Tuesday, December 23, 2008
We moved Mira up to 100mg on the Lyrica over this past week and frankly, it doesn't seem to make a bit of difference. She seems significantly sleepier and overall a little doped up. She's having just as many seizures and the irritability factor is still present. We will give it a little while longer to see if the increased dosage will 'kick in' (maybe another week or so) but we are not optimistic. Mira's neuro didn't seem to think it would work, but it is certainly worth a try, since it's the only medication to ever work for her.
Mira is still taking the melatonin and still getting up in the middle of the night. We could either bump her dosage up or try a time-released brand of melatonin. The fact that she is able to get in a few solid blocks of sleep throughout the night is a dream (no pun intended) in comparison to what we were all going through a few months ago.
On the bed front, insurance denied our claim (the correct claim this time) with the explanation that our policy has an "exclusion" for anything but a 'regular hospital bed.' It's comical almost to imagine Mira sleeping SAFELY in a bed like that. Why yes, that seems safe to put my 3 1/2 year old child with severe dyspraxia and hypotonia in a bed with 12" side rails elevated off of the floor several feet. That makes sense.
So, we are currently looking into alternative funding sources available to us here in the area, through Mira's therapy group/school and some other local organizations. From what we have been told, there is funding available, we just need to apply. We were able to get part of Mira's stander and KidCart funded through similar groups in the past. We will see what happens and hopefully have Mira in a new bed not too far into January.
I did have a brief conversation with Mira's neuro about having her screened for a recently discovered Rett's Syndrome genetic variant called FOXG1. He is doing some research on it to see if it makes sense to have her screened for it. Mira was screened for MECP2 and CDKL5 (and the results were negative), the only two known and well documented genetic screens for Rett's. FOXG1 seems to be the third that is only recently coming into focus. At this stage, we will leave it up to her neuro to make the call whether it makes sense to have the screen done.
Sunday, December 7, 2008
We didn't really notice any difference in adding the ranitidine over the past week, so we suspect that Mira's irritability is not due to reflux, but most likely, it's all just neurological. We increased her Lyrica dosage to 75mg daily and will hold there for a week, most likely moving up to 100mg next week. We will wait and see what happens.
Sarah and I are still having fun with the camera. We tend to get some good pictures (catching Mira is strange poses as though she is telling some 'big fish' story) but we still need some guidance on getting the most out of the camera. The beauty of digital is that you can take as many shots as you want without it costing a thing.
Mira is fairly consistent in her sleep these days. We have stopped giving her the trazadone and opted to just give her melatonin only. We give her 2.5mg before bedtime and she sleeps pretty solid until about 1am, then wakes up crying. We give her another 2.5mg and she sleeps until about 5:00am-6:00am and usually wakes up crying again, probably because she is hungry. She has been consistently doing this most of this week, which is actually a nice change of pace. I am personally glad the nightly inconsolable screaming fits have stopped for now, knock on wood!
Monday, December 1, 2008
As for the seizures, we are going to do an increased trial of Lyrica after a few days of the ranitidine and see what happens. No one seemed to have any definitive suggestions at the appointment as what to try next in terms of seizure control, so this is our best option for now.
Saturday, November 29, 2008
Mira's sleep over the past few nights hasn't improved much. It took her and hour last night to stop screaming and fall asleep, only to get up for several hours again, from about 3:30am until 5:30am. Part of the problem as of late is that we have a few variables in motion, which included adding Gabapentin, which I will get into later. The other thing is that Mira has been battling constipation for months now and when she has trouble clearing out her system, it only adds to her sleeping problems and irritability. Needless to say, we have been giving her Miralax nearly every day to help with the constipation and it seems that when we miss a day or two, it really throws her whole 'chi' off and we ultimately pay for it days later. Well today was the day for her to clear out her system. This has happened several times over the past month and the result is the same. The only detail that you need to know is that another outfit has been officially ruined.
The trial with Gabapentin has been another medication disaster. I think we are now 0 for 4 with post-Lyrica medication trials. She has been on it for about 10 days now and not only have we seen a marked increase in her irritability (yes this could be attributed to her ongoing constipation, but not for 10 straight days.......) but Sarah and I are seeing a substantial increase in seizures. I swear she was consistently having one every 15 minutes today. Lots of hand wringing on her right hand side and funny movements for a few moments after every seizure too. I thought the premise of the medication was to DECREASE her seizures?! I don't know where we are going to look next, but we have discontinued trying Gabapentin for now. It was a very low dosage and there wasn't anything positive about it.
We did receive a letter back from our insurance last week, giving us written confirmation that they have denied our claim for Mira's bed. The problem is that they denied the claim based on a motorized bed, not the manual one we had requested. I'm not sure what happened during the claim process, but basically they denied the claim based on the wrong type of bed. Now we have to try to get our insurance back on track and make sure they are processing the claim for the right bed this time. More paperwork and phone calls.
After years of fighting to get decent pictures of the kids with our point-and-shoot digital camera, Sarah and I finally invested in a digital SLR. After two days, I love it. No more 5 second delays, takes and retakes, and trying to capture decent shots with the kids! The SLR is so slick and has so many foolproof options that it's hard to take a bad picture with the right lighting. Mira posed like a rockstar this afternoon in her 80's shades for us to test out the new camera.
