Tuesday, December 27, 2011

Christmas in the Big House

No, I didn't get arrested before Christmas. ;) Emily was admitted to the hospital the Monday before Christmas. She had another blood infection caused by a bacteria translocation from her gut. Unfortunately, there is nothing we can do about a leaky gut. She would get these same blood infections even without a central line. She was started on massive antibiotics, and remains on three of them still. She has had a drastic decline in her neurological status this last month, so genetics/metabolics are involved closely this admit as well. The new thing is her inability to swallow and control her oral secretions (spit). She is seeing pulmonology and ENT now for this. We have started breathing treatments and are going to try Botox to her salivary glands. Today or tomorrow she will have an Upper GI done to check the status of her fundo. We know she throws up right past it, but we want to be sure she hasn't re-herniated. Later this week she will have an MRI and MRS of her brain. This will hopefully give is an idea if what is going in with her brain, causing the neurological decline. The MRS is useful in diagnosing mitochondrial disorders as well. We are doing a new muscle biopsy when we do the salivary gland Botox. She may not have these two surgery procedures done until we are outpatient, depending on how far out surgery is booked for non emergency procedures. Hopefully Emily will remain stable, and the plan is to discharge on January 5th or 6th, when her IV antibiotics are done. I would love for the Botox and biopsy to be done inpatient before we go home, but the ENT surgeon is it of town until the 6th or so. So, we celebrated Christmas in the hospital. I was really down about it at first, but it was nice. I managed to reserve a meeting room, and all our family came. They brought food and presents, and we had a good time. Emily can't leave her room, she is on isolation until some viral tests come back. And we couldn't fit all our family in the rom at one time. But we took turns going back and forth, eating and opening gifts, spending time with family. It was quite awesome, who knew Christmas in the hospital could be enjoyable! Two Santas came, some firemen, and Emily was quite spoiled. We are truly blessed to have an amazing family (both my side and my husbands side) who made this day as good as possible. Thank you! I hope everyone had a nice holiday. I can't believe it's already almost 2012. We celebrate our 13th anniversary on New Year's Eve. I guess we'll celebrate that in the hospital as well. :)

Monday, December 12, 2011

It's almost Christmas!

It is hard to believe that it is almost Christmas. It seems like this entire year just disappeared. It doesn't help that we spent the majority of it in the hospital, I suppose. Emily is doing well. She has been home for 3.5 weeks straight right now, knock on wood! She is having some more severe gagging and retching spells lately, and I think we are missing a key piece of this puzzle. I took some video of the spells for our GI. She starts out by gasping for breath, like her airway is shut. Then she gasps and gasps and finally gags and retches. I think it is possibly some sort of airway spasms, that we haven't realized before. I always call it retching when I talk to the Drs, and so they call it retching. The nurses in the hospital and walk-in clinic always ask me how long she's been coughing and I tell them "That's not coughing, that her retching". Duh. Perhaps I should have not thought I knew everything! LOL Anyway, I sent some videos to the GI and hopefully we can get her opinion and a referral to an ENT immediately. These spells have been longer and scarier lately, and honestly if we don't hear back from her within a day or two we may end up in the ER. We have a pulse ox machine that a friend sent us, and watching her O2 sats plummet during these events is pretty scary. Other than these spells, Emily is very stable. Her seizures are well controlled, and the small ones we do see are almost always stopped by swiping her VNS. The VNS has been awesome for her!
We went yesterday to my Grandmother's house for an early Christmas with her. We took lunch, and I made a fun Christmas cake. It was a great visit. I love seeing her with Emily. :)
Today it is raining ... our backyard is a mush puddle. Alex's parents are in town until tomorrow, so they are going to come sit with Emily for a while and Alex and I are going to go out. Souplantation for lunch, and the last Christmas shopping. It will be nice to go out, alone, with my husband! It's a super rare event in our house.
I hope everyone is doing well. I know I don't get on here very often and update. I miss blogging .... I have a goal of blogging more. Hopefully I will be able to! :)

Saturday, November 26, 2011

Getting ready for the holidays and cleaning house

I am trying to take advantage of Emily being home from the hospital to get our house cleaned up. It is absolutely embarassing what it looks like right now. We had some friends over last weekend (hi Lacey!) and I realized after they left how horrible my house looks right now. It seems like we are never home long enough to complete a project. Mainly what I do is just organize the crap wherever it is, and try to keep the house functioning. What I am going to start this week is really CLEANING. Not just putting things in neater piles, but finding a home or organizing system for the things. Taking more stuff to Goodwill. Giving more things away. Selling some things we don't need anymore, to make a little extra holiday money.
While doing that, I am soaking up Emily. She is doing pretty good this week! KNOCK ON WOOD she has done well since we came home from the hospital this last stay. She is still on TPN 100%, gets medications in her j-tube, and her g-tube drains to gravity 24 hours. It's actually a gj-tube, although we are discussing with the GI putting in a seperate j-tube sometime in the near future. She relies on a LOT of medications to get through the day happily and without retching and crying all day, but it is what it is. When she is happy and smiles at you and wants to cuddle, it makes every rough moment disappear.
Jacob is doing great as well. We get his grades via email each week, and I can always keep tabs on him. His lowest grade right now is a B+. That makes my mommy heart so happy. He is such a well adjusted, smart, amazing little man. He still takes tennis lessons twice a week and is getting really good at it. It's like watching a real tennis game when they play!
We had a wonderful Thanksgiving at my parents house. The food was all delicious, and I am eating leftover Oreo cheesecake as I type this. Yum! It was great to be out of the hospital and surrounded by family and love. We took the kids to Legoland yesterday, and it was so much fun. Emily was able to go on almost every single ride, including the roller coasters! It was a great family day. Hopefully we will continue to be HOME and celebrate the rest of the holiday season here. We pulled down all the Christmas boxes this evening, and will start decorating tomorrow. I love Christmas decorations!
I hope everyone is doing good. I am behind on reading blogs and commenting and catching up. Hopefully I will have time to do that this week, in between laundry/cleaning/Emily. :)

Monday, November 21, 2011

Thankful for Thanksgiving

We are home now, and so thankful to be here! The good days more than make up for the not-so-good days. Emily is sleeping a lot lately, but when she's awake she's happy. She is always in and out and in and out of the hospital, so we love the out times. This weekend we got to spend a day at Disneyland with a bunch of friends. Emily did great! She loves going on rides, especially Small World. The girl is so happy being pushed around for hours in her wheelchair. :) We are getting ready for Thanksgiving. Cooking, cleaning, doing laundry in my brand new washing machine. It's the little things in life that make me happy sometimes. Thanks for checking in on my Princess. Things are about the same, but at least we're home. Happy for home!

The Tostinos/redbox winner is ...

The Enriquez Family!! Sorry I don't have a picture of Jacob drawing the name from a pink hospital wash bin but my camera is missing. :( Anyway, congratulations to the Enriquez Family! Please email your mailing address to me at waffle1313 @ gmail. com. :)

Thursday, November 17, 2011

Totino's Pizza Rolls and Redbox Giveaway

You all know how often our family is home together and has time to relax. Just about never. So when we are all home and no one is at the hospital and everyone is feeling good, the last thing I want to worry about is making dinner! Thankfully there are many options for frozen dinners and treats that are easy and delicious. Right now there is a great deal going on! Look for specially-marked packages of Totino’s Pizza Rolls and Party Pizzas to stock up on everything you need for a Fun Friday Night: purchase 4 Totino’s Party Pizzas or 2 Totino’s 40 count Pizza Rolls to receive a free one-day redbox DVD promo code. With more than 27,800 locations, and available nationally at Walmart, there’s always a redbox kiosk nearby! Find the closest redbox kiosk by checking redbox.com/locations. If you haven't tried redbox for movies yet, you have got to! There is a huge variety of movies from all categories, even Blu-Ray and video games. We always find something we want to watch. There is no easier way to rent movies - you can return the movie at any redbox, it doesn't have to be the same one you got it from! By us there are redbox kiosks everywhere - Walmart, Walgreens, Stater Bros. I was lucky enough to get a special package from MyBlogSpark, thanks to Totino's! I the package was a coupon for Totino's products - and can I tell you that Jacob is now in love with Pizza Rolls? There was also a coupon for a free redbox movie, two cute pizza slice plates, a great new pizza roller, and a popcorn bucket. One of YOU reading this post can receive the same package!
Here's how to enter - simply reply to this post by tomorrow, Friday November 18th, at 5:00pm. In your reply, tell me if you have ever used redbox and how you like it. One winner will be drawn at random by Jacob (the Pizza Roll eating boy). Disclosure: The VIP coupons, prize pack, information, and giveaway have been provided by Totino’s through MyBlogSpark

Friday, October 21, 2011

Admitted again

Emily had an amazing birthday party. So many of our special friends made it and she got some lovely gifts. We are very lucky to have so many great people who love our girl! The next day we went to the Mattel Children's Hospital Party on the Pier, and it was a blast. Wednesday Emily had outpatient surgery to scope her Mitrofanoff and dilate it. They did a ton of bloodwork beforehand, and they called Thursday morning to say that she had a positive blood culture. So in we went to the ER, for repeated cultures. I've been told already that the repeat culture was positive as well. I haven't seen any doctors today, so I don't know the plan. No one has started her on antibtotics yet, so I don't know what the stay will be like. It would be great to go home wit IV antibiotics, but with her history of sepsis with no fever, I doubt they will let us. Please keep Emily in your thoughts and prayers. This is admit 19 I believe, since March of this year. It's a lot for one little girl!

