Before You

Before you, I didn’t know.

I didn’t know that this world of little broken hearts existed.

I didn’t know that some babies must fight for each breath and every heartbeat. I guess, I thought I knew pain. I thought I knew desperation, but who am I really kidding? I didn’t have a clue. How can you know until you’ve lived upon the precipice of loss and prayed that today’s crisis wouldn’t bring your whole world crashing down? 

Until you’ve lived it, you can’t know what it’s like for all of your hopes, dreams, worries and cares to be wrapped in such a tiny package that you have no control of. 

In the early days, when it became clear that we were not guaranteed tomorrow, I made the conscious decision to know every little detail of you. I’ve spent countless hours studying you with an intensity that can only be found in a desperate mother’s soul. Every aspect of your precious face is etched into my heart too deeply to ever fade. But past your sweet facial features, curls, and those precious little fingers and toes (that look so very much like your father’s) I know you like no mother should.

I know the harsh murmur of your tiny surgically altered heart and the pale sheen of your scars. I know if you’re cyanotic or acidotic with just a glance. I know your baseline capillary refill and what colors your skin should be but often aren’t. I can tell your fluid status by looking into your eyes and a gentle squeeze of your tootsies. I know your sweet little eyes focused and glazed. I know you in the pits of delirium and withdrawal. I know your medications, their side effects, and your new found allergies. I know the difference between contented punches and kicks, and those of delirium or pain. I know how much of a given narcotic you can handle weaned and at what frequency. I know your cries—because of frustration, withdrawals, anger, pain, or that which you save for times of crisis. 

Before you, I thought I knew strength and resiliency. But, once again, I didn’t have a clue. I associated sickness with weakness and frailty. How could I have ever been so wrong? Seeing you, and other little loves like you fight for survival, has taught me so much in life. You are stronger than I could ever imagine being and more brave than any legends of old. Though your odysseys are wrought with uncertainty, pain, and fear—you fight on via sheer force of will. You have endured more in your infancy than I shall ever, and yet you’re capable of love and sharing sweet smiles (especially when your daddy gives you Eskimo kisses).

Precious baby, because of you I have delved into depths of pain and desperation. I have felt true and utter helplessness and vulnerability.

But, I have also soared with hope and swelled with pride. I have learned to love wholly and completely, without regard to tomorrow's unknowns. 

Before you, I never wished to know of this world of broken hearts but here we are. 

And you know what baby? 

There’s nowhere else I’d rather be. 

**I'll update on Avelyn's status and the last couple weeks soon. A lot has happened since the last update! Between blog posts if you would like to check in on Avelyn's journey please follow my Instagram. 


**If you choose to share this or any of my previous posts please do! But I ask that you do not alter my writings in any way and provide a link to the shared material. If you have any questions about this request, please ask me**

   

One Day at a Time

Though the days have not all been sunshine and rainbows, overall Avelyn is still making progress on the cardiac step-down unit. The biggest incident  in the last week was Tuesday and into Wednesday morning. Sean had brought up his concern about Avelyn’s increasing heart rate in rounds Tuesday morning, but the team chose not to investigate at that point. She had just been weaned from Methadone the day before and tachycardia is a potential withdrawal symptom. As I came back into town from working I was concerned but not overly so. Avelyn’s blood pressure was a bit lower than baseline Tuesday and she was having bad diarrhea throughout the day. Things began to cascade to a dangerous level in the early morning hours with persistent diarrhea and fluids also being pulled off via dialysis.

The resident assigned to the unit came in to evaluate around 4am and I point blank and very clearly stated she needed fluid. To me it was clear, her heart rate was up (180’s), her blood pressure dangerously low (50’s/ high 20’s) and her weight had been down the day before. As a kidney failure patient, Avelyn’s kidneys do not react to dehydration properly (aka renin-angiotensin system) and her blood pressure is highly dependent on fluid status. Back to the story, the resident decided it was best to hold off on giving her fluid and to also hold her 4am methadone dose (he thought it may lower her blood pressure further- bollocks). His reasoning was he didn’t know her case and that she is complex so he wanted to wait for the day team to arrive and make the call. I was dumbfounded. Literally.

We’re still settling in on the step-down unit and how things are run, and at that point I didn’t feel like I was going to get anywhere with the resident. I asked the nurse if there was an attending provider available. She said there was “only one for the whole hospital at nights so they stay pretty busy”. I felt lost and was busy trying to comfort a very sick baby whose Methadone was needlessly being held for hours. By the time the nurse practitioner got there around 05:30 the nurse stopped her in the hall before she had put any of her belongings down. She came in immediately and as soon as she walked in I pleasdd with her to give Avelyn a fluid bolus and Methadone. She agreed that Avelyn likely needed such but felt the fellow needed to be called first (I guess next time I need to ask for a fellow specifically :/). The fellow came in and thank goodness knew Avelyn from rotations through the PCICU. He ordered the bolus stat and pushed the methadone immediately. It took a few more hours to get her stabilized and comfortable but she bounced back fairly quickly.

