Appointment recap

Mighty Margaret brought out the big guns (read: lungs) at her appointment today.  We headed to the Heart Clinic for a post-cath check-up and it definitely was an exhausting one!  

Oh my gosh, you guys.  Screaming. So. Much. Screaming!  Today's echo was the worst yet.  I felt awful for the techs -- that's right, techS.  after Margaret started screaming a second tech came in to try to help calm/comfort her.  Nope.  Margaret just wasn't having it today.  They did their best and we took several breaks to try and help her regroup because she was crying so hard that her lips would turn blue.  Not cool, Margaret!!!

We finally gave up and I'm positive the images they "got" were terrible.  Thank goodness Margaret has such an amazing team and her cardiologist knows how sassy she is when it comes to these appointments.  It's kind of hilarious, though, because the SECOND the techs stopped trying to put the wand on her chest, she immediately stopped crying and was smiling at them.  When the cardiologist came in, Margaret was happy and smiley.  Such a little flirt.  She behaves so well for him and it's such a relief.  

Margaret's heart is functioning well overall. Her right ventricle pumps abnormally, which is expected with the restricted size of her pulmonary arteries downstream. But, the pressures in her heart are ok albeit the one measurement they were able to get showed very high pressure (remember: screaming). Her cardiologist is happy with her O2 sats and is comfortable with monthly checkups from now on until the next heart cath will need to take place. Margaret is on a low dose of medication to help keep her heart pressures down. We are expecting her to have another heart cath before the end of the year. The goal is to delay her next surgery as far out as possible, without allowing the pressure levels to damage her chambers.  We do not have an anticipated timeframe for when to expect to be back at Children's for the next procedure.  For now, we get a month pass and will just go appointment by appointment until it's time.  We didn't get to make any adjustments to her current medications as her cardiologist put it, things are working right now, her sats look phenomenal and we don't want to rock the boat.  Our job into check sats a few times daily and call the clinic if Margaret does anything "weird" like gets puffy or starts dropping her sats again.  We got this.

 

**sound asleep before we even got out of the parking ramp.  Mighty, indeed. 💕

Heart Parents

"Your baby has a rare form of heart disease."

Today is a day that marks exactly 1 year since we were catapulted into a terrifying new world.  A world we knew nothing about.  

We decided to drive separately to our appointment so that we could both head to work after. Admittedly, we were both kind of excited to get an opportunity to see our precious little miracle twice in one week.  Right away we noticed how welcoming the clinic was and felt extremely comfortable being there.  We went back for our ultrasound where we made small talk with the tech and for once, our little peanut finally seemed to "behave for the camera" (she must have known that this was a very important appointment!).  The cardiologist came in and she and the tech talked a lot as they collected various images of our baby's heart.  We had no idea what they were talking about since all of the terminology that now is second nature to us was jargon to us at that time.  It was very intimidating being in the presence of such an important doctor and we were thankful to get such personalized attention from her.

our first drawing of her heart

For the next part of our appointment, we were led across the hall into a consultation room.  It's kind of exactly how you picture it in the movies -- minimal "decorations", an easily cleanable stiff couch, small table and a couple of chairs.  As we waited for the doctors to come in, I felt myself becoming nervous but didn't really know why.  After all, we were just waiting to hear that our baby's heart was totally fine and we would be dismissed back to our regular clinic for the remainder of the pregnancy...oh boy, how wrong I was!

The cardiologist and OB came in and got straight to business.  They asked us to share with them our perspective as to why we were referred to their clinic.  We naively told them how our baby is stubborn and didn't let the previous tech and doctor get clear images of her heart so we needed confirmation that her heart was healthy.  I remember watching as the cardiologist's facial expression showed a hint of disappointment before becoming extremely serious.  The next few words that came out of her mouth changed us in a way we would never have expected.  It's all really quite blurry but little pieces of that appointment stick out to each of us.  

One of my strongest memories of this day was looking at Bill and thinking how strong he is and how I couldn't believe how level-headed he had remained while talking with the doctors.  I tried my hardest to keep my composure and not cry but it was too hard.  I finally just let myself cry and I don't recall asking any questions.  I was too stunned.  Thank God Bill was taking in as much information as he could and asking questions because I was most definitely not!

Bill remembers this moment like this:  It really felt like a ton of bricks. The next half hour was kind of a blur. The cardiologist drew us a picture and explained the diagnosis to us. She said our baby would need multiple surgeries. Oh, and try not to spend too much time on our own looking up the condition. Yeah right!

