Frequently Asked Questions

Below are answers to the most frequent questions from prescribers like you, separated by indication (CIDP or PI) or support and access.

If your questions are not covered on this page or you need additional information, please let us know. We'll contact you by your preferred method–email, mail, or phone.

Support and Access

CIDP

PI

Starting Patients on Hizentra: Access and Billing Codes

How do I bill for Hizentra?

The treating physician is solely responsible for diagnosis coding and determination of the appropriate ICD-10-CM codes that describe the patient's condition and are supported by the medical record. The billing party is solely responsible for coding of services (eg, CPT Coding).

The Hizentra Coding Guide can be found here. All codes listed in this guide are for informational purposes and are not an exhaustive list.

The CPT, HCPCS, and ICD-10-CM codes provided are based on AMA and CMS guidelines. Because government and other third-party payer coding requirements change periodically, please verify current coding requirements directly with the payer being billed.

Filling each section of the claim entirely and including all of the requested documentation is the best way to avoid payment delays.

How much does Hizentra cost?

The cost of any treatment, including Hizentra, depends on a number of factors, including insurance status and the insurer's co-pay/coinsurance policy. The actual out-of-pocket cost to a patient will depend on their individual prescription and medical coverage.

For those who cannot afford their treatment, CSL Behring is committed to providing appropriate resources to ensure patients can begin and/or remain on their therapy. These include:

Free Trial offers eligible patients a free 1-month supply of Hizentra and infusion equipment, and free at -home visits by a trained nurse. Other terms and conditions may apply.

Copay, for those who need assistance with copays, deductibles, or coinsurance specifically related to the product. Most eligible patients with commercial insurance pay $0 out of pocket.^*

Patient Assistance Program, which provides access to Hizentra for qualified patients who are uninsured or underinsured.

Assurance program, which is designed to help eligible patients, who rely on CSL Behring therapies, continue to receive treatment even if they experience a lapse in insurance coverage.^†

For more information, including terms and conditions of these programs, call Hizentra Connect^SM Resource Center, CSL Behring's single source for information about immunoglobulin therapies, at 1-877-355-4447, Monday–Friday, 8 AM to 8 PM ET.

* Subject to terms and conditions of the Copay assistance program. To read the full terms and conditions, visit cslbehring.com/patients/support-and-assistance. CSL Behring reserves the right to rescind, revoke, or amend the program at any time without notice. ^† Enrollment required. Subject to terms and conditions. For the Assurance program, visit cslbehringassurance.com.

Is Hizentra covered by Medicare Part B?

Hizentra is covered under Medicare Part B for both PI and CIDP.* Get the convenience of self-infused Hizentra, covered under the same benefit category as IVIg.^† Medicare Part B coverage means lower out-of-pocket costs for patients than with Part D, covering their infusion pump, ancillary supplies, medication, and nurse training. And no prior authorization is required.^‡

See access and billing codes for PI and CIDP.

For coverage and comprehensive support, call Hizentra Connect^SM at 1-877-355-4447 Monday-Friday, 8 AM to 8 PM ET.

^*100% coverage for those with Medicare Part B and a Medigap plan after Part B annual deductible is met. ^†Coverage of IVIg when administered in a clinical setting. ^‡Chart notes must be up to date and include diagnostic criteria.

Does CSL Behring provide assistance with Hizentra billing information?

Yes, Hizentra Connect^SM can provide coding, billing, and reimbursement information, as well as assistance with insurance verifications and prior authorizations.

For more information, call the Hizentra Connect Resource Center, CSL Behring's single source for information about immunoglobulin therapies, at 1-877-355-4447, Monday–Friday, 8 AM to 8 PM ET.

Starting Patients on Hizentra: Patient Resources

Can my patients self-administer Hizentra?

Hizentra is a self-administered immunoglobulin therapy for CIDP and PI. Patients can learn to easily give themselves Hizentra with training from a healthcare professional. The Free Trial program not only provides patients with a 1-month supply of Hizentra and infusion equipment, it also provides in-home training visits by a trained nurse–at no cost for Hizentra-naïve patients. After patients have gone through training and have successfully self-administered in front of a healthcare professional, they can self-administer Hizentra.

If patients would like a helpful teaching tool after they have been trained to self-infuse Hizentra, or if they would like a refresher later on, patients can follow easy instructions detailed in the self-administration guide.

It might be a good idea to periodically ask your patients about their self-administration technique, especially if their infusion regimen has recently changed. See Patient Resources for more information.