Friday, October 14, 2011

Tomorrow is PARTY day!

Emily turns 8 on Sunday, and her big party is tomorrow. When I say big, I mean big. It started out small, of course ... and then we have a horrendous year with her in the hospital so much. There were a couple of times where we weren't sure if he was going to survive, including just last month. So the party got BIG. I cannot wait, and I will definitely share lots of pictures afterward! It is going to look like a Hello Kitty explosion at the park. :)

Friday, October 7, 2011

We're home again ... And staying here, damnit!

Oh, I guess I didn't even mention that we were admitted again. I have a hard time updating lately, and thinking of something witty to say. Emily had TPN clinic last Wednesday. During the appointment, I caught her TPN tubing on her wheelchair while lifting her to the exam table, and broke her central line. You know, that pesky little life-saving line that goes to an artery directly next to her heart, so that she can receive the IV nutrition that is keeping her alive. We had to wait while the TPN nurses ran to the hospital next door and scrounged up a repair kit. By Friday she was retching more and irritable again, so we were told to take her to the ER for labs and a blood culture. Thankfully everything looked fine, so we got to go home. Saturday we went to a birthday party for a friend, and Emily had a blast. Sunday we went to an awesome Make a Wish party on the Santa Monic Pier, hosted by Kevin James. Every Wish kid was given an iPad 2 by Kevin James! Monday afternoon Emily pulled her gj-tube out. I put a g-tube in, and paged the GI on call. For those who aren't drowning (oops, I meant immersed, did I say drowning?) in the medical world, a g-tube goes into the stomach. A gj-tube goes through the same stoma (hole) and then threads deep down onto the intestines. Emily needs this because she doesnt tolerate anything in her stomach, including the 26 meds she takes daily. We had to go to the hospital, but they couldn't replace it until late Wednesday night. So we were admitted and sat on IV meds and waited. Not all of her meds are available in IV form and she was already in withdrawals from missing doses of methadone and Ativan, so she as a mess. Her tube got replaced Wednesday, but we stayed that night to be sure Emily would return to her new normal before going home. We were discharged Thursday morning, andi was so glad because Jacob's back to school night was that night. I have to admit that I am no longer handling this well. I am a puddle of tears and self pity. I feel like I have lost any semblance of who I am, and my only identity is being Emily's caregiver. I don't have a chance to go out and do anything that doesn't involve Emily. Friday lunches with "the girls" that I have done for years, are no longer possible most weeks. Getting my nails done ... Heck, even grocery shopping ... Nothing is easy anymore. We don't have any nursing yet, and the palliative nurse who is supposed to be helping us with this is not returning calls this week. My mother-in-law is here helping right now, and I don't know what we'll do when she goes home. Even waking Emily up to get Jacob to school, putting her in the car to pick him up, these will all be huge challenges when she goes home. I am feeling very overwhelmed and frustrated. I belong to an amazing group of moms on Facebook who all have kids with AnyCephaly - micro, hydro, liss, pmg. There is a mom whose sweet daughter had lissencephaly, and was 22 years old. She passed away this week. My first thought, while crying, was that I can't handle the thought of losing Emily. My second thought, was that I can't so this until she's 22. That is another 14 years. I can't do this for 14 more years. I am so exhausted, mentally - physically - spiritually. And then the guilt of thinking something like that, much less typing it here and admitting it? Killer. What kind of Mom thinks that? I can't imagine either end of the spectrum with Emily and that is enough to tie my brain in a knot and leave me quivering in tears. Sometimes this life is incredible. Most days I think how lucky we are to be blessed with Emily. Most days she's just this awesome being, who gives so much more than she takes. Some days though, like now, I just think that I can't handle this much longer.

Sunday, September 11, 2011

Okay, here is what I really wanted to blog ....

I posted that last post because I didn't want to sit and type out what is on my mind and in my heart. I just wanted to update that we are home, and perhaps it would come across as a happy post. Which it is, because we are home. But my heart is not happy. :( Emily is on a LOT of medications. She is on sedatives, narcotics, pain meds, and more. She is SO knocked out, because it is the only way to keep her comfortable. She slept for 16 hours last night through this morning. Unheard of for her, she normally sleeps maybe 8 hours a night and usually wakes up in the middle a few times. She went to bed at 8 last night, and only got up at noon today because she had pottied her whole bed up. Who knows how long she would have slept. Now she's in her chair, in front of the TV. She can't sit up anymore, so she can't be on the ground with her toys. She lays on the ground but WANTS to sit up, and it is heart-wrenching. She tries and tries and tries to sit up, while whining and looking at you for help. If she manages to sit up, or you help her up, it lasts a few seconds and then she falls right over. It's so sad. :( I told my husband that as bad as it sounds, it would be better if her cognition went down as her physical abilities do. She still WANTS to sit up, she knows she used to be able to, and so she tries over and over. She just can't do it. She can hold her head up still, but not for long. It flops back against you, or down on to the tray of her chair. In bed she just lays on her back, in the nest of pillows we put all around her. She does wake up and smile, and tries to be silly with us. It's fairly infrequent though. She just sleeps most of the day, and looks drugged and dazed the time she is awake. I know she's on a lot of meds. I know she was inpatient and really sick. I know she COULD overcome this and return to herself. But I can't help but look at her and cry ... I feel like we are losing her. Slowly and painfully. She is becoming less and less herself. :( We follow up with all her doctors over the next 2 weeks. I have some serious questions for them. Tomorrow morning I am supposed to talk to the hospice company. Emily isn't going on hospice, but she is going on palliative care which is headed by a hospice company. Hopefully the woman can help us get some RN care at home, because this is too much for any of us. She has meds 12 times a day. That includes midnight, 3 am, and 6 am. No one is getting enough sleep, and watching her drift away from us is so hard ....

Settling in at home

Emily came home Thursday from the hospital, and we are so glad to be at home! Beyond glad though, we are tired. She gets meds 12 different times through-out the day. Plus cathing, emptying her g-tube drain bag, IV antibiotics, etc ... it is non-stop. I imagine that some day in the future I will be able to cook dinner again, right?

Sunday, September 4, 2011

An update from Club Med ...



Emily has been inpatient for almost 4 weeks straight now. She has battled two blood infections, a urinary tract infection, a line removal and a line placement, multiple xrays/CT scans/ultrasounds/labs/cultures/etc. She is still dependant on the pain pump to get through her days. We are supposed to start her on an oral med though her j-tube, in order to get her off the pain pump. Unforunately no one at this hospital is an "authorized prescriber" of the drug. What the hell - are we really putting something this serious and scary in my tiny little princess?

In the meantime, we hang out here day after day. School starts on Wednesday. I will be home with Jacob Tuesday night and Wednesday for her first day, and then back to the hospital. He will be shuffled around from friend to family member to friend and back again, as we try to keep his life as normal as possible. Luckily he doesn't have tennis lessons this week, so it's one less thing to stress about.

Yesterday, Jacob and I went to this amazing place called Nurtured by Nature, thanks to the Starlight Foundation. We go to meet and play with armadillos, porcupines, snakes, and otters. It was INCREDIBLE! I am so thankful for a day of fun with my boy, he so often gets skipped over when Emily is in crisis.



