IDF Guide for Nurses

Immunoglobulin Therapy for Immunoglobulin Therapy Primary for Primary Immunodeficiency Immunodeficiency Diseases Diseases

IDF GUIDE FOR NURSES

IMMUNOGLOBULIN THERAPY FOR PRIMARY IMMUNODEFICIENCY DISEASES

THIRD EDITION COPYRIGHTS 2004, 2007, 2012 IMMUNE DEFICIENCY FOUNDATION Copyright 2012 by the Immune Deciency Foundation, USA.
Readers may redistribute this publication to other individuals for non-commercial use, provided that the text, html codes, and this notice remain intact and unaltered in any way. The IDF Guide for Nurses may not be resold, reprinted or redistributed for compensation of any kind without prior written permission from the Immune Deciency Foundation. If you have any questions about permission, please contact: Immune Deciency Foundation, 40 West Chesapeake Avenue, Suite 308, Towson, MD 21204, USA, or by telephone: 800.296.4433.

This publication has been made possible through a generous grant from

IDF Guide for Nurses

Immunoglobulin Therapy for Primary Immunodeciency Diseases

Third Edition

Immune Deciency Foundation 40 West Chesapeake Avenue, Suite 308 Towson, MD 21204 800.296.4433 www.primaryimmune.org

Editor: M. Elizabeth M. Younger CRNP, PhD

Johns Hopkins, Baltimore, Maryland Vice Chair, Immune Deciency Foundation Nurse Advisory Committee

Associate Editors: Rebecca H. Buckley, MD

Duke University School of Medicine, Durham, NC Chair, Immune Deciency Foundation Medical Advisory Committee

Christine M. Belser
Immune Deciency Foundation, Towson, Maryland

Kara Moran
Immune Deciency Foundation, Towson, Maryland

Contributors: Carla Du CPNP, CCRP, MSN

University of South Florida, St. Petersburg, Florida

Kristin B. Epland FNP, MSN

Midwest Immunology Clinic and Infusion Center, Plymouth, Minnesota

Elyse Murphy RN, BSN

CSL Behring, King of Prussia, Pennsylvania

Immune Deciency Foundation Nurse Advisory Committee: Loris Aro, RN

Sussman & Associates Immunology

Amy Meyer, RN, CPNP-PC

Childrens Hospitals & Clinics of Minnesota

William Blouin, MSN, ARNP, CPN

Miami Childrens Hospital

Maggi Dodds, RN, MS, CPNP

Texas Childrens Hospital

Carla Du, RN, BSN, CCRP

University of South Florida

Kristin Epland, FNP - Chair

Midwest Immunology Clinic

Vanessa Howard, BSN, MSN, FNP

St Jude Childrens Research Hospital

Terry Raburn, RN, BSN, ACRN

Texas Childrens Hospital

Jeanette Scott, BSN, RN

Santa Clara Valley Medical Center

Debra Sedlak, CPNP

Duke University Medical Center

Gretchen Vaughn, RN, MSN, CPNP

Cincinnati Childrens Hospital Medical Center

M. Elizabeth M. Younger, CPNP, PhD - Vice Chair

Johns Hopkins Hospital

TA B L E O F C O N T E N T S

About the Immune Deciency Foundation . . . . . . . . . . . . . . . . 2 Introduction to Primary Immunodeciency Diseases . . . . . . . . 5 Clinical Uses for Immunoglobulin Replacement Therapy . . . . 7 Product Selection and Characteristics . . . . . . . . . . . . . . . . . . 10 Delivery of Immunoglobulin Replacement Therapy . . . . . . . . 15 Intravenous Immunoglobulin Therapy . . . . . . . . . . . . . . . . . . 20 Subcutaneous Immunoglobulin Therapy . . . . . . . . . . . . . . . . 30 Additional Responsibilities . . . . . . . . . . . . . . . . . . . . . . . . . . . 36 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 40 Appendix A: Quick Nurses Guide for Troubleshooting SCIG Administration . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 46 Appendix B: Immune Deciency Foundation Resources. . . . 49

IMMUNE DEFICIENCY FOUNDATION

ABOUT THE IMMUNE DEFICIENCY FOUNDATION

The Immune Deciency Foundation (IDF) is the national nonprot patient organization dedicated to improving the diagnosis, treatment and quality of life of persons with primary immunodeciency diseases through advocacy, education and research. IDF was founded in 1980 by parents of children with primary immunodeciencies and their physicians. At that time, there were few treatments for primary immunodeciency diseases, almost no educational materials for patients, no public advocacy initiatives, and little research being done. In the past thirty years, IDF has pursued an aggressive agenda to remediate these problems and has made tremendous strides in the following areas:
I

Immune Deficiency Foundation

Helping the patient and professional communities gain a broader understanding of primary immunodeciency diseases through comprehensive education and outreach efforts; Promoting, participating in, funding and supporting research that has helped characterize primary immunodeciency diseases and given healthcare providers substantially improved treatment options for the care of patients with primary immunodeciency diseases; Addressing patient needs through public policy programs on local, national and international levels by focusing on issues such as insurance reimbursement, patient condentiality, SCID newborn screening, preventing genetic discrimination, ensuring the safety and availability of immunoglobulin therapy, and maintaining and enhancing patient access to a full range of treatment options; Establishing supportive networks of patients and professionals to ensure that the needs of patients with primary immunodeciency diseases are recognized and addressed.

IDF GUIDE FOR NURSES

Primary immunodeciency diseases represent a group of more than 150 rare disorders. In the United States, approximately 250,000 people are diagnosed with primary immunodeciency diseases. Thousands more go undetected. These individuals live throughout the country and experience a number of problems which have been documented by IDF. These patient problems include:
I

Difculty in nding specialized healthcare by immunologists or care providers knowledgeable about immunodeciency An inordinate delay in reaching proper diagnoses Problems with availability of appropriate treatment Difculties nancing healthcare and treatment Finding instructional materials about the specic diseases Educating the community and those with whom they come in contact about their disease and particular needs Lack of peer support and connection to others with whom they can share experiences

The goal of IDF is to address these issues and help affected individuals to overcome these difculties, thereby enabling them to live healthy and productive lives.

IMMUNE DEFICIENCY FOUNDATION

IDF Nurse Advisory Committee A Resource for Nurses and Patients

The Immune Deciency Foundation established the Nurse Advisory Committee in 1999. The committee is comprised of nurse experts who have many years of managing and providing care for patients with primary immunodeciency diseases. The goal of the committee is, rst and foremost, to improve the quality of healthcare received by patients with primary immunodeciency diseases. This goal is primarily achieved by educating and providing resources for patients caregivers. The Nurse Advisory Committee also increases awareness of primary immunodeciency diseases through professional education and outreach on local, national and international levels. The Committee is instrumental in increasing educational and peer support opportunities for individuals and families affected by primary immunodeciency diseases. The Nurse Advisory Committee is available as a resource for nurses providing therapy for or treating patients with primary immunodeciency diseases. The committee is also available for patients requiring assistance. Members can be reached by contacting IDF at 800.296.4433 or idf@primaryimmune.org. Immunoglobulin replacement therapy is indicated for a signicant number of patients with primary immunodeciency diseases. The IDF Nurse Advisory Committee is proud to offer this guide to help nurses to administer this therapy, safely and effectively. By doing so, nurses are in a unique position to improve the treatment experiences and provide a better quality of life for patients living with primary immunodeciency diseases.

IDF GUIDE FOR NURSES

I N T R O D U C T I O N TO P R I M A RY IMMUNODEFICIENCY DISEASES

The World Health Organization recognizes more than 150 primary immunodeciency diseases some are relatively common, others are quite rare. Some affect a single cell within the immune system; others may concern one or more components of the system. These diseases are classied according to the part of the immune system involved, either the adaptive or innate immune system. Immunodeciencies involving adaptive immune responses are characterized by impaired antibody production or function. Problems with innate immunity are those which involve natural killer lymphocytes, neutrophils, monocytes, macrophages or the complement system. Regardless of whether the problem is with the adaptive or innate system, patients affected with primary immunodeciencies are at risk for infection with virtually any pathogen. Even organisms which are not pathogenic in immunocompetent hosts can be pathogenic for people with immunodeciencies. These infections can be unusually severe or recurrent and they can sometimes be difcult to treat with conventional therapy. For the most part, primary immunodeciencies are rare and, because of this, may go unrecognized. Often patients experience many years of recurrent infections before they are appropriately diagnosed. Some primary immunodeciencies are caused by a problem with a single gene; others are caused by defects in multiple genes. There can be a clear inheritance pattern, such as with those immunodeciencies that are x-linked diseases; for other diseases the inheritance pattern is less clear. It is believed that some primary immunodeciencies develop over time and may be the result of a combination of genetic and environmental factors. Therefore, primary immunodeciencies may present and be diagnosed at any age. Similarly, there can be tremendous phenotypic and immunologic variability among individuals with the same diagnosis.

IMMUNE DEFICIENCY FOUNDATION

Many patients with primary immunodeciencies have signicant co-morbidities. Some of these co-morbidities may be related to the immunodeciency itself. For example, a patient with recurrent pneumonias may have irreversible lung damage (bronchiectasis) because of the infections. It is also known that patients with primary immunodeciency diseases may have a predisposition to autoimmune diseases including such problems as autoimmune cytopenias, inammatory bowel disease or rheumatoid arthritis. Sometimes the immunodeciency is diagnosed after a presentation of autoimmune disease. Some immunodecient patients may also have a greater risk for lymphoreticular cancers, such as lymphocytic leukemias, multiple myeloma or lymphomas, compared to that risk in the general population. Patients with antibody disorders are the largest group of people with primary immunodeciencies. These include patients with selective IgA deciency, by far the most common primary immunodeciency disease; patients with hypogammaglobulinemia and impaired antibody responses; and patients with combined B and T cell problems. For some of these diagnoses, but not all, immunoglobulin replacement therapy is the standard of care. This therapy provides antibodies from thousands of plasma donors to those who do not have and/or cannot make protective levels of antibody.

