Monday, October 31, 2011

Sleep Deprived Halloween

Reagan was up A LOT last night. She didn't go to sleep til close to midnight and then she was back up by 12:30pm fussing. I stayed up with her from 12:30-4:40am! She wasn't happy either. She was either fussing, full out crying, or just being an all around pain the entire time! She woke back up at 5am and Mike laid down with her til she fell back to sleep. It was a looooong night. To top it all off, our nurse sends me a text just before 7am telling me that she's sick and not coming in today! Talk about perfect timing!! Reagan slept in (of course she did), but I had to get up and give her all of her medicines. Thankfully my mom was here so she was able to help me out a little. I decided to go ahead and take her in to the pediatrician's office...just in case. We woke her at 11 to take her in and she woke surprisingly happy. Her pediatrician said she has a little fluid in her ear but nothing horrible. She is congested though and gave us some nasal spray to calm down the inflammation in her nose. She's been pretty good most of the day, although at times she can be very high maintenance and fussy. We were able to take her trick or treating down our street tonight though. She seemed to enjoy it and she was probably the cutest Dorothy ever!!! Here are a few pics...







Sunday, October 30, 2011

Off day

Reagan slept well last night. She'd make a noise or move around here and there but she never fully woke up. Today she's been happy, but "off". She sounds a little gunky. Her nose is a little runny (I don't even remember the last time she had a runny nose), her throat sounds a little gurgly (I think it's sinus drainage), and she's got a low grade temp. She's also been very high maintenance. One second she's fine and smiling...the next she's got a fat lip and she's about to cry! Lots of little fusses out of her throughout the day and a few meltdowns. I'm not sure if she just feels lousy or what. We may take a trip in to the pediatrician's office tomorrow (just in case). It's actually been a loooong time since she's had to go the pediatrician. Reagan has managed to stay well for quite a while now (thank God). Hopefully she's feeling alright for trick or treating tomorrow! Our house is all decorated and we've got our costumes ready. We're doing a Wizard of Oz theme. Reagan is Dorothy (she's had the costume since last year), my mom is Glinda the good witch, and I'm the wicked witch! I was supposed to be the good witch but I've been demoted since the costume won't fit over my belly. Oh well. In Reagan's eyes that's probably about right. Grandma is the good one. I'm the bad. I have also been feeling a little respiratory something or other in my chest and my allergies are a little worse than usual. Hopefully we both get better quickly. We don't have time to be sick! Please keep us in your prayers!! Also please keep this little boy, Max, in your prayers. His dad works with Mike...he's in really bad shape and they're taking him off life support Tuesday...he needs a miracle!

*For those of you that didn't see the blog yesterday...go back and watch the video of Reagan. It's too cute!*

Saturday, October 29, 2011

Cutest video ever

Reagan had a bit of a rough night last night. Normally when she's nauseous, it only affects her while she's awake. Last night she kept waking up making choking noises, I'd get up and run in her room to check on her, pat her a little and then she'd be OK (a little gurgly, but OK). She did this a lot. I was really worried that she might be getting sick, because she did sound a little congested. But this morning (when she woke for good), she was smiley and happy. Not at all what I was expecting. She was still a little gaggy and didn't seem quite herself, but I think she was tired more than anything. She was very clingy all morning. I held her and she'd fall asleep on me for short periods of time. Finally this afternoon she started acting more herself. She was more energetic and less gaggy. She was even "singing" along with my mom to Phantom of the Opera! (I have the cutest video of her doing this when we were in the Bahamas and today she did the exact same thing! So precious!) Our neighbor had another garage sale benefiting the UMDF this weekend. I feel bad because I wasn't able to help much with the actual sale this time around but I did collect things in advance to sell at the garage sale. She's become determined to raise as much money as possible for the UMDF...she's a pretty amazing woman. Unfortunately, some of our neighbors don't really like the fact that we've had two garage sales...regardless of the fact that it's benefiting a charity (so we're not going to be able to do this anymore). It's pretty crazy when you think about it. For two days they are slightly inconvenienced with more cars than usual parked on our road. But we live with the effects of Mitochondrial Disease every single day! This is for CHARITY people! But people don't care. They don't have to live this life. They don't have to think about their child dying from a terrible disease! It's true, I guess...you just don't care about these sorts of things until it affects you personally or someone you know. Well, hopefully we can get more people to care...about sweet little girls like this one!
This is her singing in the Bahamas (video shot on cell phone)...pause the blog music at the bottom of the blog so you can hear her!


video

Friday, October 28, 2011

Nausea

Reagan slept in today til NOON! I'm not really sure what that was all about. She woke super smiley and happy. She did miss her one and only Friday therapy. Grandma surprised us this morning and showed up a day early (with her new dog). Reagan was definitely happy to see her grandma...we haven't seen her since the Bahamas! Although Reagan was in a great, easy going mood today...she's been very nauseous. Tonight especially. This afternoon she was really just a little chokey here and there, but tonight she's been gagging nonstop! I drained her g-tube and lots of fluid, foam, and air came out but it didn't help her nausea AT ALL. I'm just hoping she'll be able to fall asleep and sleep it off. Please keep her in your prayers!!!

Thursday, October 27, 2011

Ophthalmology appt and more CA pics...

