OK so I have yet again failed to keep this journal updated. We were in survival mode here for a while, as Claire kept getting illness after illness. She had a head cold for 3 weeks, and the postnasal drip made her vomit that entire time. At least we think so, none of her Dr's could find any other reason for the vomiting. Whatever the reason, she lost all the weight she had gained since going off the diet. Twice we ended up in the ER for IV fluids. The second time I all but begged to be admitted, I felt so overwhelmed and helpless, thinking "Obviously nothing I am able to do for her at home is good enough, she's only getting worse!" But the Dr. couldn't find any reason to justify admitting her.. At that point, she wasn't even keeping down just pedialyte at a rate of 30cc/ hour (roughly one shot glass of fluid per hour.)  Long story short, after being sick for most of the last 3 months, she's been well for about 2 weeks now.

The felbamate is helping her tremendously! She was seizure free for 5 days, and since then has had anywhere from 1-5 seizures a day, down from 40 per day just 2 months ago. Her movement problems have gotten more noticeable, her arms and legs are never still. That is a cerebral palsy thing and not a seizure thing, just something we need to live with. She's comforted by tight holding and swaddling, so I am doing that and just massaging her as much as I can. She's making some great progress in physical therapy. She's gone from very low muscle tone all over to tight tone in her elbows and ankles especially, so were having to work extra hard to keep her full range of motion in those joints. But the good part of that is she will now hold her head on a plane with her torso when you grab her hands and pull her from laying down to sitting. She still can't hold her head from falling forward, and she can't always pick it up once it falls forward. We are working on her back and neck strength, and if this keeps up I can imagine her sitting on her own at some point in the future.

Nora's doing great, she is just a sponge for words and actions.

Alright, that's as much time as the girls are going to give me for blogging. Bye!

I have fingers in many online pies, I keep my FB up to date with little bits of Claire news, but this journal has suffered. I'm sure I've said it before, but I will try to do better from now on...

So lets see, as of the last post we were fresh out of the hospital after discontinuing the ketogenic diet. Claire is now a lovely 23lb 5oz, and that extra 3 lbs is making such a difference in her overall look.

As expected with her condition, she has good and bad periods. For about a week, ending Monday morning, she was so active and social. I forget if I posted that she now makes brief eye contact, and she smiles, 2 things I never thought I would get to see. Since Monday she's been kind of out of it with a lot of myoclonic jerks. What can we do but ride it out and wait for the next good time?

A few weeks ago, I had to give her Diastat for the first time. Diastat is a rescue medication for a seizure or cluster of seizures that doesn't end. Claire was having tonic seizures that were then generalizing, over and over again. The Diastat basically puts a person to sleep so they can't seize anymore. A few hours later she woke up and was OK.

Claire's primary seizure type now is tonic, lasting up to 10 seconds. She will do this 20-40 times a day depending on if she is having a good or bad day.

Nora turned one on Sept 2. Man did that year fly! We had a birthday party for her on Sunday, she mashed some cake and loved her presents. Chuck loved the excuse to light a bonfire :) Nora is such a joy! A couple weeks ago she took her first steps, and really hasn't wanted much to do with walking since then. Today, however, she has started letting go of the couch and walking to me, just a few feet but still it's progress. Above all else, Claire has taught me patience, and that babies will do things in their own good time.

We ended up doing 7 nites in the hospital, and got home yesterday. Claire is completely off the ketogenic diet, and I can't wait for her to put on some weight. She still has the upper respiratory infection, but she's doing some good coughing.
Now I would never wish to be in the hospital, but I do miss having just me and Claire, and being able to give her my full attention. She's noticeably less chatty since we got home, because I can't focus on her all day long :(

We are on day 3 of our inpatient stay to discontinue the ketogenic diet. It's sad that another treatment has failed, but I will not miss the side effects. Claire was 20.3 lbs when we were admitted on Monday, down from 25 lbs last August when we started the diet.
Today she is taking 50% ketocal, 50% regular formula, and is doing just fine. No increase in seizure activity. The stay to discontinue keto is pretty long, we'll probably be in until Saturday or Sunday.
Claire hasn't had a long term EEG in almost 2 years, and it's so much more difficult to move her around now than it was. She's 35 inches long, totally hypotonic, with wires sticking to her head that attach to a backpack with a wire running to the wall. Fun fun fun. So she's spending most of her time in bed or in her wheelchair, since holding her is next to impossible.
And oh my God do I miss Nora.. 