Tuesday, November 18, 2008
After pleading with neurology for some help, her neurologist recommended trazodone, which is a sedative. It's not uncommon to use for kids with developmental disorders and sleep issues, so we figured it is worth a shot. We started her on a low dosage and have since moved up to 25mg at bedtime. It seems to work for a while, letting her sleep solid usually until midnight or so. She consistently gets up and has a few of her screaming fits throughout the evening, in which we give her melatonin again to help her settle back down. It's a vicious cycle of sleep deprivation and irritability with Mira at night and we are just frankly exhausted from it all. We will be starting her on gabapentin (brand name is called Neurontin) which has a nearly identical mechanism of action as Lyrica, to help combat her seizures. I still believe that the root of all of her sleeping issues are neurological and anything within reason that might help is certainly worth trying.
We have been putting Mira in her stander every day, with her AFOs and she doesn't seem to mind it. She loves to try and throw herself backwards once upright, but eventually settles down and gets used to being in a standing position. It turns out to be a two-person job just to get her in the stander, since she is the queen of wiggling, squirming, and kicking. One has to take extra precaution when she is wearing her AFOs, since they are basically pointed plastic weapons once they are on her feet. They are rough on the chins (mine and Sarah's that is).
We did hear back from our insurance this morning on Mira's bed and our company denied the request. We could appeal, but having heard from other parents requesting similar equipment, it's a long painful battle and unlikely to end positively. Therefore, we are looking at $4850 for the bed. We are planning on calling the manufacturer directly tomorrow to ask them specifics on the bed and see what options are necessary and whether we might be able to reduce the cost some.
A few weekends ago we made the trek to Illinois for a few days at Auntie Aura + Uncle Ware's house. We went to an awesome pirate birthday pool party for Addy and had a wonderful time. Did someone say scurvy dog?!
Despite all of the broken sleep and ongoing trials with Mira, she seems to be taking it all in stride. Some days she does really well in therapy, is happy, and seems to be a chatterbox, vocalizing all day long. The next day, she might be on the opposite end of the spectrum, crying and fussing all day. If there is one thing consistent about Mira is that she is inconsistent - you never know what the day is going to bring. Kudos to my wonderful wife Sarah, who endures the daily nuances and at times, complete chaos in our house (appropriately referred to as 'crazytown' more often than not) day in and day out. You are awesome and I love you.
Monday, November 3, 2008
I have been corresponding via email with Mira's neurologist about what we might try next, but I think we are not going down that path for at least a week or so to give Mira a break. Her neuro wants to think for a while on what might make sense at this point to control her seizures.
Let the good nights continue!
Sunday, November 2, 2008
We haven't seen any sort of withdrawal symptoms after stopping the ethosuximide, nor have we noticed any spike in seizure activity, which is all very promising so far. Let's hope she can go two nights in a row!
Saturday, November 1, 2008
While Sarah and I think we have been seeing slightly less seizures with Mira over the past week, the constant screaming and crying in the middle of the night is more than we can stand. I have said this time and time again - either the medication works or it doesn't and in the case of ethosuximide, it was only partially working and the side effects for Mira were horrendous. We took her off of it today. We gave her a small dosage of melatonin this evening to help her hopefully sleep better.
We are so tired of the medication-go-round over the past few months. It is so exhausting for everyone, especially for Mira. We basically have few options left in terms of medications and are jaded by all of Mira's poor responses to everything we have tried since Lyrica. We will probably push on her neuro to try Gabapentin (which has nearly the exact mechanism of action as Lyrica) if things get worse again, but we are going to give Mira a break for a while. She has to be exhausted.
On a positive note, Halloween turned out to be picture perfect for all of the kids. Great weather and great costumes. Mira was a monkey, Jonah was a banana, and Eli was a Star Wars Jedi. Sarah rigged up Mira's switch on her Kid Cart tray so that when she smacked it, it said 'trick or treat'. Worked like a charm!
Monday, October 27, 2008
Sunday, October 26, 2008
We increased her meds to 3ml twice daily on the ethosuximide last week and I suppose we are seeing less seizures. However, we always seem to trade one symptom for another with many of the medications, whether it be irritability, insomnia, or something worse. I just wish SOMETHING would work positively for her like Lyrica did.
We did manage to find something that Mira enjoys to eat lately (besides her usual daily quota of sweet potatoes) which is pintos and cheese from Taco Bell. Go figure. She gobbles it right up. We figured we could make the equivelant at home with refried beans, cheese and some salsa so we aren't making daily trips to TB and the fact that who knows what is actually in it. I think she tends to get bored with the usual food fare we put in front of her, so perhaps Mira figures ANYTHING is better than sweet potatoes, applesauce, yogurt, or oatmeal.
Sunday, October 19, 2008
Last night she was up for several hours just crying and screaming. There is little we can do to calm her down when she is on a roll - she is just inconsolable. There is always a correlation between her appetite and her poor sleeping. Yesterday, she wouldn't eat a bite at breakfast, lunch or dinner. She finally came around at lunch today and ate a decent meal. Tonight as I am writing, she is still having a tough time winding down and is crying and fussing herself to sleep.
One of things Mira has been enjoying of late is her globe toy. She has had it for ages (she actually has a spare one thanks to her former vision therapist who snagged another one for her on eBay) and has always enjoyed playing with it. Lately, she has been getting extremely intense with it, banging and smacking it so hard I think she is going to pulverize it into a thousand pieces. She will go for 45 minute stretches just beating it into submission until she is exhausted. She then sneaks in a 10 minute catnap in her chair, then lights it up for another half hour. She never gets tired of it.
Mira's orthotics (AFOs) came in last week and she had them fitted and adjusted at Children's Mercy. She will probably wear them for an hour a day, mainly during therapy. They should last a little less than a year.
We are still waiting to hear back on her bed at the moment. I called the beginning of October and they told me it was was going to be another 6-8 weeks before insurance will process it. This doesn't mean they will even approve it. If they deny it, we may have to appeal or go another route. I'm not sure why it takes so long - more insurance red tape I suppose. In the meantime, Mira keeps trying to sit up in her crib, which often results in her banging her head into the rails when she launches forward. She is just getting to big and too mobile for it to make sense for her.
The plan for now is to hold steady on the medication fromt. Mira has some room to go up on the ethosuximide, so we are optimistic at this point that it will help in some capacity. She is currently on about 30mg/kg and can in theory go up to 60mg/kg. We are taking it week by week, as usual.
Wednesday, October 8, 2008
One of the things I did notice being home with Mira all day is just how many seizures she is having. I seriously lost count after 20 or 30. Most of them rarely last more than 5 seconds - where she lunges forward with arms extended for a few seconds, then comes out of it and pretty much goes back to what she was doing. It's the fact that she is having so many of them that is disconcerting.
There definitely seems to be a direct correlation between the seizure activity and her irritability. More seizures - more crying. There were a few moments over the weekend where she was just screaming and kicking like crazy and I could not calm her down, other than get her outside and go for a walk with her - just a change of scenery works wonders as a distraction for her. Her fussiness has continued into this week. The past several nights haven't been all that great too - getting up screaming and crying for 45 minutes to an hour each night.
We did take Mira off of Keppra last week, due to the fact that it wasn't doing anything and seemed to be agitating her. She had a very similar reaction with the Depakote - a poor one. So, we are essentially back in full swing on the medication merry-go-round. I spoke and emailed neurology most of last week, trying to determine what to do next. Mira's neuro agreed to try ethosuximide (brand name Zarontin - there is a generic equivalent which Mira is on) only because it has a very similar mechanism of action as Lyrica. Since Lyrica is basically the ONLY medication that has ever helped, we need to try and find similar medications with similar mechanics, but there just aren't many out there. She has been on the ethosuximide for only two days, so it's too early to tell anything.
Sarah took Eli and Mira to the dentist this morning and both did extremely well. No cavities, no problems, and no screaming!
Friday, October 3, 2008
Monday, September 29, 2008
Mira spends some part of her day, every day, being irritable. Some days it's a half hour; other days it's sun up to sun down. Other days, nothing makes or keeps her happy and we feel like we are always two steps behind whatever she wants or needs. Sarah and I call it LGF (Low Grade Fuss) where she will just whine, whimper, or all out cry and there is seemingly nothing we can do about it.
On a good note, my Mom came in from Saint Louis for the weekend and was able to see Mira's gymnastics moves, live in action. Mira's mobility increases a little every week. She is still popping right off of the floor into a sitting position. She will do it over and over until she is exhausted. It's great exercise for her and she seems pretty excited when she is in an upward position. Who knows - maybe she can solidify a spot on the 2016 Olympic squad soon.
All that aside, she seems to be adjusting to the Keppra fine - no increased irritability for the past few days, then again, no decrease in seizure activity. I thought I didn't see anything for the first two days, then yesterday I swore I saw a half dozen before noon. We will just keep watching and waiting.
We did add some vitamins back into her diet, since going back on additional medications. We added B12 and folic acid, added to the B6 and omega 3 she has been taking for some time.
Sunday, September 21, 2008
We did get a double stroller today, which is something Sarah desperately needed, just to get around with errands and such. Lugging around Jonah and Mira (even while Eli is in school) is a challenge to say the least. The double holds up to 50 lbs. per seat, so I think we have some breathing room with Mira. It also has a decent five point harness in it to keep Mira contained and upright. We already have a Jeep double stroller, but it's more of an umbrella type and isn't very accommodating for Mira or Jonah, at this point. Eli outgrew it a while ago too.
Tomorrow, all three kids gets caught up on their vaccinations, including Mira. Wish us luck - it's going to be fun!
Friday, September 19, 2008
The AFOs should be ready in about 3-4 weeks and we have an appointment scheduled in mid-October to pick them up and try them out. We will see if insurance is going to cover them - so far we have been extremely fortunate in that they have covered just about everything.
Wednesday, September 17, 2008
We are still seeing quite a few head drops from her everyday, which has been going on for months now. No better, no worse - they just happen. Now that she has been sitting up (and staying up for 20-30 seconds stretches) I am always nervous about her having a seizure while sitting up an falling flat on her face. The few times I have seen it, she just sort of bends forward, but still stays up. They don't appear to be strong enough to topple her over.
Mira's neuro recommended starting her on a low dose of Keppra to help with the head drops, but we are holding off until she gets over the congestion she has been battling over the past few days. After her latest bout with Depakote, we aren't too anxious to spin the medication wheel again, but will try if we think it will help.
On Friday, Mira is going to get fitted for some orthotics, also referred to as AFO's - Ankle and Foot Orthotics. Basically what AFOs are leg braces that will help Mira to maintain alignment of the bones in her feet and ankles. As she becomes more mobile, the AFOs will also help keep her from rolling her ankles and provide needed stability, should she try to stand. She won't be wearing them all the time, but will easing into them gradually for a few hours a day. We should learn more on Friday on the AFOs.
Monday, September 8, 2008
Less than 2 weeks after starting Depakote, we took Mira off it. We noticed a drastic change in her mood soon after starting it. She became increasingly irritable all of last week, which escalated to her screaming Friday night and turning purple she was getting so upset. She had a lot of trouble decompressing at night and did a lot of screaming all week. It did nothing for her seizures so we decided Friday was her last day on it. Today she is pretty much back to her old self.
We had a similar episode when she tried Felbatol in early 2007, only we took her off of that after only a few days. That was really awful for her. We haven't seen such a poor reaction to a medication since Felbatol, thankfully, but Depakote was a good contender for worst meds.
