Follow-up this month

Sorry for the radio silence the last last few weeks, but there hadn't been any new news to share. We spent the first half of May finishing up appointments with various specialties, and then we've been waiting to hear what the plan would be moving forward. 

Our cardiologist at Lurie's called yesterday, and, of course, it was when I was in the midst of a self-checkout with children melting down all around me. 🤣 But anyway, she said she wants us to bring Ramona up for a follow-up appointment on June 11. Ramona will have her usual echo and ekg, and it sounds like we'll sort of get the final input from some of the doctors on what they think we should do. 

I asked if we should plan/pack as if we'll be there a while, and, at the moment, it sounds like we should expect an outpatient visit. It also sounds like they might be leaning toward possibly listing her at a lower status with her waiting at home at least to begin with, but we really don't know yet.  Everything is so uncertain and flip-floppy, so  we will continue to hold our "plans" loosely and see how the chips fall. Because I am who I am, I will be doing some excessive packing and prep work at home before the 11th, just in case. :)

I think that's it for now. Ramona continues to be in good spirits and is still her sweet, helpful, and hilarious self. We continue to pray for the doctors to have wisdom in what is the best plan for her, for us to steward our family well, and for healing for her heart. ❤️

Test Results & Next Steps

 

Sister snuggles!

Ramona had her procedure/testing yesterday morning. She had to stay flat on her back for four hours following the cardiac cath, but she was pretty sleepy and snoozed through most of it. The procedure itself went smoothly. Due to the high risk a cardiac cath poses with her condition, they had several precautions in place in case they ran into any complications (including having a surgery team with an ECMO [heart & lung machine] waiting in the hallway). Thankfully, the procedure went off without a hitch.

 Unfortunately, though, the test results were not what we were hoping for. Her condition can cause the pressure inside her heart to rise, which can also cause increased pressure in her lungs. Unfortunately, both of those are high, in addition to heart not functioning (contracting & relaxing) as well as it used to. 

The doctors feel that it is definitely time to list her for a transplant. And then things got confusing.  Initially they were going to admit her until Tuesday, which is when the transplant team would meet together as a whole and decide the urgency level at which to list her. But, now they’re sending us home today for the time being. They said it will take a few days to get all of her lab results in, plus some of the consults that need done can’t be done on the weekend anyway. Then, once they can get everything finalized, they’ll meet and determine the level of urgency to list her at. The doctor said he thinks this is what will take the majority of the time when they meet.

There are three levels of urgency: 

— 1A is the highest priority, and children listed at this level live at the hospital until they receive a transplant (which could be weeks, months, or more).

— 1B is second priority, and these children could be at the hospital or at home, depending on their needs.

— 2 is the lowest priority and waits at home.

Based on what we’ve heard so far, my feeling is that she’ll probably be listed at 1A, but we won’t know for sure for at least a few days. But, we’re glad they feel comfortable with her going home at the moment. None of us had been expecting a long admission right off the bat, so we’re thankful it works out for us to regroup (and repack). We have a lot to process and a lot of logistics to figure out. We were able to get a room at the Ronald McDonald House last night, which was a blessing, so hopefully that will work out again when we return. 

We had a few really hard times, but overall, Ramona in great spirits during this admission. She was upset when she almost had to get a poke this morning, but her diligent nurse was able to get blood through the IV she already had in (thank you, Lord!). Other than that, Ramona has been cheerful! She even said, “This has actually been pretty fun!” at one point (as she was watching YouTube, ordering ice cream with supper, and being waited on hand and foot 🤣).

One funny thing is that the head transplant doctor told us today that there are two groups of trouble makers at the hospital - dads and grandmas. And it’s funny because it’s true! 

I also need to give a shout-out to Mimi for helping with Dottie up here the last few days, and to Papa and Grammy for tag-teaming and juggling the other kids at home. We’re fortunate to have wonderful families! Thank you, also, to the people who have reached out to us the last few days, even though we haven’t gotten back to most of you in all the chaos. We appreciate your love and support. ❤

Getting some more pictures. 

Living her best life visiting sister! 

Game time! 

A Busy Week in Chicago

 

Crazy cotton candy after her
appointment on Monday. 

