Sad

Three days ago another member of the mito community died. He was an adorable little boy a year younger than Eve. This news has rocked me. It seemed to come out of the blue (in my perspective). I think I have been living in world of near denial. But I think that's how I manage to survive. If I constantly thought about the awful nature of this disease, I just couldn't function. So as a matter of survival, I focus on the day to day stuff and try not to think about the "what ifs" and the reality of what will be. But then something like this happens and it's like a bucket of cold water in the face. I think what really got me was that this little boy was able to walk and talk. I always figure Eve is so severely affected because she is nonverbal and non-ambulatory, but I have to count my blessings and realize that she is relatively healthy. In fact she came down with a cold a week ago, and has already managed to shake it. She hasn't been in the hospital in a year and a half. But that won't always be the case. I *ache* for the family of this little boy. And I am so scared of what will happen when I am in their shoes.

sleuthing

We got Eve a Medic Alert bracelet. I've always been kind of paranoid that I'd get into a car accident while driving with her and be unable to explain to the paramedics her condition. So, her bracelet is on her 24/7 and reads: mitochondrial disease; non verbal; allerg to peanuts.
When the hospital calls the number, they can get access to more information including her current meds/dosage. We also chose the basic design, so it would be obvious in an emergency. Bonus: the bracelet comes in a pink decal!


I did a little deducing and may have figured out the source of Eve's UTI's. I've been using a diaper cream recommended by the pediatrician that has 3 ingredients I mix myself in a little Tupperware tub. Well, I had noticed (and ignored) a connection between using the diaper cream and then smelling funky pee a day or so later. I brushed it off because why would a cream make her pee smell? Her pee started smelling again and a lightbulb the size of the sun turned on in my brain. What if the tub had some contamination!! What if I changed a dirty diaper, stuck a finger in to get a glob of cream, and inadvertently contaminated the tub? So I called the pediatric surgeon to see if he still wanted to do the VCUG. He said that we should cancel it, but if she gets smelly pee/UTI again that we should schedule it again (which would indicate that the frequent UTI's weren't due to the cream, but instead might be kidney reflux). In the meantime, Eve's pediatrician put her on a course of antibiotics. So we are starting with a clean slate. I've tossed the tub of cream and will use straight Desitin from a tube if necessary.

Eve also had her 5 year well visit. She is 34.5 lbs and 39 inches tall. All is well. School is going well for both Eve and I. I have 5 classes this semester (!), so I am a busy girl. Eve has settled in quite nicely (or so it seems) in her class. She has stayed back in Pre-K this year. Her teacher is a Special Ed teacher and we thought she would really benefit from another year in that classroom, reinforcing concepts she learned last year. It's sad that most of her class from last year are in Kindergarten this year, but she will make new friends and visit with the old ones in the hallway. Not to mention, this is such a small community that we *literally* cannot go anywhere without someone saying, "There's Evie!" Usually it's kids from school (that I don't always know). I think Eve is pretty recognizable (thanks in large part to her wheelchair).

Hope you are having a nice weekend. Jim is camping with Paul, so it's just the girls here. Josie is in charge of security. She already saved me from a large spider. Right now she's dreaming of being tough.

pediatric surgeon visit

So, we went to Springfield (again) today to see the pediatric surgeon (who functions as a urologist). Eve was doing fine in the car, and about 15 minutes from the place I heard her wimpering. I turned around and the lower half of her face was covered in blood. The best I can tell is that in some freak coincidence aiming, she managed to stick her finger up her nose and get herself good. I pulled over and was able to clean her up. But, boy was it scary.

The doctor's appointment went well. I liked him a lot. I explained that Eve had kidney reflux that had supposedly reversed itself, but there had been several occurrences of possible UTI's over the last 9 months (some of which did not show up in urinalysis). I explained about most recent event (smelly pee on vacation), and how it went away with the medicine. He told me that he has had some patients that have had kidney reflux, it reversed, and then after a few UTI's and a new scan it turned out they had it again. So, he is going to schedule a VCUG (an xray test to diagnose kidney reflux) at Baystate (in Springfield) and then we will follow up with him. If she does have reflux again, we have two choices: 1. Prophylactic dose of antibiotics (like in GA) or 2. Surgery that does not involve an incision, but rather goes up the urethra and deposits a substance that prevents urine from going back up into the kidneys (reflux). I asked him if he thought I was being rash or paranoid over a couple of instances of stinky pee, and he said if he thought that, he wouldn't be scheduling the VCUG.

Finally, I told him about the bloody nose and I asked if he thought I should call the pediatrician when I got home. He said no and agreed it was probably freak-aiming (unless it happens again).

neuro today

Today we went to Eve's neurology check-up. She's really lost some tone in her trunk and he noticed she is having more trouble keeping her head up. He also thinks her eyelids are a little more droopy. She is definitely cognitively behind her peers as well. This is something I know, but it is still hard to hear. I also know this is the nature of the beast. She's not going to get better. She's not going to stay the same (always). It's still hard when you have to face it.
I asked the dr. if there was anything we could do about her excessive drooling. He prescribed a medicine that we will try. There are some side effects, but we will start on a low dose and see how it goes. She's been choking/gagging on her saliva if it pools in the back of her mouth and she just soaks her shirts and bibs. Follow-up is in 6 months as usual.

On to more fun stuff. Last week we had a birthday party for two of Eve's stuffed animals. Eve just loves a birthday. She got a new haircut. A little bob with 50's style bangs. Cute.

Here Eve is fooling around with stickers.


School starts next week for both Eve and me. I'm not sure who is more excited.