M. ELIZABETH M. YOUNGER CRNP, PH.DTHE JOHNS HOPKINS UNIVERSITY

BALTIMORE, MARYLAND

Development of National Guidelines for Immunoglobulin

Therapy

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 deficiencySecondary 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 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 reviewExplore regional variationsEvidence based practiceDraft guidelinesCirculate guidelinesCome together to reach consensus

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