Adjusting to Acrocyanosis

As my more faithful readers know Lil Miss was diagnosed with Acrocyanosis this year.

It was a fantastic diagnosis for us finally knowing that yes indeed her heart is just fine and it's not some connective tissue disorder or anything with her bone marrow that has been causing her to turn blue.

I had thought for a long time it was her heart because Lil Miss was born premature with a hole in her heart and had unexplained Bradycardia as a baby. I was concerned that perhaps she was still suffering from bradycardia (that's where the heart rate drops really low really fast... kinda scarey to watch) and that the Dr.s just weren't seeing it. After all she has been suffering from this since birth, and I spent way to much time in the hospital that first month watching her heart crash.

We have had lots of testing done including 2 ECG's, 24 hour holter monitor to record her heart, an echocardiogram, and countless bloodtests done... and that's just the past 4 months.

All of this has led us to Acrocyanosis, or Reynauds Syndrom. Essentially Lil Miss's vascular system goes into spasm and that decreases the oxygen to the skin. It's considered largely cosmetic, and totally non life threatening!

Worst case scenario for us is if she stays blue too long it can cause necrosis. OK that is a bit on the scarey side. I like her fingures and toes right where they are and am so not a fan of little limbs turning black and needing to be amputated.

Having a diagnosis for us though means that we can learn more about what triggers it and how to prevent it.

So who wants to know the big triggers? Well I'm gonna tell you anyway.

Being cold. Yup cold. Now I know you think well thats easy but it is a bit more complicated, you see cold to us means put a cold drink in a mug not in a glass cause otherwise her entire hands and right up her arms will go blue. Or another example was last summer camping. It was like 34 degrees outside (close to 100 degrees for my American friends) and Lil Miss stepped in a stream with her runners on. For most of us the cool water is refreshing. For Lil Miss it meant her turning blue from head to toe. Seriously even her back was blue.

Emotions can also be a trigger. Lucky for us Lil Miss is the worlds most optimistic person EVER! To her everyday is the best day in the history of the world, it's amazing really. It's a good thing too because she really could cry herself blue.

Lil Miss is going to be 5 in a couple weeks and being so young I was worried about her learning how to cope with acrocyanosis. It is amazing to me that now that we have a diagnosis and we have talked about what causes her to change colour she is being so self aware.

She will be careful to make sure she has her hat and mittens on, and usually that her jacket is done up. Lil Miss is taking into consideration more often that perhaps if it is raining out that she make sure she has a sweater and warmer pants on.

It both breaks my heart and makes me grateful when she recognizes that she is cold so shouldn't be playing outside but rather should come in for other activities to warm up.

It's hard to listen as she tells me, 'I have to dress warm cause I have a-row-sigh-ohhs-is" (OK it's kinda funny how she pronounces it)The thing is though, she knows there is something about her that is different from the other kids and I worry about how that could affect her.

Really she is adjusting to this much better than I could ever have imagined. Lil Miss knows her limitations (I hate calling it that) and she lets people around her know when she needs to warm up.