Speaker Series Webinar: "Current Treatment Options for GBS, CIDP, MMN, and Variants"

Posted on June 13, 2024

Speaker Series – Episode 6 Summary

In this episode of our Speaker Series, we dive into the world of treatments for GBS, CIDP, and MMN as well as understanding them from Dr. Saperstein, a neurologist on our Global Medical Advisory Board.

Video Recording

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GBS

Two main treatments: Plasmapheresis or IVIg/Subcutaneous Infusion

Both are clinically proven to be equally effective with the same benefits
IVIg is more easily accessible in most hospitals whereas plasmapheresis requires more special equipment making it less practical for some patients
Most do well with each treatment, yet people can still have limitations years after their GBS episode

Is there ever a circumstance where GBS is not treated with IVIg?

The recommended window of treatment is within four weeks of diagnosis, so consult your doctor on starting treatment if you are past the four week mark from the start of your symptoms
Be Aware: Some insurances will advocate that if a patient has some function they do not need any treatment. Understand that their perspective comes from a financial standpoint rather than a patient-support perspective. Therefore, continue to advocate for yourself and you are always welcome to ask for a doctor to doctor consult from the Foundation.
It is important to note that some countries do not have access to IVIg for a number of reasons, leaving patients without access to IVIg or Subcutaneous Infusion
- Small Volume Plasma Exchange is an option https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6104782/

What if a patient does not respond to the first round of IVIg?

Most patient will not see results within a week, every patient is different so it is hard to predict the exact moment when patients will see results
If a first round of IVIg does not help a patient, most doctors will not approve a second round
- The GBS treatment guidelines and clinical evidence do not show data favorable of a second infusion.
- They will often try to use steroids or see if a patient has a variant typically unresponsive to IVIG
Variants like Miller-Fisher, AMAN, and AMSAN are usually treated the same way as GBS with IVIG and do not have variant specific treatments

Long Term Treatments

There aren’t any great treatments for fatigue, yet most doctors recommend exercise and physical therapy, and medication like Lyrica for pain management
Medications are prescribed for pain management like Lyrica, modulating the immune system, and treating nerve pain
If there as some residual effects present for more than a year, talk to your doctor because it may be a mimic or another condition affecting your wellbeing
Please remember to stay in touch with specialist who originally helped you with your GBS diagnosis because it you develop long-term effects from GBS they can serve as a resource!

CIDP

What is the difference between On label and Off label medications and how does it affect CIDP?

On label means that a medication is FDA approved where as off-label is not FDA approved
All immunoglobin from healthy donors, the only difference is a pharmaceutical’s method of stabilizing the immunoglobin
All IG for GBS is off-label while it is on-label for CIDP because of FDA’s indication

Subcutaneous Treatment or SCIg

SCIg is a self-injection of immunoglobin under the skin into fatty part of the body such as the thighs
A typical schedule with Subcutaneous IG involves a once-a-week infusion, and then the frequency can change as illness improves
Frequent infusions of IG at a lower dose can keep the body at a steady level without side effects like weakness, headaches, etc. that can occur with a higher dose
Gives patients more accessibility and convenience with self-injection
Most doctors see it as a second option to IVIg that patients can transition to

Concentration

Each brand has different concentrations such as 5% and even more like 10% (10% is mostly for IVIg)
Some IVIg brans can be administered subcutaneously
Some brands use 20% for CIDP and can still be given subcutaneously, the determining factor is a patient’s tolerance and responsiveness
Hydration is recommended to helping with IVIg and Subcutaneous
Concentration dosage is determined by a patient’s weight in kilograms

Hyqvia (Brand of IVIG; administered Subcutaneously)

Hyaluronidase is first injected to disrupt hyaluronic acid to make a pocket for fluid to go into
It is typically injected in the abdomen and is followed by the injection of IVIG
It takes 2-3 hours which is shorter than IVIg and longer than SCIg, given every 3-4 weeks so there are less infusions
It is recommended for patients who get more side effects from typical IVIg
It can be self-administered or with a nurse’s assistance

What are some alternatives to IG (Immunoglobin)?

In some cases, the patient’s body is not tolerating IVIg or SCIg rather than solely not working
Steroids is an option and less expensive, some patients take IVIg and Steroids at the same time
Plasmapheresis or plasma exchange: there are some risks like clogging veins and is in limited settings
Immunosuppressant: results may vary and take months to occur, it is often used with other therapies
- Rituxan or Rituximab (brand): target cells that will become plasma cells that will make antibodies so it prevents antibodies affecting myelin therefore preventing CIDP

MMN

Treatments

IVIG is the only proven treatment that works
Steroids either do not work or worsen the illness and condition
Immunosuppressants were used in the past
The focus of treatment is to tolerate IVIg or Subcutaneous IG

Anti-Mag

Treatments

Most patients do not respond to IVIg or steroids
Plasmapheresis does not work well
Immunosuppressants are tricky because they work depending on the patient
Rituxan is sometimes used to treat this condition with mixed results

Waiting for FDA Approval

Efgartigimod

An FCRN receptor blocker pulls bad immunoglobin out of the blood stream
Once a week infusion for an hour or use hyaluronidase resulting in shorter infusion times
⅔ of patient responded well but it is unclear if the long term effects lead to long-term remission, reverse a patient’s progress, or is a superior treatment to IG therapy
Currently being reviewed by the FDA as of April 2024

Other Potential Therapies

Several potential therapies are developing for GBS, CIDP, and MMN
Some of these potential therapies target the complement cascade within the immune system
FCRN inhibitors are another potential therapy option being studied in clinical trial