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Immunoglobulin Therapyfor Primary Immunodeficiency Diseases

IDF Guidefor NursesImmunoglobulin Therapy forPrimary ImmunodeficiencyDiseases

IDF GUIDE FOR NURSES

IMMUNOGLOBULIN THERAPY FORPRIMARY IMMUNODEFICIENCY DISEASES

THIRD EDITION

COPYRIGHTS 2004, 2007, 2012 IMMUNE DEFICIENCY FOUNDATIONCopyright 2012 by the Immune Deficiency Foundation, USA.

PRINT: 12/2013

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This publication has been made possiblethrough a generous grant from

IDF Guide for NursesImmunoglobulin Therapy for

Primary Immunodeficiency Diseases

Third Edition

Immune Deficiency Foundation40 West Chesapeake Avenue, Suite 308

Towson, MD 21204800.296.4433

www.primaryimmune.org

Editor:

M. Elizabeth M. Younger CRNP, PhDJohns Hopkins, Baltimore, Maryland

Vice Chair, Immune Deficiency Foundation Nurse Advisory Committee

Associate Editors:

Rebecca H. Buckley, MDDuke University School of Medicine, Durham, NC

Chair, Immune Deficiency Foundation Medical Advisory Committee

Christine M. BelserImmune Deficiency Foundation, Towson, Maryland

Kara MoranImmune Deficiency Foundation, Towson, Maryland

Contributors:

Carla Duff CPNP, CCRP, MSNUniversity of South Florida, St. Petersburg, Florida

Kristin B. Epland FNP, MSNMidwest Immunology Clinic and Infusion Center, Plymouth, Minnesota

Elyse Murphy RN, BSNCSL Behring, King of Prussia, Pennsylvania

Immune Deficiency Foundation Nurse Advisory Committee:

Loris Aro, RNSussman & Associates Immunology

Amy Meyer, RN, CPNP-PCChildren’s Hospitals & Clinics of Minnesota

William Blouin, MSN, ARNP, CPNMiami Children’s Hospital

Maggi Dodds, RN, MS, CPNPTexas Children’s Hospital

Carla Duff, RN, BSN, CCRPUniversity of South Florida

Kristin Epland, FNP - ChairMidwest Immunology Clinic

Vanessa Howard, BSN, MSN, FNPSt Jude Children’s Research Hospital

Terry Raburn, RN, BSN, ACRNTexas Children’s Hospital

Jeanette Scott, BSN, RNSanta Clara Valley Medical Center

Debra Sedlak, CPNPDuke University Medical Center

Gretchen Vaughn, RN, MSN, CPNPCincinnati Children’s Hospital Medical Center

M. Elizabeth M. Younger, CPNP, PhD - Vice ChairJohns Hopkins Hospital

About the Immune Deficiency Foundation . . . . . . . . . . . . . . . . 2

Introduction to Primary Immunodeficiency 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 Nurse’s Guide for TroubleshootingSCIG Administration . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 46

Appendix B: Immune Deficiency Foundation Resources. . . . 49

TABLE OF CONTENTS

I M M U N E D E F I C I E N C Y F O U N D A T I O N | 1

ABOUT THE IMMUNE DEFICIENCY FOUNDATION

Immune Deficiency FoundationThe Immune Deficiency Foundation (IDF) is the national non-profit patient organization dedicated to improving the diagnosis,treatment and quality of life of persons with primaryimmunodeficiency diseases through advocacy, education andresearch. IDF was founded in 1980 by parents of children withprimary immunodeficiencies and their physicians. At that time,there were few treatments for primary immunodeficiencydiseases, almost no educational materials for patients, no publicadvocacy initiatives, and little research being done. In the pastthirty years, IDF has pursued an aggressive agenda toremediate these problems and has made tremendous strides inthe following areas:� Helping the patient and professional communities gain a

broader understanding of primary immunodeficiencydiseases through comprehensive education and outreachefforts;

� Promoting, participating in, funding and supporting researchthat has helped characterize primary immunodeficiencydiseases and given healthcare providers substantiallyimproved treatment options for the care of patients withprimary immunodeficiency diseases;

� Addressing patient needs through public policy programs onlocal, national and international levels by focusing on issuessuch as insurance reimbursement, patient confidentiality,SCID newborn screening, preventing genetic discrimination,ensuring the safety and availability of immunoglobulintherapy, and maintaining and enhancing patient access to afull range of treatment options;

� Establishing supportive networks of patients andprofessionals to ensure that the needs of patients with primaryimmunodeficiency diseases are recognized and addressed.

2 | I D F G U I D E F O R N U R S E S

Primary immunodeficiency diseases represent a group of morethan 185 rare disorders. In the United States, approximately250,000 people are diagnosed with primary immunodeficiencydiseases. Thousands more go undetected. These individualslive throughout the country and experience a number ofproblems which have been documented by IDF. These patientproblems include:� Difficulty in finding specialized healthcare by immunologists or

care providers knowledgeable about immunodeficiency � An inordinate delay in reaching proper diagnoses� Problems with availability of appropriate treatment � Difficulties financing healthcare and treatment� Finding instructional materials about the specific 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 affectedindividuals to overcome these difficulties, thereby enabling themto live healthy and productive lives.


IDF Nurse Advisory CommitteeA Resource for Nurses and PatientsThe Immune Deficiency Foundation established the NurseAdvisory Committee in 1999. The committee is comprised ofnurse experts who have many years of managing and providingcare for patients with primary immunodeficiency diseases. Thegoal of the committee is, first and foremost, to improve thequality of healthcare received by patients with primaryimmunodeficiency diseases. This goal is primarily achieved byeducating and providing resources for patients’ caregivers. TheNurse Advisory Committee also increases awareness of primaryimmunodeficiency diseases through professional education andoutreach on local, national and international levels. TheCommittee is instrumental in increasing educational and peersupport opportunities for individuals and families affected byprimary immunodeficiency diseases.

The Nurse Advisory Committee is available as a resource fornurses providing therapy for or treating patients with primaryimmunodeficiency diseases. The committee is also available forpatients requiring assistance. Members can be reached bycontacting IDF at 800.296.4433 or [email protected].

Immunoglobulin replacement therapy is indicated for a significantnumber of patients with primary immunodeficiency diseases.The IDF Nurse Advisory Committee is proud to offer this guide tohelp nurses to administer this therapy, safely and effectively. Bydoing so, nurses are in a unique position to improve thetreatment experiences and provide a better quality of life forpatients living with primary immunodeficiency diseases.


The World Health Organization recognizes more than 185primary immunodeficiency diseases – some are relativelycommon, others are quite rare. Some affect a single cell withinthe immune system; others may concern one or morecomponents of the system. These diseases are classifiedaccording to the part of the immune system involved, either theadaptive or innate immune system. Immunodeficienciesinvolving adaptive immune responses are characterized byimpaired antibody production or function. Problems with innateimmunity are those which involve natural killer lymphocytes,neutrophils, monocytes, macrophages or the complementsystem.

Regardless of whether the problem is with the adaptive or innatesystem, patients affected with primary immunodeficiencies areat risk for infection with virtually any pathogen. Even organismswhich are not pathogenic in immunocompetent hosts can bepathogenic for people with immunodeficiencies. Theseinfections can be unusually severe or recurrent and they cansometimes be difficult to treat with conventional therapy. For themost part, primary immunodeficiencies are rare and, because ofthis, may go unrecognized. Often patients experience manyyears of recurrent infections before they are appropriatelydiagnosed.

Some primary immunodeficiencies are caused by a problemwith a single gene; others are caused by defects in multiplegenes. There can be a clear inheritance pattern, such as withthose immunodeficiencies that are x-linked diseases; for otherdiseases the inheritance pattern is less clear. It is believed thatsome primary immunodeficiencies develop over time and maybe the result of a combination of genetic and environmentalfactors. Therefore, primary immunodeficiencies may presentand be diagnosed at any age. Similarly, there can betremendous phenotypic and immunologic variability amongindividuals with the same diagnosis.