Thank you to everyone who has helped us in any way. Financially, emotionally, physically, spiritually. If you would like to donate to our ChipIn fund still, please CLICK HERE!

Monday, August 29, 2011

An easy link

We are still inpatient, things have gotten much worse :(


Emily was weaned off the pain pump, only to have unexplainable pain episodes all day. We have run every scan and test imaginable, and no one can find anything. She screams and writhes around and bites herself (or me). She tears out her hair, bangs her head, and screeches. It is horrible. It comes on suddenly - she will be fine for a minute or two, and then go abruptly into this. It can last anywhere from a few minutes to a full hour. There's no more than 30 minutes break in between episodes.

She is back on the pain pump, at more than twice the dose she was on before, and still having these episodes. She is also on ativan and seroquel around the clock, with no relief.

The doctors all agree that this must be a progression of her underlying mitochondrial disorder, which is not specifically diagnosed. They were blunt today, they said that they do not feel they will be able to find a reason for her pain. Their goal is to get her comfortable enough to go home.

Please pray for Emily. Pray for us. Pray for her 11 year old brother, who doesn't understand why the doctors aren't fixing his baby sister. Pray for the doctors to find a way to make her comfortable. Pray for a miracle!

And I hate to add this in to this post, but we are getting very behind financially because of being inpatient over 90 days this year. The drive to the hospital is 50 miles each way, and my husband and I try and switch off every couple days for sanity breaks. The cost of gas and the cost of eating every meal in the cafeteria are adding up. Alex's car costs $11 a day to park at the hospital, luckily mine only costs $4 with the handicap placard. It all adds up, and Alex is taking days off every week. Someone has to be home with Jacob, and someone has to be with Emily. It's getting pretty desperate. If you are in a position to help, please donate to our ChipIn fund. It will go directly to us. I hate to even ask or put this here, but we really need help!

Saturday, August 13, 2011

Adding to the previous post., since it won't let me edit it!

blogger sucks.

(ETA) I realized that I posted this update from FB here, and didn't post before this. So we were told her stomach ultrasound showed possible kidney stones. Then we did an xray to confirm and it was shown that she has a possible obstruction or ileus in her intestines. So we did a CT scan, that showed a large abcess next to her bowel, with contrast in it, most likely caused by a small bowel perforation. So she was sent to ct scan guided surgery to drain the abcess and put a drain it. They called after 2 hours and said there is no abcess, the CT is showing a large loop of bowel. So no surgery, no answers, no relief. The GI says the radiologist that saw an abcess needs to talk to the radiologist that saw a loop of bowel and determine what the hell they are seeing.

Update from the hospital

Just heard from Alex. Emily's broviac line is growing yeast. :( She is starting on antifungals for it, and the line will be pulled Monday in the OR. A new broviac won't be placed until they are sure the infection is cleared. GI is upset about the CT scan issue. He is insisting that the two radiologists sit down Monday and discuss WHAT they are seeing, since they are not agreeing on what it is. Obviously SOMETHING is wrong with her bowels or something near her bowels, so what the hell is it. They will be doing more imaging this weekend or Monday to determine for sure. Her fever is back up to 103. Her PCA (pain pump) ran out of medication and for some reason it's taking pharmacy forever to send more up. She is shaking, crying, jerking, miserable. I am so glad that Alex is there and I get a break, but it breaks my heart to not be there. :( Her bladder is huge on the scans, so they want more frequent catheterization. I just told Alex to ask the nurse if we can just place a catheter in and LEAVE it. Let it just drain. Her stomach hurts so much, to catheterize her Mitrofanoff makes her scream, I can't imagine doing it even more often.

I don't know what causes a yeast infection in a line. I am wondering if drooling can cause it? She drools SO much, and the dressing on the broviac has come undone three times this week from it. In fact when we came up to the floor from the ER on Wednesday, the Broviac was uncovered and she was drooling on it. I tell them as soon as I notice it coming undone but it's hard to watch it 24/7. :(

Please pray for Emily, think good thoughts, whatever it is you do. She has never been this sick in her life and it is horrible. They keep saying that she is "on PICU watch", so they are very concerned about what she will do next.

Today, I am home with Jacob. He needs some love and attention and "normal'. We are going to lunch and the Smurfs movie. I will update when I hear from Alex later!

Thursday, August 11, 2011

Quick update, need prayers!

For anyone who doesn't follow us on Facebook, Emily is back in the hospital. She has either an ileus or a partial obstruction. Her pain is out of control and she is really, really sick. She finally got a pain pump of Nubain, on top of all the other lines/meds getting pumped into her. She is knocked out. I am going to try and sleep, although I am so heartbroken and sick to my stomach I don't know if I can. Please pray the the intestines either wake up or unblock soon, so she doesn't need surgery ... Or worse.

I am really scared. I don't think she has ever been this bad. :(

Friday, August 5, 2011

Cycles

Emily seems to have this horrible cycle of days that we can't get over. Just when she has a few good days in a row ... they come to a halt. Tuesday Emily had three long seizures, after not seeing anything major in almost two weeks. Then she went to sleep that night, and woke up seizing. She was struggling to breath and wouldn't come out of it. I didn't feel comfortable giving diastat when her breathing was so weird, so I packed her up and headed to the ER. By the time they triaged her, her sats were back up to 92 and she was a bit more responsive. They put us in a room quickly, and her sats were at 98 and she was fine. The ER doc asked what I wanted him to do, and I said send us home. Whatever it was, it was over and she was fine.

The next day we had TPN clinic, with our GI. The gj-tube we are trying to get for her wasn't in yet, so we still couldn't schedule the placement and endoscopy. Hopefully it will be in soon. The hope is that she can get medications through the j-tube and the g-tube can drain constantly. Right now I have to clamp her g-tube for an hour after meds, and it drains 24 hours otherwise. The problem is that even after an hour, most of her meds drain out of her stomach. They just aren't digesting. So she isn't getting most meds, and she is still gagging and retching when I give the meds. She throws them up half the time. Because we had been in the ER the day before with seizures, the neuro squeezed us in that same day because we were already in the clinic. He doubled the strength of her VNS cycles, and changed it from every 3 minutes to every 1.8 minutes.

Like I said, it's a cycle. The day we had TPN clinic, I told the GI that she was doing decent. Retching every morning, but not all day. Laughing more, playing with toys, more like herself. This morning she woke up retching and it never stopped. She threw up her morning meds. She threw up her next meds. I didn't even bother trying to give her any more meds.

She is asleep now, but keeps waking up retching. Luckily she only wakes partially up, and is going back to sleep. But it means I am nervous to go to bed, because what if she throws up in her sleep and doesn't wake up.

I hate these cycles. I wish Dr. Gregory House was real - I would take Emily to him in a heartbeat!

Friday, July 29, 2011

Funky town ...



Sometimes, we all get in a funk. I'm in one now, up to my shoulders. The easiest thing to do is hide myself away in the house, soak up the misery, and wait it out. Unfortunately, Emily is very high needs and soaking in misery is not an option. Rather, I am running around all day doing her medical care and trying to sprinkle some play and love and cuddles in, while splashing in the misery puddle.

I know it's not easy having a special needs child, and I know it's not something to take for granted. There are a million amazing wonderful fantastic things I have gained by being Emily's mom.

But sometimes it's just HARD. It's summer, and everyone around us is running around and doing fun things. The fair is here, but Emily wouldn't like it. She can't handle the heat, she doesn't eat the delicious and disgusting fried foods, she hates the smell of animals, she wouldn't be able to ride hardly any of the rides. She gets bored and grumpy very easy. We live mere miles from the beach, but there's no way we can go there. Emily hates sand, she hates water, not to mention that she has a central line that would NOT be good to get sand/water in. We would have to just sit on a blanket (after carrying here and all her connected accessories because her chair can't roll on sand). She would be miserable, and in turn so would we.

The biggest frustration is that we can't just pick up and go. Most people with kids can, even though they might think they can't. You grab a diaper bag or a bag of snacks and you go. In my case, I would have to pull up meds for every few hours, make sure her TPN is running okay, get her IV bag ready for the afternoon, and prep the next round of TPN. Not to mention figure out where to change her diaper/clothes when she has an explosion, and how to clean her up if she retches all over herself while we are out.

