Ella's Pages

Ella’s periodic checkup with Dr. Duffy of Epilepsy at Children’s Hospital went well. He is always very encouraging and feels that, considering Ella’s condition, we are very lucky. The fact that she doesn’t have more seizures than she does, given her physiology, is very surprising. Reading about other parent’s trials with their children’s Hemimegalencephaly leads me to think he is right. He also said that the stagnation of her motor skills development is a. to be expected with the medication she is on and her condition and b. will be overcome after surgery.
The fact that she is developing cognitively is a good sign and hopefully she will be able to catch up quickly once the hemispherectomy is done. It looks like the operation will happen at the beginning of May. This is both amazingly frightening and a source for hope. I am reading ‘Half a brain is enough‘ and it gives a lot of hope for the best case scenario. It tells of a boy who’s had an hemispherectomy (right side, same as Ella) at the age of three and developed wonderfully doing everything a normal boy his age would do and more.
There are however scenarios besides the best case… Ella’s WILL be the best case scenario. Right?

The days are passing by so quickly and we are so busy. My one regret is not being able to spend much time these days with Ella. Everything seems to be going well with Yvette. She and Ella have definetly bonded. Etan tells me that Keri (her PT) told him that Ella starts whining and won’t cooperate during therapy unless Yvette is in the room.

Oh, I forgot to write that we finally got a long letter from Dr. Stoler, the geneticist. She mentioned a few things that should be watched out for in Ella’s condition like rickets and skin problems in her nevus. She described Ella’s condition as linear nevus syndrome with hemimegalencephaly secondary to that. Her research and letter is very thorough — all 4 pages of it. Concerning genetics, she (as others) determined that Ella’s condition is not caused by any known gene and is a sporadic occurence. Once again, I am impressed by Mass General’s efficiency as compared with Children’s Hospital. The bad news for today is that Ella seems to have had a slight seizure, that lasted about a minute and a half. It was kind of an absence-type seizure similar to the one she had about a month ago. Even though it seemed mild, this is still scarey and must be watched.

Though Ella’s motor skills have remained nearly the same, we feel that her cognitive skills are going through major changes. Ella is making herself much clearer in her likes and dislikes. If she likes something she will be all smiles, giggles and laughter, kicking and moving all limbs to show her excitement and pleasure. If things are not to her liking she is not beyond screaming to high heaven letting us know something is wrong. Figuring out the specifics she leaves to us.
The helmet has brought on two nasty sores in the back of her head. They are slowly improving but are still there, we alternate helmet time and air time to let the sores dry and get better. The helmet seems to be working in that the flat side of her head is starting to round out. Quite amazing how these things work. On the other hand it leaves her head sweaty and smelling like the Romanian wrestling team. She doesn’t seem to mind it though. We were loaned two seats for Ella to use. One, a kiddie cart. Is like a stroller with more support, also has a tray for food or toys. It holds Ella well and she enjoys being moved around the house and feeling like she is part of the action.
A couple of days ago we were watching a movie. It ends with everyone on screen bursting out in wild laughter. Ella was quite taken by that and continued laughing way after the movie was over. (she is not very critical that way).