During the turmoil Avelyn’s clonidine patch was removed in an attempt to get her blood pressure up. Once it was off the team figured it was as good of a time as any to start weaning it. So now she’s being weaned from both clonidine and methadone. We don’t know the plan for the clonidine wean, despite Sean and I both asking multiple times. As you can probably tell, the lack of urgency and specifics up on the floor has been difficult for us after having spent so much time in the PCICU. :/

Other than medication weans Avelyn has been working on feedings. We have to get her on a regimen that helps bring her triglyceride levels down and that she can tolerate without chronic intestinal irritation and bleeding. That’s been a major challenge. Right now she is getting a special formula mixed with breastmilk. We’re praying she tolerates the new combination because there really aren’t any other obvious safe options. She’s persistently having tiny flecks of blood in her stool but so far it isn’t worsening and so we’re praying the irritation resolves with time.

As of now we’re looking at about another month to come off of clonidine, get her blood pressure regulated, feeding sorted out and onto bolus feeds (if possible). Although it’s a lot, after all she’s been through we can see a light at the end of the tunnel for this hospitalization.

After discharge, we’ll be keeping Avelyn in Nashville for a time, mainly to be safe but also because she’ll have so many outpatient appointments and daily dialysis will make day trips impossible. We hope to get her home to Johnson City before spring arrives but we have no plans to rush the process. Then, if all goes well, she’ll need to return for another heart cath sometime between March-May. Dependent on those results and how her left kidney functions with time to recover, we’ll be able to know when and if she’ll need her kidney transplant (still extremely likely within the next few years).

In the meantime, we’re doing our best to live a day at a time and savor ever bit of progress and every milestone. It’s not an easy life and we often get overwhelmed with the uncertainty and pain of it all. But Avelyn is still giving her all and who are we to not give ours?

All in all she is making steady progress in the being a baby department and is learning to play with toys, enjoy the sunshine and get food all over her face. All of which are major milestones for our wonder girl!

As always, thanks for checking in and I hope all is well with you and yours!

Between blog posts if you would like to check in on Avelyn's journey please follow my Instagram. 


If you choose to share this or any of my previous posts please do! But I ask that you do not alter my writings in any way and provide a link to the shared material. If you have any questions about this request, please ask me.

 

          

Moving Up

Sorry to have gotten a bit behind on here, things have been busy the last few weeks. I guess I have not updated since Avelyn’s heart cath a couple weeks ago. She went into the procedure in a better state and more stable than she has been in a while. She was on .3-.1 liters of oxygen and tolerating feeds well.

Sending her down for the cath was very difficult for us. We knew it would make her worse in the short term. We knew it would affect her kidneys and her pulmonary status. We knew she would be intubated and sedated, but just because you know something does not mean you can prepare for it in this cruel CHD world.

Avelyn did well for the actual cath procedure but findings were not what we had hoped. Her pulmonary artery anatomy is worse than expected. Recent echos had been suggesting growth of her left pulmonary artery, unfortunately this was not the case. Her left pulmonary artery was so small that if the cardiologist hadn’t intervened the artery itself would have withered away in a few months’ time. Meaning her most invasive open heart surgery, that sent her into renal failure and onto ecmo (when she all but died on the operating table and was maintained with chest compressions) would have been for nothing. So we are thanking god that the team pushed the heart cath and we agreed to it. Otherwise, I don’t think Avelyn would have a chance. We later learned that going into the cath Avelyn’s left lung was receiving an estimated 5-10% of her pulmonary blood flow.

Anyway, while she was in the cath lab we got a call saying that a stent would need to be placed in the left PA. I relayed this information to Sean in shock. Maybe in the future getting a stent won’t be so bad, but for now it means surgical removal in a few years, aka another open heart surgery. Sean and I were both so disappointed, honestly disappointment doesn’t touch the feeling. We held it together, knowing and trusting the doctors and surgeons would make the best decisions concerning Avelyn’s wellbeing. We went into meet Avelyn’s cardiologist after the procedure believing that he had stented the left PA. However it turns out that just prior to placing the stent, he decided to try a more aggressive form of ballooning. Surprisingly her artery responded very well. Her superior vena cava was then ballooned in the same way. Her left PA is now about twice the size it was prior to the cath. Her left sided pulmonary blood flow is now about double what it was, which still is less than half of what it should be. This causes her right sided heart pressures to remain elevated, too elevated. At current levels, she’ll eventually go into right sided heart failure. To prevent this, she’ll need to have multiple more heart caths to continue working on her pulmonary artery narrowings. We have hope that eventually her right sided heart pressures will decrease to a manageable level, though it’s very unlikely that Avelyn will ever have normal right sided pressure.

