Treatment Journey

Surviving The Neurology Ride

The Patient Experience With The Neurology Ride

A visit to Gary’s neurologist often brings to mind when you step up to ride the tallest, most twisty ride in an amusement park. Your ride begins by slowly inching along, anticipating a thrilling experience with just a hint of fear. Then, the bottom falls away, and down you go. Sometimes the ride is exhilarating, everything you could hope for. Often, the anticipation and hype don’t live up to your expectations.

Do We Expect Too Much?

Gary just finished with his last neurology appointment of 2018. We’ve had an outstanding year, as far as finding solutions to some of his problems. We’ve been lucky to be assigned an indulgent doctor who listens to my oft-times long-winded list of concerns. I am aware I can be a high-maintenance caregiver. However, without a little direction and prompting, Gary rambles through triage sessions.

Why does it always feel like it’s never enough? Superficial siderosis is a slowly progressing condition early on, but there seems to be a point of acceleration that is alarming to witness. 22 years passed before symptoms became disruptive to our life. Now every new event seems magnified.

Appointment Day

You arrived at your appointment with a list of changes, and it hits you in the first few moments, your rotating resident hasn’t bothered to read the file very thoroughly.

“So Mr. Daniel, you’re here about headaches?”

” No, he’s here because he has superficial siderosis.”

“You have a problem with your lungs?”

I am now silently slamming my forehead against the imaginary wall in my mind.

Tracking The Changes

Fast-forward ten minutes to when I’ve finished with my standard lecture on superficial siderosis. We begin to discuss Gary’s changes. Now from a medical standpoint, I understand not every symptom change is critical but I’ve learned it’s best to track these changes in our healthcare providers notes. This way, if circumstances do become extreme there is a trail for each new resident to follow. Every symptom change we brought up was met by a look of wonder. That too? He decided he had better take a moment and start reading.

Assesing The Changes

Gary’s short-term memory has been rapidly changing for the worse in ways that have been painfully obvious to those around him. We met a couple in the grocery store we’ve known for years. Gary couldn’t remember his friend’s name. He knew his face, and he knew he should know him, but his name was gone until I prompted him, “Hi Bob, where’s Linda?” This glitch stood out because for the first time it wasn’t short-term memory that failed. Gary’s long-term memory has always remained extremely good.

Gary was given an in-office Mini-Mental State Exam (MMSE). Gary has been given a lot of these type of tests over the years. Most often they use the Saint Louis University Mental Status Examination (SLUMS) or Montreal Cognitive Assesment (MoCA). My complaint? The MMSE is not sensitive to mild cognitive impairment. Gary has zero problems copying blocks or clock faces. Scoring a short-term memory exam seems extremely skewed when the person administering the exam provides repeated word prompts and clues. I undertand this is standard practice but the patient is not usually allowed to be scored with giving a correct response.

Resident: Mr. Daniel, can you repeat the five words I told you earlier?
Gary remembers two words.
Resident: The next word was a color. It’s bright. The color of a fruit you eat.
Gary: Red?
Resident: Can you name me the flower I said?
Gary: Yes, the word was “flower.”
Resident: No Mr. Daniel, it was a flower. It’s white. Children collect them and make chains.
Gary: Daisy?
Resident: Perfect Mr. Daniel! Can you repeat the last word? It’s a place you go every week. A place you go to pray.
Gary: ?????????…………………

Gary was never asked to repeat the names of the three animal pictures he was show earlier. The test was scored as three correct answers. In the four years since Gary’s been taking these type of in-office tests, he hasn’t scored higher than a 21 in recent years. The resident scored Gary with a 27/30. How nice Mrs. Daniel! It looks like your husband’s memory is getting better!

Wonderful. I’ll be sure and tell Bob.

Two Steps Forward

Our visit with Gary’s neurologist was a little more productive despite the first year resident’s rosy assessments and pencil whipping of unperformed tests. Gary will be scheduled for a GI motility test soon. His visit with the otolaryngologist suggested his increasing acid reflux and weekly bowel movements were the results of poor gastric motility. His increasing lumbar pain was moving into the intolerable stage so physical therapy will be scheduled for three time a week. If physical therapy can’t provide some relief, then a pharmacological route will have to be explored.

