Primary immunodeficiency treatment

Treatment for Primary Immunodeficiency

Depending on your particular type of PI and your health situation, there are several potential treatment options for primary immunodeficiency (PI).

It's important to keep in mind there is not a right or wrong treatment option—there is the treatment option that is best for you. Only you and your healthcare team can determine which treatment option is best for you.

Types of PI Treatment

There are several options for PI treatment. Depending on your particular type of PI and your treatment needs, your doctor might recommend certain treatments as the best options for you.

Shire Ig Treatments for PI

If you and your doctor have decided that you should be using immunoglobulin (Ig) treatment, Shire offers a few different ways you can receive your Ig treatment, as shown in the following chart:

Indicated for adult patients with primary immunodeficiency
Subcutaneous (SubQ) 1
Infused under the skin in the fatty tissues of the abdomen or thighs
Infusion includes the administration of Recombinant Human Hyaluronidase prior to the administration of immune globulin
Administered once a month (every 3 to 4 weeks)*
Dose given in 1 site
A second site can be used at the discretion of the physician and patient based on tolerability and total volume
Can be self-administered after patient is trained by a healthcare professional or administered by a healthcare professional
Infused at home after being trained, or at a hospital or clinic
Gammagard liquid logo
Indicated for patients two years of age and older with primary immunodeficiency
Intravenous (IV)2
Infused directly into the bloodstream through a vein
Administered once every 3 to 4 weeks
Dose given in 1 site
Administration requires a healthcare professional
Infused at hospital, clinic, or home with nurse present
Gammagard liquid logo
Indicated for patients two years of age and older with primary immunodeficiency
Conventional SubQ2
Infused under the skin in the fatty tissues of the abdomen, thighs, upper arms, or lower back
Administered once a week
Dose given in multiple sites
The number of simultaneous sites should be limited to 8
Can be self-administered after patient is trained by a healthcare professional
Infused at home after being trained
Indicated for patients 2 years of age and older with primary immunodeficiency
Conventional SubQ20
Infused under the skin in the fatty tissues of the abdomen, thighs, upper arms, or lower back
Administered at regular intervals from daily up to every 2 weeks
Dose given in up to 4 sites simultaneously based on the discretion of the physician and patient based on tolerability and total volume
Can be self-administered after patient is trained by a healthcare professional
Infused at home after being trained, or other appropriate setting

*Based on clinical response
Can also be administered by a caregiver

GAMMAGARD S/D [Immune Globulin Intravenous (Human)] IgA < 1 μg/mL in a 5% solution]

For patients with PI 2 years and older who need an Ig treatment with low levels of the type of Ig called IgA, this intravenous Ig treatment may be an option.13

Please see the Indication and Important Safety Information below.

Please see FDA-approved patient labeling for HYQVIA, FDA-approved patient labeling for GAMMAGARD LIQUID, and FDA-approved patient labeling for CUVITRU. Please see Full Prescribing Information for GAMMAGARD S/D.



Please expand for Indication and Important Safety Information.

