Development of National Guidelines for Immunoglobulin Therapy M. Elizabeth M. younger CRNP, ph.D The johns hopkins university Baltimore, maryland

Disclosures CSL Behring: Consultant and Research Support RMS: Clinical Advisory Panel Immune Deficiency Foundation: Chair, Nurse Advisory Committee

Immunoglobulin Replacement Therapy The Standard of Care for treatment of those immunodeficiencies characterized by hypogammaglobulinemia and impaired antibody responses to antigenic stimuli

Immunodeficiencies requiring Ig replacement Primary Immunodeficiencies: Common variable immunodeficiency Agammaglobulinemia (X-linked or autosomal recessive) Severe combined immunodeficiency (pre-transplant and post transplant until humoral immune reconstitution occurs) Hyper IgM deficiency Secondary immunodeficiencies Agammaglobulinemia secondary to monoclonal antibody therapy (e.g. rituximab) Hypogammaglobulinemia secondary to CLL

Goal of Therapy To deliver therapy safely and effectively To achieve this goal: therapy should be facilitated by professionals who have been educated regarding the rationale for therapy and its administration

Considerations Site of care Mode of Administration Home Hospital Infusion Suite Medical Office Mode of Administration Intravenous Peripheral Line Central Venous Line Subcutaneous Pump Push Facilitated subcutaneous

More Considerations Administered by Physician Nurse Practitioner Registered Nurse Vocational Nurse Patient Other trained lay person

And Still More Intravenous Infusions Frequency: every 2-4 weeks Product (excipients, stabilizers) Product Concentration (5, 6, 10%) Rate of Infusion Pre-medications (?) Patient risk factors/co-morbidities

Still More… Subcutaneous Infusions Frequency: Daily to every 4 weeks Product Concentration (10, 16 or 20%) Number of Sites (1 to ……) Time of Infusion (rapid push to hours) Premedication?

Process Guidelines were developed to achieve the goal for therapy while taking into account all the considerations

Process Literature review Explore regional variations Evidence based practice Draft guidelines Circulate guidelines Come together to reach consensus

Beth Younger, Kris Epland, Amy Meyer, Maggie Dodds, Carla Duff, Deb Sedlak, William Blouin, Jeanette Scott, Loris Aro