The walker is moving up into the line-up more and more with Gary’s worsening balance and gait. His PCP has an appointment scheduled for us to pick-up a wheelchair this January. At this point in time, the wheelchair is more for cardiac-based endurance issues rather than balance.

One Step Back

Other negatives for this year besides the balance and ataxia issues, in spite of two different models of hearing aids this year, his hearing is getting markedly worse. His audiologist doesn’t want to visit the cochlear implant option unless it’s the last resort. Even though Gary lost his sense of smell along with the ability to distinguish flavor a long time ago, his five basic tastes that come from his mouth and tongue are now disappearing. Salty is still pronounced as is sweet but the others have begun to fade. Our most pressing concern is getting his lower back pain under control. Sitting, standing, or riding, the pain is always there.

The High Points

Some positives for 2018 included finally getting Gary’s horrible headaches under control. He goes to the pain clinic quarterly for Greater Occipital Nerve blocks. Gary is injected with a mixture of Marcaine and Kenalog four times a year. He still has headaches, but they are more typical in duration now. The traditional Chinese herbal medicine is still keeping the burning pain in his feet under control though he still suffers from ice cold extremities at times.

Neurology is now transitioning Gary into a status of stable. He will return to neurology every six months in 2019 while continuing to make the rounds between multiple specialties.

Here’s hoping for more positives in 2019.

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Rori Daniel

Living With Superficial Siderosis began as a way to keep family and acquaintances updated after my husband Gary was diagnosed with Superficial siderosis in 2014. In 2019, Livingwithss.com became a partner in the Superficial Siderosis Research Alliance. Together our alliance has expanded into research, advocacy, and patient education. Rori Daniel, Editor livingwithss.com,

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4 Comments

  1. My husband had a cochlear implant in 2011 for which he got very little benefit. We did not know at the time that he had superficial siderosis. He has also lost his sense of smell. Sometimes ice cream tastes salty so he doesn’t ever eat it anymore. Otherwise, he says everything tastes like sawdust. He has a voracious appetite for chocolate, probably because he remembers he liked it.

    Regarding the implant: he can hear some sounds but the speech reception is extremely poor. Communication is difficult and I have to write most things down. Sometimes he reads lips a bit but it is inconsistent. Since his MRI last December, we have been waiting for a new outer device for the implant and I don’t see any drop in his already poor communication skills since he has no aid. The implant never worked well for him and the hassles we went through to get the magnet removed to get the MRI done were tremendous. Now I wish they never put the magnet back because he says he refuses to go through that procedure again.

    I am telling you this because what I read about SS is that it attacks the 8th cranial nerve and if this is damaged, the implant won’t work or won’t work well. I know others with cochlear implants that have great success stories but they do not have SS.

    Once you have the implant done, it is much more complicated to get future MRIs done. I would think long and hard before doing this.

  2. This could be me writing about Paul, every symptom is the same, except Paul is now in an electric wheelchair, has no hearing, due to a mri, that damaged it….once you go to cochlear inplants, you can never go back to aids, and they often fail due to the nerves dying, that there attached to…pauls short term memory is near non existent, but long term is still good…good luck..xx

  3. My lower back pain is terrible too, but I thought it was because of an old injury and that the SS just made it worse.
    Did Gary have a prior injury in his lower back?
    Has anyone ever considered the chicken pox virus one of the factors that weakens the natural defenses against getting SS?
    I see a new neurologist the end of January , being that I’m just riding this thing out, not traveling to the city will be a plus to me.

    1. When Gary had his original surgery to remove the tumor in his neck a leak formed. Spinal fluid would make a large bubble at the back of his neck. His surgeon attempted to reduce the pressure by spinal tap into his lower back. Due to a defect in the needle it broke off when it was in Gary’s spine. They had to take him back into surgery to remove the needle. He also has very slight disc problems starting at the same area so there may be several reasons/causes of his pain. They have already tried a muscle relaxer but that was completely useless.

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