I'm just frustrated. I know that I am incredibly lucky to be Emily's mom, and I wouldn't change it for the world. Please don't tell me how blessed I am. I know. I am also tired, sad, frustrated, and bored at home.

And if you are one of our friends in real life and reading this, PLEASE do not be upset or offended by this. It happens every day, whether you invite us somewhere or not. PLEASE do not stop inviting us to every thing. The rare times that I *CAN* get out and go do things with you, are worth all of the "No, sorry" replies. ((love))

Thursday, July 28, 2011

Knock on wood ....


I spoke to the GI on the phone yesterday morning and we decided to wean off the Baclofen that was started to help with Emily's retching. I told her that we hadn't noticed a difference. Then my husband reminds me that Emily didn't wake up retching yesterday morning. She just woke up. It was such a NORMAL thing, that it didn't strike me as to how abnormal for her that was! This morning, no retching.

Could it be? I need the most gigantic piece of wood ever to knock on as I say that two mornings in a row she hasn't retched ..... KNOCK KNOCK KNOCK.

I just emailed the GI again to let her know that I am not weaning the Baclofen after all and perhaps it's working!!

We are going to try a gj-tube again for Emily. It will be placed by the GI herself during an endoscopy. This is a last ditch effort to get meds in Emily. Please cross your fingers, pray, turn in circles, and hope it works!

Tuesday, July 26, 2011

We went on a little vacation!


We decided to go out of town last weekend, to visit friends and support them with a fundraiser. They had a second annual bbq fundraiser to raise money for the Foundation for Children with Microcephaly. Emily doesn't like being away from home and was a grump most of the trip, but it was SO nice to get out of town for a few days! We visited the Charles Schulz (Charlie Brown creator) museum, and Jacob had his picture taken picking the nose of every statue we saw. :)

We are home now and settling in. Emily is doing the same, not very well. Her g-tube is draining 24 hours a day, except for right after giving meds. She is still retching and vomiting, and crying. I emailed the GI this morning but haven't heard back from them yet.

Something has to give, this girl needs a break!!!

And for all of you who live local to us, mark your calendars - October 15th is going to be Emily's giant 8th birthday bash! :)

Thursday, July 21, 2011

Where we go from here

We had TPN clinic yesterday, and I think I left equally frustrated as I arrived. It went beyond the fact that we weren't seen until 3:45, and our appointment time was 2:40. I make appointments for as early in the day as possible when we go to UCLA, because the traffic coming home gets horrid. It took me over 2 hours to get home, and it's only 42 miles.

The good news is that Emily weighed 15.9 kg, which is officially 35 pounds! So her nutritional status is good on TPN, at least it's doing one good important thing.

Her retching and vomiting however are uncontrolled. We discussed a few things, and for now we are going to try Baclofen. The thought is that it will relax the muscles in her stomach, and hopefully ease the retching. As a bonus, it should relax the spasicity and clonus in her feet/ankles. The downside is that it will further relax the rest of her body, which is already low tone. He said that if her drooling gets worse and she's choking more on secretions, we will increase her robinol. So we add a med, which will make us increase another med ... and then he has the audacity to say "She's sure on a lot of meds, we should see what we can discontinue soon". Seriously? Ugh.

If the baclofen doesn't work, we will consider a seperate j-tube. She doesn't tolerate gj-tubes, she vomits bile and screams with j-tube feeds. The thought behind doing it is that at least we could get her meds in her, without them being retched up. We leave her g-tube open to gravity 24 hours a day, except for right after giving meds. We're supposed to clamp it for 30 minutes to let them absorb, but I wait 60 minutes. Even at 60 minutes, a large portion of them drains out.

We go back in 2 weeks. I am crossing my fingers that the Baclofen works, without having too much detrimental effect.

Wednesday, July 20, 2011

TPN Clinic today

Emily has an appointment at the TPN clinic today. I am nervous. She is in a bad way again. Retching all morning, throwing up meds, grumpy most of the day. I hate this for my girl!!!

I am fearing that they will want to admit her again. I am going to be strong and tell them NO. We have a 4 day trip planned, we are leaving Friday through Monday. We are going to visit some friends and have a relaxing weekend. They can admit Emily as soon as we return if they want.

And then I realize how insane it is that I talk about admits like this ... but I just counted up on her medical sheet that I printed for the TPN appointment, and she has been in the hospital 16 times since January. Please cross your fingers and pray that they listen to my wishes!

Sunday, July 17, 2011

Slow and steady

We are doing good at home so far, KNOCK ON WOOD! :) Emily retches every morning for a while but it's not too bad. She is her normal happy self otherwise! As long as Fresh Beat Band plays over and over (thank heavens for DVR) she is content. She is playing with her toys and happy. It's so nice to be back at our "new kind of perfect". :)




I am in the midst of a huge organizing stretch. This house has gotten out of control while we were in and out of the hospital for the last 4 months. The first thing I tackled was Emily's medical supplies. You have no idea how happy this shelving unit makes me. I am such a supply geek!







Friday, July 15, 2011

Home again!


I'm sorry I haven't updated since the last post, which was quite a crisis time. She stabilized that night, and hasn't had another episode like that. Thank goodness! The metabolic geneticist came in the next day and talked to us in depth. He feels that the episodes were not true metabolic crisis but rather a reaction to pain. It makes a lot of sense, when we review it looking back. Her lactic acid was high, but that is expected when you are in pain or stress. This was the first time we had met the geneticist at this hospital, and he had a lot of good information for us.

He truly believes that Emily has some sort of maternally inherited mitochondrial disorder. I have reflux, migraines, fibromyalgia. Jacob has migraines and reflux. Emily has everything under the sun. We are waiting on authorization to do a mitochondrial gene array, and a whole exome study. He is also referring us to a different metabolic Dr, who is the leading mitochondrial specialist on the West Coast. His waiting list is about 12 months long, but hopefully he will get us in sooner when he sees how fast Emily has been going downhill.

GI wise, things are the same. She is on TPN for 22 hours a day. She retches a lot in the morning, but it's not too bad during the day. She manages to keep most meds down now. They started her on Rifaximin for bacterial overgrowth. It's a nasty, thick, orange medication. As long as we give it at a different time than her other meds, she usually keeps most of it down. As a side effect, her poop is bright orange colored which is interesting.

We go to GI clinic on Thursday, and then we are going out of town for four days. We are going to visit some friends we haven't seen in a while. It will be SO nice to get away for a bit! I can't wait. We have told Emily that she is NOT allowed to relapse, as we have vacation plans! :)

Saturday, July 9, 2011

Back in and not so well

Emily was re-admitted to the hospital on Thursday. She is back to retching, gagging, screaming. Her g-tube is draining so much that she is on an IV of fluids in addition to her TPN. Today she has had three horrendous episodes of what appear to be metabolic crisis. She screams hysterically, cramps up, her legs and arms get bent and stuck, she shakes and trembles, and drools and gags. The last time she did it today the nurse called the Dr's in. They immediately ordered stat labs to check her metabolic function and found her lactic acid to be high. They said it wasn't quite "acute" but was elevated enough to be concerning. It shows that she is verging on metabolic acidosis. She is stable and asleep for the night, and we are going to see the genetic/metabolic/mitochondrial team in the morning. Please pray for some answers. The neuro and GI still think that everything that is going on is a progression of her underlying disease. Without knowing what that disease is, it's hard to know how to treat or how far to push her. Please pray for answers and guidance!

Tuesday, June 28, 2011

Jacob's 11th birthday






I cannot believe my baby is eleven! I can't believe I am old enough to have an 11 year old! Wow. He had a Harry Potter themed party on Saturday and it was a good time. It was the most relaxed, calm party he's ever had. He only invited 5 kids, and it was calm and they just played and went to the park for a while. We did a pinata, and make your own potion station (little containers from Ikea and different colored Sixlets candy). He chose meatballs in my "special sauce" as he calls them, hotdogs cut up in bbq sauce, hawaiian sweet rolls, and every flavor of Takis chips. We had a Harry Potter cake and double brownie ice cream. I realized that you can tell your kid is growing up when instead of a bunch of toys as presents, he gets Itunes and Target gift cards! Then this morning, which is his actual birthday, he left for Seattle with my in-laws for a week. I'm going to miss him!

Tuesday, June 21, 2011

It's been a rough road but it seems to have calmed down!

