Heart Day: 6 Years!!

Poppy’s cath back in December turned out to be an overwhelming blessing in every way!! Not only was the cath doc totally in our corner…affirming our gut feeling check everything thoroughly..her heart looked BEAUTIFUL!!! No evidence of CAD or rejection!!! PRAISE THE LORD!!!! Coming out of anesthesia was by far one of the roughest, but now we know for next time, that her body much prefers fentanyl to the presidex! 😉 We got to visit with Dr. Kahoud for a while during her recovery, which was a gift.  He helped take care of Poppy at Seattle Children’s, and his presence in the PICU here has been a sweet relief…a familiar face from the early days of this journey…someone who knows where we’ve come from…God is so gracious.

 

It seemed to take another month for Poppy’s heart failure symptoms to subside, but they pretty much did…thank you so much for praying!!!  She continues to have sudden random bouts of screaming pain and vomiting.  At times it seems they coincide with a storm, but not every time.  We are convinced one of her shunts is beginning to fail/ failing intermittently, but it’s really hard to say.  Most people with hydrocephalus can go in for imaging to check for shunt failure (enlarged ventricles/increased fluid/pressure on the brain), but since Poppy’s had so many revisions/been accessed so many times, her ventricles have a lot of scar tissue, which means they don’t expand much when there is added fluid/pressure on the brain, making imaging a poor diagnostic tool for her.  The only way to truly know her pressures is by putting in a ‘bolt’/intracranial pressure monitor, which is super invasive…and since she’s immunosuppressed, she is more prone to infection…so we wait until her symptoms persist over a couple days…ultimately we are praying these symptoms would completely lift!! She is so tough and has such a high pain tolerance..it’s so hard to see her in such pain…if even brief..

In February, it became clear that Poppy’s kidneys were starting to struggle.  Her BUN and Creatinine had both doubled and remained consistently high for over a month.  Since she is on the Cyclosporine for immunosuppression, which is metabolized through the kidneys, it is often the culprit when it comes to kidney problems in transplant patients. Since it is one of the only anti-rejection meds she tolerates, we just prayed.  What do we do? We consulted with Nephrology…  Poppy was already getting plenty of fluid/day (2L to be exact)…is it 6 years of cyclosporine use finally taking it’s toll? Is this the beginning of kidney failure? The only other thing it could possibly be was her diet.  She has been on the modified atkins/keto diet since last June in hopes of weaning down her seizure meds, praying it would help her talk and encourage development…(which it TOTALLY DID!! Girl is halfway off of Keppra and has seen tremendous gains with her verbalizations, processing time, and attention!!! Praise the Lord!!! 🙂

Nephrology said that we should try adding back a few carbs, and we would re-check labs in a week.  If there was no improvement, she would have to go in for a kidney biopsy to check for cyclosporine toxicity.  After just a few days of adding back a few carbs/less protein, her BUN and Creatinine came back PERFECT!!!! Bittersweetness…praising the Lord for His protection over her kidneys… and at the same time, remaining on our knees, desperately praying that the vast improvements we had seen since starting the modified atkins diet would not be hindered.

Kidney health trumps brain health …it HAS to be this way for her…we are REALLY trying to avoid a kidney transplant someday (fairly common in transplant patients, as the transplant anti rejection meds are very hard on the kidneys)…she NEEDS the cyclosporine for the rest of her life…kidney health takes priority over brain health… 😦 I don’t like it..and yet, we just keep praying and trying the next thing…

It has been a couple of months since Poppy has been off the diet, and while we do see a difference in her attention and overall demeanor when she has had carbs (don’t we all..;), her verbalizations and processing time remain!!! Hallelujah!! What an experience..what a grace..all of it.  I’m so thankful for the Lord’s insight to even just try the diet…come to find out, sugar and excess carbs really do impact our brain function and overall brain health!!!