INTRODUCT ION TO PR IMARY IMMUNODEF IC IENCY D ISEASES


Many patients with primary immunodeficiencies have significantco-morbidities. Some of these co-morbidities may be related tothe immunodeficiency itself. For example, a patient withrecurrent pneumonias may have irreversible lung damage(bronchiectasis) because of the infections. It is also known thatpatients with primary immunodeficiency diseases may have apredisposition to autoimmune diseases including such problemsas autoimmune cytopenias, inflammatory bowel disease orrheumatoid arthritis. Sometimes the immunodeficiency isdiagnosed after a presentation of autoimmune disease. Someimmunodeficient patients may also have a greater risk forlymphoreticular cancers, such as lymphocytic leukemias,multiple myeloma or lymphomas, compared to that risk in thegeneral population.

Patients with antibody disorders are the largest group of peoplewith primary immunodeficiencies. These include patients withselective IgA deficiency, by far the most common primaryimmunodeficiency disease; patients withhypogammaglobulinemia and impaired antibody responses; andpatients with combined B and T cell problems. For some ofthese diagnoses, but not all, immunoglobulin replacementtherapy is the standard of care. This therapy providesantibodies from thousands of plasma donors to those who donot have and/or cannot make protective levels of antibody.


Concentrated human immune globulin preparations first becamewidely available during World War II and were used forprophylaxis against infectious diseases such as hepatitis,measles and polio. The use of immunoglobulin as replacementtherapy for primary immunodeficiency was described by Dr.Ogden Bruton in 1952. Dr. Bruton treated a boy diagnosed withX-linked agammaglobulinemia with subcutaneous injections ofimmunoglobulin from immunocompetent human plasma donors.

Initially, immunoglobulin was given predominantly byintramuscular injections. These injections were painful, and themaximum doses that could be given were limited because of thevolumes involved. In the early 1980’s, preparations that couldbe safely given by the intravenous route were first licensed in theU.S. Intravenous immunoglobulin replacement therapy or IVIG,also referred to as IGIV, was generally well tolerated by mostpatients and became the standard of care for treatment ofpatients with primary immunodeficiencies with antibodydeficiencies. Larger doses of immunoglobulin could be givenvia this route, more closely mimicking the body’s own productionof antibodies. Better infection prophylaxis was achieved,resulting in significant improvements in the patients’ conditionsand outcomes.

In 2006, the first commercial preparations for subcutaneousimmunoglobulin replacement therapy (SCIG) were approved bythe United States Food and Drug Administration (FDA). Thesepreparations, given in smaller doses and more frequently thanIVIG provide very stable, consistent levels of IgG as opposed tothe peaks and troughs associated with the intravenous route.For some patients, this is an important consideration.

CL IN ICAL USES FOR IMMUNOGLOBUL IN REPLACEMENT THERAPY


All immunoglobulin preparations currently available in the U.S.are manufactured using donor pools from 10,000 to 60,000 unitsof donated human plasma. They contain IgG antibodies againsta broad spectrum of vaccine antigens and infectious agents. Allpreparations contain ≥ 96% IgG. Most also contain some IgAand trace amounts of other plasma proteins. There aredifferences in the manufacturing processes and in the stabilizingagents used for each manufacturer’s products.

Immunoglobulin (Ig) therapy is indicated as replacementtherapy for primary and secondary immunodeficiencies inthose patients who do not make sufficient amounts of specificantibodies to adequately protect themselves from infectiousdiseases and those whose antibodies do not functioncorrectly or those people with poor immunologic memory.Two examples of primary immunodeficiency conditionsrequiring replacement therapy are agammaglobulinemia(either x-linked or autosomal) and common variableimmunodeficiency (CVID). Examples of secondaryimmunodeficiencies include hypogammaglobulinemiacaused by chemotherapy or monoclonal antibody therapy, aswell as immunosuppressive therapies.

In addition to antibody replacement, immunoglobulin also hasanti-inflammatory and/or immunomodulatory effects. As such, itis sometimes used to treat patients with a variety of conditionsother than primary immunodeficiency diseases. Immunoglobulintherapy has been demonstrated to be efficacious in thetreatment of such diseases as idiopathic thrombocytopeniapurpura (ITP), Kawasaki disease and some neuromusculardiseases. However, some of these other uses are experimentaland/or “off-label,” which means that the FDA has not approvedthe use of immunoglobulin for those particular conditions.


Table 1: FDA Approved Uses of Immunoglobulin (Ig)

Clinical Condition Ig Indication

Chronic InflammatoryDemyelinating Polyneuropathy(CIDP)

Improve neurological symptoms

Primary HumoralImmunodeficiency

Antibody replacement therapy

Kawasaki DiseasePrevent coronary arteryaneurysms

Idiopathic ThrombocytopeniaPurpura (ITP)

Increase platelets counts toprevent and control bleeding

B-cell Chronic LymphocyticLeukemia

Prevent recurrent bacterialinfections

Table 2: Immunodeficiencies that ALWAYS Require Ig Replacement Therapy

Agammaglobulinemia (X-linked, autosomal, or acquired)

Common Variable Immunodeficiency

Hyper IgM Syndrome

Severe Combined Immunodeficiency (SCID) before and sometimesafter bone marrow transplant

Table 3: Immunodeficiencies 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


There are several different brands of immunoglobulin andhyperimmune products currently licensed for use in the UnitedStates. The U.S. Food and Drug Administration (FDA) hasmandated that all immunoglobulin administered in the U.S. mustbe manufactured from plasma donated in this country. Allmanufacturing must be done in FDA approved facilities.

Immunoglobulin is a plasma product. Thousands of carefullyscreened and tested donors provide plasma for a single lot ofproduct. It is produced via a multifaceted manufacturingprocess designed to remove and/or inactivate bacterial and viralpathogens. These processes vary from manufacturer tomanufacturer but include such steps as cold alcoholfractionation, low pH incubation, nanofiltration, chromatographyand solvent/detergent treatment. While the immunoglobulinmanufactured in the U.S. is a very safe product, the possibility oftransmission of existing or emerging pathogens cannotabsolutely be ruled out.

All immunoglobulin products are mostly IgG (> 96%). They alsocontain trace amounts of IgM and IgA. The remainder of theproducts is made up of stabilizing agents. Products vary inconcentration, pH, stabilizing agents, osmolarity and osmolality,as well as sugar and sodium content. There is variability inadministration factors as well, including the form of the drug(lyophilized or liquid), shelf life, approved means ofadministration (intravenous and/or subcutaneous) andprescribed infusion time. All of these factors need to becarefully considered when choosing a product for a particularpatient. (See Table 4)

1 0 | I D F G U I D E F O R N U R S E S

PRODUCT SELECT ION ANDCHARACTER IST ICS

I M M U N E D E F I C I E N C Y F O U N D A T I O N | 1 1

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PreparationsImmunoglobulin products are supplied as liquids or lyophilizedpowders (freeze dried powder that requires reconstitution).Some liquids require refrigeration; others are stored at roomtemperature. It is important to follow the manufacturer’sspecifications regarding storage. Any liquid which has beenfrozen should be discarded. Refrigerated products should beallowed to warm to room temperature before administration, asadverse effects can be associated with the administration ofproducts that are too cold.

Lyophilized products can be stored at room temperature beforereconstitution. It is possible for these products to be prepared atmore than one concentration depending on the amount ofdiluent added. Possibilities for different concentrations arespecified in the manufacturer’s prescribing data. Nurses may beasked to reconstitute lyophilized products in the home or theinfusion clinic. It is critically important to be aware of and tofollow manufacturer’s guidelines, prescriber’s orders and aseptictechnique, when reconstituting these products.