IDF GUIDE FOR NURSES

CLINICAL USES FOR IMMUNOGLOBULIN REPLACEMENT THERAPY

Concentrated human immune globulin preparations rst became widely available during World War II and were used for prophylaxis against infectious diseases such as hepatitis, measles and polio. The use of immunoglobulin as replacement therapy for primary immunodeciency was described by Dr. Ogden Bruton in 1952. Dr. Bruton treated a boy diagnosed with X-linked agammaglobulinemia with subcutaneous injections of immunoglobulin from immunocompetent human plasma donors. Initially, immunoglobulin was given predominantly by intramuscular injections. These injections were painful, and the maximum doses that could be given were limited because of the volumes involved. In the early 1980s, preparations that could be safely given by the intravenous route were rst licensed in the U.S. Intravenous immunoglobulin replacement therapy or IVIG, also referred to as IGIV, was generally well tolerated by most patients and became the standard of care for treatment of patients with primary immunodeciencies with antibody deciencies. Larger doses of immunoglobulin could be given via this route, more closely mimicking the bodys own production of antibodies. Better infection prophylaxis was achieved, resulting in signicant improvements in the patients conditions and outcomes. In 2006, the rst commercial preparations for subcutaneous immunoglobulin replacement therapy (SCIG) were approved by the United States Food and Drug Administration (FDA). These preparations, given in smaller doses and more frequently than IVIG provide very stable, consistent levels of IgG as opposed to the peaks and troughs associated with the intravenous route. For some patients, this is an important consideration.

IMMUNE DEFICIENCY FOUNDATION

All immunoglobulin preparations currently available in the U.S. are manufactured using donor pools from 10,000 to 60,000 units of donated human plasma. They contain IgG antibodies against a broad spectrum of vaccine antigens and infectious agents. All preparations contain 96% IgG. Most also contain some IgA and trace amounts of other plasma proteins. There are differences in the manufacturing processes and in the stabilizing agents used for each manufacturers products. Immunoglobulin (Ig) therapy is indicated as replacement therapy for primary and secondary immunodeciencies in those patients who do not make sufcient amounts of specic antibodies to adequately protect themselves from infectious diseases and those whose antibodies do not function correctly or those people with poor immunologic memory. Two examples of primary immunodeciency conditions requiring replacement therapy are agammaglobulinemia (either x-linked or autosomal) and common variable immunodeciency (CVID). Examples of secondary immunodeciencies include hypogammaglobulinemia caused by chemotherapy or monoclonal antibody therapy, as well as immunosuppressive therapies. In addition to antibody replacement, immunoglobulin also has anti-inammatory and/or immunomodulatory effects. As such, it is sometimes used to treat patients with a variety of conditions other than primary immunodeciency diseases. Immunoglobulin therapy has been demonstrated to be efcacious in the treatment of such diseases as idiopathic thrombocytopenia purpura (ITP), Kawasaki disease and some neuromuscular diseases. However, some of these other uses are experimental and/or off-label, which means that the FDA has not approved the use of immunoglobulin for those particular conditions.

IDF GUIDE FOR NURSES

Table 1: FDA Approved Uses of Immunoglobulin (Ig) Clinical Condition Chronic Inammatory Demyelinating Polyneuropathy (CIDP) Primary Humoral Immunodeciency Kawasaki Disease Idiopathic Thrombocytopenia Purpura (ITP) B-cell Chronic Lymphocytic Leukemia Ig Indication

Improve neurological symptoms

Antibody replacement therapy Prevent coronary artery aneurysms Increase platelets counts to prevent and control bleeding Prevent recurrent bacterial infections

Table 2: Immunodeciencies that ALWAYS Require Ig Replacement Therapy Agammaglobulinemia (X-linked, autosomal, or acquired) Common Variable Immunodeciency Hyper IgM Syndrome Severe Combined Immunodeciency (SCID) before and sometimes after bone marrow transplant

Table 3: Immunodeciencies that MAY Require Ig Replacement Therapy Severe Cases of Transient Hypogammaglobulinemia of Infancy Selective Antibody Disorder Wiskott Aldrich Syndrome DiGeorge (22q11 deletion) Syndrome Ataxia Telangiectasia Pediatric HIV

IMMUNE DEFICIENCY FOUNDATION

PRODUCT SELECTION AND CHARACTERISTICS

There are several different brands of immunoglobulin and hyperimmune products currently licensed for use in the United States. The U.S. Food and Drug Administration (FDA) has mandated that all immunoglobulin administered in the U.S. must be manufactured from plasma donated in this country. All manufacturing must be done in FDA approved facilities. Immunoglobulin is a plasma product. Thousands of carefully screened and tested donors provide plasma for a single lot of product. It is produced via a multifaceted manufacturing process designed to remove and/or inactivate bacterial and viral pathogens. These processes vary from manufacturer to manufacturer but include such steps as cold alcohol fractionation, low pH incubation, nanoltration, chromatography and solvent/detergent treatment. While the immunoglobulin manufactured in the U.S. is a very safe product, the possibility of transmission of existing or emerging pathogens cannot absolutely be ruled out. All immunoglobulin products are mostly IgG (> 96%). They also contain trace amounts of IgM and IgA. The remainder of the products is made up of stabilizing agents. Products vary in concentration, pH, stabilizing agents, osmolarity and osmolality, as well as sugar and sodium content. There is variability in administration factors as well, including the form of the drug (lyophilized or liquid), shelf life, approved means of administration (intravenous and/or subcutaneous) and prescribed infusion time. All of these factors need to be carefully considered when choosing a product for a particular patient. (See Table 4)

10

IDF GUIDE FOR NURSES

Table 4 : Examples of Factors to Consider in Choosing a Immunoglobulin Product

Potential Patient Risk Factors Osmolality

I I

Potential Immunoglobulin Risk Factors pH IgA Content

Volume Load

Sugar Content

Sodium Content

Cardiac Impairment
I

Renal Dysfunction

Anti IgA Antibodies

I I

Thromboembolic Risk

IMMUNE DEFICIENCY FOUNDATION

I I I I I

(Pre) Diabetes

Elderly Patients

Infants/Children

11

Immunoglobulin products are supplied as liquids or lyophilized powders (freeze dried powder that requires reconstitution). Some liquids require refrigeration; others are stored at room temperature. It is important to follow the manufacturers specications regarding storage. Any liquid which has been frozen should be discarded. Refrigerated products should be allowed to warm to room temperature before administration, as adverse effects can be associated with the administration of products that are too cold. Lyophilized products can be stored at room temperature before reconstitution. It is possible for these products to be prepared at more than one concentration depending on the amount of diluent added. Possibilities for different concentrations are specied in the manufacturers prescribing data. Nurses may be asked to reconstitute lyophilized products in the home or the infusion clinic. It is critically important to be aware of and to follow manufacturers guidelines, prescribers orders and aseptic technique, when reconstituting these products.

Preparations

Stabilizers include different sugars and/or amino acids that are added to immunoglobulin products to stabilize the IgG molecules and prevent them from aggregating. These stabilizing agents may pose a risk for some patients. For example, products containing glucose should be used cautiously in patients with diabetes. Similarly, some sucrose containing lyophilized products have been implicated in causing or exacerbating renal disease.

Stabilizers

12

IDF GUIDE FOR NURSES

There are small amounts of IgA in all immunoglobulin products. If a patient has an absence of IgA they may have anti IgA antibodies, then that patient could be at risk for anaphylaxis. Unfortunately, there is no commercial assay available for measuring IgE antibodies to anti-IgA. Fortunately, antibody decient patients are seldom able to mount IgE responses, so this is not a widely prevalent problem. Patients with low or undetectable levels of IgA may be able to tolerate all immunoglobulin without problems; however, these patients (particularly the CVID patients) should be carefully monitored. The rst infusion should always be administered in a controlled setting where emergency treatment can be administered immediately should problems occur. If the infusion is tolerated, the patient is not likely to have subsequent problems with IgAcontaining products.

IgA Levels

All products should be carefully inspected before administration. The packaging should be inspected for tampering as should the vials and their closures. Any evidence of tampering should be reported to the supplier and/or manufacturer and the product should not be used. Reconstituted and liquid products should not be given if there is particulate matter, precipitate crystals or bers in it. Products that have been frozen should not be given. For the most part, immunoglobulin should be clear although there can be a slight amount of cloudiness at times. The manufacturers package insert will provide information about the range of color as this can vary from one product to another. If the nurse or patient has any doubts at all about the integrity of the product at all, it should not be administered.

Product Integrity

IMMUNE DEFICIENCY FOUNDATION

13

Documentation
All IVIG infusions should be carefully documented. Documentation should include:
I

The patients current health status and any changes in this status in the period between infusions. The name and dose of the product, AND the lot numbers of the product used. Any pre medications which were given. How long it took for the infusion and specic rate titrations which were made. Any problems the patient experienced during the infusion and what the response to these problems was. How long the infusion took.

Similar documentation is important for SCIG infusions. Documentation of SCIG infusions should also include patient teaching interventions and documentation of the patients ability to administer their own infusions.

14

IDF GUIDE FOR NURSES

D E L I V E RY O F I M M U N O G L O B U L I N REPLACEMENT THERAPY
Nursing Responsibilities

Whether the nurse is administering an intravenous infusion or teaching patients to administer their own subcutaneous infusion, safety should always be the rst priority. The prescribers orders should be carefully followed and any problems with the orders should be addressed and resolved before the infusion. Communication of potential issues and problems so that they can be proactively addressed is critical. The following are broad guidelines for nursing interventions prior to, during and after administration of immunoglobulin replacement therapy. These guidelines are offered to help infusion nurses minimize problems and adverse effects, and safely provide a successful infusion experience for the patient.

Key Pre-infusion Assessments

I

Assess that the immunoglobulin product ordered is appropriate for the patient. Communicate potential problems to the prescriber. It is important to be aware of the differences between the various products available. As previously discussed, the qualities of a particular product may affect the tolerability and success of an infusion. Remember, not all immunoglobulin products are the same and are, accordingly, NOT interchangeable. The rst dose of any product should be administered in a controlled setting, where emergency equipment and treatment is readily available. The transition to home infusions can take place after it has been demonstrated that a particular product is tolerated. Should it be necessary to change products, the rst infusion of the new product should, again, be in a controlled setting. Assess product integrity. If the protective seals are not intact, the dispensing pharmacy should be notied immediately and the product should not be given.