Poppy had her annual transplant appointments earlier this month, and everything came back GLORIOUS!!!! Her heart is doing amazing!! Her growth remains on the curve (barely, but we’ll take it! She’s finally over 40lbs and her bone age is right on with her age!!) Endocrine was thrilled! Dermatology was happy! Nephrology was happy! Even Infectious Disease was happy! Her heart, kidney, and liver labs, her antibodies, she is still CMV negative and EBV negative…ALL OF IT came back AMAZING!! Thank you, Jesus!!! Not super typical to have such a report 6 years post transplant…thank you Lord!!! Her next cath to check for CAD will be over Christmas break again, unless concerns arise before then. We will continue to do monthly labs and see transplant every 3 months for an echo and visit.

While we miss ALL of our precious people at Seattle Children’s, God has totally provided here at Mayo.  I don’t know that we are everyone’s favorite…we ask lots of questions and hold closely to how we were trained to care for Poppy (transplant/hydrocephalus, etc)…by a different team with a different protocol that is comfortable and safe to us, but Mayo has been overall very receptive and accommodating…I think we could be growing on them? 😉 Overall it’s been really incredible to see how the Lord has used this season of change and challenge and blessing for His glory.  He really has…though we miss our family and providers and the NW desperately, He has made the time here beyond blessed in so many ways…

Poppy continues to LOVE music, dancing, books, swinging, biking, jumping..she’s pretty fun…except for when she’s hungry… 😉 She is OVERJOYED to be able to have chips again 😉 She continues to eat ALL of her calories by mouth, and receive her meds and fluids by g-tube.  Since she gets 2L of fluids/day in order to keep her kidneys happy, and her meds 2x/day, I am relieved she still has her tube, so I can insure she’s getting what she needs.

When we lived in MT, our vision resource gal from the MT School for the Deaf and Blind had brought up the possibility of a Cortical Visual Impairment http://littlebearsees.org/what-is-cvi/, as it can occur with brain injury (strokes, bleeds, ECMO, bypass). It’s always been in the back of our minds, and as the years progress, it has become more apparent that CVI is the case when it comes to Poppy’s vision.  Modifying everything we do to support her vision when homeschooling, in therapies, as well as in daily life is something I am still learning SO much about!! Poppy’s vision teacher and other homeschooling mom friends who are also accommodating for CVI have been a wealth of information and support! Though it’s indeed a challenge that I oftentimes feel totally ill-equipped to take on…He never fails….just look at how He provides what we need..the Lord takes such good care of us ❤️

Poppy’s mobility is strengthening!! She continues to crawl and use furniture and walls to get around the house, her walker for shorter distances, and her wheelchair for longer distances.  We got a tray that fits her wheelchair, and it’s such a blessing especially for the homeschool co-op we attend!! It has been such a gift to see the sweetness of her classmates come along side of her and love on her..cheer her on..she’s just another kid in class and she LOVES that!!!  ❤️ God is so good ❤️

 

As we prepare for Poppy’s 6th Heart Day celebration, we reflect on the last 6 years…grief and awe…tremendous joy and deep aching…the daily challenges and long awaited victories…it’s ALL so good because He is good….He doesn’t waste any of it..it’s ALL for His glory..

Grace up on grace…He has allowed us this precious gift of time with our girl…we don’t deserve it any more than the next parent, and yet here we are.  Her donor family is missing a part of their family…so many in Poppy’s cohort are missing a member of their families..we are thankful…life is such a gift…there just aren’t words to describe the feeling of grace and mercy we have been given by getting to watch our girl grow up with us..

There isn’t a day that goes by that we don’t pray for total and complete healing..for her to have the freedom to express herself verbally…to have the strength and ability to get up and run around with her siblings..no doubt in heaven someday..but God is using it ALL…the joys…the challenges..the day to day of being a family just trying to do our best for our kids…and falling short, just like everyone else….He knows and He is perfect..and  He uses the hard things to draw us to Him..to find our rest in Him. Hallelujah.

Happy Heart Day #6 sweet girl!!! You are SO loved!!! ❤️

 

^^ Just got the news: Poppy is getting a heart!!!

^^Getting ready to be rolled into the OR to get her new heart!!

^^ New heart!!! ❤️