We put a call in to neurology to see what her neuro suggests. Frankly, there isn't much left that she hasn't tried, other than Neurontin (Gabapentin), Zarontin (Ethosuximide), Gabatril (Tiagabine) and Inovelon (Rufinamide), which isn't available in the U.S. Since Lyrica is about the only thing that has worked for her, it would be nice to find a medication that has a similar mechanism of action.
Thursday, September 4, 2008
Mira's crabbiness has subsided somehwat over the past few days. Perhaps it is her system getting acclimated to the Depakote and/or the fact that she is eating a little better these days.
I thought I would post some video of Mira sitting up again - this one is pretty amazing. After venting some frustration, she managed to maintain some balance once she was up for about 20 seconds! I thought she was going to a faceplant at first, but she kept right on bouncing around. Go Mira!
Sunday, August 31, 2008
We started Mira on a small dosage of Depakote last week and it doesn't seem to be agreeing with her. She has been much more irritable since starting it, waking up screaming in the middle of the night 3 out of the last 4 nights. I'm not sure what affect it has on appetite, but I'm sure it's not good, Her eating is still hit or miss, which doesn't help the nighttime screaming when you are hungry on top of it. Then again, this isn't really new behavior for her, she was pretty much the same before starting the additional meds. Only now, she seems more irritable, more often. We are going to give the Depakote a few weeks (our neuro says at least 6 weeks) and hopefully it will help with her seizures.
We are still waiting on the cable for her KidCart, which was supposed to be in on Friday. We also have someone from the company that we ordered the chair through to come out and give us an assessment on a new bed for her. Mira has been periodically kicking out the rails on her crib (which she is just too big for anyway) and is constantly hitting her head against the sides when she is moving around. We looked into this bed and are going to see if we can work something out with insurance, if not, we have several alternate plans.
Friday, August 22, 2008
The biggest hurdle we have had of late is Mira's Kidcart debacle. Her Kidcart is basically a three part system - a chair (which is the actual seat she sits in) a high-low base (for getting her around the house) and a stroller base, for getting her around outside. There is an intricate cable system that broke on the chair that enables the seat to lock into the high-low base, as well as her stroller base. It is critical that have the chair working so that we can just get her around without having to carry her everywhere. The cable started to unravel and fall apart several weeks ago. We were proactive, calling the company that services her chair and placed the order for a new cable. As it turns out, the cable broke completely yesterday morning, with the chair locked into her high-low base. Good and bad. Good news is that it locked in the upright position. Bad news is that you can't get her around in the high-low base, other than in the house.
So we called the company to find out the status on the cable and as luck would have it, they hadn't even ordered it yet, citing it was pending insurance approval. Sarah explained our dilemma so they are going to expedite our order. By 'expediting' they mean you still won't get it until sometime next week. Even if we were somehow able to get a hold of a cable, we still need someone to install it. Basically we are stuck trying to get Mira around in a stroller (which offer no support) and lugging her locked chair/high-low base combo, which is clumsy and awkward to get in and out of the car, not to mention weighs 40 lbs.
Sunday, August 17, 2008
And now for the bad. Mira pretty much stopped eating 4 days ago. Her hunger strikes have increased in frequency and intensity over the past few months, but this latest one is a whopper. Sarah and I cannot get her to eat anything these days. She will only drink from a bottle. I don't know whether it's regression, stubbornness or my poor cooking, but she flat out will not eat anything. It is incredibly frustrating. We have tried every tactic outside of force-feeding, but have been unsuccessful for 4 days now. We usually manage to get a few bites (at best) and then the snubbing begins. We just wait another meal or another day until she decides she wants a solid meal.
The third and final issues these days is the screaming. I have no doubt that Mira's eating woes go hand in hand with her sleeping problems. Over the past few nights, she has woken up screaming and inconsolable at various times throughout the night. The first 2 nights were at 2am, then 3-4am the following night, then for several hours last night just before midnight. She has had a really difficult time decompressing before she goes to bed which is not uncommon, but lately has been waking up screaming her lungs for out until she is exhausted. We haven't seen her do this consistently (as in multiple nights in a row) in quite some time. We didn't hear a peep out of her after midnight last night, so a 6 hour stretch scream-free is a good sign of things to come we hope.
On a side note - Eli is really excited about starting kindergarten tomorrow. I can't believe he is already starting school! Jonah is also doing very well - Sarah and I got some great 'cooing' and smiles out of him last night - too bad it was at 1:30am!
Thursday, August 7, 2008
Mira has been sitting up a lot this week, while not uncommon, she has been doing it for longer stretches. Sarah told me that yesterday Mira propped her self up and stayed up for about 15 seconds! I took some video of her doing some good 5 second stretches, which is great. She is starting to gain some balance when she swings upward and almost gets into a sitting position. The coordination is there - she just needs to use those arms as props!
Tuesday, July 29, 2008
The three of us returned Saturday afternoon and Mira was pretty crabby all day, screaming and crying the last 50 miles home. Her irritability continued into Sunday, when I think she finally was able to catch up on some sleep Sunday night. As I said before, her sleeping is usually pretty poor on trips, which tends to affect her eating as well. The combination of poor sleep and her hunger strikes makes for a rough time for her.
We have decided to try a trial of GABA supplements with Mira before we dive straight into the Depakote, again. Mira tried Depakene (same medication, different form - liquid versus capsules) back in November of 2005 and it didn't do anything, so we are not in a huge hurry to try it again. GABA is a simple amino acid that is used for anxiety and seizures, although its efficacy is questionable and unproven (as are many medications mind you), since supplemental GABA doesn't cross the BBB (blood-brain barrier). GABA itself acts as a neurotransmitter in the central nervous system and is suppose to regulate neuron activity. We were going to try GABA supplements last year, but side tracked with medications before we tried it long-term. GABA is pretty safe and harmless, so a trial of it for a few weeks is worth a shot.