IMMUNE DEFICIENCY FOUNDATION

15

Assess product temperature. The immunoglobulin should be at room temperature before the infusion. Solutions should be allowed to come to room temperature naturally. Product integrity may be compromised (denatured) by freezing or heating. NEVER put product into the microwave for warming. Assess level of patients understanding of therapy. Assess the patients general health and hydration status. It is important to document and inform the prescriber of any new health problems which have arisen since the last infusion and/or any new medications the patient is taking as these may have an impact on prescribed therapy. If the patient is poorly hydrated, consideration should be given to the possibility of providing some hydration, either enterally or parenterally, before the infusion. Assess for any weight loss or gain. Immunoglobulin replacement therapy is prescribed based on weight. Any signicant change (greater or less than 10%) may indicate a need for dosage increase or (less likely) reduction. Assess heart rate and respiratory status. Patients with congestive heart failure or who are at risk of uid overload, especially, should be assessed carefully before beginning the infusion. Both the volume of uid infused and the characteristics of the uid (osmolality, sodium content) could exacerbate these problems. Patients should be reassessed frequently during the infusion to be sure that there is no change in respiratory status, which could indicate uid overload. Diuretics may be prescribed before, during or after the infusion to prevent or relieve respiratory distress and/or complications associated with uid overload in these patients. Assess for fever prior to the start of infusion. If fever is present, the prescriber should be notied for directions for proceeding with or deferring the infusion. If the patient has an acute febrile illness or other indications of an infection are present, the infusion may need to be postponed until the patient is treated with antibiotics and/or the fever subsides. Administration of intravenous immunoglobulin when the patient has an acute infection may lead to adverse effects due to the formation of immune complexes.

16

IDF GUIDE FOR NURSES

Assess the need for premedication. Although the patient should have communicated any adverse events associated with previous infusions to the prescriber, this may not have happened. It is important to establish that the patient tolerated his/her previous infusion without problems. If problems did occur, then the prescriber should be notied and asked if premedication should be given. Premedications may be indicated to diminish the risk of infusion-related adverse events. Examples of premedications include systemic corticosteroids, antihistamines, antiemetics, acetaminophen and/or NSAIDs. Assess the need for localized anesthesia and obtain an order as necessary. Children, especially, may prefer to have topical anesthesia applied in advance of needle insertion to numb the sites at which needles or intravenous catheters will be placed. Assess preparedness for emergency situations. Emergency equipment should be readily available during the infusion. Emergency medications including epinephrine, diphenhydramine and parenteral uids should be checked to ensure that they have not expired. The nurse should ensure that he/she is prepared to respond to an emergency and that orders are in place for this response. A phone with which to call 911 should always be available. A protocol for communicating with the prescriber for both routine and emergency issues should be in place. Assess need for laboratory blood work prior to start of infusion. For patients receiving intravenous immunoglobulin, trough levels of IgG are an important monitoring tool. These levels need to be drawn immediately before beginning an infusion. The nurse should review the results of previous lab work with the patient and communicate with the prescriber to ensure that routine monitoring labs are done as ordered. Assess the patients experience with previous infusions. It is important for the nurse to listen to the patient and ensure that established routines are followed to avoid causing undue stress. Children, in particular, may have routines in place to assist them in dealing with both the physical and psychological impacts of infusions.

IMMUNE DEFICIENCY FOUNDATION

17

Key Intra-infusion Assessments

I

Assess the patient to ensure that the infusion is being tolerated. The nurse should listen carefully to any complaints and be sensitive to any alteration in the patients baseline status. Vital signs should be assessed as ordered and as indicated. Assess for any problems occurring after the infusion which may be infusion related. These can include headaches, myalgias, fever, arthralgias, rashes or a subjective feeling of general unwellness. If these problems are postulated to be infusion related, alterations to the infusion protocol may be necessary. Assess the need for premedications for future infusions and ensure that the premedications will be available for the next infusion. Assess the patient for his/her knowledge about the next infusion. It is important for the patient to know when the next infusion is due and what his/her responsibilities regarding this infusion are.

Key Post-infusion Assessments

I

Immunoglobulin replacement therapy can be administered intravenously (IVIG) or subcutaneously (SCIG). There are multiple factors to consider when choosing the route of administration; careful consideration of these factors and their relationship to the individual patient is critical to ensuring success. The patients wishes and whether these factors represent a pro or a con to the patient should be considered. Factors and questions to consider include:
I

Routes of Administration

Efficacy of Therapy: IVIG is usually given every three to four weeks. There is a peak in the level of IgG when the infusion is given and then the level declines to a trough before the next infusion, so there is a predictable rise and fall in levels. With SCIG infusions, the drug is slowly absorbed and is given more frequently, usually weekly. Once steady state is reached, the level of IgG is remarkably consistent. This consistency of level may be important for patients with conditions such as protein losing enteropathies or for patients on IVIG who have frequent breakthrough infections.
| IDF GUIDE FOR NURSES

18

Adverse reactions: There is a greater risk for systemic reactions with IVIG; local reactions are more common with SCIG. Patients who experience adverse reactions with IVIG and need premedication for their infusions may not experience these problems with SCIG.

Time Factor: IVIG infusions generally require three to four hours a month in a single sitting. SCIG infusions require less time but are given, at least, weekly.

Patient Compliance: What is the patients level of commitment? Will the patient do unsupervised home infusions of SCIG and follow up with appointments and lab work or is closer management/supervision required?

Cost of Therapy: In addition to the cost of drugs, there is an additional cost for nursing and/or overhead administration (infusion suite) costs with IVIG infusions. These costs are not uniform; the patients insurance benets and out-of-pocket costs need to be investigated.

Comorbidities: Does the patient have another illness which will be affected by therapy? For example, patients with cardiac disease may do better with the smaller amounts of uid used in SCIG. Conversely, some patients may need a higher peak of IgG than can be achieved with SCIG and consequently IVIG may be a better choice for them. IV Access Issues: Is monthly IV access difcult? The American Academy of Allergy, Asthma and Immunology strongly discourages the use of permanent indwelling ports or central venous lines in antibody decient patients due to the risk of infection and thrombotic events. If peripheral access is consistently difcult, SCIG may be a viable option.

Availability of Nursing Resources: Home infusion nursing services are not always available in every area of the country and patients may not live close to infusion centers, making self-infusion a desirable alternative.

IMMUNE DEFICIENCY FOUNDATION

19

I N T R AV E N O U S I M M U N O G L O B U L I N THERAPY

Intravenous immunoglobulin replacement therapy (IVIG) is generally given every three to four weeks at a dose of approximately 400-500 mg/kg/dose. It is well tolerated by the majority of patients, but it is important to note that, just as each patient may require a different immunoglobulin product, each may also require an individualized infusion regimen in order to achieve the desired therapeutic response. Once a successful regimen has been developed, it should be carefully followed with every infusion. This includes not only the rate of the infusion and necessary premedications, but the specic product, as well.

Administration
Different products vary in their compatibility with normal saline, sterile water or D5W, and manufacturers guidelines should be followed carefully. Administration of concomitant medications through the same IV line should be avoided. Should medications be required prior to or during an infusion, it is recommended to ush the line with at least 5-10 ml of compatible uid prior to administering the medication. Some medications will precipitate when in contact with IVIG, especially furosemide or diazepam. No medications should be directly administered into the same line simultaneously with the IVIG. If multiple medications are required, a second IV line should be placed so as not to interfere with the infusion. Another option is to piggyback the IVIG into the closest port in a line where a compatible uid is already running. The compatible uid can then be used as a ush if necessary.

20

IDF GUIDE FOR NURSES

Intra-infusion Assessments
I

Assess the rate of infusion. Prescribers orders and manufacturers recommended rates of infusion should determine length of time for an infusion to take. Generally, a ramp-up procedure is used for IVIG rates of infusion. The infusion is started slowly and the rate increased incrementally approximately every 15 to 30 minutes, as tolerated, until the patients maximum rate of infusion is reached. Although there is some literature reporting the tolerability of higher infusion rates, a general guideline to follow would be not to exceed the manufacturers recommended maximum rate. If a product change is necessary, the process for assessment of tolerability and potential rate increases must again be taken slowly. Assess the vital signs prior to each rate change to ensure that the infusion is being tolerated. Hyper- or hypotension, increased heart rate, increased respiratory rate or effort, and fever could all be signs of problems. It is important to assess the clinical relevance of any alterations in vital signs. For example, if a comfortable patient falls asleep, his/her blood pressure, heart rate and respiratory rate may decrease and may not represent a pathologic concern. Similar ndings in another patient may be signs of signicant problems with the infusion. Assess the need for comfort measures during the infusion, particularly if side effects occur. Both pharmacologic and nonpharmacologic interventions (supplying blankets or pillows, heating pads and encouraging the use of relaxation techniques) may be indicated. Assess for signs of anaphylaxis. Although true IgE medicated anaphylaxis in antibody decient patients is rare, if a patient has difculty breathing, signs of tongue or throat swelling, a feeling that the throat is closing, stridor, wheezing and/or chest tightness, generalized urticaria, or extreme anxiety, the infusion should be stopped and immediate emergency treatment, including calling 911, should be initiated.