The depakote wean is going better than I could have dreamed. We are down to 50mg morning and night, and her afternoon dose is completely gone. We started at 200mg 3x daily, so we've really come far. Claire still has good and bad days, but her good days are better and better, and her bad days aren't as bad. There's a possibility she said "Dada" last week at PT. I wish I had been there, but I was home due to wisdom tooth extraction. Apparently she was in the pool with her therapist, with Chuck watching from the other room (it's like 100 degrees in the pool room). Therapist asked "Do you want your daddy to come get you out of here?" and Claire said Dada. I don't know what to make of it. Even on her best days, the only sounds Claire makes are some random coos and the occasional shout.

Speaking of words, Nora said "duck" in response to me handing her a toy duck on June 1. She said it a few more times, but not since. Little booger. She started doing "mamamamamama" over the weekend, which is music to my ears. Her sleeping pattern has been erratic and terrible.. some days no nap, bedtimes pushing 10PM, waking up several times a night. Hoping for some normalcy in that area really soon.

Gotta go.. lots to do, and very little motivation or energy to do it.
 

So last ThursdayClaire got the stomach bug.  It had been long enough since Nora had it that I thought we were safe, but I guess not. Thursday and Friday she vomited after both of her bolus feedings. She even vomited her meds on Thursday. Thank goodness I didn't see too much of an increase in seizures after that. We had to feed her in small amounts, and make sure not to touch or move her for an hour afterward. Just miserable stuff. She handled her overnight feeds just fine, all we did was reduce the hourly rate and dilute the ketocal more than usual.
The worst part about it all is Claire is very high risk for aspiration pneumonia, and when she vomits there's high potential that she'll aspirate it. The last time she was sick was on Saturday, and so far her lungs are sounding clear. We're still on pneumonia watch for a few more days though, just because I am paranoid mommy at times like this.

We went ahead with the baptism on Sunday, since she was doing OK at that point. I spent all morning worrying she'd be sick at church, but as it turns out, Nora was the one I needed to worry about. Pretty much as the pastor was crossing her forehead, she farted. Loud enough for at least the first 5 rows to hear. Chuck and I did our best to keep straight faces, but it wasn't easy. I've gotta find out if anyone has a video of it- I'm fairly sure we'd make it onto America's Funniest Videos.

With the decreases in her depakote, Claire is becoming more alert and responsive, which is awesome. The only downside is that she now cries during her seizure clusters. It's heartbreaking, all you can do is hold her and soothe her until it's over. From what I understand, it's normal for kids with West syndrome to do this.. figures the one time she picks to be normal, and it's a negative. But, it's awesome when she is awake and looking around and responding to touch, etc.

Wow, I had a friend point out to me how long it's been since I updated here.. let's see, what's happened since March.

Claire continues to make developmental progress. I forget if I posted that she has learned to roll onto her side.. umm.. her head control is getting better every day.

In medication news, we continue to try increasing / decreasing meds, in hopes of replacing depakote w/ Banzel. So far so good. She has withdrawal symptoms for a couple days after each depakote drop, but so far nothing unmanageable. She has developed an almost normal sleep pattern- awake with a few power naps during the day, and sleeping for all but a couple hours at night. We'll be inpatient for a few days this summer for a long term EEG, to see if there's any change. This will also help the Dr. decide if the ketogenic diet has been helpful enough to make it worth all of the side effects. Weight gain is just about impossible on the diet, so Claire remains at 22 lbs, and is very bony. And don't even get me started on the constipation.. Oy. She just had an increase in calories, and we have a manageable poop routine, so as long as she gains some weight, I am OK with continuing the diet.

Mother's day weekend, Claire sustained a fractured wrist :( Daddy was carrying her up the front steps but tripped. He took almost all of the hit, but Claire still dinged her arm on the front door frame. It's so minor that the ortho who reviewed the xray didn't even have a splint or soft cast placed on it. We have a follow up today with a pediatric ortho.

Nora is 8 months old and awesome. She has trouble in her eyes :) Once she's mobile, life is going to get very interesting. She still doesn't laugh, but when amused wrinkles her nose, squints her eyes, and makes a hissing sound through her nose. She claps for everyone and everything. Just a charming little person. At the moment, she has a stomach virus, so she's understandably been difficult. I'm just trying to keep her happy, making sure she gets rest and as much fluids as I can get into her. She'll be home with her great-aunt today while I'm out with Claire, thank goodness. 