Monday, July 21, 2008
I figured the easiest way to give an update is to pick up where I left off, which was right after Mira's birthday. We actually had a larger birthday party for Mira in the park, a few days after her actual birthday. Turned out to be a beautiful day and Mira managed to take some nibbles of of a cupcake. Thanks to everyone for all of the nice gifts, cards, flowers, and balloons. I know Mira really enjoyed having everyone there.
The day after (or the day before I don't remember - again the baby thing as my default) we went to a 4th of July celebration for the city (called Villagefest) where it turned out to be another perfect day out weather wise. Who expected back to back days in the beginning of July at 82 degrees?! I just remember sweating bullets last year this same time at Villagefest and it being nearly 100. Anyway, Eli had a blast that day for two reasons. First, he was able to indulge in one of his new found interests, which is anything and everything to do with the military, particularly guns. The police department has a display of assault rifles, handguns, masks, and other weaponry for kids of all ages to enjoy, I guess.
The annual Village fest 4th of July celebration wouldn't be complete for Eli though, without running into his old pal Sluggerrr. Eli somehow new that this bitter rivalry would come to a head that day, so he opted to wear his Cardinal's shirt, just to taunt Sluggerrr if he saw him. The showdown finally took place about high noon, where Eli caught him out of the corner of his eye. He first threatened to kick him, but Sluggerrr stood tall, picked Eli up and gave him a big bear hug. Eli immediately took off running after Sluggerrr got the best of him. Final score: Sluggerrr 2, Eli 1.The following week we headed to the air show in nearby Gardner, Kansas, where Jonah got his first taste of real summer heat. We didn't last long and tried to stay in the shade (which often meant under the shadow of the Chinook helicopter propeller blades) but survived. Again, Eli whose love these days of weaponry, old cars, and military vehicles knows no boundaries, exclaimed "this is my luckiest day of war" as we passed through the gate. He saw an old 1932 Roadster, a gigantic Chinook helicopter, and a plane armed with guns, all in the first 30 seconds.
Last week was fairly uneventful, although we were dealing with one of Mira's hunger strikes most of the week. She went a couple days and we realized that she was probably constipated. At the same time, she was battling more phlegm issues off and on for a few days during the week. I woke up one morning, started preparing a bottle for her and heard her making some funny sounds. Sarah and I both ran in to find her vomiting everywhere, probably due to the mucus and being so upset. It's funny to know your child's noises and mannerisms so well that you can immediately sense when something is not right. Mira makes a lot of noises and sounds throughout the day, but we know when the sound she makes is not right. Just tonight she let out this weird cry, again we both rushed into her room to find her with her binky caught in her mouth. Time to be done with the binky at night. Most of the time when we hear a strange cry and she has her leg or arm wedged between the rails in her crib. Time to modify her bed.
Now that she is more active, we are finding it really difficult to find places for her to roll around on the floor. Our house is only so big (yes - a whopping 900 square feet) and she rolls into everything. She often gets stuck in weird positions - between the couch and chair, getting tangled in her blanket, etc.
So as it goes, we were getting into a little bit of a rhythm this past week, until we had Mira's EEG last Thursday. The expectation for an EEG is that you keep her up as late as possible (Children's Mercy says to keep her up until midnight, which is impossible, we rarely get to 10:00pm) and then wake her up at 4:00am (again, impossible if we can get her up and keep her up earlier than 5:00am) so that she is sleep deprived (a scenario we are all experiencing at the moment) for the EEG itself and will fall asleep for the beginning of it. This EEG, being her 12th or so, we were actually able to keep to that schedule fairly well and Mira turned out to be a real trooper for the whole thing. I ended up staying with her at CMH until our neurologist appointment afterwards, while Sarah stayed home with the baby. The EEG was scheduled for 7:30am, which went fine, but getting into neurology is a whole other ballgame. We were almost an hour late by the time we were sitting down with her neurologist, which seems to be the norm with that department. Great people, great doctors and nurses - just never on schedule.
Despite having a seizure free period of nearly six months, Mira's EEG hasn't changed much since last November. I don't quite understand the specifics of her EEG (ironically this is the first time someone actually tried to explain it to us) other than she is having frequent spiking and bursts, which interrupts her ability to focus and learn though we have seen considerable progress since she started on the Lyrica. Why does her EEG look like this is the million dollar question. Mira did manage to have one head drop toward the end of the EEG, which they caught on tape. Unfortunately, it doesn't offer any insight as to why - it's just that she has the potential for seizures all the time, but we are only seeing a fraction of them on the surface.
We discussed genetics again and believe we have exhausted all of the testing at this point in time. There is one final test to perform for now, which is a mosaicism of idic15, which would completely rule out any sort of idic15-related scenario for her. A mosaicism of idic15, as opposed to the 'typical' inverted or duplication of chromosome 15 is extremely unlikely, occurring in about 1:100,000 births. Children with a mosaicism (which is merely a mutation, inversion, or duplication occurring in a small percentage of cells as opposed to every cell in the body) are usually much more high functioning than kids with the 'typical' version of the genetic disorder. Our neuro thinks it would be very unlikely, nonetheless, he thinks it's a good idea to finish out the testing. We basically are banking her DNA for future testing and should something come up that they believe she should be tested for in the future but for now, we are done.
