What a crazy couple months it’s been! We found out on January 10, 2014 that we are expecting our 3rd child! The next several months whizzed by as our anticipation grew into finding out if Asher and Eli (our twin 3yrs olds) would have a baby sister or brother. At our 20 week ultrasound, we were thrilled to find out we were having a daughter, and decided to name her Lively Mae Hembree!
The ultrasound tech at our 20 week apt said that she couldn’t see Liv’s heart as clearly as she would like to, and called in the doctor, who also couldn’t get a good view (despite many tries to get Liv to reposition). They referred us to the high risk doctors (Maternal Fetal) “just to be safe”. Because we saw many Maternal Fetal doctors during Beth’s pregnancy with the twins (who were perfectly healthy), this didn’t seem like a big deal to us. Three weeks later, on our 4th wedding anniversary, a follow-up appointment with the Maternal Fetal doctor revealed that “just in case” quickly turned into the worst news we’ve ever been given.
It took several more appointments and tests to figure out the full story, but we eventually came to understand that Liv has Hypoplastic Left Heart Syndrome (HLHS), a Cleft Lip and Palate, and a partial deletion of chromosome 22. In addition, she seems to be small, but it’s unclear whether that is related to any of her other conditions. HLHS means that the left side of her heart is too small and isn’t pumping as much blood (none in our case) as it should. It also turns out that of the various congenital heart defect that exist, HLHS is one of the worst. As we researched more about HLHS, we found that the “treatment” is very extreme and more of a patch than a repair. Liv will need to undergo 3 open heart surgeries (the 1st within her first week of life) within about 3 years to essentially re-pipe her heart so that the right side can do all of the work that was meant for the left side. She will spend significant time in the hospital / ICU and the list of potential complications and additional procedures longer than we ever thought possible. After all of the surgeries are complete, there is the chance for a reasonably “normal” life, but always looming is the high chance of heart failure (because the right side of your heart is not designed to carry the full load) and the need for a heart transplant somewhere down the road.
Having a cleft lip & palate and chromosome deletion only add to the complications and complexity. We continually see that adding any additional issues to HLHS causes the survival rate to plunge and the risk for potential complications to increase. The cleft lip & palate repair requires a couple surgeries, which would normally be pretty low key in comparison, except for the fact that any additional surgery on a child with HLHS is a pretty scary ordeal. Also, we’ll have to work with the surgeons to fit the cleft lip & palate surgeries in around the heart surgeries, which will be delayed to some extent.
It’s hard to determine how the partial Chromosome 22 deletion fits into the whole picture. Liv’s deletion actually overlaps two other “known” syndromes (DiGeorge syndrome and Distal 22 deletion). Deletions in this region are known to cause heart defects and cleft lip / palate issues; however, neither of these syndromes are linked to HLHS – it’s actually extremely rare to have HLHS and a Chromosome 22 deletion. So while the chromosome abnormality can bring a lot of other symptoms (immune deficiencies, developmental delays, etc.), it’s difficult to determine how many or how severe these will be. The only way to know is to check at birth and as she develops, and even then, it may be difficult to determine the full impact. The good news in all of this is that with so many ultrasounds, the doctors have very thoroughly checked for all sorts of other issues, and thus far have not been able to find much. She is small, which could be related to her chromosome abnormality, and could also create issues for the HLHS surgeries. So far her growth seems to be continuing and not slowing down much further, which is good news.
Because the medical treatment options are so severe (and the probabilities of survival are low), we considered for a long time the option of comfort care: choosing not to perform any surgeries and allow Liv to live out her short life without ever having to know the pain of surgery or other extreme medical treatment. After several weeks of prayer, discussion, and research, we’ve started to turn away from this option, in part due to the finality of this decision (without surgeries, there is a 100% chance that Liv will not live longer than about 10 days). We’re currently getting ready to go to Texas Children’s Hospital (TCH) in Houston (the closest hospital that performs all 3 surgeries for HLHS, and also happens to be #2 in the country for this type of treatment) so that Liv can be born and evaluated there. TCH is where we plan to have all 3 of Liv's heart surgeries, any other heart procedures, and her cleft surgeries. Deciding to do the HLHS surgeries is a huge decision, but it is only one of many that will present themselves over the next several months and even years, beginning the day she is born in September. We pray for strength and guidance for every single step of this journey.
The challenges with Liv’s physical body has given us more reason lately to dig into to our relationship with God and has challenged how we view life and our role and purpose as parents. We so easily focus on the fleeting physical attributes and milestones, that we sometimes miss the fact that our children are an incredible blessing from God. What truly matters for Liv (and Asher & Eli) is that their lives bring glory and joy to God, and our role as parents is to teach, nurture, and shepherd them in this manner. Our Liv is truly a blessing and we will praise Him for bring her into our lives.
“I praise you because I am fearfully and wonderfully made; your works are wonderful, I know that full well.”