New Equipment

I am trying to bury the previous post. (It is LONG... the summary is that Sophia had two seizures in December, but is on seizure medication now and is doing okay so far... Now you don't need to read it!)

Never mind about the previous post... how frustrating.... I was about to post it, but I highlighted the text to try to change the font, and -- poof -- it disappeared. I am discouraged because it took hours and lots of energy to write. Anyway, here's this one (I'd better post it before its text disappears too):



Sophia has some new equipment. The first is a new gait trainer / walker. She wasn't quite ready for it, but our insurance would pay 100% of it last year, and we received it Dec. 31, so it was fully funded by insurance! Hooray! The following photo is from January 1st. She couldn't hold herself up at all. I wondered whether she would ever be able to use this thing.



In early February, she received foot orthotics (braces that support her feet and lower legs). I need to take a picture of them. We had to pay nearly $300 for them, and insurance covered the remainder (no more 100%). She wears huge shoes that make her tiny, unused feet look big, but the orthotics make her much sturdier. The following photos are from February 18th. She can't propel the gait trainer herself and her feet cross over each other when she steps, but look! She can support herself (with the gait trainer's supports and the orthotics helping) with her arms and legs!! Now I think that she may someday actually be able to get around in this thing! (Though her therapist thinks that a wheelchair will be her primary mode of transportation.)

We're also borrowing a different, more supportive stander from Early Intervention. I was disappointed to go from the less-supportive stander to the more-supportive one, but it is a nice piece of equipment and is what she needs. She loves to bite the bin that comes out of the tray.

All done!

I'll try to do birthday posts soon -- April and Sophia turn 2 on Sunday!!

Another Diagnosis

In September, we took Sophia to an opthalmologist. The last time she saw an opthalmologist, she was 4 months old, still small, and home for just a couple of months. At that time, the visit's purpose was an ROP follow-up to make sure that she still showed no signs of retinopathy of prematurity. We also expressed to the doctor our concern that she didn't seem to be seeing and tracking. The doctor seemed rushed, and didn't explain things to us to our satisfaction, but we recall him telling us that both babies' eyes looked good, and that Sophia might have CVI (which is a vision impairment due to the brain not correctly processing what the eyes see). It was this doctor who made the referral to the neurologist, who subsequently diagnosed Sophia's PVL, cerebellum atrophy, CP, and CVI. The neurologist told us that there was no point to taking her back to the opthalmologist since she couldn't see much of anything.

Jumping ahead to last spring, when Sophia began to reach for and track objects held close to her eyes, the developmental pediatrician she sees twice a year recommended we take her to an opthalmologist again since she was seeing more. The thought was that perhaps something should be done for her crossed eyes (to improve binocular vision) since her vision was improving. So, I made the appointment with a different opthalmologist and waited with building anticipation for months for it to come.

I expected that we would leave the appointment with a plan to address her strabismus (in her case, crossed eyes). I thought we would either be told to patch her eye(s) for a certain number of hours per day, or she would have a prescription for glasses, or she would be scheduled for corrective surgery. I hoped for the surgical option, since that would straighten out her eyes and, I hoped, help her vision. I was extremely optimistic about his appointment.

Instead, we left the doctor's office with a new diagnosis. Sophia has severe optic nerve hypoplasia (ONH). This means that her optic nerve didn't develop properly. She has only about 15% of what she should (sounds kind of like the cerebellum diagnosis, huh?). The doctor said she may have CVI too, given her neurologic history (= brain damage), but that her ONH is severe enough to singularly account for her visual status. The icing on the cake is that this condition often also affects the pituitary and growth. This is called SOD, and we need to have her tested for that.

Although it doesn't matter, I don't understand why both the first opthalmologist and the neurologist missed this. Was the opthalmologist just pretending to look in her eyes? Was the MRI image so poor that the optic nerves looked normal? However, this diagnosis does answer some oddities that CVI didn't seem to explain. Sophia's light sensitivity was not typical to CVI, but people with ONH sometimes have sensitivity to light. Her CVI had seemed worse in relation to her physical impairments than it should have, according to a doctor she saw last year. So some "mysteries" have been explained.

This diagnosis didn't change Sophia's vision. She is the same cute, smiley little girl she was before the appointment. However, what it did was change my hope. With CVI, I had hope that her vision would continue to improve as her brain learned to better process the images it receives. I dared to hope that she might be able to learn to read, at least in large print. I hoped that she would be able to navigate safely through space. I hoped... We still don't know exactly what she sees since she can't tell us, but now those hopes are gone. Her brain doesn't receive all the visual signals that it should. The optic nerve can't be healed or regrown (though there has been some promising research in mice, so perhaps someday...). It is finally, heartbreakingly, accurate to say that my baby is blind.