So back to the story…. Avelyn did well with the cath procedure but with the amount of work she had done, the cardiologist warned us to be prepared for a severe reperfusion injury to her left lung. Meaning significant left sided pulmonary edema. We were told to be prepared for about a week of intubation. However, as usual Avie played by her own rules. She did great with the cath from a pulmonary standpoint. She was extubated that night to my and Sean’s amazement. But then things started to get much harder for our sweet baby. She grew more and more delirious and was in pain. She couldn’t sleep and cried for hours on end because of adverse sedative medication effects. It was a really hard few days on her. All the while her feeds were held, and then when they were restarted it was at a low rate because her feeding tube was pulled back to her stomach rather than her intestine (which needed to be done for her to eventually go home).

She tolerated the feeds into her belly well for a day and then started vomiting and having blood in her stools. So feeds were, once again, stopped and she was placed on yet another NEC rule out. In this time she was found to have an intestinal infection, likely due to bacteria overgrowth from being on vanc (which is from her NEC and bacteremia episode last month) for so long. So the vanc was stopped and different antibiotics (flagyl and Cipro) to treat the new infection were started. After three and a half days, feeds were reintroduced and she tolerated them from a digestive standpoint. For the first few days she was vomiting a lot, nearly hourly. She also seemed to be in pain. I took my fair share of flagyl for a couple years while fighting Lyme disease and for some people, it’s rough. I guess our poor Avelyn takes after me in yet another unpleasant area. She struggled on the flagyl and eventually I asked the infectious disease doctors to discontinue it early. To me, it wasn’t worth the intestinal irritation, vomiting, loss of calories, and risk for aspiration it was causing. Since it was discontinued she has done much better. She’s still somewhat gaggy but it’s nothing compared to before.

Throughout all this, Avelyn was transferred to the cardiac step down unit. I had always hoped she would be in a more stable position before going up to the 7^th floor, but life never really works out like we hope. Thankfully Avelyn has done well since transferring up. She hasn’t been requiring oxygen, the gagging and retching have improved and  she hasn’t shown any further signs of infection. She continues to struggle with skin integrity, due to malnutrition after going so often without feeds in the last month and a half. She also continues to have these episodes that appear like an allergic process. Her eyes, hands, feet, and inner legs  flash very red and then fade over an hour or so. However the incidents are not as often as they were earlier in the week. So I am hoping this is a weird side effect from the Cipro antibiotic, which she finished her course of today. She is now officially antibiotic free! For the first time in months! It’s a big deal and I am praying her natural flora can repopulate and her little intestines will have a break. She has been having diarrhea for months, which is just so sad.

All in all, it’s been a very trying couple of weeks with huge milestones. Avelyn finally got out of the ICU after 293 days! She has been outside every day since transferring and now knows what sunshine and wind feel like on her face. She has been able to get more rest in a quieter and less acute setting. All of which has been great for her. But it’s a big transition. Sean and I spent 292 days in the ICU right alongside her, getting the know the staff, routines and environment. So much is different on the 7^th floor and it’s been a big adjustment. But after all we’ve been through, it’s not as overwhelming as I thought it may be. We get to participate more in her care and treatment plan now that she is more stable. Sean and I often are the ones to take her vitals and administer her medications, which allow us do so how and when is best for Avie. Believe it or not, we now spend even more time at Avelyn’s bedside than ever. We don’t leave her alone since going up to the floor. The nursing staff is spread more thin and the doors are opaque.
(though she is still on constant telemetry) I worry too much to leave without someone there to comfort her if she gets upset or lift her up if she vomits to prevent aspiration.

As time goes on, I hope my to share Avelyn’s growth and development. I hope to show our girl continuing to conquer challenges head on like she always has, but I hope to also share more normal accomplishments. Like more photos of our strolls out to the patio!

As always, thanks for checking in. I hope all is well with you and yours.   

Between blog posts if you would like to check in on Avelyn's journey please follow my Instagram. 

If you choose to share this or any of my previous posts please do! But I ask that you do not alter my writings in any way and provide a link to the shared material. If you have any questions about this request, please ask me.
Thank you so much! 

An array of photos from the last couple weeks are displayed below chronologically. She's cute, enjoy!