Complexity of needs in amyotrophic lateral sclerosis (ALS) patients using the ENP-E scale in the north-eastern region of Spain.

OBJECTIVES: This study aimed to explore the clinical characteristics of amyotrophic lateral sclerosis (ALS) patients in Spain's north-eastern region, their inclusion in chronic care programmes, and their psychosocial and spiritual needs (PSNs). METHODS: A longitudinal descriptive study in adult patients with ALS. We analyzed clinical variables and participation in chronicity and PSNs assessment using the tool Psychosocial and Spiritual Needs Evaluation scale in end-of-life patients (ENP-E scale). RESULTS: 81 patients (average age 65.6 ± 11.7) were studied. At the study's outset, 29.7% employed non-invasive ventilation (NIV), increasing to 51.9% by its conclusion. Initial percutaneous endoscopic gastrostomy (PEG) utilization was 14.8%, rising to 35.85%. Chronic care programme participation was as follows: home care (24.7% initially, 50.6% end), palliative care (16% initially, 40.7% end), case management (13.6% initially, 50.6% end), and advance care planning registration (6.2% initially, 35.8% end). At study start, 47.8% of patients (n = 46) showed moderate-to-severe complexity in PSNs assessment using the ENP-E scale, without showing differences in age, sex, and time of evolution; whereas, on the evolutionary analysis, it was 75% (n = 24). A higher evolutionary complexity was observed in males <60 and >70 years, with no PEG and evolution of ALS of <2 and ≥5 years, and not included in chronicity programmes. When assessing concerns, physical pain and family aspects stand out in all measurements. Forty-eight percent of patients at study start and 71% at end of study showed external signs of emotional distress. SIGNIFICANCE OF RESULTS: Most ALS patients showed a high degree of complexity and were not integrated in chronicity programmes. A "care path" is proposed to integrate ALS patients in these programmes and systematically assess their needs.

La esclerosis lateral amiotrófica (ELA) desde la Atención Primaria. Epidemiología y características clínico-asistenciales / Amyotrophic lateral sclerosis (ALS) from the perspective of Primary Care. Epidemiology and clinical-care characteristics

La ELA es una enfermedad poco frecuente en atención primaria (AP), representa un desafío para el médico de familia especialmente en atención domiciliaria. Objetivo: Conocer la incidencia y prevalencia de la ELA en un área de gestión de AP, las características clínicas y la utilización de recursos sanitarios. Diseño: Estudio observacional. Emplazamiento: Dirección de AP Costa de Ponent, Región Sanitaria Metropolitana Sur, Barcelona, Cataluña, España. Participantes: Pacientes con ELA ≥18 años diagnosticados hasta el 01/03/2017. Mediciones principales: Edad, sexo, características: forma de inicio (espinal, bulbar, otras), intervalo entre inicio de síntomas y diagnóstico, portadores gastrostomía percutánea, ventilación no invasiva o invasiva. Identificación en AP como paciente crónico complejo o con necesidades paliativas. Inclusión en programas de atención domiciliaria (PAD). Modelo de atención hospitalario. Resultados: Ochenta y un pacientes, edad media 65,6 años (±11,7), varones 49,4%. Forma de inicio: espinal 69%, bulbar 21%, otras 4%. Intervalo entre inicio de síntomas y diagnóstico 12 meses. Identificados como paciente crónico complejo o con necesidades paliativas 13,6%, incluidos en PAD 29 pacientes (35,8%). Atendidos en modelo hospitalario integral 79 pacientes (97,5%). Prevalencia 6,1/100.000 habitantes en 2017. Incidencia anual entre 1,2 casos/100.000 habitantes/año en 2012 y 3,5 casos/100.000 habitantes/año en 2016. Conclusiones: Utilizar gastrostomía percutánea en la ELA favorece la identificación como paciente crónico complejo o con necesidades paliativas e inclusión en PAD. Utilizar ventilación no invasiva favorece la inclusión en PAD. Los datos de incidencia y prevalencia de ELA son superiores a los descritos previamente en la misma área. Es necesaria la identificación precoz de estos pacientes en los modelos de atención a la cronicidad en equipos de AP.(AU)

