213The purpose of this chapter is to provide an overview of some less common, but often home-administered infusion therapies that do not fall under the broad headings of the previous chapters. These infusion drug therapies are associated with uncommon or rare diseases and the infusion drugs may be associated with significant adverse effects. In almost every case, these drugs are not administered as first doses in the home setting. Patients who do not experience significant adverse reactions or side effects may be transitioned to home infusion. Some of the drugs require reconstitution in the home and it is important to read the specific drug product inserts prior to preparing the infusion. The home care nurse should have an excellent understanding of the disease processes and the specific infusion therapies prior to managing these unusual patient populations. Additional resources, as available, are included in each section. It is important to note that this chapter is not inclusive of all possible home-administered therapies. As the practice of home infusion therapy grows, as the safety profile for home-administered medications is established, and as new medications are approved by the Food and Drug Administration (FDA), this list will grow. It is not uncommon for home care organizations to receive referrals for infusion medications that are not usual. Before accepting a referral for an infusion therapy, it is important to investigate the safety of the infusate, the risks especially as they relate to a home infusion, and the rationale for home administration. This requires interprofessional collaboration between the physician, the pharmacist, and the nursing organization. Patient safety is paramount in such decision making.
After reading this chapter, the reader will be able to:
■ Identify indications for other infusion drugs that may be home administered
■ Discuss key aspects of assessment and monitoring of these infusion drugs
214ALPHA1-PROTEINASE INHIBITOR (ARALAST NP, PROLASTIN-C, GLASSIA, ZEMAIRA)
Description
■ Purified human alpha1-proteinase inhibitor, which is also known as alpha1-antitrypsin (AAT). Product is prepared from large pools of human plasma; manufacturing processes are used to reduce risk of potential viral transmission.
Indications
■ Long-term chronic replacement therapy for the treatment of AAT deficiency, a hereditary condition that results in chronic obstructive pulmonary disease.
■ AAT protects lungs from a natural enzyme (neutrophil elastase) that fights bacteria and cleans up dead lung tissue; this enzyme can also damage the lungs if not neutralized by AAT.
■ Symptoms are primarily shortness of breath with activity, decreased exercise tolerance, and wheezing beginning between 20 and 40 years of age.
■ AAT deficiency is diagnosed with a blood test.
■ The goal of treatment is to slow or stop the progression of lung destruction by replacing the deficient protein. The therapy cannot restore lost lung function and it is not considered a cure. There is some evidence that augmentation therapy can reduce the frequency and severity of pulmonary exacerbations.
(Alpha 1 Foundation: www.alpha1.org).
Infusion Guidelines
■ Prolastin-C: Manufacturer recommends immunization against hepatitis B prior to initiating treatment.
■ 215It is administered as an IV infusion over 30 to 60 minutes, most often as a gravity infusion.
■ It is usually administered with a peripheral venous catheter; started with each dose.
Establish venous access prior to reconstitution (due to high drug cost, cannot risk inability to infuse due to IV placement issues).
■ Alpha1-proteinase inhibitor is a lyophilized powder or ready-to-use solution.
The powder is reconstituted in the patient’s home just prior to administration; several vials may be used for one dose; reconstituted alpha1-proteinase inhibitor is pooled into an empty IV container for infusion. Follow package insert instructions.
Key points related to reconstituting: Use strict aseptic technique, allow adequate time for product to dissolve, may gently roll vial but never shake.
(Gahart, Nazareno, & Ortega, 2016)
Monitoring/Adverse Reactions
■ It is generally well tolerated.
■ Adverse symptoms are infrequent but may include pharyngitis, headache, cough, delayed fever, lightheadedness, and dizziness.
■ Alpha1-proteinase inhibitor is contraindicated in persons with IgA deficiencies who have developed antibodies against IgA; anaphylactic reactions could occur.
