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.

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