Parent of a Child with Albinism

Lyra had an appointment with her pediatric ophthalmologist this month, a check-up that was originally scheduled for the end of September. Several weeks ago, we lost one of the lenses from Lyra’s glasses. After over a week of searching, I gave up. We knew we had to get her a new pair. I didn’t want to buy her a new pair of glasses right then, knowing there was a chance that her prescription might change in September.  So, I called Lyra’s PO to see if they could get her in sooner. Luckily, they were able to schedule her an appointment later the next week. In the mean time, they said it was okay for Lyra to wear her old glasses…just until the appointment.
The day before Lyra’s appointment…the missing lens to her other glasses magically appeared. There it was, just sitting on the end table in the living room. I don’t know how it got there. We asked Rebekah and Dominick and neither of them knew how it got there. I suspect Lyra may have found it on the floor somewhere and just picked it up and put it on the table, but as much as I GRILLED her…she just wouldn’t confess…lol.   Maybe it was the dog.

Lyra’s check-up went really well. They dilated her eyes and all that fun stuff. Her refractive error didn’t change, so her prescription remained the same. So much for my reasoning for Lyra needing a sooner appointment. Dr. Grin did note that Lyra was focusing on objects/noisy toys at a further distance. She was looking at and paying attention to the wiggling barking stuffed dog on the shelf across the room. …well okay then.
Regarding Lyra’s nystagmus, Dr. Grin said that when she is focusing on something close up, her nystagmus REALLY slows down. Since the appointment, I’ve been paying attention to that more, and I agree. I’ve even noticed that for very brief moments when she’s focusing on something, her nystagmus stops completely.  Her next appointment will be in 6 months.

Dr. Grin did not, nor does she ever give us any type of visual acuity numbers for Lyra. I’m okay with that. She said she’s seen children have a visual acuity of 20/200 and then at an appointment 6 months later that same child see at 20/80. Those numbers fluctuate and they are not a definitive or complete indication of what Lyra sees.  However…just for the sake of numbers, we do now have a number.  Last month at the NOAH conference, we had the opportunity to get those visual acuity numbers. Doctors from the University of Minnesota were seeking volunteers to participate in a research study. It consisted of some basic information forms and a questionnaire and also included a vision test. They used a preferential looking test, specifically Teller cards, and based on that test, at that time Lyra’s acuity was 20/130. Those numbers mean more to other people than the do to me really. When people ask us, “So what IS Lyra’s vision like?”…that number is really what they’re asking for. So now I have a number I can give them…instead of just a range. I know her acuity may be better or worse than that on any given day depending on the environment and situation, but knowing whether its 20/100 or 20/360 doesn’t make any difference or serve any purpose. For those people who ask and just HAVE TO know… 20/130 is what I’m goin’ with.

Just over the last month I’ve actually had a more than a few opportunities to respond to those people, asking how well Lyra sees. After talking to those people and just thinking about comments people have made in the past, I’ve come to a conclusion… I think Lyra’s glasses are misleading. I think a lot of people assume that with her glasses on, she has 20/20 vision. When I’ve told people that Lyra’s vision is 20/130 WITH her glasses ON, many of them are shocked. These are people who have known Lyra since birth. A common response is, “Really! I had no idea her vision was that bad.” People knew Lyra had vision problems but apparently thought her glasses “fixed” all of those problems. It just really makes me wonder now, how many other people in Lyra’s life think this.

I can understand how easy it would be to think Lyra sees better than she does. She does function very well and puts on quite an act, fooling people into thinking she has perfect vision. I admit, sometimes she has me fooled, forgetting that she has any visual impairment. Then one day, Lyra walks up to a pile of laundry sitting on the couch and says, “Hi Daddy!”…and I’m reminded, oh yeah, she can’t see perfectly. Fortunately those sad moments are rare. Honestly, MOST moments are happy moments, when the focus is on silly, beautiful, sweet Lyra…and not on her visual impairment.

Okay so I found some time to make this video.