The girls are being baptized on May 24th. We found the same dress in both their sizes. I just hope that Nora still fits it by then. Not sure if she's lost any weight with being sick, but before that she was almost 21 lbs. She's wearing 12 month clothes, but is just about ready for 18 month. Yikes.

OK, been a while, let's see..

Claire is doing OK.. she is just finishing up a round of antibiotics for white blood cells in her pee. I'm going to have her tested again, and if they are still there, I'll push to have her checked for kidney stones. It's a possible side effect of the ketogenic diet, and my Mommy-sense is tingling.

Nora sits up all on her own now, it is so cool to watch. She's just becoming so much her own little person every day.

Really just average daily life here, not a lot to post.

Saturday nite Claire had a particularly nasty seizure cluster thing.. I'm not really sure what to call it. She had fallen asleep at 5PM, and woke at 10PM. Her spasms were really strong and close together, only a few seconds between them. There were some tonic events mixed in. Then she was out of it and twitchy. The whole thing went on for about 1/2 hour. A nurse was here, which was a great comfort. It's only the second time in her life that I've seriously considered using diastat. But, she was returning to baseline between events, and her O2 sats were good the whole time, so we rode it out. She stayed up for an hour or two, then fell back asleep until about 7 the next day. She never sleeps that long at a time, so it must have really taken a lot out of her. She's been OK since, so I'm not sure what that was all about.

Nora's on the floor at the moment with no pants on, swinging around a (clean!) diaper that was supposed to be on her booty by now. But she wanted none of being diapered, and I've learned to pick my battles. I'll try again in a few minutes, and in the meantime hope she doesn't wee on the blanket.

Update- She did in fact wee on the blanket and her shirt somehow.. all good after an outfit change.

1. Claire started Banzel Tuesday nite. She was sleepy during the day a lot yesterday, but other than that it looks to be going well.
2. For her half-birthday on March 2, we gave Nora bananas. She was not pleased.
3. Nora loves pears.
4. Time to put away the 6-9 month clothes and start wearing the 12 month clothes. Nora is a very big girl.
5. I am so eager for Nora to crawl, though I am sure I will regret ever saying that once she is into everything.
6. Claire is pulling her legs up and rolling to her side more frequently now, it's so great to see.
7. Claire has 3 appointments today, all here at home thank goodness. PT, Special instruction, and a visit from her home nursing case manager.

Let's see...

Claire has been doing great at PT (at the center) the last 2 weeks. I had mentioned to the therapist how Claire moves a lot more after a bath and during outfit changes, basically when she's naked. So for the last 2 sessions, she's done PT with Claire in just a diaper, and it's been helping a lot. She rolls onto her side by herself, and lifts her knees up into her chest, all kinds of things that she otherwise wouldn't do.

We also picked up her DAFOs last Friday (her ankle braces). They look like little plastic boots with some velcro straps. We're getting her used to wearing them, and this coming Tuesday she will get to try out a stander. If all goes well, one will be ordered for here, so she can use it for a little while each day.

This past week has been nutty.. I got sick with lord knows what.. it had characteristics of a cold and of a flu, but didn't last long enough to be the flu I don't think. The worst was the exhaustion.. literally to the point where Nora was screaming for 5 minutes right next to me before I could get it together to help her. A few days later Chuck got sick too, he's just about over it now. And Nora's had a cough for the last 2 days, and is on antibiotics for a double ear infection. Fun fun fun. Thank God Claire has so far avoided it all. Basically I've been doing just enough to keep the girls and myself alive, and it shows. I've got lots of house stuff to catch up on. Very glad that's over.

Claire has a neuro appt this Thursday, I'll update afterward.

One of the unexpected great things about being Claire's mom is that I see amazing things everywhere I look. I wonder if the people involved have any idea.

It is very easy to dwell and make myself miserable over all of the moments that I will never share with Claire. Much of the time, it's an honest to goodness effort to be happy. But over the 2 years since her diagnosis, I've gotten much better at turning it around, and instead of mourning what will never be, appreciating what is.

What got me thinking this time around, and what ultimately made me write this post, was a trip to ShopRite the other day. I got stuck behind a school bus. Of course my first thought was, "Christ, now I get to stop every 300 feet while some booger faced 7th grader stares at me out of the back window." But then, at each stop, I saw little kids getting out and running up their driveways, and moms waiting with open arms and big smiles. This is one of those times that I had a choice, sadness or seeing the beauty. First, there was the deep pang imagining what it would be like to see Claire running at me with her little backpack on, but then I just started smiling. I only hope those moms realize how awesome a moment that really is.