Reagan slept well again last night. She was still asleep when we put her in the car to head to her ophthalmology appt. She actually woke at 8am, when we took her out of the car and put her into her stroller. She woke just as smiley and bright eyed as can be! They dilated her eyes and then we waited for almost an hour and a half to see the doctor (I really thought they had forgotten us). The doctor looked in her eyes and checked her pressures and everything looks good. So good, that we don't have to come back for a year. I asked her about Reagan's vision. I remember reading something on another blog about a test they can do to tell what exactly a child is seeing. Our doctor, who is actually a neuro-ophthalmologist and an expert in CVI, said there are two different tests available both of which are completely useless to us. One is wrought with false positives and false negatives and nearly unusable and with the other the child has to focus on a specific spot (and it's actually fairly accurate). Problem is...how are you going to get a child like Reagan to focus on a specific spot for any length of time?? You can't. Another thing she said, which makes perfect sense, is if the child has significant developmental delay...there's really no way for them to convey to us what they are seeing. Their vision could be WAY better than what we think but there's just no way to get an accurate response out of them to know one way or the other. So, unfortunately, there's really no way for us to know (at this time) how much or how little Reagan actually sees. The doctor did say (and has said before) that CVI often gets better over time, so it's possible we'll see her vision improve. For now we'll just take what we can get. She has been a precious girl today, that's for sure. She had vision therapy right after we got home from her appt (what's the chance of that). She was trying to fall asleep, but we forced her to wake up and she actually did a great job. Her vision therapist came with the OT from the school and they did a session together. Then Reagan's teacher came. She was fine for the first 45min but then she fell asleep! She took a little nap and then had PT this afternoon. She stood in her stander for over an hour (which is good since it's been a while) and then has been precious, even laughing for us tonight. Such a sweet girl.
(Any Rangers fans watch that game? Painful.)


More pics from our trip to San Francisco (the day Reagan had her brain scan)...


We drove over the San Francisco-Oakland Bay Bridge and by stopping on Treasure Island we somehow managed to avoid paying the toll (which is good because we didn't have any cash at the time)!



View of San Francisco from Treasure Island (on an overcast morning)




At Ghirardelli Square...yum!



Mike is watching crazy people swim out in this cold water! (behind the ship you can see Alcatraz)




We ate at this restaurant almost 11yrs ago on our honeymoon! We ate clam chowder and listened to live jazz music (and watched a guy out on the street jump out from behind things and scare people walking by)!



This is that same guy!! Still doing the same thing 11yrs later (he's hiding behind the branches)!!!

Shutterfly Christmas Cards

It's that time of the year again!!! I LOVE Christmas! It truly is the most wonderful time of the year (and this year I'll be just as round as Santa)! Christmas is coming and that means Christmas cards from Shutterfly. In the past we have always had cards with our dog Bear on them. And then Reagan came along and it was all about Reagan. Now, getting a good picture of her to use on a card is not always an easy thing (or the dog for that matter), but hopefully we'll find one (all we need is one) and we'll have a super cute card again this year. Last year I also bought calendars for the whole family. A new pic of Reagan for each month! You can't beat that! It was an easy way to give a super personal gift that everyone loved!!! They also have other gifts you can give like ornaments, mouse pads, stationary, coffee mugs, even playing cards that you can personalize with any photo!!! I would highly recommend going to Shutterfly for your Christmas needs! I know I will. For those of you that are interested...please leave me a comment at the bottom of the blog and I'll draw names to give away 25 free cards to three different people!!

Are you a blogger? Want a chance at 25 free cards this holiday season? Register here:
http://goo.gl/DDw7Q

Wednesday, October 26, 2011

Big news and more California pics

Reagan slept well last night. She's still not going to bed til right around midnight every night and then sleeping in late...Mike thinks she's still on California time (I think she's just on Reagan time)! She woke up this morning around 10:30am when her pump started beeping! She was all smiles but she did have a big throw up shortly after waking up. She was fine after that and didn't do it again (or even seem to be nauseous) the rest of the day. She had lots of therapies again today. First OT, then speech, and then speech again in the afternoon (through the school system). She did well for everything, especially after a short nap (she was even vocal during speech for once). She's been a real sweetie today. She's still a mover but I think the chorea has calmed down a little. Her movements today seem to be more purposeful, less chorea like. Hopefully she sleeps well again tonight...we've got an 8am appt with the ophthalmologist!! And in other news...Reagan is going to have a baby BROTHER! I wanted a girl and Mike wanted a boy, but in reality we just want healthy. Everything looks fine on the ultrasound so please keep those prayers coming for a happy, healthy, EASY baby this go round!!!


Here are more pics from our first full day in CA. Reagan had an echo/EKG in the morning and then we drove out to Half Moon Bay and then came back for an appt with Dr. Enns in the afternoon...



The sweetest most well-behaved girl to ever get an echo!!


The view on the way to Half Moon Bay...



Half Moon Bay Golf Course...








The beach at Half Moon Bay...


The Ritz Carlton at Half Moon Bay...






If you look closely (in the middle of the pic) you can see a black fin sticking up. We SWORE we were seeing whales but apparently it was just large porpoises because they don't get whales in this area.

Tuesday, October 25, 2011

Lots of energy

Reagan slept fairly well last night. I heard her up talking a couple of times but she did go back to sleep. She actually slept in til 11am or so this morning. She woke up with smiles and has been in a good mood all day. Super vocal..."talking", squealing, and laughing nonstop! She's been a ball of energy! A little hyperactive really. Still having lots of chorea, kicking, moving so much she's hard to hold. Her heartrate is elevated too. Staying in the 130s-150s. I still think the propranolol did something to upset the calming effect of the clonidine. I haven't seen chorea like this since before starting her on daily clonidine. As of now, we're going to stay off the propranolol until we see if she can get back to the way she was before. That's one reason I hate making changes when things are going good. Please keep it in your prayers that we can get her stable again and in another stretch of good days!! On the pregnancy front...I'm 20weeks along and tomorrow I have my ultrasound to find out the sex of the baby. Mike is (of course) hoping for a boy. I'm hoping for a girl (we have boxes and boxes of girl clothes). But more than anything we are both just hoping for happy & healthy!! Thank you for your continued prayers!


Reagan asleep in her own king size bed our first night in California...