Selected Important Safety Information about HYQVIA
  • HYQVIA can cause blood clots. Call your healthcare professional if you have pain, swelling, warmth, redness, or a lump in your legs or arms, other than at the infusion site(s), unexplained shortness of breath, chest pain or discomfort that worsens on deep breathing, unexplained rapid pulse, numbness or weakness on one side of the body.
  • Do not take HYQVIA if you are allergic to IgG, hyaluronidase, other blood products, human albumin (in the hyaluronidase solution), or have IgA deficiency with antibodies to IgA.
Indication and Important Safety Information
HYQVIA [Immune Globulin Infusion 10% (Human) with Recombinant Human Hyaluronidase] Solution
  • HYQVIA is a liquid medicine containing immune globulin and Recombinant Human Hyaluronidase. HYQVIA contains IgG antibodies, collected from human plasma donated by healthy people. The antibodies help your body to fight off bacterial and viral infections. The hyaluronidase part of HYQVIA helps more of the immune globulin get absorbed into the body to fight infection.
  • HYQVIA is indicated for the treatment of Primary Immunodeficiency (PI) involving the humoral immune system in adults.
  • Safety and efficacy of chronic use of Recombinant Human Hyaluronidase in HYQVIA have not been established in conditions other than PI.
  • HYQVIA is infused under the skin (subcutaneous).
  • If you and your healthcare professional decide that home self-infusion of HYQVIA is right for you, then be sure you get instructions and training from your healthcare professional before using HYQVIA at home.
Important Safety Information
    • HYQVIA can cause blood clots.
    • Call your healthcare professional if you have pain, swelling, warmth, redness, or a lump in your legs or arms, other than at the infusion site(s), unexplained shortness of breath, chest pain or discomfort that worsens on deep breathing, unexplained rapid pulse, numbness or weakness on one side of the body.
    • Your healthcare professional may perform blood tests regularly to check your IgG level.
    • With your consent, your healthcare professional may provide blood samples to Shire plc, to test for antibodies that may form against the hyaluronidase part of HYQVIA.
    • Do not infuse HYQVIA into or around an infected or red swollen area because it can cause infection to spread.
    • Talk to your healthcare professional if you become pregnant. Women who become pregnant during HYQVIA treatment are encouraged to enroll in the HYQVIA Pregnancy Registry by calling Medical Information at 1-866-424-6724.

    Do not take HYQVIA if you:

    • Are allergic to IgG, hyaluronidase, other blood products, or human albumin (in the hyaluronidase solution).
    • Have IgA deficiency with antibodies to IgA.

    HYQVIA can cause serious side effects. Call your healthcare professional or go to your emergency department right away if you get:

    • Hives, swelling in the mouth or throat, itching, trouble breathing, wheezing, fainting or dizziness. These could be signs of a serious allergic reaction.
    • Bad headache with nausea, vomiting, stiff neck, fever, and sensitivity to light. These could be signs of swelling in your brain.
    • Reduced urination, sudden weight gain, or swelling in your legs. These could be signs of a kidney problem.
    • Pain, swelling, warmth, redness, or a lump in your legs or arms, other than at the infusion site(s). These could be signs of a blood clot.
    • Brown or red urine, fast heart rate, yellow skin or eyes. These could be signs of a liver or blood problem.
    • Chest pain or trouble breathing, blue lips or extremities. These could be signs of a lung problem.

    What are the possible or reasonably likely side effects of HYQVIA?

    • After HYQVIA infusion a temporary, soft swelling may occur around the infusion site, which may last 1 to 3 days, due to the volume of fluid infused. Mild or moderate pain, redness, swelling, or itching may occur at the site of infusion and generally go away in a few hours. Local reactions are less likely after the first few infusions.
    • The most common side effects of HYQVIA are headache, fatigue, nausea, fever, and vomiting.
    • Antibodies to the hyaluronidase component of HYQVIA were formed in some patients taking HYQVIA. It is not known if there is any long term effect. In theory, these antibodies could react with your body’s own PH20. PH20 is present in the male reproductive tract. So far, these antibodies have not been associated with increased or new side effects.

    These are not all the possible side effects with HYQVIA. Talk to your healthcare professional about any side effects that bother you or that don’t go away.

    Before starting HYQVIA, tell your healthcare professional if you:

    • Have or had any kidney, liver, or heart problems or history of blood clots because HYQVIA can make these problems worse.
    • Have IgA deficiency or a history of severe allergic reactions to IgG or other blood products.
    • Are pregnant, trying to become pregnant or are breast feeding.

    You are encouraged to report suspected side effects by contacting FDA at 1-800-FDA-1088 or www.fda.gov/medwatch or Shire Drug Safety at 1-800-999-1785.

    The risk information provided here is not comprehensive. To learn more, talk about HYQVIA with your healthcare provider or pharmacist.

