Year in Review

I have put off writing about the last year of our lives because, I don’t think the words to accurately describe all we have been through exist. We started the journey hoping and praying that Avelyn would have one open heart surgery and a few heart cath’s.  That dream (or delusion) shattered when Avelyn’s surgeon discovered much more complicated anatomy than he was expecting.

Since that point we’ve been on a roller coaster from hell and have seen our child suffer unimaginably. For those that may not have followed her story closely, here’s a recap of what our Avelyn has endured:

• Central shunt and right pulmonary artery banding open heart surgery at 5 days old with a chancy post op stabilization complete with unstable blood pressures and high lactate levels.
• 2 episodes of lactic acidosis with levels in the mid-teens, one requiring re-intubation.
• Over circulation causing poor perfusion and feeding interolerance.
• Basal ganglia ischemic stroke.
• Diagnostic heart cath.
• Endocarditis and nearly fatal septic shock, requiring intubation and terrible saturation instability (desat’d into the 40’s with the slightest provocation for about a week.)
• Early unifocalization, right PAplasty, VSD revision, and tricuspid valve rebuilding. Avelyn’s surgery lasted nearly 20 hours and she coded on the table requiring CPR. She only survived due to a portable bypass machine call ECMO.  Avelyn sustained her kidney injury due to this surgery and all its complications.
• 4 days on ECMO, where she experienced 2 subdural bleeds, uncontrollable bleeding into her chest that required continuous blood infusions for the first day and also NEC. She also required an in room open “exploration” of her open chest to find and stop the source of bleeding.
• Chylous Effusions
• ECMO disconnection.
• Delayed chest closure.
• Another diagnostic heart cath that discovered surgically induced coarctation of the aorta, and tricuspid regurgitation requiring intervention-this cath also caused a peri-renal bleed further damaging her right kidney.
• Another open heart surgery to make her VSD smaller into a fenestration, correct her coarc (via thoracotomy), revise her tricuspid valve, and more PA work.
• Respiratory infection from resistant Enterobacter.
• Building fluid overload.
• Started dialysis via PD drain in her belly.
• Continued chylous effusions
• Bronchoscopy showing right lung compression due to a large hematoma.
• Hematoma evacuation via full sternal incision.
• Tenchoff placement for more permanent means of peritoneal dialysis.
• Leaking tenchoff catheter, preventing usage and causing near fatal fluid overload situation.
• Extreme pain and delirium due to long term usage of high dose opiates, benzos, and other narcotics.
• Diastolic dysfunction, maxed out ventilator settings-causing pneumothorax and other lung damage.
• Massive diuresis over the course of a week that completely changed her clinical picture.
• After 3 months, chylous effusions resolved and chest tube was pulled.
• Successful extubation on the first attempt after 4 months of being intubated.
• Completely weaned from HIGH doses of Ativan.
• Culture negative sepsis and near septic shock.
• A week of anaphylactic symptoms related to fish oil.
• NEC and translocation of bacteria causing sepsis/bacteremia.
• Weaned from precedex.
• Extreme hypertriglyceridemia (maxed out past 2700)
• NEC and translocation causing sepsis/bacteremia again- septic shock with tanked blood pressure and lactate level of 18.
• Rhino virus nearly causing respiratory arrest.
• Hyperkalemia of 8.9
• Severe dehydration causing medical emergency.
• Feeding intolerance issues.
• Norovirus causing terrible diarrhea and leading to UTI which translocated into the bloodstream causing sepsis. Again.
• Fluid overload and airway compromise requiring intubation.
• Bronch study with finds of trachio/bronchiomalacia
• Successful extubation

Currently she is working on feeds, narcotic weans, staying away from bacterial and viral infections, settling out on a stable dialysis regimen, GROWING and PLAYING.

After writing all of this out (from memory, so I likely missed quite a bit and definitely understated most all…) I am truly beside myself with of all my poor child has endured. At this point, I haven't processed what all has happened and can't while still in survival mode. Right now we focus on doing our best each day to be there for Avelyn fully and advocate for her as she needs us to. That takes all we have and encompasses our lives and minds.