IMMUNE DEFICIENCY FOUNDATION

21

Although most patients do well with IVIG, there is the potential for adverse reactions. It is estimated that 15-30% of patients experience some kind of reaction to their IVIG infusions. These reactions can range from mild to severe; however, most reactions occur during the initial 30 to 60 minutes of the infusion and are mild and self-limited. These reactions include anaphylactoid problems such as headaches, chills and rigors; allergic reactions like urticaria and, potentially, anaphylaxis; and other problems such as aseptic meningitis. The most common problems are related to the rate of the infusion and the temperature of the product. Reactions are more frequent with patients who are therapy nave, when therapy is given with a different product than the patient has previously been used to receiving, and/or in those who are not truly antibody decient or those who have been off of therapy for a period of time. It is important to note that most reactions occur during the initial 30 to 60 minutes of the infusion and are mild and self-limited. Regardless of the severity of a reaction, managing these problems requires timely interventions on the nurses part. A nursing policy and orders must be in place for dealing with these issues. There are risk factors that may identify persons at greater risk for having a reaction to IVIG. It is advisable to read the specic package insert for the IVIG product used, as the incidence and types of adverse events varies from product to product.

Adverse Reactions

Types of Adverse Reactions

I

Pyogenic Reactions: These reactions are marked by a signicant rise in temperature and are usually accompanied by other systemic symptoms. Fever is the most common side effect in children. Management of acute pyogenic reactions includes the use of antipyretic medications such as acetaminophen or ibuprofen. Persons who repeatedly experience temperature elevations during administration of IVIG may benet from premedication with an antipyretic/anti inammatory such as acetaminophen, 30 to 60 minutes prior to initiation of the infusion.

22

IDF GUIDE FOR NURSES

Anaphylactoid Reactions: These reactions most commonly include headache, dizziness or lightheadedness. Patients can also experience chills sometimes progressing to rigors, nausea and/or vomiting, back or hip pain, malaise, myalgias and arthralgias. Frequently the patient reports anxiety and in some cases a sense of impending doom. The most frequent cause of these reactions is infusion at an excessively rapid rate or infusion of a drug which is colder than room temperature. Often, the patient will have elevated blood pressure rather than hypotension.

Vasomotor Symptoms: These can occur with or without additional cardiac manifestations. Blood pressure can either increase or decrease, and may be accompanied by ushing or tachycardia. Patients experiencing such reactions may report shortness of breath or tightness in the chest.

Allergic Reactions: True IgE mediated allergic reactions are rare in antibody decient patients; however they can occur. In some cases, reactions to IVIG mimic those of true IgE mediated allergy but are actually due to activation of complement or other mediator systems by the IVIG. Allergic reactions can lead to acute anaphylaxis and shock. If these reactions occur, future use of IVIG is not precluded but, of course, must be closely monitored in a controlled environment (NOT in the patients home). These reactions often begin with a generalized nonspecic feeling of unease. Patients may describe an uncomfortable feeling, such as a tightening around the neck, chest or abdomen. There may be difculty swallowing, a choking sensation or difculty breathing. Other symptoms may include wheezing, ushing, hives, rapid or weak pulse, hypotension, sweating or an upset stomach with or without nausea, vomiting or diarrhea.

IMMUNE DEFICIENCY FOUNDATION

23

Table 5: Potential Nursing Interventions for Dealing with Adverse Reactions to IVIG Reaction Nursing Interventions Stop infusion. Administer prescribed medications. When symptoms resolve, restart the infusion at the rate the patient was tolerating before the symptoms occurred Administer acetaminophen or NSAID as prescribed. The patients hydration status may aect the development of headaches; the patient should make sure he/she is adequately hydrated on the day of the infusion. Pharmacologic: Administer prescribed anti-migraine medications as soon as the rst signs of a migraine occur. Oral or IV steroids may help decrease the intensity of the headache and should be given if ordered. Non-pharmacologic: Include comfort measures such as reducing auditory and visual stimuli, and applying cold compresses to the head or back of the neck. Resting after an infusion may help to minimize muscle aches or pain and to decrease excessive fatigue. Acetaminophen or NSAIDS and ensuring adequate pre-infusion hydration may help with this problem.

Chills/ Rigors

Headache

Migraine Headache (patients with a history of and under treatment for headache problems)

Malaise/Flu-like Symptoms

24

IDF GUIDE FOR NURSES

Table 5: Potential Nursing Interventions for Dealing with Adverse Reactions to IVIG (cont.) Reaction Nursing Interventions Stop the infusion. Contact the prescriber. Administer prescribed antihistamines and/or steroids. Observe for signs of true anaphylaxis; if they occur administer epinephrine and activate the emergency response system (911). Stop infusion. Follow the prescribers order for uid bolus, diuretics or other interventions or administer uid with hypotension, based on prescriber order. Administer diuretics on prescribers order if uid overload is likely. Stop the infusion. Administer prescribed antiemetic medications. Provide comfort measures. Stop or slow the infusion. Administer acetaminophen or NSAIDS for the discomfort. The use of a heating pad may be benecial.

Urticaria

Vasomotor Symptoms (Hypotension, Hypertension, Flushing or Tachycardia)

Nausea/Vomiting

Back Pain/Hip Pain/Arthralgias/ Myalgias

IMMUNE DEFICIENCY FOUNDATION

25

Potential Post-infusion Reactions

Post-infusion reactions can occur immediately or as long as 72 hours following the infusion. Symptoms associated with postinfusion reactions are usually less severe in nature but can interfere with a patients quality of life. Common post-infusion reactions may include headache, low-grade fever, nausea, arthralgias and generalized malaise. Often patients describe a u-like feeling. These reactions are generally managed with over-the-counter analgesics, antihistamines and may require a short course of corticosteroids. Headaches are more frequent in patients who have a history of migraine or cluster headaches. Some patients, particularly those with histories of migraines at other times, may have severe headaches and/or typical migraines up to 72 hours after their infusion. Over-the-counter analgesics are usually effective in treating these headaches, but they sometimes require the addition of oral steroids. Severe, persistent posterior occipital headaches may be a sign of aseptic meningitis, which has been reported in some patients after IVIG infusion.

Table 5 presents common adverse reactions and potential nursing interventions. It is important to follow the prescribers orders when dealing with any infusion reaction. The prescriber should always be notied that a reaction has occurred and may wish to change immunoglobulin products or order premedications for future infusions. Reactions to IVIG can diminish with subsequent infusions, so the need for premedications needs to be reassessed periodically.

26

IDF GUIDE FOR NURSES

Complications Associated with IVIG

I

Thrombotic Events: Thrombotic (vascular occlusive) events have been reported in association with IVIG. The mechanisms for these episodes may include increased blood viscosity after high-dose IgG and/or the presence of procoagulant proteins in the IVIG preparation. These episodes have been noted with increased frequency in patients following rapid infusion protocols or patients with risk factors such as prior thromboembolic events, thrombocytosis, or immobility. Thrombotic events are serious in nature and include chest pain, myocardial infarction, congestive cardiac failure, transient (cerebral) ischemic attack (TIA) and stroke. Patients with risk factors for thrombotic events should follow a conservative infusion protocol, using a product with a low (5%) concentration, and proceed slowly and cautiously with incremental increases in the rate of infusion to a maximum of 4 ml per kg of body weight per hour. Patients should be given clear instructions regarding what post-infusion symptoms should be reported immediately to their prescriber.

Transmission of Bloodborne Pathogens: IVIG products are manufactured from large numbers of carefully screened human donors who have been tested for the absence of hepatitis B surface antigen, hepatitis C antibody and HIV antibody, and by nucleic acid testing for HIV and HCV. In addition, all products are produced using techniques to remove or inactivate potentially contaminating viral pathogens. Viral inactivation and removal processes have demonstrated reduction of the potential presence of pathogenic prion agents that have been associated with the development of transmissible spongiform encephalopathy such as variant Creutzfeldt-Jakob disease. Each manufacturers package insert will delineate these processes.

IMMUNE DEFICIENCY FOUNDATION

27

Aseptic Meningitis: Cases of aseptic meningitis with headache and positive meningeal signs have been reported with the use of IVIG in both standard replacement therapy dosing and high dose therapy. The symptoms may occur during the infusion, but more typically they usually develop within 24 hours of the infusion. A previous history of migraine headaches has been noted to be a risk factor. A neurologic exam is indicated for these patients to rule our bacterial or viral meningitis. Patients with aseptic meningitis have pleocytosis but no organisms in their cerebrospinal uid. Treatment is symptomatic. The development of aseptic meningitis is an indication for a change in the immunoglobulin product used for future infusions. Premedication with corticosteroids is also indicated for those with a previous history of infusion related aseptic meningitis.

Renal Adverse Events: Potential adverse effects involving the kidneys include acute renal failure, acute tubular necrosis, proximal tubular nephropathy and osmotic nephrosis. There have been rare reports of increased serum creatinine, oliguria and acute renal failure occurring from one to seven days after IVIG administration. Hyperosmolality and the presence of sucrose have been implicated as factors contributing to renal adverse events. Patients who are not adequately hydrated prior to onset of the infusion, who have diabetes mellitus or any pre-existing renal insufciency, those receiving nephrotoxic antibiotics, those who have paraproteinemia, and/or those who are over age 65 are at the greatest risk for these problems. Renal function (serum Creatinine and BUN) and urine output should be carefully monitored in patients at risk for developing renal adverse events. Using slow infusion rates during administration of IVIG and assuring adequate hydration are advised for such at-risk patients. As with patients at greater risk for thrombotic problems, patients with the potential for renal adverse events should be given clear instructions regarding what post-infusion symptoms should be reported immediately to their prescriber.

28

IDF GUIDE FOR NURSES

Transfusion-related Acute Lung Injury (TRALI): TRALI is a rare but potentially devastating complication of blood component therapy characterized by severe respiratory distress, hypotension, fever, dyspnea, and tachycardia. Patients exhibit pulmonary edema, hypoxemia, abnormal left ventricular function, and fever with a typical onset within one to six hours after infusion of the product. Pulmonary embolism and lung dysfunction due to "transfusion related acute lung injury" have also been observed during or immediately after IVIG infusions. Patients at risk include those with a history of atherosclerosis, those who have multiple cardiovascular risk factors, those of advanced age, those with impaired cardiac output, and/or those with known or suspected hyperviscosity or hypercoagulable disorders. This last group of patients include women taking oral contraceptives, especially if they also smoke, and anyone who has had prolonged periods of immobilization. Patients with TRALI may be managed using oxygen therapy and appropriate ventilatory support with symptoms usually resolving within 96 hours. If TRALI is suspected, appropriate tests should be performed for the presence of anti-neutrophil antibodies in both the product and patient serum.