Lest anyone think I am trying to sound like Saint Tamsen here, I'll admit that I still mourn more than I admire. And, if Claire wasn't special, I too would never realize the everyday miracle of a child springing out of the bus after school.

Long time no post.. through the course of last week, Claire recovered from her pneumonia. She looks and sounds wonderful. Her therapy schedule is getting back on track. (She has 4 each week- in home PT, OT, and special instruction, and also PT at a center.)

Not much time, just wanted to let everyone know that after 5 weeks, Claire is finally well.

Claire ended up being seen in the ER Tuesday night, because her abdomen distended twice more during the day Tuesday. We realized we were about to be snowed in on Wednesday, and took the better safe than sorry approach. Xray showed no obstruction, but she was F.O.S. (If you have to ask, you don't want to know). When she got home, we did an enema with very little result, which was worrisome. But Wednesday night, she finally went.. oh man she went. Respiratory wise, she sounds worlds better. Still a lot of congestion, but she is actually coughing well now and I am able to get most of it out before she swallows it again. Hooray for albuterol!

Yesterday we moved Claire's bedroom downstairs. She's not so big that we can't get her up and down the stairs safely, but we had the time and the motivation. Plus, in a couple months, she'll likely have a stander, and the larger bedroom will definitely be needed to store and use it.

In all this commotion, a major milestone was not announced. On Monday, Nora rolled from her back to her belly, and neither of us saw it! She was on a folded blanket on the floor, Chuck was on the couch w/ Claire right next to her, but focused on Claire. I was in the other room. Next thing I know he's calling me to get her, because she was on her belly and angry.

Speaking of angry, I hear her now. Gotta go, duty calls.

Claire's been sick for a month now. We went back to the Dr. again this morning because her eye was oozing yellow stuff and looked irritated, and was not responding to antibiotic eyedrops. Also her cough and congestion had gotten really bad again.

So, the infection in her right ear is back.. she had it a month ago in the ER, but not 2 weeks ago when we saw the Dr. Her eye is not pinkeye, but likely irritated from the ear infection, and from her habit of rubbing at her eyes when she is upset or hurting. The big thing was the congestion, the Dr. heard a definite crackle in one lung.. and crackle = pneumonia.. so apparently she wasn't really getting better when we thought she was. An albuterol treatment in the office improved her lung sounds, so we now have a nebulizer and albuterol here to use as needed. She is on another 10 day course of antibiotics. If this one doesn't fix it, we're looking at the possibility of hospitalization for IV antibiotics, so please send good thoughts.

But the really tense part was this evening, when her abdomen became distended and firm. This has never happened before.. I keep wondering what she will throw at us next, and she keeps finding new and awful things to answer me. Anyway, I called the Dr. and explained what was happening. He said to vent her, and to give her a glycerin suppository. Venting is when you plug a tube and syringe into the g-tube, and open it up to allow stomach contents and air to come out. He said as long as these 2 things fixed the distension, and she had a BM from the suppository, we would be OK. But, if she had no BM and/or her abdomen stayed hard, we had to go to the ER to check for a bowel obstruction. Here comes the really gross stuff.. while venting, several syringes full of partially digested food and LOTS of mucous came out. Her belly became soft and normal sized again. The suppository yielded one little turd, enough to keep us out of the hospital. Thank God that Nora had already gone to bed at this point, and we were both able to focus fully on Claire. I don't think any words can quite get across how intense it all was.. the entire time this was happening, she was coughing and whining and screaming, and I kept suctioning mucous out of her mouth and nose. I hooked her up to the pulse-ox a few times during this episode to make sure she was breathing OK and getting enough oxygen. Just a really scary situation.

At the moment, Claire is sleeping peacefully on the couch, breathing softly and smoothly. After one month of respiratory infection, I think we are maybe seeing the light at the end of the tunnel. When is my girl going to get a break???

Now I am just waiting for her nurse to arrive so I can update her on today's events.. then I am going to bed, where I will sleep the sleep of the dead, at least until Nora wakes me up. 