PATIENT EDUCATION: KEY POINTS
■ Indications and rationale for treatment
■ Signs/symptoms of adverse reactions to report
■ Lifelong treatment
■ Importance of therapy adherence and keeping physician (MD)/laboratory appointments for regular follow-up
■ Patient (and clinician) resource: Alpha 1 Foundation: www.alpha1.org
216DEFEROXAMINE MESYLATE (DESFERAL)
Description
■ An iron chelating agent; binds with iron to form ferrioxamine, a chelate that prevents iron from entering into further chemical reactions
■ Removes iron from free serum iron, ferritin, and transferrin
■ Also used in the chelation of aluminum in patients with chronic renal failure
Indications
■ In the home care setting, deferoxamine is used for the treatment of chronic iron overload often associated with patients who have a history of repeated blood transfusions (e.g., with sickle cell anemia, aplastic anemia, chronic hemolytic anemia) or secondary hemochromotosis, an iron overload disorder.
■ Without treatment, excessive iron accumulation results in joint problems, decreased liver function, cardiac damage, and endocrine disorders.
Infusion Guidelines
■ Routes include subcutaneous and intravenous infusions
■ Most often given as a subcutaneous infusion of 1 to 2 g per day (adult dose) over 8 to 24 hours; dosage rate should not exceed 15 mg/kg/hr; weight-based dosage adjustments in children (dosage should not exceed 40 mg/kg/d)
■ Requires an infusion pump
■ Intermittent infusions often administered during nighttime hours for patient convenience (Eckes, 2011)
■ Resource: www.drugs.com/pro/deferoxamine.html#fcf7eafe-cf86-4e12-b6a8-83cd2f0d103b
Monitoring/Adverse Reactions
■ Hearing and visual problems can occur.
■ Hearing and vision are regularly tested during chronic therapy.
■ Side effects associated with too rapid infusion include abdominal discomfort, allergic type reactions, diarrhea, fever, hypotension, and leg cramps.
■ 217Laboratory monitoring:
Serum iron concentration
Serum ferritin levels
PATIENT EDUCATION: KEY POINTS
■ Indications and rationale for treatment
■ Signs/symptoms of adverse reactions to report
■ Turns urine color reddish color
■ Importance of therapy adherence and keeping MD/laboratory appointments for regular follow-up (Eckes, 2011)
■ Infusion-related teaching: Subcutaneous site access, infusion pump and related management—patients usually become completely independent with infusions
FACTOR REPLACEMENT FOR HEMOPHLIA: RECOMBINANT PRODUCTS
Description
■ Most often factor replacement products used are derived from recombinant DNA rather than processed from donated plasma. This allows for home administration. Most patients will learn to self-administer.
Indications
■ Hemophilia A is a deficiency of coagulation factor VIII that occurs in about 1:10,000 males (Hitch, 2013). Factor VIII replacement either is used routinely for patients with severe hemophilia or may be used situationally in moderate to mild cases, such as patients undergoing prophylactic therapy with planned surgery or with a traumatic event.
■ Hemophilia B is a deficiency of coagulation factor IX that occurs in about 1:50,000 males (Hitch, 2013).
Infusion Guidelines
■ Factor replacement doses are in units and each lot contains a different number of units per vial. The cost of factor replacement is high and dosages are usually prescribed as a range as it is 218unlikely to find a vial with an exact number of units to match the prescribed weight-based dosage (Hitch, 2013).
■ It is administered by slow IV push infusion, no faster than 10 mL/min (Gahart et al., 2016). Always refer to specific manufacturer’s directions.
■ Dosing varies based on treatment versus prophylaxis, type of bleeding episodes (minor, moderate, or major), or type of surgery (minor or major).
■ It is packaged in kits that include a vial of factor (powder), a diluent, and a mixing device; most come with a built-in filter. Sometimes, a steel needle IV device is included in the kit.
■ It is generally supplied by the blood center and stored in refrigerator or at room temperature (as directed by the manufacturer); shelf-life up to 2 years.
Monitoring/Adverse Reactions
■ Most common side effects include headache, dizziness, nausea, rash, pain in extremity, and oral paresthesia depending upon the specific product.
■ Factor VIII: The most frequently reported adverse reactions in clinical trials were headache, pyrexia, and pruritus (www.kovaltry-us.com/hcp).
■ Factor IX: Most common side effects include headache, dizziness, rash, hives, injection site reactions/pain, and nausea (www.drugs.com/monograph/factor-ix-recombinant.html).
■ Patients can develop antibodies to replacement factor, making it more difficult to control bleeding. This may require higher doses or a bypassing agent that allows clotting to occur without factor VIII or IX (Hitch, 2013).
PATIENT EDUCATION: KEY POINTS
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