Patient-centered chronic care management is a new model for the management of rare chronic diseases such as primary immunodeficiency disease (PIDD). This approach emphasizes helping patients become experts on the management of their disease as informed, involved, and interactive partners in healthcare decisions with providers. Because only a few patients are affected by rare illnesses, these patients are forced to become knowledgeable about their disease and therapies and to seek treatment from a healthcare team, which includes physicians and nurse specialists who are equipped to manage the complexity of the disease and its comorbidities. Importantly, therapy for PIDD can be self-administered at home, which has encouraged the transition toward a proactive stance that is at the heart of patient-centered chronic care management. We discuss the evolution of therapy, the issues with the disease, and challenges with its management within the framework of other chronic disease management programs. Suggestions and rationale to move case management of PIDD forward are presented with the intent that sharing our experiences will improve process and better manage outcomes in this patient population.
The patient-centered model for the management of PIDD is applicable to the primary care settings, where nurse case managers assist patients through education, support them and their families, and facilitate access to community resources in an approach, which has been described as “guided care.” The model also applies specifically to immunology centers where patients receive treatment or instruction on its self-administration at home.
Patient-centered management of PIDD, with its emphasis on full involvement of patients in their treatment, has the potential to improve compliance with treatment, and thus patient outcomes, as well as patients' quality of life.
The patient-centered model expands the traditional model of chronic disease management, which relies on evidence-based medicine, provider expertise, clinical information systems, and patient education. This approach supports patient self-management with strategies that empower and prepare them for their role as expert patients.
Janet Burton, MSc, RGN, is the Lead Nurse for Clinical Immunology for the National Health Service, Oxford, England. She has many years' experience of working with immunodeficient patients, particularly teaching self-infusion at home and providing ongoing support for patients to self-infuse at home.
Elyse Murphy, BSN, RN, is a Medical Science Liaison at CSL Behring, King of Prussia, Pennsylvania. She has over 30 years of nursing experience with chronic disease management, with 20 years' clinical experience in immunoglobulin therapy specializing in areas of immunology.
Patty Riley, RN, BAN, CRNI, is a Medical Science Liaison at CSL Behring, King of Prussia, Pennsylvania, supporting the therapeutic area of immunology. She has extensive clinical expertise with complex home infusion therapies. Clinical responsibilities have included healthcare provider and patient education of IVIg and SCIg for treatment of PIDD.
Address correspondence to Janet Burton, MSc, RGN, Clinical Immunology, L4 Academic Centre, John Radcliffe Hospital, Headington, Oxford OX3 9DU, England (email@example.com).
The authors have no conflict of interest.
Funding for this article was provided by CSL Behring.