After having to reschedule a couple of times for various reasons, we've got a busy week at Lurie Children's Hospital this week for Ramona. We drove up for the day on Monday to meet with a doctor who is only in the office on Mondays, and we headed back up again today. We'll be in meetings with various doctors/specialties all day tomorrow (Thursday) - from 8:00 a.m. to 4:00 p.m. Then, on Friday she'll have some testing done with a cardiac cath. 

We're anxious to see what the doctors here think and what they'll recommend going forward. 

The Ronald McDonald House is already full tonight, so we couldn’t stay there this time. But, Uncle Jesse is letting us stay with him instead, which isn’t too far from the hospital, and includes Bluey and popsicles!

We'd appreciate your prayers, especially in the following areas:

- for us to understand and remember all the information we're going to get tomorrow 

- bravery for Ramona, as she will be under anesthesia Friday, which has made her pretty anxious in the past

- safety during her testing & anesthesia

- clarity and wisdom moving forward

- healing

Thank you for praying for and caring for our sweet girl and our family!

Do you not know?

    Have you not heard?

The Lord is the everlasting God,

    the Creator of the ends of the earth.

He will not grow tired or weary,

    and his understanding no one can fathom.

29 

He gives strength to the weary

    and increases the power of the weak.

30 

Even youths grow tired and weary,

    and young men stumble and fall;

31 

but those who hope in the Lord

    will renew their strength.

They will soar on wings like eagles;

    they will run and not grow weary,

    they will walk and not be faint.

Isaiah 40:28-31

Aquarium at Lurie's 

Ramona took lots of pictures of the city. 

More Testing Needed

We met with a doctor in Chicago today, and, it turns out, she was one of the doctors who cared for Ramona when she was admitted at Lurie as a baby! We met sooo many providers at that time that I didn't remember her specifically, but it's nice to know it's someone we've worked with before. She also specializes in Ramona's specific condition, which is great. 

The appointment went well today. They don't have enough up-to-date information to be able to weigh the possibility of transplant at this point, but the doctor thinks that it's worth doing some more in-depth testing so their team can get a better look at how her heart is doing. 

So, for starters, she needs to get some bloodwork done soon. They said it didn't have to be today, which was excellent, because she does NOT do well with having that sprung on her. I mean, it's not great when she knows it's coming either, but it's even worse when it's unexpected. 

After that, she'll need to go back to Lurie's for 3 to 4 days to have a cardiac catherization along with other tests. She hasn't had that done since she was 1, so it will be good to get an updated look. They're going wait until after Christmas, so we're looking at some time in January, but we don't know when yet. Then, we'll see what happens from there. Depending on what kind of results they get, they could hold off on listing her for a transplant, or they could list her at varying degrees of urgency. So we'll just wait and see. 

For now, we just need to get past the impending bloodwork and then on to Christmas. But, first, we need to enjoy Ramona's birthday! She's been counting down for a while and tells literally everyone how many days away it is (it's 5, currently).

PRAYER REQUESTS: 

- a smoother-than-usual blood draw

- joy and focusing on the good

- plans and preparations for January

- our weary hearts and minds

- for Ramona's heart to be healed

- for the other kids not to feel lost in the shuffle

PICTURES: 

We spent the night at her uncle's 
house, which she loved. I didn't
get a picture with them, though. 

We stopped to see her aunts on
the way home. 

Thanksgiving day

They had to go out in the snow 
before church on Sunday! 

Building a lego set when we stopped to visit. 

Back to Chicago

 

There it is. And boy am I glad. 

I utterly hate, HATE that Ramona had to go through so much the last few weeks, but we know it needed to be done. Her heart was pausing for such long stretches that her heart rate was sometimes dropping to under 10 beats per minute at night. Crazy. Just crazy. The doctor said that if it got much slower than that, it could have started causing brain damage. I'm so thankful they replaced the loop recorder when they did, and I'm so thankful she has the pacemaker now (and that both are over with). I'm also thankful that God was watching over her when we didn't even know there was trouble. 

Ramona is doing really well physically. She's had very little pain (almost none), and her wounds look great. She's been more emotional and grouchy than usual, but that's understandable. She has been on a roller coaster. 