StabilizersStabilizers include different sugars and/or amino acids that areadded to immunoglobulin products to stabilize the IgGmolecules and prevent them from aggregating. Thesestabilizing agents may pose a risk for some patients. Forexample, products containing glucose should be usedcautiously in patients with diabetes. Similarly, some sucrosecontaining lyophilized products have been implicated in causingor exacerbating renal disease.


IgA LevelsThere are small amounts of IgA in all immunoglobulin products.If a patient has an absence of IgA they may have anti IgAantibodies, then that patient could be at risk for anaphylaxis.Unfortunately, there is no commercial assay available formeasuring IgE antibodies to anti-IgA. Fortunately, antibodydeficient patients are seldom able to mount IgE responses, sothis is not a widely prevalent problem. Patients with low orundetectable levels of IgA may be able to tolerate allimmunoglobulin without problems; however, these patients(particularly the CVID patients) should be carefully monitored.The first infusion should always be administered in a controlledsetting where emergency treatment can be administeredimmediately should problems occur. If the infusion is tolerated,the patient is not likely to have subsequent problems with IgA-containing products.

Product Integrity All products should be carefully inspected before administration.The packaging should be inspected for tampering as should thevials and their closures. Any evidence of tampering should bereported to the supplier and/or manufacturer and the productshould not be used.

Reconstituted and liquid products should not be given if there isparticulate matter, precipitate crystals or fibers in it. Productsthat have been frozen should not be given. For the most part,immunoglobulin should be clear although there can be a slightamount of cloudiness at times. The manufacturer’s packageinsert will provide information about the range of color as thiscan vary from one product to another. If the nurse or patient hasany doubts at all about the integrity of the product at all, itshould not be administered.


Documentation

All IVIG infusions should be carefully documented.Documentation should include: � The patient’s 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 specific 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 patientteaching interventions and documentation of the patients’ abilityto administer their own infusions.


Nursing Responsibilities Whether the nurse is administering an intravenous infusion orteaching patients to administer their own subcutaneous infusion,safety should always be the first priority. The prescriber’s ordersshould be carefully followed and any problems with the ordersshould be addressed and resolved before the infusion.Communication of potential issues and problems so that theycan be proactively addressed is critical. The following are broadguidelines for nursing interventions prior to, during and afteradministration of immunoglobulin replacement therapy. Theseguidelines are offered to help infusion nurses minimize problemsand adverse effects, and safely provide a successful infusionexperience for the patient.

Key Pre-infusion Assessments � Assess that the immunoglobulin product ordered is

appropriate for the patient. Communicate potential problemsto the prescriber. It is important to be aware of thedifferences between the various products available. Aspreviously discussed, the qualities of a particular productmay affect the tolerability and success of an infusion.Remember, not all immunoglobulin products are the sameand are, accordingly, NOT interchangeable. The first dose ofany product should be administered in a controlled setting,where emergency equipment and treatment is readilyavailable. The transition to home infusions can take placeafter it has been demonstrated that a particular product istolerated. Should it be necessary to change products, thefirst infusion of the new product should, again, be in acontrolled setting.

� Assess product integrity. If the protective seals are not intact,the dispensing pharmacy should be notified immediately andthe product should not be given.

DEL IVERY OF IMMUNOGLOBUL INREPLACEMENT THERAPY



� Assess product temperature. The immunoglobulin should beat room temperature before the infusion. Solutions should beallowed to come to room temperature naturally. Productintegrity may be compromised (denatured) by freezing orheating. NEVER put product into the microwave for warming.

� Assess level of patient’s understanding of therapy.� Assess the patient’s general health and hydration status. It is

important to document and inform the prescriber of any newhealth problems which have arisen since the last infusionand/or any new medications the patient is taking as thesemay have an impact on prescribed therapy. If the patient ispoorly hydrated, consideration should be given to thepossibility of providing some hydration, either enterally orparenterally, before the infusion.

� Assess for any weight loss or gain. Immunoglobulinreplacement therapy is prescribed based on weight. Anysignificant change (greater or less than 10%) may indicate aneed for dosage increase or (less likely) reduction.

� Assess heart rate and respiratory status. Patients withcongestive heart failure or who are at risk of fluid overload,especially, should be assessed carefully before beginning theinfusion. Both the volume of fluid infused and thecharacteristics of the fluid (osmolality, sodium content) couldexacerbate these problems. Patients should be reassessedfrequently during the infusion to be sure that there is nochange in respiratory status, which could indicate fluidoverload. Diuretics may be prescribed before, during or afterthe infusion to prevent or relieve respiratory distress and/orcomplications associated with fluid overload in these patients.

� Assess for fever prior to the start of infusion. If fever ispresent, the prescriber should be notified for directions forproceeding with or deferring the infusion. If the patient hasan acute febrile illness or other indications of an infection arepresent, the infusion may need to be postponed until thepatient is treated with antibiotics and/or the fever subsides.Administration of intravenous immunoglobulin when thepatient has an acute infection may lead to adverse effectsdue to the formation of immune complexes.


� Assess the need for premedication. Although the patientshould have communicated any adverse events associatedwith previous infusions to the prescriber, this may not havehappened. It is important to establish that the patienttolerated his/her previous infusion without problems. Ifproblems did occur, then the prescriber should be notifiedand asked if premedication should be given. Premedicationsmay be indicated to diminish the risk of infusion-relatedadverse events. Examples of premedications includesystemic corticosteroids, antihistamines, antiemetics,acetaminophen and/or NSAIDs.

� Assess the need for localized anesthesia and obtain an orderas necessary. Children, especially, may prefer to have topicalanesthesia applied in advance of needle insertion to numbthe sites at which needles or intravenous catheters will beplaced.

� Assess preparedness for emergency situations. Emergencyequipment should be readily available during the infusion.Emergency medications including epinephrine,diphenhydramine and parenteral fluids should be checked toensure that they have not expired. The nurse should ensurethat he/she is prepared to respond to an emergency and thatorders are in place for this response. A phone with which tocall 911 should always be available. A protocol forcommunicating with the prescriber for both routine andemergency issues should be in place.

� Assess need for laboratory blood work prior to start ofinfusion. For patients receiving intravenous immunoglobulin,trough levels of IgG are an important monitoring tool. Theselevels need to be drawn immediately before beginning aninfusion. The nurse should review the results of previous labwork with the patient and communicate with the prescriber toensure that routine monitoring labs are done as ordered.

� Assess the patient’s experience with previous infusions. It isimportant for the nurse to listen to the patient and ensure thatestablished routines are followed to avoid causing unduestress. Children, in particular, may have routines in place toassist them in dealing with both the physical andpsychological impacts of infusions.


Key Intra-infusion Assessments � Assess the patient to ensure that the infusion is being

tolerated. The nurse should listen carefully to any complaintsand be sensitive to any alteration in the patient’s baselinestatus. Vital signs should be assessed as ordered and asindicated.

Key Post-infusion Assessments� 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 infusionrelated, alterations to the infusion protocol may be necessary.

� Assess the need for premedications for future infusions andensure that the premedications will be available for the nextinfusion.

� Assess the patient for his/her knowledge about the nextinfusion. It is important for the patient to know when the nextinfusion is due and what his/her responsibilities regarding thisinfusion are.