Infusion Resources: Managing Therapy

How are infusion issues managed?

See the Managing Therapy page for a detailed list and downloadable guide of troubleshooting suggestions.

Infusion Resources: SHARE Nurse Training Center

Why do I need to register for the online SHARE training program?

Registration is required so we can help you keep track of your progress in each module and provide a certificate once you've passed the exam.

How do I access SHARE training materials?

You can access SHARE training through the SHARE page of this site. Registration is required.

What if my staff has already been trained on administration of other SCIg therapies?

SHARE training is specific to Hizentra administration. While your staff may be familiar with certain aspects of SCIg administration, there are specific clinical and administrative aspects that are unique to Hizentra and therefore necessary for proper education.

How long does SHARE training take?

It's hard to say exactly how long training will take because you will be able to move at your own pace. However, most people are able to finish in less than an hour.

Will my staff have to be retrained in the future?

It is recommended that your staff go through training regularly, especially if they don't train patients on a frequent basis.

What happens when someone completes training?

After training, you can take the exam to test your knowledge of Hizentra administration. Upon passing, you will receive a certificate and be provided the option to add your organization to the Nurse Locator.

I have taken the SHARE training. How do I take the exam?

To take the exam, visit the SHARE page and select the option to take the exam. You will need to log in using your email and password used when registering for a SHARE Nurse Training account.

I am having problems logging in to SHARE or viewing/printing my certificate, what can I do?

If you have forgotten your password, you can have the password sent to your registered email address by completing the "Forgot Password" form. For any other technical issues, please contact Hizentra Connect at 1-877-355-4447, Monday–Friday, 8 AM to 8 PM ET.

Problems viewing your certificate are commonly caused by the Adobe^® Reader application. Ensure your Adobe^® Reader is up-to-date.

Hizentra Connect^SM Support

Where can my patients get more information about financial and copay assistance?

Hizentra Connect can provide information on programs designed to help patients with access to Hizentra therapy. They can also answer other related financial assistance questions.

For more information, call Hizentra Connect, CSL Behring's single source for information about immunoglobulin therapies, at 1-877-355-4447, Monday–Friday, 8 AM to 8 PM ET.

Proven CIDP Efficacy and Safety

How effective is Hizentra at reducing CIDP relapse?

A multicenter, double-blind, randomized, placebo-controlled, parallel-group, phase III study evaluated the efficacy, safety, and tolerability of 2 different weekly doses of Hizentra (0.4 g/kg body weight and 0.2 g/kg body weight) vs placebo in 172 adult subjects with CIDP and previously treated with IVIg (PATH Study).⁵

CIDP relapse was defined as a ≥1-point increase in adjusted Inflammatory Neuropathy Cause and Treatment [INCAT] score compared with baseline.

The main endpoint was the percentage of subjects who had a CIDP relapse or were withdrawn for any other reason during the subcutaneous treatment period. Both Hizentra doses demonstrated superiority over placebo for the main endpoint (32.8% for 0.4 g/kg Hizentra and 38.6% for 0.2 g/kg Hizentra compared with 63.2% for placebo, p<0.001 and p=0.007, respectively), with no statistically significant difference between the Hizentra-treated groups.⁵

81% and 67% of Hizentra-treated subjects remained CIDP relapse free (0.4 g/kg body weight and 0.2 g/kg body weight, respectively); 44% of placebo subjects remained relapse free for up to 24 weeks.⁵

Is Hizentra proven effective with adjustable dosing?

Initiate therapy 1 week after last IVIg infusion

Recommended subcutaneous dose is 0.2 g/kg body weight per week*
- A dose of 0.4 g/kg body weight per week was also safe and effective
If CIDP symptoms worsen on 0.2 g/kg consider increasing to 0.4 g/kg per week*
Most patients remained relapse-free on either dosing option, with the 0.4 g/kg dose showing a lower rate of relapse^†‡
- If CIDP symptoms worsen on the 0.4 g/kg dose, consider reinitiating therapy with IVIg

* Administered in 1 or 2 sessions over 1 or 2 consecutive days. † CIDP relapse was defined as a ≥1-point increase in adjusted Inflammatory Neuropathy Cause and Treatment [INCAT] score compared with baseline. ‡ Statistical tests between the two doses were not conducted.

View additional dosing information for Hizentra.
Learn more about the efficacy of Hizentra.

How effective is Hizentra at helping to maintain functional ability in patients with CIDP?

