Gracie update: Within 48 hours of starting Cipro, she is definitely resembling herself again. However, as much as Cipro knocks out bugs, it also knocks out her gut and gives her a horrible diaper rash... the side effects are brutal for her and, thus, we only give it as a last resort. Unfortunately, it's nearly the only antibiotic that works on her bugs. She had a good day at school, but is not tolerating her feedings well at all and is still pretty weak.Two weeks ago, Gracie's Endocrinologist called to discuss the lab results from the day of her heart cath.
The good news - - unlike they initially thought the day they drew her blood, they
did, in fact, draw enough to run all labs the Endo ordered. So, no trip to the lab this month. Good.
Other good news - - Gracie's Vitamin D and Calcium levels were normal. I was floored. I thought for sure, given the difficulty we have getting nutrition in her, she'd be deficient in both areas. She's not. That's good.
Now... for the rest of the news - - Gracie's growth factors are both low. They always have been, but not in the "we need to treat" range, rather in the "they are low and we'll monitor every two years" range. One is low, but not low enough to justify treatment on its own. The other growth factor is very low... normal is 1.5... Gracie's is 0.7.
So, what does this mean? For the moment, it means we wait. No treatment action will be taken at this point until we spend the next several months getting all of her specialists to weigh in on the pros and cons from their perspective. Then we'll put everything together, including our own opinions along with the advice and wisdom of parents who've already walked this road, and try and make the best decision for Gracie.
At this point, what we
do know is this:
- She is clearly very tiny... which we've known for a long time,
obviously. (She's between 37.5"-38.5" depending on how stretched and straight she is and she's 25 pounds. Despite every effort, we cannot keep weight on her. Her heaviest weight was at age 4 and she was 30.5 pounds. Her highest weight in the last 16 months is 26.5 pounds.
However, she is incredibly small regardless of her nutritional status. I've kept an obsessively accurate log of her caloric intake and, in theory, she should still be bigger in weight and height.)
Her doctor (whom I like very much) kindly and gently made a point to tell me that the decision is ultimately ours as her caregivers. She wants us to consider, first and foremost, how we would handle Gracie getting bigger as we continue to get older. Obviously, our dream is that Gracie will progress with her ability to stand, walk and transition independently. We won't give up believing that she can do it. Likewise, we won't give up believing in her ability to make great strides and live a more independent life someday. As time moves further and further along though, we also have to consider much more seriously the fact that these things might not happen for a really, really long time... or perhaps not at all. It doesn't mean that we aren't hopeful for our daughter; our dream is for her to reach her fullest potential - - whatever that may be. As her parents, we give her every tool to which we have access (and fight for access to those tools we don't have) and we don't allow a definitive line to be drawn in the sand about anything regarding her potential. That being said, we are keenly aware of what reality will look like if those things don't happen.
This piece of the "do
we want her bigger?" decision is easy for us. We are not considering ourselves... we will figure out how to manage if she gets too big for us to easily care for as we age. If she's smaller, then it's obviously much easier for us to carry/transition/help her with all mobility tasks. But... we don't want to keep her small for our sake when there is still hope that she'll be independently mobile. There is plenty of equipment to help us with those things and if we need to hire help someday when we're older, we will. And, in all reality, even though she has a lot of very tall family, there are some shorter women, including me... I'm 5'4" (according to my driver's license!) so Gracie wasn't necessarily destined to be huge... and likely wouldn't have tremendous growth with growth hormone treatment.
- Typically, with Gracie's numbers, an MRI of the brain would be ordered to get a good look at her brain, specifically the pituitary gland. While the labs are very informative, more information about the brain would give clarity as to exactly how deficient she truly is. Gracie's last MRI was done in August 2004. There were some abnormalities noted about the pituitary gland, but her doctor would prefer to get another MRI done now that she's nearly seven. Unfortunately, because of her pacemaker, she cannot have an MRI. So, we base it on the labs, her size and the MRI from 2004.
- With the Orthopedist, we need to discuss the fact that Growth Hormone is contraindicated for patients with scoliosis (because with more rapid growth can come faster and more severe curvature). He might not consider the scoliosis piece to be too much of a concern as Gracie is already slated to have spine surgery someday... when she is
bigger, ironically. Perhaps his biggest concern about her spine will be how much/how fast the curve deteriorates rather than
if it deteriorates.
There is also a potential side effect (though not common) for hip issues. Since her Orthopedist is already concerned about her potential for hip growth plate separation (thus the full-body brace that's been casted... that experience is another post in and of itself), he might be less inclined to support GH treatment for that reason alone.
- We need to discuss with the Cardiologist, too. She will,
when she is bigger, need an additional open heart surgery to replace her mitral valve. It is our assumption that her Cardiologist would be in favor of this treatment for that reason. Obviously, with growth, she'd be more closely monitored to be certain that her heart can handle the work for her increasing body size. (But, really, we are not talking about rapid growth. If we treat, we'd start incredibly slow and monitor her very closely with all of her specialists.)
- With GI, we need to discuss what the risks would be if we do treat with GH and yet continue to have a difficult time increasing her tolerance for caloric intake. How would she be affected? Would we definitely need to go the route of TPN (IV nutrition) and bypass her digestive system entirely? This is something we continually debate for several reasons... one of which is the big concern about her psychological tolerance of being continuously attached to an IV... she most certainly would
not tolerate it.
- Another possible (though, again, not common) side effect is for increased brain pressure and headaches... sadly, all too familiar territory for Gracie.
That's "it," in a nutshell. We ask, we listen, we think... and at some point in the next handful of months, we make a decision.
This time, last year, Gracie lost her first tooth. It wasn't much later that she lost the one right next to it. This week, she lost two more... for a total of four. Her bottom two permanent teeth are growing in (in opposite directions!) and now she's toothless on the top! As far as grins go, she couldn't look more like an almost seven year-old! Tooth fairy is scheduled to arrive and collect both teeth sometime in the wee hours tonight!
