Ella's Pages

Great progress today. And on two points.

One because she actually drank grape juice from a regular cup! We were at great aunt Karen’s for dinner and since it’s so close to home I only took some food for Ella and no bottle. After she ate I took a plastic cup with some grape juice and tried to get her to drink. As usual she turned sideways and locked her lips as soon as she figured out I was trying to get her to take a sip. So I took some juice on my finger and splashed it on her lips. Okay she got the idea. Then I sipped the juice myself out of the cup right in front of her. She got that idea as well and then took a sip by herself. After that she was enthusiastically trying to grab the cup and she sipped! The second point here is that she learned by copying my example. I think that proves a good ability to learn by imitation. We’ve been trying all week to get her to take the sippy cup with very little success so this was great. Otherwise she was in a good mood today and played with all her cousins and Gaul of course. One of her favorite games is passing the beach ball with Yardaena and Ayellet although her “pass” is more like an accidental push than anything else.

Now comes the wrestling matches with the insurance companies. We are trying to get Ella few pieces of equipment that she needs to work on her standing and mobility. Little by little she is starting to do things with her legs but there is a clear asymetry with the right leg being much stronger than the left. I think that neurologically Ella doesn’t understand yet that legs have some use. We’ve been told that that poses some problems developmentally because a child that doesn’t stand by about 18 months doesn’t develop proper hip sockets. So the child uses a “stander”. Tufts insurance doesn’t cover that automatically and we aren’t sure they cover it at all. Letters are needed from the PT, the doctor, a committee needs to review it, blah, blah, blah. Usually Early Intervention has a stander but they don’t have one right now either so time passes and Ella doesn’t have this equipment. The other thing we’ve been working on is the transition from the bottle to the cup and mashed food to solids. Ella is great at eating crackers, cookies, even bread but she throws all peices of vegetables, fruits, meat and milk products. Her pitcher’s arm is improving but not her chewing skills. She’s very stubborn. Usually when I approach her with her sippy cup she locks her lips and turns away (always rightward). She knows what she doesn’t want and she knows how to express it!

Ella had her shunt adjustment done at the beginning of the week. Her final setting is now 100. She is fine with that and didn’t show any discomfort or bad side effects. (There is always a risk that the new setting will be too low and she could get sick). We’ve passed the critical 48 hour adjustment period and all is okay. I was a little concerned before the adjustment b/c she was doing so well at 120, but apparently 100 is okay too. Ella started two playgroups last week. One is through the Perkins School for the Blind. This is open to children with visual impairments and their parents. The adults go off to meet together and the children stay in a group with one-on-one caregivers assigned to each child. It is important to me that Ella has some time with other children in play settings although it doesn’t seem too critical to her. She looks around a bit, gets used to the crowd and then plays with the great toys they have there without noticing or caring about anything or anyone else! At the EI playgroup Ella is the least mobile but she does get to try out all the cool toys in the company of other kids. Beth will take her from this week on, but I took her last week for the first time. She got very excited at circle time and even patted the hand of one of her little playmates (so begins intimacy?).

We made it back to the U.S. in one piece (although two Palestinian suicide bombers killed over 20 people in Tel Aviv the morning we left). Ella was fine on the way back. I think all in all she enjoyed traveling with us and seems to have made some significant progress (see pictures…). She seems to be stronger while sitting and of course, now she is starting to have some baby-talk vocabulary. She loved playing in the pool in Eilat. She splashed vigorously and had a smile on her face the whole time she was in the water. Sometimes she appeared overwhelmed by so many new faces like when we visited all Etan’s father’s side of the family the day before we left. Of course, Ella can always concentrate on her toys. In any case, whenever she focuses on us– Gaul, Etan and me — she looks happy and content. On the plane ride back, Ella slept most of the time. I think the trip home was easier than the trip going. Well, when the endpoint is your own crib with your own musical mobile overhead the world is a happy place. Now Ella truly is a world traveler and impressing her therapists with all her worldly experience…

We are in Israel now, and despite the tense political situation here, everything is going fine and we are having a great time. Ella seems to enjoy being with us all the time. She had some trouble adjusting to the time change and she apparently had a pretty bad stomach ache right after we arrived. We can’t be sure it was a stomach problem but for one of the first and only times in her life she cried and was completely unconsolable. Eventually she had some prune juice and fell asleep quite late that night. Otherwise two days ago Ella made a break through in speech. She actually started pronouncing consonants. She does her screeching thing which has become much more refined. Gaul says “She sings opera”; but now she has been saying “Naw, Naw, Naw”! So if she keeps this up we feel she’s off to a great start. We are going to Eilat in a few days with a long drive down south. Hopefully she’ll be okay with that. So far, she’s been a real trouper, staying up late at night and dragging around as we visit all our friends and relatives. She keeps a smile on her face much of the time.

Ella’s progress is slow but steady. I’ve been doing this new exercise with her where she stands on my stomach. Of course she can’t stand on her own but I straighten her knees with my hands. With her knees locked she rests lightly on my thighs that support her from behind. It seems to help her get the idea of standing and she seems stronger in the legs from time to time. She’s very happy while in the “standing” position — I put on her favorite tape (Rifka Michaeli’s trip to the zoo with Guri) and she gets so excited she starts screeching and throwing her arm out to the side, which I think is Ella dance move. Yesterday at aqua therapy, Donnalee was very excited about her progress. According to Beth, Ella made some movements that looked like attempts to stand. This is great! Otherwise, Donnalee thinks that the new shunt setting is helping alot. Ella is more alert, more focused and happier — if that’s possible. We are gearing up for the big trip to Israel next week. I have the feeling Ella will enjoy traveling and being with us all the time.