Routes of AdministrationImmunoglobulin replacement therapy can be administeredintravenously (IVIG) or subcutaneously (SCIG). There aremultiple factors to consider when choosing the route ofadministration; careful consideration of these factors and theirrelationship to the individual patient is critical to ensuringsuccess. The patient’s wishes and whether these factorsrepresent a “pro” or a “con” to the patient should be considered.Factors and questions to consider include:� Efficacy of Therapy: IVIG is usually given every three to four

weeks. There is a peak in the level of IgG when the infusion isgiven and then the level declines to a trough before the nextinfusion, so there is a predictable rise and fall in levels. WithSCIG infusions, the drug is slowly absorbed and is givenmore frequently, usually weekly. Once steady state isreached, the level of IgG is remarkably consistent. Thisconsistency of level may be important for patients withconditions such as protein losing enteropathies or for patientson IVIG who have frequent breakthrough infections.


� Time Factor: IVIG infusions generally require three to fourhours a month in a single sitting. SCIG infusions require lesstime but are given, at least, weekly.

� Adverse reactions: There is a greater risk for systemicreactions with IVIG; local reactions are more common withSCIG. Patients who experience adverse reactions with IVIGand need premedication for their infusions may notexperience these problems with SCIG.

� Cost of Therapy: In addition to the cost of drugs, there is anadditional cost for nursing and/or overhead administration(infusion suite) costs with IVIG infusions. These costs are notuniform; the patient’s insurance benefits and out-of-pocketcosts need to be investigated.

� Patient Compliance: What is the patient’s level ofcommitment? Will the patient do unsupervised home infusionsof SCIG and follow up with appointments and lab work or iscloser management/supervision required?

� Comorbidities: Does the patient have another illness which willbe affected by therapy? For example, patients with cardiacdisease may do better with the smaller amounts of fluid usedin SCIG. Conversely, some patients may need a higher peakof IgG than can be achieved with SCIG and consequentlyIVIG may be a better choice for them.

� IV Access Issues: Is monthly IV access difficult? TheAmerican Academy of Allergy, Asthma and Immunologystrongly discourages the use of permanent indwelling ports orcentral venous lines in antibody deficient patients due to therisk of infection and thrombotic events. If peripheral access isconsistently difficult, SCIG may be a viable option.

� Availability of Nursing Resources: Home infusion nursingservices are not always available in every area of the countryand patients may not live close to infusion centers, makingself-infusion a desirable alternative.

INTRAVENOUS IMMUNOGLOBUL INTHERAPY

Intravenous immunoglobulin replacement therapy (IVIG) isgenerally given every three to four weeks at a dose ofapproximately 400-500 mg/kg/dose. It is well tolerated by themajority of patients, but it is important to note that, just as eachpatient may require a different immunoglobulin product, eachmay also require an individualized infusion regimen in order toachieve the desired therapeutic response. Once a successfulregimen has been developed, it should be carefully followed withevery infusion. This includes not only the rate of the infusion andnecessary premedications, but the specific product, as well.

Administration

Different products vary in their compatibility with normalsaline, sterile water or D5W, and manufacturer’s guidelinesshould be followed carefully. Administration of concomitantmedications through the same IV line should be avoided.Should medications be required prior to or during an infusion,it is recommended to flush the line with at least 5-10 ml ofcompatible fluid prior to administering the medication. Somemedications will precipitate when in contact with IVIG,especially furosemide or diazepam. No medications shouldbe directly administered into the same line simultaneouslywith the IVIG. If multiple medications are required, a secondIV line should be placed so as not to interfere with theinfusion. Another option is to piggyback the IVIG into theclosest port in a line where a compatible fluid is alreadyrunning. The compatible fluid can then be used as a flushif necessary.


Intra-infusion Assessments� Assess the rate of infusion. Prescriber’s orders and

manufacturer’s recommended rates of infusion shoulddetermine length of time for an infusion to take. Generally, aramp-up procedure is used for IVIG rates of infusion. Theinfusion is started slowly and the rate increased incrementallyapproximately every 15 to 30 minutes, as tolerated, until thepatient’s maximum rate of infusion is reached. Although thereis some literature reporting the tolerability of higher infusionrates, a general guideline to follow would be not to exceed themanufacturer’s recommended maximum rate. If a productchange is necessary, the process for assessment of tolerabilityand potential rate increases must again be taken slowly.

� Assess the vital signs prior to each rate change to ensure thatthe infusion is being tolerated. Hyper- or hypotension,increased heart rate, increased respiratory rate or effort, andfever could all be signs of problems. It is important to assessthe clinical relevance of any alterations in vital signs. Forexample, if a comfortable patient falls asleep, his/her bloodpressure, heart rate and respiratory rate may decrease andmay not represent a pathologic concern. Similar findings inanother patient may be signs of significant problems with theinfusion.

� Assess the need for comfort measures during the infusion,particularly if side effects occur. Both pharmacologic and non-pharmacologic interventions (supplying blankets or pillows,heating pads and encouraging the use of relaxationtechniques) may be indicated.

� Assess for signs of anaphylaxis. Although true IgE medicatedanaphylaxis in antibody deficient patients is rare, if a patienthas difficulty breathing, signs of tongue or throat swelling, afeeling that the throat is closing, stridor, wheezing and/or chesttightness, generalized urticaria, or extreme anxiety, the infusionshould be stopped and immediate emergency treatment,including calling 911, should be initiated.



Adverse ReactionsAlthough most patients do well with IVIG, there is the potentialfor adverse reactions. It is estimated that 15-30% of patientsexperience some kind of reaction to their IVIG infusions. Thesereactions can range from mild to severe; however, mostreactions occur during the initial 30 to 60 minutes of the infusionand are mild and self-limited. These reactions includeanaphylactoid problems such as headaches, chills and rigors;allergic reactions like urticaria and, potentially, anaphylaxis; andother problems such as aseptic meningitis. The most commonproblems are related to the rate of the infusion and thetemperature of the product. Reactions are more frequent withpatients who are therapy naïve, when therapy is given with adifferent product than the patient has previously been used toreceiving, and/or in those who are not truly antibody deficient orthose who have been off of therapy for a period of time. It isimportant to note that most reactions occur during the initial 30to 60 minutes of the infusion and are mild and self-limited.Regardless of the severity of a reaction, managing theseproblems requires timely interventions on the nurse’s part. Anursing policy and orders must be in place for dealing withthese issues.

There are risk factors that may identify persons at greater risk forhaving a reaction to IVIG. It is advisable to read the specificpackage insert for the IVIG product used, as the incidence andtypes of adverse events varies from product to product.

Types of Adverse Reactions � Pyogenic Reactions: These reactions are marked by a

significant rise in temperature and are usually accompaniedby other systemic symptoms. Fever is the most common sideeffect in children. Management of acute pyogenic reactionsincludes the use of antipyretic medications such asacetaminophen or ibuprofen. Persons who repeatedlyexperience temperature elevations during administration ofIVIG may benefit from premedication with an antipyretic/antiinflammatory such as acetaminophen, 30 to 60 minutes priorto initiation of the infusion.


� Allergic Reactions: True IgE mediated allergic reactions arerare in antibody deficient patients; however they can occur.In some cases, reactions to IVIG mimic those of true IgEmediated allergy but are actually due to activation ofcomplement or other mediator systems by the IVIG. Allergicreactions can lead to acute anaphylaxis and shock. If thesereactions occur, future use of IVIG is not precluded but, ofcourse, must be closely monitored in a controlled environment(NOT in the patient’s home). These reactions often begin witha generalized nonspecific feeling of unease. Patients maydescribe an uncomfortable feeling, such as a tighteningaround the neck, chest or abdomen. There may be difficultyswallowing, a choking sensation or difficulty breathing. Othersymptoms may include wheezing, flushing, hives, rapid orweak pulse, hypotension, sweating or an upset stomach withor without nausea, vomiting or diarrhea.

� Vasomotor Symptoms: These can occur with or withoutadditional cardiac manifestations. Blood pressure can eitherincrease or decrease, and may be accompanied by flushingor tachycardia. Patients experiencing such reactions mayreport shortness of breath or tightness in the chest.