    Please see the FDA-approved patient labeling

    GAMMAGARD LIQUID [Immune Globulin Infusion (Human)] 10% Solution
    • GAMMAGARD LIQUID is indicated for the treatment of primary humoral immunodeficiency diseases (PI) in patients two years of age and older. GAMMAGARD LIQUID is a ready-to-use, liquid medicine that contains immunoglobulin G (IgG) antibodies, which protect the body against infection.
    • GAMMAGARD LIQUID is made from human plasma that is donated by healthy people and contains antibodies that replace the missing antibodies in patients with PI.
    • GAMMAGARD LIQUID is given into a vein (intravenously) or under the skin (subcutaneously). You and your healthcare provider will decide which way is best for you. Most of the time infusions under the skin are given at home by patients or caregivers. Although it is possible to give yourself infusions into a vein at home, they are more often given in a hospital or infusion center by a nurse. Only use GAMMAGARD LIQUID by yourself after you have been instructed by your healthcare provider.
    Important Risk Information

      GAMMAGARD LIQUID can cause the following serious reactions:

      • Severe allergic reactions causing difficulty in breathing or skin rashes
      • Decreased kidney function or kidney failure
      • Blood clots in the heart, brain, lungs or elsewhere in the body
      • Severe headache, drowsiness, fever, painful eye movements, or nausea and vomiting
      • Dark colored urine, swelling, fatigue, or difficulty breathing

      Do not use GAMMAGARD LIQUID if you:

      • Have a known history of a severe allergic reaction to IgG or other blood products.
      • Have selective (or severe) immunoglobulin A (IgA) deficiency with antibodies to IgA.

      GAMMAGARD LIQUID can cause serious side effects. Call your healthcare professional or go to the emergency department right away if you get:

      • Hives, swelling in the mouth or throat, itching, trouble breathing, wheezing, fainting or dizziness. These could be signs of a serious allergic reaction.
      • Bad headache with nausea, vomiting, stiff neck, fever, and sensitivity to light. These could be signs of swelling in your brain.
      • Reduced urination, sudden weight gain, or swelling in your legs. These could be signs of a kidney problem.
      • Pain, swelling, warmth, redness, or a lump in your legs or arms. These could be signs of a blood clot.
      • Brown or red urine, fast heart rate, yellow skin or eyes. These could be signs of a liver or blood problem.
      • Chest pain or trouble breathing, or blue lips or extremities. These could be signs of a serious heart or lung problem.
      • Fever over 100°F. This could be a sign of an infection.

      Whenever giving yourself treatments at home, you should have another responsible person present to help treat side effects or get help if you have a serious adverse reaction occur. Ask your healthcare provider whether you should have rescue medications available, such as antihistamines or epinephrine.

      What are the possible or reasonably likely side effects of GAMMAGARD LIQUID for PI?

      The following one or more possible reactions may occur at the site of infusion. These generally go away within a few hours, and are less likely after the first few infusions: Mild or moderate pain, swelling, itching, redness, bruising, and warmth.

      During the infusion of GAMMAGARD LIQUID, look out for the first signs of the following common side effects: Headache, migraine, fever, fatigue, itching, rash/hives, cough, chest pain/tightness, chills/shaking chills, dizziness, nausea/vomiting, faster heart rate, upper abdominal pain, increased blood pressure, muscle cramps, and sore throat.

      These are not all the possible side effects. Talk to your healthcare professional about any side effects that bother you or that don’t go away.

      You are encouraged to report suspected side effects by contacting FDA at 1-800-FDA-1088 or www.fda.gov/medwatch or Shire Drug Safety at 1-800-999-1785.

      The risk information provided here is not comprehensive. To learn more, talk about GAMMAGARD LIQUID with your healthcare provider or pharmacist.

      Please see the FDA-approved patient labeling

      GAMMAGARD S/D [Immune Globulin Intravenous (Human)] IgA < 1 µg/mL in a 5% solution
      INDICATIONS

      GAMMAGARD S/D is indicated for the treatment of primary immunodeficiency (PI) associated with defects in humoral immunity, in adults and pediatric patients two years of age or older. This includes, but is not limited to, congenital agammaglobulinemia, common variable immunodeficiency, Wiskott-Aldrich syndrome, and severe combined immunodeficiencies. GAMMAGARD S/D is also indicated for prevention of bacterial infections in patients with hypogammaglobulinemia and/or recurrent bacterial infections associated with B-cell CLL, for the treatment of adult patients with chronic ITP to increase platelet count and to prevent and/or to control bleeding, and for the prevention of coronary artery aneurysms associated with Kawasaki syndrome in pediatric patients.