As far as seizures are going, Mira is doing about the same. We are still seeing several dozen head drops a day, but nothing more major than those small myoclonics. Our neuro is very hesitant to go any higher on the Lyrica, based on how little is known about this kind of use of the drug. Possibly, Lyrica has lost some of its efficacy, which is not uncommon with AEDs. Our plan is a conservative one regarding her meds. For now we will drop Mira back down to 50mg daily of lyrica and add a small dosage of Depakote in about two weeks. Depakote is in the same family of GABA-related AEDs as Lyrica, so the theory is that it will work in conjunction with the Lyrica to help combat the head drops. It's all trial and error with the medications and it will always continue to be at this point. We will only try the Depakote for 6 weeks unless she is not tolerating it well or it stops her seizures.
Despite the seizures and the occasional hunger strikes, Mira is doing very well overall. Thanks to everyone for your continued support, gifts, and prayers.
Sunday, June 29, 2008
Thursday, June 26, 2008
Eli is adjusting to the baby very well. He seemed pretty freaked out when Jonah was screeching at the top of his lungs the first day back from the hospital, but has gotten used to it by now. Mira cackles every so often when Jonah is crying, since his cry is reminiscent of a mix between a high-pitched baby pterodactyl and a squeak toy. It's distinct to say the least.
Mira started one of her hunger strikes over the weekend and she finally caved at dinner this evening. I was able to get her to finish off a small bowl of sweet potatoes, which is the first 'full' meal she has had in days. Nothing seems to break her cycle of not wanting to eat - we try and switch her meals up and offer up new things, but nothing makes a difference. She just decides that she wants to start eating again and we are forced to wait it out.
Since we have been seeing an increase in her head drops and jerks, we decided to move her dosage back up to 75mg daily. It hasn't been a dramatic increase, but a slow and steady one over the past few months in the frequency and intensity of her spasms/episodes. Sitting through her 1 1/2 hour therapy session yesterday, I saw at least a half dozen. I spoke to neurology yesterday and we agreed to move her dosage up over the next few days. We may just hold steady at 75mg until our appointment in the middle of July and see where she is at then. Still, no full blown tonic-clonics or anything, which is good.
Tuesday, June 24, 2008
Sunday, June 22, 2008
Mira has been really fussy the past 2 or 3 days, going on one of her hunger strikes and getting up in the middle of the night again for hours. Poor Auntie Aura stayed up with her last night while we were in the hospital. It might take a while to get back into a rhythm once the new baby is home, so wish us luck!
Friday, June 13, 2008
Our neurologist (and the neurology clinic in general) doesn't have the experience with kids taking Lyrica, since it is so new to the market. We agreed that it has really been the miracle medication for Mira and we are not about to abandon it yet. This sort of 'reverse' trial dosage is to really see if Mira is really reacting to the Lyrica or to see if we need to look at alternatives or adjunct therapies. I hope that it is just that we need to do some tweaking with her dosage and the aim is to not have her so over-medicated that she isn't able to do anything. There's some action or mechanism at the cellular level (I guess) with Lyrica that has been helping Mira, which could be a clue as to why she is having seizures. If she reacts to lowering the dosage, we will know for sure.
We are also going to push for another EEG, since Mira hasn't had one since last November and all of this new seizure activity would warrant another.
Tuesday, June 10, 2008
We have still been seeing a fair amount of head drops with Mira, so there is concern that the dosage increase hasn't done much yet. We are going to give it another week at the higher dosage and see what happens. If things don't improve, we will either take her back down or perhaps try something different. Mira's temperament has improved a little over the past few days, so there is hope that the increase is potentially helping in other ways.
Mira had her first haircut today! Her hair was getting seriously unruly and Sarah seemed to spend half her day getting knots and rats out of her hair, which Mira really detests. She did great, all things considered. We sat her on my lap while the stylist did some quick work, taking about 4 inches off the back. It took Sarah and I together, with all hands on Mira, just to keep her from flinging and thrusting backwards. Mira is getting very strong and has developed 'abs of steel' from squirming and arching so much.
I posted a few videos on Mira's site (see link to Youtube.com at the right). One is of Mira's new swing that we installed on the trellis in the back yard, which she loves. Sarah managed to find a swing for her that puts her in more of a reclined position and it works great. The other video is of Mira's new found love - hearing the trumpets from Sarah Vaughn's 'Whatever Lola Wants'. Yes - completely random how we discovered it. We had the iPod on one day and Mira was over in her chair just chuckling away, but only at certain parts in the song. We discovered it was the trumpets that really cracked her up.
Last but not least, the countdown continues for the baby. Sarah had some contractions over the weekend, but nothing after that. Still waiting!
Thursday, June 5, 2008
Tuesday, June 3, 2008
She also started one of her hunger strikes a few days ago, where she won't eat anything and it is a struggle just to get her meds in her. She is getting really good at resisting pills, spoons, and is partial only to one thing - her bottle. And yes, the bottle MUST be warm or she will snub it before you can say 'fussbot'.
Speaking of meds, we decided to up her dosage today, since she is completely snot-free and still having some sort of seizure activity. We are hoping that bumping her up will help get rid of the head drops, but we have been down this path many times before. For 6 months, Lyrica was the miracle medication for her and we hope that it continues to be. It will take a few days for her to feel the increase, so we will watch her closely.
Have to go - the fussing continues.......
Friday, May 23, 2008
Mira's skin has cleared up dramatically since we started giving her acidophilus every day. I don't know what it is about probiotics, but if we stop giving it to her, the rash and irritation comes back the next day. You have to love the friendly bacteria. This rash on her face has been around for a long, long time and it worsens when she is rolling around on the floor.
