1. < Autoimmune Diseases
Mentor

CLIPPERS: Looking to connect with others

Posted by Becky, Volunteer Mentor @becsbuddy, Nov 14, 2019

Have you ever been told that you have Clippers disease and you’re thinking: “ what? I’ve never heard of such a thing! What on earth”? Well Clippers is real and is one of the newer Autoimmune Diseases—it was only given a name in 2010. It’s similar to many other AD I’ve read about on Connect: vague, random symptoms that can be debilitating and difficult to diagnose. An MRI and brain biopsy are needed to confirm diagnosis.

My journey with Clippers (Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids) began with unexplainable vomiting. After many trips to the ER and being shrugged off be doctors, someone decided that it must be a gallbladder problem, so it was taken out, but nothing changed. The vomiting got worse and, eventually, I couldn’t walk without help, I choked on foods and water, my voice changed, and I lost 20 pounds. I was finally taken seriously by doctors when husband again took me to the ER and I was basically unresponsive. I was admitted and given an MRI (which I had repeatedly begged for) and the scan showed a large area of demyelination of my brain. None of the doctors knew what was going on, but the neurologist finally decided on intravenous steroids and a brain biopsy. I have no memory of that hospitalization. When I got to rehab, my husband took me to University of Colorado medical center where I continue to get treatment in their neurology-immunology department.

My message is to encourage anyone, with unexplainable symptoms, to tell the doctors to “think outside the box” and to advocate for themselves. Have someone go with you to appointments and to take notes! Don’t keep information only in your head!

Anyone else out there?

Interested in more discussions like this? Go to the Autoimmune Diseases Support Group.

donnyboy | @donnyboy | Jun 7 10:32am

@becsbuddy @suec147 @catartist

Thanks to everyone for sharing all this information about your experience or trials with rituximab! An amazing amount of information.

Becky, I already had the article you sent me references for, and it is a very good one from 2018. Thanks. It looks like Clippers treatment requires finding a medication regimen that needs to be changed as the condition of one's body changes. I haven't heard of anyone sticking with rituximab indefinitely, although the woman in the article was, at last report, on it for four years after the frequency of the rituximab infusions was increased from once every six months to once every four months. No update is available about whether she has stayed with the rituximab past four years (maybe one could ask the article's authors?). I guess while we hope for and pursue finding a long-term treatment regimen, we have to live with always monitoring ourselves for the emergence of new symptoms and then dealing with their impact. Yuck! But staying on the hope side as long as possible!

Sue, hope your appointment next Wednesday leads to a positive outcome for you, including the return of vision in your left eye.

Chris, I've gone through weight and muscle loss but am coming back significantly with occupational and physical therapy over he past 3-4 months. Just a few weeks ago, after a short relapse, I looked as out of shape as I've ever seen myself (women looking at me naked, instead of being impressed, would be depressed - ha), but I am getting back to normal by exercising (swam 22 laps this morning). I realize my condition is unique, but do try to get your muscles back if you can. As they say, use it or lose it.

Don

REPLY
1 reply
catartist | @catartist | Jun 8 9:42am
In reply to @donnyboy "@becsbuddy @suec147 @catartist Thanks to everyone for sharing all this information about your experience or trials..." + (show)
@donnyboy

@becsbuddy @suec147 @catartist

Thanks to everyone for sharing all this information about your experience or trials with rituximab! An amazing amount of information.

Becky, I already had the article you sent me references for, and it is a very good one from 2018. Thanks. It looks like Clippers treatment requires finding a medication regimen that needs to be changed as the condition of one's body changes. I haven't heard of anyone sticking with rituximab indefinitely, although the woman in the article was, at last report, on it for four years after the frequency of the rituximab infusions was increased from once every six months to once every four months. No update is available about whether she has stayed with the rituximab past four years (maybe one could ask the article's authors?). I guess while we hope for and pursue finding a long-term treatment regimen, we have to live with always monitoring ourselves for the emergence of new symptoms and then dealing with their impact. Yuck! But staying on the hope side as long as possible!

Sue, hope your appointment next Wednesday leads to a positive outcome for you, including the return of vision in your left eye.