      DETAILED IMPORTANT RISK INFORMATION

      WARNING: THROMBOSIS, RENAL DYSFUNCTION and ACUTE RENAL FAILURE

      • Thrombosis may occur with immune globulin products, including GAMMAGARD S/D. Risk factors may include advanced age, prolonged immobilization, hypercoagulable conditions, history of venous or arterial thrombosis, use of estrogens, indwelling vascular catheters, hyperviscosity, and cardiovascular risk factors. Thrombosis may occur in the absence of known risk factors.
      • Renal dysfunction, acute renal failure, osmotic nephrosis, and death may occur in predisposed patients with immune globulin intravenous (IGIV) products, including GAMMAGARD S/D. Renal dysfunction and acute failure occur more commonly with IGIV products containing sucrose. GAMMAGARD S/D does not contain sucrose.
      • For patients at risk of thrombosis, administer GAMMAGARD S/D at the minimum dose and infusion rate practicable. Ensure adequate hydration in patients before administration. Monitor for signs and symptoms of thrombosis and assess blood viscosity in patients at risk of hyperviscosity.

      CONTRAINDICATIONS

      • GAMMAGARD S/D is contraindicated in patients who have a history of anaphylactic or severe systemic hypersensitivity reactions to the administration of GAMMAGARD S/D with < 1 µg/mL IgA in a 5% solution.

      WARNINGS and PRECAUTIONS

      • HYPERSENSITIVITY: IgA deficient patients with antibodies to IgA are at greater risk of developing potentially severe hypersensitivity and anaphylactic reactions. Severe hypersensitivity reactions and anaphylactic reactions with a fall in blood pressure have occurred in patients receiving GAMMAGARD S/D, including patients who tolerated previous treatments with GAMMAGARD S/D, even though it contains low levels of IgA. If hypersensitivity reaction develops, discontinue GAMMAGARD S/D infusion immediately and institute appropriate treatment. The risk of anaphylaxis may exist despite the fact that GAMMAGARD S/D < 1 µg/mL, contains < 1 µg/mL IgA.
      • RENAL DYSFUNCTION/FAILURE: Monitor renal function, including blood urea nitrogen, serum creatinine, and urine output in patients at risk of acute renal failure. Ensure that patients with pre-existing renal insufficiency are not volume depleted. For patients at risk for renal dysfunction or thrombotic events, administer GAMMAGARD S/D at the minimum infusion rate practicable.
      • THROMBOSIS: Thrombosis may occur following treatment with immune globulin products, including GAMMAGARD S/D. Monitor for signs and symptoms of thrombosis and assess blood viscosity in patients at risk for hyperviscosity.
      • Aseptic Meningitis Syndrome (AMS) may occur with IGIV treatment and has been reported with GAMMAGARD S/D. AMS may occur more frequently with high dose (2 g/kg) IGIV treatment.
      • Hemolytic anemia can develop subsequent to GAMMAGARD S/D treatment due to enhanced RBC sequestration. Monitor patients for clinical signs and symptoms of hemolysis and delayed hemolytic anemia.
      • Transfusion-Related Acute Lung Injury (TRALI): Non-cardiogenic pulmonary edema has been reported in patients following treatment with IGIV products, including GAMMAGARD S/D. Monitor patients for pulmonary adverse reactions.
      • GAMMAGARD S/D is made from human blood and may carry a risk of transmitting infectious agents, e.g., viruses, the variant Creutzfeldt-Jakob disease (vCJD) agent and theoretically, the Creutzfeldt-Jakob disease (CJD) agent.
      • Hyperproteinemia, increased serum viscosity, and alterations in serum sodium levels: Hyperproteinemia and increased serum viscosity may occur in patients receiving GAMMAGARD S/D. Alterations in serum sodium levels, such as hypernatremia acutely, or pseudohyponatremia after equilibration of the sodium, may occur with the administration of GAMMAGARD S/D. The amount of sodium in the product may add materially to the recommended daily allowance of dietary sodium for patients on a low sodium diet. In these patients, calculate the amount of sodium from the product and use it when determining dietary sodium intake.
      • Passive transfer of antibodies may transiently impair the immune responses to live attenuated virus vaccines such as mumps, rubella, varicella, and measles. This passive transfer may also yield false positive serological testing results, with the potential for misleading interpretation.

