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The burden of familial chylomicronemia syndrome from the patients' perspective.
Gelrud, Andres; Williams, Karren R; Hsieh, Andrew; Gwosdow, Andrea R; Gilstrap, Alan; Brown, Alan.
Afiliación
  • Gelrud A; a Center for Pancreatic Disorders , University of Chicago , Chicago , IL , USA.
  • Williams KR; b Akcea Therapeutics Inc, A Subsidiary of Ionis Pharmaceuticals , Cambridge , MA , USA.
  • Hsieh A; b Akcea Therapeutics Inc, A Subsidiary of Ionis Pharmaceuticals , Cambridge , MA , USA.
  • Gwosdow AR; b Akcea Therapeutics Inc, A Subsidiary of Ionis Pharmaceuticals , Cambridge , MA , USA.
  • Gilstrap A; b Akcea Therapeutics Inc, A Subsidiary of Ionis Pharmaceuticals , Cambridge , MA , USA.
  • Brown A; c Division of Cardiology , Advocate Lutheran General Hospital , Park Ridge , IL , USA.
Expert Rev Cardiovasc Ther ; 15(11): 879-887, 2017 Nov.
Article en En | MEDLINE | ID: mdl-28847199
BACKGROUND: Familial chylomicronemia syndrome (FCS) is a rare, inherited lipid disorder characterized by high levels of plasma triglycerides and chylomicrons, which may cause life-threatening acute pancreatitis. Currently no FDA-approved treatment exists. Management is low-fat diet (<20g fat/day), which is difficult to maintain. With the restricted diet, triglycerides may remain elevated. We conducted discussions with patients and caregivers to better understand the burden of FCS from their perspectives. METHODS: A panel of FCS patients and caregivers was assembled to discuss and assess the clinical and psychosocial burden of FCS. RESULTS: Ten adults with FCS (median age 48 yr) and their spouses/caregivers were asked specific questions about their experiences living with FCS. Patients with FCS stated their symptoms were abdominal pain, nausea, diarrhea, constipation, bloating, and fatigue. Patients reported a median of 34 episodes of acute pancreatitis over their lifetimes; half of these led to hospitalizations, each with an average stay of 6.5 days. The psychosocial burden of FCS was primarily associated with the restricted diet, anxiety and stress of FCS. CONCLUSIONS: Living with FCS imposes a significant clinical and psychosocial burden on patients and caregivers, who reported reduced quality of life, limited employment opportunities, socialization and increased burden on family.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Calidad de Vida / Triglicéridos / Quilomicrones / Hiperlipoproteinemia Tipo I Aspecto: Patient_preference Límite: Adult / Aged / Female / Humans / Male / Middle aged Idioma: En Revista: Expert Rev Cardiovasc Ther Asunto de la revista: ANGIOLOGIA / CARDIOLOGIA / TERAPEUTICA Año: 2017 Tipo del documento: Article País de afiliación: Estados Unidos Pais de publicación: Reino Unido
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Calidad de Vida / Triglicéridos / Quilomicrones / Hiperlipoproteinemia Tipo I Aspecto: Patient_preference Límite: Adult / Aged / Female / Humans / Male / Middle aged Idioma: En Revista: Expert Rev Cardiovasc Ther Asunto de la revista: ANGIOLOGIA / CARDIOLOGIA / TERAPEUTICA Año: 2017 Tipo del documento: Article País de afiliación: Estados Unidos Pais de publicación: Reino Unido