It was extremely hard to not get stuck in the "why us?" mentality.  We had been through so much: several years struggling with unexplained infertility, two big surgeries for me, several failed rounds of IUIs, 1 failed round of IVF which resulted in an ectopic pregnancy and then finally pregnant with our rainbow baby.  We honestly thought we "deserved" this pregnancy and we "deserved" to bring a healthy baby into this world.  Thankfully, our selfish mindset didn't last too long and we knew in order to prepare for this new adventure we were going to need to shift our focus.  We agreed to spend the next 2 days allowing ourselves to be sad, angry, confused and cry but come Monday morning, we were not going to let each other sink into a dark place because of our baby's diagnosis.  We needed to continue to be excited and prepare for our little miracle's entrance into this big crazy world.  We were being entrusted with an extra special baby and it was going to be our job to protect her.

After our appointment, we both called into work and started our weekend early.  We needed time to begin to process everything.  I distinctly remember being so grateful that I rarely call my sister since we usually text.  I think the phone rang maybe once before Jessie had answered and she knew something was up right away.  Talking with her while I drove home helped since, remember, Bill and I both had driven separately to the appointment.  Sharing our "news" with the rest of our families and close friends was difficult.  We remember dreading having to tell people of our baby's diagnosis.  Often, they had more questions than we had answers to and it was exhausting trying to field all of them.  We knew they were going to get on Google so at times we withheld information with hopes of not freaking them out even more.  You guys, if I have any advice for you: STAY OFF GOOGLE! Seriously - get your medical information from medical professionals!! 😉

Our new team had engulfed us at the clinic had already started putting various services, appointments and whatnot into place.  We made it very clear that we needed to speak with any and all professionals who would be working with us and our baby, including needing to meet with our child's soon-to-be heart surgeon --- still so hard to believe.  We received so many hugs from everyone and at the same time, the sadness was met with hope.  We left this office feeling so many mixed emotions but can confidently say, among them, was a feeling of being loved.  We were going to be okay and our baby was going to be closely monitored.  My former principal's staple phrase of "We got this" became a phrase I would say to myself daily just to remind myself that we were not in control and the only thing we could control is how we reacted to the cards we had been dealt.  💕

*side note -- we managed to go through every single detailed (and frequent!) ultrasound without finding out that our little warrior was actually a princess warrior -- kudos to the amazing techs, doctors and nurses who helped us keep our goal of not finding out until birth! 

on the spectrum of TOF, our baby was on the more severe side

Post-Cath

"blow-by" oxygen for a little boost

If there's anything we've been reminded of this week, it's just how unique Miss Margaret's heart truly is.  Although she has the diagnosis of Tetralogy of Fallot with Pulmonary Atresia and MAPCAs, her version of this is pretty unique.  We were also reminded this week that things rarely go "as planned" and slowly we are becoming more comfortable with expecting surprises and going with the flow.  

Margaret's heart cath was overall successful and when we got to see her right after, her O2 sats were in the 90s -- excellent.  We expected her sats to be a little better than they had been prior to the cath since her pulmonary arteries were basically widened therefore letting more blood flow to the lungs more easily.  

So, if everything went well and she looked good, what happened to extend her stay in the hospital?  --  many are wondering this so I'll do the best I can to explain...

slept through the echo 

First - remember how wonderful the entire cardiology team is at Children's.  Margaret was monitored very closely the entire time she was in the hospital and her team did an excellent job of explaining what they could to us.  They also were quick to come up with a plan moving forward.

Margaret started to drop her sats and at first this wasn't too concerning as anytime you mess with the heart it takes some time to adjust.  Unfortunately, she started to drop more consistently and when she fell asleep she was starting to be in the 60s (she even popped into the 50s).  This is where it became concerning.  We hadn't noticed right away, but she also was starting to get puffy and was retaining extra fluid (again, remember that she got admitted early so she could get fluids and be well hydrated for the procedure...).  The team decided to increase her diuretic to try and get the excess fluid out and also drew some labs.  Margaret's hemoglobin was borderline low so a transfusion was ordered.  Margaret had also started to look dusky and that's not something that we typically see with her.  Even when she's dropped sats in the past, we have only seen her look dusky once.  For me, it was reassuring to hear the doctors and nurses verbalize that they noticed the dusky color as well because frankly I often question whether or not I'm actually seeing a change in her color or not.  Turns out, we all were.  

