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Role of Palliative Care in the Supportive Management of AL Amyloidosis—A Review

Authors :
Muhammad Hamza Habib
Yun Kyoung Ryu Tiger
Danai Dima
Mathias Schlögl
Alexandra McDonald
Sandra Mazzoni
Jack Khouri
Louis Williams
Faiz Anwer
Shahzad Raza
Source :
Journal of Clinical Medicine, Vol 13, Iss 7, p 1991 (2024)
Publication Year :
2024
Publisher :
MDPI AG, 2024.

Abstract

Light chain amyloidosis is a plasma–cell disorder with a poor prognosis. It is a progressive condition, causing worsening pain, disability, and life-limiting complications involving multiple organ systems. The medical regimen can be complex, including chemotherapy or immunotherapy for the disease itself, as well as treatment for pain, gastrointestinal and cardiorespiratory symptoms, and various secondary symptoms. Patients and their families must have a realistic awareness of the illness and of the goals and limitations of treatments in making informed decisions about medical therapy, supportive management, and end-of-life planning. Palliative care services can thus improve patients’ quality of life and may even reduce overall treatment costs. Light chain (AL) amyloidosis is a clonal plasma cell disorder characterized by the excessive secretion of light chains by an indolent plasma cell clone that gradually accumulates in vital organs as amyloid fibrils and leads to end-organ damage. With progressive disease, most patients develop diverse clinical symptoms and complications that negatively impact quality of life and increase mortality. Complications include cardiac problems including heart failure, hypotension, pleural effusions, renal involvement including nephrotic syndrome with peripheral edema, gastrointestinal symptoms leading to anorexia and cachexia, complex pain syndromes, and mood disorders. The prognosis of patients with advanced AL amyloidosis is dismal. With such a complex presentation, and high morbidity and mortality rates, there is a critical need for the establishment of a palliative care program in clinical management. This paper provides an evidence-based overview of the integration of palliative care in the clinical management of AL amyloidosis as a means of reducing ER visits, rehospitalizations, and in-hospital mortality. We also discuss potential future collaborative directions in various aspects of clinical care related to AL amyloidosis.

Details

Language :
English
ISSN :
20770383
Volume :
13
Issue :
7
Database :
Directory of Open Access Journals
Journal :
Journal of Clinical Medicine
Publication Type :
Academic Journal
Accession number :
edsdoj.8229db53c24a93935320332a4076be
Document Type :
article
Full Text :
https://doi.org/10.3390/jcm13071991