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Meningitis and PNH with Dr. Bhumika Patel

Dr. Bhumika Patel explains why meningitis can occur in patients with PNH and how it is both treated or prevented.



[00:00:30]    Hi everyone. This is Podcasts for Patients with the Aplastic Anemia and MDS International Foundation. I'm Tricia Maddrey Baker, Communications Manager. Our podcast series is brought to you thanks to generous support from patients, families, and caregivers like you and our corporate sponsors. Thanks to everyone for supporting the series. Today, we're talking about meningitis and PNH with Dr. Bhumika Patel at Prisma Health in Greenville, South Carolina. Welcome, Dr. Patel.

Bhumika Patel:    Thank you for having me today. And I would like to thank all of our patients and I would like to thank our sponsors and AAMDS for having me today.

Tricia:    Thank you. What is meningitis?

Bhumika Patel:
[00:01:00]    Meningitis is an inflammatory disease of the leptomeninges. It's the tissue that surrounds our brain and our spinal cord. It's characterized by an abnormal white cell count that shouldn't typically be in the fluid surrounding our brain and spinal cord in a majority of patients. Meningitis can be caused by bacterial infections, viral, and fungal infections, but what's important to keep in mind in our PNH patients, we're worried about the bacterial meningitis and which is, which we'll be talking about as we move along.

Thanks. What are the signs and symptoms that would help identify meningitis in a PNH patient?

Bhumika Patel:
[00:04:00]    Some of the signs and symptoms PNH patients on, um, active complement inhibition therapy that, uh, for meningitis, they could experience fever, nausea, vomiting, headaches, neck stiffness, photophobia, irritability, and they may have altered mental status. And to explain that further, that would be they are not acting right, they're more confused, they may have altered behavior, they may act very agitated, disoriented, and they may have inappropriate behavior which their family members or caregivers are not used to seeing. And so it's very critical that if your loved ones or you're experiencing these signs and symptoms, it's important for you to seek immediate medical attention for further evaluation, which may include, in the emergency room, include, um, brain imaging, lumbar puncture, and, um, and immediate antibiotic treatment. And at that time your clinical healthcare provider will hold your complement inhibition therapy until the infection has been resolved to decrease the risk of further complications.

Tricia:    Why do PNH patients need to be concerned about meningitis?

Bhumika Patel:

[00:06:00]    So, individuals, um, with PNH need to be concerned about meningitis, the reason being is that the complement system plays a pivotal role in our immune system. And when you're on complement inhibition therapy with the current FDA-approved therapies, which includes eculizumab, which, uh, another name is Soliris, ravulizumab, Ultomiris, and Empaveli, pegcetacoplan, these individuals at higher risk for developing infections because they're, we're inhibiting, uh, important components of the complement system which are actually supposed to be protective. And there's actually a black box warning on the package insert, which is why it's important for patients to be vaccinated two weeks prior to initiation of therapy, but  in the setting of urgent situations, they may be put on prophylactic antibiotics and then be bridged with vaccinations and, continued on therapy.

Tricia:    Dr. Patel, do all PNH patients need to be vaccinated for meningitis?

Bhumika Patel:

[00:08:00]    All patients with PNH, uh, do need to be vaccinated for meningitis. And it's normally the standard recommendation is if the patient is clinically stable, patients need to be vaccinated at least two weeks prior to initiation of any complement inhibition therapy. Currently, there are three FDA-approved therapies for PNH which include eculizumab, uh, the brand name of Soliris, ravulizumab, Ultomiris, and pegcetacoplan, Empaveli. It's critical to follow the package insert directions for vaccinations for these patients and also follow the CDC and Advisory Committee on Immunization Practices to make sure patients are being properly vaccinated for meningococcal infections, uh, Haemophilus influenzae type b, and also for Streptococcus.

Does an adult who was already vaccinated as a child need to be vaccinated again?

Bhumika Patel:

[00:09:00]    It is important, depending on their, um, immunization records, if they were not properly vaccinated, they would be vaccinated again, um, in the, in the setting if they're being put on complement inhibition therapy. But it is important prior to receiving vaccinations to mention to your healthcare provider if you have any allergies to any vaccinations in the past or any allergic reactions to ensure safety prior to initiation of vaccinations.

Tricia:    Thank you. What happens if a PNH patient contracts meningitis? Are there different treatments available or just one?

Bhumika Patel:

[00:10:00]    So if a, if a patient is experiencing any of the signs and symptoms of meningitis, it's critical to seek immediate urgent medical attention. And when they're in the emergency room, it's important for patient or the, caregivers to mention that the patient is a PNH patient on complement inhibition therapy and they're at high risk for meningococcal infection. Once they're in the emergency room, they would obtain proper imaging of the brain, lumbar puncture, which is one of diagnostic testing for diagnosing meningitis, and, uh, appropriate antibiotic treatment would be initiated for these patients. Many studies have shown that a delay in the administration of antimicrobial therapies can cause adverse effects on patients with PNH, which is important to make sure that you get there in a timely fashion and seek immediate attention if you have any signs or symptoms.

Tricia:    Thank you.

Bhumika Patel:

[00:10:30]    There are treatment options for meningitis. So once your diagnostic workup has been initiated and obtained, antimicrobial selection will be determined by your provider. And it's usually a penicillin or a third-generation cephalosporin plus or minus vancomycin, which may be initiated in the setting of your infection while, um, your studies from the cerebrospinal fluid studies are coming back. And you will be admitted to the hospital for further, treatment and management.

Tricia:    Thank you. What else do patients with PNH need to know about meningitis?

Bhumika Patel:

What's some important key factors that, individuals with PNH need to know is that current clinical practices, um, which includes, our CDC guidelines, our advisory of immunizations, and package inserts on all of the complement inhibition therapy, that the practices mitigate the risk of meningococcal infections but does not completely eliminate it. So it's really critical to watch for signs and symptoms and be an advocate for yourself. It's important that patients remain vigilant for signs and symptoms and important to seek immediate medical attention and notify healthcare providers of their diagnosis of PNH on complement inhibition therapy, until the, emergency, caregivers that, you know, "Please, um, you know, I'm on complement inhibition therapy, which puts me at high risk for meningitis."

[00:12:00]    Keeping an ongoing record of your immunizations is very critical because you need to be re-vaccinated every three to five years and so that's important to keep a record of. And, you know, all in all, you know, what's important is that your healthcare provider who prescribes the complement inhibition therapy needs to be notified, uh, if you are in emergency setting because at that time they will hold off on further treatment till your infection is resolved.

[00:12:30]    Thank you so much, Dr. Patel, for sharing your time and your expertise with all of us. You can find out more about PNH and other bone marrow failure diseases on our website at, by following us on Facebook, Instagram, Twitter, and LinkedIn, or by calling our helpline at 800-747-2820. Thank you for listening. See you next time.