I haven't kept this blog updated, and I feel bad for that. I hope that most of you have found me on facebook, because I am better at updating there. I have lost almost 50 followers over the last 4 months. :( Hopefuly I can earn them back!

So here's the recap. Emily had been retching and vomiting every morning for months. It started in January, and progressively got worse. She had been getting three bolus feeds of blenderized diet during the day, and 12 hours of g-tube feeds overnight through the pump. She started throwing up in the morning to the point that she was losing weight and becoming dehydrated. The GI said he thought it was seizure related.

In March it was so bad one day that I took her to the local ER, because I assumed it was out of control seizures. They loaded her with ativan and such and sent us home. The next day it was just as bad, so I drove to UCLA where we see neuro. We were admitted through the ER for an overnight VEEG. The vomiting and retching were unrelated to seizures. We were discharged after two days. We made it a week at home, and then had a GI appointment. I explained that it was NOT seizure related, and it was getting worse. The GI said (in his exact words) "She looks like shit. You need to be admitted right now". We were admitted to CHOC and began testing. An Upper GI showed a loose but intact fundoplication and no hernia. An endoscopy showed redness consistent with persistant vomiting but no abnormalities. Biopsies were normal. Her g-tube was changed to a gj-tube, and we tried feeding through the j port instead. A gj-tube goes in the stomach and then threads into the jejunum (intestine) to allow you to feed the patient and bypass the stomach. This made things much worse, she would scream in pain and vomit bile non-stop. She had a PICC line placed, and was sent home on TPN.

We were home for 3 days and then we had a pediatrician's follow-up appointment. She sent us straight to the ER. Emily was still retching non-stop, even on TPN, and had a UTI. They discharged us with meds.

Two days later Emily had her VNS (electric implant for epilepsy control) surgically placed at UCLA, she went home the same day. The next day we went to CHOC's ER for continued vomiting. This time she had an Upper GI done with small bowel follow-through, although it was done incorrectly so it didn't give them any information. They started worrying about pancreatitis or gallbladder problems. We did ultrasounds four times, a CT scan, and many xrays. She has sludge in her gallbladder but nothing more. During this stay she pulled her PICC line out and had to get a new one. We were inpatient for 8 days and discharged still on TPN.

Two days later we took her to the ER at UCLA because CHOC had said they didn't know what else to do with her, and she was still retching. She was diagnosed with another UTI and had another Upper GI done. She was discharged after 3 days.

Two days later (notice the 2 day at home pattern) we had to go back to UCLA to the Urgent Care, because she was throwing up the antibiotic they had prescribed for the UTI. She was then prescribed IV antibiotics which I did at home with the TPN.

The day after this ER trip, we went to the ER at CHOC because she was draining blood from her g-tube. They said it was to be expected when you retch as much as Emily has been. Her blood counts looked okay, so we were sent home.

Two days later (see!) we were back in the ER for screaming and retching. We were admitted to CHOC for almost 3 weeks. They ran more ultrasounds, xrays, etc. Durning this stay she finally had a Broviac placed. It came out two days later and was replaced. She was sent home again on TPN, and CHOC said they had no clue what to do with her. They kept saying she should go to UCLA and be considered for a small bowel transplant. But they couldn't tell me what was wrong with her, so how could they say this? It was a nightmare. At one point during this three week hospital stay we were told we should take her home on hospice. Ugh. The pain team was consulted and she got some relief. Thankfully we decided not to do hospice since, again, they had NO idea what was wrong with her.

We were home for almost a week and then took her to the ER with a fever. Anytime you get a fever over 101 with a broviac (central line) in place, it's an immediate ER trip for blood work. Luckily her labs were good, so they said it was a virus and safe to take her home. Two days later (damn this two day pattern!) they called back and said to return her immediately to the ER. Scared the crud out of me. Turns out she had a UTI and they needed to prescribe antibiotics. They couldn't just call them in to the pharmacy?

We made it almost three weeks at home, and then the retching got worse again. We went to a GI appointment and were told "Emily is a mystery. Somtimes we don't even try to figure her out anymore". That is not very reassuring! So we took her to the ER at UCLA because their GI and motility departments are award winning. She was admitted for 10 days. She was diagnosed yet again with a UTI. She had a gastric emptying study, electrogastrogram, and some test where they determine your caloric needs based on your O2/CO2.

During this last stay at UCLA, they managed to get her off of 6 meds CHOC had put her on. They started her on a couple of new meds. The biggest thing we did her was to compress her TPN to only 18 hours a day, instead of 24. In between, she now tolerates some blenderized diet again! Before we feed her, we give her something called "magic mouthwash" through her g-tube. It's a mixture of viscous lidocaine, benadryl, and mylanta. It numbs up her stomach enough that she tolerates some feeds.

She has finally gained some weight, she is 33 pounds! They would like her at 35. Remember she's almost 8 years old. She's teeny. :) She is finally into 5T clothing for once. We still don't know for sure what is going on, but we have narrowed it down a bit. We know her stomach works decently, it's all the small bowel and intestine that don't. What we will do about it depends on how she does over the next couple of months. The hope is that her GI tract will wake up a bit and she will be able to get off of TPN.

That is the long version. :) In the meantime, Jacob graduated from 5th grade and is now officially a middle schooler. Emily is a 3rd grader now. We are getting ready for Jacob's birthday party this Saturday, I cannot believe I have an 11 year old! I will post again soon, and hopefully be a regular updater again. :) Thanks for not giving up on us!

Wednesday, May 25, 2011

Blogger is being a Bother!

I have typed up an update three times, and all three times it has been eaten by Blogger. I will try again later.

Equally annoying, if not MORE annoying, is that Blogger won't let me comment on anyone else's blog. It has me log in to the google account, then enter the "word scramble thing". Then when I hit submit it has me log in again. Then unscramble. Then log in. Then unscramble. Rinse and repeat.

So, I have read up on lots of our friends this morning. I have many things to say, but evidently Blogger doesn't want you to know.

I will try and find time later!

Friday, May 6, 2011

What's going on

Emily is still in the hospital. She had a broviac placed yesterday, since she pulled her PICC line out for the second time. She is getting TPN 24 hours a day through the line. She is still not tolerating anything in her stomach, except for tiny amounts. She can handle a 5 ml dose of medication, but usually the ones that are 10 ml get retched up still. Her Upper GI (three of them), endoscopy, ultrasounds, xrays, 2 CT scans, are mostly normal. The only thing that the GI can find that is abnormal is that her gallbladder has some sludge in it, and her bile ducts are dialated. The HIDA scan on her gallbladder showed it functioning okay, although it is over-reactive and hyper-something. The GI wants her gallbladder out because it's the only thing we can find that COULD be causing her symptoms. Surgery says the tests are not strong enough to convince them it should come out. Emily's regular surgeon is in Belize until Monday, so we are in a holding pattern until he comes back. Our regular GI is on the hospital round starting Saturday, so it will be good to have people who really know us. Our GI is going to try and convince surgery to remove her gallbladder, since after 8 weeks we have to do SOMETHING to try and give her some relief.

Emily has moved from the non-stop retching and throwing up into screaming in pain/agitation all day and night. She is getting many sedatives, pain meds, etc every 4 hours and still screams. Her heart rate shoots up and she is just a mess.

We have been in the hospital since March 13th, with only a few days home here and there. PLEASE pray that they can figure out soon what is going on! I just asked about ovary or uterine cysts, they are going to review her previous tests and see if they could see something there. We are really grasping here, but something is causing her pain and no one knows what.

The other thought they have is that this is simply a "progression of her underlying disease" which no one can diagnose. Remember that overall, Emily has no diagnosis. They are repeating a lumbar puncture today, she had one in 2005. They are testing for neurotransmitter disorders. She is being tested via blood for palmitoyl—protein thioesterase deficiency. If she goes into surgery, they will be repeating her skin biopsy, which was also done back in 2005. They have repeated her chromosome micro-array, and it was normal.

If no one can figure out what is wrong and they cannot find a source for her pain, they say that it is a neurological issue and nothing can be done for it. They will then continue having the pain team work with us and transition us to hospice.

PLEASE pray for guidance for our doctors!! We are to our limit. I am so sad that we will be in the hospital for Mother's Day. My 10 year old Jacob needs his family all together!

Sunday, May 1, 2011

Quick update

We are still in the hospital. I will try and get on here tomorrow to post an update.

Monday, April 18, 2011

Can I say "again"?