Monday, October 24, 2011

Pumpkin patch pics & EPI-743 explanation

Reagan did not go to sleep until 1am last night (of course she would do this, now that we no longer have a regular night nurse)!!! She did wake up once and fussed a little, I checked on her and noticed she kept pulling her hair, so I put a sock on her hand and eventually she settled down and went back to sleep (not sure when, I was so tired). Then she slept in til almost 10am this morning!!! Turkey. She woke up happy (and laughing) but has been quite hyperactive today. Lots of chorea, some crazy laughs and yelling out (and lots of clear fluid draining from her stomach), but overall she's been happy and very vocal. She still has the "bad day smell" but that may just be from her being so active and sweating more than usual. I'd like to think that she's going to skip over the bad days this go round. Please keep that in your prayers!!! So here's a quick (semi-simplified) explanation of EPI-743, the study medication that Reagan is now taking. EPI-743 is a molecule that is similar in structure to CoEnzyme Q10 but works 1000x more efficiently and readily crosses the blood brain barrier. Patients that have a mitochondrial disorder have a problem with one or more of the complexes of the electron transport chain which is responsible for generating energy for the body to function. Molecules like CoQ10 and EPI-743 help the electron transport chain to function more efficiently by moving electrons down the chain. They don't necessarily fix the problem, but they help to bypass the problem area. Studies have been done in cells from patients that had a deficiency in the transport chain and were destined for cell death and EPI-743 actually rescued these cells from dying. As as scientist, things like this really excite me. Now experiments don't always translate to success in the body...but just talking with random nurses and doctors at Stanford...they have seen drastic improvements in the children taking this medication (which is even more exciting)! So we're hopeful. This medication is literally the ONLY medication out there that actually treats mitochondrial disease (instead of just treating the symptoms of the disease). Reagan will start off at a dose of 50mg 2x day, then it will increase to 100mg 2x a day, and then it will increase to the highest dose of 100mg 3x day. They gave us a 7week supply when we left and then they'll ship the next shipment to us. We're supposed to go back after she's been on the medication for 13weeks (but they are all booked up that week), so instead we'll go back on week 12 (Jan 12-13...I'll be 7 weeks from my due date). When we go back they'll run some more tests (including another brain scan), take our account of any improvement or side effects we've seen, and then we can decide if we want to keep her on the medication or take her off. From what I understand, nearly everyone chooses to keep their kids on it (indefinitely). Please let me know if you have any more questions about the medicine itself or the protocol that Reagan is on...I'd be happy to answer them (if I can)!

Below are a few pics from our trip to the pumpkin patch in CA...

Loved this little piggy.









Hand in the mouth (and hand in the hair)...of course!

Sunday, October 23, 2011

Off day

OK...so no pictures and no in depth explanation about Reagan's new medicine EPI-743...but we had a long night and Reagan's been a little high maintenance today so I haven't gotten much done. Definitely in the next couple days I'll get to it! Reagan did not sleep well at all again last night. She woke up literally the second we laid our heads down on our pillows! She was very restless and moany so I had to pick her up. She fell back to sleep fairly quickly, so I was able to get her back in bed maybe 15min after I picked her up. She woke again at 3am fussing and did not want to go back to sleep. Mike and I both got up with her at first. I stayed up with her from about 3:45-5am...when she finally dozed back off. With all the traveling and going non-stop, this lack of sleep is really wearing on us! She woke this morning still very on edge and moany. I laid her down for a second to change her diaper and she had a big throw up. Poor girl. You could just tell she didn't feel well. I have drained a ton of clear fluid from her g-tube today. She was feeling pretty lousy most of the morning and then this afternoon she started up with the crazy laughs for Daddy. She's actually preferred her Daddy today and been a little more fussy for me. She's still having tons of chorea and her hands and feet are a little clammy, but she's been less moany this afternoon so hopefully she feels a little better. Please keep it in your prayers that she can get some much needed rest tonight and that she starts feeling better soon!

Saturday, October 22, 2011

Houston, we're home!

Reagan didn't sleep much at all last night (and neither did we). I'm not sure if it had anything to do with the new medicine (which could potentially give her more energy) or not. She was very restless and a little moany. Mike laid down with her the first shift and then I took the second. She did finally fall asleep sometime around 6-6:30am. She woke a little fussy, lots of chorea, and very sweaty. Thankfully she cheered up and was a good girl for us! We packed up all of our stuff and left for the airport early. In Houston we waited through ridiculous lines to get through security and then had to walk all the way to the other side of the airport because our flight was in another terminal...needless to say we barely got to our flight on time. Today in San Jose things went much more smoothly. Of course, it always helps to be early, but there wasn't really any line at all to get through security. The only hold up was them scanning every single medicine out of Reagan's bag...individually. And this time instead of having to walk half way across the airport...our terminal was the very first terminal as we walked out of security! Reagan was as good as gold on the flight. She just sat in her chair pulling her hair and chewing on her fingers. (What milestone is that? She has that one mastered!) Toward the end of the flight she was getting a little vocal and we couldn't tell if she was getting agitated or just talkative. She held it together though and we all made it home safely around 7:30pm. Reagan started yawning and then fell asleep sometime around 10pm. Hopefully she stays asleep, because we all could use some rest tonight! (I promise to post pictures tomorrow and give everyone more detailed info about the new medication, EPI-743, she's taking.)

Friday, October 21, 2011

First dose EPI-743

Busy day, busy day. We haven't rested much on this trip, that's for sure. Last night Reagan was a little restless. We're not sure how much actual sleep she got, but we could hear her moving around when she should have been sleeping. She was asleep when we woke up this morning though, so that's good. The urine bag the nurse put on her yesterday after her procedure resulted in a failed attempt with a diaper full of pee, so this morning I had to put a new bag on her and try again. Thankfully, this mommy knows all too well how to put these bags on and I was able to get a good urine sample before we left this morning. We were supposed to meet the study coordinator at 9am, but she called us about 15min before (while we were still driving) and asked us if we could come an hour later...so we went a had a nice breakfast with our extra time. There were a few setbacks in meeting up with everybody, the coordinator was late and it took us a while to find the MRI nurse to draw Reagan's blood from the iv, so we didn't do the first dose of EPI-743 til 10:50am (almost two hours later than we expected). They did one blood draw before the medicine, a 1hr post dose blood draw, a EKG 2-3 hrs later, and a 3hr post dose blood draw (all of this went very smoothly because we were able to leave in the iv port from the day before, so they wouldn't have to stick Reagan multiple times today). Afterward we were free and clear to go! They gave us a cooler bag full of medicine to take back with us. We left the hospital and drove back toward Half Moon Bay so we could stop at one of the many pumpkin patches we saw a couple days before. We took lots of pictures...go to this post to see some of them! Then we drove along Hwy 1 toward San Francisco. What a beautiful drive. We made it to San Francisco (again) and walked along a pier where we had really good views of Alcatraz and the Golden Gate Bridge (less foggy in the afternoon). Then we drove to the other side of the bridge so we could see the sun set over the Pacific. We found a nice waterfront place to have dinner...where we had the best clam chowder and seafood pasta ever (although we're both quite sick now from all of the rich food). We got back to our hotel late and couldn't find any parking (they had some event going on and it was packed with non-guests). They had even filled up every last handicap parking place (not one car had a tag or plates)!!! We were a little irritated by that. Mike dropped us off a the front and had to park a long way away. Reagan was good all day today but she's been having a lot of chorea...moving nonstop and pulling at her hair constantly. She was looking tired and dozed off for a bit tonight but then woke suddenly and frantically, so I sure hope she goes back to sleep and gets some much needed rest tonight!!! Keep us in your prayers, we have a long day tomorrow heading back to Houston!!!