so puffy... but still smiling

The transfusion seemed to help and the diuretic had her filling diapers up in record time.  Her sats started to not dip quite as low, but then they started to go down again.  The next step was to add in a new medication to help with Pulmonary Hypertension, which Margaret has never actually been diagnosed with but the stenosis of the right ventricle could possibly be helped with this medication.  It relaxes the muscle and therefore should theoretically help the right ventricle pump better.  We also had a few extra doses of the diuretic to keep her from retaining fluid and the puffiness started to go down.  It's always weird to see Margaret with puffy eyelids since her eyes are ALWAYS open nice and wide and round.  🐸😊

so mad :(

Next up, oxygen.  We had to get Margaret some supplemental oxygen to get her sats back up to an acceptable level.  She had previously gotten "blow-by" oxygen where the tubing is set next to her and she breathes it in without having a nasal cannula.  This requires a LOT of oxygen and that's why we can't do that at home.  Margaret's doctors wanted to see how she would do with a nasal cannula overnight and whether or not the oxygen helped her keep her sats at a better level AND consistent.  Margaret was NOT happy.  So so mad.  She slept horribly and we were up more than we have ever been since she was born.  It was awful.  Of course, this didn't help her mood (or mine!).  Eventually we were allowed to take the cannula off and go back to the blow-by oxygen since that seemed to work well.

THANKFULLY, her sats started to look much better so we were allowed to see how she did without the oxygen.  She actually started to maintain her sats when she was awake in the mid-80s and even started to creep back up into the high-80s --- great news!  When she fell asleep for naps, she would drop her sats but not nearly as low.  She was bouncing around in the 70s now instead of 60s --- again, excellent, although we still need her to bump up a big higher to at least the 80s.  80 and above is where she needs to be.  

looking SO much better

So that's where we've kind of stopped.  Before we got discharged Margaret took a nap and kept her sats in the low 80s but we still came home with oxygen.  We need to be prepared for anything and unfortunately with Margaret, she likes to throw some curve balls.  We will use the oxygen when she needs it and monitor her O2 levels more often than we had in the past.  Using the oxygen at night will help keep us from worrying more than usual, that's for sure!  If Margaret ever looks dusky or her sats drop below 80 we will be able to put oxygen on her and get her back up.  We will also have it readily available for whenever she may need it and that makes the upcoming cold/flu season a little bit less terrifying.  

finally getting some sleep

so excited to be unhooked!

"let me out!!"

Heart Cath #2

Heart Cath #2 is done!  🎉

We checked in yesterday afternoon so that Margaret could have her pre-op testing done and then stay overnight to get some fluids.  It was important to make sure that she was well hydrated for this procedure.  

Ready!

Pre cath snooze...

The original plan was to get started right away this morning at 8:30am but unfortunately things got delayed a few hours because there was an issue with the humidity in the operating area on the floor.  Thankfully, Margaret's procedure just got pushed back a few hours unlike a couple of other procedures which got moved to different days.  

After -- so confused

Margaret was taken back around 10:45am.  She was definitely not happy to be in someone else's arms and screamed the whole way there right through the CVICU.😟  
This morning we were able to connect with the cardiologist who would be doing the heart cath and we discussed the risks and benefits of what they hoped to accomplish today.  We were reminded that Margaret's Pulmonary Arteries are really quite small and there was the possibility of only being able to address one of them during today's procedure.  The plan was to balloon the arteries and see how much they could increase their size and place stents if needed in order to keep them open wider.  Remember, she has had some work done with these and there is scar tissue that makes them a little tougher to work with because it causes the tissue to be more stiff (like what you experience with any type of scar tissue).  The decision to coil any of the MAPCAs would be made during the cath after they could determine wether or not necessary for now.  Right now the #1 priority is to address the pressures in the right and left ventricles and get those to be at a more desirable "balance".  

At 2:15pm we got the call from the nurse that the procedure was done!  Things went much quicker than anticipated, but results are promising and we are happy!

Here's what we know about what the cardiologist was able to do during this heart cath...

