Harlie Update and fantastic news!

It has been so long since my last post.  I am going to write some updates, and post them as I finish.  They will likely be in random order, based on what I feel like writing about first.

Harlie Update

She's been on home bound schooling since the week before Thanksgiving.  It has definitely been a nice change for us.  But, at some point, I am going to have to send her back.  I was so hoping to send her back with NO oxygen.  But, that isn't to be right now.  When I ordered her monthly supplies last week, I had to make sure I replaced all the empty oxygen tanks so we would be ready for that again.  Ugh.

As far as when to send her back... as things stand now, we are supposed to see a pulmonologist at Children's National in DC.  We are waiting on that date now, but am told it should be within the next two weeks.  Her docs there (cardiologist, ENT, pulmonologist and her social worker) have been emailing back and forth to come up with a plan.  Once we meet with this pulmonologist (who we have not met yet) he will go over some things with me and determine if he thinks a sleep study is warranted.

To recap why they were talking about doing a sleep study.... One cause of higher pressures in the Fontan (her heart circulation, sort of) is a higher level of carbon dioxide (CO2) in the blood.  A cause of higher CO2 can be sleep apnea.  They would like to rule out sleep apnea before they start treating her with medication.  But, given that she already has a trach, I am thinking obstructive sleep apnea isn't likely (since the trach prevents anything from obstructing her airway).  Since she sleeps with a pulse ox (which indicates the patient's heart rate and the amount of oxygen in the blood) I am doubting she stops breathing at night, which would indicate central sleep apnea.  I would have thought by now that we would have seen her desat while sleeping.  But, who knows?

Anyway, I guess her ENT and the pulm must have talked about this because now we are to meet with him and then he will decide if she needs a sleep study.  I'm grateful they really want to think this through before putting her (and me) through a sleep study.  Given Harlie's opposition and great dislike to anything sticky, the probes that they put in their hair (or on their head) would be torture.  But, if it's warranted, then we will get through it.  And they have spoken with the director of the sleep lab and will get her in asap if that's what they decide.

So, now I'm thinking of keeping her home until we have this appointment, so he can see her at her baseline best.

My gut tells me that in the long run, we won't get many answers.  This might just have to be something we accept as her new normal and we try meds, and wait and see if they work.  I am not looking forward to the wait and see game.  So far, she had a problem (or many of them), we figured out what was a priority, and we "fixed" it, or made it better or in some cases tried to make it better.

This is something we can't just fix.  Surgery isn't an option.  So, this is new territory for us.  And now I can see the effects of this pressure change - in her oxygen requirement and continuing clubbing fingers and toes.  Basically, the clubbing is due to prolonged oxygen deprivation aka low oxygen saturation her whole life.  Here are her toes...

I know it could be worse but it still kills me just the same.  I can see her heart defects when I see her fingers and toes and it makes my stomach hurt.  It is a constant reminder of what battle is going on inside her body.  And no one wants to see that.

Soon after her heart cath in mid-December we tried weaning her from the oxygen during the day.  She did okay, I guess for a few days.  She seemed to be in the low 80s and then in the high 70s.  A typical, healthy person is near 100.  One day I noticed that I couldn't get all her food in her that day.  It just seemed that every time I went to tube her a feeding, she had a lot of residuals (formula was still in her stomach from the last feeding) and complained of feeling full all day.  You can't force it in, so she didn't get all four cans.  Then Brandy told me the same thing after she was with her all day.  This happened for several days in a row.  This could be a sign of poor perfusion to her stomach due to the low oxygen saturation levels.  Basically the body is sending oxygen to the most important organs.  Once that happened, I put her back on oxygen and haven't taken her off again.  It's not worth that.

So, what if we can't stop the pressures from getting higher?  What if meds don't work?  What then?  Waiting for those answers aren't going to be easy.  I know this has happened to other kids and the outcome wasn't good.  It's just hard, living with this kind of worry.  I am trying very hard to focus on the positive.  She's happy and seemingly unaware of anything amiss inside her body.  She willingly wears the oxygen and doesn't complain.  We give her little breaks - like to take a bath.  I think she could probably tolerate a quick trip somewhere without it (like to Target or something).  But, at this point, the worry I have about something happening (car accident, me running into a friend and talking for an extra 15 minutes, a sudden change with her, etc.) makes it not worth it.  I would rather her have it when she needs it.

She has shown a crazy sudden interest in food.  She even asked to eat once.  Tom gave her some yogurt and she took a few bites all on her own - holding the spoon herself, with no reward other than a "Good job, Harlie!"

She wants to watch Tom cook and she wants to stir it, or put the salt and pepper on it.  We let her and Tom tells her what everything is as he is making dinner.  "This is celery, Harlie."  She's also been tasting stuff with her finger - like syrup, butter, salt, sugar, etc.  And we've been telling her if it tastes sweet or salty (since those are things she's never experienced and likely didn't even know the language).

So, that's where we are with her.  Plugging along one day at a time.  Focusing on the haves instead of the have nots.

And to end with a FABULOUS piece of news... My dear friend Lynda (founder and organizer of We Heart Harlie) applied to be a beneficiary of the Deep Run High School Marathon Dance.  Every year the high school students organize this event and raise money for 12 local organizations.  Last year they raised just under $245,000!  Isn't that amazing?  Well, right before Harlie's heart cath last month they had an event at the bowling alley with all the applicants.  Groups of students on the committee went around and spoke with each applicant's group to get to know them better.  Lynda and I talked for over two straight hours.  Harlie was there and thank goodness I had Brandy with us, because I could never have been able to speak without her there.  Anyway, it was emotionally exhausting talking about Harlie and the past seven years to so many people in one night!

Well, we just found out that We Heart Harlie was selected to be a recipient this year!  Can you believe it?  We are more thrilled, blessed, lucky, etc. than words could say!  To see We Heart Harlie among the other organizations was surreal.  I will never be able to thank everyone enough.  Never.

Too grateful for words.

Much love,
Christy xo