Striking the Right Balance: Shared Decision-Making in Primary Immunodeficiency Care


Sponsored by CSL Behring

Niraj C. Patel, MD

Niraj C. Patel, MD

Flexibility and convenience are staples in most aspects of our lives due to new technology. Healthcare is no exception with the availability of instant access to medical records, online appointments and efforts to decrease wait times through better scheduling technology. With these advances, people expect greater flexibility and an experience centered around patient convenience.

But what about the clinical side? Niraj C. Patel, MD, Associate Professor, Pediatric Allergy and Immunology, Emory University, Children’s Healthcare of Atlanta, Atlanta, GA, who specializes in treating patients with Primary Immunodeficiency (PI), a genetic condition that impairs the immune system, participated in a Q&A with CSL Behring to discuss a recent survey centered around treatment preferences for people living with PI, and how he considers both convenience and flexibility in his practice. He emphasizes the importance of physicians and patients working together to determine the right treatment option.

How are considerations around patients’ lifestyles becoming more integrated into treatment decisions?

Today, more and more patients prefer and prioritize flexibility and convenience when it comes to their medical treatments.

People living with a rare disease like PI, or any chronic disease, require lifelong medical care and often lifelong treatments. For these persons especially, creating a patient experience that equally prioritizes flexibility, convenience and outcomes is critical.

How do you ensure you treat each patient with an individualized approach?

The physician/patient relationship is all about finding a balance between the science and the unique human needs of the patient. Both sides need to be heard and work in harmony to achieve optimal outcomes.

It starts with taking the time.

A physician’s day can be unpredictable as well as emotionally and physically taxing. Physicians often feel they don’t have enough time for thorough conversations with each patient, and appointments can feel rushed at times.

The patients can see and feel that.

Before I enter an appointment with a patient, I take a few seconds to clear my mind and focus all my attention on them. I try to create an environment where my patients feel comfortable sharing what’s working well and what can be improved with their treatment plan.

What are the key factors you consider when developing a treatment plan for your PI patients?

For people living with life-long conditions like PI, finding a treatment that offers both efficacy and convenience is paramount in ensuring a favorable treatment experience and overall quality of life. People living with PI often receive regular infusions of immune globulin (Ig) replacement therapy to maintain their health, and this can interfere with their daily routine if they need to go to treatment centers to receive their medicine. A recent Harris poll survey of 104 PI patients sponsored by CSL Behring found that 94% of PI patients surveyed agree that flexibility and options are important in personalizing Ig treatment.

So, taking the time to understand a patient’s lifestyle can be an important factor when determining treatment. If a patient prioritizes flexibility and convenience, we may need to discuss whether they are candidates for subcutaneous immune globulin (SCIg), like Hizentra® (Immune Globulin Subcutaneous [Human] 20% Liquid), which allow PI patients to self-infuse in the comfort of their home and on a schedule that is convenient for them.

During my initial conversations, I try to get a feel for how involved the patient wants to be in their treatment administration. Some patients prefer intravenous immune globulin (IVIg) – having someone else infuse their treatment – because they are nervous or unsure if they can handle sticking themselves with needles. For many patients, unfamiliarity with a treatment process can make them apprehensive. In these instances, I aim to arm my patients with information and resources, such as training videos and the reassurance that a nurse will be available for training patients to self-infuse. I believe we need to give patients the time and tools to make an informed decision and allow them to explore their options.

How often do you approach discussions about treatment options with patients?

I recommend asking patients at each appointment about their treatment experience and if there are concerns that arise, schedule a separate appointment to have a robust dialogue around all options, as well as background on their lifestyle, to inform the decision. I firmly believe that physicians must work in tandem with patients to align on a treatment plan. PI patients have a number of choices when it comes to treatment, but the thing I like to point out is whether we are talking about IVIg or SCIg, all Ig products approved by the FDA for treatment of PI meet certain standards in terms of efficacy. From there, it’s our responsibility to check in with our patients about their treatment experience to determine if there are any adjustments that need to be made. I’ve found that some issues with SCIg can be mitigated by making adjustments like changing the needle length or infusion site. We also need to ask about the impact of their treatment schedule on their quality of life – are they missing time with family or friends because of side effects or time spent infusing – and adjust treatment as needed.

According to the aforementioned survey sponsored by CSL Behring, at least 97% of surveyed PI patients using SCIg prefilled syringe reported being satisfied with the ease of administration and overall convenience. I find that patients who are busy with work, school, and family and are looking for the next thing in terms of convenience, are willing to give it a try.

At the end of the day, if a patient isn’t happy with their treatment, they are unlikely to adhere to it. Many physicians engage in some shared decision-making, but not to the extent that it should be, and it goes back to the obstacle of time.

What advice do you have for your fellow physicians to ensure their patients are on a treatment that best fits their needs?

Physicians need to take a holistic treatment approach and evaluate individual patient needs to customize treatment based on efficacy, as well as flexibility and convenience, to fit into the everyday life of those living with a life-long disease. We need to keep patients informed of any updates and new products to ensure they are on the best possible treatment for their unique needs.

Important Safety Information

WARNING: Thrombosis may occur with immune globulin products, including Hizentra. 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.

For patients at risk of thrombosis, administer Hizentra 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 for hyperviscosity.

Hizentra is contraindicated in patients with a history of anaphylactic or severe systemic reaction to human immune globulin (Ig) or components of Hizentra (eg, polysorbate 80), as well as in patients with immunoglobulin A deficiency with antibodies against IgA and a history of hypersensitivity. Because Hizentra contains L-proline as stabilizer, use in patients with hyperprolinemia is contraindicated.

IgA-deficient patients with anti-IgA antibodies are at greater risk of severe hypersensitivity and anaphylactic reactions. Thrombosis may occur following treatment with Ig products, including Hizentra.

Monitor patients for aseptic meningitis syndrome (AMS), which may occur following treatment with Ig products, including Hizentra. In patients at risk of acute renal failure, monitor renal function, including blood urea nitrogen, serum creatinine and urine output. In addition, monitor patients for clinical signs of hemolysis or pulmonary adverse reactions (eg, transfusion-related acute lung injury [TRALI]).

Hizentra is derived from human blood. The risk of transmission of infectious agents, including viruses and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent and its variant (vCJD), cannot be completely eliminated.

The most common adverse reactions (observed in ≥5% of study subjects) were local infusion-site reactions, as well as headache, diarrhea, fatigue, back pain, nausea, extremity pain, cough, upper respiratory tract infection, rash, pruritus, vomiting, upper abdominal pain, migraine, arthralgia, pain, fall, and nasopharyngitis.

The passive transfer of antibodies can interfere with response to live virus vaccines and lead to misinterpretation of serologic test results.


Hizentra®, Immune Globulin Subcutaneous (Human), 20% Liquid, is indicated for:

  • Treatment of primary immunodeficiency (PI) in adults and pediatric patients 2 years and older.
  • Maintenance therapy in adults with chronic inflammatory demyelinating polyneuropathy (CIDP) to prevent relapse of neuromuscular disability and impairment.
  • Limitation of Use: Maintenance therapy in CIDP has been systematically studied for 6 months and for a further 12 months in a follow-up study. Continued maintenance beyond these periods should be individualized based on patient response and need for continued therapy.

For subcutaneous infusion only.

Please see full prescribing information for Hizentra including boxed warning.

To report SUSPECTED ADVERSE REACTIONS, contact the CSL Behring Pharmacovigilance Department at 1-866-915-6958 or FDA at 1-800-FDA-1088 or

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