Happy Solstice, Beloveds!
Zoe is blind, that we know, and at any other time prior to this point it would be safe to say that she would be blind for life. But with technology and scientific advances happening almost as fast as they can imagine it, there is a chance that Zoe may get to see some day.
I had heard of people traveling to China to receive stem cell therapy for optic nerve regeneration, but not only are the treatments prohibitively expensive (at least $20K per treatment, and they recommend multiple treatments), but the results they have are limited. It all seems a bit too experimental and unregulated to me to even consider considering it.
Closer to home I discovered the Schepens Eye Research Institute online and made use of their "patient liaison", Richard Godfrey, to get educated on what they know about developments in optic nerve regeneration. The following is some text extracted from literature he sent me.
Regenerative Research:Well, that all sounds promising, eh? But it's not only optic nerve regeneration that might allow Zoe to see in the future. Technology may light up the darkness for her.
As our knowledge of stem cells has expanded, so has the breadth of our research. A few short years ago, all the focus was on embryonic stem cells and research was limited by the ethical issues and related federal funding ban. Since then, we also have access to progenitor cells, adult stem cells that also have great potential without some of the limiting factors of embryonic stem cells mentioned above. Now, we have discovered that the human body has already existing, dormant stem cells in the central nervous system (CNS) which includes the spinal cord, brain, optic nerve and retina. With both embryonic stem cells and adult stem cells, additional research needs to be done to both develop consistent sources of stem cells and develop a surgical or other stem cell delivery technique. This new approach of activating already existing, dormant stem cells may eliminate the need for these added steps. In addition to knowing that these cells exist, we also now know the molecules that are responsible for that dormancy and we have identified the drug that can activate them. This drug then is likely to become the foundation of a new drug that will both activate and coax these cells to become the kind of cells needed in our goal to restore vision.
In optic nerve regeneration, we know that there are three barriers against regeneration we need to overcome in order to develop a therapy. We already know that the original drug will overcome the first barrier (dormancy) and the second barrier (scar), but the third barrier still must be overcome. This barrier comes from the proteins of the myelin (sheath covering the optic nerve) that send signals preventing regrowth. So the goal is, using the original drug as a foundation, build a new drug to overcome all the barriers safely and reliably.
At the Schepens Eye Research Institute, an affiliate of Harvard Medical School located in Boston, our regenerative center, The Ocular Regeneration Research Center, is made up of 3 separate laboratory teams exploring the great potential of stem cell and gene therapy technologies. The goal of all the labs is the repair/regeneration of the retina and optic nerve. Both are made up of nerve cells, which share the same barriers to regeneration that we are working to overcome. The real strength of Schepens' regenerative research is these 3 labs, 3 research teams, with varied approaches, working collaboratively, are making the idea of vision a reality in coming years. It is a very powerful combination.
Some of the group's individual accomplishments include:
- Dr.Feng Chen's first ever regeneration of the optic nerve in mice
- Dr. Michael Young's years of transplanting brain and retinal stems cells into mice and pigs in preparation for the next step, human retinal damage repair/regeneration
- Dr. Kameran Lashkari's novel, new discovery of adult progenitor cells from the retinas of premature babies that seem to migrate to damaged optic nerve and retina
- Dr. Chen's discovery of the mechanism to reawaken already existing, dormant stem cells in the retina, optic nerve and brain
- Dr. Young's discovery of the molecule in stem cells that is the key to integrating transplanted nerve cells into damaged tissue.
The Brainport Device is an amazing doo-dad in development that allows blind people to actually experience vision in the brain via the nerves on the tongue.
Say what?
The article (linked above) can explain the workings of the device better than I can, but remember that it's not really the eyes that see, it's the brain that sees--the eyes just give it the information it needs to experience vision. The Brainport device simply gives the brain the information it needs through different nerves. It's all very heady stuff, but I am just so impressed with the brain that thought of this whole concept in the first place.
For those of you who'd like to see the unit in action, check this video out:
(And, speaking of videos--check the YouTube link for about five recent video uploads of Zoe stuff.)
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On the home front, we're still all doing really well--so nice to be redundant on that subject!
We took Zoe in for a year-post-surgery-check-up with Michael Cunningham (always a pleasure to see him), and he was well satisfied with Zoe's progress. Recent CT scans showed definite signs of bone remodeling in her skull, as well as a gradual evening out of her bumpy-headed appearance. He felt like we shouldn't even consider the idea of a follow up surgery until Zoe's about 5 years old (as opposed to the usual 3 years of age for most craniosynostosis patients); he just felt like she's been through too much already and that another procedure so soon could 'break her".
We're fine with that approach.
Zoe also had a check-in with her transplant team at SCCA with accolades on that front as well. The extensive labs they drew showed that she "has an immune system" and that it is more robust than most patients this soon after transplant. To be sure, we still have to take care not to expose her to sickness as she is immunocompromised compared to the average person, but good news nonetheless!
Zoe is thriving on the whole food purees we are feeding her fresh from the Vitamix through her G tube. Although certain to be filed under "To Much Information", her poops are finally normal for the first time in a year, making it lovely for her not to have to suffer awful, chronic diaper rash (and for us not to have to deal with diarrhea all day long). Although concocted to meet supposedly all of her nutritional needs, the formula she was on for 9 months simply wasn't agreeing with her as much as this whole food mix we're making. Go figure.
Though she's entirely tube fed, Zoe's recently discovered that she absolutely LOVES crackers. Not to eat, but to suck and lick and taste. Her official first word is "Keh-Keh" (cracker) and she asks for them all the time. I mean, incessantly. First thing when she wakes up (at 5am) in the morning, and pretty much all day until she goes to bed. Crackers have supplanted the brief mommy obsession she had--which is both a relief and a "Hey.....! Really...?"
Really.
Jeff and I are good, so good. To be sure we're dealing a bit with cabin fever, but that's a sickness we are happy to manage. I'm back to learning Braille after a long hiatus; Jeff's teaching himself Beatles tunes on the piano. This Christmas is very low-key--no presents, no decorations, no fuss. We will spend time with family both from in and out of town, so the gathering of the Newton-Cormey-Rapp-Nielsen-Bell-Connor clans will be a good (and loud) time.
Zoe will add to it with her screams no doubt--she's been perfecting them and I'm looking forward to showing them off. Oh, and her cuteness, of course.
