Unexpected Lessons

We just returned home today after a weekend retreat with the folks at Michigan Parents of the Visually Impaired (MPVI).  Everybody in the family had a fantastic weekend, and we all came away with new friends along with new energy toward sharing knowledge and improving the world around us.

This is the first time since 1996 that Brian and I have done anything with the “blind community.”  Back then, Brian was still working at the IRS in a job that he got through a training program at Lion’s World Services for the Blind in Little Rock, AR. Understandably, there were a lot of other blind people working in that job with him, most coming from the same program, and they tended to hang out together.  We started bowling with the local blind bowling league and did that for several years, even heading to the national championship one year.

Since leaving the Washington, D.C., area, we had not gotten involved again in anything blind-oriented, other than staying in touch for a while with old friends Brian had from TSB and Criss Cole.  We never felt the need for support groups in Texas, even while Brian was using Commission services during his 2002 layoff.  Once we got to Michigan, we met one blind friend here very quickly (which is a pretty cool story of its own!), but again never felt the need for a support group.  Eventually we had blind children (unexpectedly!), but I let other people’s experience color my judgement and still didn’t reach out much.  My local friend told me stories of how little support she received during her time in the schools and how many blind people she knew who were not independent.  My mother-in-law’s experiences with “parents of the blind” support groups involved sheltered kids with smothering parents who treated their children like helpless china dolls.  Between the two, I didn’t feel any need to connect with others, feeling safer and more comfortable with Brian and my friend, and our online friends from the Blindhomeschooler list.

We were missing out.

All this weekend, we encountered parents who were encouraging their kids to do things for themselves, to walk independently, to give them canes at a young age, to let them climb rock walls and ride on zip lines. We met some of the young counselors the kids had at Lions Camp.  We met some absolutely awesome organizers who really do want the same things we want for our children: independence, self-confidence, competence as blind people and as people in the world. What they were lacking, perhaps, was exposure to more competent blind adults (like Brian and my friend) as role models, along with some different ways of doing things, but their hearts are taking them in the right directions.  Whenever we mentioned the different ways we do things, like buying our own canes, learning and teaching Braille ourselves, all the things we think of as just part of life and homeschooling, we found other parents eager to hear a different story.

Lesson learned: Don’t just go on other people’s judgements about people or groups.  Go find out for yourself.

One of the activities offered Saturday morning was “blindfolded cooking.”  The idea was to give sighted parents some idea of the obstacles that blind people face when doing everyday tasks.  It was a lot of fun.  I’ve always enjoyed practicing “being blind.”  I got to help some people out with suggestions and tips, and I got some help of my own from the older blind kids who participated with us parents.  I was a little disappointed that the set-up was not adapted in any way, so that it more mimicked being dropped into a sighted stranger’s kitchen rather than the way a blind person would manage their own kitchen, but it was fun nevertheless.

One thing bothered me the rest of the weekend, though.  The cooking activity spanned two different rooms, a large dining area and a big kitchen.  I was very careful in orienting myself once the blindfold was on, and had no trouble during the first hour or so, moving between tables and to my coffee that I had stashed in a windowsill.  Then it came time to head to the kitchen and use the stove.  I made my way to the hall door I had already found, found the entrance to the kitchen, and started trailing myself around it counterclockwise.  I wanted to orient myself to the entire kitchen, and I could hear that folks were congregating to my left as I entered.  I followed along the counter that was open to the dining room, the counter along the wall, the double oven at the end of the counter, the closet next to the oven where extra tables were stowed.  Then somebody came and led me to the stove so I could start my cooking (I was roasting pumpkin seeds in a cast-iron skillet, for a spiced nut mix).  After that, the kitchen cleared out a bit, but I got thoroughly confused and disoriented.  I felt like the stove was not where it should be, and I had trouble finding the door when it was time to leave the room.  After I finished in the kitchen, I went back into the dining room, where I was led to another table to work on a second baking project (smores cups, yum!).  Once again, I became disoriented, even though I had been navigating this room well previously and pretty much knew where I had to be in the room.  I floundered when I went for my coffee again, and floundered some more when it was time to put the cups in the oven.

