Ella's Pages

Our appointment with the plastic surgeon was cancelled this morning due to power outages at Children’s Hospital. We have to reschedule on Monday. More telephone calls. Ella’s head and face seem to be growing much larger on her right side. Her skin has a different, thicker texture on the right side and she has much less hair on that side. Her eyebrow is very thin on the right and she has only half on eyelash on the right eyelid.

Today I took her to the pediatrician (Dr. Brown) because she seemed to be having a discharge from her head. Enough to wet her sheet at night. Turned out to be just bad cradle cap. Every little thing I link to her condition, yet much of what worries me may just be normal baby stuff.

Ella’s head is much firmer now and she seems to be directing it to the direction she wants it to go. This makes holding her much easier. Up until recently she was decidedly floppy and enormous care needed to be given otherwise her head would just flop over if you leaned in one direction when holding her. Another discovery is that you can wake her up by calling her name. She opens her eyes and kind of looks around. What is not yet happening is her tracing the source of sound. If you make a noise, it doesn’t seem like she can detect where it is coming from and face it.

Tomorrow we are meeting with Dr. Mulken. He is a well known Craino-Facial plastic surgeon. We don’t expect him to suggest any action right now but we want to get an idea of when things are usually done. From reading other kids case studies, it sounds like the first procedure Ella will go through will be a thing called ‘de-bulking’, namely removing some tissue on the right side of her face in an attempt to make it more symmetrical. Gaul spent a long time today sitting with Ella and describing his latest conquest having to do with a computer game. Ella responded by making a much higher than average number of cute noises as if responding to his drone. Cooincidence? I don’t think so.

I can look at Ella for hours. Especially her face. I guess that’s a good bonding sign. Sometimes I look at her and she looks like a completely “normal” baby. Other times, from other angles, her cheek and ear look huge. It hurts to think what this might mean for her in the future. Her skin seems different on the right side, more callous or something.

She has the line down the middle of her nose indicative of Linear Nevus Syndrome, a skin condition associated with HME. She also has other discolorations on the right side of her face (Epidural Nevus Syndrome). I hope these won’t cause problems in the future. Day by day…

Mom went home today. They (mom and Danny) were here on their way to a trip to Alaska and while that was happening we discovered Ella’s condition. Mom immediately decided to stay on and she managed to change her flight and spend an extra week with us.

We very quickly adjusted to the extended family scene and it was wonderful having her around, helping and being optimistic, (which was great, because we often don’t manage that). It was obvious to me that mom and Ella were forming a bond, It is a pity that Ella won’t see her for a while. Maybe mom will come back in October… If we are lucky.

Ella slept from about 11 pm last night to a quarter to seven in the morning! Yeah! I woke up at 4 am with slightly engorged breasts and no one to nurse. Aba (Dad) woke up shortly after, asked me if I had nursed and then rushed to check that Ella was okay. Up to now she’s been sleeping mostly 3 hour stretches at night so needless to say, I had a very good sleep last night.

It is getting to be so much fun to be with Ella. She is definitly noticing us now, having more control in her head muscles. She focuses for longer times and seems interested. When awake, she is constantly cooing and making cute noises.

She is generally such a happy camper, seeming to be ok with nearly everything (Doctor visits being the exception). Her current state of being makes you want to just concentrate on her now and not think about the problems that lay ahead. But I manage to keep that thought for just a few minutes at a time.

Ella’s gaining much better control of her head and neck. She seems to prefer looking to the right and tends to rely on her heavy cheek as a pillow.

I wonder if she has limited vision on the left side because she glances only momentarily to that side.

We spent two nights in the hospital. It was a very difficult time for us with much heartarche and fear for our little girl. After seeing the seizures getting more frequent and more pronounced we called the Neurology Dept. at Children’s Hospital at 4 am. They suggested coming in the morning to the ER. There we waited and then different medical personnel kept asking us the same questions over and over. She finally had a seizure before them and a number of different doctors — the ER attending physician, the resident, the attending neurologist, etc. etc. — admitted her. Dr. Darras, the neurologist on-call, had her start a large dose of Phenobarbital through an IV. This is called “loading”.

Because they needed to observe her response to the medicine and dosage, she was admitted and they moved us to the neurology ward where Ella had a little raised crib. I stayed with her to nurse and one of us, either Etan or myself, were with her constantly. Thankfully, she responded well to the medicine and her last seizure was at 8 pm that night.

Dr. Duplessi’s news was the worst case scenario. We didn’t dare imagine that possibility before meeting with him. It was totally surreal to us. We were standing in his office, on the left a poster showing the brain as it is supposed to look and on the right the MRI of Ella’s brain. It looked sickeningly different than the poster. A lot of what he said is somewhat of a blur. The minute we got the gist of what he was saying, we couldn’t very much listen to the rest, and kept tuning out. Two things he made very clear, Ella will have challenges ahead (the hospital way of saying problems), and she is very likely to have seizures, as the structure of her brain more or less guarantees it.

Sure enough today, two days after the talk with him, we started identifying seizures in Ella. It is difficult to make sure they are seizures in a little baby. Babies tend to be moving around in a twitching way normally. Dr. Duplessis gave us some identifying marks for a seizure. To our horror Ella started displaying them. With a faraway look in her eyes she rhythmically contracted several muscles in perfect synchronization in a repetitive fashion. This started to repeat itself at an increasing rate. At first it showed up every couple of hours, it would last about a minute. It is a totally helpless situation for you because there is absolutely nothing you can do to stop it and watching her twitch this way makes you want to bury yourself in a deep hole and never have to witness it again.

Our meeting with the neurologist assigned to our case, Dr. Duplesis, was devastating. He examined Ella, quickly reviewed the MRI films as we sat there and he looked at the EEG report. He confirmed the diagnosis, and very solemnly told us that these children face “many challenges” (an easier way of saying that before they even begin life it’s like they’ve been run over by a truck…). He said that Ella is at high risk for seizures and will have developmental delays. he suggested that we stay off the internet (in hindsight I think that was a warning to keep us from complete breakdown and depression). Just as scary was looking at the films on the light box.

The inside of Ella’s head looked so skewed. After that things became a blur for me. I’m not sure I was really all there.