Saturday, December 27, 2014
Lu has still slept A LOT today, but she has had brief periods of time of being awake and smiling. We all even ate breakfast and lunch at the table together and she ate quite a bit! This morning as we were getting her in her chair, Chad and I were commenting on how she had a little color in her cheeks and we told her she was looking more like herself, and a few minutes later this is what she said:
Friday, December 26, 2014
Lu woke up on Christmas Eve morning sounding phlegmy. She had her morning feed and napped, and when she woke up she had the "sick eyes" and was getting pale. She rested throughout the day, and we tried to enjoy a little gathering we have at my Nanny and Pappy's house at supper time, but she just couldn't handle being awake anymore. When I took her temperature before bed it was 102.2. She coughed and gagged and fussed all night long. She slept for pretty much all of Christmas Day, and by the evening her breathing had become rapid and shallow and very, very loud from the gunk trapped in her throat. We do not have a suction machine and she just couldn't seem to get the junk out of her throat. When her fever meds wore off, her temp was back up to 102.9 and then even on the medicine her fever was not going down a whole lot.
So, around 1:15am, Chad called the after hours doctor line through Grisinger and told the nurse Lu's symptoms and she said we had better go to the ER. When the ER doctor first listened to Lu's lungs she said, "They sound a little wet." My first instinct was to shout, "How dare you!" Let me add a little back story here. I have mentioned many times that pneumonia is a very common and very serious complication of Rett Syndrome. Children with Rett get it a lot. I have seen many girls spend weeks in the hospital trying to recover from pneumonia. In addition to the usual ways people get pneumonia, children with Rett can also get aspiration pneumonia from aspirating on their food or drink. So, any time in the past that Lu has been coughing, phlegmy, and had any kind of fever, I take her to the doctor...just to make sure. And each and every time the doctor has said her lungs sound good. And then this morning they sounded "a little wet". However, fortunately, after a lung x-Ray it was determined that she did not appear to have any pneumonia. The doctor did say though that aspiration pneumonia can take a little longer to show up, so we followed up with the pediatrician this afternoon. She said she sounded barely wheezy at all now.
While at the hospital during the night, Lu had two breathing treatments of albuterol, more Tylenol, a steroid, and swabs for the flu. The nurse also was able to suction a bunch of junk from the back of her throat. But, she is still full of snot. So we left with a prescription for a nebulizer to use at home. We have done two treatments on our own and we are staggering Advil and Tylenol to keep her temperature down.
Here is what Lu has done for the past two and a half days:
And here is what she wanted to express while at the doctor appointment this afternoon:
So she doesn't have pneumonia and we are so, so, so very glad for that. But I'll tell you what, when that woman said her lungs sounded "a little bit wet" I felt like she had just slapped me across the face. Or socked me in the gut, or tore my heart right from my chest. It was difficult to accept the facts when we determined that Lu needed a feeding tube, but Rett Syndrome just takes control of her mouth and her body, and no matter what I tried to do to stop her from losing weight, it just didn't help. And seizures, well, there just isn't much I can do about those either. If they are going to happen, they will happen, and our only line of defense at this point is medication. But getting sick, getting "wet" sounding lungs, I have a whole heck of a lot more control over that. And so I might be a bit of a zealot about trying to keep Lu from getting sick. I don't send her to school. I don't let therapists come when they are even a little sick. But I know I can only do so much and we still certainly go out to all kinds of public places and have people over...I don't keep her in a bubble (yet).
The plan now is to just get the sickness out of her before it settles in and tries to become pneumonia, or an ear infection, or whatever else it might want terrorize her with. She's just so pale and lethargic. I miss her. I miss her being awake and talking to us and smiling. I'm a little sad that she slept through Christmas. I am mostly sad because she and I worked really hard for months on homemade presents for everyone and she missed them being opened. She always gets pretty excited to give gifts and see people get excited about them. There's always next year.