All that floundering is what has puzzled me since.  I thought and thought and thought, and I think I figured out what the problem was.  I was being led.  First in the kitchen, interrupting my trailing, and then again when I left the kitchen.  When I was led across an open floor, I lost my bearings.  Some of this may be a matter of practice, I’m sure, but it reminds me of a principle that I follow and believe in for more general parenting and education: children and adults learn things better when they do it themselves, instead of having it done for them.  When I was doing all my navigating myself, I might have been slow, but I was getting there, and I was keeping my own mental map straight.  When somebody else did it for me, I didn’t learn anything from it.

I think it is important to remember this lesson when dealing with our children, especially our special needs children.  The impulse is so often to step in and do it for them, to help them.  We don’t want it done poorly or slowly, we don’t like to see them flounder and appear slow and helpless.  Every time we take over, though, we are depriving them of an opportunity to learn.  Even in something as seemingly simple and obvious as providing a sighted guide to lead a blind person across an open floor.

Unspoken Rules

A conversation with some other moms with spectrum sons reminded me of my early experiences in the workplace.  My first real full-time job was working as a secretary for an intellectual property law firm outside of D.C.  I started out as a temp, then got hired full-time to be the primary secretary for two younger lawyers as well as the backup for the entire firm.  For the first few months, I was regularly in the office until 7, 8, even 9 at night, trying to get all of the days’ assignments completed.  I had been told that my job was salaried, with no overtime, but I had also been given a logbook so that extra hours could be taken as comp time.  I dutifully filled it out every day. Poor Brian would get off work and be waiting for me to finish and go home.  Finally, the office manager took pity on me, and explicitly told me that finishing all of my assignments every day was not expected of me.

Yes, I had to be told that it was okay to leave some work unfinished for the next day.  I’ve had problems with that in my personal life, too.  I use lists and routines to help keep myself in order (or else I’ll do things like forget to brush my teeth or wear a bra!), but then I get stressed about not finishing everything every day.  It’s kind of a “damned if you, damned if you don’t” situation.  In the case of work, though, I had just gotten out of school, with the expectation that an assignment is to be done that day, unless it explicitly has a later due date set.  I just assumed the same was true in the workplace, and it never occurred to me that there might be unspoken rules here that were different.

It never occurred to my supervisors that I might need to be told those unspoken rules.  Rules like:

  • Don’t do your job too well.
  • Don’t wear a shirt with the shoulders cut out.
  • Don’t try to take any personal days too soon.
  • Don’t keep your desk too neat.
  • Don’t try to help the firm if it falls outside your job description.
  • Don’t try to learn new things if the skillset is above your pay grade.
  • Don’t make jokes.
  • Don’t complain loudly when your food is eaten out of the break room refrigerator. Especially not by mass email.

I don’t know if those are all the rules, or if that’s exactly how they go, but based on the times I got in trouble, got lectured, etc., those are the rules I broke.

There are other places where this has tripped me up.  At least in the workplace, my first office manager in particular learned that I needed to be explicitly told what I’d done wrong when I broke one of the unwritten rules.  There seems to be a whole set of unspoken rules regarding interactions with other women and other mothers that trips me up on a regular basis.  I’ll get shut out of groups without the slightest idea what I’ve done wrong, and nobody ever comes back and says, “Look, you can’t do [x, y, z] around us, and that’s why we’re now going to shun you and never speak to you again.” They just skip to the shunning.  Kind of like high school.

Things are getting better. The neurodiversity movement, TV shows like the Big Bang Theory, the push for mainstreaming autistics, even the spread of the goth subculture, all of these have contributed to a little more understanding in society at large of those of us who don’t get or follow those unwritten rules.  That could just be the subset of our society that I currently move in, which admittedly has a high density of geeks, nerds, and spectrum issues.  I’d like to think, though, that somebody getting thrown into the workplace as an aspie today, with a diagnosis and an understanding of how they see the world differently…I’d like to think they’d have an easier time than I did.

Regrets

“Regrets. I have a few.  But then again, too few to mention…” — Frank Sinatra

I’m going to mention some, though.

I regret not being more forceful in trying to figure out what was wrong with Caitlin’s and Jarod’s eyes before Kender was born.  Ever since Jarod was a baby, and his eyes seemed to be going down the same path as Caitlin’s, we knew there had to be something genetic going on.  Obviously Caitlin’s eyes weren’t just the result of ROP and cerebral palsy.  I remember in the two weeks before Kender was born, we had an appointment with our pediatric ophthalmologist.  I sent Brian, both because I was in pre-labor and not really up to the all-day trip required, and because I thought Brian, with his better communication and people skills, might finally be able to wring an answer to this question out of her.  Even Brian could only get, “I don’t know.”  I still don’t know how, after more than 2 years of looking into Jarod’s eyes every 4 months, she never once saw that he had a retinal fold blocking his bad eye.  There are so few pediatric ophthalmalogists around here, though, that we never sought a second opinion.

I regret trying to patch Jarod’s eyes.  Although we now know that he has stage IV FEVR in his bad eye, and that it would never be able to just magically get stronger, at the time our ophthalmologist said it was just lazy eye.  We tried patching, but he fought and screamed and hollered every time we put the patch on.  (Obviously…we were effectively blindfolding him!)  Since just patching didn’t seem like it was going to do any good with a child who was essentially throwing a hissy fit the whole time, we tried using atropine drops, which dilate and blur the vision in the good eye to force the child to use the bad eye.  Remember, that bad eye had Stage IV FEVR, with a retinal fold that covered more than half of his lens capsule.  That eye was blind. But we put drops in his good eye to force him to use the bad one.  I know that we were only operating on the knowledge we had at the time, but dear gods I regret that more than anything else I did to my children in the name of medicine.

I regret the two surgeries that Caitlin had before receiving her FEVR diagnosis.  They kept insisting that she just had some strabismus because of her cerebral palsy, and if they just went in and snipped a nerve or a tendon here and there, they could straighten out her eye.  Caitlin has Stage III FEVR in her bad eye, with a peripheral retinal fold and a dragged macula.  These days, she is still losing vision in that eye, with active disease along the fold that is worsening the dragging and decreasing her vision.  The first surgery she had didn’t do the trick, so they wanted to do a second one, and I went along.  Again, I was only operating (no pun intended) on the knowledge I had at the time.  But those unnecessary surgeries are probably near the top of my regret list.

I deeply, terribly, horribly regret that I waited so gods-damned long to force the issue of Kender’s eyes, and his autism.  With his eyes, in his bad eye he has had a vitrectomy, a retinal membrane peel to pull a fold off his lens capsule, and a lensectomy after a cataract developed in response to the first surgeries.  Then the disease in that eye continued to progress, and he blew the pupil out (uveal ectropion).  Then he developed a bleed, along with glaucoma.  The blown pupil removed his ability for his pupils to contract in response to light.  If you’ve ever had your eyes dilated for an exam, and then walked outside into the daylight, imagine that NEVER GOING AWAY.  That’s what he lived with for 2 years, from the time he was 18 months until he was nearly 4, before I started investigating pain as a contributor to his developmental delays.  We finally got him started on atropine drops a year ago.  Atropine is a dilating drop, which seems counterintuitive (after all, he’s permanently dilated).  However, the problem with his bad eye is that, even though the pupil is blown and can’t contract, the muscles are still there, and they still respond to the light stimulus.  Even though that eye is now effectively blocked by the remnants and scarring from the last bad bleed, it still responds to light, forcing the muscles of the pupil to try to contract.  But they can’t, and so they cramp.  He was in constant pain. The changes we saw in him once we started the atropine drops were nothing short of phenomenal.  It was like he’d been stuck as a baby, and suddenly he started to progress.  He went from constantly being in a little ball, not interacting with anything or anybody, to finally exploring the world.  Gods, do I regret those 2 years.  What did we cost him?  What pathways in his brain might have solidified in immaturity, or atrophied from disuse?

