Hello all,
My son Niko was diagnosed with MOGAD ADEM (with Optic Neuritis and Transverse Myelitis) almost a year ago on 11/4/20. To date he has received 6 plasmapheresis, 5 days of high dose steroids, 6 rounds of Cytoxan chemo w/dexamethasone (after some research I learned dexamethasone penetrates the blood brain barrier whereas methylprednisolone does not), and 10 IVIG’s to date.
His onset was severe. One of the most severe Dr.’s have seen. After five months of investigative approaches - 4 rounds of genetic work ups (2 via UW Pathology and 2 via Prevention Genetics), 4 infectious disease work up, 2 brain biopsies, and a leg muscle biopsy (a definitive way to determine any mitochondrial disorders)…all results yielded negative thankfully.
Suspected cause at this point? A flu vaccination approximately 4 weeks before rapid progression, in which subtle symptoms started presenting within a few days of the vaccination but I didn’t realize it. I thought he was just experiencing a growth spurt and/or caught a bug and even took him to urgent care on 10/31/20 and to his primary on 11/2/20. After months of research, A VAERS report was ultimately filed by Niko’s neurologist.
Niko is now 10 (admitted when he was 9) and an inpatient at Seattle Children’s currently almost a year. The hospital and team have been wonderful although we are all still struggling to figure out Niko’s persistent MOGAD.
Niko’s latest MRI results as of 10/2021:
No further atrophy since February.
Yes, some demyelination shows.
Of course, Dr. wants to entertain Rituxan but Dr. cannot guarantee what the PML risks would be. Nor can Dr. guarantee if Rituxan would even work.
At the same time Dr. is impressed that Niko looks good and despite demyelination and slow recovery appears stable. (As upon admittance Niko went from being able to talk, walk, see, to none of the above in a matter of 2-3 days. Niko also surprisingly never lost his independent breathing but was intubated just in case, he now has a trach and vented only at night to give him a break).
Now Niko is starting to visually track…but you can tell he still has vision challenges. Niko is moving his legs all the time and wiggling his toes. And for the first time in almost a year he tried to vocalize and talk a few days ago but they were mumbles. (I told him how proud of him I am and told him not to give up. That it will take time but he will talk again one day). In the meantime Niko has learned a secret handshake (thanks to an dedicated RT who spent 20 minutes hanging out and teaching him), able to tap his fingers on command, and give a thumbs up. We just need to give him a few seconds to respond.
As stated above, Niko also has trach. However, Niko is on a HME all day and we put him on the Astral vent only at night to give him a break and is on the lowest vent settings.
Anyhow, on the Rituxan I just don’t know what to do. I told Dr.'s my answer remains no on Rituxan because the gamble is too great. Especially since there continues to be a mixture of unexpected surprises.
I realize there are risks to anything in life, but as we know some risks are far greater than the average risks of getting into a car or crossing the street vs. injecting something directly into your body. The gamble/risks for Rituxan seem too great…but I will research on it more.
What I have learned so far:
The side effect would be PML (a brain infection) due to the reactivation of the JC virus that many are carriers of and stays dormant until activated (which there is no way to test if one is a carrier).
If he is a carrier (due to this monthly IVIG’s = 1000 donors per bag), chances are he would get PML which there is no known treatment for and it could kill him.
They also cannot guarantee that Rituxan would even work.
Hence my hesitation and current stance on no.
Don’t know what to do. Trust the lord and wait and give Niko more time? Especially since his steady improvements remain steady? Yes slow but steady.
I also asked if Niko’s TM went away and I was informed that yes it has. And while most of his demyelination went away he does still have persistent MOGAD and some demyelination. But nowhere as initially.
As of this point my decision on Rituxan remains no due so many unknown risks…especially since to this day they cannot identify why his condition is ongoing for so long.
Your thoughts?
I am not asking for subjective/objective advice per se as I know the decision is ultimately up to me and my husband. But rather I am asking for perspectives from others in case there’s something/an insight we haven’t considered. Especially since Dr.’s cannot seem to isolate reason for Niko’s persistent MOGAD.
Of course, quality of life is a concern, but I don’t want to lose Niko. And my gut is telling me to steer clear of the Rituxan, but of course my logic is all over the place. And while science is wonderful, there’s so much to be learned still with regards to ADEM.
Niko is currently wheelchair bound and immobile for the most part.
I advocate for regular PT/OT/SLP therapies regardless of goals.
I advocate for learning labs and readings partnered with the hospital school.
Daily chair time and showers are given to Niko in which I have bio-oil skin care massaged onto his body daily.
We’ve gotten close with many of the nurses in which they have been an amazing support system.
We also have gotten close to two of the hospital chaplains in which they make visits at least 2-3 times a week.
My husband and I partner with the nurses to play shows, music, audiobooks for him daily to keep his brain stimulated.
We also play relaxation sleep meditative music for him nightly.
And every single night either my husband or I stay with him at the hospital nightly.
I usually do Monday nights-Thursday nights after work.
And my husband usually does Friday nights-Sunday nights depending on his work schedule.
Thank you for taking the time to read this message. May God bless to you all.