Steven’s Story

Site created on October 19, 2019

Welcome!. This site is to allow me to keep people updated about my C3 vertebra replacement neurosurgery, its aftereffects, and my recovery.

If this is your first visit, this (rather long) narrative will give you a pretty detailed overview about what I’m going through -- just click "Read More" below! Ongoing dated progress reports are available in the “Journal” link above.

As of Friday 2020-10-02:

I had neurosurgery on Tuesday 2019-10-29 to replace the C3 vertebra in my neck with a plastic substitute, because a "marrow-replacing" tumor had destroyed it from the inside and was impinging on my spinal cord. The symptom that took me in was neuropathy (numbness and tingling) in both my arms and hands. The neurosurgeon said that had I not had the surgery when I did, I was probably a month or two away from the tumor completely cutting off my spinal cord and leaving me paralyzed.

The plastic C3 vertebra substitute is enclosed by rods screwed to the C2 and C4 vertebrae above and below. The removal part of the surgery involved coming in through a surprisingly small incision in my throat and moving aside my larynx, blood vessels and nerves to get at the vertebra and screw in rods from the front. After that, they flipped me over and opened me up from the back to screw in those rods.

At some point either during shortly after the surgery, a clot or plaque was dislodged and triggered a sizable stroke in the right occipital lobe of my brain. This has given me a partial hemianopsia, or “visual field cut:” my brain no longer sees all objects in the left half of my field of vision, independent of which eyeball is doing the seeing. (One easily-graspable effect is that I now have degraded peripheral vision to my left — I might not notice things, especially in the top of my field where the deficit is greatest — but it’s more complicated than that.) The stroke thankfully occurred in a part of the brain mostly concerned with processing vision, not  in an area affecting my language or cognition. Apparently my brain has a lot of “neuroplasticity” --  that is, a lot of interconnected pathways that allow the brain to route around the damage and continue functioning. Given the size of the damage, I am somewhat miraculously not “presenting” typical signs of stroke such as drooping facial muscles, slurred speech, etc.

After surgery I was transferred to an in-patient rehab facility in Los Gatos for a week of physical and occupational therapy before being discharged home on Thursday 2019-11-14.

The in-patient occupational therapies involved retraining my attention (a dicey prospect even before the stroke!) to adjust for my brain’s decreased ability to e.g. notice some visual interruptions etc. without effort. My condition was reported to the DMV, and I will not be able to drive until I retrain and pass a test to verify I can do so safely. The in-patient physical therapies mostly focused on post-stroke issues like balance and core stability. I tested normal for cognition and language.

The post-surgery biopsy of the tumor confirmed that I have multiple myeloma, a cancer of the plasma cells in the blood that affects the marrow. A pre-surgery MRI showed lesions at other points on my spinal cord that hinted at this diagnosis. Myeloma is a treatable but incurable cancer. It is currently manageable to the point where patients routinely have pretty reasonable quality of life for 10 years or more. I have been under the care of a hematologist-oncologist at my clinic in California, who outlined the general course of treatment I decided to follow.

Not all of the tumor was surgically removable, given that it was impinging on the spinal cord and major blood vessels in the spinal column. I completed ten radiation treatments on Monday 2019-12-16 to wipe out the tumor remnants near my vertebra, in anticipation of starting the myeloma treatment itself, which is pretty standard now.

The treatment started in California on Friday 2020-01-24 with multiple three-week cycles of VRd, at-home “induction” chemotherapy:

* a weekly subcutaneous injection of Velcade;
* a daily pill of Revlimid (generic name lenalidomide -- yes, a cousin of the notorious thalidomide), two weeks on and one week off;
* a weekly dose of dexamethasone, a corticosteroid.