I regret that it took so long to get Kender’s autism diagnosis.  Part of that was the confounding factors of his blindness, and the delays caused by the chronic pain.  However, I suspecting the autism from the time he turned three, and I took him to the psychologist who originally diagnosed Caitlin with Asperger’s.  That…that man…had the gall to look me in the face and tell me that my child was just stubborn.  That it was impossible for an autistic child to show affection for his mother, or to play games, or to explore a room.  I wish I could face him now, although I don’t know what I would say.  We have the diagnosis now, which gives us new tools to work with in helping Kender.  But that man…I trusted him, because he first looked at Caitlin and said, “No way,” but then he tested her and admitted that she was absolutely on the spectrum as Asperger’s, that her condition was initially clouded by the fact that she was a triplet with NT triplet siblings.  I trusted him, and so even though I felt in my heart that he was wrong, I waited almost another year before seeking out another opinion.  A whole year wasted, waiting, when we could have had his dog already by now, we could have known.  Maybe not my biggest regret, but it still makes me mad.

Yeah.  I have a few regrets.  Maybe some of our doctors should have them, too, although they probably never give me and my children a second thought.  One day, I know, I’ll get over it.  The PTSD from the triplets’ birth has faded after 13 years, although it is not gone entirely.  This will pass, too.  Someday.

FEVR Conference

Better late than never. It’s always so crazy around here. Here’s my summary of the FEVR conference we had here in Michigan last month:

If you’ve been reading this blog regularly, you know that our ophthalmologist, Dr. Michael Trese, is the world’s leading pediatric retina specialist. People come from all over the world to see him and his colleagues, Drs. Capone and Drenser. Last month, their office, Associated Retinal Consultants, together with ROPARD, held a conference for families affected by Familial Exudative Vitreo-Retinopathy (FEVR), the disease causing the blindness in our family. It ended up being a two-day event, with social opportunities the night before and lectures given by the doctors on Saturday.

For many (most?) of us there, it was our first opportunity to actually meet other people and families with this disease. FEVR is so rare, most doctors will see at most one case of it in their entire careers. Our local low-vision specialist flat out told us that he didn’t even study this disease in school because he never expected to see it. Many of us have been through misdiagnoses and mistreatment from our local “specialists” who just didn’t realize what they were dealing with. The opportunity to share our stories was priceless.

Friday night, ROPARD helped organize a dinner at the hotel we were staying in. We all ate in one of the hotel’s conference rooms, and then there was time to hang out and chat. Paula from ROPARD brought some items to display, special toys and things. One blind gentleman she brought with her brought a Mountbatten brailler for us to check out, and I brought a box of Braille and tactile books. Thanks to how the child-care situation ended up not working out, there weren’t many children with us, but there were a few.

I was a little embarrassed, because people kept coming up to me and saying, “Oh, it’s so nice to finally meet you, you always seem to have so much information!” Of course I’ve met people online before, but usually just one or two at a time. This was a little weird!

One thing happened after dinner Friday evening that was just so amazing, I keep telling everybody I know about it. I’m always a big advocate for mobility in young children, and I kept telling everybody I spoke to that they needed to get canes into their children’s hands, no matter how young, no matter what the schools or anybody else said. While we were standing around chatting, a little boy came up to Brian. The boy looked to be about Kender’s age, with a little better vision than Kender. He grabbed Brian’s cane, curious, and I whispered to Brian to let the boy have it. This little boy, who had never held a cane before, took off with Brian’s cane around the room, using it in a completely appropriate manner, finding all sorts of obstacles with it. You couldn’t ask for a better demonstration of how natural cane use is for these children when they start young, and how easy it can be.

Later, a bunch of us parents got together in the hotel lounge and hung out until the bar closed down, chatting and sharing stories. Brian got to talk with some other fathers who also have FEVR, although none of them were blind and all found out about it as adults. There were other families there with 3 and 4 children with FEVR (although none with six childrens altogether!), and even a couple of families from Michigan! Max Bodnar and Brian really hit it off, and started making plans to set up another “conference” next year, mostly as a get-together for the parents and children this time.

