Thursday, July 30, 2009

Real Quick

Real quick: Harley's blood clot has doubled in size over the last few days. Cameron's coming to pick me up from work now to talk about what we're going to do about it. Will update as soon as I can.

7-29-09 Bad News

Quick update because I’m working the night shift tonight. Harley has a blood clot in the upper right chamber of her heart now, most likely from the IV in her neck (which is now out). They are giving her an anti-coagulant (makes the platelets [things that cause clots in blood] not so sticky so the clot can dissolve) and are going to do another ultrasound on Friday to check it. Only reason they found it is because they did an ultrasound on her heart to check her VSDs (small pinholes in her heart that are closing and they aren’t worried about them, just watching them still: she’s had them since she was a week old) to make sure blood wasn’t leaking out of her heart and they saw it.

More bad news to report is that her liver is starting to fail. The exact words were ‘not processing proteins’ and that her liver had ‘high enzymes’. For those of you who don’t know this is the next phase of Alpers Syndrome. They didn’t really tell me a plan of action for her liver failing, just that they’re trying to get all the levels of things like protein and potassium stable in her blood so she can go home and we can spend time with her. The only time frame I was given was weeks to months, it just depends.

Her bloating is a little better, but she’s not back to her normal self by no means. Apparently protein helps with bloating by keeping fluids in your blood stream instead of them leaking out and causing bloating. Well, the Lasiks, on the other hand, helps bloating by flushing out the excess fluids, including the protein. So they’re trying to keep protein in her and flush out the excess fluids, so it’s a vicious cycle. They are cutting back on the Lasiks and the doctor said she should be off of it in a few days. Also, this new ‘Boost’ formula supposedly has more protein in it and that’s why they switched from PediaSure. What I’m thinking is if her liver isn’t processing the protein correctly, then is that why she’s bloated? Just my train of thought…

Lastly, her seizures are still pretty bad. A little better today because she got more sleep, but still pretty bad. I talked to the doctor today and he said he’d look into raising her Phenobarbitol (the anti-seizure med they cut back on) back to the normal level it was at when we were home.

So, once again, there’s really nothing any doctor here on Earth can do for her, so it’s up to God now. Please, if you do pray, just say a quick one for her so that she’s at least comfortable and can enjoy the time she has. Thank you.

Tuesday, July 28, 2009


Last night I didn't get much accomplished. They did finally take the IV out of her neck and put one in her left hand! Yay! That helped the swelling in her face, too. They apparently changed her formula AGAIN to something called Boost. It's 'Complete nutrition for kids age 1-10', vanilla flavor. She's still bloated, still having seizures, still sleeping alot, still won't talk to us. They are giving her albumen (I was told it was protien and when I looked it up it's the whites of eggs, which would explain it being a good source of protien, but my husband is allergic to eggs: he can only eat 1 or so then he throws them up. He's had reactions to the flu shots, which has eggs in them, too. Harley hasn't really eaten enough eggs to tell if she's like her daddy or not.) which they say helps the swelling. She fell asleep last night, so they weren't concerned about her seizures because they stopped... because she passed out from exhaustion! Urrg! I guess tonight is round 2... wish us luck!


Harley wasn’t doing too well yesterday and last night. She’s really swollen and this time it doesn’t seem to be going down any. They’re giving her Lasiks more often now and her potassium was high (which apparently causes bloating) so they stopped supplementing the potassium in her formula. She’s also having seizures more frequently now, too.

When I ask the nurse about the constant bloating she just says her Lasiks was upped and the potassium was stopped. Other than that, I guess they don’t care. When I tell the nurse about her seizures she just says she charted it and will let the doctors know. This is really upsetting us that they aren’t addressing our concerns. Last time Harley was in there they did very well at ‘making us happy’ and addressing our concerns and I don’t understand why this time would by any different. If she’s still swollen by tonight I’m going to ask to talk to the doctor myself and let him/her know how upset we are by all this. Poor baby is so uncomfortable from the swelling and excess seizures she won’t talk to us anymore and is sleeping all the time again. I don’t understand why she was doing so well and now she’s back to where she was before. I guess we’ll just have to wait, probably do more med changes and see.

Saturday, July 25, 2009


Harley is officially off the ketogenic diet and on Pediasure! The neurologists are blaming Harley's inability to make ketones on her Alper's Syndrome, but honestly it doesn't matter. She can have SUGAR again! Now I can sneak her tastes of yogurt, ice cream and other candy again! Yay!