Hizentra has been shown to maintain functional ability across multiple secondary endpoints^5*

Stable MRC score^† – 0.0 median change from baseline; p=0.0026^‡
Stable INCAT score^† – 0.0 median change from baseline; p<0.0001^‡
Stable grip strength^† – -1.7 kPa median change from baseline; p=0.0223^‡

Hizentra also demonstrated better R-ODS scores vs placebo^5§

Median change from baseline to last postdose observation was -3.0 points for the placebo group, -2.0 points for the 0.2 g/kg Hizentra group, and 0.0 for the 0.4 g/kg Hizentra group (p=0.0002 for all). ^||

*Study design: Data from a randomized, multicenter, double-blind, placebo-controlled, parallel-group, phase III study of 2 doses of weekly Hizentra versus placebo for the treatment of chronic inflammatory demyelinating polyneuropathy (CIDP)—the PATH Study. †Statistically significant vs placebo at the last postdose observation. The MRC (Medical Research Council) sum score was assessed in the following 8 bilateral muscle pairs: shoulder abduction, elbow flexion, wrist extension, index finger abduction, hip flexion, knee extension, foot dorsiflexion, and great toe dorsiflexion. The INCAT score is a 10-point scale that covers the functionality of legs and arms. Grip strength was measured in both hands by the Martin Vigorimeter. Grip strength was for dominant hand. ‡Overall p-values are based on both Hizentra dosing groups compared to placebo. §The Rasch-built Overall Disability Scale (R-ODS) measures a patient's ability to perform a wide range of activities ranging from easy tasks, like reading a book, eating, and brushing teeth, to more difficult tasks such as standing for extended periods of time or running. Exploratory endpoint. ||The difference between the 0.2 g/kg Hizentra group and the placebo group was not significant.

What were the adverse reactions in the clinical trials of Hizentra for CIDP?

In the PATH Study, the most common adverse reactions (ARs) observed in ≥5% of study subjects receiving Hizentra and at a higher frequency than placebo were local infusion-site reactions*, headache, nasopharyngitis, fatigue, upper respiratory tract infection, fall, back pain, arthralgia, and pain in extremity.⁵

Patients on Hizentra reported a 3.6-fold lower rate of systemic adverse reactions per infusion vs IVIg.^† 93% of 4,225 total Hizentra infusions were free of any reported ARs.⁵

Overall, safety findings in the Open-Label Extension (OLE) study to PATH were consistent with those from the PATH study and the established safety profile for Hizentra.

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.

*Includes infusion-site erythema, infusion-site swelling, infusion-site pain, infusion-site induration, infusion-site warmth, infusion-site hematoma, and infusion-site pruritus. †Data represent systemic side effects reported during the 13-week single-arm restabilization phase compared to those reported in the 2 Hizentra groups during the 24-week postrandomized phase. However, there was no parallel group of subjects receiving placebo in the IVIg restabilization phase.

Which of my patients with CIDP would be appropriate for Hizentra subcutaneous Ig treatment instead of IVIg therapy?

While IVIg treatment helps control CIDP, challenges may remain for many patients. For example, in some patients, poor venous access can make infusion difficult.¹ Also, systemic adverse reactions such as headache and fever can occur.² Patients also have lifestyle burdens. IV infusions can be costly and constraining³ and traveling to an infusion clinic can be time-consuming and require missing work.³

Consider Hizentra for your patients with CIDP who:

Experience IV-related systemic ARs
Have venous access issues
Require more frequent IV infusions
Seek, flexibility, freedom, and control

CIDP Dosing and Administration

What dose should CIDP patients start on?

Initiate Hizentra therapy 1 week after the last IVIg infusion, with the recommended subcutaneous dose of 0.2 g/kg (1 mL/kg) body weight (bw) per week.

In the PATH study, both 0.2 g/kg and 0.4 g/kg doses were found to be safe and effective.

What should patients with CIDP do if they miss a dose of Hizentra?

Patients should contact their healthcare provider for further instructions and monitor for returning symptoms.

How many infusion sites should be used with Hizentra for patients with CIDP?

A Hizentra dose can be infused into multiple sites, based on dose and tolerability. The number and location of infusion sites depends on the volume of the total dose. Use up to 8 sites per infusion. Infusion sites should be at least 2 inches apart. Change the actual site of infusion with each administration. New sites should be at least 1 inch from the previous site. Recommended infusion sites include the thighs, upper arms, stomach, and hips.

How long does it take to infuse Hizentra for patients with CIDP?