      ADVERSE REACTIONS

      There were no serious adverse reactions attributed to GAMMAGARD S/D in the clinical trials. The most common adverse reactions observed in ≥5% of clinical trial patients during or within 48 hours of infusion were headache, nausea, chills, fatigue, pyrexia, upper abdominal pain, diarrhea, back pain, infusion site pain, hyperhidrosis and flushing.

      Serious adverse reactions reported postmarketing were anaphylaxis, acute renal failure, myocardial infarction, cerebrovascular accident, transient ischemic attack, deep vein thrombosis, pulmonary embolism, aseptic meningitis, acute hemolysis and TRALI.

      Please see the Full Prescribing Information, including Boxed Warning.

      You may report side effects to FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.

      You may report side effects to Shire Drug Safety at 1-800-999-1785 or drugsafety@baxalta.com.

      If you have a medical question regarding the use of GAMMAGARD S/D, please ask your healthcare provider.

      CUVITRU [Immune Globulin Subcutaneous (Human)], 20% Solution
      • CUVITRU is a ready-to-use, liquid medicine that contains immunoglobulin G (IgG) antibodies, which protect the body against infection.
      • CUVITRU is indicated for the treatment of primary humoral immunodeficiency (PI) in adult and pediatric patients two years of age and older.
      • CUVITRU is made from human plasma that is donated by healthy people. CUVITRU contains antibodies collected from these healthy people that replace the missing antibodies in PI patients.
      • CUVITRU is given under the skin (subcutaneously).
      • Most of the time infusions under the skin are given at home by self infusion or by caregivers. Only use CUVITRU by yourself after you have been instructed by your healthcare provider.
      Important Risk Information

        CUVITRU can cause the following serious reactions:

        • Severe allergic reactions causing difficulty in breathing or skin rashes
        • Decreased kidney function or kidney failure
        • Blood clots in the heart, brain, lungs, or elsewhere in the body
        • Severe headache, drowsiness, fever, painful eye movements, or nausea and vomiting
        • Dark colored urine, swelling, fatigue, or difficulty breathing

        Do not use CUVITRU if you:

        • Are allergic to immune globulin or other blood products.
        • Have selective (or severe) immunoglobulin A (IgA) deficiency with antibodies to IgA.

        CUVITRU can cause serious side effects. Call your healthcare professional or go to the emergency department right away if you get:

        • Hives, swelling in the mouth or throat, itching, trouble breathing, wheezing, fainting or dizziness. These could be signs of a serious allergic reaction.
        • Bad headache with nausea, vomiting, stiff neck, fever, and sensitivity to light. These could be signs of irritation of the lining around your brain.
        • Reduced urination, sudden weight gain, or swelling in your legs. These could be signs of a kidney problem.
        • Pain, swelling, warmth, redness, or a lump in your legs or arms. These could be signs of a blood clot.
        • Brown or red urine, fast heart rate, yellow skin or eyes. These could be signs of a liver or blood problem.
        • Chest pain or trouble breathing, or blue lips or extremities. These could be signs of a serious heart or lung problem.
        • Fever over 100°F. This could be sign of an infection.

        What are the possible or reasonably likely side effects of CUVITRU?

        The following one or more possible side effects may occur at the site of infusion: mild or moderate pain, redness, and itching. These generally go away within a few hours, and are less likely after the first few infusions.

        The most common side effects that may occur are: headache, nausea, fatigue, diarrhea, and vomiting.

        These are not all the possible side effects. Talk to your healthcare professional about any side effects that bother you or that don’t go away.

        You are encouraged to report suspected side effects by contacting FDA at 1-800-FDA-1088 or www.fda.gov/medwatch or Shire Drug Safety at 1-800-999-1785.

        The risk information provided here is not comprehensive. To learn more, talk about CUVITRU with your healthcare provider or pharmacist.

        Please see the FDA-approved patient labeling