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Chronic inflammatory demyelinating polyneuropathy (CIDP), an immune-mediated peripheral neuropathy, is frequently treated with long-term maintenance intravenous immunoglobulin (IVIG). However, disadvantages of IVIG are the systemic adverse reactions, lengthy infusions, and need for vascular access. Subcutaneous immunoglobulin (SCIG) addresses many of the issues encountered by those unable, or unwilling, to tolerate the treatment burden of long-term IVIG. Subcutaneous immunoglobulin, a 20% solution stabilized with L-proline, is US Food and Drug Administration-approved for CIDP maintenance therapy in patients after being stabilized with IVIG. Approval was based on a randomized, double-blind, placebo-controlled trial where SCIG demonstrated superiority over placebo and was safe and efficacious in maintaining function. In addition to reviewing the primary efficacy results from the clinical trial, this article aims to update the neurology nursing community on a new option for long-term management of CIDP, including the practicalities of initiating and maintaining patients on SCIG therapy.

Subcutaneous immunoglobulin (SCIg) infusions are an option for patients requiring immunoglobulin therapy. Nurses are uniquely positioned to advocate for patients and to teach them how to successfully manage their infusions. The purpose of this review is to describe SCIg therapy and to provide teaching instructions as well as creative tips to ensure treatment success.

A number of ancillary supplies are used in the process of administering subcutaneous immunoglobulin. The particular type of ancillary supplies used (needles, tubing, and tape) may contribute to the development of issues at the local infusion site. Patient case studies demonstrate that changes in the choice of ancillary supplies can often alleviate these issues. The use of alternative ancillary supplies should be considered prior to the possibility of changing immunoglobulin replacement products in patients experiencing local infusion-site issues in order to improve outcomes and increase compliance. A treatment progression algorithm of ancillary supply adjustments has been developed.

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.

Immunoglobulin (Ig) replacement therapy, given as regular infusions of pooled human Ig, is the recognized treatment of humoral immunodeficiencies characterized by hypogammaglobulinemia and impaired antibody responses. It is a safe, effective therapy when delivered by nurses who have been educated to oversee and/or provide these infusions. Guidelines for administration have been developed by the Immune Deficiency Foundation Nurse Advisory Committee to provide a framework and guidance to those nurses administering this therapy.

Primary immunodeficiency diseases (PIDDs) include a large class of genetically heterogeneous disorders which predispose patients to significant risk of serious and chronic/recurrent infections, as well as reduced quality of life (QoL). Intravenous immunoglobulin (IVIG) therapy improves the well being of PIDD patients; however, the need for venous access and potentially severe side effects frequently require administration in medical facilities. We evaluated the long-term (12-month) experience with home-based self infusions of subcutaneous immune globulin (SCIG) in patients with PIDD on health-related QoL, rates of serious bacterial infections, and all other infections.Adults (n = 42) and children (n = 9) with PIDD, previously treated with clinic-based IVIG, were trained to self administer SCIG at home. QoL (SF-36(R) and CHQ-PF50 questionnaires), serious bacterial infections, serum immunoglobulin G (IgG) levels, overall infections, and incidence of adverse events were recorded at predetermined intervals.All patients had improved perceptions of general health (adults P = 0.047, children P = 0.037). Adults also had marked improvement in the bodily pain and vitality assessments, and parents had improved perceptions of personal and family activities. Serum IgG levels were maintained at mean levels 25% higher than previous troughs on IVIG. There were 162 infections overall for an annual rate of 3.42/patient, but only 1 serious bacterial infection was observed (0.03/patient/yr). An average of 4.5 days/yr was missed from work or school per patient.Home SCIG therapy was safe and led to improved perceptions of general health, higher serum IgG levels, and very low rates of infections and days missed from work/school.

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.

Increased use of specialized infusion therapies has necessitated training of health care providers and patients. The Starting Hizentra Administration with Resources and Education (SHARE) program provided 709 US participants with information to educate patients with primary immunodeficiency disease (PIDD) on self-administration of 20% subcutaneous immunoglobulin (SCIG). Postprogram surveys assessed participants' experience and opinion of 20% SCIG. The most frequent questions about 20% SCIG regarded subcutaneous challenges (29%). Participants stated that all attributes of SCIG were beneficial (51%), and they expressed interest in future programs on non-PIDD diseases (26%). Survey results will assist in future SHARE and other relevant educational program optimization.