These were accompanied by a number of other pills daily, to combat various secondary infections that can occur because the chemo drug also damages the immune system while it’s reducing the cancer…

I had a bone marrow biopsy on Wednesday 2020-04-22 that detected NO measurable myeloma in my bone marrow and confirmed I was responding well to the VRd treatment. In the words of my hematologist-oncologist, that was “excellent news” and meant I was ready to proceed to the next stage of treatment. I chose to have an Autologous Stem Cell Transplant (ASCT), as opposed to continued traditional chemotherapy. (This is not a surgical transplant; keep reading…)

I consulted at the Mayo Clinic in Rochester, Minnesota (I am originally from Minnesota and a brother still lives there) and Stanford University before ultimately choosing to have the ASCT at the University of California San Francisco (UCSF), where I wouldn’t have to travel while SARS-CoV2 is still rampant, and where more of the process would be handled in-patient rather than out-patient.

After some schedule delays, the process began on 2020-08-14 with self-injections of Neupogen, a drug which causes the marrow to manufacture extra stem cells in the bloodstream. After four days of Neupogen injections, I underwent two days of “collection” at UCSF on 2020-08-18 and 2020-08-19. The collection process involved cycling my blood through a centrifuge that extracted the by-then minimally-cancerous stem cells my marrow was producing. (Check out the journal entries for August 18, 2020 and August 20, 2020 for details, as well as the Gallery for photos of the cool technology involved.)

I was admitted on Wednesday 2020-08-26 to the Malignant Hematology wing of UCSF’s Medical Center for the actual transplant process itself, which began that evening with a single large dose of Melphalan -- “good old-fashioned chemo” -- which wiped out as much of the remaining cancer by attacking cells that reproduce “too rapidly.” (This is why chemotherapy causes hair loss, nausea, diarrhea as side effects -- hair, skin, and gastrointestinal cells also reproduce rapidly and are thus affected by Melphalan.)

With the chemo having reduced the myeloma to absolutely minimal levels, on Friday 2020-08-28 (two days after Melphalan), they gave me a transfusion of five bags worth of own previously-harvested stem cells. And here’s where the combination of modern medical technology and human biology gets magical: the minimally-cancerous stem cells put back into my bloodstream started replacing (“transplanting”) my bone marrow with minimally-cancerous marrow cells, which will put the cancer into remission and allow me to start low impact “maintenance chemo” that affords a pretty normal quality of life. Multiple myeloma treated by ASCT typically stays in remission for around 3-6 years at a time, although I’ve heard of people staying in remission longer than ten years. The myeloma will, however, flare up periodically and require re-treatment. It is, however, subject to a lot of ongoing research, so a silver lining is that the treatment of each recurrence can benefit from the most recent treatment discoveries.

I was discharged from the hospital on Thursday 2020-09-10 (after two weeks) to continue recovery at home. My sister Leanne was with me as a caregiver for the first week at home, followed by my brother Eric the second week. They have both since returned to their homes, and I am managing the rest of my recovery on my own.

I am currently on my second stint of short-term disability leave from work, which began with the Neupogen injections on Friday 2020-08-14, and will continue until Saturday 2020-11-14 -- three months total while my immunocompromised body recovers from the damage of chemotherapy and the transplant.

I will also have to have a schedule of vaccinations over the following 24 months to restore the immunity that I received from childhood vaccines.

Although my voice was initially affected by the surgery, I seem to have recovered my normal singing voice. I feel like my top end is still not fully “back,” but it has felt good enough that I have been actively contributing performances to some online cabarets -- including accompanying myself in public for the first time in my life.

SARS-CoV-2 quarantining has both changed and not changed things for me. I was back at work from 2020-01-24 (the end of my first disability leave for the surgery) until I went back on leave for the transplant, starting 2020-08-14. They have been very supportive about my working from home as much as I needed even before the quarantine was extended to everyone, so shelter-in-place didn’t directly change things for me as much as it has others.

I’m in… generally okay spirits about all of this. It has gotten genuinely overwhelming at times. But all of your kind well wishes and comments are appreciated more than I currently have the time or energy to let you all know individually. I’m literally brought to tears multiple times a day by something that reminds me of how lucky I am to be so well-regarded by so many wonderful people. Thank you.

My love and best wishes to you all.