For some reason it keeps turning this pic sideways (even though I saved it correctly)...

Brain scan...done!

Everything went well today. Reagan woke up early this morning at 6:40am and was all smiles! We decided to go ahead and get up early too so that we could go do something. We didn't get out of the room til 8:30am or so, but we decided to head over to San Francisco. We drove over the double decker San Francisco-Oakland Bay Bridge and stopped on Treasure Island to take some pictures. It was very overcast and cool but still a nice day. Then we drove over to the Oakland side and basically made a u-turn and headed back to San Francisco. It was at this point that I got a call from the anesthesia nurse wanting us to come in early (they had a cancellation). I explained to her that we had just gotten to San Francisco and we were not going to just turn around and head back! We parked our car and walked along Fisherman's Wharf. We were here almost 11yrs ago for a few days of our honeymoon and loved it. We went back to Ghirardelli Square and had some hot chocolate and a caramel sundae! By then it was time for us to make our way back. We (of course) got stuck in some more awful CA traffic but we made it to Stanford only a few min late for our appt. It still took them some time to take us back, but everyone was very nice and they even got Reagan's iv in on the first try!!! Unfortunately the one they put in was too small a gauge to take blood from (for labs), so they had to stick her again in her arm but they were able to leave that iv in so we can try to draw from it tomorrow to avoid additional sticks! Even the anesthesiologist was nice, which is surprising because usually they are very no nonsense and technical. They seemed surprised that she wasn't more affected by the versed they gave her to calm her before doing the iv. She was slightly less energetic, but definitely not sleepy. We did explain to them ahead of time all of the issues Reagan's had in the past with anesthesia (taking a long time to come out and crying and holding her breath when she woke up) as well as the incident she had with methadone. The anesthesiologist stressed that if Reagan was having any breathing difficulties afterward...they would want to keep her overnight to monitor her. We went down to the room with them and watched as they had all kinds of problems finding equipment they were looking for. At one point I looked down at Reagan's iv and noticed that nothing seemed to be attached to it. I asked the anesthesiologist about it and he looked and saw that he hadn't tightened it well enough and it had come apart (and dripped on the bed). No wonder she wasn't getting sleepy yet. Once the medicine made it's way in, she really started dozing. That's when we left and were told it would take 30min for the tracer to absorb into her brain and then another 30min for the scan. We went and got coffee and a snack and then went back to the waiting room. About 1hr 20min later, the nurse came and told us she was doing fine, they were just waiting for her to wake up a little more. Another 10-20min later they came and got us. Reagan was still asleep when we got to her bedside but Mike spoke to her and immediately she opened her sleepy little eyes and looked at him. She was sleepy but kept opening her eyes here and there for us, so that was a great sign. The anesthesiologist came by and told us that they did have one little issue during the procedure. About 10min before they finished, she started waking up (they were using very light sedation). So the doctor gave her some more anesthesia (because if she moved, they would have to start the scan over from the beginning). Apparently he gave her a little too much, because he said she held her breath and turned purple!!! It only lasted 30sec or so and they gave her some oxygen and she was fine. He was very nonchalant about it...I guess because he's afraid of getting sued! We were both a little in shock about the whole thing. Reagan looks fine though, so I guess all is good. She was still very sleepy but since she woke up no problem, they let us leave the hospital. She woke up for good on our drive back to the hotel and has been awake ever since. So much for being sleepy the rest of the day (I was looking forward to a nap)!!! Then tonight while sitting in our hotel room, we experienced an earthquake! The epicenter was about 30miles away in Berkley but we could definitely feel it. Kind of like an unsteady boat, we rocked back and forth for about 10 seconds or so. Apparently there was another one earlier in the day around 2pm, but it was shorter and we didn't feel that one. Needless to say, we've had a very busy day! Time for some rest!! Do you hear that Miss Reagan?!?!


A very overcast San Francisco (I have more pics, but they're on my good camera and the cord is at home! I'll post more after we get back!)




After the scan...waiting for our car.




In the car 15min later...wide awake!!

Thursday, October 20, 2011

Good girl

Reagan has been a good girl all day today. Very smiley and happy from the get go. She slept all night (once we finally got her to bed) and then woke up this morning when we put her in the car to head to the hospital. The Echo/EKG didn't take long so we decided to head out to Half Moon Bay before our afternoon appt with Dr. Enns. We drove to the Ritz Carlton and then walked around the hotel and golf course (which, of course, is right on the water). The views were amazing and we did take quite a few pictures, but I didn't bring the cord to connect the camera to the computer, so you'll have to wait til we get home to see those pictures! I did take a few of Reagan in the car this morning with my cell...she was just so bright eyed and happy! After Half Moon Bay we drove back to Stanford and stopped off at the bookstore to buy a few t-shirts. We had just enough time to shop and go because we had to get over to our appt. At our appt, Dr. Enns wanted us to go over Reagan's history from the beginning and then he did a quick examination of Reagan. Afterward he went over some info about EPI-743 and then told us about the brain scan Reagan is going to have tomorrow. I'm not super excited about her being put under, but from what I understand is they use light sedation and try to avoid intubating (so that's always a good thing). The scan itself is something similar to a CT scan (which Reagan has never had) but different. It should be interesting to see the difference in her scan before and after taking this medication. Definitely keep all of this in your prayers (that they can place an iv easily, that she doesn't have any problems with anesthesia, and that she responds positively to this new medication)!! We're praying for good things!!