Long story short, we didn't get transferred on Friday. We got discharged and went home. Sunday was about the worst day she had so far, so we went to the ER. This time we went to the hospital where our neurology team is. Emily is hooked up to an EEG right now, and then we will be getting a GI second opinion. They agree here that her quality of life has gone downhill to a point that is unacceptable.

Please pray that the doctor here have the wisdom to get to the bottom of these things soon! Today marks the sixth Monday in a row, in a hospital.

Friday, April 15, 2011

Still in ...

We are still in the hospital. Our Children's Hospital can not figure Emily out, so we are being transferred today to the hospital where our neurologist and neurosurgeon are. We will be consulting with them, and getting a second opinion from their GI team.

Emily has been in and out since March 13th, with only 5 or 6 nights home in that time. I am exhausted.

Her GI tract is a mess. She can hardly keep her g-tube meds down, usually even that gets retched up. She is TPN dependant. Extreme diarrea. Retching, gagging, drooling. All GI tests are normal. Upper GI, small bowel follow-through, endoscopy, CT scan, ultrasounds, xrays. Everything.is.normal. How frustrating!

So we are sitting around today waiting on transport to transfer us to the other hospital. It's an hour to two hours away depending on traffic. Of course, now it's rush hour time so the drive will be horrible. I'm hoping that it will after rush hour time, although being admitted late at night sucks. Then we're heading into the weekend ... I imagine we will do a lot of nothing between now and Monday.

Please pray that the second hospital is able to get to the bottom of Emily's problems! My little girl needs a break, and we all need to get HOME!

Sunday, April 10, 2011

In again ....



(I need to state first for the record, that the picture above is a bit deceiving. Emily looks like she has a little paunch belly, doesn't she! She is really skin and bones, this was bloating. I wish her tummy was round like that!)

Emily had her VNS surgery on Wednesday, everything went great with that! She was in recovery for about an hour and a half, and we were discharged. Then Thursday morning she woke up retching and gagging. Her home nurse gave zofran through the g-tube, she retched it up immediately. She redosed it, and it managed to stay down. So 20 minutes later her nurse gave all her morning meds, and she retched them up. In between all this she had three explosive diapers, so things weren't good. We immediately packed up and headed to the ER. Good thing I had her nurse with me to sit in the row with Emily, so she could suction her as needed while I drove.

We are back in the hospital, still with her gut shutdown completely, still with no answers. We did xrays, ultrasounds, more xrays, and more ultrasounds. They found a lesion/cyst/something on her gallbladder. We are waiting until tomorrow (Monday) and then going to do an MRI. The doctors have to contact her neurosurgeon first to get the proper information on how to do the MRI now that she has a VNS implanted. Once we get the MRI results we'll know more. They warn me that this may have nothing to do with what is going on .. that she could have a congenital cyst that's been there forever ... or it could be "the answer". Who knows. Depending on what it shows and how she does, the GI might redo the Upper GI, this time doing a small bowel follow-through as well.

I am exhausted. We have been in and out and in and out of hospitals non-stop lately. I am so sick, bad head cold with sinus headaches. The stress, poor eating, and bad sleep aren't helping the situation.

Please pray that they get to the bottom of these problems soon. My little girl is being SO good in the hospital, but how much can we all take? When she's not nauseas or in pain, she is her normal smiling self. She has all the nurses wrapped around her little finger. They literally argue over who gets her case each day. I am so glad that people love my girl!!! I just hope we are done with this soon.

Tuesday, April 5, 2011

In and out, in and out

That's how we have been lately. In the hospital, out of the hospital. In the hospital, out of the hospital. Three weeks ago it was two trips in a row for seizures. Then on the 24th it was in for feeding intolerance, retching, pain. That stay lasted 12 days and we were finally discharged, but TPN dependant. Her g-tube was switched to a gj-tube, and she doesn't tolerate j-feeds any better than she does g-tube feeds. An endoscopy showed significant irritation in the esophagus, but nothing serious. An Upper GI showed that her fundo is intact, although a little loose. She had a PICC line placed (twice, since she pulled the first one out). We came home Friday on TPN 24/7. By Monday morning she was retching, gagging, crying again. Luckily we had a pediatrician's appointment already scheduled, and she sent us straight to the ER. They ran labs, bolused extra fluids in, and diagnosed a UTI. The GI said we should go home with zofran and antibiotics, and we were on our way. This morning she retched just as badly. I called the GI first thing this morning, because he told the ER he would see us today. Now it's 11:30 and they still haven't called back to tell me when we'll be seen today.

She is going in tomorrow at the other hospital for her VNS placement. I have explained all that is going on, and everyone is okay with her proceeding with the surgery.

Please pray she does well with the VNS placement, and that we really get to go home after like they say we should. Please also pray that someone can get to the bottom of her GI problems. Her gut is shut down for whatever reason. It's a mess.

Tuesday, March 22, 2011

Ugh ....

It's been a long time since I posted again! I wish I could come up with an extra 30 minutes every day to sit and post.

Emily has been in and out of the hospital. Right now she's inpatient. She has air in her bowel wall and possibly her chest, she isn't tolerating feeds, she's losing weight, she has blood in her stool, etc.

The GI thinks it's related to whatever mitochondrial disorder she may have, otherwise just an overall gut shut down. He is hopeful that it is temporary.

In the meantime, her VNS was supposed to be placed Friday. Surgery has been postponed indefinitely. :(

Please pray for Emily. She needs to get better quick!

Sunday, March 6, 2011

Chex Mix Giveaway


This is the week of giveaways on my blog! :) I am excited to share some information about Chex Mix with you, and offer you a chance to win a prize package.

I received a box of Rice Chex, a measuring cup set and a Chex® Party Mix tote bag from General Mills through MyBlogSpark. We made Muddy Buddy Chex Mix, and it was a huge hit! Even my 90 year old Grandmother loved it.

For decades, savvy home cooks have been using crunchy Chex® cereal as the starting point for their family-favorite party mix recipes. Many Chex® Party Mix recipes can be made in the microwave in just 15 minutes and call for ingredients you already have in your pantry. From holiday gatherings to impromptu get-togethers, Chex® Party Mix provides a snack idea that will impress your guests in no time.

Whether you’re looking for sweet or salty, Chex® Party Mix has something to offer every taste bud, making it the perfect go-to snack for entertaining this holiday season. Four gluten-free varieties of Chex cereal help ensure that even party guests with gluten restrictions can enjoy Chex® Party Mix recipes like the NEW Chex Lemon Buddies! Remember Multi-Bran and Wheat Chex are not gluten-free.

I am offering a prize pack to one of my readers! All you have to do to enter is leave a comment on this post and tell me your favorite way to eat Chex cereal. Do you like sweet mixes, salty party mixes, or do you prefer to just eat your Chex with milk?



Jacob will draw one name from a hat on Wednesday, March 9th, to determine the winner. Be sure to enter now!

Saturday, March 5, 2011

More information, and a Yoplait winner!

First off, congratulations to Joan's Blessings - you are the winner of the Yoplait giveaway! Please email me your name and address. MyBlogSpark will then mail you your gift pack. :)

Okay, so I took a few days to stew over what the metabolic dr talked to me about, and I am in a better place with it. We know that Emily has some genetic condition that is yet to be diagnosed, this is not a surprise. We know that it is causing her to decline lately, that is obvious. The seizures are the biggest cause of her regression right now. Hopefully once we get the VNS placed and turned on, we will see some relief of them.

We see the geneticist on Friday. I am supposed to tell her that the metabolic dr thinks we should re-run her micro-array. It was done 4 years ago and the test now detects so many different genetic mutations than it did 4 years ago. Also he wants her to re-run Emily's SCN1A test. It was done by UCLA at a lab that is evidently notorious for wonky results. It came back with a mutation, which could explain Emily's Lennox-Gastaut Syndrome. Unfortunately, it's an "unknown mutation" so it could also mean nothing. Hopefully if we rerun this test through Baylor University, where we do all our metabolic labs through, it will come back with more information. Or perhaps the geneticist will be able to interpret the results we have - we'll see on Friday.

We go back to metabolics in 3 months. He would like to order a High ThroughPut Mitochondrial Screening. He said that it tests over 700 mito genes. The other thing he wants to do is try and get us in a trial of a Whole Genome Sequencing test, that's for the whole family. The affected child, non-affected sibling, and both parents. Neither of these tests would give us a diagnosis that would be curable. At this point, we get that. Whatever Emily has is not something that can be cured. All we can do, and will do, is treat her medical conditions as they come. Make sure she is happy and comfortable. And spend every minute of every day loving on her.

