Our adventures continue into the summer of 2016.  I decided to take the summer off, a first in basically forever.  It's been nice to have nothing I "have" to do and to be able to focus on us for once.  Quinn has been doing great since her heart surgery.  She is growing (we finally hit 17 pounds in August) and thriving.  She is an amazing little girl and she has taught us how strong our love can be.  We had a bout of Pneumonia this summer, but other than that she's been great.  She's crawling now and always into things, she already knows she can get away with anything.  Her beautiful eyes draw everyone in, people can't help but stare at her and they can't figure out why.  Although it probably has something to do with the slight Noonan features, she is pretty dang adorable.  Chris and I found out today that we are not carriers with Noonan, this means Quinn has a random mutation.  Something she will have to think about as she gets older.  But a beautiful soul who can do anything, I can't wait to see what she does in life. (well maybe I can since she's my last baby).

Chris started a new job on July 25 with Probation and Parole, this means 10 weeks of training, 8 of which are in Madison.  Soon enough Tahlia will be starting Pre-K and Mylee in 1st grade.  Everyone is growing fast.  

A little TLC goes a long way

Great appointment today, don't have to go back for a month. Quinn's heart rate is in the 130s.  Doc diagnosed her issues in the hospital as Post pericardiotomy syndrome.  We will continue with the Naproxen for another week and then wean her off over the next week, as long as she doesn't develop a fever or agitation we are good to go.  She can go back to daycare Monday and life can go back to mostly normal (we hope)   Now we just have to fatten this baby up as she is barely 16 lbs after the surgery.  She's in a very good mood, rolling around, and playing like a sweet normal 10 month old.

Happy Early Mothers Day

After moving into PEDS on May 6, we were prepared to stay until Monday as that is what the PICU doctor thought was best.  However, Quinn's cardiologist surprised us in the morning and asked if we wanted to go home.  Quinn isn't going to be any better or worse whether she is at home or in the hospital and since she was eating, playing, and happy it was time to go!  Her heart rate is still a little higher than they would like and the PVC's are a little more frequent than they like.  However, as the doctor explained today, we should think of the PVC's as a gift from God because it means her heart understands that it needs to change.  The area causing the PVC's is trying to help the area that was fixed, because of this it's sending a signal to the heart to speed up.  The hope is that her heart will figure out soon how to work together.  Her echo showed nothing bad and they did NOT check the gradient (on purpose) because they want to give the heart time to heal.  Quinn is on Naproxen which is another name for Aleve.  This is to help with the inflammation that is still in her heart from the surgery.  Quinn was in no mood for a nap and has been happily tucked in bed for 2 hours now.  The hospital ruined her, she now needs light to fall asleep (she preferred all darkness) and needs some noise to comfort her (should be interesting with dueling lullabies when the older girls get home).  She is pretty much back to herself, tried out her tummy but didn't stay too long, and can't sit up on her own, but hopefully she regains her strength soon.  She had some applesauce tonight which is the first real food she has eaten since last Sunday, although she threw up shortly after, baby steps!

So happy to have her home.  We have a follow up on Thursday.

Getting better... little by little

Today we moved to the PEDS floor (at 9PM).  Which is great news since in the morning they said another night in the PICU.  They were hoping the potassium and magnesium would help with the PVCs, but it didn't although now her labs are coming back good with the boost in electrolytes and now that she is eating bottles pretty regularly she's doing quite a bit better.  They believe that the right side of her heart, because it was so overworked before, doesn't know how to relax and is working over time.  So they are giving her digoxin and milrinone to help with low cardiac output system and the PVCs  We stopped the milrinone in the morning to see how she does off that.  So far her heart rate has dropped to the 150s, yesterday it was the 170s, the goal is the 130s but things seem to change all the time so maybe we can leave sooner.

They did an Echo today to check for fluid around the heart and there was none and they determined the PVCs are benign and that her heart just needs to figure it out.  The doctor last night thought maybe we could go home Monday (wishful thinking would be Sunday, the nurse asked if we were going home Saturday, so maybe she knows something we don't).

Quinn had lots of visitors on Friday.  Mylee and Tahlia came, Grandma and Grandpa, and Aunt Shelly.  Daddy also came back for the weekend.  She was pretty wild with all her visitors.  Still didn't sleep very well, but it's a new room so I am sure that is why.

Today was a good day...

Quinn is acting, well like Quinn.  Being a little stinker today trying to pull out her cords and wires (and there are a lot of them.  Her PVC's have gone down a lot throughout the day (happening way less frequently), we learned that they think its because of the inflammation of the heart and just needs a little more time to heal.  She is still on about 3 IVs, and had a slight fever this afternoon, but her mood is better.  She played hard today (hopefully that means she sleeps longer tonight)! Grandma came to visit and I held her twice.  It's a little intimidating holding her knowing her chest is wired shut and that you have to pick her up supporting her neck and butt for the next 6 weeks (no picking up under the arms).  She spent the day trying to eat her toes, but was unsuccessful with all the wires and cords... good effort though!

Looking forward to getting this little one home.  Hopefully we will have a good day tomorrow and can get out of the PICU.