They gave us a mask to put on Reagan because they were doing construction in the building and stirring up a lot of dust. She kept pulling it off her face!

Wednesday, October 19, 2011

Long day but we're here!

Reagan didn't sleep very well last night. She was up late again (after midnight) and then woke in the middle of the night fussing. I picked her up and she calmed down, so I put her in bed with us. She kept making grunting noises and seemed unsettled, but eventually she did fall back to sleep. I got up at 5:45am and we left the house at 6:30am. It's been a long day...lugging all of our stuff through the airport. Reagan was perfect on the plane. She sat in her chair and never made a peep. I picked her up (just to let her move around), but she probably would have sat there the entire time no problem. We arrived in San Jose around 11:30am. We almost forgot our carseat...again. When we went to the Bahamas and then again today, we picked up all of our checked luggage and then walked out to get on the rental car shuttle before realizing we didn't pick up the carseat. Both times Mike had to run in to get it. Needless to say, getting around airports with all of Reagan's stuff can be a very stressful thing. The weather here is great though and we can totally see why people love living here in CA. We checked into our hotel and Mike went about a mile away to meet with one of his customers (total fluke that I booked a hotel so close). Tonight (around 6:30) we headed out to go look around and we got stuck in some killer California traffic. We ended up eating dinner at a little mexican food restaurant in Palo Alto. It was packed, but the food was great and we had a nice time. Reagan was as good as gold too, so that always helps. We were kind of hoping that we could get her to go to bed a little earlier tonight (seeing as how we gained two hours going from central to pacific time...keep in mind my posts will be two hours later as well). She wasn't looking too tired but Mike laid down with her and she's starting to look a little sleepier. Hopefully we all get some rest tonight because it's been a looooong day and we have to be at the Heart Center for an Echo/EKG at 9am tomorrow morning.

Monday, October 17, 2011

Stanford itinerary

Reagan finally slept last night. She really fought it, she was up til 12:30am or so, but she did sleep through til 11 this morning. She's been a good girl all day today. She still is having the occasional crazy episode of chorea and cold hands and feet, but overall she's been content (and we've even gotten some laughs). I'm exhausted. I've been packing all night. I actually packed the suitcase full and then found out it was 10lbs overweight so I had to divide it up between two bags. What a pain. It's 12:30am right now and we've got to leave here by 6:30am...so I'm going to make this quick. Here's our itinerary for Stanford...

Weds, Oct 19:

9am check-in for 9:30 Echo/ECG at Heart Center

3pm clinic appt with Dr. Enns + Labs


Thurs, Oct 20:

1pm anesthesia prep for 3pm brain spect


Fri, Oct 21:

9am series of blood draws, dose study drug, 1 more ECG.. done!

Please keep us in your prayers!!! Time for bed!

Sunday, October 16, 2011

Better

Reagan was up again last night (and so was her mommy). I was exhausted and kept falling asleep myself, but she wasn't. She finally fell back to sleep sometime between 3-4...and then she slept til 12:30!! I'm glad someone got some sleep! Overall, she's been much happier today. When she first woke she was pretty mellow and zoned out, but we've gotten some laughs out of her and although she's been a little crazy (still having lots of chorea), she's seems to be feeling better. Her hands and feet are still ice cold, but I think she's finally coming out of the bad days. This is good because we leave for Stanford Tues morning. I'm not joking when I say, I haven't packed a thing yet. Nothing. Hopefully I can get everything rounded up and ready...even if it means I have to stay up late tomorrow night. I'm a little sick of traveling. It's such a hassle. The packing, the unpacking, getting through security, getting on a plane with all of our junk! Ugh. Although, there is one place I wouldn't mind going back to...







Saturday, October 15, 2011

Rotten but getting better

Reagan was rotten last night. We even resorted to trying diazepam to see if it would calm her down (a 2mg pill her doctor prescribed for her bad days...not the rectal form you use for seizures). Needless to say, it didn't do a thing. There were maybe 2-3 min that she calmed down and was quiet and then she was back to crying again. She did get more sleep last night. She dozed off for a bit with Mike, but that only lasted maybe an hour or so. Then I got up with her from 2-4. She fell asleep around 3:15 in my arms but I was afraid to move so I waited to lay her in her chair til 4. Of course, then she slept in til 9:40 (I had to get up at 7 to get all of her medicines done before I left for my tennis tournament). She woke just as nasty as she was last night and she gave her Daddy a hard time in the morning. But she turned it around in the afternoon. She started laughing (a crazy laugh, but laughing none the less). Her hands and feet are like icicles and she's having crazy amounts of chorea (including super hyperactive bouts of running in place), but she's not biting at her hands any more so we could finally take off her arm restraints (now she just wants her hands in her mouth or hair at all times). Tonight she gave us another round of fussiness that lasted an hour or so, but that passed and now she's back to her the laughs. Hopefully she's turning a corner and headed out of this bad streak! Only a few more days til we leave for Stanford...we need a good girl by then! (By the way...my partner and I won first place in the tennis tournament! Not bad for a pregnant woman!)

Friday, October 14, 2011

Bad days

The bad days are back. Well, at least I think that's what's going on. I'm not sure if it had anything to do with adding the propranolol or not but that's the only change we've made lately. We had gone a month and 10 days without any real bad days. A few "off" days here and there, but no screaming crying, awake all night sort of days in such a long time. But Reagan was headed in that direction yesterday and last night she started up with the full out screaming crying. She'll be OK one second and then the next second, she's screaming bloody murder. The weird thing is that her hands and feet haven't been clammy at all (well her feet are a little cold tonight) but her heartrate is through the roof, so I'm not sure how much good the propranolol is doing at this point (of course we're only giving her 3mls and the doctor wanted us to give her 5mls). She didn't sleep much last night (I stayed up with her all night and Mike didn't get any sleep either because she was so loud). She slept maybe 45min to begin with and then slept another 3hrs later on. I only slept about 2.5-3 hrs total last night. Definitely not the best timing, since I'm playing in a tennis tournament today and tomorrow!! She's been miserable!! Lots of shrieking and screaming all day. Lots of chorea...including little fits of it where she's kicking so much it looks like she's running in place. Definitely biting. We've had to keep an arm restraint on her at all times and I've felt teeth on me quite a few times today! It's been a loooooong day (and night). I just can't believe we're here again. I was really hoping that maybe we were done with these days altogether. Please keep it in your prayers that she comes out of this quickly (for her sake and for our sanity) and that all "bad day" symptoms are gone by the time we leave for Stanford!