So much going on…Yesterday I spent almost the whole day at Children’s Hospital. Ella had a helmet fitting appointment, then she had her blood drawn and then she had 2 opthamology appointments. The sore at the base of her scalp was pretty nasty looking by the time we saw the ortontist (sp?). He thought the wound looked infected. I didn’t. It just needed to dry out. He cut out the helmet padding so that it wouldn’t rub there. I’m not convinced he took out enough. We put the helmet on last night and her sore looked a bit oozy again this afternoon. We are keeping it off a bit more. When they drew Ella’s blood, needing one poke in each arm, she sure belted out the wailing. That is a welcome sign and much more normal in my eyes than the apathy which she has shown before. At the vision testing she performed marvelously. At first the doctor, Dr. Mayer, thought she had a field cut. Then after about 45 minutes of seeing Ella respond to all sorts of lights, shapes, toys, cards, stripes, etc. she determined that she “suspects” that Ella doesn’t have a field cut. Ella was a real trooper in any case. She looked every which way and really proved herself! To her Mom anyway. Then we went on to the opthamologist. This is the second time we’ve had such a nerve wracking appointment. I’d hate to be in those doctors’ shoes. Hours of looking through various lenses into the evasive gaze of an infant, lining up lights and lenses over and over again until???? Well, I was losing patients with the system and so was Ella. She got very tired and fussy. I hinted to the doctor in a mildly assertive tone that this was enough and he ran out to bring one of his supervisors. Upon their return, Ella was dressed in her coat, asleep in her carriage and ready to go home. The older doctor (didn’t catch his name) was able to re-examine her while she slept. Apparently, they confirmed some kind of abnormality in her left eye. They couldn’t figure out how that would be connected to her condition or what it was. Could this be new problem? They suggested a follow up appointment in April. Back at home, today, we have two new problems. Ella has a worsening rash on her legs around the edges of her diaper. It looks more like an allergic reaction than diaper rash. It is actually not under the diaper but more on the sides, on her thighs. The other thing is that she has a fatty lump on the back of her scalp. I noticed that before but it now seems to have grown larger. I think we need to go to Dr. Becker’s tomorrow for her to take a look. Uhhh…. what a slew of problems.

Well I made it back to work okay this week and now I’m 3 weeks into the semester as well. Also, we survived the couple of weeks of many guests…grandma Lilly was here for her 70th birthday, uncles and aunts too for the occasion. All this and I’m relieved to say that Ella is more than okay. She is making noticeable progress now. Over the past two weeks she’s been more alert and lively. She’s not sleeping so much but also she is smiling and laughing often. Maybe the helmet is helping too. She often opens her eyes wide and looks around including alot of clearly looking leftward. The difference between left and right hands is becoming more pronounced. On the one hand this is too bad. However, it’s to be expected. She is doing more grabbing (almost reaching) with the right hand. She pulls her bib which ends up making quite a messwhile she is eating. She also scratches a bit of diaper rash she has on her upper thigh. Too bad about the rash but GREAT about her making useful movements with the hand! She has a little sore under her helmet which I think has been caused by the helmet rubbing on a fatty area at the base of her skull. I cut away the hair and put a bandaid on but it is still red and sore. Next week she’s having the helmet adjusted so hopefully they’ll take care of that. Otherwise, we have seen how her head is getting rounder in the back. Amazing how fast the helmet makes a difference. Yeah!

Surprisingly, Ella doesn’t seem to mind the helmet. She wears it nearly all day, and all through the night. It does make her head a bit heavier and so holding it up gets even harder. A strange thing is going on. As far as Ella’s motor development goes, we see nearly no discernible change. She is still unable to sit, roll over or convincingly hold on to anything. On the other hand there are undeniable changes happening as far as her perception and responses go. She is much more focused. She will look at you directly for long periods and be much more responsive to noises and movements you make. She is also developing a strong notion of likes and dislikes.
In contrast to how it was till now, she will outright cry if something is wrong, she is uncomfortable or tired. Not just her usual little moan but a real bawling. I don’t know how objective I can be (I seem to think I am), but looking at you the way she does feels like there is some deep intelligence on the other side, looking you over, examining.
We are waiting to hear back from Dr. Black the neurosurgeon. He was supposed to present Ella’s case to the epilepsy department and get their opinion about the timing of Ella’s surgery. This was to have happened two days ago. Not totally surprisingly, we heard nothing as of yet.

Even though Ella is not making much developmental progress there are very small nuances that prove some progress. For example, she responds to my hands placed on her chest when she is lying on her back by bringing her hands onto mine. She does this both with the right and the left hand which is quite amazing. She still doesn’t put anything into her mouth, not even her fingers. Although her head control is clearly coming along slowly but surely, she is still very floppy due to her low muscle tone. She has been fussier lately which is both good and bad. On the one hand, something could be bothering her and we can’t figure out what it is. How often can a baby be tired and hungry, right? But, at least she is interacting and letting us know that somethings not right. She never cries, but whines and wimpers. We’ve added more solid food. She’s eating avocado, mashed sweet corn and now oatmeal cereal. And alot of it too. Saturday we took her on a trip to Gloucester. She hung out in the snuggley, got fussy, was appeased by the good ol’ pacifier and finally fell fast asleep in the car seat at a restaurant. It was fun for all. By the way, last Wednesday (almost a week ago now) I think I saw one seizure in the morning as I was changing her. She seemed very out of it for about a minute. Her eyes were fixed. She snapped out of it, took a big breath and promptly fell asleep. Ella is always very excited on that table which almost certainly confirms that that was seizure. We watched her like a hawk after that and haven’t seen any more. Fingers crossed.

