A patient journey map for people living with autoimmune pulmonary alveolar proteinosis

Abstract

Introduction

Patients with autoimmune pulmonary alveolar proteinosis (PAP) face a complicated journey (physically, emotionally, and financially) to receive the correct diagnosis and treatment. We developed a patient journey map (PJM) to describe the experiences and needs of patients with autoimmune PAP in the USA.

Methods

This PJM was developed in four stages: (1) analysis of existing literature; (2) patient advisory board meetings (n = 7); (3) an online survey (n = 19); and (4) a validation workshop (n = 6).

Results

Four phases of the patient journey were identified: (1) symptoms and experience before diagnosis; (2) diagnosis; (3) treatment; and (4) ongoing monitoring. Patients reported heterogeneous and indirect diagnostic pathways, often waiting months or years for the correct diagnosis. The majority reported at least one misdiagnosis, most commonly pneumonia. Treatment pathways varied substantially, and current treatments and off-label therapies were frequently described as burdensome, emotionally taxing, and/or financially worrisome. Patients described their journey as an “emotional rollercoaster,” especially during pre-diagnosis and treatment. Patients reported common barriers to care, particularly insurance problems and access to expert care. Patients specifically cited the need for improved education on autoimmune PAP within the medical community and increased help with insurance challenges related to current treatments.

Conclusions

This PJM provides insights on patients’ journeys with autoimmune PAP. Patients reported inconsistent, burdensome, and circuitous journeys. This PJM provides the medical community with valuable information on patients’ needs and increases awareness of this rare disease. Over time, these factors may improve diagnosis, treatment, and the holistic experience of patients with autoimmune PAP.