I’ll try to post another year in review, from the emotional side of things soon.

Love to you all and a very, very, very Happy Holidays and Merry Christmas!

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**

 

      

Endurance

Endurance.

Before Avelyn was born, I prided myself on my endurance, but once again my child has shown me a different plane of strength and resiliency than I ever knew existed. This last month Avelyn has endured more strife, pain, and discomfort than most experience in a life time. Watching her suffer and struggle brought me to my knees in brokenness and desperation. My child has suffered more than anyone should ever and while some has been unavoidable, it breaks me to admit this last month’s wasn’t.

Avelyn was doing well (which I always say with an air of relevancy, well for her is still incredibly ill), when she was exposed to norovirus. Within 24 hours she was having bloody stools. At the time, we figured she was once again on the road to NEC (necrotizing enterocolitis-a potentially fatal intestinal infection what she has experienced 3x). We were at a loss, she had been doing so well with feeds and the team was finally being slower with her transition to the low fat-high protein formula (that she needs due to high triglyceride levels and protein loss from dialysis). That week things only declined. Avelyn spent the entire week vomiting, retching, and having mass amounts of diarrhea. At the time, tests had not yet revealed norovirus and it was believed she was presenting as a child with “short gut syndrome” would. I thought the team was pushing her too hard, too fast and begged (like actually begged) the feeding plan be slowed.

I hit a new low that week and continued to sink lower as Avelyn endured blow after blow. The nutritional losses from a week of extreme diarrhea and vomiting started to mount. Avelyn grew weaker than she has been in a very long time. Her color was terrible, she couldn’t hold her head up, she started having trouble breathing on her own, and she started to get wounds/skin tears all over her body. Then I noticed what I believed to be signs of an UTI. It took a couple days of me mentioning something was wrong with her urine for cultures to be ordered (Avelyn doesn’t pee much and therefore it’s hard to diagnose an UTI, but her low urine output also puts her at a much higher risk). Then it took a day before enough urine could be obtained for a culture, which was needed before antibiotics could be started. So by the time antibiotics were started, the UTI was raging and Avie was in a lot of pain (even with opiates on board). At this point she had just come off of antibiotics (from her last episode of bacteremia), prior to norovirus and restarting them was another major blow to her intestines.

So antibiotics were started but Avelyn still just looked awful. She spiked a pretty high fever and blood cultures were obtained, only coming back to show that the infection in her bladder had spread to her blood. Once again she was septic, I couldn’t believe it. Our poor little baby was once again fighting for her life. Throughout all of this time (now nearly a week out from norovirus) she continued to struggle from a respiratory standpoint. It was bad enough that I was calling in providers multiple times a day, and even cursing at some of our favorites (I’ve since apologized profusely). But in my heart I was terrified by how hard Avelyn was having to work to breathe. She looked awful and exhausted. Her nutrition status was crap and I just knew she did not have the caloric reserve to keep up the effort required to breathe.

I asked them to intubate her to secure an airway as her airway was swelling closed—because by this time she was also dealing with extreme fluid overload. That evening, after many tears, prayers and a few curses… a new diuresis plan was put in place and Avelyn rose to the challenge. She lost a kilogram of excess fluid in just a couple of days. Though this helped her breathing some, she still struggled significantly. Later that week it was decided that she needed a new central line and the only safe way to place it was to intubate her. Once intubated, the team felt it best that she have her airway evaluated by pulmonology and ENT. As much as it hurt and terrified us to send her back into the OR, we trust our Vanderbilt team (who in many ways have become like family), and we agreed.

Findings were not what we had hoped. Avelyn was diagnosed with significant tracheomalacia of the lower airways, especially the left side (given the unequal presentation I wonder if it’s more bronchomalacia, but maybe that’s just my own OCD..). The pulmonology team came in and told us, there was a chance that Avie would never again breathe on her own and that it would not be unlikely for her to require a tracheostomy and home vent. Given all that our sweet girl already endures (complex CHD, renal failure that will eventually require transplant, hypertriglyceridemia, GI issues, chronic failure to thrive, ect.) this is just not something Sean and I are willing to do to Avelyn. Because as parents of a chronically critically ill child, it is possible to reach a point of doing things ‘to your child’, rather than ‘for your child’ and Avelyn’s quality of life is always at the forefront of our minds (hence why this month has been so devastating). The last month has brought us so very close to this boundary and we viewed a trach as a tipping point.

