One tentative step into the unknown...

Cady started her Growth Hormone (GH) injections two weeks ago.. she gets a shot six nights a week, with Wednesdays off. So far she's had 12 of them. Since my last post, she's gained quite a perspective on receiving them.. she no longer cries, not even a whimper. She doesn't flinch, and will even remind you when it's time to give it. (Mom! It's 7:30!) Not only that, but she has given herself two of the injections. I want her to be independent with her care, she knows how to tube feed herself, and which meals to eat at what times, this is no different. With some verbal coaching, we've allowed her to perform the process, beginning to end. She does wonderfully. Cady has always just been the sort of person that does what you expect her to do. If you expect her to learn to walk, she will. If you expect her to learn to tube feed herself, she does. When we expected her to learn to give herself these injections, she didn't protest or complain. She simply did it. It's our way of life, it never dawns on her there is any other possibility.

She did, however, give me a big scare on the night of her birthday. We had been partying hearty, it was her Sweet 16, and we rented bouncy castles and blow up obstacle courses for the kids to enjoy.. And enjoy they did! For like four solid hours. For a child with reduced endurance, that's like going all day long. She was hot, sweaty, and behind on dinner by an hour when we finally got home. I quickly went to the fridge, administered the GH shot and set her dinner down in front of her. She took one sip of the smoothie I'd prepared, and ran off to the bathroom to vomit. When I went to the bathroom to see what was going on, she appeared to be having a seizure. She was clutching the toilet and bouncing one shoulder off the wall next to the toilet. I grabbed  her, wiped her face (she had tears and snot running down her face from the vomiting), and asked her what was wrong.. "I don't know.", she replied. I asked her how she felt; she said, "Dizzy."
Ahhh.. so I checked the website devoted to info on GH and found a statement about low blood sugar, but nothing on seizures. Well, now it started to make sense.. Hot, playing, sweaty, dizzy, late for dinner.. maybe low blood sugar. I tested her with a fingerstick and her sugar was 178.  WHAT??!!

Ok, high blood sugar. So, I grabbed the pamphlet insert that came with the GH pen. No info on low blood sugar mentioned, but detailed at length the probability of high blood sugars.. at least for the initial adjustment period. After that, blood sugars generally stay under 100 and HgbA1C is unaffected. Good to know. Now I just have to figure out how to stabilize her metabolism and not let the hyperglycemia spiral into ketoacidosis and the rest of the cascade that bodes so ominously for her. Oh, brother.

Cady's condition is a tenuous one. I wasn't kidding or exaggerating with the 'tightrope' analogy. I know more about metabolism, anabolism and catabolism than anyone should ever have to know. I have an unnatural and fairly comprehensive understanding of biochemistry (meaning I have never actually *studied* it, but am able to understand it and apply it anyway). And I know more about Cady's  metabolism than anyone on the planet. Period. It all rests on me. If I don't figure something out, it does not end well for her. (MacGuyver ain't got nuttin' on me, guys.)

So, I pull out my handy dandy diet tracker (I'm gonna put a plug in for Myfitnesspal.com here.. I used it to lose almost 50 pounds and keep it off.. it's wonderful!) to analyze Cady's diet and see if I could adjust it somehow to compensate for the GH causing the high blood sugars. Right away I see the flaws, and years of training in muscle building, nutrition and nursing have given me the insight on how to fix it (I hope... At least, how it works on normal people..)

So, I make the needed adjustments, and implement them. And right away, she straightens up, has more energy, no nausea, and.. HERE'S THE KICKER:  she wants to eat all the time! This is HUGE, people! She is a life long anorexic child with a feeding tube! And she WANTS to eat! I am so blown away by the immediate and gradiose results that I also fear it is an anomoly.. not to be trusted, and will probably not last..

But, here we are, at least five days in to the new diet, and she's still trucking right along! If this were something of a fluke and she wasn't going to handle it, she'd have decompensated by now.

I have nothing but thankfulness in my heart that she is doing so well with these changes.. There have been opposite results in the past, I very nearly lost her on other occasions. It's a scary dance.

And while I hear praises all around, aimed at me, over Cady's progress and stability, I don't feel they are deserved.  I've had mother after mother tell me, "You do so wonderfully with her, I could never figure out that complicated mess and keep her alive. I wouldn't be able to understand it."

The reason I don't deserve any of this praise is simple to me: It is a very complicated business. Metabolism is, for most people, an enigma. I have an in depth understanding of human metabolism, an intricate understanding of human biochemistry, and a detailed knowledge of the breakdown in Cady's presentation of Propionic Acidemia. And the only formal training I've ever had was an entry-level college chemistry class. It's not just UNnatural.. it's SUPERnatural. I am simply a vessel of this knowledge. I did not study to attain it, it was not through my own works that I received it (it wouldn't have done much good to go that route, there never was any time to do those things. I had to know  it, and I had to know it NOW.) And so, the product of this knowledge (Cady's good health) is not a product of my own works either.  To brag about this gift that God has given me so freely would be like bragging about being tall. Or blue eyed.  I didn't do anything special to attain it.. it's just the way God created me. God deserves the praise for this miracle child, not I. He has sustained her and kept her all the days of her life. He has snatched her from death when I was powerless to do anything but weep. He has allowed me to be a tool in her building, and that is something I count as a great blessing.  Oh, what a great blessing.