Day 3...it was a little rough

Today, was interesting to say the least.  Sometime in the AM alarms started going off because of irregular PVCs and multifocal PVCs. Dr. Google in the middle of the night is never a good idea.  Something to do with irregular extra heart beats that disrupt the normal beat.  Her fever is gone.  They thought the problem was the chest tube, that came out with no troubles.  But didn't help the PVCs. Cardiologist wasn't too worried, but ordered and Echo because she heard a rub along with the heart murmur (whatever a rub means).  The Echo looked pretty good, gradient down to the 30s which is what they hoped for.  She was pretty out of it most of the day.  She was having some trouble with her BP and a high heart rate, so they did a bunch of labs.  The PVC's continued throughout the day.  The on call cardiologist read the echo more closely around 5PM and noticed her heart was struggling a bit and was working a little too hard for what it should be, so they put her IVs all back in and started her on some magnesium, potassium, and switched her to a flow of basically ibuprofen because they think she is having a bit of pain.  They also have her on something to help her heart so it doesn't have to work so hard.  They'll see how that goes tonight and tomorrow and then go from there.  They'd like to figure out the PVCs and so far they are still happening about as frequently as they were earlier.

Quinn perked up a lot when we video chatted with Mylee and Tahlia tonight, that was a good sign.  She hasn't been to interested in her bottles, but hopeful she'll start getting hungry soon.

Her incision doesn't look scary at all.  That's a good sign.  Can tell she's had a rough day from her morning pic to her evening pic.

Love this sweet baby so much!

We survived Open Heart Surgery

   After a delay in surgery, or maybe someone told us to be here at 8:30 and we didn't have to be here until 10.  Quinn headed back around noon.  It took them some time to get her ready for surgery and she was on the bypass machine around 1.  The heart surgery itself was pretty quick.  They repaired a Patent Ductus Arteriosus (PDA ligation).  According to Dr. Google, this is a problem because "When the ductus arteriosus stays open, oxygen-rich (red) blood passes from the aorta to the pulmonary artery, mixing with the oxygen-poor (blue) blood already flowing to the lungs".  They fixed her PFO (hole in her heart) which was bigger than expected.  They also did a pulmonary valvotomy to widen the narrowing of her pulmonary valve and also reshaped her valve (it will never be perfect).  They are pretty confident at this time that the gradient will be down to the 30's which can mean no more problems for the rest of her life (that is the goal and the hope).  They will watch it for life. 

  She was pretty crabby when she started coming out of anesthesia.  They didn't let us in to see her until after 5, which was pretty stressful, but they wanted to calm her down.  After we met with her normal cardiologist, they let us in.  It was a pretty rough night, she was supper agitated.  They kept giving her different variations of meds to keep her calm.  Finally found a good one in the middle of the night.  She's been spiking a fever, was at 103.1 last night, but they are working on getting it down today.  She's a lot calmer on day 2.  Still has a chest tube in with some bleeding, they hope to remove that tomorrow.  She's slowly starting to wake up now (3:15PM).  They have her off of oxygen, she's comforting herself with a pacifier (which she doesn't normally take), but we can't hold her with all of the tubes and IV's (she's got one in her neck, one in her arm, one in her foot, plus a catheter, and the chest tube).

Quinny Binny

When Quinn was born, we learned she had a heart murmur.  After being referred to a Pediatric Cardiologist in August of 2015, we learned that she had a congenital heart condition called Pulmonary Valve Stenosis.  This restricts blood flow to the lungs caused by a narrowing of the valve.  The Cardiologist also mentioned that she thought Quinn might have something called Noonan Syndrome which can be diagnosed through genetic testing.  In March, 2016 Quinn went through a balloon catheterization procedure to try to stretch the valve open so it worked properly, at this same time we pursued genetic testing.  The balloon catheterization decreased the gradient in her heart by 20%, but was not enough to give her lasting relief.

Quinn was diagnosed with Noonan Syndrome in April, 2016.  She was found to have a mutation of the PTPN11 gene which is the most common form of Noonan Syndrome.  (Noonan Syndrome is usually hereditary, but can be caused by a random gene mutation) Noonan Syndrome affects many areas of the body, including a common finding of Pulmonary Valve Stenosis.  Typically, it is characterized by mildly unusual facial characteristics, short stature, heart defects, bleeding problems, skeletal malformations, and other signs and symptoms.  There is no way to know which problems Quinn will have associated with Noonan Syndrome, it can be very minor, or more severe.  There are numerous things they will check throughout her lifetime, but no way to predict the future.  A good website with information about Noonan Syndrome is http://www.teamnoonan.org.

Blood testing shows that Quinn may have bleeding issues due to a mild coagulation Factor VII deficiency and Low von Willeband Factor activity.  These both affect how her blood clots and might cause excessive bleeding, these are both common findings in people with Noonan Syndrome.  Nothing has been diagnosed regarding bleeding disorders as one blood test can’t be definitive.  However, the Pediatric Hematologist on her case has plans in place should anything occur during her open heart surgery.

The plan for Quinn is for Open Heart Surgery, her team of Cardiologists believe this is the time to do it so she can have the fullest quality of life as she starts to grow.  The wait and see approach has shown no improvement in her heart as she gets bigger with the gradient being around 79 (this is diagnosed as moderate severe pulmonary stenosis and right ventricular hypertrophy) The effects of her heart defect right now are tiring easily, and some minor blue spells, she is also slightly behind in her gross motor skills.  Overall, she is a very happy, healthy baby and brings great joy to our lives.