Ella's Pages

So we are all set for May 1st right? Maybe. (read on) Yesterday we spent 9 hours at children’s hospital, really. We met with Dr. Duffy, Ella’s neurologist, Dr. Black the neurosurgeon that will operate on Ella and the pre-op clinic for final tests and instructions before surgery. So here is the thing. The anesthesiologist at the pre-op clinic felt that Ella’s cold might introduce an additional risk and recommended pushing off surgery by three weeks. It is still unclear whether that will happen. Dr. Black would like the final decision to be made by the anesthesiologist who will actually be performing anesthesia rather than the doctor at pre-op. There might also be some sort of power struggle involved. The bottom line is that we will not know till Monday (two days prior to surgery) whether or not it will happen next week. This of course calms us down to no end and allows for relaxed planning and consideration.
So you can imagine we are climbing the walls. An additional piece of information was disclosed with our conversations with Dr’s Duffy and Black. The right hemisphere has grown more than anticipated and has started to encroach on the left hemisphere. The danger from the right hemisphere is no longer ‘just’ for seizures and electrical activity. At this point the danger is physical. The one comforting thing about that is that the dilemma concerning surgery just evaporated. There is no choice. The danger is very real. Another comforting issue was Dr. Black explaining to us why it is virtually impossible for them to remove the wrong hemisphere. This came about because I disclosed I have these reoccurring nightmares where Ella is operated on and they remove the wrong side. He told us he shares that nightmare. I can imagine. Just when we think we have a plan, maybe. Poof! Then again. Maybe we do have a plan. Ella’s cold is getting slowly better.
I hope that by Monday it will be completely gone.

We were hoping to get to the surgery date without any more seizures. That was not to be. Saturday night, Ella started seizing again. After a long reprieve, they were back. The seizures this time were very unpleasant. They were less subtle than the previous ones, and lasted longer. The left part of Ella’s body rhythmically twitching and her face slowly turning gray, eyes turned straight up. The effect on us is nearly as bad as the effect on her.
The seizures repeated themselves during the night and the next morning. For the first time ever we used the emergency Diastat gel. This is a rectal syringe that is supposed to stop seizures in their tracks. To be used only with prolonged seizures. Sunday morning we contacted the pediatrician’s office and went in to see him. It seems like Ella has an ear infection and that could explain the resulting fever and seizures. (We hope) She is now on antibiotics and Tylenol, and has not had any more of those big seizures. We have noticed a return of the periodic left leg ticks that started out the previous seizure attack. Lets hope that these will also fade away.
All we wanted was to get to surgery without another bout of seizures, was that too much to ask for, We should have learned by now… Ella is back to being her smiley little self and was totally into listening to music last night. One week to go.

Yesterday was the MRI. The good thing about it was that the process went smoothly. No long waiting time. Ella went right to sleep after taking the sedation meds. One hour later we met her in the recover room and soon after she was her old self again. Whew! I guess that is not too surprising since the medication they use for sedation is similar to phenobarb which she is very accustomed to already.

The alarming part about the MRI was looking over the films afterwards. Again, we see such an abnormal looking brain. To me, a layman in this, it seems incredible that she smiles, eats, laughs, moves. The left side of the brain is dwarfed by the right and the two can’t even be compared as the same organ. She has a huge opening on the right side that would be the ventricle. Of course, it depends on the angle the cross-cuts are taken at but on none of them is there any basis for comparison. I guess we’ll hear on Wednesday if everything still looks good for the operation. I’m just hoping there won’t be any new, unexpected information that will have some ominous bearing on the prognosis. I guess we, Etan and I, are feeling the anxiety as we get closer to the operation. I enjoy focusing on other things (like studies and work) but disturbing thoughts about possible complications and what we are going into are never far, especially when Ella has been so cute and lovable lately.

She is such a little doll.