Speaking of rolling, we have been spreading a quilt out on the floor for her every day and she loves having roll time. The trouble is, she is getting increasingly quick and will end up on the hardwood floor in no time. Sarah also ordered a swing online that we are going to start using, in an effort to get her excited about moving around. She is constantly moving and squirming and we want her to be able to be as active as possible out of her chair and not be on the floor so much. We occasionally put her in the swing at the park, but she can only lean forward and it's a lot of work for her to keep her head up and forward in those swings. This swing is in more of a reclined position so hopefully it will work better.
Other than that, not much else happening here. Still seeing some head drops and other quirks, but nothing major with Mira. Sarah is really exhausted these days, hitting the last month before the baby gets here!
Tuesday, May 13, 2008
Sarah and I have been seeing a lot of head drops lately, maybe up to a half dozen or so a day that we catch. I saw a couple this morning and one when Eli and I were out in the park with Mira. Despite this, Mira is still in a great mood, eating well, and drinking well - all of the things she should be doing.
Mira has taken on the name 'Thumper' since one of her favorite things to do these days is kick. If you hold her in your arms, with her facing away from you, she kicks and 'thumps' like a miniature tornado - arms and legs going everywhere. She is also constantly arching her back, which makes it nearly impossible to sit her on your lap, let alone try and hold her. She seems to be in constant squirm mode.
Changing her diaper has become a real challenge, since all she wants to do is throw her legs up in the air (usually at you) all while twisting, turning, squirming, and generally trying to kick her way out position. Don't get me wrong - it's fantastic to see her moving so much and exerting an incredible amount of energy, but it is a total 5 minute workout for Sarah or I just to get her changed.
Wednesday, May 7, 2008
We are crossing our fingers that we don't get rained out for hippotherapy tomorrow, but we have been getting rain off and on today and more is forecast for tomorrow.
We moved forward with the aCGH (genetic micro-array) testing on Monday. Children's Mercy is sending it through a private genetic company for testing, so as it was with the other genetic screens, it will be back in about 6-8 weeks.
I had the pleasure of sitting through a 4 hour city planning commission meeting last night, for yet another cell tower proposal. Cingular threatened back in 2005 to put a cell tower about 50 feet from our house, in the middle of a park no less. After an exhausting fight, they were denied a special use permit and eventually the issue went away in 2006. Now, T-Mobile wants to put a massive 120' monopole about three blocks from us, which we could still see from our back yard. Again, the application was denied. Fortunately, we have really proactive neighbors (some of which are attorneys, conveniently) who are always willing to fight such proposals.
Saturday, May 3, 2008
As I was getting her ready for bed tonight, she had a small head drop and her eyes fluttered very briefly. All of it lasts less than a second. I know it was something, because I have seen it thousands of times before - I was just hoping they would never return. Anyway, Sarah and I have noticed these small twitches/episodes every so often over the past week or so, but we aren't getting too worried at this point. If it happens, it will happen and we have a plan of attack.
Sarah is enjoying a very brief girls' weekend in Saint Louis with her sister and Auntie Tisha. Daddy gets to stay at home in KC and do laundry!
Thursday, May 1, 2008
I was able to take the day off (Sarah still isn't feeling well) and actually saw what Mira goes through on her heavier therapy days. This morning she had PT in house, then vision therapy in-house after lunch, then hippo in the late afternoon.
Tuesday, April 29, 2008
At this point, we are probably going to do one more additional screen, called an Oligo Array CGH (Cognitive Genomic Hybridization). Basically, Mira had some similar genetic screens back in 2005, soon after her seizures started, but it was at a very low resolution, meaning it only picked up major genetic organization. The technology for genetic screening has advanced so much over the past 2-3 years, that they can now perform a much more exhaustive scan at a much lower cost. This scan (neurology refers to it as a 'micro-array') is an extremely high resolution scan that is able to identify many more deletions/mutations in DNA than ever. Before this can happen, we need to clear it with insurance, since it costs about $4,000.
Mira has been battling a cold over the past few days, but is doing alright. Lots of snot. Need I say more? Sarah has been dealing with the same thing now for two weeks. I think she is exhausted from it. I am hoping that Spring is just around the corner........
Sunday, April 27, 2008
............Started searching for a new house. Looked at many, many houses and drove many, many neighborhoods. Fixed supposed cracked foundation to stop leaky basement to prepare our house. Put our the house on the market. Had almost 20 showing in 10 days with no offers on our house. Could not find a house worth putting an offer on in the neighborhoods we liked. Took our house off the market after 10 days and decided not to move. Basement flooded 3 times over the past two weeks since house has been off market. Still have a leaky house. Still have packed boxes in friend's garage and in storage...........
Sarah and I are pretty exhausted from this whole experience, but it has been a very eye-opening one at the same time. We are quite content with staying put and with the market as soft as it is currently, we felt like it was the best decision for us right now. We will deal with the bedroom situation as it comes. We figure we might be singing a different tune in 6 months (the baby is due in 8 weeks!) and might consider making another go at moving. But for now, it's nice not to have so many irons in the fire and it's just one less thing to worry about.
In addition to the house, the winter sickness and colds have been working their way around the house for seemingly forever. Sarah has been battling a cold and some congestion for over a week and it doesn't seem to be going away. It's getting passed around, with Mira and myself getting touches of it over the weekend. The weather isn't helping either - 65 one day then 30 the next.
As for Mira, she has been doing well too. I am happy to report that she is still seizure free, although she has been having what I swear I thought were head drops at one point. They are very subtle and you wouldn't know she was doing it, unless you have seen it as many times as Sarah and I have. She had a few (at least they looked like really quick ones - not completely sure) on Wednesday and then one today. I emailed her neuro and asked and his recommendation was just to keep a close eye on her and we could always up her Lyrica dosage. She has been on it for nearly 6 months and has not had to increase yet, so it isn't unreasonable to think that based on her growth and weight, that she might need to increase the dosage.