In the PATH Study, patients successfully infused Hizentra at a median of ~1 hour per infusion session (patients received two infusions per week).^5* Your patient's experience may vary, depending on a number of factors, including infusion rate, infusion volume, number of infusion sites used, type of tubing, and patient tolerability.

What volume of Hizentra should be used per infusion site for patients with CIDP?

For patients with CIDP, the first infusion of Hizentra should not exceed a volume of 20 mL per infusion site. The volume may be increased to 50 mL per site for the subsequent infusions as tolerated.

At what rate do you infuse Hizentra for patients with CIDP?

For the first infusion of Hizentra, the recommended maximum flow rate is 20 mL per hour per site. For subsequent infusions, the flow rate may be increased to a maximum of 50 mL per hour per site as tolerated.

Why might some patients prefer prefilled syringes?

Prefilled syringes may be a compelling option for your CIDP patients since they are simple, convenient, and ready to use. Prefilled syringes may reduce steps and effort during infusion preparation vs. vials, depending on dose. And prefilled syringes may also simplify the set-up and transfer of Hizentra for patients who have difficulty drawing from vials. Prefilled syringes are available in a variety of sizes for added convenience when customizing treatment to patient needs.

Learn more about Hizentra prefilled syringes

How long can Hizentra be stored at room temperature?

Hizentra can be stored at room temperature (up to 77°F [25°C]) for its entire shelf life, up to 30 months. Because Hizentra is stored at room temperature, there is no need to refrigerate. Room-temperature storage enables patients to infuse when they are ready, without waiting to bring the product to room temperature before use.

Proven PI Efficacy and Safety

How effective is Hizentra at reducing infections in patients with PI?

Hizentra provides proven protection against infection, with over 6 million exposures since 2010.^4* In the US clinical trial,^† the annual rate of serious bacterial infections (SBIs) was 0 per subject year, while the annual rate of any types of infections was 2.76 per subject year. This means that patients did not experience any serious infections (eg, bacterial pneumonia, sepsis, osteomyelitis, visceral abscess). On average, patients had fewer than 3 infections of any type per year.⁶

In the long-term (1.7 years) extension study,^7‡ Hizentra was demonstrated to provide safe, sustained, effective protection from infections. In this study, there were zero reported serious bacterial infections (per subject year, annualized rate), zero treatment-related serious adverse events, and zero subjects who discontinued because of treatment-related adverse events.⁷

*Estimate based on amount of Hizentra grams sold worldwide from 2010 through June 2018. †Demonstrated in patients 2–65 years of age and older in the 12-month US phase 3 trial with Hizentra weekly. Hizentra was initiated at a weekly dose of 153% of the previous IVIg dose in the US study. Serious bacterial infections were defined as bacterial pneumonia, bacteremia/septicemia, osteomyelitis/septic arthritis, bacterial meningitis, and visceral abscess. ‡During the US extension study, 2 SBIs (bacterial pneumonia) in 2 subjects were reported, giving an annualized rate of 0.06 infections per subject. Serious bacterial infections were defined as bacterial pneumonia, bacteremia/septicemia, osteomyelitis/septic arthritis, bacterial meningitis, and visceral abscess.

What were the adverse reactions in the clinical trials of Hizentra and what adverse reactions can my patient with PI expect from an infusion with Hizentra?

In the US clinical study, the most common adverse reactions (observed in 5% or more of study subjects receiving Hizentra) were local reactions (ie, swelling, redness, heat, pain, and itching at the injection site), headache, diarrhea, fatigue, back pain, nausea, extremity pain, cough, rash, pruritus, vomiting, upper abdominal pain, migraine, and pain.

Local reactions were mostly mild (93.4%) or moderate (6.3%). Because Hizentra is infused under skin, local reactions are common and expected. In a clinical study, local reactions decreased over time, from ~20% following the first infusion to <5% by the end of the study.*

Please see the full Prescribing Information for a complete list of adverse reactions.

*Three subjects withdrew from the European study due to adverse reactions.

Flexibility for Patients With PI

How are the trough IgG levels affected when switching a patient with PI from IVIg to weekly or biweekly (every 2 weeks) dosing of Hizentra?

When switching from IVIg to weekly dosing of Hizentra, the target serum IgG trough level is projected to be approximately 16% higher than the last trough level during prior IVIg therapy. For biweekly (every 2 weeks) dosing of Hizentra, the trough IgG level is projected to be approximately 10% higher than the last IVIg trough level.^*†

*No difference in the clinical benefit of SCIg and IVIg serum profiles have been demonstrated by substantial clinical evidence or experience. †Weekly dose based on dose adjustment factor of 1.37 when switching from IVIg. Biweekly dosing = 2x weekly dose, based on adjustment factor of 1.37 when switching from IVIg.