Anyway, Chad and I had about two hours of sleep last night and some snippets of cat naps here and there throughout the day, so we are exhausted. I'll post a healthy picture when Lu gets better, which will hopefully be soon!
Wednesday, December 17, 2014
So, about the shaking episodes... Dr. Sasha increased the depakote a second time and I kept a log for two weeks to see if there was improvement. She only had the episodes on 5 out of 15 days, whereas the first update I provided, before the second increase, was that she had episodes on 5 out of 9 days. So Dr. Sasha said to increase it just a little more. She said the medicine is helping. I asked if we were to assume then that they are in fact seizures, and she said, "Right." So, that's that then. Lucy is in fact having seizures. The depakote would not help if they were not seizures.
Other big issues on our list of symptoms to try avoiding to whatever extent possible is pneumonia and scoliosis. Both of these have more of a possibility of being avoided, whereas seizures, and feeding issues, apraxia, just manifest from the disease. I think Lu has been (mildly) sick twice since leaving school almost a year ago, so we are doing well in the battle against pneumonia. As for scoliosis, we do our best to have her positioned straight, but comfortably as much as possible. No curvature has been detected in her spine so far.
When I look at pictures like this one:
I can't help but think back to my oblivious frame of mind at this time in Lu's life, and feel so sad. I guess maybe we did have those 8-9 months where we thought life was going to be run of the mill for her and us, but I can almost never look at it that way. I look at it more like we were fools back then to think that life was going to be easy, or fair. Even up until a few months ago we thought maybe Lu could be in the small percentage of girls who don't have seizures. And two years ago we thought hopefully that she might also be able to continue to eat on her own. And when she was a year old we assumed she would learn to walk. And on September 27, 2009, when I found out I was pregnant, we never, ever assumed that our daughter would have a rare condition that was going to steal so many things from her. It's hard to not take things for granted when you have no reason not to.
Speaking of assumptions, I keep forgetting to mention some assumptions that Chad and I would not allow to be made in these past months of trying to determine what was happening with Lu. First we went to the pediatrician who of course said the could be seizures, but he would talk to a neurologist in Danville. He spoke to the neuro on call, who has never even met Lu, and he said that he didn't even need to see the videos we had to be certain that they were seizures, since she has Rett Syndrome. Well, that is malarkey, and a dangerous assumption to make solely based on her diagnosis. As I have said, there are breath holding spells, and "Rett episodes" and all types of other "events" that can happen in girls with Rett besides just seizures. Unfortunately, Dr. Sasha was out of the country for the month when these first began, so we could not consult her.
When we went to an appointment in Danville with the neurologist that has seen Lu in the past, he too was going to instantly assume they were seizures and just start her on medicine. We said, no, we thought an extended EEG should be done to try and determine if they were seizures. As I have mentioned, none were apparently seen during the EEG. However, when we did finally get up to see Dr. Sasha, she said they just could've missed it since Lucy's brain has abnormal seizure activity all the time. She still wasn't sure, but we trusted her decision to try the medicine because she is a specialist and we always have comete faith in her to do what's right for Lu. And now we know for sure. But, I don't believe it is in anyone's best interest when doctors just make assumptions based on a minimal amount of information and say, "Let's throw medicine at it!" I mean, the arrogance of that first doctor to say that he didn't even need to see the video! Rett Syndrome is a diagnosis, but it is not a mold that all girls came out of. Everyone is different.
I guess my point is, don't let doctors push you around! Not about your own health, or your child's health. I think they do that a lot, to a lot of people, because they think they need to seem important, like they are the alpha dogs and we are all just mongrels in their pack. And I guess another point I tried to make is do your best to not take what you have in life for granted. Back when Lu was 9 months old, the new pediatrician we went to see because we had just moved here encouraged me to NOT have her evaluated by Early Intervention, even though she was not crawling, pushing up on her arms, or bearing any weight through her legs. That was my first experience with saying to myself, "He might be the doctor, but he is not the alpha dog of me and I'll do whatever I think is best for my baby!" And I did have her evaluated, and at that time she (oddly) didn't qualify, but 3 months later, when she still had made no progress, she did. And that was also the beginning of learning what it does really mean to not just be able to assume and take for granted that things in life will go your way.