Cranky pants

So...today hasn't been the best day for little Miss Reagan. She slept all night but woke at 7:30am...no smiles. This morning she was OK. You could tell she was a little agitated, but she kept it together. She would grunt here and there, we weren't really sure what that was about. I sensed she had some stomach upset. She was a little more spitty than usual and a little gurgly in her throat. We put the drain on her g-tube and lots of air kept coming out. She fell asleep at the beginning of vision therapy this morning and was still asleep when her teacher called at 11:30. I thought she was going to keep sleeping, so I told her teacher not to come. Reagan woke up, but it was probably better she didn't come, because someone had a bad attitude. She was a little squirmy while the guy took off the EEG leads, but she pulled through and somehow made it through PT. Afterward we gave her a bath to wash all the gunk out of her hair and we thought maybe she'd start feeling a little better. Nope. Things only got worse. For the last hour or so that the nurse was here, she was really on edge. She kept yelling out, kinda sounded like a laugh, kinda like a cry. Then she proceeded to cry off and on for two hours tonight. She seemed to do better when I wasn't holding her and she was just sitting in her chair. She'd be fine one second and then screaming the next. Definitely not good. I'm hoping we get away with another mildly bad streak again (although tonight didn't feel that mild). We're going to Stanford next week to start her on EPI-743, so at least she's getting this out of her system now. Hopefully she sleeps tonight...I'm playing in a tennis tournament in the morning, so I really need some rest!

Wednesday, October 12, 2011

Already sick of the EEG

As far as I know, Reagan slept the whole night. I was OUT. I'm not sure why I was so exhausted, but Reagan and I both slept in til 9am this morning!! She's been a good girl again today. I'm sure she's already sick of having those leads on her head. The bandaging that was wrapped around her head/hair to keep it all out of hands reach has completely come off. It was all bunched up at the top of her head this morning. She had a hat on so we didn't see it for a while, but the gause was all up on top of her head, giving her a bit of a cone head! I just took it all off and put one of her little crocheted hats over it. It's not super important that we get perfect tracings from here on out. Her doctor really just wanted a 24hr EEG, but these home companies/insurance make you do a minimum of 48hrs. It can't hurt to have additional time, but it certainly is a hassle for everyone (as the cords on her head don't stretch very far so you have to keep the bag only a foot or two from her head at all times...her feeding pump is SO much easier to deal with). She's still really going for those cords too! We've been fighting with that hand all day! She's a turkey! She had OT this morning and speech this afternoon and was great for both. She has been a little antsy at times, but it's hard to tell if it's just her being irritated with the stuff on her head or if she's heading toward a mildly bad day. Tonight her nurses are helping us out so Mike and I can join some people from our Bible study in taking dinner to the Ronald McDonald House at Texas Children's. This one is for the families of children in the ICU at Texas Children's (there's another one in the medical center that is a big free standing building). We've done this before and it's something that Mike and I feel especially drawn to. The Wednesday day nurse came in an hour late and she's going to stay an hour later than usual and the night nurse is going to come in 3 hrs early...just so Mike and I can both go tonight! Hopefully Miss Reagan behaves herself for everyone!

Tuesday, October 11, 2011

Home EEG

Reagan has been a super good girl again today! Even though the pulse ox alarm woke her up this morning (she had it on because the night nurse was here), she was just as sweet and smiley as can be. She had OT this morning and then "school". Her teacher helped her make "hand art". She made a little picture with ghosts made from tracings of her hand. We already have quite a few hand art pictures this year!! This afternoon she was a perfect angel while the EEG technician put the leads on her head (for her in-home EEG). She actually was dozing off a little as he did it...I think the hum of the air compressor (he used to dry the glue) was making her sleepy. She never did fall asleep all the way though and once the leads were on, she's been quite the handful to keep away from the cords! Her head is wrapped up enough to prevent her from pulling the leads directly off, but the cord in the back is within her reach, so we've either had to keep a sock or an arm restraint on that left arm at all times. She's also been super squirmy. Very hard to hold and a little resistant to falling asleep tonight. Even asleep, she's very restless! Hopefully she sleeps. They need to get time with her awake and asleep while the EEG is on. Goodnight!


Monday, October 10, 2011

Super precious

Reagan has been a total doll again today...so super precious! I woke up around 6am and saw her moving around in her bed. I thought she was awake, but apparently she was just moving around in her sleep. She was very restless, but her eyes were still closed! I don't know if she did this all night or what. She slept in til 9am and woke super sweet and smiley. She had speech at 11 and then fell asleep right after. She took a nice little nap (maybe she was still tired) and woke right before we had to leave to go get her tube replaced. She woke just as sweet and smiley as can be. She was really hamming it up for the people in IR. The girl that took her back couldn't get enough of her smiles. Reagan was only gone for maybe 10-15min and then they brought her right back. Piece of cake (knock on wood)...they've never had a problem placing her GJ tube. I've heard nightmares about other kids getting them, but for Reagan it's never been a problem. No sedation, super quick...we've been lucky, I know. When we came home we took Reagan out for a ride on her tricycle. We went to a couple of our neighbors' houses to show them her new bike. Her head control was great and she really seemed to enjoy herself...smiling the whole time! She rode around for 30 min or so, definitely the longest amount of time she's ever been on it (although some of that time she wasn't actually moving). She did end up with a couple mosquito bites and that was even with her Off clip-on attached to her bike (although it probably would have been 15 bites without it)! She had fun, got outside, got some exercise, socialized...good times!