Chris, I've gone through weight and muscle loss but am coming back significantly with occupational and physical therapy over he past 3-4 months. Just a few weeks ago, after a short relapse, I looked as out of shape as I've ever seen myself (women looking at me naked, instead of being impressed, would be depressed - ha), but I am getting back to normal by exercising (swam 22 laps this morning). I realize my condition is unique, but do try to get your muscles back if you can. As they say, use it or lose it.

Don

Jump to this post

Thanks Don for replying! And yes PT and exercise IS so important and helpful. But my issue is a deteriorating disease that it atrophying my muscles regardless. RA can lead to this Cachaxia, and mine is made worse by steroid wasting syndrome - I am looking for others experiencing this. Hard to know what to expect. I have already lost most of both shoulder (cuff) and two of 3 glutes - only have Maximus working - no swimming for me! But pool water resistance is good. And chair yoga, walking ( with walker now). Any one???

REPLY
1 reply
donnyboy | @donnyboy | Jun 8 3:30pm
In reply to @catartist "Thanks Don for replying! And yes PT and exercise IS so important and helpful. But my..." + (show)
@catartist

Thanks Don for replying! And yes PT and exercise IS so important and helpful. But my issue is a deteriorating disease that it atrophying my muscles regardless. RA can lead to this Cachaxia, and mine is made worse by steroid wasting syndrome - I am looking for others experiencing this. Hard to know what to expect. I have already lost most of both shoulder (cuff) and two of 3 glutes - only have Maximus working - no swimming for me! But pool water resistance is good. And chair yoga, walking ( with walker now). Any one???

Jump to this post

Gosh, that's tough. Hope someone has some suggestions for you.

REPLY
1 reply
catartist | @catartist | Jun 8 7:29pm
In reply to @donnyboy "Gosh, that's tough. Hope someone has some suggestions for you." + (show)
@donnyboy

Gosh, that's tough. Hope someone has some suggestions for you.

Jump to this post

Thanks again- I am not giving up! I lost a fair amt. of weight too - part of it apparently (holding 98 past few days). Fingers crossed.

REPLY
suec147 | @suec147 | Jun 11 3:03pm
In reply to @donnyboy "@suec147 @becsbuddy Sue, I moaned as I read what you said about years of searching for..." + (show)
@donnyboy

@suec147 @becsbuddy

Sue, I moaned as I read what you said about years of searching for the right medication and I gasped when I read about your right eye blindness and your recent fall. You poor dear. I wish I could help immediately, but maybe something I say about my own condition and experience might lead to something of assistance to you.

My diagnosis of Clippers came on May 3rd, after my neurologist, who focuses on auto-immune issues, had tested for and ruled out a variety of possible diagnoses that would be possible for someone with a demyelination disease (e.g., MS, NMO). My symptoms, which began with left eye vision impairment, and the hospitalizations that ensued, only began last September, and finding the right prednisone regimen has been a major issue and question. Based upon earlier changes and their results, I am now on 20 mg prednisone per day, every day, plus gabepentin, while pressing forward with OT and PT. Some consideration is being given to shifting me to what Becky has started, rituximab infusions.

I’ve been very active in exploring Clippers, for example by joining Mayo Clinic Connect and doing online investigation and buying Bill Crum’s A Book on CLIPPERS. My neurologist has lined up an appointment for me in 10 days in NYC with a specialist well versed with Clippers for a second opinion or evaluation. I have also been invited for consultation anytime with a Mayo Clinic doctor in Minnesota.

Given all this, I have to ask if you are comfortable with your neurologist. Even though she gave you a Clippers diagnosis way back in 2017, is he/she an autoimmune focused neurologist? Have you received a second opinion or evaluation? Has your bone density been monitored regularly, at his/her direction?

A suggestion for using MC Connect: if you plan to write something for more than a few paragraphs, and maybe you might want to digress to look up something to include, do it off MC Connect, in Word, or Pages, or something on the side, then copy and paste it into MS Connect. I didn’t do this and lost everything I wrote earlier this afternoon when writing to you!

Hope you are feeling at least a little better now, Sue, than when you first posted a few days ago.

