Ella's Pages

Will this always be the case? The minute we remark on how well Ella is doing, the powers that be will decide we are committing the sin of hubris and it is time to show us who is boss. This morning we noticed a subtle type of convulsion taking place in Ella’s left side. It looks and feels like both her hand, arm, foot and leg are contracting simultaneously. It isn’t aggressive or abrupt, but it is rhythmic and it goes on for long periods of time. We called Dr. Duffy and he suggested we bring her in.

We held out for a few hours because the seizures seemed to go away, and also the thought of going through the emergency room again was daunting. Finally at around 4:00pm we brought her in. Michelle spent hours in the emergency room and finally was admitted to the ward. The contractions continue and they are going to run an EEG (that beloved mess of wires). And think of adding a medication. Dr. Duffy doesn’t think that these seizures in themselves are very harmful but is wary that they might lead to bigger and more aggressive ones. Therefore it is important to control them. Ella is in the hospital again…

We are back from our trip to Baltimore for a consultation with Dr. Freeman at Johns Hopkins. They were very professional and efficient. Gave us a feeling of being on top of things and dedicated a lot of time to the examination and dealing with our questions. The picture we got was a bit different than what we expected though. Hemispherectomies are done only in response to uncontrollable seizures, so there really isn’t any discussion of surgery for Ella at this point since her seizures are under control (mostly). When the situation ‘presents itself’; (as Dr. Freeman called it) a decision about a hemispherectomy might be taken. Dr. Freeman said that it is surprising that Ella has had relatively few seizures. The physiology of her brain would have you think she would have more. So that is a good sign. Another was the level of interactivity that she is showing in smiling, laughing and responding. Dr. Freeman’s examination was different than most of the neurologists who saw her till now. For one, he only bumped and measured her a bit. He then proceeded to google and coo with her, interacting with her, making her laugh and look about. The other piece of good news we heard there was that children in Ella’s condition start compensating and having functions from the abnormal side of the brain being assumed by the normal side of the brain, from birth. They don’t have to wait for a hemispherectomy for that process to start taking place. So by the time a hemispherectomy is done, usually, much of the functionality has already migrated. The less than good news (marketing term for bad) is that her condition is a complex one, not one easily defined as mere hemimegalencephaly, since she has more accompanying symptoms such as the hemihypertrophy and linear nevus about which even less is known about than hemimegalencephaly. The trip itself was a lot of fun. We got to see a bit of Baltimore, and also to stop and say hi to Danny, Tammy and Yonatan in New York.
Ella was in amazingly good spirits throughout the trip, constantly making noises, looking about and being generally amused. It is strange how much cuter she is than other babies. I find all others quite boring, yet I can watch Ella for hours. Also when other babies laugh or smile, it is just a mundane non-event. When Ella does, it has the power of changing your mood.

Maybe they are just too symmetrical.

Well we made it home at 10 pm last night. Ella did well all day yesterday and although the Coordinated Care doctor, Dr. O’Brien, suggested staying another night just to be safe, we thought it best to come home. Ella’s color was good, she had perked up and she really was pretty much her old self. What luck to only spend one night in the hospital!

Today she was well for the most part but she did give us a scare this morning when she had one seizure while I was giving her a bath at about 10 a.m. She didn’t have anymore although she did have those nerve wracking twitches on her left side which usually come in clusters. We are increasing the Topamax according to Dr. Duffy’s instruction to 15 mg./day. I have to say I’m quite exhausted. I’m worried about her so much of the time and I get very little done otherwise. It seems that our life has changed very drastically for the time being and it seems like a real treat to be able to meet with friends, catch up on correspondence or just to get out of the house. I have been able to keep up on my studies which are a welcome diversion. But that is about it: reading, writing and Ella!

On our way to Dr. Duffy for Ella’s monthly appointment we were remarking what a good month it had been, how well she was doing with her smiling, interaction and batting things around. I reminded Michelle that we had had a very similar conversation just before the meeting With Dr. Duplessis for her periodic exam. That exam went well; everyone stated how well Ella was doing, no seizures and nice progress. The next day the seizures started. Today Dr. Duffy said she was doing very well and that he was hopeful she could hold this status for a while, (‘holding Pattern’ is what he called it). When we got home Ella threw up and then stopped responding.
Michelle took Ella to the pediatrician who Said She wouldn’t risk it and we had better hospitalize Ella, since she cannot find an explanation for what was happening. We are well versed in the procedure we picked up our pre- packed bags and made our way to the hospital. We went through the same endless process going from the emergency room to the ward, through IV insertion (Painful), six doctors, three beds and seven hours. (How come on ER , the TV series, patients are always rushed through the ER, doctors running along side the beds making clever observations and insightful remarks?) There isn’t anything specific that is going to be done here (at least not now). The intention is to watch her and see if any reason can be found for her current condition. Ella is such a flirt that any nurse that comes close instantly gets a big smile, causing them to think we are just making the whole thing up. It is tough seeing her so lethargic though, I think we are going to be going through a long period such as this after Ella’s surgery where she isn’t herself yet (In fact she will never be that same self again). I Hope this hospital stay won’t be too lengthy and that Ella will wake up tomorrow morning all smiley and energetic (and hungry!) So they will let us go home.