We saw the neurosurgeon, Dr. Black, last Wed. and Ella now has a helmet too since Friday. Our frustration with Children’s Hospital continues. I just don’t get it. At one point, I understood there was going to be coordination between the Cranio-facial surgeon and the neurosurgeon. Now it looks like there isn’t even coordination between the epilepsy neurologist and the surgeon. I just give up. I feel like we have to make all this happen somehow or it just won’t. I sure look forward to the day when we won’t be so dependent on each of these different specialties. Dr. Black picked Ella up from her car seat and held her as he talked so softly to us that we could hardly hear him. At first he said he would like to wait 6 months, but then after listening to us he suggested we come back after 3 months and then as we got up to leave he said that if we’d really like to do the operation sooner that he would be okay with that. Since then Etan and I don’t see much point in waiting. If she were developing well it would be different but since most of the time it just seems like she is drugged up and so subdued, we think we’d like to go ahead. Black told us the operation will take 2 to 4 months to schedule anyway. Ella is really cute in her colorful helmet but she woke up screaming last night during her first night sleeping with it. She barely cries so we took it seriously and took off the helmet. She screamed again this morning upon waking up from a morning nap with it. Her whole head is sweaty when she wakes up like that even though while awake it doesn’t seem to bother her at all. I guess she’ll just have to ease into it.

“Progress slow, but consistent.” That is how Tracy, Ella’s OT (occupational therapist), reports on her sessions. It is true. Not much progress in gross motor skills. But, today, Ella had a very good day. Evette, her nanny was with her and must have spent hours singing and talking to her, in French yet! Evette reports how responsive Ella is and how joyful she is. Okay. We are happy to hear that. After writing down Ella’s waking times yesterday and watching today, she seems to have come out of the over-sleepiness. Maybe she was under the weather, as Etan and I were, over the weekend. She had therapy today and yesterday and was interactive and cooperative. While siting in her makeshift therapeutic chair today at dinner with us she was so funny. She just started laughing. First it seemed to be because Gal came to the table. Then it was the clanking of a pot lid. Then it was just because….well by that time we were all laughing. But Ella’s laughs are the funniest thing. Really cute. Kind of a cross between repetitive noisy exhaling and a big smile. She always looks like she knows something that the rest of us just don’t get. Oh well — maybe she does.

Tomorrow we have a meeting with the neurosurgeon who will probably perform the hemispherectomy — Dr. Black. That is, we hope we have an appointment with him. You never know with these guys.

Ella’s flat development curve leaves us very worried. There has been no substantial change in her abilities in the past few months. Granted, her head control is a bit better, but all other motor skills have remained basically stagnant. Turning over, sitting up or crawling don’t seem anywhere near. This, I guess is to be expected. It isn’t clear what is responsible, her condition or the massive amounts of medication she is on (or maybe a combination of both). The result is clear. She is moving ahead very very slowly. Our hopes focus on the changes the hemispherectomy will bring. But even then, there are no guarantees. Our task now is to get her to surgery with as few as possible seizure events on the way. Still, I often wonder what her sense of reality looks like. Reading newsgroups of Epilepsy patients, I often bump into grown up’s testimonials of how this or another medication effected them. Harrowing stories. (Leaving them depressed, unable to laugh or very flat emotionally), And these are adults that can verbalize the transformation, and can ask for something to be done. How does this effect a little baby at such a formulative time in her development, when she is supposed to be forming her picture of reality? She is a lot of fun though, and very cute. In the past few days she has been sleepier than usual and we don’t know if she is coming down with something (we are all in various stages of flu like conditions) or whether her medication blood levels are, for some reason, higher. We are keeping a log of her sleeping hours to know exactly how this is changing.