18 months - Sophia

Sophia is 18 months old today!

In a departure from my typical format to date, I'm going to journal a snapshot of Sophia today, at a year and a half old. She is a cute, little girl and we love her dearly. She seems "sweet" because her body doesn't let her get into much trouble, and she is not easily upset. However, she enjoys roughhousing and fun play, so I think her true personality may be much more fun-loving. She is fairly mellow, but when she is upset, she remains (sometimes) difficult to soothe. For example, we went camping a couple of weeks ago, and Sophia fell asleep in the stroller on a walk before bedtime. I woke her when I changed her diaper, and then April crawled all over her, and then NOTHING worked to pacify her for an hour and a half. (Which, I'm sure wasn't much appreciated by the neighboring campers.) This is very unusual, though.

This week, the physical therapist who sees her twice a month in our home through Early Intervention brought a stander for Sophia to try. This one offers minimal support, and if it works for her, that would be great -- less support means more work for her, which means more potential strength building. However, her trunk is not very strong, so she can lean forward way too much (not helpful):
Here are her cute little feet and toes (she also has some cute little Adidas shoes that she wears in this). Her feet always want to roll to the inside (scissoring), and her toes scrunch up during weight bearing. She also tends to lift her heels. I just position her feet for her when placing her into the stander. The knee pads help to align the legs and feet correctly.

Ahh, here is better posture. We tap firmly on her back to encourage her to stand up correctly, and, if this fails, we place one hand on her chest and one on her back and simply lift her to the correct position. Then we help her grab the bars, and that way she can use the bars to help hold herself upright.
Sophia is generally pretty smiley, but this is the first time I'd been able to get some good smiles from her while in the stander. I think her body is working so hard that it is just too much to smile. I don't think she minds the stander, though. In fact, when she's on the floor, she says "up" (which sounds like "uuub") and I think she is asking to either stand or sit up (or maybe just be held). She also says "ball" for her absolute favorite toy -- a ball on loan from the EI vision specialist -- and it also sounds like "uuub," but I really do think they are two separate words. "Ball" ("uuub") was her first word, and she began using it less than two months ago. We are excited that she has a few words now, as she is at risk of being non-verbal. We hope there are many more words to come! Her other word is "more" ("mmm-la") and she also verbalizes when she is hungry ("mmm-mmm" or a variant). Back to the stander -- when she's finished with it (currently about 15 minutes), she lets us know by fussing!
Statistically, if Sophia doesn't learn to sit well by the time she is two years old, her chances of walking someday are minuscule. We work on sitting every day. I sit behind her and place her in a sitting position (although we are also working on showing her how the transition goes from back-lying to sitting). I bend her knees so her feet are touching (to reduce the effect of her hamstring tightness on her hips). Then, I pull her behind back a bit to help her correctly tilt her pelvis. Finally, I help her weight bear on her arms to remind her to use them for balance as needed. This is her normal sitting posture, or sometimes she falls forward even more than this:
I help her adjust her balance by pressing down on the thigh opposite of the direction she is tilting, if necessary. Here is a notably good sitting moment:
It doesn't last long, however. After a few seconds, she does this:
And then this:
I place her in the following position to show her how to get down to the floor from sitting. This time, she held herself up with her arms before lowering to the floor. Although she was placed in this position, I look at this picture, and in my imagination she is just about to crawl off to play:
Sophia continues to be a relatively good eater. She swipes her left hand across her tray in an arc, looking for finger foods. When her hand touches something, she rakes it up and brings it to her mouth. She can eat with her right hand too (which is actually her less tight hand), but for some reason she generally uses the left. I think her right hand and arm may help her stabilize herself. If the food is beyond the reach of her arc, she doesn't get it. She doesn't seem to use her vision much while she is eating, although she sees and reaches for her sippy cup within about 15 seconds when I bring it to her and hold it in front of her. She handles her sippy clumsily, but has figured out how to maneuver it quite well and seldom fusses for help. Sometimes she drinks from it upside down, which works as long as the cup is fairly full. She drinks whole milk, occasionally formula (if we are out it is easy to transport), and loves juice, although I rarely give that to her. She'll drink a little water before she decides it isn't quite what she wants. She doesn't know how to drink out of a regular cup and doesn't have the slightest idea what to do with a straw (she chews on it). She eats most things, and gags only when she has too big of a bite of something. She needs to improve her chewing. Every meal, I am grateful that she eats so well.
Sophia rolls both directions. She will roll more than once in a direction, although she has yet to do anything like roll across the room. Today, she was close to the border of the family room, and I told her I would love to find her in the kitchen (due to her own mobility) one day. Yesterday, I vacuumed around her and she was excited about the vacuum cleaner. She rolled toward it in both directions. (I did stop and let her briefly touch it). Her vision is about the same as the last report. Her eyes are very crossed. She sees in near space and has a delay in reaching toward objects. Typical of CVI, she usually does not look directly at an object as she reaches for it. I hope her vision continues to improve. She has tracked me as I walk across the room several feet away from her, although since it is difficult to tell where she is looking, I can't really say whether this is due to visual or auditory cues.