Tomorrow Ella is going for an MRI. This is the last test before surgery. After this, next week we have a pre-op meeting where hopefully, we will get more information about the process ahead; the logistics and timetable. We are also meeting with Dr. Black and Dr. Duffy to hear from them what they see in the test results and what the plan is surgically based on that. Ella is blissfully unaware of what lays ahead. The only way to go into surgery in my opinion. Especially this type of surgery. She has been doing very well these past few weeks. No huge strides forward, especially not with motor skills, but a steady increasing awareness of her surroundings, sounds, music and the discovery that almost all her fingers can fit in her mouth simultaneously. She is also very clever about managing to scratch herself ‘down there’ when being changed. At this point she seems to me to have ‘normal’ sleep patterns. She is no longer drowsy and out of it for long periods.
Could be that since we haven’t increased her medication, it is in effect lower than before since she is growing and putting on weight. Her body is probably more accustomed to it by now. I just hope that she doesn’t break into seizures before the operation, maybe she can hold on just a bit longer.
The looming date ahead is making it very hard to concentrate on other things, such as life. With the current state of things in Israel and the world, maybe that isn’t such a bad thing…

The preparations for Ella’s surgery begin. Today I took Ella to Children’s Hospital for the first pre-surgery test, an EEG. The procedure went very smoothly with Ella asleep during the whole messy setup. 28 electrodes were glued to her scalp and then her brain activity was recorded when she was asleep and when she was awake. Several things showed up on the EEG both disturbing and reassuring. Though I am no EEG expert, I could pick up several things going on in Ella’s brain activity. First of all the activity is totally asymmetrical. Her left side churns along calmly with smooth peaks and valleys. Her right side is a picture of chaos. Sporadic sharp peaks and valleys, much more aggressive than the left. The technician confirms this: ‘definitely seizure activity’. This is quite amazing, she is living with a constant drone in her brain. It is a wonder she manages to do anything. Another thing that was very apparent was the fact that when Ella moved around, (moving her arms and legs). The brain activity on the left side closely corresponded to that movement. Though, the activity on the right side showed no change whatsoever. This has led me to several conclusions (some can be classified scientifically as wishful thinking but still…):

1. Ella, though she seems to have the seizures under control is in a constant state of near seizures. We are lucky that they aren’t breaking through to form full blown seizures, but they are there, right under the surface.
2. It seems like the activity of even the left side of her body has already started migrating to the left side of the brain. When she moves her left arm, the left side of the brain shows that activity. The right doesn’t.
3. This makes the argument for surgery stronger since it doesn’t seem like she will be loosing functionality with the loss of the right hemisphere. Also the constant chaotic brain activity in the right side is, for sure, affecting her ability to develop.

So there.
Still I cringe every time I think of the impending surgery ahead. Later today we went for a last helmet adjustment (since surgery is going to stop the use of the helmet). And then to draw blood for levels. Ella was very good and totally interested in the noise, people and action in the hospital.
Don’t worry Ella. We’ll be back.

Sometimes we get caught up in the visualization of the different problems that will face Ella throughout her life (and that is totally ignoring the trials everyone goes through growing up). Her first is coming up, the hemispherectomy. On May 1st, half her brain will be removed. Through her life she will have to go through a series of operations to correct the structural abnormalities stemming from her hemihypertrophy. These will involve jaw and facial operations, dental surgery and plastic surgery to deal with the additional mass in the right side of her face.
There will have to be all kinds of treatments dealing with the nevus on her face (the darker birth mark) and sporadic hair growth on the right side of her scalp. How will she manage through all these often painful processes? I just can’t imagine.
I am hoping her calm and happy disposition won’t be affected by all of this, but is that at all possible? Well, first she has to survive the hemispherectomy, we all do, and come out the other end with flying colors, overcome her developmental delays prove that you can do anything if you put half your mind to it.

