Little Bella has captured the hearts of our family and everyone that meets her. We want to share her story with all.

Sunday, March 8, 2009

Pulmonary Hypertension

Last night Sister Rolfson brought us dinner and I expressed my confusion about everything going on.  She really encouraged me to seek more understanding, so I expressed my confusion to our nurse today about Bella.  She relayed my concerns.  When they talk about her they say, “trisomy 21, transitional AV canal, blah, blah, blah, pulmonary hypertension, blah, blah, blah.”  I get most of my information from eavesdropping (and interrupting) during rounds.  They tell me I know more than most parents, but honestly I have a kindergartener understanding of their graduate level knowledge.  So I research a lot and try to learn more and more.  But I couldn’t understand what Bella is doing right now. 

Her repair was last Tuesday and the surgeon came out and said (as you remember) that it was textbook. The holes weren’t that big.   So in my mind, OK, fabulous, we have solved the problem, we’ll get better now.  Not so fast.  We are still sitting here on Sunday on the vent.  She is still sedated.  She is still fighting the same battles.  I’m confused.  If the heart was the issue, why aren’t we better?  So my cardiology fellow (the same one that sat down with me before she was presented and answered my questions and then told me himself that she was scheduled for surgery) came in and talked with me today.

This is what we talked about.  I’m still trying to make heads and tails of it.  Why didn’t I take anatomy in college?  The heart and the lungs are so interconnected.

If all the problem with Bella was the heart, she would be on the 15th floor getting ready to go home already.  But she has pulmonary hypertension.  Yeah, but y’all said it wasn’t too bad.  Right.  What they meant was it wasn’t so bad that they wouldn’t do the repair.  Oh – glad I didn’t know that before.  Her pressures were a 7 when measured on the cath.  Normal is 1, maybe 2.  OK.  So is her PH mild or moderate (I’m not going to even say severe)?  Moderate.  Ahhh….So, all the drugs and support she is receiving at this point are for her lungs. 

He also explained about the NO (nitric oxide).  It’s pretty easy to come down on the NO till around 5 (a measuring level).  The cells in the pulmonary artery get saturated around 2, so coming down from 20 to 10 – easy.  Coming down from 10 to 5 – doable.  Going down from 5 to 2 and then 2 to nothing – much harder.  That’s when it’s actually happening.  Yesterday they tried to turn her down, and she decided that wasn’t a good idea.  She’s currently at 1.9 /2 and she’s happy.  They want to lose that today.  We’ll see.

He said that they are letting her guide them.  If they rush her, she will go into pulmonary crisis (not sure I want to know what that is) and so they are giving her time.  She could be here another week.  That being said, they are still hopeful that she can be off the vent (and the NO) in a day or 2.  She is on the sildenafil and they will continue to increase that till she is where she needs to it to be.

The chyalis is leaking from her chest cavity.  They said sometimes the thoratic duct gets nicked during surgery and then it’ll drain into the chest cavity.  This is a problem because it puts pressure on the lungs and then they can’t inflate properly.  That’s why the chest tube is in there – to pull the fluid out.  If it doesn’t get better, they can go in and remove the duct.  The doctor feels that it will resolve on its own, especially since she did this before when she was in the NICU.

I have more to learn about pulmonary hypertension, but at least I understand a little more what Bella is doing.


Dolores said...

Oh Shelly. I'm empathetic with you and your sweet baby, all that you've been through and are still going through. So much to learn, so much to hope for. Your faith is helping you. I know there must be moments when the tears just flow. Sometimes it seems like there isn't any way we could possibly deal with anything more. I too have learned far more about medical things than I'd ever hoped to know. What a trial and yet a blessing. Heavenly Father knows your strength. We know Bella's worth every minute, a child of God to love and cherish. He knows and loves her-- and all of you, and wants the best for you.

I hope and pray that this will all soon be accomplished and Arabella can go home and continue her life with Mommy, Daddy and sisters!

Heather said...

Patience is so very difficult right now.But that is exactly what you have to try and be.The doctors are so right ... these kids set the pace.Especially,these kids and this surgery.Zoey was on the vent for 5 days before they even attempted to extubate.Some are longer, some are shorter but Arabella will show you when it's her time.She does look really good.Not as puffy.I'd say she is more than on her way.Prayers continue for your precious Arabella.

CYNDI said...

what is the vent? And by the way, you sound really knowledgable. I would put you at least junior high level. :) can't wait to see you, even if it is in the hospital.

Claudia said...

I totally understand your feelings, as I always thought as well, if the heart is fixed, everything is ok - but as you said, it is not.

Now, pulmonary hypertension is so complicated. From the german doctors I know that everybody fears it, because it is so hard to handle. I don`t want to scare you, but I know how important it is to know where you stand! Arabella will need probably some more days to let her body get used to the new circle due to her surgery. I would like to know, how is she reacting to Sildenafil? Is it good for her?

I do pray for you and for Arabella getting healthy without pulmonal crisis.

Cammie Heflin said...

Addysen had PH too, I'd be happy to answer any questions.