Newest Update

Journal entry by Steven Knight

I had my consultation with my specialist today. She said she is SO HAPPY with the results!

That said, things weren’t quite as good as I thought, because I misunderstood the interaction between the “Complete Response” categorizations and the MRD test result.

So I don’t cause needless worry by burying the lede: Yes, I am in remission. And yes, I am MRD-.

However, I thought that the MRD test, being especially sensitive for the amount of myeloma in the bone marrow, conferred some kind of “super-remission” status. Turns out it’s more complicated than that. (Because: biology!)

Yes, MRD- means I have no measurable myeloma cells in my marrow. And that’s good! When I started a year ago, I apparently measured about 40% of my marrow being myeloma cells. Some people start with a lot more, and plenty do not respond as well to treatment as I have.

But. In addition to plasma cells in the marrow, there are other indicators for myeloma, because it's not just biology, it's complicated biology. (As if there's any other kind.) So MRD- means that I aced one indicator. A very important indicator, sure, but I didn’t ace all of the indicators.

Specifically, another indicator is the presence of free immunoglobulin light chains in the blood. These light chains come in two types in mammals: kappa and lambda.

(Stick with me here.) 

Where there are light chains there are also, you guessed it, immunoglobulin heavy chains, which come in five classes in mammals: IgG, IgD, IgA, IgM and IgE.

Light chains and heavy chains team up to make Y-shaped immunoglobulins, of which there are thousands of types, and which are also known as antibodies -- yes, the things that fight bacteria and viruses in our system, and which we’re all counting on for eventually combatting things like SARS-CoV2.

Myeloma shows up when one of the plasma cells that produce these different kinds of antibodies starts proliferating in the marrow out of control -- that is, becomes cancerous -- and consequently not only starts taking over the bone marrow, but also overproduces the type of antibody it was designed to produce. In my case, the type being overproduced falls in the IgG and kappa classes. It can also start producing too many free light chains.

The number of free kappa and lambda light chains in the blood should not be too high, and the ratio of the two should also not be too high (or low). I have a few too many kappa light chains showing up, and the ratio is also a little higher than the normal range.

My specialist said this isn't problematic and doesn’t change my prognosis, but it does knock me out of the “stringent Complete Response” designation. But hey, if they're out of "sCR" on the menu, I’ll gladly settle for “CR” with a side order of “MRD-”...

My specialist also confirmed that my PET scan showed that places where the myeloma was previously present -- especially my neck, where the tumor was -- are now clear of myeloma. Hooray!

(If you’ve never heard of a PET scan before, you’re in good company. I hadn’t before the first of the three I’ve had now. A PET scan involves getting an injection of radioactive sugar that spreads throughout your body and gets absorbed by cancer cells, after which you get scanned by an MRI-like machine to create an image of where the cancer is. There are other things besides cancer that it can scan for, depending on the substance injected. The scan itself isn’t bad, it’s much quieter than an MRI. The daunting thing about the process, though, is you have to wait for about 45 minutes in a darkened room for the sugar to spread through your body and get absorbed, and they tell you to try not to think because that will increase radioactive blood flow to the brain and too much radioactivity near your brain isn’t good. Well! There’s certainly no better way to get someone’s thinking to calm down than to warn them that thinking is dangerous!)

As expected, my specialist recommended a pretty standard maintenance treatment of 10mg Revlimid, in a cycle of three weeks on, then one week off. I won’t have the Velcade or dexamethasone that were part of the induction chemotherapy. (And yes, that’s the dexamethasone that everyone got really concerned about Trump being hopped up on…) This will be under the care of my local clinic hematologist-oncologist.

I’ll be back at UCSF in three months, though, to start getting my childhood immunizations again.

In the meantime, I also have to follow up with my UCSF neurologist, once they have received copies of my brain MRI images, to assess what’s going on with the stroke.

And, apropos nothing else, it might amuse you to hear that my hair is, at the moment, growing back surprisingly soft and fuzzy. We'll see if that keeps up.

As always, thank you all so much for your interest and support.

Much love to you all,

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