• Margaret's left Pulmonary artery was ballooned and a stent was placed.  This artery went from around 3mm to around 7mm -- Awesome!  Once this was done the pressures on each side of her heart already looked better!  Great news!!
• The right Pulmonary artery is smaller than the left and a bit more difficult to work with.  The cardiologist was able to balloon it and it went from around 1mm to around 2.5mm -- too small to make it worth it to put a stent in at this point.  
• We will have a follow-up appointment next week sometime and talk about the results of the Cath and do a quick check on how Margaret's doing since discharge.
• In about 6-8 weeks we might have to get a CT scan to get more images of Margaret's heart -- she had one of these done when she was born.  It gives the team good images to make a plan.  
• The pressures between her left and right ventricles look much better having intervened with her Pulmonary Arteries.  They are not where we hoped they could be, BUT they are much better and her heart should like the change.  😉
• Possibly the next heart cath could be as soon as before the New Year. 
• The overall reality is that Margaret will have many heart catheterization procedures but this is okay because these are much easier to recover from than open chest and open heart surgeries.  We hope to not have to put her through another big surgery until it is time for her Pulmonary Valve to be replaced -- we are estimating that to be around 2 years old.
• It was decided not to attempt to coil any of Margaret's MAPCAs at this time.  They do not seem to be causing any issues and one larger one actually seems to be helping pick up the slack for her teeny right PA.  

Our evening has been mostly uneventful.  Margaret even was able to jump back into full feeds at 8pm after doing great with a full feed of Pedialyte.  Feeds always stress us out because we know she still struggles with bad reflux and we hate to see her suffer with that on top of being sore from her heart procedures.  

We've been a little late to post an update because we have been so tired and really Margaret has been happy and wanting to play so all of our attention has gone to entertaining her.  We are very much looking forward to being discharged tomorrow!  

SO happy but SO exhausted.

Thank you for the love, prayers, good vibes, etc.  We continue to be thankful to have such a huge village of family and friends surrounding our family as we continue to navigate the world of Congenital Heart Defects.  As tough as this can all be, we continue to be in awe at how blessed we are! 💖

Feeding Clinic Follow-up

All smiles after her appointment 

brushing her new teeth

Hey all!  After our last post ended up having a kind of downer attitude, we decided we should do a quick update tonight.  

Margaret had an initial evaluation with a new feeding clinic this week and we are SO excited about how things went.  Now, we are well aware of the fact that feeding issues take time, usually a long time, to overcome but we think we have found a place where we feel more comfortable.  Our last team just didn't seem like the right fit for us but that doesn't mean they aren't fabulous and haven't had a lot of great success with other families.  We just didn't feel our needs were being met the way that we thought they should so decided to move on.  Our new clinic is much closer to our house as well.

We went into our appointment expecting the team to have a basic understanding of Margaret's complicated medical history, but it turns out, they hadn't received any information yet.  Yikes!  As soon as I started talking the therapists cleared their schedules so they could spend more time with us --- talk about feeling like you're being listened to!  

she likes the spoon - w/o food ha

We got everyone updated as best as we could and talked about what our hopes and goals are for Margaret: eating orally being the #1 priority.  In order to get to this goal, obviously we have to meet other milestones first.  

a girl and her pepper LOL

Margaret was nervous at first and had a nice tight grip on my shirt until she realized that she wasn't going to be poked or prodded by these new people.  She quickly warmed up to the therapists and before we knew it she was interrupting our conversations to squeal and scream as if she was offended that all of the attention wasn't on her quite yet. 😜

Margaret's new team is happy with the feeding schedule she is currently on and super impressed with her growth.  They were glad to hear that her reflux has improved even if she still struggles with it - most of the time she doesn't appear to be in any pain from it but it's definitely still a big issue.  The team was also happy to hear that we were able to transition Margaret off of the elemental formula onto a "regular" type.  The team agrees that we need more guidance and we will now have weekly, that's right WEEKLY, Occupational Therapy (OT) sessions.  Margaret needs to learn to trust and become comfortable with the therapists so a lot of what we will be doing for a while will be strategizing and coming up with things to try each week.  Bill and I feel excited that we will have an OT working with us to help us move Margaret forward.  Up until now, most of our experience with feeding clinics has been "don't do this or you will cause her to have an oral aversion" -- it just really felt like more focus was on what not to do instead of what to try.  

We are optimistic and refreshed.  Margaret is growing so fast and we want to make sure the teams working with her are taking into consideration her growth and we move forward with her therapies as well.  It's going to be long and frustrating, but by golly, we're going to get this girl to eat someday!  

Check it out --- she's been willing to bite down on bottles lately and even appeared to swallow a little bit of formula, too!  Baby steps!