comprises a group of rare genetic diseases characterized by neurovisceral crises that are manifested by abdominal pain and neurological and/or psychological symptoms that interfere with the ability to lead a normal life. Our objective was to determine the burden of the disease in one year and the health-related quality of life (HRQoL) in patients with AHP. RESULTS: 28 patients were analyzed. The mean age was 36.6±10.2 years, 89.3% were women, and the average number of crises was 1.9±1.5. The average annual cost per patient was 38,255.40. 80.2% of the costs was direct medical costs, 17.5% was associated with loss of productivity and 2.3% was direct non-medical costs. 85.9% of the total cost corresponded to the crises. The intercrisis period accounted for the remaining 14.1%. The global index of the EQ-5D-5L (HRQoL) was 0.75±0.24. The dimensions of pain/discomfort, anxiety/depression and daily activities were the most affected. Leisure, travel/vacations and household activities were the most affected daily activities. 53.6% of patients required a caregiver due to AHP. 92.9% did not present overload and 7.1% presented extreme overload. CONCLUSIONS: Patients with AHP are associated with a high economic impact and an affected HRQoL in the pain/discomfort dimension, with a negative impact on the performance of daily activities and a risk of psychiatric diseases.
Asunto(s)
Porfirias Hepáticas , Calidad de Vida , Humanos , Femenino , Adulto , Persona de Mediana Edad , Masculino , Depresión/etiología , Costo de Enfermedad , Dolor/etiologíaRESUMEN
Introduction: Home parenteral nutrition (HPN) improves quality of life, allowing patients to receive nutrition at home and providing a social and labor integration to these patients. Objective: To assess the direct costs of HPN in adult population in Spain. Methods: A literature review of the records of HPN in Spain, carry out by NADYA-SENPE Group (years 2007-2014), was performed. The analysis included the evolution of: patients requiring HPN, number of episodes/patient, mean duration of episodes, description of delivery routes and complications rate. HPN consumption and cost were estimated. Patients were grouped according to their pathological group: benign and malignant. Direct costs (, 2015) included were: parenteral nutrition bags, delivery sets and costs due to complications. Results: The number of patients who receive HPN has increased over years (2007: 133 patients; 2014: 220 patients). The average number of episodes per patient ranged from 1-2 episodes per year. The average duration of those episodes decreased (2007: 323 days; 2014: 202.8 days). Tunneled catheters were the most used and septic complications were the most common. The average annual cost per patient was estimated at 8,393.30 and 9,261.60 for benign and malign disease respectively. Considering that 220 patients required HPN in 2014, an annual cost of 1,846.524.96 ( 1,389,910.55 directly due to HPN) and 2,037,551.90 ( 1,580,937.50 directly due to HPN) was estimated for patients with benign and malignant pathologies respectively. Conclusions: These results can be used to develop future economic evaluations on HPN and to establish effi cient prioritization strategies to allocate available resources.
Introducción: la nutrición parenteral domiciliaria (NPD) mejora la calidad de vida de los pacientes permitiéndoles recibir nutrición en su domicilio y facilitando su integración social y laboral. Objetivo: analizar el coste de la NPD en España. Métodos: se realizó una revisión bibliográfica de los Registros de NPD en España (años 2007-2014), elaborados por el Grupo NADYA-SENPE. Se analizó la evolución de: pacientes que requerían NPD, episodios/paciente por los que se administró NPD, duración media de los episodios, vías de acceso y tasa de complicaciones. Se estimó el consumo y coste de la NPD. Los pacientes fueron agrupados según patología: benigna o maligna. Los costes directos (, 2015) incluidos fueron: bolsas administradas, vías de acceso y complicaciones. Resultados: el número de pacientes que recibió NPD aumentó a lo largo de los años (2007: 133 pacientes; 2014: 220 pacientes). El número medio de episodios/paciente osciló entre 1-2 episodios/año y su duración media disminuyó (2007: 323 días; 2014: 202,8 días). Las vías de acceso más utilizadas fueron los catéteres tunelizados y las complicaciones sépticas fueron las más comunes. El coste directo anual medio por paciente se estimó en 8.393,30 y 9.261,60 para patología benigna y maligna, respectivamente. Considerando que, en 2014, 220 pacientes requirieron NPD, el coste anual fue 1.846.524,96 (1.389.910,55 debidos a la fórmula de NPD) y 2.037.551,90 (1.580.937,50 debidos a la fórmula de NPD) para patología benigna y maligna respectivamente. Conclusiones: estos resultados sirven de base para futuros análisis económicos de la NPD y para establecer estrategias de priorización eficiente de recursos disponibles.
