Ella's Pages

Wow! I’m shell-shocked. We spent 6 and a half hours at the hospital today. This was for the long awaited Cranio Facial “team” appointment made about 3 or 4 months ago. As Etan said, at Children’s Hospital, they use the term “team” very loosely. We were just saying last night in the support group how these meetings are never what you expect them to be. Again, we were passed around to different doctors, floors and departments but most of the time was spent waiting. After seeing Dr. Mulliken (plastic surgeon) for about 45 minutes we ended up waiting at least that long for the Maxillo-facial surgeon to come by. He talked and talked to us. By this time we had found out that the blood that we had drawn yesterday, (yes, we spent most of the afternoon yesterday at the Children’s as well), didn’t come out right and we had to get the test redone. Then we rushed to another floor to see the audiologist. We decided not to have the hearing test done. Okay, back to Cranio-facial…..then down to get fitted for a helmet….then Dr. Duffy’s nurse informed us that there is a suspicion from Ella’s blood result that her liver is not functioning perfectly normal. Then they need a waiver from us and half the payment upfront for the $700 helmet. So we rush around Fegan and Hunnewell buildings totally confused about what we are supposed to do and where we are supposed to go. Anyway, for right now, Dr. Mulliken suggested getting a helmet for Ella to help shape her head and avoid the flattening that is a result of her head turning rightward and from spending so much time lying flat on her back. The other thing he will try to do in May-June is to do a test abrasion on her nevus to see if that helps at all. Otherwise, more major facial surgery will have to be put off for several years. I guess from all this, I did come away feeling hopeful, but exhausted….. We got Ella’s blood labs drawn before leaving and then spoke to Duffy and the on-call epilepsy doc once we got home. The liver fuctions are mildly high. We may have to go in again for blood tests next week. If they keep rising, we could have a problem with the meds. Huh, I’m really tired. That’s an awful lot for one day.

Ella has a new toy. We call it the spaceship. Bought in a yard sale, it consists of two saucers placed one above the other. Ella is placed suspended from the top one with her feet touching the bottom one. This requires lots of effort from her. She needs to balance herself, holding up her head and can also bounce up and down as she kicks down on the bottom part. She seems to be enjoying it, with smiles and yelps when it goes well. It is a huge effort for her and she is completely knocked out after a few minutes on the contraption. Another thing that always gets her attention is social activity, any time there are people around talking, music or movement, and she is much more alert and interested. We had a chance to test that out in several New Year parties. Ella continues to be the best natured person imaginable. Danny found an article that equates the right side of the brain to negative experiences and state of mind and the left to positive. If there is anything to that, it certainly explains Ella’s amazing disposition. We are getting increasingly apprehensive regarding upcoming seizures. It has been quite a while since she had any. We are very thankful for that, but know that with her condition, it is not likely to continue indefinitely. All we can do is to watch her very closely.

Although Ella’s motor skill development doesn’t seem to be progressing one iota, we’ve discovered that she has the sweetest disposition. She almost never makes a nasty face yet she often gives expressions of sheer joy and happiness. Her toothless smile is really adorable. There are some places where she smiles almost invariably: on the changing table, in her bed (especially when she is somewhat tired and really wants to be there) and when there are lots of people around chatting. She seems to like the hum of conversation and sometimes squeeks and coos as if she’s trying to join in.

The funniest thing lately is that she laughs at sneezes. When any of us sneeze close by her, she takes a second, thinks about it and then breaks into a great big smile accompanied by a laugh. Her laughs are these tiny little grunt-type giggles. Then it’s really funny. So we’ve been trying to get her to laugh that way. Up to a few days ago she seemed to be able to pick out the real sneezes from the fake ones, but lately we have been able to stump her.

Ella is six months already. There’s not much to report which on the one hand is good. No seizures or any crucial health issues. On the other, not much progress either. Ella remains very interactive but also very limited in her gross motor skills as Etan wrote last time. I hope the medication is partly responsible, after all, these medications subdue abilities. Also, it might be that the abnormal right hemisphere and the “noise” it emits, hinder the good left hemisphere. We just don’t know so it is very hard in that way.

We have no idea what Ella will be able to do when. I hope, between moments of frustration and disappointment, that once she has the hemispherectomy she’ll be able make good progress but, there are no guarentees. She has a whole repetoire now of “solid” foods that she eats: banana, potatoe, corn, cereal, apples, plus she’ll have a good breast feeding with a meal as well. It seems that her appetite has picked up.

It’s kind of amazing that we have been a whole six weeks without a hospital stay. Yeah!

