Primary immunodeficiency diseases: a primer for PCPs.

Primary care providers (PCPs) play a key role in identifying patients with primary immunodeficiency diseases (PIDDs). This diagnosis has implications for PCPs, as patients continue to require primary care and management after a PIDD diagnosis has been made. This review presents essential information for PCPs regarding PIDDs.

Subcutaneous immunoglobulin therapy home.

Subcutaneous immunoglobulin (SClg) replacement therapy is a new clinical service development in adult respiratory care undertaken at the Royal Brompton and Harefield Foundation Trust. SCIg therapy reduces appropriate patients' susceptibility to recurrent acute and chronic respiratory infections. Patients with specific chronic respiratory conditions and antibody deficiency can be treated with SCIg therapy at home, rather than with intravenous immunoglobulin (IVIg) therapy in hospital. Using a personalised teaching programme and managing the daily demands of the clinical service promotes greater concordance with treatment. With over 34% of our immunoglobulin therapy patients now on SCIg replacement therapy, this treatment innovation has brought about high levels of patient satisfaction and cost savings to trust budgets.

Primary immunodeficiency disease: a model for case management of chronic diseases.

PURPOSE/OBJECTIVES: 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. PRIMARY PRACTICE SETTINGS: 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. FINDINGS/CONCLUSIONS: 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. IMPLICATIONS FOR CASE MANAGEMENT: 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.

Developing practice guidelines for the administration of intravenous immunoglobulin.

As the use of intravenous immunoglobulin (IGIV) continues to expand, infusion nurses have a greater need for a comprehensive understanding of the product, patient risk factors, and comorbidities when developing guidelines for administering IGIV. Because immunoglobulin therapy is a blood derivative product, many nurses may not have as much experience administering this type of infusion. This article provides an in-depth overview of immunoglobulin therapy and helps to define the infusion nurse's major role in coordinating, assessing, and ensuring patient safety during IGIV administration.

Clinical evaluation and treatment of the adult patient with suspected primary immunodeficiency disease: a case analysis.

PURPOSE: To describe immunoglobulin G2 (IgG2) subclass deficiency in the context of primary immunodeficiency disorders, their pathophysiology, epidemiology, clinical evaluation, and management. DATA SOURCES: Actual case study and extensive review of the scientific and medical literature. CONCLUSIONS: Consideration should be given to primary immunodeficiency diseases as one possible cause of recurrent upper and lower respiratory tract infections in patients at any age. Early diagnosis and intervention can significantly reduce the burden of these diseases. IMPLICATIONS FOR PRACTICE: Advanced practice nurses need to keep pace with the ever-expanding field of immunity. Knowledge of basic principles of the immune system facilitates a logical approach to the evaluation and management of primary immunodeficiency diseases.

Intravenous gamma-globulin: clinical applications in pediatric care.

Intravenous gamma-globulin is being used with increasing frequency in the care of children. This article reviews the physiology of the immune system, the pharmacology of intravenous gamma-globulin, and presents four case studies. Administration guidelines, adverse reactions, and appropriate interventions are discussed. A table comparing four commonly used products is presented.