Behind The Scenes Of "Normal" (i.e. Boring)

Life is busy, and I just can't seem to keep up with the blog. Mostly because I think that life is pretty "boring" these days -- as in, who would want to read about our never-ending construction downstairs, how stinky Jordan's spica cast is, how insane the weather is (83'F one day, freezing & hail the next), how desperately the van needed cleaning out, etc, etc. How about one more video of Jordan munching on a banana through her mesh bag? (Actually, now she eats cantaloupe, marshmallows, biter biscuits, mango, & sweet potato, too, but she really does prefer banana). It's just our plain old "normal" life -- boring.

So, here it is: Speaking of Jordan, she's doing very well these days. If you haven't seen her since last fall you would hardly recognize her! So many things have changed, I don't know how to decide which things are most important, but I'm sure they've all contributed to her gradual "awakening." Blending & feeding her real food each day, adding CoQ10 to her diet, gaining seizure control with medications, fixing her ears & airways, relieving the pain of her dislocated hip, and last but not least, having daily nursing support have all contributed.

Nursing support is not only important to us as a family, but also to Jordan as a person. She now has a significant amount of 1-on-1 work each and every day, something that most 2-year olds would enjoy, but don't need in the way that Jordan needs it. She needs to be hugged & snuggled, and fed with a mesh bag, and talked & read to, and just attended to in much the same way a newborn needs these things -- to learn that she's not alone in the world and that people do respond to her needs. She also needs extra care to learn how to use her mouth, and her vision, and her muscles (including her brain). She has therapies stacked upon therapies to keep her body working & prevent deterioration. With a "normal" newborn skills build up over weeks and months, but Jordan's almost 28 months old and she's just starting to progress out of the newborn stage -- and not by accident I might add. She needs to be guided to the development of a 1-month-old, and the gains she's been making towards that don't come easily. There's just no way for a "normal" family alone, with work, and another kid, and frankly, sleep, to spend the kind of time directly with Jordan that she requires!

Medically speaking, nursing has also helped keep her out of the hospital this winter. With the exception of 8 hours for her tubes & adnoidectomy in February, and 5 days for her hip surgery in March, even with a few nasty colds & multiple ear infections we were able to keep her off the floor at Emanuel all through the winter. In fact, she hasn't been admitted for illness since October -- it's truly amazing! Another testament to the strength we're all building by having nursing care to help with her.

But like I've said before, things with Jordan still do not come easily. For example, I'm constantly researching & learning about new syndromes and diseases, hoping that one day we'll find the one that fits (although I'm pretty sure we won't). We're at the point now that when we see her doctors for 6-month meetings, I'm the one suggesting possible syndromes to test for, and they're more than happy to do what I say. I guess that just goes to show how stumped they really are at diagnosing her. Currently we're waiting to get the spica cast off to get a lumbar puncture for Folate Deficiency and Non-Ketotic Hyperglycinemia. No, she doesn't present classically with either of these, but hell, every diagnostic test she's had in the last 28 months has come back "normal" -- so clearly she doesn't present classically for anything. And trust me, there have been ALOT of tests. Tens of thousands of dollars worth of tests. All normal. Her most recent normals (within the last 3 weeks) were for Tetrahydropbiopterin deficiency, and Carbohydrate Deficient Glycoprotein.

It's great that doctors are listening to me and even letting me take the lead on things, but lets face it, as a geologist by training, it takes an inordinate amount of research for me to come up with things for the metabolicists, geneticists, and neurologists to test for. I mean, this is their career, they shouldn't be counting on my hit-or-miss internet research alone! It's classic "thinking inside the box!" They just need to get themselves out of those boxes and contribute, too! It takes a village (of doctors & parents), ya know! The great news is that I've made some truly wonderful friends both locally & all over the world via internet groups, and I really feel like I would be a lost sheep in this world without them. Believe me, parents who've "been there, done that" are invaluable when you're dealing with kids, no matter what the topic, but especially with a mystery child like Jordan.