Also, they started her on physical therapy yesterday! She came in and worked with Harley and even came back today! I'm so relieved they actually listened to me! I knew it wasn't like them to just ignore my request, but that's the impression I got everytime I asked.

Harley's swelling has gone down DRASTICALLY. They started giving her Lasiks every 12 hours to help her pee out the excess fluids and wow, what a difference! Her skin isn't as tight anymore and she stopped whining and talking all night: she actually sleeps through the night again! Poor girl was just so uncomfortable she couldn't sleep before.

Another thing accomplished is that they lowered her dosage of Phenobarbitol (seizure med)! She seems more awake without an increase in seizures! We're pretty sure they did this because her levels of Phenobarb in her blood lab were high, not so much at our request, but either way we'll take it!

She's also seen the pediatric GI doctor and all that he said was as long as she doesn't throw up any more blood he's not too concerned. If not then that means it was probably from some bruising and irritation from her GJ tube. If she does (it's highly unlikely since she hasn't since Monday) then they'll go down her throat into her stomach with a camera and look around for hemmorages, ulcers, etc. He's pretty confident he won't have to do that, though.

Harley on the outside is also doing GREAT! She's so much more awake (probably the most awake she's been since before her first stay in the hospital!) and looking at everyone and talking to us! I could've swore I heard her say 'Mama' a few times now (or it's just wishful thinking, but maybe not!) and when we ask her questions sometimes she'll say 'Uh-huh' like she's saying yes! Coincidence? Maybe, but it's sure cute anyways!

Well, that's all for now. Hopefully with the next update I'll be telling you all that Harley's home again!

Thursday, July 23, 2009


On Monday morning, Harley threw up blood clots. Cameron called 911 right after calling me to come home. I raced home and arrived just as the fire truck was pulling up. Harley seemed to be okay other than throwing up blood; she was acting normal and looking around at all the paramedics. It was 10-15cc of mostly clotted blood so they decided to take her to St. Joe’s Hospital (at our request).

In the ER they said she was dehydrated and wanted to start an IV for fluids. They poked her and poked her but couldn’t get one started. They even had the SWAT nurse, supervisor and another nurse from the PICU come to the ER and try to get an IV started with no luck. At this point they gave her fluids through her G tube for lack of anywhere else to give them to her. Then, they had phlebotomists come from the lab to draw blood from Harley (2 of them) and they poked and prodded her even more with no luck, either. The ER doctor then came and tried to do a central line IV in her thighs with no success (he even used ultrasound to make sure he was poking her in the right place). At this point they said they would have to basically put an IV in her knee cap (straight in the bone!) to get her fluids because they’d exhausted all their other sources unless the PICU doctor had any other ideas. He ended up coming down to the ER from the PICU and put a central IV line in her neck, first try. Poor baby didn’t have any pain killers and STILL did GREAT! After that they were able to draw blood so they could do the lab that would tell if she needed to go to the PICU or a normal room; we ended up in a normal room.

Of course since we already knew everyone in the PICU on the 4th floor and on the 5th floor pediatric unit they would send us to the 2nd floor where we know no one. It’s been okay on the 2nd floor, though. WAY more confusing on where to go (we still have to stop and ask directions!), but we can handle that. First night we were in a 3 patient room with another family (that had NO bathroom in it; we had to walk past the nurse’s station down the hall and use a really short toilet for kids) and the second day I asked if we could move next door because 1.) It had a bathroom and 2.) The baby girl that was in there was discharged. Our nurse talked to her supervisor and got us our own room (a 2 patient room, but it’s all ours’!) with our own bathroom & its right by the nurse’s station! Only bad thing is there are no cots on that floor so that night Cameron & I slept uncomfortably on the couch until we woke up in the middle of the night and Harley’s nurse said she brought the chair from our old room over for us. How sweet!