However, as time when on-in true Avelyn colors- she started to recover faster than anyone anticipated. In a couple days the team felt she was ready for extubation, and again in true Avelyn colors, she actually did better breathing on her own than she did while being ventilated (per blood gases).

Also feeds were started back and have slowly been titrated up. The plan was to start her back on breastmilk, which she needs to repopulate her normal gut flora, and she how she did from a triglyceride standpoint. Each day the breastmilk rate was increased by 3 ml an hour while watching daily triglyceride labs, once her levels got to 1600 (16x normal, which is the level when pancreatitis becomes a very high risk) then the low fat-high protein formula would be very slowly added in to reach goal calories.

Miraculously each day breastmilk was increased and each day her triglycerides either went down or never changed. Today she reached goal feeds, with a triglyceride level of 520. We are all blown away and no one really has concrete answers as to why she is now tolerating a higher fat diet than she has in months, while maintaining a lower triglyceride level. Though we also still don’t have a concrete cause as to why her triglycerides ever became so out of control, I don’t doubt that all of our prayers are being heard.

I asked you all to pray for a birthday and Christmas miracle for our sweet Avelyn and I choose to believe that’s what we are witnessing. Our baby has been through so much and, though I have said it many times before, we hope to have finally found our road home. Thank you all for keeping her in your thoughts and prayers through such a difficult and trying time in our lives. I will never be able to repay you or the world for the kindness bestowed upon our family throughout this year, but, I plan to spend my life trying.

Be on the lookout for a super emotional first birthday post to come this next week!  

All my love to you all.

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**

             

Strength through Adversity

The last month has been one of Avelyn’s most difficult. In that time she nearly coded and very narrowly escaped septic shock due to bacteremia (suspected central line associated staff epi infection). During this acute period she suffered a lactate level peak of 18.7 and required 8 ml’s per kilo of bicarb, a blood transfusion and a ton of fluid- in addition to the antibiotics.

The next weekend she sustained a potassium level of 8.9 (which is insane) and started have dysrhythmias and bradycardia before the interventions to bring down the potassium in her blood took effect. The insanely high level was not the first time she had been hyperkalemic and Sean and I have raised concerns before due to poor maintenance of her potassium levels. Trouble arises due to her need for dialysis, as well as recent inconsistency in her feeds and fluid status related to acute illnesses. Since that time, I have nearly lost my temper due to the time it takes to receive her potassium results from the lab. The ICU instensivists have listened to my concerns and agreed, and hopefully begun processes to better care for all dialysis dependent patients in the future.

The next week’s challenge was rhino virus and another bacteremia scare. Avelyn’s first symptoms of rhino were coughing, running/stuffy nose but over the course of a few days things escalated to a dangerous level. The event that got her rapided back down to the ICU was her upper airway spasming closed. The next day this continued to happen, despite being on a ton of steroids. The medication to keep her upper airway open (inhaled racemic-epi) caused her lower airway to spasm closed. This essentially sent her into respiratory arrest. During the crisis we had a nurse that understood that if Sean and I are urgently insisting that Avelyn needed to see the attending than that means she really needed the attending. By the time the attending rushed in, Avie had already gone from purple to blue and was quickly graying. He bagged/CPAP’d her for half an hour- forcing her airway open with the positive pressure and albuterol. She was then placed on high flow respiratory support for a few days and her feeds were held once again (for fear of poor perfusion to her gut). Avie’s respiratory status took the better part of a week to improve, even with round the clock albuterol treatments. It’s no exaggeration when I say we nearly lost her to the common cold.