The High ThroughPut test right now costs about $16,000. He said our insurance may cover it, but may not. If we wait 6-12 months, Baylor is going to offer the same test and he thinks it will only be about $5000. The insurance should approve that no problem. Every time we've done gene sequencing (they isolate and sequence one gene, that is how we got her SCAD Deficiency diagnosis confirmed) it costs about $2000. Wow! We've done tests for Angelman Syndrome, CDKL5, MeCP2, FOXsomethingforrett, Cornelia deLange Syndrome, Ethylmalonic Encephalopathy, and on and on and on.

The metabolic dr said that of his 3000 or so patients, Emily is one of the top 5 who have had the most testing done without getting a diagnosis. Wow. She really is a mystery.

So that's a bit more of an explanation of our appointment last Thursday. It was not a fun appointment. Oh, and her weight is down to 28 pounds now. She's 39" long. 7 years old - 28 pounds. She is SO skinny now. We are working on how to add more calories to her blenderized diet during the day. Our dietician is great, we are lucky to have someone who approves of our blenderized diet.

This is a busy week. My mother-in-law is coming in tomorrow for a few days. Jacob has Robotics Club after school this week, for a competition at JPL next week. That is exciting! Add in his regular tennis lessons, Emily's regular appointments, etc .... I am going to need a nap through-out the week for sure!

Lastly, I have Emily's room almost completely redone now thanks to Make a Wish. They had delivered the last items last weekend, but I didn't get a chance until today to get it all put together. I will try and get some pictures tomorrow to share. Her room looks great!! :)

Friday, March 4, 2011

So much to do and not enough time to even think about doing it all


I didn't have Jacob draw a name yet for the Yoplait giveaway, I will have him do it this weekend. Sorry for the delay.

Emily has had a crappy seizure week. She got Klonopin many, many, many times for seizures. She got Diastat twice this week. Once was at a metabolics appointment, where we threw her on a pulse ox machine and she was satting at 88 percent. Not great. :( I called her insurance case manager and was told that the VNS authorization should be in by next week. Three weeks later?? Insane. So we are just waiting (im)patiently for that.

We had a long appointment with our metabolic dr this week. It was not a great day. Emily was doing horrible, and the doctor really loves her. We talked and talked, and we ended with both the Dr and I in tears. We had The Talk. You know, the one where you talk about the future and what it looks like. The one where you compare Emily today to Emily a year ago. Or even three months ago. Her decline is rapid and scary as shit. :(

We talked a lot about mitochondrial disorders and how the metabolicist has always felt she has one. We did a muscle biopsy 5 years ago and it was normal, but he says they were only looking for a few disorders. We are looking into some much, much more extensive testing.

He was quick to tell me that any answer we get at this point won't be good. There is nothing we can diagnose her with at this point that will be treatable, or have a cure. It will just give us an answer, and information for the future.

This appointment SUCKED. I am just so thankful that I really like this Dr, and I know he loves Emily. It was clearly hard for him to talk to me this bluntly, as it was for me to hear it.

We see the geneticist next Friday. We haven't seen her in 4 years. I don't expect anything earth shattering to come of that appointment, other than her reiterating what the metabolic Dr said already.

We have a busy weekend coming, lots of house cleaning and organizing. My mother-in-law is coming for the week, so it will be nice to see her. She hasn't seen the kids in forever. Hopefully Emily will have a better week next week, while she is here.

Wednesday, March 2, 2011

Yoplait Smoothie Giveaway!



I was happy to get a package from MyBlogspark.com recently! Inside was coupon to try Yoplait Frozen Smoothies, a drawstring backpack, a water bottle and a fitness journal. We went and bought our Smoothie packages. We got two packages of the Strawberry Banana flavor, because it says that one package is only 2 servings and there are three of us.

Including Yoplait Frozen Smoothies in your diet is a quick and easy way to help you get important nutrients, like vitamin C or calcium your body needs. And with seven out of every ten Americans not eating the recommended two to four servings of fruit per day, Yoplait Frozen Smoothies could help you reach that goal.

Each package of Yoplait Frozen Smoothies contains one full serving of fruit (1/2 cup) and 110 to 120 calories per serving. Not only does Yoplait yogurt contain live and active cultures, but when prepared with skim milk and made according to package directions, Yoplait Frozen Smoothies are a good source of calcium. So raise your smoothie glass and toast to a new you!

Found in the frozen fruit aisle of your local grocery store, Yoplait Frozen Smoothies come in four delicious flavors. Be sure to check out the newest flavor, Blueberry Pomegranate, along with other favorites like Triple Berry, Strawberry Banana, and Strawberry Mango Pineapple.

I am able to offer a gift package to one of YOU! :) To enter, all you have to do is leave a comment on this post. Tell me what your favorite healthy snack or dessert is, that your kids actually eat and enjoy! These smoothies are definitely going to be a common dessert in our house. They are so cool and tasty!



Jacob will be drawing one name from a hat on Friday, March 4th to choose the winner. Be sure to comment now to enter!

Thank you to Yoplait, who provided me with the product, and the information through MyBlogSpark.

Tuesday, March 1, 2011

Sweet Maggie ....


One of the best parts of being online and having friends with special needs kids is that we all support one another, help one another, and fall in love with each other's kids. One of the worst parts of being online and having friends with special needs kids is that our heart breaks as we lose our sweet loves.

Little Maggie held a special place in my heart. Her mom, Michelle, had contacted me when Maggie was little. She was so similar to what my Emily was like as a teeny-tiny. Our girls went through so many similar things.

My heart broke this week when I read about Maggie's health worsening. Then the blog post that we all were afraid to read. Sweet Maggie has passed away. :( My thoughts stray to her adorable little face all day. I cannot imagine the heartache her family is going through.

My prayers are with the Agnew family during this hard time. (((hugs)))

Wednesday, February 23, 2011

February is already half way over ..

That means we are almost one month closer to summer! Which means I am almost one month closer to NOT being PTO president anymore! LOL I am soooo over this.

In more pertinent news, Emily is going to the neurologist on Monday for a follow-up. We haven't actually seen our neuro since she got the diagnosis of Lennox-Gastaut Syndrome back in November. We have been inpatient since then and seen his partner (who is fantastic), but we haven't been back to his office since then. I have quite a few questions and concerns for him, so I hope he's prepared for us!

Emily is having such insane rage episodes. She gets SO angry. She screeches and cries and pulls her hair or glasses or whelchair straps or whatever she can get her hands on. She scratches herself, or whoever's holding her, or whatever she can reach. She flings things if you try to give her a toy to distract her. And the worst is the biting. She bites constantly. If you try and soothe her during these episodes, you will get bitten. She has bitten my shoulder, my head, my ear, my hands, my arms, my thigh, even my boob. Ouch. :( The worst part is that she is biting herself now when she's in a rage. Both of her sweet little hands have tooth marks on them. :( I know that Keppra has rage as a known side affect, but she's been on Keppra for over a year. This problem has been getting worse and worse for the last few months, but the last two months have gotten unbearable. She's on Banzel also, and then Klonopin and Diastat as rescue meds for seizures. It's just out of control now, and I definitely am going to talk to the neurologist about it. She can't continue to bite us or herself!



Her seizures are about the same. The Banzel really has helped reduce the number of drop seizures she has, but she still has them daily. And her myoclonics and absense seizures are just as bad. This weekend was terrible, it was one seizure after another. There's no use going to the ER for this, as all they do is say "Well she is always going to have seizures". It's frustrating.

We are still waiting on authorization for her VNS placement. I know it's not a guarantee that it will help and I am not putting TOO much hope in it, so that we won't be too disappointed if it doesn't work. But I can't help but pray that it does slow the seizures down. They are slowly taking Emily from us.

In happier news, Emily got part of her Make a Wish already, and the rest is coming next week! She got her Sleep Safe Bed on Friday, and the EmFit monitor. It is SO nice to have a big, safe bed for her to be in at night. What a relief it is! We haven't playing with the EmFit monitor enough yet to know if it is awesome or not, but I'm excited about it. This weekend we will spend time figuring it out. The rest of her wish comes next Wednesday, with her formal presentation. They are bringing some storage solutions, decor, etc. It is SO fantastic! Thank you, Make a Wish!!