How are the trough IgG levels affected when switching a patient with PI from weekly dosing of Hizentra to biweekly (every 2 weeks) or more frequent (2 to 7 times per week) dosing?

When switching from weekly Hizentra dosing to biweekly dosing, the target trough is projected to be approximately 5% lower than the last trough level on weekly therapy. When switching from weekly dosing to more frequent Hizentra dosing, the target serum IgG trough level is projected to be approximately 3 to 4% higher than the last trough level on weekly therapy.^* See the steady-state Ig levels at any Hizentra dosing interval.

*Weekly dose based on dose adjustment factor of 1.37 when switching from IVIg. Biweekly dosing = 2x weekly dose, based on adjustment factor of 1.37 when switching from IVIg.

PI Dosing and Administration

Can biweekly (every 2 weeks) and more frequent dosing be used in pediatric and geriatric populations with PI?

Data from more than 150 unique subjects were analyzed, including a subset of pediatric (over age 2) and geriatric (over age 65) subjects.^* No age-related effects were observed; thus, no specific dose adjustments for biweekly or more frequent dosing are needed for either pediatric or geriatric populations.

*Biweekly and frequent regimens are based on pharmacokinetic modeling and simulations.

When should I check IgG levels after changing a patient with PI to Hizentra?

Over time, the dose may need to be adjusted to achieve the desired clinical response and serum IgG trough level, regardless of the frequency of administration. To determine if a dose adjustment should be considered, measure the patient's serum IgG trough level 2 to 3 months after switching to Hizentra.

What should patients with PI do if they miss a dose?

Patients should contact their healthcare provider for further instructions and monitor for symptoms of infection.

How many infusion sites should be used with Hizentra for patients with PI?

A Hizentra dose can be infused into multiple sites, based on dose and tolerability. The number and location of infusion sites depends on the volume of the total dose. As few as 1 site and up to 8 sites can be used in parallel. Infusion sites should be at least 2 inches apart. Change the actual site of infusion with each administration. New sites should be at least 1 inch from previous site. Recommended infusion sites include the thighs, upper arms, stomach, and hips. To see how Hizentra can help fit patient needs and preferences, go to the PI patient profiles.

How long does it take to infuse Hizentra for patients with PI?

In the US clinical trial, the median duration of a weekly infusion ranged from 1.6 to 2 hours. Hizentra allows flexible dosing for your patients. The duration of infusions can vary, depending on a number of factors, including dosing schedule, number of infusion sites used, type of tubing, needle gauge, and patient tolerability.

What volume of Hizentra should be used per infusion site for patients with PI?

For the first infusion of Hizentra, do not exceed a volume of 15 mL per infusion site. The volume may be increased to 25 mL per site as tolerated.

Learn more about dosing and administration

At what rate do you infuse Hizentra for patients with PI?

For the first infusion of Hizentra, the recommended maximum flow rate is 15 mL per hour per site. For subsequent infusions, the flow rate may be increased to a maximum of 25 mL per hour per site as tolerated.

Learn more about dosing and administration

Does the infusion rate or volume administered per site change depending on the dosing schedule of Hizentra for patients with PI?

No. The maximum volume per infusion site is the same with any of the dosing regimens and is based on patient tolerability. Depending on several factors, the infusion rate might be the same.

Example based on 10-g dose.To determine your patient's specific dose per his or her dosing regimen, use the PI dosing calculator.

Why might some patients prefer prefilled syringes?

Prefilled syringes may be a more compelling options for PI patients since they are simple, convenient, and ready-to-use. Elimination of vial transfer may reduce steps and effort, depending on dose. Prefilled syringes are transferred to a pump syringe using a syringe-to-syringe transfer device. Prefilled syringes are available in a variety of sizes for added convenience when customizing treatment to patient needs.

Learn more about Hizentra prefilled syringes

How long can Hizentra be stored at room temperature?

Frequently Asked Questions

Support and Access

CIDP

PI

Starting Patients on Hizentra: Access and Billing Codes

Starting Patients on Hizentra: Patient Resources

Infusion Resources: Managing Therapy

Infusion Resources: SHARE Nurse Training Center

Hizentra ConnectSM Support

Proven CIDP Efficacy and Safety

CIDP Dosing and Administration

Proven PI Efficacy and Safety

Flexibility for Patients With PI

PI Dosing and Administration

Hizentra Connect^SM Support