IMMUNE DEFICIENCY FOUNDATION

29

S U B C U TA N E O U S I M M U N O G L O B U L I N THERAPY

In 1952, when Dr. Bruton began to treat his antibody decient patient, he actually used subcutaneous immunoglobulin (SCIG). In the United States, the therapy evolved into intramuscular injections and then intravenous infusions. Since the 1980s, SCIG has been widely used in Europe. In January 2006, the U.S. Food and Drug Administration (FDA) approved a preparation of SCIG for use in the U.S. Currently the only FDA approved indication for SCIG is as antibody replacement therapy. At this time products for subcutaneous infusions are available in concentrations of 10 or 20%. For the majority of patients, IVIG and SCIG are equally efcacious; however, there are differences between the therapies. While IVIG is usually given as a single large infusion every three to four weeks, SCIG involves giving smaller doses more often, once or twice a week in most cases. Fractionating the total dose into smaller portions decreases the changes in serum IgG levels that are the hallmark of intermittent IV infusions. This fractionation of the dose may eliminate some of the systemic adverse effects associated with IV infusions. Since infusions are given more often, the low trough IgG levels that occur just before the next IVIG infusion are also eliminated. With SCIG, the serum IgG concentration becomes remarkably consistent once steady state is achieved and the patient is compliant with the therapy. SCIG is self-administered by the patient. For children or those patients with some physical limitation, someone else can assist with the infusion. Dependent on the volume of drug to be infused, multiple small needles may be used simultaneously. The drug can be administered via a small syringe driver pump or via a manual push. Many studies have demonstrated that patients can tolerate relatively rapid infusions and those patients who deliver their infusions via a manual push can do so in a matter of minutes.

30

IDF GUIDE FOR NURSES

A great deal of exibility in the regimen, including the number of sites, duration of the infusion, and frequency of infusions, is possible. Because of these multiple permutations, the patient can design a regimen which works for him/her; that is one with which he/she can be compliant. For example a dose of 10 grams/week if given with a 20% solution would have a volume of 50 ml. The typical adult could receive this dose via a once weekly infusion, using two needles connected to bifurcated tubing and a pump; the infusion could take approximately 30-40 minutes, although it could be made to go faster or slower based on the patients tolerability and wishes. This dose could be split into a twice weekly infusion, using a single needle for each infusion. Some patients could even choose to give themselves 10 cc on ve days every week. This exibility in custom designing a regimen can be very attractive to patients who seek greater control over their illness and treatment. Deciding how, where and when the infusion occurs may help to minimize time lost from work or school, and allow greater freedom for patients who travel frequently.

The nursing role in SCIG is primarily that of an educator and facilitator. The goal for care is to help the patient/caregiver to become independent. Patients and/or caregivers will need to be taught the skills necessary to administer their infusions in a safe and aseptic manner. A systematic, step-wise teaching approach is usually effective. This starts with the nurse rst demonstrating the procedure, then allowing the patient to practice the skill, and nally observing a return demonstration by the patient/caregiver to demonstrate mastery. After the patient is independent, follow up and support are critical in managing issues and/or problems. There may be multiple small tweaks necessary to ensure success. These might include changing the gauge or length of the needle, a recommendation about using a different site, or changing the rate of the infusion. The important thing to remember is that the vast majority of patients can be successful with this therapy.

Nurses Role

IMMUNE DEFICIENCY FOUNDATION

31

The most important factors in assuring the success of subcutaneous therapy are teaching and support. The nurse needs to develop a teaching plan which takes into consideration the patients ability to learn; independence; self-motivation; compliance; ability to read and/or follow instructions; physical limitations, especially regarding manual dexterity; and presence of someone to assist or actually perform the infusion, if necessary. Much of the education for SCIG administration includes basic nursing, i.e. hand washing and aseptic technique. A systematic approach to setting up the equipment and drawing up the product, inserting the needle(s), monitoring local effects, discontinuing the infusion, and safely discarding the used equipment and needles needs to be developed.

Specific teaching topics can include:

I

Storage and handling of medication Traveling with medication, supplies and pumps Using aseptic technique for drawing up the drug Priming tubing Subcutaneous site selection and preparation Insertion, securing and removal of needles Checking needle placement to ensure that it has not been inadvertently placed in the intravascular space Setting up the pump if a pump is going to be used Anticipating and troubleshooting infusion problems Discontinuing infusion Comfort measures and site care Appropriate waste disposal

32

IDF GUIDE FOR NURSES

Another important teaching topic is ensuring that the patient understands adverse reactions and/or complications, as well as how to initiate the appropriate action should something untoward occur. Expectations regarding site reactions and management should be discussed. The patient must be taught the signs of anaphylaxis and what to do should they occur. EpiPen training should be provided when an EpiPen has been prescribed. Documentation of the patients mastery of skills is important. The number of sessions required for the patient to master all of these steps may vary widely depending on the individuals capacity for learning, coupled with their anxiety level.

Systemic reactions such as headache, nausea, fever, chills, and more serious adverse reactions are less frequent with SCIG than with IVIG, and published studies on SCIG consistently demonstrate a low rate of systemic reactions. The most common reported adverse events with SCIG are localized site reactions including itching, a burning sensation, mild redness and/or swelling. These local reactions are very common when a patient starts SCIG therapy because initially the body does not recognize the drug and perceives it as an irritant or something foreign. The normal inammatory cascade is activated, so there is swelling and erythema at the site(s). In almost all cases, these symptoms resolve within 12 to 24 hours. The intensity of these local reactions decreases with every infusion as the body comes to recognize the drug. If redness, irritation and swelling persist after the patient has been on therapy for more than a month or six weeks, it may be an indication of a mechanical issue such as a too-short needle causing drug to leak into the dermal layer or an infusion rate higher than the patient can tolerate. It may also be an indication that the patient needs to work up to the desired number of sites, volume per site and rate. As with IVIG in patients with humoral immunodeciencies, true anaphylaxis with SCIG is extremely rare. Local site reactions can be managed with adjustments to the infusion regimen, gentle massage, warm or cold compresses, and/or mild pain medications such as ibuprofen or acetaminophen.

Adverse Reactions

IMMUNE DEFICIENCY FOUNDATION

33

Table 6: Potential Nursing Interventions for Dealing with Adverse Reactions to SCIG Reaction Nursing Interventions Apply cold compress (do not apply cold pack directly to skin). For future infusions, consider: Use of a longer needle. Decrease volume per site and working up to desired site volume gradually. Ensure that a dry needle insertion technique has been used. Topical diphenhydramine. Over-the-counter topical steroid. Apply cold or warm compress depending on which the patient feels will help. Consider irritation from tape or adhesive and change this product. Assure patient that redness should decrease with each subsequent infusion. Clamp o catheter for 5-10 minutes, if desired. Slow the rate of infusion. Cold compress. Distraction techniques for younger children. Consider removing the needle and replacing it in another site. Assess needle placement as the needle may be partially intramuscular instead of subcutaneous. Consider the use of a shorter needle. Assess antiseptic used for skin prep.

Local Itching

Redness

Burning

34

IDF GUIDE FOR NURSES

Table 6: Potential Nursing Interventions for Dealing with Adverse Reactions to SCIG (cont.) Reaction Swelling (There will always be some degree of swelling as the patient is infusing uid under the skin.) Nursing Interventions Warm compresses for 5-10 minutes. If using a heating pad, use low setting. Gentle massage. Move area as tolerated, e.g. if using a leg site: take a walk to help mobilize the uid. Stop infusion. Contact prescriber for instructions and to determine if infusion should continue. Antihistamine. Stop SCIG. Contact prescriber for instructions and to determine if infusion should continue. Consider the possibility of tape or latex sensitivity. Slow infusion. If intolerable pain, needle may be intramuscular, remove the needle and change sites. Warm compresses. Gentle massage. Over-the-counter analgesics can be used, but are rarely necessary.

Urticaria/Hives

Rash

Discomfort

IMMUNE DEFICIENCY FOUNDATION

35

ADDITIONAL RESPONSIBILITIES

The nurse, the prescriber and the patient form an interdependent triad. Each person in this triad has individual as well as overlapping responsibilities. All must work together to achieve the goals for care.

The prescribers responsibility is to make a diagnosis and educate the patient about the diagnosis. Decisions for therapy should be made collaboratively. The prescriber should explain available options for therapy. The therapy regimen, both the rationale for the therapy and practicalities involving the therapy, need to be explained clearly. The prescriber should make plans and expectations clear regarding follow up and sick visits, referrals to other providers and laboratory monitoring.

Prescriber

Patients need to assume responsibility for themselves while maintaining close connections with the prescriber and the nurse. They should identify the need for education and/or assistance and should communicate problems or issues, especially potential barriers to care, appropriately, effectively and in a timely manner. Ultimately, it is the patient who establishes the parameters and/or boundaries for the partnerships in this interdependent triad with nurse and prescriber.

Patients

36

IDF GUIDE FOR NURSES

The nurses rst responsibility is to safely and effectively provide the prescribed therapy. However, the nurse has multiple other responsibilities:

Nurse

Compliance Monitoring
The nurse may oversee the establishment of monitoring parameters for infusions and infusion related issues, including patient compliance. Compliance monitoring should include clear instructions for the patient regarding his/her therapy. The patient should know the name and dose of the drug that he/she is receiving. The patient should be taught to record specics of each infusion in a personal diary or infusion log. Information that should be recorded includes:
I

Expiration dates and lot numbers of drug, the site(s) used, Length of time for infusion to be complete, Adverse events, and Any other pertinent information.