This is my attempt at zooming in on Lyra’s eyes so that you can see the degree/severity of her nystagmus. I apologize for my crappy camcorder skills. Between me not holding the camera steady and Lyra’s wiggling, it’s hard to know what shaking is actually her nystagmus. Every once in a while I was able to catch her holding still with her eyes wide open. Look for those moments and then you should see her nystagmus.

The first part of the video was filmed in the early afternoon and the second half was right before her bedtime. Sometimes her nystagmus is more noticeable when she is tired. See if you can tell a difference. I couldn’t.

Nevermind my singing…LOL… I promise I really do have a degree in music education.

I have been meaning to post this for several weeks. Lyra visited her pediatric ophthalmologist for a check-up last month.

They dialated her eyes this time to see if her refractive error had changed, and it did. Her prescription changed from +2.50 to +3.25 in her left eye and from +2.75 to +3.75 in her right eye. It’s not a huge jump or anything, but to change that much in only 6 months freaks me out just a little. Dr. Grin said her biggest issue is her astigmatism. She said it is fairly common for people with albinism to have a significant astigmatism.
So she is getting new glasses. (more…)

Lyra has been exceeding all of my expectations with her vision. She has seen things or done things I thought would never happen. Like all kids with albinism, Lyra has multiple eye problems that contribute to her overall low vision. (more…)

Lyra wears her glasses now all the time, rarely taking them off except for at night. This is wonderful, however now they are nearly too small for her. They are beginning to leave marks on her nose where they are too tight, and they barely reach over her ears. This STINKS! I can’t believe we are already going to have to spend another $200+ for a new pair of glasses. (more…)

Lyra had another appointment with her pediatric ophthalmologist a few weeks ago. Her vision continues to improve, however the best estimate we can get as far as accuity is still 20/200. She is too young to obtain visual acuities with standard measures. Dr. Grin is not seeing any recurrence of the strabismus and her nystagmus is less apparent. Lyra hasn’t developed any noticable head tilt that is sometimes associated with nystagmus. (more…)

Lyra finally has her glasses. The first time we put them on, they were off in less than 30 seconds. Every time we tried to put them back on her, she would shake her head “no” and scream.

Now, 2 weeks later, she’s kept them on for up to 20 minutes at a time and she LETS us put them back on her. I’ve noticed they start to slide off her nose after a while, giving her the NERD look. I’m going to take them in to be adjusted to fit better. They also have to order new lenses, because these were supposed to be transition lenses…which we paid for…but they’re not.
I can’t decide if she looks cuter with them on or off.

I think they’re helping her see better, but it’s hard to tell. She seems to have more precision with fine motor skills when she is wearing them…putting a small ball in a cup, putting puzzle pieces in, reaching for objects. So the glasses are improving her close/near vision, but what if it screws up her distance vision? I guess my glasses for nearsightedness don’t screw up my close-up vision. These are the kinds of stupid things I worry about.

Her prescription isn’t very strong…+2.5 in one eye and +2.75 in the other eye, but when I look through them, they look really wacky. She’s leaving them on longer, so it can’t look too wacky from her perspective.

She has her next appointment with her pediatric ophthalmologist on Dec. 5th.

It’s going to be a long month and a half for impatient mommy.

Poor Dominick is so sad…he’s the only one in our family who “doesn’t get to have glasses”…other than the dog.

This
work is licensed under a
Creative Commons Attribution-Noncommercial-No Derivative Works 3.0 United States License.

Lyra is walking pretty much all the time. I rarely see her crawl anymore. This is AWESOME…except that she is like a pinball when she walks. I keep the toys, books, blankets and sippy cups picked up as much as possible, but Lyra drags everything out all over the place. Then she walks around the room tripping on this, bumping into that, and sideswiping everything else. I know toddlers fall a lot when learning to walk, but I think its more than just being a toddler. The only identifiable issue that we can try to correct, is that Lyra doesn’t look down, as she walks across a room. She just PLOWS right on through. Her TVI says this (not looking down) is fairly typical of kids with visual impairments. Also contributing to her frequent bumps and bruises is her poor depth perception. She’ll misjudge how close she is to the wall or climbing gym and clip the side of her head on it.
We are working with her and trying to use some sound cues to get her to stop and look down. And lots of kisses for the bumps and bruises. She will be having an evaluation with an Orientation and Mobility specialist in a month or two. This is a standard evaluation done once a child starts walking. The O&M specialist will be able to help us recognize/identify which trips and falls are a result of her low vision and which are just typical toddler falls.