� Anaphylactoid Reactions: These reactions most commonlyinclude headache, dizziness or lightheadedness. Patientscan also experience chills sometimes progressing to rigors,nausea and/or vomiting, back or hip pain, malaise, myalgiasand arthralgias. Frequently the patient reports anxiety and insome cases “a sense of impending doom.” The mostfrequent cause of these reactions is infusion at an excessivelyrapid rate or infusion of a drug which is colder than roomtemperature. Often, the patient will have elevated bloodpressure rather than hypotension.


Table 5: Potential Nursing Interventions for Dealing withAdverse Reactions to IVIG

Reaction Nursing Interventions

Chills/ Rigors

• Stop infusion.

• Administer prescribed medications.

• When symptoms resolve, restart theinfusion at the rate the patient wastolerating before the symptomsoccurred

Headache

• Administer acetaminophen or NSAID asprescribed.

• The patient’s hydration status may affectthe development of headaches; thepatient should make sure he/she isadequately hydrated on the day of theinfusion.

Migraine Headache(patients with a history ofand under treatment forheadache problems)

Pharmacologic:

• Administer prescribed anti-migrainemedications as soon as the first signs ofa migraine occur.

• Oral or IV steroids may help decreasethe intensity of the headache andshould be given if ordered.

Non-pharmacologic:

• Include comfort measures such asreducing auditory and visual stimuli,and applying cold compresses to thehead or back of the neck.

Malaise/Flu-likeSymptoms

• Resting after an infusion may help tominimize muscle aches or pain and todecrease excessive fatigue.

• Acetaminophen or NSAIDS andensuring adequate pre-infusionhydration may help with this problem.


Table 5: Potential Nursing Interventions for Dealing withAdverse Reactions to IVIG (cont.)


Urticaria

• Stop the infusion.

• Contact the prescriber.

• Administer prescribed antihistaminesand/or steroids.

• Observe for signs of true anaphylaxis; ifthey occur administer epinephrine andactivate the emergency response system(911).

Vasomotor Symptoms(Hypotension,Hypertension, Flushing orTachycardia)

• Stop infusion.

• Follow the prescriber’s order for fluidbolus, diuretics or other interventions oradminister fluid with hypotension,based on prescriber order. Administerdiuretics on prescriber’s order if fluidoverload is likely.

Nausea/Vomiting

• Stop the infusion.

• Administer prescribed antiemeticmedications.

• Provide comfort measures.

Back Pain/HipPain/Arthralgias/Myalgias

• Stop or slow the infusion.

• Administer acetaminophen or NSAIDSfor the discomfort.

• The use of a heating pad may bebeneficial.

Potential Post-infusion ReactionsPost-infusion reactions can occur immediately or as long as 72hours following the infusion. Symptoms associated with post-infusion reactions are usually less severe in nature but caninterfere with a patient’s quality of life. Common post-infusionreactions may include headache, low-grade fever, nausea,arthralgias and generalized malaise. Often patients describe a“flu-like” feeling. These reactions are generally managed withover-the-counter analgesics, antihistamines and may require ashort course of corticosteroids.

Headaches are more frequent in patients who have a history ofmigraine or cluster headaches. Some patients, particularlythose with histories of migraines at other times, may have severeheadaches and/or typical migraines up to 72 hours after theirinfusion. Over-the-counter analgesics are usually effective intreating these headaches, but they sometimes require theaddition of oral steroids. Severe, persistent posterior occipitalheadaches may be a sign of aseptic meningitis, which has beenreported in some patients after IVIG infusion.

Table 5 presents common adverse reactions and potentialnursing interventions. It is important to follow the prescriber’sorders when dealing with any infusion reaction. The prescribershould always be notified that a reaction has occurred and maywish to change immunoglobulin products or orderpremedications for future infusions. Reactions to IVIG candiminish with subsequent infusions, so the need forpremedications needs to be reassessed periodically.


Complications Associated with IVIG� Transmission of Bloodborne Pathogens: IVIG products are

manufactured from large numbers of carefully screenedhuman donors who have been tested for the absence ofhepatitis B surface antigen, hepatitis C antibody and HIVantibody, and by nucleic acid testing for HIV and HCV. Inaddition, all products are produced using techniques toremove or inactivate potentially contaminating viralpathogens. Viral inactivation and removal processes havedemonstrated reduction of the potential presence ofpathogenic prion agents that have been associated with thedevelopment of transmissible spongiform encephalopathysuch as variant Creutzfeldt-Jakob disease. Eachmanufacturer’s package insert will delineate these processes.

� Thrombotic Events: Thrombotic (vascular occlusive) eventshave been reported in association with IVIG. Themechanisms for these episodes may include increased bloodviscosity after high-dose IgG and/or the presence ofprocoagulant proteins in the IVIG preparation. Theseepisodes have been noted with increased frequency inpatients following rapid infusion protocols or patients with riskfactors such as prior thromboembolic events, thrombocytosis,or immobility. Thrombotic events are serious in nature andinclude chest pain, myocardial infarction, congestive cardiacfailure, transient (cerebral) ischemic attack (TIA) and stroke.Patients with risk factors for thrombotic events should follow aconservative infusion protocol, using a product with a low(5%) concentration, and proceed slowly and cautiously withincremental increases in the rate of infusion to a maximum of4 ml per kg of body weight per hour. Patients should begiven clear instructions regarding what post-infusionsymptoms should be reported immediately to their prescriber.


� Renal Adverse Events: Potential adverse effects involving thekidneys include acute renal failure, acute tubular necrosis,proximal tubular nephropathy and osmotic nephrosis. Therehave been rare reports of increased serum creatinine, oliguriaand acute renal failure occurring from one to seven days afterIVIG administration. Hyperosmolality and the presence ofsucrose have been implicated as factors contributing to renaladverse events. Patients who are not adequately hydratedprior to onset of the infusion, who have diabetes mellitus orany pre-existing renal insufficiency, those receivingnephrotoxic antibiotics, those who have paraproteinemia,and/or those who are over age 65 are at the greatest risk forthese problems. Renal function (serum Creatinine and BUN)and urine output should be carefully monitored in patients atrisk for developing renal adverse events. Using slow infusionrates during administration of IVIG and assuring adequatehydration are advised for such at-risk patients. As withpatients at greater risk for thrombotic problems, patients withthe potential for renal adverse events should be given clearinstructions regarding what post-infusion symptoms shouldbe reported immediately to their prescriber.

� Aseptic Meningitis: Cases of aseptic meningitis withheadache and positive meningeal signs have been reportedwith the use of IVIG in both standard replacement therapydosing and high dose therapy. The symptoms may occurduring the infusion, but more typically they usually developwithin 24 hours of the infusion. A previous history of migraineheadaches has been noted to be a risk factor. A neurologicexam is indicated for these patients to rule our bacterial orviral meningitis. Patients with aseptic meningitis havepleocytosis but no organisms in their cerebrospinal fluid.Treatment is symptomatic. The development of asepticmeningitis is an indication for a change in the immunoglobulinproduct used for future infusions. Premedication withcorticosteroids is also indicated for those with a previoushistory of infusion related aseptic meningitis.


� Transfusion-related Acute Lung Injury (TRALI): TRALI is a rarebut potentially devastating complication of blood componenttherapy characterized by severe respiratory distress,hypotension, fever, dyspnea, and tachycardia. Patientsexhibit pulmonary edema, hypoxemia, abnormal leftventricular function, and fever with a typical onset within oneto six hours after infusion of the product. Pulmonaryembolism and lung dysfunction due to "transfusion relatedacute lung injury" have also been observed during orimmediately after IVIG infusions. Patients at risk include thosewith a history of atherosclerosis, those who have multiplecardiovascular risk factors, those of advanced age, those withimpaired cardiac output, and/or those with known orsuspected hyperviscosity or hypercoagulable disorders. Thislast group of patients include women taking oralcontraceptives, especially if they also smoke, and anyonewho has had prolonged periods of immobilization. Patientswith TRALI may be managed using oxygen therapy andappropriate ventilatory support with symptoms usuallyresolving within 96 hours. If TRALI is suspected, appropriatetests should be performed for the presence of anti-neutrophilantibodies in both the product and patient serum.