To record infusions and medical information, patients can use the Immune Deciency Foundation (IDF) eHealthRecord, which is a free-of-charge, online personal health record developed specically for the primary immunodeciency disease community: www.idfehealthrecord.org. Some patients keep handwritten infusion logs, which are often available as downloadable PDF les from manufacturers websites. The eHealthRecord can keep all medical history, current medications, infusions logs and more all in one place. Either way they choose to log infusions, patients need to understand the importance of keeping a log to record lot numbers and dosages as recalls of products and/or specic lot numbers do sometimes occur. Patients have a right to know if there is a potential problem and to seek appropriate help if there is a concern. Patients can enroll in a patient notication system for information on product withdrawals and recalls at: www.patientnoticationsystem.org.

IMMUNE DEFICIENCY FOUNDATION

37

Documentation
Documentation is another key nursing responsibility. As with all blood products, the nurse needs to keep a record of the product, lot number(s) and expiration date(s). This data needs to be readily retrievable in the event of a product recall or if a reportable patient problem occurs. Other infusion related data including dose, duration of the infusion and assessments of the patient should also be carefully recorded. This data is important when trending information about the patients replacement therapy and his/her overall health status.

Communication
The nurse has a critical role in establishing parameters for communication between all members of the triad. Variables to be determined regarding communication include the mode (telephone, e-mail, written), what needs to be communicated to whom, to whom specic problems should be communicated and communication in the event of emergency. The patient needs clear, written directions and needs to demonstrate understanding of these directions. A printed instruction list with relevant contact information is useful as a reference guide in the patients home.

38

IDF GUIDE FOR NURSES

Education and Advocacy

The nurse plays an important role in patient education and advocacy and should assess the patients knowledge about the disease state and treatment, and provide necessary education. There is a multitude of resources available to meet educational needs. IDF has patient education materials readily available: www.primaryimmune.org. Through IDF, patients can also connect with other patients. Additionally, IDF has frequent outreach programs for patients and families. Information about such things as new modalities of treatment, legislative initiatives and insurance issues can be valuable resources. For complete list of resources available through IDF, see Appendix B. The nurse should provide ongoing support and education and assist patients in locating resources for such diverse issues as insurance problems, pediatric patients transition to adulthood and assumption of their own care, attending school/college, concerns regarding traveling and vacations, pregnancy and any other life cycle changes. This includes providing patients with information about their product manufacturers patient assistance program. Again, information regarding these issues can be found on the IDF website: www.primaryimmune.org. Nurses should advocate for their patients with primary immunodeciency diseases and help them to bring issues to the prescriber about ways to improve the patients quality of life not only during replacement therapy administration but also in other areas in which the patients primary immunodeciency has an impact on their life. Patients should not be dened by their disease; the ultimate goal should be to empower them to take control of their lives.

IMMUNE DEFICIENCY FOUNDATION

39

REFERENCES

Ahsan N, Intravenous immunoglobulin induced-nephropathy: A complication of IGIV therapy. Journal of Nephrology. 1998;11(3):157-161. Ballow M, Safety of IGIV therapy and infusion-related adverse events. Immunologic Research. 2007;38(1-2):122-132. Ballow M, Berger M, Bonilla FA, et al. Pharmacokinetics and tolerability of a new intravenous immunoglobulin preparation, IVIG-C, 10% (Gamunex, 10%). Vox Sang. 2003;84(3): 202-210. Berger M. Subcutaneous immunoglobulin replacement therapy in primary immunodeciencies. Clinical Immunology. 2004;112(1):1-7. Berger M. A multicenter, prospective, open-label, historically controlled trial to evaluate efcacy and safety in primary immunodeciency diseases (PID) patients of Flebogamma 5% DIF, the next generation of Flebogamma. Journal of Clinical Immunology. 2007;27(5):503-509. Berger M. Principles of and advances in immunoglobulin replacement therapy for primary immunodeciency. Immunology and Allergy Clinics of North America. 2008;28(2):413-417. Berger M, Pinciaro PJ. Safety, efcacy and pharmacokinetics of Flebogamma 5% [immune globulin intravenous (human)] for replacement therapy in primary immunodeciency diseases. Journal of Clinical Immunology. 2004;24(4);389-396. Berger M, Murphy E, Riley P, Bergman G, VIRTUE Trial investigators. Improved quality of life, immunoglobulin G levels, and infection rates in patients with primary immunodeciency diseases during self-treatment with subcutaneous immunoglobulin G. Southern Medical Journal. 2010;103(9):856-863. Bhole MV, Burton J, Chapel HM. Self-infusion programmes for immunoglobulin replacement at home: Feasibility, safety and efcacy. Immunology and Allergy Clinics of North America. 2008;28(4):821-832. Bonilla FA. Pharmacokinetics of immunoglobulin administered via intravenous or subcutaneous routes. Immunology and Allergy Clinics of North America. 2008;28(4):803-819. Bonilla FA, Bernstein IL, Khan DA, et al. Practice parameters for the diagnosis and management of primary immunodeciency. Annals of Allergy, Asthma & Immunology. 2005;94:S1-S63. Brennan VM, Salome-Bentley NJ, Chapel HM. Prospective audit of adverse reactions occurring in 459 primary antibody-decient patients receiving intravenous immunoglobulin. Clinical and Experimental Immunology. 2003;133(2):247-251. 40 | IDF GUIDE FOR NURSES

Burks AW, Sampson H A, Buckley RH. Anaphylactic reactions after immunoglobulin administration in patients with hypogammaglobulinemia: Detection of IgE antibodies to IgA. New England Journal of Medicine. 1986;314(9):560-564. Busse PJ, Razvi S, Cunningham-Rundles C. Efcacy of intravenous immunoglobulin in the prevention of pneumonia in patients with common variable immunodeciency. Journal of Allergy and Clinical Immunology. 2002;109:1001-1004. Centers for Disease Control and Prevention. Renal insufciency and failure associated with immune globulin intravenous therapy; United States, 19851998. MMWR Morbidity and Mortality Weekly Report. 1999;48:518-521. Chapel H, Cunningham-Rundles C. Update in understanding common variable immunodeciency disorders (CVIDs) and the management of patients with these conditions. British Journal of Haematology. 2009;145(6):709-727. Chapel HM, Spickett GP, Ericson D, et al. The comparison of the efcacy and safety of intravenous versus subcutaneous immunoglobulin replacement therapy. Journal of Clinical Immunology. 2000;20: 94-100. Church JA, Leibl H, Stein MR, et al. Efcacy, safety and tolerability of a new 10% liquid intravenous immune globulin (IGIV 10%) in patients with primary immunodeciency. Journal of Clinical Immunology. 2006;26(4):388-395. Cox JA, Westbrook LJ. Home infusion therapy: Essential characteristics of a successful education process: Grounded therapy study. Journal of Infusion Nursing. 2005;28(2):99-107. Cunningham-Rundles C, Seigal FP, Smithwick EM, et al. Efcacy of intravenous immunoglobulin in primary humoral immunodeciency disease. Annals of Internal Medicine. 1984;101(4):435-439. Cunningham-Rundles C, Zhou Z, Mankarious S, Courtier S. Long-term use of IgA-depleted intravenous immunoglobulin in immunodeciency subjects with anti-IgA antibodies. Journal of Clinical Immunology. 1993;13(4):272278. Dalakas M. High-dose intravenous immunoglobulin and serum viscosity. Neurology. 1994;44:223-6. Daw Z, Padmore R, Neurath D, et al. Hemolytic transfusion reactions after administration of intravenous immune (gamma) globulin: a case series analysis. Transfusion, 2008;May 2.

IMMUNE DEFICIENCY FOUNDATION

41

De Albuquerque Campos R, Sato MN, da Silva Duarte AJ. IgG anti-IgA subclasses in common variable immunodeciency and association with severe adverse reactions to intravenous immunoglobulin therapy. Journal of Clinical Immunology. 2000;20(1):77-82. Duff, KA You Can Make a Difference in Administration of Intravenous Gammaglobulin. Intravenous Nursing Supplement 2006 Durandy A, Wahn V, Petteway S, Gelfand E. Immunoglobulin replacement therapy in primary antibody deciency diseases: Maximizing success. International Archives of Allergy and Immunology. 2005;136: 217-229. Eijkhout HW, van Der Meer JW, Kallenberg CG, et al. The effect of two different doses of intravenous immunoglobulin on the incidence or recurrent infections in patients with primary hypogammaglobulinemia: A randomized, double-blind, multicenter crossover trial. Annals of Internal Medicine. 2001;135:164-175. Elkayam O, Paran D, Milo R, et al. Acute myocardial infarction associated with high-dose intravenous immunoglobulin infusion for autoimmune disorders. A study of four cases. Ann Rheum Dis. 2000;59:77-80. Fasth A, Nystrm J. Quality of life and health-care resource utilization among children with primary immunodeciency receiving home treatment with subcutaneous human immunoglobulin. Journal of Clinical Immunology. 2008;28: 370-378. Food and Drug Administration and Center for Biologics Evaluation and Research. Guidance for industry: Safety, efcacy, and pharmacokinetic studies to support marketing of immune globulin intravenous (human) as replacement therapy for primary humoral immunodeciency. U.S. Department of Health and Human Services, Food and Drug Administration. 2005. Gardulf A, Borte M, Ochs HD, Nicolay U, The Vivaglobin Clinical Study Group. Prognostic factors for health-related quality of life in adults and children with primary antibody deciencies receiving SCIG home therapy. Clinical Immunology. 2008;126(1):81-88. Gardulf A, Nicolay U. Replacement IgG therapy and self-therapy at home improve the health-related quality of life in patients with primary antibody deciencies. Current Opinion in Allergy and Clinical Immunology. 2006;6(6):434-442. Gardulf A, Nicolay U, Asensio O, et al. Children and adults with primary antibody deciencies gain quality of life by subcutaneous IgG selfinfusions at home. Journal of Allergy & Clinical Immunology. 2004;114(4):936-942.

