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Not your typical Thursday…

December 31, 2010


We are currently sitting in day medicine watching blood drip ever so slowly into our baby girl.  Dr. R  came over to discuss the details of the day, and reminded me again that transfusion is really our only option for alleviating Lucy’s cardiac symptoms.  On Christmas Eve we received a call from Lucy’s cardiologist.  The results from Lucy’s Holtor monitor revealed what we suspected.  Lucy is tachycardic (high heart rate), and her tachycardia increases even more with changes in position (orthostatic).  Lucy’s heart rate issues are as a result of her autonomic dysfunction, which is affecting her sinus node.  They have diagnosed Lucy with sinus tachycardia, and postural orthostatic tachycardia syndrome, POTS.  We discussed medications, specifically the use of beta blockers to help control Lucy’s cardiac symptoms; unfortunately, the side effects of the medicines would exasperate many of Lucy’s mito symptoms.  Beta blockers are heavy duty meds for “normal” people, but for a kiddo like Lucy they are very risky. 


Last week we learned, after several discrepancies, that Lucy’s blood type is not the same as mine or Drew’s.  Lucy is A negative, one of the rarer blood types in Caucasian people, leave it to Lucy to be different.  Because of this, we were unable to do a directed donor blood donation.  Lucy is receiving packed A negative red blood cells from the Delmarva blood bank, many thanks to those of you who donate blood!


After several attempts, IV team placed a peripheral IV line in Lucy’s left foot. It’s not pretty, but it gives us a point of access to transfuse the blood.  We are able to keep Lucy’s TPN running at the same time as the blood, which helps us avoid the whole hypoglycemia issue.



Everything went as well as could be expected today.  Lucy’s body appears to have tolerated the whole procedure.  It’s too early to say for sure, but it looks like her heart rate has come down slightly, we have not seen too many numbers above 170.  We will monitor her closely for the next few days so that we can get a better idea of where her numbers fall.

Many thanks to all of you for your thoughts and prayers, we feel so supported!  We would especially like to thank the brigade of loving friends who watch over our three other children so that Drew and I are able to sit bedside with Lucy.  Your love and unwavering support means the world to us!       

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Susan said...

So thankful the transfusion seems to have gone well. Hopefully this will make her more comfortable? I hope you all were able to enjoy the blessings of Christmas this year. I heard about the Sunday you had your children dedicated. I can't believe that was the ONE Sunday that we've not been at Willowdale in months! I'm really sorry we missed it, but from what I heard, it was beautiful. How wonderful that you were able make that happen. Hope to see you soon!
In Him, Susan

Gretchen Johnson Photography said...

Hi Nicole..My husband wants me to tell you that he's A- and wants to donate blood for Lucy. Just let me know when and where and he'll be there.

Looking forward to some fun with the kiddo's and the camera really soon.

Always thinking and praying for your beautiful family!

~plaid said...

I'm also A negative. I feel more inclined to get to the next blood drive now. I'll think of Lucy as I donate. I am sorry for the problems mito gives her and you guys. We still don't know for sure if that is why our two kids died or why the rest of us have so many things not as well as they should be. But whatever the case, I am hoping to help find more answers for those who are affected or may be. If we can improve testing methods and find treatments that are more effective... I am just sorry. Thank you for being willing to share your journey. Sometimes I feel concerned about the things we cope with, but seeing what others are coping with helps me not feel so alone in the world of medical frailty and uncertainties. I hope your new year is blessed and filled with the strength to endure through such a difficult disease.

Jessica said...

Glad the transfusion went well. Eithene gets blood every 7-10 days at this point ( was only about once every 6 weeks when she was Lucy's age) but it is definitely one of the best methods we have to keep her heart rate down. We did use a beta- blocker in Eithene for almost a year, and it was great for her, but now that she has hygpotension problems it's no longer an option.
I hope that you see several weeks of good improvement from the blood and that she won't need more for a while.
Is her anemia from low iron, or something else? If it's from low iron you might try IV iron infusions. They will cut down on the number of transfusions needed by a lot, and have many fewer risks than blood. We did them for about 3 months, but E's anemia is from a bone marrow problem (and she didn't respond to procrit shots) so we are stuck with chronic transfusions.
Happy New Year, and prayers for a year of God's grace and mercy.


Anonymous said...

Hoping that this treatment give Lucy relief from tachycardia for a long while. Barbara