And, the ride's not over yet. The doctors here have been sending her files and pictures up to Lurie Children's Hospital in Chicago, which is where she was admitted for a while when she was 1. At that time, Ramona was sent there to see if it was time to look into a transplant, but, thankfully, a medication issue was resolved and everything stabilized. She hasn't been back to Lurie's since then. With everything going on, her doctors here decided it would be good to have a team that deals with transplants take a look at her again. We will be heading back to Chicago to see a doctor there on Tuesday. So we'll see what comes of that. She isn't supposed to have any procedures done, just some of the regular testing she's used to, so that's good. 

PRAYER REQUESTS: 

- safe travels next week

- that God will continue to watch over her and that we would trust his sovereign plan

- that her heart will be healed

Here are a few more pictures from last week, if you're interested:

Video chatting with Daisy 
while waiting for discharge.
Precious.

Her prized souvenir - the loop
recorder they took back out.
She was so excited they let
her keep it! 

Home at last.
So tired; so happy.
(Sweet sign by sweet Daisy) 

"Come to me, all you who are weary and burdened, and I will give you rest."

Matthew 11:28

A Sigh of Relief

It was a long day, but it ended well. Really well. Ramona is settled into her room for the night, where Nathan will stay with her. She feasted on chicken nuggets and ice cream, while snuggling an army of stuffed animals and watching movies. Her grandparents and siblings were able to visit her around supper time, which she was very excited about. Dottie and I are back at the Family House for the night, and she's sound asleep. It all feels like a sigh of relief. 🤍

Thank you so much to everyone who was praying over her throughout the day. It had it's ups and downs, but. overall, everything went about as well as we could have hoped. She was very upset when it was time to go back for her procedure again. They gave her some medicine before heading back that was supposed to help her calm down, but it was no match for her fiery determination. If you know her at all, that's no surprise 🤣 I was able to go with her to the operating room this time and stay with her until she fell asleep, which I am so thankful for. She was very upset, so I'm glad I was able to be with her. 

Ramona wanted me to ride with her,
and the staff kindly obliged.

The procedure went wonderfully, and she did great with the anesthesia. She was very emotional and disoriented for the first couple of hours after waking up, but they said it was nothing out of the ordinary. Once she got past the part where she was crying to go home and asking where we were, she kept asking over and over if Daisy could come see her yet. That was pretty precious. 

They'll be giving her Tylenol overnight to make sure her pain is managed, and she's scheduled to get a chest x-ray in the morning (standard follow-up), and then hopefully head home. She'll have a few days of antibiotics to help ward off infection (they want to be extra cautious since they were going right into her heart). Oh, and the loop recorder they put in last week was removed, and Ramona is very excited that Dr. Ferns is letting her keep it. 🤣 

I feel like I'm forgetting something, but that's all my tired brain can think of at the moment. Thank you again for praying for our family, and especially for Ramona!

PRAYER REQUESTS:

- mainly just thankfulness for God's goodness today

- thanks that "He gives us more grace"

- for a good night of sleep for everyone

- for the next few weeks of recovery

- for her heart to be healed

The silly sisters, together at last.

The bravest girl 🤍

Do you not know?

Have you not heard?

The Lord is the everlasting God,

the Creator of the ends of the earth.

He will not grow tired or weary,

and his understanding no one can fathom.

29 

He gives strength to the weary

and increases the power of the weak.

Isaiah 40:28-29

Pacemaker Tomorrow

Though I haven't used this blog in nearly 2 years, I've had "write a new blog post" on my to-do list since the last update in January 2022 (which is a pretty accurate representation of my to-do lists). We were fortunate that there hadn't really been anything significant enough to warrant an update going on in all that time - just check ups every 3-4 months with things remaining stable overall. Ramona did give us a bit of a scare last fall when she was sick for nearly 4 months straight, which we feared was heart-related, but whatever it was ended up blowing over. We have, however, noticed her getting worn out more easily than she used to, which led to her having some restrictions in physical activity for the first time. 

As you may recall, Ramona had a loop recorder (heart monitor implant) put in when she was one to help find the cause of some symptoms she was having, but the batteries in it expired some time ago. The electrophysiologist who put that device in left the clinic, so the device was just left in place for the time being. 

Fast forward to two weeks ago - Ramona had a regular heart check-up, but we also met a new electrophysiologist at that appointment. This doctor was eager to get the loop recorder replaced. Between the severity of her condition, and the symptom of getting tired so easily, she thought it was best to get a monitor in soon to make sure there wasn't anything troublesome going on. Even with a full schedule, she was able to squeeze us in Monday of last week. 