42

IDF GUIDE FOR NURSES

Gardulf A, Nicolay U, Asensio O, et al. Rapid subcutaneous IgG replacement therapy is effective and safe in children and adults with primary immunodeciencies-A prospective, multi-national study. Journal of Clinical Immunology. 2006;26(2):177-185. Gaspar J, Gerritsen B, Jones A. Immunoglobulin replacement therapy by rapid subcutaneous infusion. Archives of Disease in Childhood. 1998;79:48-51. Gustafson R, Gardulf A, Hansen S, et al. Rapid subcutaneous immunoglobulin administration every second week results in high and stable serum immunoglobulin G levels in patients with primary antibody deciencies. Clinical and Experimental Immunology. 2008;152(2): 274-279. Gelfand E. Critical decisions in selecting an intravenous immunoglobulin product. Journal of Infusion Nursing. 2005;28(6):366-374. Gelfand EW, Goldsmith J, Lederman HM. Primary humoral immunodeciency: Optimizing IgG replacement therapy. Clinical Focus on Primary Immune Deciencies. 2003;11: 3-13. Gelfand EW, Hanna K, The IGIV-C Increased Maximum Infusion Rate Study Group. Safety and tolerability of increased rate of infusion of intravenous immunoglobulin G, 10% in antibody-decient patients. Journal of Clinical Immunology. 2006;26(3):284-290. Gelfand EW. Differences between IGIV products: Impact on clinical outcomes. International Immunopharmacology. 2006;6:592-599. Go RS, Call TG. Deep venous thrombosis of the arm after intravenous immunoglobulin infusion: case report and literature review of intravenous immunoglobulin-related thrombotic complications. Mayo Clin Pro. 2006;75:83-5. Hagan J. Efcacy and safety of a new 20% immunoglobulin preparation for subcutaneous administration, IgPro20 in patients with primary immunodeciency. Journal of Clinical Immunology. 2010; 30:734-745. Hamrock DJ. Adverse events associated with intravenous immunoglobulin therapy. International Immunopharmacology. 2006;6(4):535-542. Hansen S, Gustafson R, Smith CIE, Gardulf A. Express subcutaneous IgG infusions: Decreased time of selivery with maintained safety. Clinical Immunology. 2002;104(3):237-241. Henderson K. Training and support to enable home immunoglobulin therapy. Nursing Times. 2003;99: 28-31. Jolles S, Sewell WA, Misbah SA. Clinical uses of intravenous immunoglobulin. Clinical and Experimental Immunology. 2005;142(1):1-11. Kittner JM, Grimbacher B, Wulff W, Jager B, Schmidt RE. Patients attitude to subcutaneous immunoglobulin substitution as home therapy. Journal of Clinical Immunology. 2006;26(4):400-405.

IMMUNE DEFICIENCY FOUNDATION

43

Kivity S, Katz U, Daniel N, et al. Evidence for the use of immunoglobulins-A review of the literature. Clinical Reviews in Allergy and Immunology. 2010;38:201-269. Lucas M, Lee M, Lortan J, et al. Infection outcomes in patients with common variable immunodeciency disorders: Relationship to immunoglobulin therapy over 22 years. Journal of Allergy and Clinical Immunology. 2010;125:1354-1360. Misbah S, Sturzenegger MH, Borte M, et al. Subcutaneous immunoglobulin: Opportunities and outlook. Clinical & Experimental Immunology. 2009;158:51-59. Moore ML, Quinn JM. Subcutaneous immunoglobulin replacement therapy for primary antibody deciency: Advancements into the 21st century. Annals of Allergy. Asthma & Immunology. 2008;101:114-120. Nicolay U, Kiessling P, Berger M, et al. Health-related quality of life and treatment satisfaction in North American patients with primary immunodeciency diseases receiving subcutaneous IgG self-infusions a home. Journal of Clinical Immunology. 2006;26(1):65-72. Notarangello, LD. Primary immunodeciencies. Journal of Allergy and Clinical Immunology, 2010;125(2): S182-S194. Nydegger, UE, Sturzenegger, M. Adverse effects of intravenous immunoglobulin therapy. Drug Safety, 1999;3:171-185 Ochs HD, Gupta S, Kiessling P. et al. Safety and efcacy of self-administered subcutaneous immunoglobulin in patients with primary immunodeciency diseases. Journal of Clinical Immunology. 2006;26(3):265-273. Orange JS, Hossny EM, Weiler CR, et al. Use of intravenous immunoglobulin in human disease: A review of evidence by members of the Primary Immunodeciency Committee of the American Academy of Allergy, Asthma, and Immunology. Journal of Allergy and Clinical Immunology. 2006;117(4):S525-S553. Quartier P, Debre M, De Blic J, et al. Early and prolonged intravenous immunoglobulin replacement therapy in childhood agammaglobulinemia: A retrospective study of 31 patients. Journal of Pediatrics. 1999;134(5):589596. Quinti I, Soresina A, Guerra A, et al. Effectiveness of immunoglobulin replacement therapy on clinical outcome in patients with primary antibody deciencies: Results from a multicenter prospective cohort study. Journal of Clinical Immunology. 2011;31(3):315-322. Pierce JR, Jain N. Risks associated with the use of intravenous immunoglobulin. Transfusion Medicine Review. 2003;17(4):241-251. Roifman CM, Berger M, Notarangelo LD. Management of primary antibody deciency with replacement therapy: Summary of guidelines. Immunology and Allergy Clinics of North America. 2008;28:875-876.

44

IDF GUIDE FOR NURSES

Schleis TG. The process: New methods of purication and viral safety. Pharmacotherapy, 2005;25(11 pt2):73S-77S. Sekul EA, Cupler EJ, Dalakas MC. Aseptic meningitis associated with highdose intravenous immunoglobulin therapy: Frequency and risk factors. Annals of Internal Medicine. 1994;121(4):259-262. Shapiro R. Subcutaneous immunoglobulin therapy by rapid push is preferred to infusion by pump: A retrospective analysis. Journal of Clinical Immunology. 2010;3:301-307. Shearer WT, Cunningham-Rundles C, Ochs HD. Primary immunodeciency: Looking backwards, looking forwards. J of Allergy Clin Immunol. 2004; 113: No. 4. Shehata N, Palda V, Bowen T, et al. The use of immunoglobulin therapy for patients with primary immune deciency: An evidence-based practice guideline. Transfusion Medicine Reviews. 2010;24(1):s28-s50. Siegel J. The product: All intravenous immunoglobulins are not equal. Pharmacotherapy. 2005;25(11 pt2):78S-84S. Stein MR, Nelson RP, Church JA, et al. Safety and efcacy of PrIVIgen, a novel 10% liquid immunoglobulin preparation for intravenous use, in patients with primary immunodeciencies. Journal of Clinical Immunology. 2009;29(1):137-144. Toubi E, Etzioni A. Intravenous immunoglobulin in immunodeciency states: State of the art. Clinical Reviews in Allergy and Immunology. 2005;29(3):167-172. United States Food and Drug Administration. Letter to manufacturer of immune globulin intravenous (Human) (IGIV) required updates to product labeling. 2008. Available at http://www.fda.gov/cber/ltr/IgIV101603.htm. Accessed August 30, 2011. Wasserman R. Pharmacokinetics and safety of subcutaneous immune globulin (human), 10% caprylate/chromatography puried in patients with primary immunodeciency disease. Clinical and Experimental Immunology. 2010;161:518-526. Wasserman RL, Melamed I, Kobrynski L, et al. Efcacy, safety, and pharmacokinetics of a 10% liquid immune globulin preparation (GAMMAGARD LIQUID, 10%) administered subcutaneously in subjects with primary immunodeciency disease. Journal of Clinical Immunology. 2011;31(3):323-331. Wood P, Stanworth S, Burton J, et al. Recognition, clinical diagnosis and management of patients with primary antibody deciencies; A systematic review. Clinical and Experimental Immunology. 2007;149:410-423.

IMMUNE DEFICIENCY FOUNDATION

45

APPENDIX A: QUICK NURSES GUIDE FOR T R O U B L E S H O O T I N G S C I G A D M I N I S T R AT I O N Leaking at the site

I I

Assess catheter: is it xed securely?

Assess placement: may be in a location that is subject to movement advise regarding selection of site, assess amount of subcutaneous tissue vs. muscle. Assess length of catheter: may be too short can suggest catheter brand change. Assess volume that is being infused: may be too much volume per individual site.

Local irritation, i.e. redness, swelling, itching

I

Assure that this is normal reaction should diminish in 24-48 hours, denitely by 4 days.

Assess size: mosquito bite, raised wheel, quarter, plum, peach, or grapefruit? size should be consistent with volume being infused and amount of subcutaneous tissue on patients; thinner patients may have more prominent raised area and may need to decrease amount of volume per site. Assess length of catheter: may be too short, can suggest longer catheter length or brand change to avoid. Advise use of gentle massage or warm compress postinfusion. Assess if tape allergy: change to paper/hypo-allergenic tape. Assess if rotating sites appropriately; may decrease frequency of rotation. Decrease volume per site and/or increase infusion time. When priming the subcutaneous needle sets, do not allow excessive drops of IgG to cover needle or prime dry leaving a small amount of air before needle. It has been suggested that the IgG tracked through the intradermal space can cause site reactions such as redness and itching in some patients. Apply topical Benadryl cream to site during and after infusion.
| IDF GUIDE FOR NURSES

46

Extreme discomfort with needle

I I

Assess length: may be too long and irritating abdominal wall.

Try catheter that allows introducer needle to be removed (Minimed Sof-Set). Try Emla cream or topical anesthetic prior to insertion.

Blood return observed

I

In single-site tubing, remove and discard. Use new set. Notify supplier of need for replacement sets. In multi-site sets, clamp off the tubing that shows the blood return and then remove the catheter from that site. Check with prescribing physician regarding selecting alternative for accommodating fewer sites. Infuse the drug with the remaining appropriately located sites, thus increasing volume per site. May need to recalculate to a slower rate of infusion if appropriate. Consider previous history of site reaction and other factors. Infuse the original amount of volume per site with the sites that are in place. When completed, repeat the infusion session with new site to accommodate the remaining volume from the site that had blood return. Change entire set up and start over.

Long infusion times

I

Assess technique for infusion: solution brought to room temperature?

Check patency of tubing, number of sites, and volume per site. Decrease volume per site. Assess infusion rate settings, correct selection of tubing size and length to match infusion rates, check pump function, battery function, etc. Arrange observation of patient technique (SPP or ofce visit).
IMMUNE DEFICIENCY FOUNDATION | 47

Needle contaminated by touching, dropping, etc.