Don

Jump to this post

@donnyboy
Oh thank you so much for your message. My Neurologist is an autoimmune dr. He sent me for a 2nd opinion to make sure he was on the right track. University of PA confirmed.
I am startin to get less steroid side effects. I think my body is getting used to it again. I would love to be back to 5mg but that does not work. I am going to get that book you mentioned.
Thank you for the heads up on typing in word first!
Hope you are having a good day today!
Sue

REPLY
donnyboy | @donnyboy | Jun 18 11:36am

@becsbuddy

I am happy to add to the information about treating Clippers with rituximab that was contained in the article "Effective treatment of CLIPPERS with long-term use of rituximab". I emailed the author of the article, Dr. Veronica Cipriani of UChicago Medicine, to ask her whether the patient she described in that 2018 article was still on rituximab today. Dr. Cipriani very graciously responded as follows: "I'm glad you reached out! I am happy to say that the patient from that case report is still on rituximab and doing very well. We were eventually able to take her from every 4 month rituximab to every 6 month, and recently we made the move to dosing every 9 months. Although CLIPPERS is often very responsive to steroids, we usually need an exit strategy because of all the long term complications of steroid use." So this patient of hers has been continuously on rituximab for 11 years! This is very positive news to me, since my neurologist is contemplating moving me from steroids to rituximab.

Dr. Cipriani also shared some information about CLIPPERS patients positive for MOG antibiodies: "I have another patient with a CLIPPERS presentation who is on IVIg and doing well on that. This is because he tested positive for MOG antibodies. I didn't know this when we wrote that case report, but there have been quite a few cases with positive MOG antibodies. Per the Mayo clinic, MOG is best treated with IVIg, so that is why I chose IVIg for that patient. This was written by some colleagues of mine: https://www.sciencedirect.com/science/article/abs/pii/S2211034821001413&#034;.

REPLY
1 reply
Mentor
Becky, Volunteer Mentor | @becsbuddy | Jun 18 12:56pm
In reply to @donnyboy "@becsbuddy I am happy to add to the information about treating Clippers with rituximab that was..." + (show)
@donnyboy

@becsbuddy

I am happy to add to the information about treating Clippers with rituximab that was contained in the article "Effective treatment of CLIPPERS with long-term use of rituximab". I emailed the author of the article, Dr. Veronica Cipriani of UChicago Medicine, to ask her whether the patient she described in that 2018 article was still on rituximab today. Dr. Cipriani very graciously responded as follows: "I'm glad you reached out! I am happy to say that the patient from that case report is still on rituximab and doing very well. We were eventually able to take her from every 4 month rituximab to every 6 month, and recently we made the move to dosing every 9 months. Although CLIPPERS is often very responsive to steroids, we usually need an exit strategy because of all the long term complications of steroid use." So this patient of hers has been continuously on rituximab for 11 years! This is very positive news to me, since my neurologist is contemplating moving me from steroids to rituximab.

Dr. Cipriani also shared some information about CLIPPERS patients positive for MOG antibiodies: "I have another patient with a CLIPPERS presentation who is on IVIg and doing well on that. This is because he tested positive for MOG antibodies. I didn't know this when we wrote that case report, but there have been quite a few cases with positive MOG antibodies. Per the Mayo clinic, MOG is best treated with IVIg, so that is why I chose IVIg for that patient. This was written by some colleagues of mine: https://www.sciencedirect.com/science/article/abs/pii/S2211034821001413&#034;.

Jump to this post

@donnyboy. WOW, you are certainly resourceful! Thank you! I’m sure everyone in this discussion thanks you. Please stay with Connect—we need your brain!

REPLY
Mentor
Becky, Volunteer Mentor | @becsbuddy | Jun 18 4:36pm

If anyone else is interested in reading the article, here is the link:
https://www.neurology.org/doi/10.1212/NXI.0000000000000448
It’s pretty heavy, but we're all smart, aren’t we!

REPLY
suec147 | @suec147 | Jun 24 1:52pm

Hello everyone,
How do you deal with the heat. My favorite time is anything outside, so staying in is not an option I like.
I have been off balance lately. I have been doing my exercising in the pool, easier on the joints. Plus if I fall, it is a soft landing.