Claire's almost done being sick.. she was doing well for a while, but got quite bad again the 11th-12th... bad enough we saw the Dr. again on the 12th. Today she's looking quite bright and cheerful, with only a few coughing fits. We went to clinical PT (as opposed to home-based PT) today and she did well. And, she's even "talked" a little today, which she hasn't done in a long time. Very cool.

Nora's doing well.. her schedule's shifted a bit, so morning nap is no longer 2-3 hours, but maybe an hour.. afternoon nap is now where it's at, and when I get most of my house stuff done. Claire still has no sleep-wake schedule that I can discern, so I try to roll with her and give as much attention as I can when she is awake and Nora is otherwise occupied. The two of them keep me busy, each day I try to get one load of laundry and one other household task done.. most days I succeed, go me!

Really nothing much to report. Just figured I'd post because I haven't in a while.

Both girls saw the pediatrician Monday, Nora for her 4 month check up and Claire for her ER follow up.

Claire's doing well, the coughing fits have all but stopped and she looks 1000 times better. She had PT Tuesday and OT today and did well at both. A little tight and out of practice because I've been coddling her, but OK.

Nora's a chunk-monster, 16lb 12 oz, placing her in the 97th percentile for weight. She wants nothing to do with rice cereal, so we'll try again in a few weeks. Dr. said we could easily wait til 6 months for solids, since she obviously doesn't need the calories. She was also 24 3/4 inches tall, about the 75th percentile. She'll pull herself to sitting now if she takes your hands in hers, but cannot stay that way without support yet. She reaches and grasps for toys. And she *loves* to make noise. Her smile knocks my socks off every time I see it, even when she wakes me when it's still dark out. She's decided that 5:30AM is a fine time to start the day.. sometimes I can get her back down, sometimes not. But, she still consistently takes a nice 2-3 hour morning nap starting at 8 or 9. Gotta love it. As it gets dark, she starts to get very fussy, and usually doesn't go down for the night without a screaming fit. She just gets so overtired. 

Dad's service is on Saturday. Just a nice simple memorial, I think he would approve. Even having his ashes here, it still doesn't feel quite real to me.

2008- If you can't say anything nice, don't say anything at all. With the obvious exception of Nora's arrival, I don't have much good to say about this year. To put it bluntly, it's been one wad of crap after another. There were just enough good bits to keep us all from running screaming into the night, I suppose.

The year ended much as it began, a middle of the night trip to the ER for Claire due to fever and respiratory distress. This was Monday morning at 2AM. Her fever continued to climb after some tylenol, and her O2 sats were dropping as low as 91.. not scary low, but low for her. We had to use her oxygen for the very first time- I had her on it for the entire ride to the hospital. In the "thank God for small favors" category, Nora was spending the night with Grandma J. The intent was for me to get some sleep, but it's very good that both Chuck and I were able to go to the hospital without worrying about Nora. They gave her the rocefin shot, which was not nearly as horrifying this time as it was in January. She was found to have a nasty ear infection in one ear, and a minor pneumonia in both lungs. Certainly explains why she'd been in a funk all day Sunday. I wish she had any way to tell us what was wrong.. all we ever know is that she is unhappy, and from there it's a guessing game as to why. Monday evening, I had her on oxygen a lot, but she hasn't needed it since. So now she is a few days into a 10 day course of antibiotics, with a pediatrician follow up on Monday. She's doing OK now, a lot of wet coughing, but she doesn't know how to handle the resulting phleghm, so we're having to keep a good eye on her to keep aspiration to a minimum.

Good riddance to 2008, with hope for a brighter 2009.

I've not talked much about my father here, but he's been battling emphysema for over 20 years. Over the last few years, his health had declined rapidly.

My father passed away this morning. I got the call yesterday morning that he had hours left, even with the most aggressive treatment. I decided to let them begin comfort care, and they took him back to the nursing home and his familiar room. I was able to stay with him all night, and I held his hand almost every minute. He was never conscious, thank goodness, and was heavily medicated to avoid any pain. This morning, around 6:20, his heart slowed to a stop, and he peacefully left. His ventilator was turned off, and his long brave fight came to an end. I think he was ready.. the last time I saw him conscious was Tuesday. He was too weak to talk around the trach, but when it was time for me to leave he squeezed my hands in his and smiled. I keep thinking I can't possibly have any tears left, but they keep coming. I miss him, and I miss all of the stories and answers he took with him.

He'll be cremated, and I'll be holding a memorial service in January to allow his family, scattered around the country, to enjoy Christmas with their families and make travel arrangements.