SUBCUTANEOUS IMMUNOGLOBUL INTHERAPY

In 1952, when Dr. Bruton began to treat his antibody deficientpatient, he actually used subcutaneous immunoglobulin (SCIG).In the United States, the therapy evolved into intramuscularinjections and then intravenous infusions. Since the 1980’s,SCIG has been widely used in Europe. In January 2006, theU.S. Food and Drug Administration (FDA) approved apreparation of SCIG for use in the U.S. Currently the only FDAapproved indication for SCIG is as antibody replacementtherapy. At this time products for subcutaneous infusions areavailable in concentrations of 10 or 20%.

For the majority of patients, IVIG and SCIG are equallyefficacious; however, there are differences between thetherapies. While IVIG is usually given as a single large infusionevery three to four weeks, SCIG involves giving smaller dosesmore often, once or twice a week in most cases. Fractionatingthe total dose into smaller portions decreases the changes inserum IgG levels that are the hallmark of intermittent IV infusions.This fractionation of the dose may eliminate some of thesystemic adverse effects associated with IV infusions. Sinceinfusions are given more often, the low “trough” IgG levels thatoccur just before the next IVIG infusion are also eliminated. WithSCIG, the serum IgG concentration becomes remarkablyconsistent once steady state is achieved and the patient iscompliant with the therapy.

SCIG is self-administered by the patient. For children or thosepatients with some physical limitation, someone else can assistwith the infusion. Dependent on the volume of drug to beinfused, multiple small needles may be used simultaneously.The drug can be administered via a small syringe driver pumpor via a manual push. Many studies have demonstrated thatpatients can tolerate relatively rapid infusions and those patientswho deliver their infusions via a manual push can do so in amatter of minutes.


A great deal of flexibility in the regimen, including the number ofsites, duration of the infusion, and frequency of infusions, ispossible. Because of these multiple permutations, the patientcan design a regimen which “works” for him/her; that is one withwhich he/she can be compliant. For example a dose of 10grams/week if given with a 20% solution would have a volume of50 ml. The typical adult could receive this dose via a onceweekly infusion, using two needles connected to bifurcatedtubing and a pump; the infusion could take approximately 30-40minutes, although it could be made to go faster or slower basedon the patient’s tolerability and wishes. This dose could be splitinto a twice weekly infusion, using a single needle for eachinfusion. Some patients could even choose to give themselves10 cc on five days every week. This flexibility in “customdesigning” a regimen can be very attractive to patients whoseek greater control over their illness and treatment. Decidinghow, where and when the infusion occurs may help to minimizetime lost from work or school, and allow greater freedom forpatients who travel frequently.

Nurse’s RoleThe nursing role in SCIG is primarily that of an educator andfacilitator. The goal for care is to help the patient/caregiver tobecome independent. Patients and/or caregivers will need to betaught the skills necessary to administer their infusions in a safeand aseptic manner. A systematic, step-wise teachingapproach is usually effective. This starts with the nurse firstdemonstrating the procedure, then allowing the patient topractice the skill, and finally observing a return demonstration bythe patient/caregiver to demonstrate mastery. After the patient isindependent, follow up and support are critical in managingissues and/or problems. There may be multiple small “tweaks”necessary to ensure success. These might include changingthe gauge or length of the needle, a recommendation aboutusing a different site, or changing the rate of the infusion. Theimportant thing to remember is that the vast majority of patientscan be successful with this therapy.


The most important factors in assuring the success ofsubcutaneous therapy are teaching and support. The nurseneeds to develop a teaching plan which takes into considerationthe patient’s ability to learn; independence; self-motivation;compliance; ability to read and/or follow instructions; physicallimitations, especially regarding manual dexterity; and presenceof someone to assist or actually perform the infusion, ifnecessary.

Much of the education for SCIG administration includes basicnursing, i.e. hand washing and aseptic technique. A systematicapproach to setting up the equipment and drawing up theproduct, inserting the needle(s), monitoring local effects,discontinuing the infusion, and safely discarding the usedequipment and needles needs to be developed.

Specific teaching topics can include: � 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


Another important teaching topic is ensuring that the patientunderstands adverse reactions and/or complications, as well ashow to initiate the appropriate action should something untowardoccur. Expectations regarding site reactions and managementshould be discussed. The patient must be taught the signs ofanaphylaxis and what to do should they occur. EpiPen trainingshould be provided when an EpiPen has been prescribed.

Documentation of the patient’s mastery of skills is important. Thenumber of sessions required for the patient to master all of thesesteps may vary widely depending on the individual’s capacity forlearning, coupled with their anxiety level.

Adverse ReactionsSystemic reactions such as headache, nausea, fever, chills, andmore serious adverse reactions are less frequent with SCIG thanwith IVIG, and published studies on SCIG consistentlydemonstrate a low rate of systemic reactions. The mostcommon reported adverse events with SCIG are localized sitereactions including itching, a burning sensation, mild rednessand/or swelling. These local reactions are very common when apatient starts SCIG therapy because initially the body does notrecognize the drug and perceives it as an irritant or somethingforeign. The normal inflammatory cascade is activated, so thereis swelling and erythema at the site(s). In almost all cases, thesesymptoms resolve within 12 to 24 hours. The intensity of theselocal reactions decreases with every infusion as the body comesto “recognize” the drug. If redness, irritation and swelling persistafter the patient has been on therapy for more than a month orsix weeks, it may be an indication of a mechanical issue such asa too-short needle causing drug to leak into the dermal layer oran infusion rate higher than the patient can tolerate. It may alsobe an indication that the patient needs to “work up” to thedesired number of sites, volume per site and rate.

As with IVIG in patients with humoral immunodeficiencies, trueanaphylaxis with SCIG is extremely rare. Local site reactionscan be managed with adjustments to the infusion regimen,gentle massage, warm or cold compresses, and/or mild painmedications such as ibuprofen or acetaminophen.


Table 6: Potential Nursing Interventions for Dealingwith Adverse Reactions to SCIG


Local Itching

• Apply cold compress (do not apply coldpack directly to skin).

For future infusions, consider:

• Use of a longer needle.

• Decrease volume per site and working upto desired site volume gradually.

• Ensure that a dry needle insertiontechnique has been used.

• Topical diphenhydramine.

• Over-the-counter topical steroid.

Redness

• Apply cold or warm compress dependingon which the patient feels will help.

• Consider irritation from tape or adhesiveand change this product.

• Assure patient that redness shoulddecrease with each subsequent infusion.

Burning

• Clamp off catheter for 5-10 minutes, ifdesired.

• Slow the rate of infusion.

• Cold compress.

• Distraction techniques for youngerchildren.

• Consider removing the needle andreplacing it in another site.

• Assess needle placement as the needle maybe partially intramuscular instead ofsubcutaneous. Consider the use of ashorter needle.

• Assess antiseptic used for skin prep.


Table 6: Potential Nursing Interventions for Dealingwith Adverse Reactions to SCIG (cont.)


Swelling(There will always besome degree ofswelling as thepatient is infusingfluid under the skin.)

• 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 legsite: take a walk to help mobilize the fluid.

Urticaria/Hives

• Stop infusion.

• Contact prescriber for instructions and todetermine if infusion should continue.

• Antihistamine.

Rash

• Stop SCIG.

• Contact prescriber for instructions and todetermine if infusion should continue.

• Consider the possibility of tape or latexsensitivity.

Discomfort

• Slow infusion.

