Aunt Hys here, standing in for Gracie's Mom, who's been released from the hospital. This comes with great news and busy news.
The great news: Hey! They're out! Who can argue with that??
The busy news: Gracie's mom has racked up some new responsibilities which, before Saturday, were held by the flotilla of nurses who moved in and out of Gracie's hospital room at better than two hour intervals.
As you can imagine, this is a full-time job on a triple shift. The best I can do from far, far away with my oft-mentioned plague-ridden babies is to pinch-blog, so here I am.
Gracie's doing well, by any standard. Her white cell count was 10.1 (well into the normal range! yay!) on Saturday. No neutrophil count was available when I talked to Sarah, but it seemed not to be of too much concern, as the total count was so nice and high. She still goes to the hospital daily for clinic, during which her blood is drawn to test for any need for blood product (whole blood or platelets), and she still receives ERT weekly, at least for the next three weeks. After that, Gracie's blood will be tested to see if she's producing enzyme on her own yet. As her need for blood transfusions diminishes (which it will, as she is better able to produce her own cells), her clinic visits will be less frequent, and she will begin looking toward home (big, BIG yay!).
In the meantime, Gracie's medication schedule is enough to make your head spin. Here's a quick rundown:
Mucomyst: 4x daily (2am, 8am, 2pm 8pm) given by IV infusion over 1 hr. each dose (!) through her central line.
This medication is normally given as a mucus thinning agent for people with pulmonary problems whose mucous secretions are too thick or otherwise abnormal. For this use, it's given as an aerosol through a nebulizer, and is inhaled. It has another indication, specifically for reversing liver toxicity due to acetominophen overdose. For this, it's given orally or intravenously. I don't actually know, but I will speculate that Mucomyst is on Gracie's menu to combat hepatotoxicity the rest of her drug regimen. Anyone with a hard answer to this one, please pipe up!
Another side note on Mucomyst: the preferred way to give it to Hurler patients is in an oral suspension. Unfortunately, it's a sulfa drug and, as promised, smells and tastes nasty like a rotten egg. Gross! So the poor patient, who's so bulldozed by chemotherapy that she can barely swallow drops of water without barfing, is expected to swallow 4 MILLILITERS of rotten egg syrup EVERY SIX HOURS! Gracie said, in her inimtiably effable way, "Hell-to-the-NO!" So she, like over 60% of her cohort, is getting it through the central line.
Um, thank goodness.
Vancomycin: 1x daily IV through her central line, antibiotic for the enterococcus she picked up in the hospital.
All the rest of Gracie's meds are given orally. They are:
Bumex: 3x daily, loop diuretic
The diuretic is to help her get her water balance back on track, after hammering her kidneys with chemotherapy.
Cyclosporine: 2x daily, anti-inflammatory. This is to combat Graft-versus-Host Disease (GVHD) which is a systemic inflammation problem that happens when the new graft attacks the body cells of the patient. So far, Gracie hasn't had any trouble with this one.
MMF (mycophenylate mofatil): 2x daily, another anti-rejection drug, given in conjunction with cyclosporine to suppress immune system activity, or inflammation.
Protonix: 1x daily, proton pump inhibitor. This is a tummy tamer. Usually given to patients with gastroesophageal reflux disease (chronic heartburn), it reduces acidity in the upper GI tract, and makes your tummy feel better. A good thing for someone whose food is bypassing her stomach (she is fed intravenously), but who still suffers from nausea and vomiting.
Fluconazole: 2x daily, antifungal. This prevents the growth of fungus in her central line. Because she receives food , including plenty of fatty substances, through this line, there is always reason to worry about infection here, and the antifungal is precautionary.
Bactim: 2x weekly (Mon., Tues.), antibiotic. Similar to the fluconazole, this is a precautionary drug, as there is a good deal of infection risk associated with TPN (IV feeding).
Ativan: mild sedative, given as needed for the general discomfort you would imagine is associated with all this health care :-)
Zofran: anti-nausea, given as needed to combat the nausea that is commonly associated with chemotherapy and other drug therapies.Interestingly, Gracie is most nauseated in the morning, like many pregnant women, I'm guessing because her sleeping metabolism is slowed, and her drugs are not cleared as as rapidly as when she is awake.
So, if you were wondering why Sarah never has time lately for blogging...
Thanks to everyone for checking in. We love to read all your comments and guestbook notes. Gracie, and all the rest of us, can't tell you enough how much we appreciate all your thoughts, prayers, kind words and every other kind of support you have given. We are one lucky family to be blessed with all of you!