Her sleeping has not been so great as of late. She has been getting up at all hours of the night, usually crying out. Most of the time, she settles down after a few minutes, but other times she really gets worked up. It has also been difficult for her to 'decompress' at the end of the day and she will fuss in her crib for an hour off and on before she finally gets to sleep. None of this is out of the ordinary for her - she has had erratic sleep patterns her whole life. It comes and goes.
We don't have anything lined up on the books with neurology, since nothing has changed much over the past few months. We will probably get Mira in for a visit sometime over the summer, unless something changes drastically before then. We haven't heard anything back on the Dravet's testing, which was ordered at the end February. Apparently, it's not uncommon for the results to take 2 months or more. We will keep waiting.
I did manage to upload several videos (see 'Other Mira videos' link on the right side of the page) of Mira doing some funny things. She really gets wound up, often for no apparent reason - one episode I was able to capture on tape back in January. We also found that she gets particularly excited over the food processor and the dust buster. Not sure what it is about certain appliances and noises, but it cracks her up.
Hope everyone is doing well and apologies for not getting our ducks in a row to do the Trolley Run this year. There was so much on our plate over the past few weeks that we just couldn't get organized to do it. The actual run was today.
Monday, March 10, 2008
On the flip side, we discovered that one of Mira's new found friends is pot roast. Sarah found an excellent recipe for a roast and creamy potatoes and Mira cannot seem to get enough of it. I think she would eat is for three meals a day if we let her. She will snub yogurt all day long, but put a spoonful of pureed roast in front of her and she snaps right out of it and chomps it down. Needless to say, looks like we will be cooking it more often!
As of March 2nd, Mira has been seizure free for 4 months solid! She periodically rolls her eyes upwards, sometimes with a 'fluttering' motion, but it doesn't appear to be seizure related. It looks like it is more self-stimulating than anything. She is constantly squirming, so trying to hold her is no longer an option - her movements (back-arching and throwing herself backwards) make it increasingly difficult to carry her and get her in and out of the car sometimes. In her crib, she is often thrashing from side to side, flinging her legs up in the air, and is still putting her hands in her mouth, all the time. Most of this behavior surfaced after she became seizure free, so to us, it's a sign that she is making some progress.
We are doing some spring cleaning around the house, donating clothes and books, shredding old papers and getting the house in order to get on the market in about 2 weeks. In the meantime, we are narrowing our house search to a particular area. This after driving neighborhoods and checking out schools over the past month.
Thursday, February 28, 2008
Dravet's, also referred to SMEI (Severe Myoclonic Epilepsy in Infancy) is usually attributed to a specific gene, referred to as SCN1A (and also SCN2A). Mira will be tested for this, but as with all of the other genetic scenarios that she has been tested for, it is unlikely that she has it.
Monday, February 11, 2008
All things considered, it has been a quiet week for Mira. I plan on talking to neurology toward the end of the week so that we can make sure we have the remaining Rett's results for her appointment at the end of the month.
Monday, February 4, 2008
It was in the mid-60's this afternoon so everyone (well, almost everyone) was able to get out and enjoy the weather here. Sarah took the kids to the park and Eli was able to burn off some energy. Poor daddy was stuck inside all day and had to sit through boring meetings and talk to other nerd architects about exhibition hall rigging capacities imposed on structural mainframes.
Monday, January 28, 2008
We also managed to get an appointment (most likely due to a cancellation) with CMH neurology, toward the end of February, so we are on the books for Mira's next appointment.
Saturday, January 26, 2008
So, I put her down about 7:30pm last night and she slept soundly until about 11:30pm. After thrashing around and vocalizing (still very loudly and Eli somehow doesn't wake up in the room right next to her) in her crib until about 1:30am, she fell back asleep, woke up again at 3:30am, screaming. I then gave her a bottle and she stayed up until 5:30am, dozed off until 6:45am, then got up again for the day.
Mira also goes through bouts of not eating, which she is on day four of not eating much of anything. A few bites here and there to get her meds down, but that's where it ends. She plugs back a ton of liquids every day, but is snubbing everything we offer her these days.
We all have cabin fever lately, since we have been braving the 0-20 degree temperatures here for two or three solid weeks now. Sarah left for Chicago yesterday, for a much needed girls' weekend out and I returned from a Nashville day trip late Thursday night, to a balmy 7 degrees here in Kansas City. Unfortunately, it is just as cold in Chicago and Nashville as it is here - so much for a change of pace. It is supposed to be in the 50's today, so hopefully we can get outside!
No news on the Rett's testing yet - my weekly harassing phone calls to neurology seem to offer no new insight, other than 'genetics is still working on it'. I will try again next week I suppose. The neurology clinic at CMH is going through some rough transition - neurologists leaving and new ones coming in, which means shifting patients and often, frustrated parents. It is extremely difficult to even get an appointment set up now. The books are supposed to open up next week, so that we can actually set an appointment, which we are told is another 2-3 months out after the schedule is posted.
Tuesday, January 22, 2008
Mira is also starting to grab at toys. Tonight, she reached several times for her Abby Cadaby toy (from Sesame Street) which have bells tied around her waist to make noise. She has been responding to Abby consistently and she gets excited every time she hears the bells. Her movements most of the time seem uncoordinated and not very deliberate, but you can tell that she is really trying to do things. Over the past few weeks though, her movements are becoming increasingly purposeful. Sarah said that Mira has been pulling her bows out of her hair throughout the day too. She is making a little progress every day by doing new things.