Anyway, back to Harley. She was dehydrated with low levels of potassium and sodium, so she got bags of fluid for each and her levels went up by that night/ next morning, only then she was bloated from so many fluids so rapidly (and it didn’t help not getting up and moving around like when we’re at home- I requested physical therapy, but my requests are still unanswered. We’d do it ourselves, but with the IV in her neck it makes it more difficult.). We saw the neurologists, who were concerned that her special formula for seizures wasn’t producing ketones in her urine like it’s supposed to do. They suggested the geneticist evaluate her for a metabolic disorder, and as of now she doesn’t have one (thank goodness… poor girl has enough issues!). They are now in the process of switching her over to normal formula because her seizures are pretty well under control without her urine testing positive for ketones anyway. The neurologists are also re-evaluating her meds (FINALLY: only took Harley being admitted into the hospital to do so!) and I’m hoping we’ll be out of there in a few days. I work Friday night and Saturday morning to make up hours I’ve missed this week, so I’ll be able to update then, too.

Monday, July 20, 2009


Wow, what an improvement! Harley has been doing GREAT. She's more awake now, is moving her head a little and has even been trying to talk! (She opens her mouth wide and moves her lips and tounge.) She's on a more normal schedule (sleeps at night, wakes up during the day with a few naps) and has had NO seizures or throwing up for 2 or 3 days now! We started giving her meds through her J tube (the extention that goes into her intestines) instead of the G (straight into her stomach) and it seems to have made all the difference! We're still trying to get a hold of the neurologist, but in the meantime we've at least found something that works for us!

Other than that, she seems pretty congested, but at least she can cough it up and out of her chest. Also, she's been doing GREAT with no oxygen during the day (for the most part)! It's just been SOOOO nice to have her more awake and interacting with us! I haven't even thought about getting a picture of her with her eyes open yet, we've been too excited about her being more awake!

Oh, and she has her little attitude back! When she grinds her teeth we put a pacifier in her mouth, right? Well, now she tries to spit it out, which is fine if she stops grinding her teeth, but if not we force it back into her mouth. She used to just accept it, but now she waits till we aren't looking (or she thinks we aren't looking) and spits it right out. It's the cutest thing! She's not grinding her teeth as hard (at least she doesn't make her whole mouth bleed anymore) so we aren't too worried about her spitting them out. That's about all for now!

Monday, July 13, 2009


Okay, time for a weekend update! Harley's been doing pretty good, all things considered. Over the weekend she grinded her teeth so hard it made her poor little mouth bleed! I suctioned all the blood out and forced a pacifier in her mouth, which seems to have corrected the problem. From now on, if she grinds her teeth she gets the paci whether she wants it or not. She seems to not mind for the most part. She doesn't spit them out unless she coughs or throws up and she's perfectly capable of doing so if they bother her.

Harley also got to meet her uncle Guy from Washington State (Cameron's brother) and visit with her Papa from Prescott Valley (Cameron's dad). They enjoyed seeing her and she seemed to enjoy the extra attention she was getting.

Well, that's about it for now. We're just trying to get her out of bed and keep her awake as much as we can. I'll keep everyone updated!

Thursday, July 2, 2009


So yesterday we had 2 appointments: 1 with her PCP/Normal Pediatrician (Dr. West) and another with the surgeon's office that did her muscle biopsy & GJ tube surgery (Dr. Avalia & Dr. Egan). Her appointment with Dr. West was at 11am and the surgeons one was at 2:30pm. Well, let's just say Harley missed her noon meds... and her 2pm meds... and her 4pm meds (which was just a breathing treatment: she's not dependent on them, so that's good). Those include a few seizure meds and some anti-nausea meds (not exactly, but best way to describe them).

Now, you would assume her being on so many meds she would be dependent on them and start having seizures and throwing up more, right? WRONG. (?) It surprised us, too! She did SOOOOO much better OFF the meds than when she's ON them! She didn't throw up ONCE all yesterday, had maybe 1 or 2 seizures (only for a few seconds) and was awake and happy! I talked to her, she'd look at me and make eye contact and she was more affectionate, too (gave me more kisses). She stayed awake for about 4 or 5 hours STRAIGHT and just seemed to be doing all around better!

Here I thought my daughter was getting so bad that she's too weak to stay awake for longer than an hour at a time and I come to find out she's just been knocked out from her meds! My poor Honey Bear! I'm going to call her neurologist and make an appointment for sooner than the one that's already scheduled: I want some of these meds GONE if we can help it (and it's looking like we can...) or at LEAST decrease the dosage.

Harley's been doing pretty good, though. She didn't throw up ALL yesterday and has only thrown up once this morning and it was only a little bit. Yay Harley! I'm sure she's happy, too. Thank you all for your continued support and I'll update again when I get a chance!