She has begun to try to grab books when I am reading them to her. If successful in her quest, she tries to eat the pages. I am delighted that she is getting into this "trouble." Also, if I hold her while I play the piano in Primary at church, she slowly reaches for the piano keys. It is really, really, hard to not let her just play the piano. I'm not sure the Primary children and leaders would share my enthusiasm about this accomplishment.

We love this smiling, happy, sitting (or at least working on it), standing (with help), talking (3 words), eating, seeing (some things), fun kid.

Big Steps!

Today, April took her first unassisted steps! This is exciting, because I thought it was still a little ways off. Yesterday, she stood alone for a few minutes. This is the longest by far I have observed her standing alone. I think she thought she was leaning against something, because her dress was lightly touching a toy wagon at Grandma & Grandpa's house. Then, today, she was standing at the back door watching her sisters outside as she often does, and I knelt down by her and encouraged her to walk to me. She stood momentarily, and then took two steps to me! Dale and I then had her go between us, and she repeated it once! Hooray! I'm sure she is still weeks off from walking any distance of note, but this is still a major milestone! She is 16 months, 2 weeks old.

As excited as I am for April, with that excitement come some strong, sad feelings about Sophia. I don't know when or if she will ever take those first steps. As Dale and I had April going between us, Sophia lay an arm's length away on her back on the floor, virtually immobile. As the months roll by and she still can't balance herself in a sitting position, and still can't scoot, the future is beginning to be a little more clear, and I don't like it.

I could, unfortunately, go on about that. However, it has been far too long since I last posted, and there has actually been some very exciting progress with Sophia in the last two and a half months.

First, probably in April, she began reaching for objects that she sees! Yes, that is right -- that she sees. She tracks slowly moving objects about eight inches from her face, and will bat her arms to find and grab the object (if she is interested in it). This has been HUGE. She has gone from no apparent vision, to a little bit. The difference between no usable vision and a little bit is of major consequence. Of course I hope that her vision continues to improve.

Also about the same time, she began rolling from her stomach to her back. It started as an accidental occurance, when she pushed up on her arms (which has slowly improved too) and lost her balance. At first, she would startle and look scared as she flopped onto her back. Now, she does it purposefully, and although she still probably log rolls, it is more graceful.

Then, at long last, about a month or so ago, she rolled from her back to her stomach, which takes more strength. She uses some back extension to do it, which is not ideal, but it is exciting nonetheless. Even better, this new movement was spurred by one of her favorite toys -- a low vision ball on loan from Early Intervention. It sounds like a rattle, and has grab bars for her to hold onto. She like to chew it, and curls around it and rolls to her side as she plays with it. It is the FIRST thing she has tried to move for when it rolls away, and thus the rolling. I actually think she is rolling less now than she was when she first discovered the new movement, however. She seems content to roll to her side and play with the ball in that position.

Both girls also have teeth now. Sophia has four -- two on the bottom, and two on the top -- with more coming soon. April has three, two below and one above. It hurts now if they try to chew on a (my) finger!

Finally, here are a couple of pictures. The first is of April, taken June 8th. The second is of Sophia on the same day. The third is not a family member, but Claire and I and my mom and brother were excited to hear him back in May. Enjoy!

Are You Sleeping?

Are you sleeping, are you sleeping, Brother John, Brother John?
Morning bells are ringing, morning bells are ringing,
Ding, ding, dong. Ding, ding, dong.

“Oh, are these twins?”

“Yes.”

“A boy and a girl?”

“No, two girls.”

“Oh, look! This one is WIDE awake, and this one is sleepy.”