Emily has a lot of appointments coming up. The neuro is Monday, then metabolics on Thursday, and then next Friday she sees the geneticist. We haven't seen her in 4 years, and I am sure she will be no closer to getting us "the answer" to Emily's medical problems, but it's always good to see her ocassionally. Lastly, Jacob is finally getting into the neurologist for his facial tic/twitch. I am hopeful that the neuro will be able to help him.

It is a cold and rainy week here in So Cal, and I am ready for summer! (Then I will complain about the heat, and how it increases Emily's seizures, but at least we won't need to wear jackets every where we go!) I hope you all have a good weekend coming up. We are going to spend some time cleaning the house and organizing Emily's room with her new furniture!

Tuesday, February 15, 2011

Appointment update

We met with the neurosurgeon yesterday, and I really liked him. He was very personable, and explained things completely. I already knew all about the VNS from researching it so much and talking to other moms, but it was nice to have it all laid out by him anyway. We went over all the "informed consent" that he required, and then I signed consent forms. Now I just wait for the authorization and then we schedule the surgery. He said it should happen within 2-3 weeks. I am hoping it is sooner than later! I know that the VNS will be placed and left off, and then turned on later and adjusted for a while. I know that the process may take some time, but I am anxious to get it started.

Emily had a re-evaluation by her OT and PT today, and it really brought to light what these seizures are doing to her. They haven't worked with her for months, as she doesn't receive regular therapy right now. She hasn't met any goals for a year, and she doesn't cooperate enough to benefit from therapy. Add in the seizures and medical instability, and there's no use going to therapy right now. Anyway, they were doing her evaluation today and it was hard to sit through. There are some things she is doing great at. She is doing great at picking things up, touching different textures, etc. She is doing great at crawling to toys, unless it's a really bad seizure day. But her balance is much worse, her posture and stances are much worse. Her protective reflexes are now delayed by much more than they were. Little things that just show how much these stupid seizures are tearing her down. We have GOT to get them under control!

In better news, the Banzel she is on does seem to be helping. She is having many less atonic seizures than she was. She can go 30 minutes sometimes while sitting on the floor without a single atonic seizure! I know that doesn't sound long, but it was literally every 30 seconds for the last few weeks. Her myoclonics are a little better as well. We are seeing a new seizure type though, where her eyes kind of roll up and around in a circle, and her lip twitches. It is very subtle, and it happens in 2-3 seconds. She does it off and on all day though.

Her gagging and retching from last week is gone. I figured out the cause finally. We were trying to increase the calories in her overnight feed, as she has been slowly losing weight. When I went back to the lower calorie feed, she did fine. Tried upping it again, retching and gagging. So I have an email in to the dietician to let her know.

Her g-tube site has been angry lately, I am going to call the GI in the morning. She has always had a BEAUTIFUL g-tube site. Never needed gauze, never leaked, not had a granuloma since the very first few months of placement. Now it's crusty, red, weepy, and raw. I am ordering some Button Buddies to help, but I think it needs to be seen by the GI in the meantime.

In some GREAT news - her Sleep Safe Bed is being delivered on Thursday!! I don't know when it will be put together, as it is part of her Make-A-Wish. I have to call them in the morning and let them know it's scheduled for delivery, to find out if the rest of her bedroom make-over is ready. Then we can set a date for the wish to be fufilled. I am SOOO excited for her new bed!! :)



As always, I like to end this post with a FUN picture. This was taken Valentine's Day weekend at a birthday party we were invited to. I love this new shirt she's wearing from 3E Love!! If you haven't checked them out, you just have to!

Monday, February 14, 2011

VNS consult is today

We meet the neurosurgeon this afternoon for Emily's VNS consult. Last night I (stupidly!) mentioned to my husband that she hadn't needed Diastat since Friday morning, and her seizures had been mild for the last two days. She then woke up at 4:30 this morning seizing off and on, and by 5:30 required Diastat. Now it's 9:15 and she just had three atonic (drop) seizures back to back and then fell asleep on the living room floor.

Please pray that the neurosurgeon can get her scheduled ASAP for the VNS. Even more, please pray that it will bring Emily some relief from these damn seizures!!

And just so you can see that Emily is still her smiling, amazing self in between these stupid seizures, here's a cute picture from yesterday morning. :)

Thursday, February 10, 2011

Remember that hole I said I retreat to?

Well I am stuck in it. I imagine I have lost a bunch of my followers since I haven't posted in FOREVER. I hope I can get you back!!

I really do retreat when things are crappy, and things are definitely crappy lately. Emily has a kleb. bacterial UTI right now. We spent the afternoon in the ER doing chest xrays and nasal flu swabs. She has been retching and gagging and coughing up this phlemy crud all week. Today at school she was doing it and she stopped breathing each time. They freaked out (go figure - it seems we are all sort of addicted to breathing!). I picked her up and went straight to the ER. We got the all clear, but they said her sinuses are a wreck. We have winds here called "The Santa Anas" and they are warm and super strong. They spur everyone's allergies, and evidently Emily is not immune to this.

We are going on Monday afternoon for her VNS surgical consult finally at UCLA. I hope and pray that they can get us in for surgery quickly. I am going to ask about having a port placed at the same time.

If you haven't given up on me, thank you! I promise to try and post more often. I have pictures and things to catch up with on my blog and I will do it soon!!

Sunday, January 23, 2011

Knock on wood as I type this ...

It's 9:30 am and I haven't seen a single seizure since Emily woke up. KNOCK ON WOOD! Bang bang bang. :) Some have asked me about her medications ... she is now on Keppra and Banzel, both twice a day. She was weaned off Trileptal. She gets klonopin dissolvables orally for seizures over 3 minutes, and then a second dose 15 minutes later if she's still seizing. If she continues to seize 30 minutes after the second dose, she gets diastat. I think that the klonopin stays with her for a while, so it's making her more dopey and wobbly. Usually the first dose of it is knocking out her seizures, but only for a few hours. So she's getting the klonopin 2-3 times a day. I know it contributes to her wobbliness and grumpiness, but it's better than non-stop seizures.

They told me in the hospital last week that if I don't break up the long clusters of seizures, they will lead to status. I am terrified of status. So we do what we have to do to keep her from clustering a long time.

In the meantime, I am doing laundry, cleaning bathrooms, writing tons of notes, and getting ready for my cruise. I leave on Friday, my 35th birthday! :)

Saturday, January 22, 2011

Quick Update

Again I find myself not updating. I am quick to update on Facebook because I can do it from my phone, but I have a harder time sitting down here to really type things out.

Emily is no better. I have had to pick her up from school this week a few times, due to increased seizures. On Friday it was the worst. She has gotten her rescue meds (clonazepam wafers and diastat) daily. Her seizures are out of control. I just emailed the neurologist, and the neurosurgeon. I know the VNS won't help immediately, but the sooner we get it done the sooner it can help. And in the meantime, this is insane. This video shows one of her seizures, it starts at about 55 seconds.



The girl can't even sit up and play without falling over. It's horrible. Her development is regressing before our eyes and it makes me want to scream. I hate this. :(

Saturday, January 15, 2011

How is Emily doing?


I have come to realize that different people blog differently. Some people are able to blog daily, regardless of how busy their lives are, and keep everyone up to date. Some people blog when things are rough, because the support we get online is irreplaceable. And then some of us, when times are tough, bury ourselves in our lives and hide from our blogs. Not that I don't want to blog and tell you all how Emily is doing, but I just can't bring myself to do it lately.

How is Emily doing? The answer depends on who's asking. Most people get the answer of "She's good, thanks for asking".

The honest answer? She's doing horrible. We just had an ER trip for increased seizures and an overnight hospital stay. She had a CT scan (these new seizures are affecting her left side seriously). She had an overnight EEG.



She is now being referred to a neurosurgeon for a VNS. If you are lucky enough to not know what a VNS is, here's a link with some basic information. This is not something that will stop all of her seizures (unless we get extremely lucky, but not likely). It is something that hopefully will lower the number she has each day, and help us control the bigger/longer ones.



Right now all I want to do is grab my girl and rock her in our comfy chair and watch cartoons. Unfortunately real life gets in the way of that, so we forge ahead and try our best to deal with this f*&n seizures.



And that is how Emily is doing lately. :(