Ramona was worried before the procedure, but, for the most part, put on a brave face until it was time for her to go into the procedure room. Then, she was hysterical - begging us not to leave her and saying she would do anything we wanted if we would just take her home. So that was really hard. But, when she woke up after the procedure, she was in good spirits and eager to go get pancakes for lunch. :) I was glad that we would be able to keep a closer eye on her heart again, and we were all glad to have that procedure behind us.

We had a follow-up visit for a wound check on Tuesday of this week, and I could tell pretty quickly that something was off compared to our usual visits. Her incision looks great and is healing up perfectly, but, in just a week of monitoring, they are already recording frequent pauses in her heart rate that are over 4 seconds long. She had some 2 second pauses noted when she had the old loop recorder in, and the doctor said at that time that they don't worry about them unless they are over 3 seconds...which we are well past now. What makes it even scarier, to me, is that they are mostly happening at night. If something happens in the day time, we always have a defibrillator with us that we could use to at least try and help her, but if something happens at night, we wouldn't even know until morning. 

So, the solution is to put in a pacemaker ASAP. They had been trying to avoid this when she was younger unless it was definitely needed, because putting a pacemaker in a toddler is major surgery. Fortunately, she is just over the threshold where they can put the device in her shoulder (rather that in her chest) and run a lead wire through an artery into her heart, which is then screwed into the heart muscle to keep it in place. 

And, again, even with a busy surgery schedule, they were able to get Ramona in this week - tomorrow (Friday) morning at 10:30. She'll be admitted overnight and hopefully go home on Saturday. She will have limited use of her arm for a few weeks to make sure everything can heal up without getting dislodged, so she'll have sling to help remind her to keep her arm at her side. We are thankful that she will have an added layer of protection with this pacemaker, and we pray that it will serve her well.

Ramona is not happy that she is having another procedure done already. During her appointment on Tuesday, the doctor explained to Ramona what would be happening and why it's important that it be done (it will be very similar to the last procedure from Ramona's perspective - check in, get changed, get some lead stickers stuck on, and go to sleep), and Ramona smiled and said "OK" to all of it. Then, as we were pulling out of the parking lot, she said "bye building! I'll never see you again!" I chuckled and asked why she said that (thinking it was some kind of joke, because she is a hilarious kid), and she said, in all seriousness, "Because I'm not coming back here and I'm not doing any of that stuff." 

So, that's where we're at. 

PRAYER REQUESTS:

- for God to give Ramona peace, courage, and joy leading up to her procedure

- for us to have wisdom in how we care for her tomorrow (and beyond)

- for her procedure to be smooth and successful

- for a quick and easy recovery

- for Ramona to tolerate the restrictions of her recovery and find joy in that process

- for her heart to be healed (I'll never stop praying for that one)

- thanks for Ramona and the precious gift she is to our family (and our other kids, too!)

- thanks for family and friends helping us fill in the gaps even more than usual the last couple of weeks

- thanks for the hope we have in Christ our Savior

Treasures far beyond what I deserve!

"And we know that in all things God works

for the good of those who love him,

who have been called according to his purpose."

Romans 8:28

Sleep study & more

Playing (I use that term loosely) her
tin whistle at Classical Conversations.

Hi there! I keep meaning to post an update with the results of all the things we were waiting for after my last post, and I've somehow let nearly a year slip by without turning those intentions into action. *facepalm* At long last, here you go!

This is from her sleep study. She gave
me a thumbs-up, but I don't think
she was feeling thumbs-up at all.

Ramona had her sleep study last spring. The study itself was...chaos? But, the results were just what we were praying for - no sleep apnea! Thank you God! Nathan stayed with Ramona for the overnight study since I had a nursing baby. Ramona did not enjoy having all of these sensors stuck all over her, and then they tried to put a sensor INSIDE each nostril. She was (understandably) having absolutely none of that. They tried to sneak them in after she was asleep, but that wasn't going to happen either. Nathan said she also woke up a couple of times and tried to rip all of the other sensors off. When he told me all of this the next morning, I was so worried we were going to have to do the whole crummy thing again, but, thankfully, they were able to get everything they needed. We are so thankful for only having to do this once and for the good results!

Another thumbs-up from her echo in
September. This is the easiest part.
It's also the part with cartoons.

