Wednesday, September 21, 2016

Worries and Hope

As most of you know, in the last few months Avelyn has battled complication after complication, including 2 bouts of sepsis, more than a week of anaphylaxis symptoms, NEC, extreme hypertriglyceridemia, hyperkalemia and others. While much of these have now resolved, each took its toll on our little baby. She is only just regaining her strength, and continues to battle renal failure, and now- hypertriglyceridemia, on top of her cardiac issues. 

This week Avelyn’s triglycerides are nearly as high as they ever have been. We continue to believe the cause is “multifactorial” and centers around her absorbing high levels of carbohydrates from her dialysis, being in a constant state of stress (acute illness and constant withdrawal from sedatives), over fortified feeds, and insulin insensitivity. However, she is also being tested for potential autoimmune disorders and hereditary diseases that cause similarly high triglyceride levels (though, we do not think this these are likely). Her records have been sent to lipidologists all over the country to try and figure out the best plan of action for treatment. At current levels she is at risk for pancreatitis, and I maintain that I don’t know if she could survive pancreatitis. At the very least, it would be yet another complication that would wreak its own havoc upon poor little Avelyn’s systems. 

To combat the risks of pancreatitis, and eventually atherosclerosis, her feeds have been changed to skimmed breastmilk and a feed fortifier containing medium chain triglycerides (which are utilized more readily by the body).  She was also started on flax seed oil the other day, due to her likely having anaphylaxis to the fish oil a few weeks back. We’ve been told that the omega 3’s may lower her triglyceride level 15-20%, although there’s no guarantee in a child so young and complicated. With time and improvement of her overall condition, we hope to see a gradual decrease. This will likely be a problem that takes years to resolve. As long as she is on dialysis, lipid specialists believe she’ll likely battle some degree of hypertriglyceridemia, and if she needs a kidney transplant the immunosuppressants will also place her at risk. 
Otherwise, Avelyn has been dealing with the effects of methadone weans and continued recovery/treatment from sepsis. She is still on IV vancomycin and will be for another week or so, it makes her stomach upset  and has been contributing to continuous loose stools. The methadone weans (and new feeds) aren’t helping on that front either. 
The methadone weans often make her feel cruddy in general. She is being weaned very slowly, at half the usual rate, due to her overall fragility.  While we feel this is necessary for her safety, it prolongs the process which is frustrating for us all. We hate to see our baby uncomfortable and struggling, as the weans cause frequent gagging, diarrhea, and likely cramps, headache, and/or body aches (because she seems to respond well to Tylenol). She is currently being weaned twice a week- Mondays and Thursdays- and it takes a couple days after each wean to see her feeling like herself but then it’s time for the next wean. Unfortunately it’ll be like this for a few months. She is on a very high dose of methadone due to her lengthy intubation and multiple surgeries. Although, this has been very difficult for our sweet baby to tolerate and we hate seeing her struggle, each wean is also a blessing. 
Avelyn has lived her whole life, with the exception of a few weeks, on sedatives. Not only do we not truly know our baby, as we haven’t seen her personality not effected by substances in so long, but Avelyn does not know life without mind altering substances. She so deserves to experience the world and life without feeling the pain and discomfort of withdrawals or the effects of opiates, benzos, precedex, or clonidine. Although we are still months out, I cannot wait to have my baby free of these drugs and their effects. 
But anyway, back to current happenings.
 Like I mentioned above, Avelyn is currently doing better than she has in a long time. Though still on dialysis, oxygen, and other support, she is stable. Therefore the doctors believe it is time for a heart catheterization to optimize her cardiac function. This heart cath has been needed since April but we have avoided it due to her poor renal function. We are blessed in that her cardiac function has not declined. Amazingly, her pulmonary arteries have actually showed independent growth in the last few months, which is a small miracle. However, despite the proximal growth of her PAs, distally she still has narrowings that are causing elevated pressures in her right ventricle. Over time, these narrowings are likely to get worse and eventually overwork the right side of her heart leading to congestive heart failure. It is believed that if the PAs can be ballooned (or less preferably stented) now during babyhood, when bodily growth is most rapid, that fewer interventions will be needed. 
Expanding her PAs will not only protect her heart, it’ll also protect her lungs and help Avelyn in the gas exchange process. Right now her left sided pulmonary artery is small and receives little blood flow. Her right lung gets much more and handles most of her gas exchange needs. While this is not currently causing respiratory distress, overtime the over circulation to the right lung will damage it. 
However, opening up her left PA is not as simple as it sounds. When that left lung starts to get blood flow it’s not accustomed to, it’ll swell (which is called a reperfusion injury) and cause that lung to be quite sick in the short term (days/weeks) because of this Avelyn may need to stay intubated for a time. We don’t know how her lungs will react to PA expanding, especially given her lengthy intubation history. We hope she handles it as well as possible, and are praying she doesn’t need to be intubated long. 
In the long run, we’re hoping that the cath lab interventions will allow her to come off of oxygen completely, but will not push it if that’s not the case yet. Taking her home on a whiff of oxygen is the least of our worries, but one less tether attached to our sweet girl would surely be amazing. 
Another major worry concerning the cath is how the dye will affect her already sick kidneys. She has recently been peeing more than she has in months, although it’s still only about an eighth of what she needs to sustain herself without dialysis. Still, this is a victory in our book and we’re not at all looking forward to another step back in the kidney department. Please join us in praying for the dye to not further damage her kidneys in the long run. 
Lastly, there’s all the usual risks of a hearth cath. Vessel perforation, irritation of her heart’s conduction system, bleeding, infection and other things. All of which are serious risks, that in and of themselves are terrifying, let alone with all of Avelyn’s specific elevated risks. 
Please keep her in your thoughts Friday. Her procedure is expected to take 5-6 hours. I promise to update (likely via my Instagram account) as soon as I can. 
Thank you all for checking in and supporting our baby girl. She continues to fight with all of her might and amaze all who come in contact with her. We are so blessed to be her parents and to have your all’s love, support and prayers.














5 comments:

  1. Thank you again baby girl for explaining in such detail that even I can grasp most of our girls situation. Harrowing as it is, I continue to pray for a miracle. I know God loves our little Wonder Girl, after all He created her just for us ❤️ We all know of her strength & tenacity, but I will continue to pray for Wisdom & Guidance for the Team and for You & Sean. She has brought more love & joy into the lives of all who know her as we witness her continue to beat the odds time & again. She's still simply Amazing and I carry so much love for this tiny Warrior. I want to be more like her ❤️😘 Father please keep Avie cradled in your loving care and give us peace & comfort knowing that you hover near her keeping her safe. Thank you for the Blessing of our precious girl and for continued Tender Mercies 🙏🙏❤️❤️

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  2. Heavenly Father, please keep this little one in your loving hands. Please guide her doctors and caregivers and give them the knowlege they need to bring her back to health. Please give her parents comfort and peace. In Jesus' Name, I ask you for this. Amen.

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  3. Lord Jesus we know Avie is in the palm of Your hand. Please restore her to complete health for the life she so richly deserves. We ask continued blessings & love to surround all of Your children as they care for, parent, grandparent, & pray for this beautiful girl. 😇 💞

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