After just seeing too many of those left leg twitches and jerks, some of which seem to involve her whole left side, I broke down and started looking for Dr. Duffy last week. Although I paged him a number of times, he didn’t return my calls and neither did his secretary. Frustrating!!! Finally, I got through to the nurse, Marian, who spoke with a Dr. Tiele. She prescribed something called Topamax right away. This added seizure medication comes in little sprinkles inside a capsule and I need to give her half at night and a quarter of a capsule in the morning in addition to the usual phenobarb. It’s tricky. I’ve been adding it so her vitamin syrup, which she likes on a spoon.

The first two days were good. We didn’t see the jerks until late the following afternoon. But now, these twitches seem to be back to how they were before. They don’t seem to bother Ella much but for me it is hard to watch. She can be smiling happily and then a jerk of the leg, eye, arm or whatever, will instantly surprise her. Other than that Ella has been in a great, happy mood lately. She’s so cute now.

With each new development Ella is achieving, there is a thought that follows asking whether she will retain this ability after surgery? What will be lost? From reading we understand that most kids do well after hemispherectomy surgery. But not all do. How will Ella do? No one can tell us. We are realizing that she is missing some of her milestones already. This isn’t all that apparent when she is alone. It becomes very apparent when we meet other babies of the same age. For now it seems that the milestones that she is missing have to do with her motor skills. She is still quite ‘floppy’. She’s made progress with holding her head up but she is still far from holding it up consistently on her own. Reaching out to grab things in front of her has also been slow, though in the last few days she has done that more than before. On the other hand she is progressing nicely with focusing on us and responding.
She definitely has a smile of recognition now and reacts differently to different people. The tick in her left leg has been persistent and after contacting the neurologist he recommended a supplementary medication that could help. We started that two days ago and noticed a definite change in the left spasms. They almost disappeared.
We’ll see how that goes (they recommended trying it out for four days). We are thinking that those ticks have a direct effect on the limpness of her left side and are hopeful that taking away the ticks might lead to an improvement there.

Last Friday I finally got a hold of the Johns Hopkins Epilepsy Center and discussed our going to Baltimore for a consult regarding the hemisperectomy. I learned alot about their approach from the coordinator. They firmly believe that the younger the better and apparently they feel comfortable performing the surgery on very small infants. Our sense is that they have alot more experience with small babies than does Boston’s Children’s. They don’t do emergency hemispherectomies and their waiting list is about 3 months long. They have quite a network set up with many support families that have been through this before and even reunions every three years. We set an appointment for early November. Other news is that our part-time Nanny (Mary) quit over the weekend and our friend Alida has been helping us out in the meantime. She loves Ella which is very nice. Ella’s been great these past few days. Very alert and sweet, especially in the evenings. She is smiling alot and longer. Plus, she becoming more and more vocal with much cooing, sighing and groaning. She almost sings!

Yesterday was the best day (Unfortunately, Etan was away in NYC). Ella was so interactive and alert. She was smiling so much — except when Tracy the physical therapist set her up for “tummy time”. She started crying then and wouldn’t stop until I nursed her again. She laughs and skirms when Gaul tickles her. She squawks when she’s alone and wants to be with us. When she hears our voices, especially mine, she smiles and laughs no matter which direction she is looking in. She doesn’t turn her head that often and we’re thinking that because of the flattened shape of her head on one side, she gets stuck and can’t turn from her side position. She’s not really reaching for anything consistently yet.

It’s frustrating to me that she is not meeting her milestones, even though that’s not surprising.

I just have to let go and accept what I have no control over. It is hard though.

Even though it is still along way off, we still find ourselves occupied more and more with Ella’s looming operation. It is such a horrible decision to have to make! Either we remove half of her brain or the chances of ‘normal’ development are compromised that is what the doctors are saying. To me it sounds like such a primitive solution. With all the knowledge held today, the knowledge of the brain is so minimal that if there is a problem we just cut out half of the brain. That part of the brain is apparently still doing something, functioning somehow, because when it is removed some functions never go back to the way they were, such as fine motor skills and restricted field of vision.

It seems to me like in 50 years (maybe less) this type of treatment will be considered a gross overkill. The flipside argument is that we can’t afford to wait and see if Ella’s development will go unimpaired because of the situation. The longer we wait the less likely that her left hemisphere will assume the functions of the right.

Very frightening.

Today Ella had a “wellness” checkup at the pediatrician’s (Dr. Becker). Yes, she does get to have some of the routine check-ups too. She also had two immunizations, which were already a month overdue. So as not to give too many at once, she’ll have 2 more immunizations next week. Dr. Becker wisely wanted to check her urine again after the UTI (urinary track infection) and also put her back on a low dose of antibiotics to be safe. It was a real struggle to get her catheterized. Twice the nurses tried and eventually gave up.

We took her home with a catchment bag pulled through a diaper. Later I took the sample, which ended up being about 3 drops, back to the hospital lab. She also needs follow-up next week to make sure her ear infection is not returning. Thursday Ella goes back to Children’s for a Coordinated Care (alias Uncoordinated Care) appointment and to see an opthalmologist. No shortage of doctor’s visits for this baby! I’m always nervous about her getting sick again and having another bout of seizures. Something we’d all rather avoid. The most amazing thing happened while Dr. Becker was examining Ella. We had just finished telling Dr. Becker that she doesn’t really reach for things yet. Then we saw Ella batting at the blood pressure gauge pump dangling to and fro from its rack on the wall above her head. She was hitting it. We were so pleased…