In a year full of changes, there's always room for a new chapter..

I am either eternally optimistic or I have the memory of  a gnat. I had fully intended on blogging here every day when I first set this up.. You'd think after 16 years of living this life, I'd get a handle on how busy it is and gain some perspective.. But I continually underestimate how little time there is in any given day. Sheesh. Oh, well, as many will tell you, it's my habitual way of NOT seeing the in-the-box limitations that has brought my beautiful Cadence this far.. So, while I intend on updating everyone frequently, let me make a caveat and say in reality, it'll probably be hit and miss..

And today is a hit.  Since my last post, things have been speeding along at a blur.. (Cue up Tesla's song "Changes")..

At Cady's last genetics visit, we discussed exploring her small stature.. Kids with PA tend to be small, and universally look younger than their stated age. At 15 1/2, Cady looks to be about 8 years old. I didn't think there was anything sinister about that, PA affects energy on a cellular level, and if you don't have the energy, you just don't grow.
At any rate, a bone-age study was ordered and as it turns out, at 15 yrs 6 months, Cady's bones actually revealed only a 12 year old. Wow! She has managed to gain four years of childhood, and because of that, her growth plates have not fused yet. Still, I didn't think much of it.. Further testing revealed she is not in or approaching puberty, but hormone levels are normal for a child. Next, we tested Growth Hormone. There was a four hour test, in which we mostly laid around the IV infusion room and watched t.v.  Cady was given an infusion of Arginine and a Clonidine tablet to antagonize a GH response. Blood was drawn every half hour for four hours, resulting in 9 tubes of blood. I was certain, like the hormone levels before, that these would be normal as well.

Well, you can imagine my surprise, when a few weeks later, I received the results and they revealed deficiency. Things actually moved pretty quickly after that, we needed an MRI to prove this was not caused by a structural anomaly, and fortunately, WVU had an MRI of Cady's brain that was taken during a recent coma she'd endured. It was determined that she had a structurally normal pituitary, and we could proceed with GH injections.

Insurance companies are notorious for fighting GH treatment.. it's expensive and long-term. They really don't seem to care that it's important. That fight results in unnecessary delay, and we were up against a clock here.. According to Cady's bone age study, her bone age represented a 93% closure, which meant we have a 7% time window to get her to grow before her growth plates fuse. Fortunately, the pharmaceutical company that makes the GH that Cady is a candidate for has a whole section of experts that set up a system of early delivery and fight the insurance company for you, so that the child receives the appropriate treatment without delay.

We received our GH shipment in the mail on Tuesday and our home health teaching on Thursday, at which time, Cady received her first injection.

Because of all that Cady has been through with this disease, and the many unpleasant treatments she's endured because of it, she is very fearful of anything smacking of a medical treatment.. Put on some gloves, and she cringes. Uncap a needle, she melts into terrified sobs. It's heartbreaking.

Cady's GH is encased in a flex pen, like people who take insulin are using nowadays. She has an uncle that is diabetic, and she enjoys giving him his shot after dinner when the opportunity arises. At least she was familiar with the procedure, since it's the same as her uncle uses. We dialed up her dose, and gave her the injection. She cried and was nearly inconsolable, but as also true to her nature, she was very obedient and stayed perfectly still during the procedure. She understands that the treatment is necessary,and if she moves, we will likely have to repeat the injection, so she is able to keep still, which makes it all the more heartbreaking to watch.

Gladly, the crying stopped there. The following night when her injection was due, she took it like a champ. The injection itself really is painless, the needle so small it's like a hair. She just needed to get used to the idea of having it done every day for the fear to wear off. Thankfully it only took one time for her to determine it wasn't so bad after all.

Now we begin this new chapter. I'm not the type that fears change, I actually embrace it. It's a new territory, new things to explore, new experiences to have. I look forward now to her growth, something we've never known before.  At four years old, Cady was still wearing 18 month sized clothes.. she could wear the same clothes for years and years, that's how subtle her growth has been. Now, at 16, she's still wearing the same child's size 12 in clothes and 13 in shoes that she's been wearing for at least half a decade.  I am excited at the prospect of being able to complain about how often I have to buy her new clothes.. I can't wait to take her shopping so frequently that I may have to get a second job at Justice (her favorite store) just to get the discount.. Our (new) future is laid out in front of us undetermined.. and I'm very excited about it.