• If intolerable pain, needle may beintramuscular, remove the needle andchange sites.

• Warm compresses.

• Gentle massage.

• Over-the-counter analgesics can be used,but are rarely necessary.


ADD IT IONAL RESPONS IB IL IT IES

The nurse, the prescriber and the patient form aninterdependent triad. Each person in this triad has individual aswell as overlapping responsibilities. All must work together toachieve the goals for care.

PrescriberThe prescriber’s responsibility is to make a diagnosis andeducate the patient about the diagnosis. Decisions for therapyshould be made collaboratively. The prescriber should explainavailable options for therapy. The therapy regimen, both therationale for the therapy and practicalities involving the therapy,need to be explained clearly. The prescriber should make plansand expectations clear regarding follow up and sick visits,referrals to other providers and laboratory monitoring.

PatientsPatients need to assume responsibility for themselves whilemaintaining close connections with the prescriber and the nurse.They should identify the need for education and/or assistanceand should communicate problems or issues, especiallypotential barriers to care, appropriately, effectively and in atimely manner. Ultimately, it is the patient who establishes theparameters and/or boundaries for the partnerships in thisinterdependent triad with nurse and prescriber.


NurseThe nurse’s first responsibility is to safely and effectively providethe prescribed therapy. However, the nurse has multiple otherresponsibilities:

Compliance MonitoringThe nurse may oversee the establishment of monitoringparameters for infusions and infusion related issues, includingpatient compliance. Compliance monitoring should includeclear instructions for the patient regarding his/her therapy. Thepatient should know the name and dose of the drug that he/sheis receiving. The patient should be taught to record specifics ofeach infusion in a personal diary or infusion log. Information thatshould be recorded includes: � 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 usethe Immune Deficiency Foundation (IDF) eHealthRecord, whichis a free-of-charge, online personal health record developedspecifically for the primary immunodeficiency diseasecommunity: www.idfehealthrecord.org. Some patients keephandwritten infusion logs, which are often available asdownloadable PDF files from manufacturer’s websites. TheeHealthRecord can keep all medical history, currentmedications, infusions logs and more all in one place. Eitherway they choose to log infusions, patients need to understandthe importance of keeping a log to record lot numbers anddosages as recalls of products and/or specific lot numbers dosometimes occur. Patients have a right to know if there is apotential problem and to seek appropriate help if there is aconcern. Patients can enroll in a patient notification system forinformation on product withdrawals and recalls at:www.patientnotificationsystem.org.


DocumentationDocumentation is another key nursing responsibility. As with allblood products, the nurse needs to keep a record of theproduct, lot number(s) and expiration date(s). This data needsto be readily retrievable in the event of a product recall or if areportable patient problem occurs. Other infusion related dataincluding dose, duration of the infusion and assessments of thepatient should also be carefully recorded. This data is importantwhen trending information about the patient’s replacementtherapy and his/her overall health status.

CommunicationThe nurse has a critical role in establishing parameters forcommunication between all members of the triad. Variables tobe determined regarding communication include the mode(telephone, e-mail, written), what needs to be communicated towhom, to whom specific problems should be communicated andcommunication in the event of emergency. The patient needsclear, written directions and needs to demonstrateunderstanding of these directions. A printed instruction list withrelevant contact information is useful as a reference guide in thepatient’s home.


Education and AdvocacyThe nurse plays an important role in patient education andadvocacy and should assess the patient’s knowledge about thedisease state and treatment, and provide necessary education.There is a multitude of resources available to meet educationalneeds.

IDF has patient education materials readily available:www.primaryimmune.org. Through IDF, patients can alsoconnect with other patients. Additionally, IDF has frequentoutreach programs for patients and families. Information aboutsuch things as new modalities of treatment, legislative initiativesand insurance issues can be valuable resources. For completelist of resources available through IDF, see Appendix B.

The nurse should provide ongoing support and education andassist patients in locating resources for such diverse issues asinsurance problems, pediatric patients’ transition to adulthoodand assumption of their own care, attending school/college,concerns regarding traveling and vacations, pregnancy and anyother life cycle changes. This includes providing patients withinformation about their product manufacturer’s patientassistance program. Again, information regarding these issuescan be found on the IDF website: www.primaryimmune.org.

Nurses should advocate for their patients with primaryimmunodeficiency diseases and help them to bring issues to theprescriber about ways to improve the patients’ quality of life notonly during replacement therapy administration but also in otherareas in which the patients’ primary immunodeficiency has animpact on their life. Patients should not be defined by theirdisease; the ultimate goal should be to empower them to takecontrol of their lives.


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Lucas M, Lee M, Lortan J, et al. Infection outcomes in patients with commonvariable immunodeficiency disorders: Relationship to immunoglobulintherapy over 22 years. Journal of Allergy and Clinical Immunology.2010;125:1354-1360.

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APPEND IX A : QU ICK NURSE ’S GU IDE FORTROUBLESHOOT ING SC IG ADMIN ISTRAT ION

Leaking at the site� Assess catheter: is it fixed securely?� Assess placement: may be in a location that is subject to

movement – advise regarding selection of site, assessamount of subcutaneous tissue vs. muscle.

� Assess length of catheter: may be too short – can suggestcatheter brand change.

� Assess volume that is being infused: may be too muchvolume per individual site.

Local irritation, i.e. redness, swelling, itching� Assure that this is normal reaction – should diminish in 24-48

hours, definitely by 4 days.� Assess size: mosquito bite, raised wheel, quarter, plum,

peach, or grapefruit? – size should be consistent with volumebeing infused and amount of subcutaneous tissue onpatients; thinner patients may have more prominent raisedarea and may need to decrease amount of volume per site.

� Assess length of catheter: may be too short, can suggestlonger catheter length or brand change to avoid.

� Advise use of gentle massage or warm compress post-infusion.

� 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 asmall amount of air before needle. It has been suggestedthat the IgG tracked through the intradermal space can causesite reactions such as redness and itching in some patients.

� Apply topical Benadryl cream to site during and after infusion.


Extreme discomfort with needle� Assess length: may be too long and irritating abdominal wall.� Try catheter that allows introducer needle to be removed (Mini-

med Sof-Set).� Try Emla cream or topical anesthetic prior to insertion.

Blood return observed � 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 withprescribing physician regarding selecting alternative foraccommodating fewer sites.

� Infuse the drug with the remaining appropriately located sites,thus increasing volume per site. May need to recalculate to aslower rate of infusion if appropriate. Consider previoushistory of site reaction and other factors.

� Infuse the original amount of volume per site with the sites thatare in place. When completed, repeat the infusion sessionwith new site to accommodate the remaining volume from thesite that had blood return.

� Change entire set up and start over.

Long infusion times� 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 office visit).


Needle contaminated by touching, dropping, etc.� Discard in appropriate waste container and use new one.

Infusion pump stops during the infusion� 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 tosite and manually push plunger forward slowly to deliverremaining volume. Depending on volume, this may takesome time.

Difficulty with manipulating syringes for filling� Lubricate the barrel of syringe for easy manipulation by

aseptically pulling back on the syringe, and moving it up anddown before drawing up solution or filling with air.

� Pull back the amount of air to be infused into the vial and thenattach the needle aseptically to the syringe.

� Mark the level of cc to which the syringe should be drawn backby placing tape on the outside barrel at the necessary level.


Immune Deficiency Foundationwww.primaryimmune.org

[email protected]

Services for Healthcare Professionalswww.primaryimmune.org/healthcare-professionals� IDF Nurse Advisory Committee: Comprised of exceptional

nurses to support the mission of the IDF. Available as aresource for nurses administering immune globulin therapy ortreating patients with primary immunodeficiency diseases.

� IDF Medical Advisory Committee: Comprised of prominentimmunologists to support the mission of the IDF. Available asa resource for clinicians diagnosing and treating patients withprimary immunodeficiency diseases.