“Yes…”

When April and Sophia came home from the hospital, although they were nearly two months old, we were able to get to know them and spend time with them as though they were newborns. One thing that I noticed within the first few weeks is that April had her eyes open a lot, and her eyes were even a bit buggy looking. I affectionately called her “bumblebee” for awhile. Sophia’s eyes were open much less. She also slept a bit more. At the time, I had not the slightest notion what was to come for Sophia, except that I thought she might be sleeping more as her brain healed from the earlier brain bleed.

By the time we were able to take the babies out into public, it had become obvious to me that Sophia’s eyes, usually downcast, were not seeing things in a typical manner. Since that time, when the girls are in their side-by-side double stroller, strangers have engaged me in the conversation outlined above time after time, after time. Especially since I thought the same thing at first, I understand the sentiment. They make the comments with a smile. They have no way of knowing that every other person to approach us makes the same observation – one awake baby and one sleeping baby.

The problem is, here is what I hear: “One of your babies looks cute, smiley, and typical. What a miracle considering she began life at less than two pounds. The other one, while also cute, doesn’t look at me because she is effectively blind. She looks like she is sleeping, but in reality the environment is simply too much for her to take in, and she closes down. She also can’t move her body in the same way as her sister. She is atypical. Poor baby.”

April & Sophia (who does hold a bottle when her hands are positioned for her - yea!!!)

I understand that this has more to do with my journey of acceptance of my daughter’s differences than it does what anyone else thinks. I also imagine that, one day soon, some people will simply look away when they see Sophia because it will be obvious that something is “wrong.” In fact, the tide is already beginning to change. Recently, people have begun asking how far apart in age the girls are, because April looks older (although their heights and weights are nearly identical, April’s head is bigger and her development is much more advanced).

So, please, keep telling me that Sophia is just sleeping. Maybe we will all wake up one of these days.

April watching swimming lessons (Jolie is in the closest corner of the pool)

Sophia's pretty eye, open wide

One Year Old

April and Sophia turned one year old on March 8th, and what a year we've had with them. I remember, during their long weeks in the NICU, once holding a friend's cute, chubby six month old baby outside of the NICU while my friend went to look at my tiny, struggling babies. One of my favorite NICU doctors walked by, and I said to him, "Here is a real baby!" I was attempting to make a lighthearted comment, but he caught the worry and discouragement in my voice, as he turned and came back just after entering the hallway. He leaned toward me, and with a kind, knowing grin and a reassuring tone, he commented, "Your babies will look like real babies before long too." Even a year later, that memory still brings tears to my eyes.



The seven weeks and two days that my babies spent in the hospital were difficult, discouraging, amazing, miraculous, and wonderful, all at the same time. I plan to record some of my experiences of that time in future blog entries. In brief summary, I had a fairly uneventful twin pregnancy until I was 28 weeks, 5 days along (about 6-1/2 months). On that day, I insisted that my doctor’s office see me because I had felt for a couple of weeks that something was amiss. Despite my premonitions, I was shocked to find that I had an advanced case of twin-to-twin transfusion syndrome (ttts). The perinatologist later told me that my babies would not have survived if I had waited four more days for my scheduled doctor appointment. After an ambulance ride to a bigger hospital an hour away, draining 3-1/2 liters of excess amniotic fluid, and six days of bedrest in the hospital, April and Sophia entered the world via c-section. April, tiny at 1 lb 14 oz, did not even require oxygen until she was several hours old. Sophia weighed in at 2 lbs, 8 oz, but has not fared quite as well as her sister.

When Sophia was one week old, we were told that she has suffered a bilateral grade 4 intraventricular hemorrhage, almost certainly before she was born and as a complication of the ttts. This meant that she had bleeding throughout her brain. She developed hydrocephalus, but was blessed in that it resolved without needing a shunt (which would have required a transfer to another hospital three hours away). By the time she had been home for a few months, however, it became obvious that her vision was not as it should be. She has cortical vision impairment (cvi­), and currently has very little usable vision. She has also been diagnosed with spastic dystonic quadriplegia cerebral palsy. These challenges are due to her brain bleed, which led to extensive periventricular leukamalacia (pvl), microcephaly, and severe cerebral atrophy. Much of this blog will be dedicated to my journey with Sophia.

Below is a picture of my beautiful, real babies on their first birthday.

I think that one child with special needs takes and gives enough emotionally, financially, physically, and otherwise to complete a family. However, we also have Sophia’s twin, April, and thus enjoy a bit of a unique twin dynamic. In addition, we have Audrey (3 this month), Jolie (6 last month), and Claire (9 in July). We love all of our daughters and are grateful that they are a part of our family. All of the past and future unexpected detours in our life will, we believe, lead through beautiful places, and we strive to enjoy the journey.