Sunday, October 9, 2011

Darling

Reagan has been a darling girl again today. I had major issues with her pump alarming over and over again last night. I went through 4 bags (one which had a hole in it and leaked all over the pump), tried both pumps, and eventually I finally got it running with the 4th bag I tried (around 1:30am)! Reagan did finally wake up this last go round but I think she fell back to sleep fairly quickly because I never heard a peep out of her (of course I was exhausted by this point). We all slept in til right around 9am this morning. Reagan woke smiling and has been super precious all day today. No laughing, but lots of smiles. I'm hoping she'll hold on and have some good days this week because we have some busy days ahead. Tomorrow she's getting her leaky gj-tube replaced (we do not have to do this under sedation...some hospitals insist that they use sedation, thankfully not ours). Then Tuesday they are coming out to put the electrodes on for the 48hr home EEG. I'm thankful that we don't have to do this in the hospital this time around, but I know 48hrs with her hooked up (even at home), isn't going to be fun either. She's been a doll today though, so hopefully she'll keep it up!




Sorry...these were taken in low light with my cell phone!

Saturday, October 8, 2011

To laugh or to cry...that is the question

Reagan seemed to sleep well last night. She woke up this morning super cute and giggly. She was laughing a lot but she also seemed a little groggy (maybe she didn't sleep as well as we thought). She was only up a couple hours before she fell back to sleep. She took a nice long nap, waking up about 10min after I decided to lay back down (I swear she can sense it when I try to take a nap)! Ever since waking up fussing, she's been in a weird sort of mood, mostly happy but a little weird. She's been laughing off an on all day today, but every now and then she'll do a little laugh/cry. It starts off with a happy sort of laugh but ends in a fussy sort of whine. I'm not sure what that's about. Maybe she's having some gas pains because we've heard quite a few toots out of her...who knows? I started the propranolol today. The new cardiologist prescribed 5mls 2x a day but I started with just 1ml (the max dose she was on previously was 2mls). I was a little leery to give her 5mls of a medicine that lowers her heart rate, when the highest dose she's ever had was so much lower. We just never know how she's going to respond to a medication. Sometimes she over responds to low doses and sometimes she under responds to high doses! Her body metabolizes medicines differently...I just didn't want to take any chances. What's weird is that Reagan's heart rate this morning (before the nap) was much lower even while she was awake (105), compared to what it was during and after her nap (115 asleep, 130 awake)...and this was after the dose of propranolol. I guess I'll try 2mls tomorrow. Her feet were a little cold today too. Not sure what that was about. The high heart rate, cold feet, laugh/crys...are all signs of impending bad days. But she should be having good days right now...not bad days. Hopefully these things were just flukes and tomorrow she'll be feeling 100%. I want a nice string of good days for my girl!


Love this pic of her in the Bahamas...floating along the infinity edge (I'm going to try to put together a slide show from the Bahamas, so you can see all of the pics...in my spare time!)

Friday, October 7, 2011

The smiles and laughs are back!

Reagan has been a precious girl today. She slept all night last night and woke around 7:30am. She was still pretty serious when she first woke up, but she started up with the laughing after a little while and has been laughing (and smiling) all day today! She was still a little spitty/gurgly when she had OT this morning and had a hard time managing her spit after licking on the lollipop...but she recovered and had a good session. Her vision therapist came back today (after what happened yesterday) and Reagan had another good session with her as well! She was such a sweet girl today. I love to see her feeling better again. Tonight after Mike got home, we took her out on her tricycle. She was smiling the whole time! Of course, the mosquitoes were out in full force, so we couldn't stay out too long. She fell asleep tonight around 9:15pm...which is pretty early for her. She sounded a little gurgly again, so hopefully she's alright tonight.

Thursday, October 6, 2011

Tummy trouble

Last night the night nurse said Reagan woke a few times, each time making a laughing sort of noise, but each time going back to sleep relatively easily (the last time she had to be patted back to sleep). Her heart rate kept jumping around all night...even while she was still asleep. Probably not the best night's sleep last night. She woke up this morning still very serious (no laughs whatsoever). She was a little spitty and looking a little groggy right before her vision therapist and school OT showed up. She was trying to doze off but they were doing their best to keep her awake, when all of a sudden she started throwing up! Lots of throw up! First clear foam, then yellow bile (and I drained a lot of yellow bile from her g-tube). She wasn't clearing it very well from her throat (I think because she was so drowsy) so she kept coughing off and on. Finally she fell asleep and rested for a little while. She woke up serious but finally sounded clear. I decided to stop her formula and went to the store and bought some pedialyte to give her for the remainder of the day. I'm not sure what exactly is going on with her GI system, but it's probably best to give it a rest. I'll start her back at half pediatlyte/half formula tonight. She did cheer up a little as the day went on and even managed to make it through PT without any problems. We've gotten quite a few laughs out of her, although they are still slightly pathetic little laughs. Hopefully she'll be feeling more herself tomorrow. And hopefully she gets a more restful night's sleep tonight.

Wednesday, October 5, 2011

Cardiology follow-up

Reagan slept the night through last night (thank goodness) and woke this morning in the same "none-too-happy" mood. She was a little whiny off and on but she did fine and made it through her early morning doctors appt without any meltdowns. This was our follow-up appt with cardiology to go over the results of the two 24hr Holter monitor studies she had done. This appt was also with a different cardiologist...one that deals more with dysautonomia (the nurse made a comment on how cold and clammy her hands were...hello, that's why we're here). The doctor mentioned that Reagan is much younger than most of the mito kids he sees with dysautonomia (who tend to be in their teens). He also seemed a little alarmed with Reagan's heart rate getting so high on her bad days (the "bad" day they have on the Holter monitor was just a MILD bad day...she's had much worse). He wants to put her back on the propranolol to try to decrease her heart rate (the last cardiologist took her off of it). His plan is for her to take it twice a day, once in the morning and once in the afternoon (and one more time at midnight if she's still up and irritable), for 2-3 weeks and then he wants to do another Holter monitor to see if her heart rate has normalized. So I guess we'll be adding propranolol back to the mix! Ugh...so many medications. Reagan fell asleep on our way home from the doctors office and was NOT happy when she woke up when we got home (she was the same way yesterday when she took a nap). She recovered though and had OT...although she wasn't too cooperative for her occupational therapist...I think they spent a lot of time just swinging on the swing. Then she had speech and she actually did amazingly well for speech (all things considered). This afternoon we've heard a few laughs out of her...no smiles, just laughs...but we'll take what we can get! We finally met up for Bible study (we've been off all summer long) and we were amazed at how well Reagan did in a very chaotic, LOUD environment (on an "off day")! She seemed a little overstimulated at times (with lots of yelling, banging kids around) but she held it together and never went over the edge! Tonight's another night nurse night, so at least I know Mike and I will be getting 7hrs or so of sleep tonight. Hopefully Reagan cooperates and gets the same (so far she's slept pretty much through the night every night the night nurse has worked so far)!!