Saturday was the official conference. The morning was taken up by presentations by Drs. Trese, Capone, and Drenser. There were two major revelations presented. The first is that they no longer believe they can identify FEVR patients with a routine dilated exam of the retina. Some Stage I patients have been slipping through this screening, only to show leakage or exudates when given flourescein angiogram. Because of this, they now are planning the fluorescein angiogram to be the standard diagnostic tool, and will be using this to screen family members once a patient has been diagnosed. This will be easier than it has been for older people (say, over 10 years old) because the doctors are acquiring a new machine that will make it easier for them to do these angiograms in the office and still get a good look at the periphery of the retina. For us, this news means that Brian’s parents need to get rechecked, especially in light of his dad’s newly-diagnosed macular degeneration and cataracts, which could be exacerbated by FEVR. The doctor down there in Texas does not do angiograms, so they will have to wait until they can come up to see Dr. Trese to get that done, but they will be able to get their blood drawn so they can be included in Dr. Drenser’s genetic study, and that should show if they are carrying the same FZLD-4 mutation Brian and the kids have. It’s important for us to find this out, because if either of his parents turn out to be carriers, Kevin’s family will need to be checked.

The second big news was that the doctors are having much better “success” rates over the last two years with preventing rapid deterioration in their FEVR patients. They have taken a very aggressive approach to laser cauterization of the retina, lasering areas with exudates, blood leakage, avascular retina, and capillary dropout. We’ve seen this in our own family, where it seems like somebody gets fresh laser every few months. This aggressive treatment has resulted in many fewer cases of what they called “surprise” leakage and exudates, where previously-quiet eyes suddenly showed massive changes on the next exam. This could be very good news for children like Tamara, Caitlin, and Jarod, who may now be at less risk than we previously thought of losing their remaining vision.

Finally, Dr. Drenser gave a very interesting and detailed description of the current understanding of the genetics and chemistry behind FEVR. There are several different known mutations that cause FEVR, as well as more that are unidentified. All the different mutations disrupt the same basic chemical process linked to the growth of new blood vessels in the retina. This process is very complex, with many known receptors at the beginning and end of the reaction chain and what Dr. Drenser called a “black box” of reactions in the middle that have not yet been fully identified. Messing up any link in the chain leads to the emergence of FEVR. Having so many different places where things can go wrong means that FEVR shows all of the known inheritance patterns (dominant, recessive, and sex-linked), and because the expression of these known genes can be up- or down-regulated by other unknown genes, the expression of the disease in two people (say, siblings) with the same mutation can be very different (like Tamara and Kender). It was really fascinating to see how much they know (and how much they don’t yet know) about how this disease works. We also got to get a sneak peek at the new genetics lab that the doctors have set up this year for further research into FEVR and other pediatric retinal diseases.

Saturday afternoon was given over to a presentation by Dr. Ference, a low-vision specialist who works out of the same building as Dr. Trese et al., and who is very highly recommended. She may be highly recommended, and I’m sure there are some advantages in her style, but Brian and I personally were very disappointed in her presentation. It came off as a 2-hour advertisement for herself, and several times she gave the impression that she could effectively “cure” somebody’s blindness through eye exercises! Many of the things she said nobody else does were things all of our ophthalmologists and low-vision specialists have done, and many of the “treatments” she prescribes are things that any child with an active parent and an engaging environment will do naturally. I’ve got notes from the presentation, and I’ll break down my criticisms in another post, but Brian and I (and really our TVI, who came along Saturday for the fun of the presentations and learning) came away very disappointed.

All in all, though, it was a wonderful 24 hours, and we can’t wait to do it again. We spoke with Dr. Trese, and we will be working with the surgery schedulers to set up a week or two next summer that can be set asides for the practice’s FEVR patients. We’ll get everybody out-of-town to schedule their annual/biannual/whatever checkups for that time, and over the closest weekend we’ll book a group of rooms at the hotel and plan some family events. We want it to be a kid-friendly get together this time, so all the kids can meet and talk and socialize. As parents, we got a lot out of just that aspect of it, and we think the kids will, too.