Another disease that comes up frequently with Jordan is a mitochondrial disease. Unfortunately, to diagnose a mitochondrial disease definitively, a fresh muscle biopsy must be performed*. That's full surgery to remove part of the muscle on one of her thighs. And to top it off, only 3 places in the US do fresh muscle biopsies (with any reputable answers, as least): Atlanta, Cleveland, and now recently, Seattle. Based on my severe PTSD from our travels to Detroit in October 2006, I haven't been able to bring myself to fly across the country with her again in search of a diagnosis. But now that Seattle is offering the service, I am more than willing to get her seen in the neurometabolic clinic at Children's Hospital there. But getting in with a guru like the one we're targeting is no small feat -- he must be convinced that it's worth his time to see her. Luckily our wonderful pediatrician here in Portland has also put me in the driver's seat and just sent the referral on Wednesday. Hopefully we'll get in soon to see him & follow that scary, but necessary path.

And speaking of mitochondrial disease, and one of the reasons I've started following the path to getting her to a guru for a fresh muscle biopsy, is that she seems to have "awakened" with the addition of CoQ10. This is a typical response with mito kids, and the next logical step is to get her onto a "mito cocktail," which is a special combination of l-carnitine, CoQ10, and many B vitamins. This cocktail is known in many mito kids to improve the function of mitochondria, which are the principal energy source of the cell. So, with anectodal evidence of the CoQ10 improvement in hand, the metabolicist was willing to write a prescription for this special "medication" based on her special needs. Now the tricky part -- convincing a pharmacist to compound it for her. It's really pretty unreasonable & unrealistic to think that I need to be chopping up & measuring vitamins 3 times per day, but that's all the "help" the local compounding pharmacist will offer -- to sell me the components. So I posed the question to my on-line support groups, and found a pharmacist in Georgia who does about 1 mito cocktail per week for people all over the US. He even seems to know how to get insurance to pay for it (grey area), and he's very excited about helping us out. Strange that I have to contact someone in Georgia just to get a vitamin & supplement mixture, but since I can't find anyone locally, I might as well have the best. Overnight mail from Georgia is the same as overnight mail from Seattle as far as I'm concerned.

On the home front the two big events on the horizon are quickly approaching. A therapist from the Washington State School for the Blind is coming on Monday to evaluate Jordan and see what kind of things we can do to encourage her to use her eyes & help her brain process the information. Cortical vision impairment is fundamentally different from other vision problems, because the problem isn't caused by a physical or mechanical problem with the eyes, but rather by a miscommunication (or no communication) between between the eyes and the brain. So treatment & therapies are totally different. We're just so thrilled that she's opening and using her eyes, we want to do everything possible to improve that skill.

And the other big news: If all looks well on the x-ray, the spica cast will come off for good on 29 April. That'll be just 1 day short of 7 weeks in that stinky thing. It doesn't even fit her anymore -- I think she was going through a growth spurt during the time the cast went on, but as long as she hasn't been moving her hip in it, which we don't think she has, she should be healed and ready to move onto the next hurdle -- 1 to 3 months in a brace. We won't be sure what brace she will have until the cast comes off and the doctors evaluate her, but I've looked at some of them on-line and they look like real "mickey-mouse" contraptions. I'm afraid that we'll be wanting the cast back once we get her into one of those things. Ahhhh, the fun never ends, I swear.

Finally, there's always an insurance battle raging. Currently I'm entwined in a battle-of-the-century, again, with the insurance company, this time over an automated Flexible Spending Account error that is being perpetuated by a computer that cannot be controlled by humans. We should all be scared. That project alone can easily eat up 4+ hours per week on the phone with various "there's nothing I can do" employees. I have every confidence that I will not have this straightened out before July, but will let you all know if I am proved wrong on that.

So, what about Avery in all of this you wonder. Avery is truly an amazing child, and we couldn't be prouder of her on so many levels! She is incredibly helpful, caring, and protective of Jordan. She's starting to understand that something else is going on, and the general comment "Jordan can't do that, she's just a baby" isn't true anymore. She realizes now that even when she was a baby she didn't have a g-tube for food, or a wheelchair, but she doesn't know anything different. She frequently explains to people that Jordan is "delicate," a term nana Noma taught her almost 2 years ago. That's a pretty good way to put it.