With regard to her loop recorder, the battery in it ended up lasting until almost Christmas! Her nurse said that was the longest she had ever seen one last. We were so thankful that we had her loop recorder and that it enabled us to get to the bottom of the scary symptoms she was having (waaaay back when she was at Lurie's for her transplant evaluation). We're also thankful that, once those problems were resolved, nothing else concerning came up the rest of the time the device was working. We will need to get the loop recorder removed at some point, so we'll probably address that at her next appointment. 

Rapunzel in tow; waiting to see Dr. Albaro.

Speaking of appointments, at her appointment in September, her doctor said that we could try spacing out her appointments to every 6 months since she has been so stable for so long. We've always had appointments every 3 months until now. It's good to know that the doctor feels confident enough to space them out. Buuuuut it's been an adjustment for me, because it turns out I really like getting that regular confirmation that everything is still okay in there. 😅

Baby brother just had his first birthday and is positively bursting with energy and adventure. He had a couple of echoes and other testing done in the first couple of months after he was born, and they came back perfectly perfect, as we expected. He does not have the gene mutation Ramona has that is known to be tied to HCM, so we expected that his heart would be normal. Since Ramona's HCM was soooo severe at such a young age, the doctors suggested (and we agreed) it wouldn't hurt to be extra cautious and make sure everything was okay.

I *think* that ties up the loose ends from last time. 😊 She's had a great year all around. She got to be in her first dance recital, started homeschooling and attending Classical Conversation, and continues to love playing with her dollhouse toys and legos. 

Her next appointment is in March, and we're praying for another good report. Ramona has already adamantly (and loudly) told me that she is not going to her appointment because she is NEVER doing a squeezy (blood pressure) again. That was discouraging, because everything went SO WELL at her appointment in September. The nurse was wonderful, and Ramona (though she was nervous) didn't fight or cry at all when she took her BP. Ramona even acknowledged that it didn't hurt afterwards (which is what she's always afraid of). She was terrified of the blood pressure machine when she was little because it squeezed so tight, and, even though the nurses do her blood pressure manually now, we've just never been able to get past that fear. Even with good experiences like her most recent appointment. *sigh* She is such a brave girl and has done so many things that no kid should have to do. Please pray that she'll be able to get over this mental obstacle. She was so little when that originally happened that I don't think she remembers it actually hurting, but the fear from it left such a strong imprint on her. 

PRAYER REQUESTS:

- That Ramona will not be so scared of having her blood pressure taken (and that I know what to do to help her better)

- That her heart will be healed

- Thanks for good test results a great year!

And, of course, some extra pictures. I am what I am. 😊

At the playground last spring.

Super Kitty and Tooth Fairy Princess

She was SO excited to turn 5!

Easter bunnies with aunt Jill.

Sunset walk with a cousin.

We were thrilled to finally get to visit
grandma in Florida! And in January
to boot. What a treat!

Their summer dance recital. They 
both had such a good time.

We tried out a one-night family camping trip with 
a baby. It was surprisingly great!

She calls this her "beauty dress" and
has been wearing it nearly non-stop
since Christmas.

"My flesh and my heart may fail,

but God is the strenth of my heart and my portion forever."

-Psalm 73:26

"I believe that I will look upon the goodness of the Lord

in the land of the living."

- Psalm 27:13

Odds and Ends

Me: Smile for a picture!
My kids: *goofy pose*
😂

 Hi there! This update covers several odds and ends from the last few months, but the overall report is that Ramona is doing great. 😊 This update is also several weeks past due, but that's because we added a baby brother to our lineup in January!

The girls adore their baby brother, and he already seems to like
them an awful lot, too! They're such good helpers and love 
to take care of him.

Ramona had her heart appointment in January, and we're thankful to report that things are still stable for her. The wonderful nurse that keeps track of the readings from Ramona's loop recorder noted that she has been having some pauses in her heart rate when she's sleeping, but they aren't anything that would be considered dangerous (anything less that 3 seconds long isn't considered dangerous). It's considered normal for that to happen to anyone when they're sleeping, however, since she has the loop recorder, we can see that it has been happening to her more frequently, so she was referred to a pulmonologist just in case it could be an indication of sleep apnea. She doesn't have any other symptoms that would indicate that she has sleep apnea, so we're hopeful (and praying!) that she doesn't have it. She's scheduled for a sleep study next month to look into it. She is actually excited about it because her daddy will get to sleep there with her, and she gets to have a breakfast date with him the next morning. 😂

Thumbs up after her echo. 👍

No tears for her (manual) squeezy!