This past week has been quiet. No seizures and Ella went to two Passover Seders. The week before last we met with Dr. Lewis at Shriner’s Burn Hospital. I had been referred to him through Dr. Stoler, the geneticist, who thought he had some experience with nevus (birthmark) syndromes. I thought he was possibly young (younger than Dr. Mulliken) and maybe had some up-to-date insights or knew of new treatments for such abnormal blotches of skin as Ella has. The meeting was disappointing because right away he pronounced, despite being of advanced age, that he had never seen anything like what Ella has before, which always puts a parent right at ease,. right? I wasn’t sure if he was referring to her nevi or to the hemi-hypertrophy in her head. In any case, he didn’t have anything new to add. Instead he seemed very interested in Dr. Mulliken’s plan of treatment and who we had seen over at Children’s. Other than that, we found the nursing and administrative staff at Shriner’s to be very attentive — different from the frantic disorganization of Children’s. The building looked brand new, exquisitely decorated (for a hospital) and a we learned that Shriner’s are older men who wear dark purple fez caps. But, Dr. Lewis didn’t contribute much to our knowledge of treatment options for Ella’s skin problems. He did say that it would be worth doing a biopsy of the nevus to distinguish if what Ella has is epidermal nevus or sebaceous nevus because the later is harder to treat and has a greater chance of developing cancerous tumors later on. This was the first time I’d heard that there was any difference between the two. The whole situation just made me tired. I spoke at length to the other family in North Andover whose son just had a hemispherectomy last November. he is doing well in general and the mother is pleased, but the whole experience sounded harrowing.

Ella was very happy over the weekend and very talkative.She seems to want to participate in our conversations When we talk directly to her she is usually quiet, however, if we talk to each other around her we find that she interjects with a particular sound that seems like an attempt at a word. This is wonderful and gets us very excited. Of course, with each tiny new development we start wondering in the back of our minds how the impending surgery will affect her. The fear is that there will be some losses; we don’t know what or for how long. That makes for alot of uneasiness. Thoughts of the surgery loom over us like a big black cloud. Got a call from the Perkins School for the Blind today. They will be assigning us a therapist to work with Ella on vision skills assuming that she will have a field cut. Sounds like they have many services over there that complement Early Intervention including parent/child/parent support groups. That was an effortless connection thanks to a referral from Dr. Mayer, the vision specialist at Children’s. Dr. Mayer is the most efficient person I’ve met over there. We got her medication levels results back from the blood tests she had last week. The pheno and dilantin levels are down. Dr. Duffy wants to go up now on the Topamax and not the other medications which could hurt her liver function. We are hesitant to even go up on the Topamax when she is doing so well but I understand his wanting to play it safe until the surgery.

Just to confuse us, Ella has started using both left leg and arm much more than before. In fact it seems like she is using them even more than their right counterparts. Where is this coming from? We have no clue. There are two scenarios here as far as I can tell: 1. Since her right hemisphere, which is supposed to control the left of the body, is abnormal, and possibly not contributing anything, the left hemisphere has already started compensating for that and has started taking over the functionality of controlling the left arm and leg. 2. The right hemisphere is not as useless as we are assuming and it has found ways to muster up some functionality. If this is the case it is quite depressing because the operation is going remove that hemisphere with any functionality that it has.
The case for surgery is still overwhelmingly strong. I am incredibly scared of it and of the whole idea of removing a part of the brain. But, it seems like their really is no choice. Ella has hardly shown any signs of development in the recent months, definitely no motor skill development. The other point making it a no brainer (the ultimate no brainer really), is that we have yet to hear of, to read about or to encounter a child who had hemimegalencephally, didn’t go through an hemispherecomy and did well. Those scenarios always end up in uncontrollable seizures, regression in development and dire consequences. I hate the idea of surgery, yet see no other choice.

The Avocado Episode: Dr. Becker (pediatrician) thought Ella is not gaining enough weight. Although she was convinced Ella is not getting enough milk from what I described, I asked about high calorie foods like avocado which Ella liked when I gave it to her a couple of months ago and then stopped (Yvette thought it too heavy). Wednesday night I got a little carried away I guess and let her eat to her heart’s desire. The next day she refused to eat or drink anything and she cried in waves throughout the whole day. By the evening she hadn’t slept and she had a slight fever. Rectal temperature-taking worked like an enema and by the next morning she was at least willing to take some milk. By Friday night she was okay again. We gave her only milk until yesterday (Sunday). We were happy to have our normal, smiley, contented Ella back by the end of the weekend and happy to have gotten through the whole episode with no seizures. The pending hemispherectomy now looms before us. Dr. Black’s office gave us a tentative date: May 1st. We have alot of hope for her development after the procedure, but there are no guarentees about anything. Now that we even have a date, it becomes that much more real and scary.