Rett Syndrome is a bully. It is the type of bully that is relentless. Chad and I are the type of people who stand up to bullies. And Rett Syndrome is the type of bully who just when you think you have fought hard enough to keep it at bay for awhile, it sneaks around a corner and kicks you in the face. I have said more than anyone over these last months that I believed Lu's episodes were seizures, but I still had a really hard time getting the actual confirmation yesterday.
Today's a new day, I'll get over it, to whatever extent a parent gets over these types of things. It just makes for one more thing to worry about when we go somewhere. It's just one more reason why no one besides my mom and dad can take care of Lu if we want to go on a date, because there is just too much to know and do for people who don't see her everyday. It's just one more thing.
Sunday, December 7, 2014
I have mentioned in the past the plethora of chairs that we have for Lu in an effort for her to be: comfortable, upright, or laying down, not falling over, able to sit on the floor, etc. A chair also needs to either have a built in way for her to not fall out (ie. straps or a harness), or she needs to be able to sink down into it. It is just extremely hard to find the right seat for her. Her big bean bag chair is great for laying down and napping, but not great for letting her sit upright and engage with the world around her. Her wheelchair is better for times like that, but if she doesn't have her braces on and her feet strapped into the foot plates, then she slides down in her seat. Sometimes she likes to sit on the chaise, but she falls over to the side a lot. And, when Lucy falls over, slides down, wants to sit up, or lay down, or has any other way she might want to alter her seating position, she just simply cannot. She just can't move her body like that. Therefore, I spend A LOT of time all day long repositioning her. Sliding her up, propping her up, sitting her back up, helping her lay down, it never ends. She doesn't even move herself much in her sleep anymore. Every night before I go to bed I turn her over so she isn't laying on one side, in the same position all night.
So, in what feels like a never-ending search for the "best" chair for Lu to relax comfortably, but still be upright, I discovered the "Chill Out Chair" made by a company called Freedom Concepts, last year. That's right, I have been longing for this chair for Lu for over a year. While it is considered a "special needs" item, it is not necessarily a piece of "durable medical equipment", and therefore it is not covered by insurance, and because it is a "special needs" chair, it has a very high price tag, because that is always the case. Hence, the year of coveting it, but not ever buying it.
But, with the help of a hoagie sale spearheaded by our family and participated in by many more family members, friends, and acquaintances, we were finally able to buy Lu her very own Chill Out Chair! And, I contacted the representative for PA and he had a few demo chairs that he offered to sell me for $500 less than a brand new one! I was ecstatic! So, for anyone else out there who also struggles daily to find a better position for their child or loved one (they come in sizes for everyone), I highly recommend a Chill Out Chair. I recommend you contact your state's rep and see if there are any demo chairs available, if you don't mind that. The cover on ours is actually antibacterial and completely machine washable, plus it matches our living room which was pretty lucky! So it's perfect! And if a person would prefer to buy a new one, they come in dozens of different fabrics and colors so they can fit right in with the furniture you already have.
I don't know if in the pictures you can see what is so special about this chair, but it has a v-shape design that Lu sits down into and the entire thing is made of very high quality furniture foam. So, she sinks down into it enough that she won't fall out, and the foam is like a hug to her, which besides helping her sink in more, it is also like a hug for her, which helps her body stay still.
And she can even nap in it!
So, I am clearly very excited about finally being able to get this for Lucy. She now has a safe and comfortable seat for relaxing, learning, watching tv, napping, whatever she wants or needs to do! As usual, we couldn't have done it without the never-ending support and love of our family and friends.
Here is a link to the Chill Out Chair website for anyone interested in checking them out: