Ella's Pages

The day started at 6:30 am when they rushed us to the pre-op room to get ready for surgery. We were the first ones there, but quickly got bumped from the queue because an emergency case was brought in. We ended up waiting three hours, and finally Ella was taken into surgery. All morning she had the most fantastic mood, playing around with toys we brought and interacting. Surgery lasted about two and a half hours, at the end of which Dr. Black reported it had gone just fine, no complications and no surprises (as we like it). Her recovery was very different than that of the hemispherectomy. Twenty minutes after surgery we were with her, she was awake, and seeming normal, just a bit groggy. There was no swelling and no visible changes except a band aid on her tummy and one on the back of her head (both ends of the tube). The pressure is going to be slowly reduced to a more normal level (turns out her level was 220 versus the normal 120). It will be done gradually to let the bone flaps slowly return to their location and connect, hopefully avoiding a situation where they overlap (whereas corrective surgery will have to be done). Hopefully no surprises or complications will arise and we will be able to go home with her on Saturday.

Well, we were afraid this would happen. And it did. The CT scan done today confirmed that Ella definitely has Hydrocephalus. The suspicion was raised when we discovered that the sutures in her skull (resulting from the hemispherectomy) have opened, and that there is now an opening about a finger wide that goes all along the top of her head. An x-ray confirmed this and the CT scan made it painfully clear. In fact the radiologist came to make sure we will be going to see Dr. Black immediately (he realized something was very wrong) and then, just to make sure, came to Dr. Black’s office as well. The visit with Dr. Black was short and blunt. Ella needs a shunt. The shunt will regulate the pressure of the brain fluids which is now simply too high. Dr. Black didn’t want to risk another day. Ella was promptly hospitalized and we are scheduled for surgery tomorrow morning. There isn’t really any other method of dealing with Hydrocephalus except the insertion of a shunt. In most cases (they tell us) once it is in there, it just works and you forget about it. Complications, however are not all that rare. They include clogging, infections and malfunction. The way the shunt works is that it regulates the pressure by transferring the liquid via tube to the stomach. About half of the children going through hemispherectomies need a shunt. We were thinking maybe this time we will get a break. Again no one asked us. Ella was in good spirits through all of this (except when then put an IV line in her arm). The prognosis is for a recovery of two – three days in the hospital. We’ll see. We are warily going into this latest chapter in the Ella chronicles. All fingers crossed.

It’s been a rough week with Ella. Last Sunday she was acting so cranky, not eating and generally just not cooperative that we got worried about hydrocephalus. She threw up on Sat. night. So, on Sunday afternoon I called and talked to Will’s mother (little boy who’s had a hemispherectomy). She told us that Will just got back from 5 days in the hospital where he was operated on again to correct the bone flap problem that he has. Upon her suggestion I called the neurosurgeon on call: Dr. Johnson. He explained more to me about symptoms of hydrocephalus and told me to check her fontanel which I assumed had closed up. Upon feeling it we discovered that there is a big gaping space between the two halves of Ella’s skull. We got very worried about that. On Monday, Dr. Black’s nurse recommended getting a skull x-ray. We debated about going through with our plans for a 2 day trip to Maine. By Monday p.m. Ella seemed to be feeling better. Upon our return we had the x-ray done (Thursday). Despite the pediatrician thinking the skull looked suspicious of something, Dr. Black eventually took a look on Friday and said the skull looks fine. He has ordered a CT scan for next week anyway just to make sure there is no pressure building up from cerebral spinal fluid (CSF). To me, the worrisome symptom at this point is the size of her head — it looks huge to me and seems to be getting bigger. I would say that from the side, the length of it looks about the same as three-quarters the length of her body. No wonder she has trouble with motor skills! Otherwise, Ella is feeling better. She is not so cranky and from time to time sits, eats and plays very well. At least that!

For the past 3 days, Ella has decided she is on a hunger strike. Yvette and I got so worried about it that I called the nurse at Dr. Becker’s office when I got home. I don’t want her to start losing weight. She was also very cranky in general. The nurse suggested, as I suspected, that it could be teething problems. When I held her yesterday evening she just seemed to be drooling all over. The treatment for that is Tylenol. When Ella decides she doesn’t want to eat, she is very clear about it. She locks her lips shut and won’t open for anything. She has been taking a bottle and nursing fine and acting pretty normal otherwise. Today and yesterday she was too tired for therapy though. The problem is she wakes up so early in the morning (today before 6 a.m.) that she is ready for a nap by 9:30 when Jackie (PT) comes.

I hope Ella is not following in the footsteps of others in the family. She is starting to get addicted to TV. Lately, when I sit with her on our bed, she stares at the empty screen in our room as if to say: “Okay, turn it on. I’m ready.” She even does that with the computer screens sometimes. It’s funny but I hope she doesn’t become too addicted to the boob tube.

Ella is off medication! Even when checked with her blood for the allergy tests her phenobarb level was 6 — it was 60 at its highest point in the past (pre-surgery). What a pleasure not to have to give her any pills. Ella continues to develop but honestly I can’t imagine seeing her ever sit by herself or stand or walk. But then, when she wasn’t even holding up her head I couldn’t imagine her doing that either and she certainly does now… Still it is hard for us to see the progress when we are with her all the time and it is so gradual. This morning we took Ella on a bike ride for the first time. Her head is so large that she fits right into Gaul’s old helmet (that he wore until around the age of 8!). Ella seemed interested although she did fall asleep in her seat on the way back. We rode to Newton Highlands where we had brunch. Ella’s favorite game at the restaurant was, “Throw The Placemat on the Floor and Have the Waiter Pick It Up”. Yesterday I had a scare. We all went to the lake to swim. I took Ella right in. I only put her in about waist deep but I guess the water temperature compared to the air shocked her or something. She started shivering. Then I noticed that her hands looked pale blue. I thought “Oh no A SEIZURE”. After almost having a heart attack I realized she probably just got chillled from the water. Today I took her into our wading pool in the afternoon and her hands took on the same blueish tinge. She was fine otherwise though.

The gauze covering the test dermabrassion on Ella’s cheek has fallen off and the place itself is nearly completely healed. The bad news is that it doesn’t look like it did much. It seems like it will look identical to it’s surroundings when completely healed. That isn’t totally fair to say yet because the area is red and one cannot really see the final coloration that will result. We shall wait and see.

Word came back from the labs and it is definite. Ella is allergic to eggs, peanuts and sesame seeds. There is a chance she will outgrow the egg allergy, but little chance she will loose the allergy to peanuts and sesame. That means we will have to be very careful with her food. Really good news! As of today. Ella is completely off any medication. She has taken the last anti seizure medication (ever, we hope). During the months since surgery we have been gradually reducing her dose till we reached zero today!! She is doing well in her physical therapy sessions and making progress in supporting herself when she sits.

Another thing we have been trying is to let her watch one of her favorite movies while on her stomach. This way she needs to hold her head up, and she does. This usually doesn’t last very long, but she can do it for about five minutes. Her sweet disposition is always with her and she is mostly entertained by what happens around her.

