Exploring a Holistic Approach to Rare Disease: Finding Flexibility and Convenience in a CIDP Treatment


Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is a rare autoimmune disorder that affects the peripheral nerves – those outside the brain and spinal cord – and damages the protective covering of the nerves, which is known as the myelin sheath. This condition most commonly manifests as symmetrical weakness and / or altered sensation in the extremities.1 Approximately 30% of people living with CIDP progress to wheelchair dependence if not treated.2 The GBS|CIDP Foundation estimates that the incidence of new CIDP cases is as high as two in 100,000 people, with the accumulation of cases over time resulting in prevalence as high as nine in 100,000 in some areas.3

Rare diseases like CIDP can be difficult to diagnose and treat, which can make patients feel isolated and uncertain.4 CIDP symptoms can progress over time and worsen, leading to significant activity limitations and a decreased quality of life for those with the disease.3

Krupa Sivamurthy, Executive Director, US Medical Affairs Immunology, CSL Behring discusses insights on the complexities of treating CIDP and the factors that come into play when working with people on individualized treatment plans.

There has been a lot of progress in the treatment of rare diseases over the years. How has the treatment landscape for CIDP, in particular, evolved?

There is no single root cause that underlies the occurrence of CIDP. Like some other rare diseases, a treatment option for CIDP is immunoglobulin replacement therapy. It is believed that immunoglobulins work through many of the pathways that contribute to the development and progression of CIDP. Two of the immunoglobulin options available today include intravenous immunoglobulin (IVIg) and subcutaneous immunoglobulin (SCIg). Both IVIg and SCIg are made from donated human plasma.5

IVIg treatment is administered through a vein with a needle or catheter. With IVIg, most patients must receive treatment at their doctor’s office or an infusion clinic. Some patients may be able to have home infusions; however, a nurse must be present to administer it.3

SClg infusions, on the other hand, are injected into fatty tissue just underneath the skin. Once trained, SCIg infusions allow patients to self-administer the treatment at a recommended infusion site location of their choice, providing greater convenience and flexibility in managing their disease.6

Harris Poll conducted a survey* on behalf of CSL Behring that included 100 people living with Chronic Inflammatory Demyelinating Polyneuropathy (CIDP), 98 of whom were currently on treatment for their CIDP. What did the results show in terms of IVIg vs. SCIg treatment options?7

The survey found that the majority of surveyed people living with CIDP believe administering treatment at home (72%) and having flexible dosing options (70%) are extremely or very important.7 Additionally, it showed that of the 98 patients surveyed who were currently on treatment for CIDP, 82% agree that they are interested in exploring more convenient treatment options.7 Given eligibility, SCIg prefilled syringes may be a more convenient option for certain individuals as it may reduce the steps needed for infusion versus IVIg. Additionally, the wide range of syringe sizes cater to a range of patient doses.

That said, some patients may prefer to have a medical professional administer their treatment, as done with IVIg, and may be intimidated by self-infusion with SCIg. Pros and cons when deciding on the right treatment option are discussed through a shared decision-making process between the physician and patient. This process aims to engage, educate and empower patients to consider a variety of treatment-impacting factors and select a therapy that works best for them.

* The survey was conducted on behalf of CSL Behring with 100 people living with Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) and 100 neurologists/neuromuscular specialists. The survey took place between February 27, 2023 and March 15, 2023. Patients were identified and recruited directly by treating physicians. Both patients and physicians were fully screened for eligibility. The survey qualification criteria for patients included being age 18+, residency in the U.S. and CIDP diagnosis. The survey qualification criteria for physicians included that they practice in the U.S., were duly licensed, and were neurologists/neuromuscular specialists who regularly saw at least one patient with CIDP.

How does living with CIDP affect the day-to-day life of patients?

In the same Harris Poll survey of 100 people with CIDP, almost all of the CIDP patients agreed or somewhat agreed that living with CIDP can be very limiting (98%).7 The majority of patients surveyed also agreed or somewhat agreed that they feel like a burden to their friends and family (80%).7 Moreover, of the 98 patients currently receiving treatment at the time of the survey, 94% agreed or somewhat agreed that finding a treatment for their CIDP made a significant positive impact on their lives, however only 61% agreed or somewhat agreed that they found a treatment that worked for their lifestyle.7* Knowing this, it is important that physicians continue to explore alternative treatment options with their patients that can potentially offer greater flexibility with managing their condition in a way that works better with their lifestyle.

How can physicians and patients better communicate about CIDP treatment plans?

Managing a rare disease is not a linear process as treatment goals and lifestyle needs change over time. However, in the Harris Poll survey less than half of the patients said they discuss potential new treatment options or switching treatments with a physician (47% and 43% respectively)7 —which can be an obstacle to identifying the best treatment approach. Given these results, more information about treatment options and innovations should routinely become a part of the patient and physician dialogue. Additionally, shared decision-making is an ongoing process that requires continual conversations to arrive at and manage a treatment option that is right for that particular patient. This process is beneficial for both the physician and patient to create open dialogue and identify an informed treatment approach.

If you’re interested in learning more about CIDP and an available SCIg treatment option, please visit Hizentra.com.

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 www.fda.gov/medwatch.

Hizentra is manufactured by CSL Behring AG and distributed by CSL Behring LLC.

Hizentra® is a registered trademark of CSL Behring AG.

1 MedlinePlus. Bethesda (MD): National Library of Medicine (US). Chronic inflammatory demyelinating polyneuropathy. Available at: https://medlineplus.gov/ency/article/000777.htm.

2 National Organization for Rare Disorders, Inc. Chronic inflammatory demyelinating polyneuropathy. Available at: https://rarediseases.org/gard-rare-disease/chronic-inflammatory-demyelinating-polyneuropathy.

3 GBS׀CIDP Foundation. What is Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)? Available at: https://www.gbs-cidp.org/cidp.

4 EURORDIS Rare Diseases Europe. Mental Health & Wellbeing. Available at: https://www.eurordis.org/mental-wellbeing.

5 Van den Bergh, PYK, van Doorn, P.A, Hadden, RDM, et al.. European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: Report of a joint task force—second revision. Eur J Neurol. 2021 Nov;28(11):3556-3583. doi: 10.1111/ene.14959. Epub 2021 Jul 30.

6 Hizentra Prescribing Information.

7 Data on file. Available from CSL Behring as DOF HIZ-016.

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