Amyotrophic Lateral Sclerosis (ALS) is a rare disease in primary care (PC), it represents a challenge for the family doctor, especially in home care. Objective: To know the incidence and prevalence of ALS in an area of ??PA management, the clinical characteristics and use of health resources. Design: Observational study. Location: PC-Direction Costa de Ponent, South Metropolitan Health Region, Barcelona, Catalonia, Spain. Participants: Patients with ALS ≥18 years diagnosed until 03/01/2017. Main measurements: Age, sex, characteristics: form of appearance (spinal, bulbar, others), interval between onset of symptoms and diagnosis, percutaneous gastrostomy carriers, ventilation non-invasive or invasive. Identification in PC as a Complex Chronic Patient or with palliative needs (CCP). Inclusion in home care programs (PAD). Model of attention hospitable. Results: 81 patients, mean age 65.6 years (± 11.7), men 49.4%. Shape of onset: spinal 69%, bulbar 21%, another 4%. Interval between the onset of symptoms and diagnosis 12 months. Identified as a CCP 13.6%, 29 patients (35.8%) included in PAD. Attended in comprehensive hospital model 79 patients (97.5%). Prevalence 6.1/100,000 inhabitants in 2017. Annual incidence between 1.2 cases/100,000 inhabitants/year in 2012 and 3.5 cases/100,000 inhabitants/year in 2016. Conclusions: The use of percutaneous gastrostomy in ALS favors the identification as CCP or with palliative needs and inclusion in PAD. The use of non-invasive ventilation favors inclusion in PAD. The incidence and prevalence data for ALS are higher than those described above in the same area. Early identification is necessary of these patients in the chronic care models in PC teams.(AU)

[Amyotrophic lateral sclerosis (ALS) from the perspective of Primary Care. Epidemiology and clinical-care characteristics]. / La esclerosis lateral amiotrófica (ELA) desde la Atención Primaria. Epidemiología y características clínico-asistenciales.

Amyotrophic Lateral Sclerosis (ALS) is a rare disease in primary care (PC), it represents a challenge for the family doctor, especially in home care. OBJECTIVE: To know the incidence and prevalence of ALS in an area of ??PA management, the clinical characteristics and use of health resources. DESIGN: Observational study. LOCATION: PC-Direction Costa de Ponent, South Metropolitan Health Region, Barcelona, Catalonia, Spain. PARTICIPANTS: Patients with ALS ≥18 years diagnosed until 03/01/2017. Main measurements Age, sex, characteristics: form of appearance (spinal, bulbar, others), interval between onset of symptoms and diagnosis, percutaneous gastrostomy carriers, ventilation non-invasive or invasive. Identification in PC as a Complex Chronic Patient or with palliative needs (CCP). Inclusion in home care programs (PAD). Model of attention hospitable. RESULTS: 81 patients, mean age 65.6 years (± 11.7), men 49.4%. Shape of onset: spinal 69%, bulbar 21%, another 4%. Interval between the onset of symptoms and diagnosis 12 months. Identified as a CCP 13.6%, 29 patients (35.8%) included in PAD. Attended in comprehensive hospital model 79 patients (97.5%). Prevalence 6.1/100,000 inhabitants in 2017. Annual incidence between 1.2 cases/100,000 inhabitants/year in 2012 and 3.5 cases/100,000 inhabitants/year in 2016. CONCLUSIONS: The use of percutaneous gastrostomy in ALS favors the identification as CCP or with palliative needs and inclusion in PAD. The use of non-invasive ventilation favors inclusion in PAD. The incidence and prevalence data for ALS are higher than those described above in the same area. Early identification is necessary of these patients in the chronic care models in PC teams.

Tratamiento sintomático de la disnea en fibrosis pulmonar idiopática: utilización de fentanilo inhalado en atención domiciliaria / Symptomatic treatment of dyspnea in idiopathic pulmonary fibrosis: Inhaled fentanyl use in home care

Este caso clínico relata la complejidad de la atención paliativa domiciliaria en un paciente con disnea. Este paciente está afecto de fibrosis pulmonar idiopática, proceso patológico en el que la disnea puede ser un síntoma de difícil control. La poca tolerancia al tratamiento con sulfato de morfina oral nos obligó a buscar alternativas. Queremos exponer nuestra experiencia con fentanilo inhalado en un paciente con disnea muy importante

This case reflects the complexity of home palliative care in a patient with dyspnea. A case is presented of a patient with idiopathic pulmonary fibrosis, in which the dyspnea was difficult to control. The low tolerance to treatment with oral morphine sulfate, led to looking for alternatives. Experience with inhaled fentanyl in a patient with significant symptomatic dyspnea is reported