She’s been doing so good… I had started to forget she had any visual impairment for a while.

It just makes me sad…

This
work is licensed under a
Creative Commons Attribution-Noncommercial-No Derivative Works 3.0 United States License.

Lyra has developed enough of a refractive error to need glasses. They are for farsightedness and the strength is +2.5 in one eye and +2.75 in the other. Her PO was going to wait another 6 months. I asked if we could go ahead and get them, as long as there was not HARM in it, so that Lyra could get more “practice” wearing them. The doctor agreed and told us to try and get Lyra to wear them 25% to 50% of the time. We are excited and nervous at the same time. We are still trying to figure out a way to get our insurance to pay for them, so we haven’t actually GOT the glasses yet.

One thing I worry is that friends and family might assume that her vision is 20/20 once she has the glasses. These glasses will only correct her vision to a point, and do nothing for her distance vision.

I’ll be sure to post some pics when we get her glasses.

This
work is licensed under a
Creative Commons Attribution-Noncommercial-No Derivative Works 3.0 United States License.

I hadn’t had any real concerns regarding Lyra’s vision development for a long time. Recently though, her TVI, Troy and I have noticed some issues with her depth perception. She tried to “pick-up” a label on her Baby Faces book as if it was a sticker, but the label was printed on the book, it was flush with the cover. Also, we got this book called “Cheerios”… it has pictures of cheerios and also has little cut-out circles for you to place real cheerios. We present the pages to Lyra with the real ones already on it and see if she can pick out the real ones from the fake ones. Anyway, she struggles with this and tries to pick up the fake ones. Also, with her walking more now, I don’t know if she is running into things just because she’s a typical toddler, or because of poor depth perception.

I talked to her PO about this and she said its common for kids with albinism to struggle with depth perception. I knew this, but I had hoped the strabismus surgery would have helped this more. I just wish I knew exactly HOW MUCH her depth perception was impaired.

This
work is licensed under a
Creative Commons Attribution-Noncommercial-No Derivative Works 3.0 United States License.

Before	After

Lyra’s surgery went very well. I think she did better than I did. The surgery itself only took about 40 minutes and then she stayed in the recovery room about an hour. That was a really long hour for me to wait. I didn’t think it would take so long for her to wake up. We were home before noon. It’s amazing what they can do with outpatient surgery these days.

Her eyes weren’t as gross looking after surgery as I expected. The inner whites of her eyes were bloody, but it wasn’t constantly dripping or oozing blood or goop. Her eyes never got so crusty that they were matted shut. Her recovery and healing has been fairly easy. The pictures above are from a week before her surgery and then 2 days post surgery. They look pretty straight to me, at least for now. I hope they stay straight. I am constantly checking to see if they start to cross again.

This
work is licensed under a
Creative Commons Attribution-Noncommercial-No Derivative Works 3.0 United States License.
Her post-op check up with her pediatric ophthalmologist was a week ago. Dr. Grin said Lyra’s eyes looked good and were healing properly. She will go back in about a month for another check-up.