Tuesday, October 4, 2011

Fighting the bad days again...

Reagan slept all night last night and then woke this morning not too happy. No smiles. Very serious. Very tense and stiff. But OK. She mellowed a little during the day. She had OT, school, and PT. She actually dozed off toward the end of school and took a little cat nap. She woke and then had PT. She did a good job with her head control while sitting at the bench, but once we went outside to ride the tricycle, the head control was gone! Poor thing. She looked pretty tired and did doze off for a little bit when we came back inside. When she woke this time, she woke startled and fussing a little. We were able to distract her though and get her calm once again. Tonight Mike watched her while I went to play tennis. He said she was a stinker while I was gone. She cried for 30min straight and then was fussy/on edge the rest of the time. She was quiet when I got home but you could tell she was agitated. Mike said while I was gone she kept putting her hands in her mouth and "acting" like she wanted to bite them...but when I was holding her tonight, she was actually trying to bite them!! I definitely think she's fighting off bad days again. Yesterday she woke a little gaggy and I drained a good amount of fluid (some yellow) out of her g-tube. Then last night you could smell that "bad smell" from her, even though she had just gotten a bath! Today her hands/feet have been a little cold and clammy and her sweet demeanor is completely gone. Hopefully we'll make it through and only have a mildly bad stretch again. Please pray that she gets some sleep tonight (we have an early doctors appt that might not happen otherwise) and that she fights off the real bad days and that we see our sweet girl again soon!!!

Monday, October 3, 2011

Sweet girl

Reagan has been a sweetie again today. She was up a little last night. I woke up around 3:30am to her "talking" to herself. She eventually went back to sleep and then woke up giggling up a storm at 7am. I can't tell you how great it is to see that smile each morning! We are definitely loving these good days!!! She's been super precious all day today. She had her usual private speech therapy, which she did great for, and then had speech through the school system (which I think she's only had one other time), and everyone was thrilled to see her in such a great mood today!!! We didn't make it on the tryke today, but her PT is coming tomorrow so she'll probably show it off during therapy tomorrow. Tonight we've got the night nurse again. Hopefully we can avoid the drama that happened the last time she was here (Reagan's feeding pump not working and trying all night long to get a replacement)! The DME company actually brought back the pump we were having all the problems with (saying they ran it and it worked fine for them), but they're letting us keep the loaner too. So at least now we'll have a back up if needed. I love how these DME companies like to act like you're the complete idiot and that they can get your pump to run just fine! Of course they aren't trying to run your child's specific formula (just water), or using the same bags that you were using, or trying to do it at 3am in the morning, etc... The craziness we've dealt with this pump is enough to drive anyone INSANE!!! I've heard a lot of people eventually get fed up with this infinity pump and switch back to the kangaroo pump...but I really don't want to do that. I like the fact that this pump is so portable (in a backpack) and small/light weight...but boy is it a pain!! Here's to NO more pump problems (I can hope, right)!?!

Sunday, October 2, 2011

Darling

Reagan was WIDE awake last night, I didn't think she was going to go to sleep, but Mike laid down with her and 10min later...she was out! She did wake up at least once last night. I heard her at 3:30am "talking" in her bed. She went back to sleep though and we all slept in a little this morning. She's been a precious girl again today. All smiles, some laughs, and big huge eyes...just a darling girl. She's still a little roly poly. Lots of energy. Moving, moving, moving. We took her out on her tricycle again this afternoon. It's much cooler outside and quite nice but the mosquitoes were all over us. We went down the sidewalk, talked to a few neighbors, and then came back. Reagan seems to enjoy it and it gets her arms and legs moving which has got to be good for her. Hopefully our good girl hangs on this week, I've still got suitcases to finish unpacking and a ton of laundry to do!!! We've got two weeks to settle in before we have to leave again...for the drug study at Stanford! Busy, busy!

Saturday, October 1, 2011

Amtryke success!

Reagan has been a precious girl again today. After the long drive last night (and a dose of clonidine), she slept well last night waking up super happy and smiley once again. This morning Grandma met up with us at the hotel and we drove over to the Elks lodge (who was sponsoring this event for the AMBUCS). They had lots and lots of trykes and I'm afraid Reagan's order got a bit mixed up! When they first brought the tricycle over, it had a seat/back that wouldn't work at all for Reagan. Actually, the one we ended up with, isn't even the one Reagan's therapist had put down on the order form...but it seems to work pretty well anyway (with the laterals and harness). The only issue now is her head (but we have a few things at home we can try for that). She seemed to enjoy it and she was SO well behaved for all of the adjustments that we had to keep making...she never made a peep! Afterward we loaded up the tryke, got in the car and drove all the way back to Houston!!! We're all a little exhausted right now. That is...everyone but Reagan!!! She's been a ball of energy today. She's been rolling all over the place, rolling onto her tube (and popping it open), "talking" a ton, hands in her mouth, biting her lower lip (making her lip/chin area permanently red). I'm not sure when this biting her lower lip thing started, but with her hands constantly in her mouth and spit all over her chin...she's a red faced mess! And her button...it's been leaking like crazy the last few days. Soaking her shirt, pants, & us! I've already checked the balloon and it's not that low on water, I think it's just time to go in and get it replaced! Thankfully, Reagan has been an absolute doll through it all. Now...if we can just get this little ball of energy to sleep tonight!!!