After a CT scan done last Wednesday, Dr. Black decided to lower Ella’s shunt setting again. The pressure, even after lowering, is still way above normal. It will take one more adjustment to bring it down to normal levels. It seems to have been successful. Everyone has noticed that Ella is more alert and connected and that she is (if this is at all possible) in better mood. The acupressure treatment continues weekly. It doesn’t seem to have a dramatic effect on her use of her left arm and hand but, there seems to be some change (related or not) in the way she uses her left. We need to address something else as well, the minimal use of her left arm leaves her neck muscles tight (maybe shortened) on her left side and she needs stretching to help with that. We haven’t been too successful in convincing her that anything else besides crackers is in fact food. She examines things briefly, but intently and if they are not crackers they are tossed aside. ‘Bring on the real stuff’.

The trip to New York to meet with the plastic surgeons left us with some new information and some new decisions. On the information side are two opposing points of view. One claims Ella’s over growth on her right side is governed by the soft matter, when it grows it stimulates the bone structures to grow along. The other point of view states that Ella’s situation is so rare that we really don’t know what the patter of growth will be. The experts, even the most experienced have only seen half a dozen cases like Ella through their careers, and you can’t base statistics off of that. One point of view that was encouraging was that perhaps (a big one), the mass in Ella’s face is composed mostly of fatty tissue and that it is above the plane where the facial nerve lays. The facial nerve is a big deal. One of the doctors said that ‘without the facial nerve, facial plastic surgery would be a piece of cake’ (not that I could think of anyone who would like that particular cake…). Unfortunately it is there and performing surgery on the face without injuring it is very difficult. So there lies the big dilemma. Do we go for surgery now? Remove the massive part of Ella’s facial overgrowth, maybe stop further bone overgrowth? Risk damage to her facial nerve? That could leave her unable to move, here face and smile. It is very frightening. This is particularly true when you look at her smiling. She does that a lot. It is a big par of her. We have another consultation here next week, we will see if there is some imaging technique that will allow them to tell for sure what the composition of the mass is and where it is compared to the nerve. Along less medical lines… Ella is making a lot of progress. For one, she is vocalizing more and more. Her ‘vocabulary’ is getting richer. She shrieks more, grunts more and tries to take part in the conversation. With her fine motor skills it’s a mixed blessing. Her use of her right hand is becoming much much more fine tuned. This however emphasizes the difference between the right and left hands and makes it all that more dramatic. She is doing nearly nothing with her left hand. We have started taking Ella for Acupuncture. It is in fact Acupressure sessions. David (her acupressurist) explained that in babies the pathways are not yet defined enough to use needles. They use pressure points and magnets and pressure instead. The treatments seem very minor and I only hope they have some effect. I have no experience with it or any particular trust (yet) but I am willing to try it if it contributes to stimulating Ella’s use of her right side. What has remained consistent and if anything, has grown is Ella’s relentless optimism and cheerful mood. It is almost uncanny. When you approach her in the morning while she is in bed waking up, she will greet you with a big smile and looks genuinely happy to be awake. Then at the end of the day when you put her in bed, she can almost not contain herself, as she kicks and laughs happy to be in bed. Almost all experiences between these two events are nearly as joyful (ok, sometimes she does get pissed off when we push her too hard with her exercises). Today we tried something new, we stopped her from using her right hand by putting it into her overall. She was forced to use her left or nothing at all. Nothing at all is what she chose for part of the time, though once in a while she actually did bat at things with her left. It is depressing to see how limited her ability is. Strangely enough, later on in the day she was sitting around, with her little xylophone when she (with no enticement from us) started batting at it with her left! Maybe its is a precursor of things to come Can’t wait.

All in all, we are feeling pretty positive about our experience in NYC. Also what we learned there was encouraging. We met with Dr. Barry Zide in the middle of the day, before rushing back to NYU at top speed to meet with Dr. DeLacure. Dr. Zide has a very plush office, quite different from the stark, 50’s looking hospital we just came from. He has done alot of work on children with what’s called “big hairy nevus” and skin expanders where good skin is overgrown using a bubble placed under the skin. Then, the extra skin replaces the nevus. We didn’t talk too much about using this technique on Ella, but more on the whole syndrome that she has including bone and soft tissue. Dr. Zide seemed to think that the nevus on Ella’s scalp might not be a problem at all over time since her hair might grow in and cover it. He described treating her overgrown ear as “a peice of cake” which at least made me feel like it’s not such a big deal. He thinks the facial nerve is a big, complicated problem and that makes the case for putting off the surgery until as late as possible. He described the surgery as a one shot deal b/c of the nerve. So now we are faced with somewhat of a dilemma with some doctors telling us that earlier is better for the surgery and others seem to think we should wait (including Dr. Mulliken here in Boston). We feel that with these conflicting approaches we need more information. We certainly don’t want to risk impacting her great smile any earlier than we have to but if it is true that the soft tissue drives the bone growth than that might be worth the risk. It is very hard to know.