REPLY
kristyinoregon | @kristyinoregon | Jun 26 5:41pm

Becky, Please let me know if this went to the whole group, or just to you. I meant it for the whole group, but not sure how it all works yet. Thanks. 🙂

Hello Everyone,
Over the past month or two, I have been reading through all of your posts, starting back with Moderator/Becky’s first post. It has been very helpful to read everyone’s experiences and questions. And, the commonalities, as well as the variation of symptoms that CLIPPERS decides to share with each one of us. I am finally feeling brave enough to share. So, here goes:

I am 58 years old and live in Banks, Oregon (west of Portland). I am thankful to have a loving husband and two adult children. I am a special education teacher. I was diagnosed with CLIPPERS by an OHSU Neurologist on May 2nd, 2024. My Neurologist has had two CLIPPERS patients in a previous practice/another state. I am currently taking Prednisone 50mg (down from 60) and Cell-Cept added 6/10.

Neurologist Visits so to current:
April 16, 2024 - Initial visit
May 2, 2024 - Follow up and Initial diagnosis. Next was scheduled for July 8th.
June 10, 2024 - Was able to get appointment before July due to an increase of symptoms and ER visit on May 28th. Added Cell Cept, advised to see primary physician to get BP meds, and ordered another MRI. Currently waiting for MRI results. Should have in a couple of days.
Next visit will be July 8th.

The lead-time up to my diagnosis took nearly a whole year. It felt very long and so unclear, but we are finally narrowing in. I will provide a separate post to list symptoms and chronicle the one year of appointments, frustration, exhaustion, worry and waiting to get into see the Neurologist, if anyone is interested. Here is what we know and have done from April 16th through current:

Tests Before the First Neurology Appointment:
Vestibular Study/Balance - 2/21/2024 Showed definite problems with brain/eye response to vestibular stimulation that should cause “eye saccades”. My saccade readings were way below normal, from very low to no response at all. The specialist and Dr. B said that either my cochlea was really messed up, or something was happening/not happening between the cochlea and the brain.

MRI - 2/22/2024 and 3/22/2023 - Showed lesions scattered throughout the Central PONS (“salt & pepper” effect visually) and brain inflammation. Cochlea and surrounding areas, both ears, are completely normal/intact, even all of the little “hairs”/nerve receptors. No tumors or masses in the brain.

Tests After the First Neurology Appointment:
Blood work - 4/16/2024 and Lumbar Puncture/Spinal Tap - 4/18/2024
These two tests analyzed together have so far ruled out MS and many other possible culprits.

Second Visit to Neurologist/Initial Diagnosis - 5/2/2024
CLIPPERS (so I guess that makes me a “Clipperhead”)
Prescribed:
60mg prednisone, to reduce by 10mg every 30 days until 20 mgs.
Visit to eye doctor, since I have long-time high eye pressure and take medication for glaucoma. Long term Prednisone can increase blood pressure and eye pressure/Glaucoma.
Take Calcium supplement. Long term prednisone can cause Osteoporosis.
Increase water intake/stay hydrated to help meds work and body heal.
Consider no alcohol, decrease salt, sugar
And, since I had 4 episodes of what I now know to be “Oscilopsia” (temporary loss of visual alignment which cause everything in my vision to slant one direction and look all pixelated for 30 seconds to 1 minute) - NO DRIVING. Wham! Ugh! Oooof !!

She didn’t say I had to take time off work, but my work is not close to my home and I was so worn out, and had no energy/desire to figure out transportation, I took time off work. I ended up being off for a total of 6 weeks. I “returned” (virtually) on May 8th to support the long term substitute with meetings and all the paperwork that happens at the end of the school year for SpEd teachers. I am no on Summer Break. Whew!

I promise my posts will not always be this long. I will now post my Chronical to Diagnosis and my symptoms list. after that, I will put longer stuff in my profile. I haven't set that up yet. Will do. KRISTY 🙂

REPLY
1 reply
View MoreView Less
Please sign in or register to post a reply.