It is very difficult to assess Ella’s development. We are worried about her missing so many ‘normal’ milestones. Her motor skills are lagging far behind; she cannot roll herself over, does not grab objects and is only now starting to hold her head up. Crawling or raising herself up are nowhere in sight. On the other hand, Ella is very smiley and reactive to interaction. She will be all alert and amused when there is any form of social activity around her. Music or singing gets her very excited. She laughs a lot.
We almost expect her to say her first word any minute, she often makes noises that sound like words in the making. This makes us very susceptible to extreme points of view, either hopeless, helpless about her situation or enthusiastic about how well she is doing. A very mercurial state of being.

We are extremely lucky that we have managed to get back into routine with Ella’s seizures well under control (knock on wood!). She had a great session with Tracy (her OT- Occupational therapist) yesterday. Tracy was impressed by Ella’s ability to hold her head so well when she was in a proped sitting position. She is improving on that but it is going slowly. When Ella is tired her head can just completely fall back and sometimes when I lift her up, she is completely flaccid. Grasping objects is going okay although it is like two steps forward to a step back. She’ll hold things in her right hand if placed there but won’t really do that with her left. I think that much of her slow development has to do with the amount of medication she’s on — especially the phenobarb. I might not be doing much more than rolling my eyes if I had as many teaspoons of medication as I did food. Oh, we’ve returned to solid food. I gave her some carrot yesterday and today she had about two full teaspoons of mashed potatoe. Ella also had another booster of immunizations yesterday and has done fine with no adverse reactions. We are thrilled about that. It seems that we can be more happy with Ella’s physical well-being than with her lagging development (and often groggy disposition). She is very smiley though and so easy to please! It is heartwarming to see her big grin as she looks into our eyes. What a sweetie!

Today we took Ella to her periodic appointment with Dr. Duffy. Dr. Duffy said he was delighted to see Ella in such a stable ‘holding pattern’ and hoped we could keep it up longer. Ella has been seizure free since the last hospitalization. The price is the staggering amount of medication that she needs to take every day (three types of seizure control medicine). After the initial period of getting used to the medication and being excessively drowsy, she is now quite alert and cheerful. In fact she is extremely cheerful, making it a joy to be with her. It seems almost everything amuses her somehow. I wish I could adapt some of that attitude. We are daring to consider ourselves lucky that Ella is doing so well.
From Dr. Duffy we get the feeling that her physiology would lead him to expect much more seizure activity and less ability to control it. For the first time Dr. Duffy was talking about the operation as a certainty. Not ‘it is likely’, or any such vagueness. He was saying, when Ella is at a stage when the surgeons think it is safe to operate. We should proceed with the operation.
I don’t know. The more time goes on and Ella develops though, I find myself exceedingly scared of that operation. What will she be loosing? (they are going to remove half her brain!) The more we get to know her the more I am frightened of loosing any part of her.

Ella seems to be making some progress in the last few days. I guess the main difference is that she seems more awake. She moves both hands and arms quite a bit now and her sides seem to move symetrically when she is laying on her back. She holds toys when we put them in her hand — especially into the right hand. We are thrilled to see her batting her toys in her bouncy chair or under her little “jungle” gym. Things are working out well with our new nanny, Yvette. She just loves Ella and she cooks too! We went to see the geneticist this morning, Dr. Stoler at Mass General Hosp. Her office seemed much more organized and thorough than what we’re used to at Children’s. Dr. Stoler was very thorough. It is important to see the geneticist and the genetics counselor; they try to find out what “syndrome” Ella has (if it has a name and is known). That gives them an indication of how to treat her over time and what we need to look out for. I have a follow up appointment with her in two weeks after she has researched her findings. On the one hand I’m afraid to find out…but on the other hand, what could be worse than what we already know?

I don’t feel as positive as Etan’s last entry. Actually, I find Ella’s situation quite depressing. Day after day, as I can see, she seems to be the same. Often she looks very drowsy most of the day. She doesn’t hold anything consciously and she doesn’t reach for any toys. Even when she sits in her trampoline chair (bouncy chair) her head usually falls to one side. I saw the ultrasoundist yesterday, Dr. Bruce Cohen. We reviewed the 16 weeks, Level II ultrasound films and I showed him the ultrasound films taken after Ella’s birth and the MRI. I don’t understand why if this condition starts developing in the first trimester, how such a gross deformity of the brain can’t be seen in the 16 week ultrasound as if everything noticeable happened from week 16 through 38. His answer was ultimately: her condition is not looked for because it is too rare. Well thanks. I guess Ella’s head control is getting better slowly. She still seems very far from being able to prop herself up in a prone position (on stomach). I stopped feeding her fruits because she was having some constipation and I also read that it is best to start solids at six months. There is no reason to rush it and they could cause her problems.