This last week’s challenge was an extension of rhino’s complications. Because she required such a high dose of steroids and for so long, her triglyceride levels soared to dangerous levels, placing her at high risk for pancreatitis. With such crazy high triglyceride levels causing hemolysis of her lab work, which rendered the results unusable,  and again placed her at high risk for electrolyte imbalances-which can be fatal considering her recent potassium issues. So…. Despite our reservations about feeding changes, given her recent history of NEC and intolerance, the risks that her high triglyceride levels were placing her at necessitated an immediate feeding change. Also complicating matters, Avelyn’s weight had been trending down (fluid losses from diarrhea related to antibiotics) and her heart rate had been trending up over the course of a couple days. I continuously brought up my concerns to staff and providers that she was growing too dry, but there seemed to be a lot of reluctance to give her IV fluid boluses. She finally was given a small fluid bolus that helped a tiny bit at the time. So in the late afternoon she was started on monogen, a feed that she was on over the spring while she battled chylothorax. The feeds were ramped up to full volume within two hours, and at the time I knew she was super dry and my biggest concern was getting fluid into her. Little did I know, her body was not tolerating the drastic change and within hours she started having absolutely terrible diarrhea. In that night she had more fluid lost in diarrhea than she got in feeds. I still feel terrible about it all. (On another note, I was able to talk the resident into leaving her IV fluids running for an extra four hours and I think that extra 80ml’s of IV fluid helped keep her from crashing in the long run.) I fell asleep around 1230 and woke at 0300 to see her heart rate in the 170’s (normal is 110-120’s) despite her being completely calm. I immediately got up and took her blood pressure, which was low and went to find help. Her nurse was busy at that given moment but came a few minutes later. Her nurse that night was great and listened to my concerns and immediately went to find the resident.  After telling the resident multiple times she needed to come off of dialysis (which was steadily pulling more and more fluid off), I just went and turned it off myself (I am sure the hospital staff loves parents like me……).  Long story short, Avelyn was rushed downstairs to the ICU and given a ton of fluid. Like half a kilo at least and she stabilized. In my panic of the situation, it helped that the attending on call was Avelyn’s primary intensivist and the fellow was the doc that once dug through the trash for her bloody diaper during a NEC incident.  So needless to say, both docs knew Avie well and I knew she was in particularly great hands.

The next day Sean and I met with Avelyn’s entire interdisciplinary team for a care conference. We discussed Avelyn’s care and goals, short term and long term. We were able to contribute input to the conversation and her feeding plan and we feel hopeful that, as long as no further complications arise, Avelyn can begin to find the road of recovery on this plan.

Aside from the new feeding plan, we discussed Avelyn’s long term goals and treatment. Which includes future needed surgeries and cath’s to get her hear t into the best shape for her eventual kidney transplant. A few months ago, I had requested that her records be sent out to Stanford which is home to the world’s best MAPCA’s surgeon. We have received word that he has agreed to take on Avelyn’s case and wants to see at Stanford for a diagnostic heart cath and likely open heart surgery in 6 months. Sean and I are grateful that the world’s best, is willing to work on Avelyn and believe he can help. But there are sooooo many reservations. First of all, Avelyn would not withstand a bypass run right now. She is just too too sick. In order for her to successfully come off bypass she would have to be fully optimized for this surgery. Meaning the next six months would have to include miraculous recovery and sustained stable nutrition. We don’t know if we can get Avelyn to that point.

There’s also the logistics and financial concerns of flying a child dependent on dialysis across the country for open heart surgery at a completely new facility and back. It’s all so truly and completely overwhelming to think about. We are waiting to hear back from the surgeon about a teleconference so we can ask our specific questions and express our concerns about everything. Hopefully we’ll know more then. Until then, we are concentrating on getting Avelyn as well as possible as soon as possible- so this incredible opportunity will continue to be on the table.

Once again, please continue to keep our beloved baby in your thoughts and prayers. Her perseverance and determination to stay with us inspires us on a daily basis. We know that if she can make it through all she’s been through, surely we can handle the logistical and financial concerns to ensure her the best possible opportunity at life.

As always, sending peace and love to each of you!


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**

 

As usual, we chose not to share photos or our sweet girl during the worst of times. Some things are just too hard, but I hope you all enjoy these. Our wonder girl grows cuter by the day! 😍

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.   

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