Avery also is possibly the busiest 4-year-old in the world right now. She is bursting with energy, and our only salvation is getting her outside into the yard, no matter how cold it is. Otherwise she just hops from one end of the house to the other -- both feet, right foot, and now she's working on left-foot only hopping, too. At pre-school they did a Hop-A-Thon for Muscular Dystrophy on Friday, and she's hooked! Ughhh, why can't I have an Avery-energy transplant?

In addition to hopping everywhere, she is taking swimming lessons (still) on Tuesday & Thursday nights, and finally is swimming on her own without the teacher holding on (okay, it's just a few feet at a time, but it's independent swimming!). It's a miracle. Just one short year ago when we started twice-a-week swim lessons, and we could barely wash her hair in the bathtub without a complete meltdown. Now, she's a regular Mark Spitz.

As if that isn't enough, she's doing gymnastics on Friday nights, and she's really good! She loves it, and her gymnastics club, Naydenov, is pretty amazing. She & nana Noma are already in full gardening swing for the year, so she goes to nana's house a couple of afternoons a week to help dig worms, haul dirt around in nana's new 4-wheel wheelbarrow, plant seeds (lots of seeds, you can never plant too many seeds when you're 4), & fertilize with gusto -- mostly when nana isn't looking. No wonder nana's tomatoes plants are 8 feet tall each summer.

Preschool in the mornings serves as a great social outlet, and she's developed many dear friends there, but academically it's not challenging at all for her**, so I'm considering some alternatives to add to her schedule. I'm very interested in getting her involved with Junior Kumon, but I'm not completely sold on the idea considering the steep pricetag that comes along with 40 minutes of instruction per week. I certainly don't want to hold her back, but I also don't want to push her too hard so that she burns out by, like, 3rd grade or something. She certainly has an interest in learning, and seems to be well ahead of most of her peers. Okay, yes, I'm bragging a bit here, but hey, I'm the mom so I'm allowed every-so-often, right?!

As for me, just keeping this all going is a full time job. In addition, I have been able to do some consulting work on the side, especially since our nursing coverage has improved to 7 days a week. It's great for me to do some adult, non-medical "work" -- I appreciate the change of pace.

As for hobbies, I have a huge stack of books piling up on my nightstand, an almost finished sweater in my knitting bag, and 3 Netflix sitting on top of the DVD player, unopened. I'd love to read & knit & watch movies & TV a bit more, but I just can't seem to fit it all in.

But mostly I've lost my "umph" with my camera. I just haven't been in the mood to take crappy pictures, so I haven't been taking many pictures at all. I'm quite sad about this, but I'm tired of having to use my flash indoors because the weather is so bleak. And I'm tired of not being able to position Jordan for a good shot. And I'm tired of Avery not being "in the mood for pictures" as she normally avers when I grab the camera. Of course, no one ever wants to help me take nicer pictures, because 1. Don't you have enough pictures of those girls already? & 2. Don't you have enough pictures of those girl already? Oh, and 3. They're hard to take pictures of. Frankly, I'm ready to give up on kids & people and just focus on animals -- if nothing else there's no attitude and they're always natural. I think in my next life I'll be an animal photographer!

That said, I still have some great photos of nana Noma's Easter Eggstravaganza that I haven't posted, and a few good ones from a little photoshoot Kelly & I did with the blossoming trees down on the waterfront in Portland. Too bad the 4-year-olds aren't nearly as excited about "modeling" for us (or even showing their faces) as the mommies are about photographing them. Although I do admit, Kate is much more camera-friendly than her tomboy best friend Avery.

After a novel like this I'm sure you'll be fascinated to know that I'm off to finish up the laundry and help Mr. Enthusiastic-cabinet-hanger by holding cabinets to be installed on the wall. I'm not sure I'm qualified for that! I guess that's why you have to trust your spouse. . . . Is there anything we're not dumb enough to not do ourselves -- from practicing medicine to home remodeling? I don't think so!

So there you have it, the boring, "normal" life of the Rowes in the Rose City, as of April 2008!


*Even with the best of the best, the experts in the field suspect that ~25% of people with mitochondrial diseases will have a negative muscle biopsy. Talk about a hard thing to diagnose!

**She already knew the entire pre-school curriculum when she started -- a full year of letters, shapes, colors, and numbers. Oh well, school in North America is NOT ONLY about academics, as we all know, so now at least she has social skills to go along with being a student.