Lyra will be having bilateral strabismus surgery next Tuesday, March 13th. Both of Lyra’s eyes cross inward. The purpose of this surgery is to straighten her eyes. By improving the appearance of her eyes it will hopefully improve her psychological and emotional development. The realignment of her eyes may also improve her depth perception and improve the likelihood of developing equal vision in both eyes. The following is from an information sheet given to us by Lyra’s pediatric ophthamologist, Dr. Trudi R. Grin called “Understanding Eye Muscle Surgery.” This explains the surgery:
” Eye muscle surgery involves re-positioning the muscles that attach to the eyeball. It is similar to releasing the tension upon reins to a horse’s head in order to control the horse. Muscles are attached to each eye to pull the eyes up, down, left, or right. Surgery to these muscles strengthens or weakens their functioning in order to straighten the eyes. This is done by putting a suture into the muscle, then cutting the muscle off of the eye and re-sewing the muscle at the appropriate position, which in turn strengthens or weakens that particular muscle.”This surgery was originally scheduled for February 27th. We chose to reschedule the surgery after we decided to have some blood testing completed first.
We did not take Lyra to have genetic testing after she was born to determine which type of albinism she had. At the time it wasn’t a priority. We knew we weren’t planning on having anymore children and it wouldn’t have affected how we cared for Lyra. During our planning and preparation for her surgery, we remembered there was one type of albinism, Hermansky-Pudlak Syndrome (HPS), that included bleeding tendencies. We hadn’t seen any symptoms such as easy bruising or prolonged bleeding that would give us reason to suspect HPS, but because we didn’t have the genetic testing, we couldn’t know for sure.
We still don’t know the answer to that question, but we do know that Lyra doesn’t have a bleeding disorder. Because of the time frame necessary to get an appointment and complete genetic testing, we decided to take her to a hematology clinic to test specifically for any bleeding tendencies. A genetic test alone would tell us if she had HPS, but we would still had to of had the blood testing to determine if the platelet dysfunction was present. Similarly, just because all of her blood tests came back normal, this does not tell us she doesn’t have HPS. Because of the risks involved with surgery, obviously testing for the bleeding tendencies was the priority. We plan to schedule the genetic testing after her surgery, hopefully within the next 6 months.

This surgery is an outpatient procedure. We have to be at the hospital by 6:45AM and the surgery will begin approximately an hour after we arrive. They said the surgery should last 45 to 60 minutes. After the surgery, she’ll need to stay in the recovery area anywhere between 30 and 60 minutes, hopefully no longer. After that she will be able to go into a “post-op” room where we can finally be with her again. They told us that assuming everything goes well, we could expect to leave the hospital before noon. Pain after this surgery is usually mild, but we may need to give her Tylenol or ibuprofen for the first day or so. Eye care following the surgery is fairly simple…basically just keeping them clean and free of crusty gunk. “Blood-tinged fluid will ooze from the eyes for a day or so following surgery.” Yipee! Doesn’t that sound like fun.
Yuck!!!
I’m not really even too worried about the actual surgery. I’m worried about her being put under general anesthetic. That’s what scares me. I know I’m going to be crying my eyes out when I have to hand her over to the nurse. It’s going to be an agonizing 1-2 hour wait. I can barely type this without crying. All I can do is pray and know that I’ll be leaving her in God’s hands.
She’ll be safe.

This
work is licensed under a
Creative Commons Attribution-Noncommercial-No Derivative Works 3.0 United States License.

Visits to the Pediatric Ophthalmologist-Dr. Trudi Grin-

1 month old- low pigment in eyes, nystagmus, strabismus, and photophobia. I’m told her vision will likely be anywhere from 20/60 to 20/2200 corrected.

3 months old- doctor still seeing the nystagmus and strabismus in both eyes, now seeing a little bit of farsightedness, but not enough for glasses. We are told that eventually Lyra will need strabismus surgery.

6 months old- At home we were seeing Lyra’s right eye turning in more. Dr. prescribes patching the left eye, 1 hr a day, everyday.

7 months- We call the doctor to tell her we are now seeing both eyes turning in equally. Dr. prescribes patching each eye, alternating every other day, for an hour.

8 months old- Strabismus surgery is scheduled for February 27th. Lyra does not need glasses for farsightedness yet, but doctor says she will prescribe tinted glasses for the photophobia in the spring.

This
work is licensed under a
Creative Commons Attribution-Noncommercial-No Derivative Works 3.0 United States License.