Wendelas treatment update interview
Thank you so much for your support and kind messages after my last update. I wanted to share how things have progressed since my last newsletter entry.
A bit of history prior to the surgery
Since October 2022, I have been looking for a specialist who would understand what was going on in my brain and what we could do about it. I first saw a neurologist in my hometown. When we found through an MRI that I had haemosiderin deposits on my brain and I asked him whether I had superficial siderosis (as I also had severe hearing loss in my left ear), he said “no,” as I didn’t show
any signs of ataxia at that time.
As I did not trust his opinion, I decided to switch to another neurologist in
town, this time with a consultancy in a hospital. As more than a year had
passed, this new neurologist wanted to repeat the MRI I had had and to
add other scans as well, like an MRI-A and a CT myelogram. He wanted
to find the source of the blood remnants on my brain, but no source
showed up. Months passed, and we were on a “wait and see” basis.
Meanwhile, in May 2024, I started showing signs of mild ataxia and my
Romberg test had changed from negative to positive.
He decided to request a lumbar puncture to sample my Cerebrospinal Fluid (CSF). I was happy he ordered that because I knew it would show if I had an active bleed dripping into my CSF, which would be transported towards my brain and cause harm.
The puncture results were positive, which was a great setback for me! The fluid was slightly xanthochromic and showed elevated red blood cells and proteins. We decided to repeat it half a year later, as sometimes this puncture can be “traumatic.” This time, as I had been doing quite a lot of reading, I requested to have the CSF pressure measured as well as the CSF ferritin levels.
It was all bad news: even more red blood cells (my CSF was pink), high CSF ferritin levels (which is a biomarker), and quite low CSF pressure (5 on a scale of 6-20 cm H2O, which indicates a leak somewhere).
By then, I also knew that sometimes the bleeding source cannot be found. After that, the last scan I had was an angiogram. This is a test where the radiologist opens an artery in your groin and injects radioactive contrast fluid to check most internal veins and arteries, making a video of the fluid moving around in the hope of seeing it escape somewhere. In my case, it did not. He concluded it had to be a micro-bleed or maybe old blood from my surgery in 1992.
Then, my neurologist recommended seeing a neurosurgeon. I saw three. The first one could not really tell what to do; he only recommended taking magnesium. The second one told me that I could not have any symptoms from surgery performed three decades ago (it was clear he did not know anything about Superficial Siderosis). The third one decided it was too risky to perform surgery, and it was not clear to him if I had a dural defect or if what was seen on the screen was scar tissue or a regrowth of the 1992 Schwannoma. He preferred to wait and see and repeat the MRI a year later. I did not want to wait
another year.
A friend, who is also a member of The Silent Bleed and the SSRA with (sadly) ample experience in neurosurgeries, suggested that I contact a renowned neurosurgeon in London. He is an expert in complex spinal surgery and has a special interest in Superficial Siderosis. He is also a member of a
multidisciplinary team that includes, e.g., the eminent neurologist Professor David Werring, who also works together with Dr. Michael Levy, a renowned researcher on SS in the United States.
As I had put all my medical information together (scans, reports, tests) in Google Drive, I decided to request an online consultation with there commended English neurosurgeon. When we got online, he clearly showed
he had studied my case in detail. Showing my scans on the screen, he said to me: ‘You have a CSF leak that causes low pressure, this low pressure draws blood to filter into the pseudo-meningocele you have (a post-surgical cyst where a nerve root tumour had once grown). Many people have a post-surgery
pseudo-meningocele, but normally it’s filled with bodily fluid, not with CSF, and it rarely causes superficial siderosis.’ In other words, I had had very bad luck. He also said that I had quite extensive siderosis on my brain and cerebellum, but few symptoms. He also mentioned I had a heterotopic bone ossification (noneuro-radiologist had mentioned that before!). He recommended surgery as I was in good shape and still quite young. The amount of siderosis on my brain worried him. The moment it starts causing a neurodegenerative symptom, that cannot be reversed.
To fast-forward: the repair surgery performed in London on June 21st was a success.
What did the surgery involve?
* Drained and filled with bio-glue and clipped afterwards the pseudo-meningocele
where blood was filtering in—causing my Superficial Siderosis.
* Removed one of my old titanium clips and replaced it with a new one.
* Cleaned out abnormal tissue growth, including a piece of bone (heterotopic ossification) that had formed due to the leak.
* Repaired the hole in the dura using a multilayered approach: stitching the defect, applying three layers of patching, and sealing it all with biological glue—and then repeating that process from the outside of the dura. This "sandwich technique" means everything is now firmly closed and supported (it
was tested through a Valsalva maneuver).
* Insertion of a lumbar drain for four days.
Post-surgery recovery
The surgery lasted five hours (out of six reserved), and I spent three weeks in London in total. Pain after the operation has been intense, which was expected—muscles and fascia had to be cut to access the area. I am currently in my 10th week post-op, and my upper thorax is still a bit sore and my neck easily starts “pulling”; the recovery process takes a long time. The pain is mostly muscular. Fatigue is another issue to deal with.
Although the surgery was a success, there was one unexpected complication: it turned out that the T1 nerve had adhered itself to the dural defect on the inside.To repair the dura properly, the surgeon had to carefully loosen it, move it out of the way, and place it back along its natural path. Nerves do not like to be
touched. The consequence of this is that I lost strength in my left hand, especially in my thumb, index, and middle finger.
A neurophysiologist present during the surgery checked and confirmed that the nerve signal was still intact, which gives me hope for recovery. However, nerve healing takes time, and we will not know the outcome for a few months.
Next step: rehabilitation
I am now receiving functional rehabilitation for my left hand, which includes electrostimulation. Fingers 4 and 5 have improved in movement and strength, but still feel slightly numb. I must admit that I am worried about my thumb, index finger, and middle finger, with the first two being the weakest. It is quite disabling as my thumb can’t yet touch the other fingers, but all the physiotherapists I’ve spoken to say: “nerves take a long time to recover.” In other words, I need to be patient and keep working on my hand and fingers. I have also started treatment with the iron chelator “Deferiprone.” The multidisciplinary team in London considered me a good candidate. This chelation process to reduce the iron on my brain is another long and slow
process.
If you have any questions, feel free to reach out. I am pleased to share my journey.
Warmly,
Wendela