The other thing we talked about at her appointment is that her loop recorder is at the end of its 3 year life span. (I can't believe it's been 3 years already!) I get a lot of peace of mind between appointments from knowing they can sort of keep an eye on her from afar with the loop recorder, and her doctor shared the same sentiment, so it sounds like we'll probably be able replace it with a new one when the current one is removed. We've got an appointment with both her regular cardiologist and the electrophysiologist (who actually does those kinds of procedures) next month to discuss it more and make a plan.

In unrelated medical news, Ramona was having some foot problems that we were referred to a podiatrist for (this girl has more appointments and doctors than the rest of our family combined!). Thankfully, the issue was short lived and hopefully shouldn't be something we need to worry about in the future. The most notable part of that whole thing was that she had to get an x-ray of her foot. If you recall, she does NOT have good memories of her most recent chest x-ray, so I wasn't looking forward to this one. She ended up having a HUGE meltdown when it was time for the x-ray, even though I had bribed her with a cake-pop when she was done. I also bribed her with a toy when we couldn't even get her on the x-ray table (no apologies and no regrets - I do what I need to do for a scared girl with more appointments than a kid should have), and that did the trick. She was so brave and ended up being fascinated by the light and huge camera when she calmed down. The person doing the x-ray gave her a little teddy bear when she was done, and I asked her if we still needed to go to Walmart or if we could just get a cake pop since she had a nice new bear, and she chuckled and said "let's go to the store." 😂 

Brave x-ray girl.

Baby brother had an echo and extra sonograms before he was born to check out his heart (everything looked good), and he had his first post-birth echo in January. Everything still looks perfect, and he slept through the whole thing. He's having genetic testing done to see if we need to worry about anything popping up in the future, so we'll have an update on that later. 

So. Tiny. 😍

I think that's everything for now. Ramona is still happy, active, and hilarious, and both girls love having their brother around. 😊 Oh, and we just had the 3 year anniversary of when we were able to come home from Lurie Children's hospital! It's hard to believe it's been that long, and wonderful to look back at how great things have been for her since then. Such a blessed 3 years. We're so thankful for all of the time she's had to just be a kid, which is such a blessing given her condition.

PRAYER REQUESTS:

- Ramona to continue to thrive, and her heart to continue to do its job well

- Thanks for how well she has been doing for so long

- Continued wisdom and skill for her doctors & nurses (we're so thankful for them!)

- Planning and preparation for removing/replacing her loop recorder

- Her upcoming sleep study - both the study itself and the results (no sleep apnea!

- Thanks that her foot issue wasn't a major problem

- Peace of mind and good results regarding baby brother's heart

- Thanks for the blessing of a new baby!

Thank you for praying for our sweet Ramona and our family! We are thankful for you!

MORE PICTURES:

Somebody had a birthday!!!

She told me months in advance that she wanted a round cake with a smiley
face with eyelashes and curly hair, with her name on the side, and with green
and purple frosting and sprinkles. She stuck to her guns and loved her cake!

Eating lunch in the van, as has been the custom most of the last year.

This is random, but I was super excited to find the perfect backpack!
Our AED fits perfectly in the bottom compartment, and the separate
top compartment serves as out diaper bag! So much easier than multiple bags!
(from Walker Family Goods)

They got swimming lessons from an amazing teacher last summer.
Can't wait to do it again this year!

All dressed up for a family wedding. Five minutes into the ceremony
Ramona said "Uuugh! When are they going to KISS?!" She loves
weddings. 😂 P.S. Check out that handsome husband! 👀

Always picking flowers.

Picnic at the playground.

Halloween with a cat and a pirate with whiskers
(who insisted she was NOT a cat pirate).

Bringing back the side ponytail. She loves to get her hair done!

Traditional homemade pizzas on Christmas Eve!

Playing their new charades game on Christmas.

She's still allll about the playmobil people.

And she's still all about everything soft and snuggly.

Thankful for some nice days to play outside!

Yes, my soul, find rest in God;
   my hope comes from him.

Psalm 62:5