On Friday Ella went through a test derma-brasion. This process is intended to see how Ella’s nevi respond to abrasion. The theory is that some nevi respond well and that when the skin grows back where it was abraided, it grows back looking more normal than before. This doesn’t work with all types of nevi, that is why the test was done. The process went smoothly and took about 30 minutes. We stayed in the hospital for a while to let Ella recover from the anesthesia. Even though it is a simple procedure, it required full anesthesia. Ella was groggy for most of the day afterwards, but recovered by the next day. To our relief it doesn’t seem to be hurting her and it isn’t even interesting her as a focus for scratching. There is a small gauze covering the area and it is supposed to fall off of it’s own accord in about four days. If this works well (we will be able to assess that in a few weeks) then the next process will be dermabrassion for all her facial nevus. The experience made me think of all the future procedures that lay ahead for Ella. All the anesthesia, waking up in the hospital pain, and then the weeks of discomfort following each. It’s just not fair. Ella’s progress is continuing, slowly. She is sitting straighter, in more types of situations. Still not sitting up herself but being a bit more sturdy. She is almost exclusively feeding herself with a bottle, which she goes through very quickly. We had a scare this week. When Michelle took her to aqua therapy, soon after breakfast, before she was able to do anything, Ella started vomiting violently. Also her body started getting covered with blotches. The aqua therapist quickly recognized that as an allergic reaction and a trip to the pediatrician confirmed it. She had two new things that morning, eggs and some cereal that contained almonds. Both are suspect. Some blood was taken and sent to be analyzed. In some cases they can figure it out. We’ll see. She didn’t have enough things to worry about, so management decided to add an allergy.

Yesterday Ella LAUGHED for the first time since her operation! She always had kind of a grunting laugh before but this was a real, hardy, cute-child laugh. I was sitting in the wading pool with her and squishing the plastic whale before her. Water comes out its spout and it squeeks. She heard a clear squeek and that made her laugh.

It was a very nice moment that we have been waiting three months for. Hopefully we will hear alot more of those as time goes on.

There seems to be so much progress now with Ella that it is hard to pick out the more important milestones. The progress is so gradual yet so pervasive that it is hard to put a finger on it exactly. One of the main things I’ve noticed is in equipment. We’ve striped the Kid Cart down to a minimum which means that it works almost like a regular high chair on wheels. On the advice of Ella’s water therapist (Donalee) I took off the head and waist supports. Now Ella has a jumpy chair and over the weekend we got her a walker seat which she sits in, can rest her feet on the floor and propel herself around by foot. She’s not going anywhere yet but it is great to see her sitting up in that with her feet on the flloor! She is also very much into the game: Push Toys Off The Tray And See If Someone Picks Them Up. Invariably someone does. Ella is constantly moving, kicking and banging and patting and scratching and….. I feel so positive about her potential for development that I think sometimes that the main problem she’ll have is how she looks. We keep hearing from cranio-facial specialists how “they can’t do this” and “they can’t do that” which leaves us (me) feeling hopeless. At this point Ella only has teeth showing on the right (big) side of her jaw. There they have grown in crooked, sharp and with big spaces between. I think her eye is getting more saggy on that side too. Her ear is huge. And doctors have told us — it will probably get worse. Great. Wouldn’t hemimegalencephaly have been enough to deal with…….

The past few days have found Ella in a really good mood. She is very smiley, playful and cooperative (well, that is her usual state of being but now, even more so). She got a bouncy chair that hangs from a doorway and that has been a surprise success. The only parts that are painful are getting her in and out of the contraption. Once she is in, she is happy as a clam (are they really that happy?), she bounces herself around, enjoys the freedom of movement and rotates herself. It is wonderful to see her little feet meet the floor. It makes it possible to imagine her standing one day, though we are very far away from that moment. Waving and saying ‘hi’ to Ella now brings (not always but almost) a response. She opens and closes her hand and sometimes raises it. Pretty clear she is trying to imitate our motions. We had a meeting with a cranio facial plastic surgeon yesterday, trying to understand what could be the plan for treating the overgrowth of the right side of her face. We came away with no better understanding but, since her condition is so rare, the doctor asked to study up on it and maybe then he would have better answers. We’ll see. One thing that has repeated itself many times now with Ella is this. Whenever there is a situation that involves many new people, she will go into a subdued mode. Glancing about and studying the people but not interacting in her normal way. I don’t think it is a question of noise. The train, plane or airport didn’t have that same effect on her. It has to do with new people entering her space en mass.