I

Infusion pump stops during the infusion

I

Discard in appropriate waste container and use new one.

Check battery or for any line occlusion. Do not override occlusion alarm and increase psi delivered.

Check sets for down line occlusion. Multi-site sets may cause occlusion alarm due to co-dependence of lines. Change catheter brands or use single independent lines that equally connected off a multi extension pigtail. Use 24 gauge catheter needle. Change type of infusion pump to simple syringe driver. Contact SPP or supplier for further information. If necessary, maintaining a closed system (leaving all connections intact), remove syringe, leave tubing attached to site and manually push plunger forward slowly to deliver remaining volume. Depending on volume, this may take some time.

Difficulty with manipulating syringes for filling

I

Lubricate the barrel of syringe for easy manipulation by aseptically pulling back on the syringe, and moving it up and down before drawing up solution or lling with air. Pull back the amount of air to be infused into the vial and then attach the needle aseptically to the syringe. Mark the level of cc to which the syringe should be drawn back by placing tape on the outside barrel at the necessary level.

48

IDF GUIDE FOR NURSES

APPENDIX B: IMMUNE DEFICIENCY F O U N D AT I O N R E S O U R C E S

Immune Deficiency Foundation www.primaryimmune.org 800.296.4433 idf@primaryimmune.org

www.primaryimmune.org/healthcare-professionals
I

Services for Healthcare Professionals

IDF Nurse Advisory Committee: Comprised of exceptional nurses to support the mission of the IDF. Available as a resource for nurses administering immune globulin therapy or treating patients with primary immunodeciency diseases. IDF Medical Advisory Committee: Comprised of prominent immunologists to support the mission of the IDF. Available as a resource for clinicians diagnosing and treating patients with primary immunodeciency diseases.

IDF Consulting Immunologist Program: A free service for physicians which provides consults with expert clinical immunologists on issues of diagnosis, treatment and disease management. USIDNet: The United States Immunodeciency Network (USIDNet), an international consortium established to advance scientic research in the primary immunodeciency diseases through peer reviewed research grants, education and mentoring programs, DNA and cell repository, and patient registries. Administered by IDF. IDF & USIDNet LeBien Visiting Professor Program: Promote improved knowledge by providing your faculty with a Visiting Professor with expertise in primary immunodeciency disease. Offers Grand Rounds and clinical presentations at medical institutions throughout North America.

IDF Online Continuing Education Course for Nurses: A free, 5-hour, accredited course for nurses that provides an update on primary immunodeciencies, immunoglobulin therapies and the nurses role with these therapies: www.primaryimmune.org/healthcare-professionals/ continuing-education-course-for-nurses

IMMUNE DEFICIENCY FOUNDATION

49

www.primaryimmune.org/patients-and-families
I

Services for Patients and Families

Ask IDF: Contact IDF with questions about living with primary immunodeciency diseases through the IDF web site: www.primaryimmune.org/patients-and-families/ask-idf. IDF has a vast reserve of innovative resources and individualized assistance to help with the unique aspects of living with a primary immunodeciency. From learning more about the diseases, to understanding insurance coverage, to lifestyle issues and more, be sure to Ask IDF.

Peer Support: Connecting people and patients who share similar relationships to PIDD.

Locate a Physician: Contact IDF to nd a physician in your area who is an expert on PIDD.

Patient Assistance Resources: Individualized assistance is available for patients experiencing problems with insurance denials for treatment, reimbursement issues, concerns with Medicare or Medicaid, disability, and accessing copayment and premium assistance. Resources and tools are available to help tackle insurance challenges. Information about Patient Rights: Patients can contact IDF to learn about their rights concerning product choice and treatment options, employment and school issues, as well as fair treatment, privacy or other rights.

IDF eHealthRecord: An electronic personal health record designed for the primary immunodeciency community to help organize health information in one place.

50

IDF GUIDE FOR NURSES

Programs for Patients and Families

I

Local Patient Meetings: Education programs featuring local experts and networking opportunities.

Operation Outreach: Patient education meetings designed to strengthen underserved areas.

IDF Youth Programs: Designed for children diagnosed with a PIDD or have a family member with this condition. IDF Teen Escape: Weekend program developed to acquaint teens diagnosed with PIDD.

IDF Retreats: Weekend events for all ages that feature medical and life management sessions.

IDF National Conference: The world's largest gathering of families affected by PIDD.

Volunteer: Network of volunteers who provide peer support, create awareness, help host educational meetings, advocate for public policy, visit plasma centers and organize fundraising events throughout the country. Scholarship Program: Awards for students living with primary immunodeciency diseases who plan on completing their secondary education.

IDF Plasma Centers Partners Program: Awareness and fundraising initiatives within plasma centers across the country arranged by IDF that highlights the work of plasma center staff members, plasma donors and IDF volunteers.

Take the Zebra Challenge!: Fundraising campaign that provides the IDF community with multiple resources to create personal fundraisers and teach the world about zebras.

IMMUNE DEFICIENCY FOUNDATION

51

All publications can be downloaded and printed at www.primaryimmune.org. Alternatively, you can order a hard copy (if it is available).

Publications

For patients and families:

I

Patient & Family Handbook for Primary Immunodeciency Diseases 4th Edition Our Immune System (Childrens Book) IDF School Guide Information about Students with Primary Immunodeciency Diseases Bill of Rights for Patients with Primary Immunodeciency Disease IDF Diagnostic & Clinical Care Guidelines for Primary Immunodeciency Diseases 2nd Edition IDF Guide for Nurses on Immunoglobulin Therapy for Primary Immunodeciency Diseases 3rd Edition Clinical Focus on Primary Immunodeciencies:

For healthcare providers:

I

Clinical Update in Immunoglobulin Therapy for Primary Immunodeciency Diseases Subcutaneous IgG Therapy in Immune Deciency Diseases Primary Humoral Immunodeciency Optimizing IgG Replacement Therapy The Clinical Presentation of Primary Immunodeciency Diseases Treatment and Prevention of Viral Infections in Patients with Primary Immunodeciency Diseases IgG Subclass Deciency Immunization Of The Immunocompromised Host

52

IDF GUIDE FOR NURSES

Communications
I I

IDF Advocate: Newsletter, published three times per year.

IDF Common Ground, www.idfcommonground.org: An online community for teens with primary immunodeciency diseases. IDF Blogs: Include updates on important issues. Allow users to comment, submit news, and share posts:

IDF Friends, www.idffriends.org: A social network exclusively for the primary immunodeciency community.

Primary Immune Tribune: E-newsletter, published monthly.

IDF Community In Action: www.idfcommunityinaction.org For all members of the PIDD community to share information about their IDF activities. Celebrates all the ways that individuals are making a difference in the IDF community.

IDF Policy Matters: www.idfpolicymatters.org Designed to inform individuals in the PIDD community about important policy issues that directly impact patients. Intended to give insight into the political world and raise awareness for IDF advocacy issues and current campaigns. IDF SCID Newborn Screening: www.idfscidnewbornscreening.org Documents the ght to establish Severe Combined Immunodeciency (SCID) newborn screening programs in all 50 states. Babies with SCID appear healthy at birth, but without early treatment, most often by bone marrow transplant from a healthy donor, these infants cannot survive. Testing for SCID is not currently included in the newborn screening panels of all states. IDF Zebra Challenge: www.idfchallenge.org For all members of the PIDD community to showcase how they took the IDF Zebra Challenge and raised funds for IDF. A place to share ideas and successes, and help inspire others to take the challenge. IDF Plasma Centers Partners Program: www.idfplasmacenterpartners.org Promotes IDF activities in plasma collection centers. Applauds all the ways that our partners volunteers, plasma donors and participating plasma centers are making a difference in the IDF Community.

IMMUNE DEFICIENCY FOUNDATION

53

Public Policy Initiatives

I

Advocacy efforts monitor public policy issues that are critical to patients at national and state levels, including Medicare Patient IVIG Access Act, SCID Newborn Screening, Health Insurance lg Guidelines and more.

Grassroots advocacy program mobilizes members of the PIDD community to contact their government representatives to promote healthcare legislation that will positively affect the community. IDF Advocacy Center features Action Alerts, enabling users to easily voice their concerns to decision makers, and the IDF Advocacy Channel, featuring patient and caregiver stories: www.primaryimmune.org/idf-advocacy-center. Insurers are Not Doctors is IDFs campaign against harmful insurance formularies for immunoglobulin replacement therapy: www.InsurersareNotDoctors.org.

Patient Notification System (PNS) www.patientnoticationsystem.org PNS is a free, condential, 24-hour communication system providing information on plasma-derived and recombinant product withdrawal and recalls. Led by the Plasma Protein Therapeutics Association (PPTA), PNS was developed by the producers and distributors of plasma products with direct input from consumers.

Additional Resources

54

IDF GUIDE FOR NURSES

Information regarding the immunoglobulin products currently licensed in the United States is available from each specic manufacturer via the individual corporate websites. The manufacturers of Ig often provide up-to-date information and added nancial resources for patients and families dealing with primary immunodeciency diseases on their websites. The resources vary over time and between manufacturers. At press time, the following is a list of current manufacturers with product name(s) and contact information: Baxter Healthcare Corporation Gammagard S/D, Gammagard Liquid www.baxter.com 800.422.9837 (800.4Baxter) Bio Products Laboratory Gammaplex www.bpl.co.uk

Product Information

CSL Behring Carimune NF, Hizentra, Privigen www.cslbehring-us.com 800.504.5434 Grifols Flebogamma DIF, Gamunex - C www.grifolsusa.com 888.474.3657 (888.GRIFOLS) Kedrion Gammaked www.kedrionusa.com 855.353.7466 Octapharma Octagam www.octapharma.us

This publication has been made possible through a generous grant from

Immune Deciency Foundation 40 West Chesapeake Avenue, Suite 308 Towson, MD 21204 800.296.4433 www.primaryimmune.org