� IDF Online Continuing Education Course for Nurses: A free,5-hour, accredited course for nurses that provides an updateon primary immunodeficiencies, immunoglobulin therapiesand the nurse’s role with these therapies:www.primaryimmune.org/healthcare-professionals/continuing-education-course-for-nurses

� IDF Consulting Immunologist Program: A free service forphysicians which provides consults with expert clinicalimmunologists on issues of diagnosis, treatment and diseasemanagement.

� USIDNet: The United States Immunodeficiency Network(USIDNet), an international consortium established toadvance scientific research in the primary immunodeficiencydiseases through peer reviewed research grants, educationand mentoring programs, DNA and cell repository, andpatient registries. Administered by IDF.

� IDF & USIDNet LeBien Visiting Professor Program: Promoteimproved knowledge by providing your faculty with a VisitingProfessor with expertise in primary immunodeficiencydisease. Offers Grand Rounds and clinical presentations atmedical institutions throughout North America.

APPEND IX B : IMMUNE DEF IC IENCYFOUNDAT ION RESOURCES


Services for Patients and Familieswww.primaryimmune.org/services� Ask IDF: Contact IDF with questions about living with primary

immunodeficiency diseases through the IDF web site:www.primaryimmune.org/services/ask-idf. IDF has a vastreserve of innovative resources and individualized assistanceto help with the unique aspects of living with a primaryimmunodeficiency. From learning more about the diseases, tounderstanding insurance coverage, to lifestyle issues andmore, be sure to Ask IDF.

� Locate a Physician: Contact IDF to find a physician in your areawho is an expert on PI.

� Peer Support: Connecting people and patients who sharesimilar relationships to PI.

� Patient Assistance Resources: Individualized assistance isavailable for patients experiencing problems with insurancedenials for treatment, reimbursement issues, concerns withMedicare or Medicaid, disability, and accessing copaymentand premium assistance. Resources and tools are available tohelp tackle insurance challenges.

� Information about Patient Rights: Patients can contact IDF tolearn about their rights concerning product choice andtreatment options, employment and school issues, as well asfair treatment, privacy or other rights.

� IDF eHealthRecord: An electronic personal health recorddesigned for the primary immunodeficiency community to helporganize health information in one place.www.idfehealthrecord.org


Programs for Patients and Families� Local Patient Meetings: Education programs featuring local

experts and networking opportunities.� Operation Outreach: Patient education meetings designed to

strengthen underserved areas.� IDF Retreats: Weekend events for all ages that feature medical

and life management sessions.� IDF Youth Programs: Designed for children diagnosed with a

PI or have a family member with this condition.� IDF Teen Escape: Weekend program developed to acquaint

teens diagnosed with PI.� IDF National Conference: The world's largest gathering of

families affected by PI.� Volunteer: Network of volunteers who provide peer support,

create awareness, help host educational meetings, advocatefor public policy, visit plasma centers and organize fundraisingevents throughout the country.

� Scholarship Program: Awards for students living with primaryimmunodeficiency diseases who plan on completing theirsecondary education.

� Take the Zebra Challenge!: Fundraising campaign thatprovides the IDF community with multiple resources to createpersonal fundraisers and teach the world about “zebras.”

� IDF Plasma Centers Partners Program: Awareness andfundraising initiatives within plasma centers across the countryarranged by IDF that highlights the work of plasma center staffmembers, plasma donors and IDF volunteers.


PublicationsAll publications can be downloaded and printed atwww.primaryimmune.org. Alternatively, you can order a hardcopy (if it is available).

For patients and families:� Patient & Family Handbook for Primary Immunodeficiency

Diseases 5th Edition� Our Immune System (Children’s Book)� IDF School Guide Information about Students with Primary

Immunodeficiency Diseases� Bill of Rights for Patients with Primary Immunodeficiency

Disease

For healthcare providers:� IDF Diagnostic & Clinical Care Guidelines for Primary

Immunodeficiency Diseases 2nd Edition� IDF Guide for Nurses on Immunoglobulin Therapy for Primary

Immunodeficiency Diseases 3rd Edition� Clinical Focus on Primary Immunodeficiencies:

• “Clinical Update in Immunoglobulin Therapy for PrimaryImmunodeficiency Diseases”

• “Subcutaneous IgG Therapy in Immune DeficiencyDiseases”

• “Primary Humoral Immunodeficiency Optimizing IgGReplacement Therapy”

• “The Clinical Presentation of Primary ImmunodeficiencyDiseases”

• “Treatment and Prevention of Viral Infections in Patientswith Primary Immunodeficiency Diseases”

• “IgG Subclass Deficiency”• “Immunization Of The Immunocompromised Host”


Communications� IDF Advocate: Newsletter, published three times per year.� Primary Immune Tribune: E-newsletter, published monthly.� IDF Friends, www.idffriends.org: A social network

exclusively for the primary immunodeficiency community.� IDF Common Ground, www.idfcommonground.org: An

online community for teens with primary immunodeficiencydiseases.

� IDF Blog, www.primaryimmune.org/blog: Includesupdates on important issues that directly impact patientswith primary immunodeficiency. Celebrates all the waysthat individuals make a difference in the IDF community.Promotes IDF programs, services and events. Allowsusers to comment, submit news and share posts.

� IDF SCID Newborn Screening,www.idfscidnewbornscreening.org: Documents the fight toestablish Severe Combined Immunodeficiency (SCID)newborn screening programs in all 50 states. Babies withSCID appear healthy at birth, but without early treatment,most often by bone marrow transplant from a healthydonor, these infants cannot survive. Testing for SCID isnot currently included in the newborn screening panelsof all states.


Public Policy Initiatives� Advocacy efforts monitor public policy issues that are critical

to patients at national and state levels, including MedicarePatient IVIG Access Act, SCID Newborn Screening, HealthInsurance lg Guidelines and more.

� Grassroots advocacy program mobilizes members of the PIcommunity to contact their government representatives topromote healthcare legislation that will positively affect thecommunity.

� IDF Advocacy Center features Action Alerts, enabling users toeasily voice their concerns to decision makers, and the IDFAdvocacy Channel, featuring patient and caregiver stories:www.primaryimmune.org/idf-advocacy-center.

Additional Resources

Patient Notification System (PNS)www.patientnotificationsystem.orgPNS is a free, confidential, 24-hour communication systemproviding information on plasma-derived and recombinantproduct withdrawal and recalls. Led by the Plasma ProteinTherapeutics Association (PPTA), PNS was developed by theproducers and distributors of plasma products with direct inputfrom consumers.


Product InformationInformation regarding the immunoglobulin products currentlylicensed in the United States is available from each specificmanufacturer via the individual corporate websites. Themanufacturers of Ig often provide up-to-date information andadded financial resources for patients and families dealing withprimary immunodeficiency diseases on their websites. Theresources vary over time and between manufacturers. At presstime, the following is a list of current manufacturers with productname(s) and contact information:

Baxter Healthcare CorporationGammagard S/D, Gammagard Liquidwww.baxter.com800.422.9837 (800.4Baxter)

Bio Products LaboratoryGammaplexwww.bpl.co.uk

Biotest Pharmaceuticals CorporationBivigamwww.biotestpharma.com800.458.4244

CSL BehringCarimune NF, Hizentra, Privigenwww.cslbehring-us.com800.504.5434

GrifolsFlebogamma DIF, Gamunex - Cwww.grifolsusa.com888.474.3657 (888.GRIFOLS)

KedrionGammakedwww.kedrionusa.com855.353.7466

OctapharmaOctagamwww.octapharma.us

Immune Deficiency Foundation40 West Chesapeake Avenue, Suite 308

Towson, MD 21204800.296.4433

www.primaryimmune.org

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