Thursday, August 28, 2014

Liv is Here!

So just when we think we’ve got our lives planned out, things change, plans are broken, and life gets crazy. The last couple days have definitely not gone the way we planned, but though God’s grace, everything has been as smooth as we could ask for.
We were planning to pack up our family and drive to Houston this past weekend so that Beth could finish her pregnancy & deliver Liv full term under the care of Texas Children’s Hospital (TCH). As we were packing on Friday evening, Beth’s water unexpectedly broke, and we drove to Woman’s Hospital in Baton Rouge to see what to do. Little did we know that Liv would be born the next day.

When we first arrived at Woman’s Hospital, we immediately asked if we could have Beth or Liv transported to TCH. After initially being told that Woman’s Hospital wouldn’t transport Beth or Liv (and after many, many phone calls), we finally were told that they could transport Beth by ambulance before she went into labor, or transport Liv by air after she was born. The catch was that TCH did not have any available room to accept Beth before she delivered Liv. Woman’s Hospital called TCH every hour through Friday night and Saturday morning to see if a bed would open up for Beth. Finally, on Saturday morning, we were told that a room had opened up at TCH! Just at that time, Beth started to go into labor and along with the doctors, we decided that no matter how badly we wanted to be at TCH for Liv’s birth, it was too risky to take the 4 ½ hr ambulance ride to Houston. This turned out to be an excellent decision, as Liv was born only 2 hrs later (quick labors at 36 weeks seem to be Beth’s “thing” – the twins were born at 36 weeks 4 days and her labor only lasted 4hrs).
(where Liv would have been born had we taken the ambulance ride)

Lively Mae Hembree; August 23, 2014 at 1:09pm; 4lb 1oz; 36 weeks 1 day

Liv is beautiful and is our pride and joy. There’s nothing that quite compares to the joy of seeing and holding our daughter for the first time. We are constantly reminded of how blessed we are to have Liv in our lives, and are overwhelmed by the joy of having a daughter and being able to see and touch her.

The TCH air-lift team is called the Kangaroo Crew. They came to the NICU at Woman’s Hospital to pick up Liv and transport her to TCH on Saturday night. They were the nicest, most caring group of people we have ever met. Every one of them were super understanding of what we were going through and went way out of their way to let us know everything that was happening and assure us that they were taking good care of Liv. They even called us at every step to let us know where Liv was and how she was doing.
(Kangaroo Crew transport to Texas Children’s Hospital)

We spent that Saturday night in the hospital in Baton Rouge, as Liv safely made her way to the cardiovascular ICU (CVICU) at TCH. Thanks to Beth’s quick and easy labor and delivery, she was able to be discharged from Woman’s Hospital on Sunday morning 19 hrs after having Liv. The only part of our original plan was that we drove to Houston on Sunday; everything else was completely out of our hands.
Because our plans didn’t work as we thought, we have had even more of a chance to appreciate how God takes care of us and provides for us at every step. It was awesome to have Liv in Baton Rouge because of the chance we had for family and friends to be close and to be able to see us after Liv’s birth. We are so very grateful for all of the help, encouragement & prayers that we received from everyone (especially so for those people who took care of Asher & Eli when we couldn’t). The boys even got to go to a birthday party on Liv’s birthday, haha.

 (Our boys having fun in the water)

Also, the medical community at Woman’s and TCH have been wonderful in taking care of us and Liv. There have been so many people already involved in her care, and each of them have been exceedingly kind and understanding of our situation.

(Just a few of the wonderful people that are taking care of Liv)
The road ahead is long and difficult. The road ahead is scary and uncertain. However, we have great joy in our daughter, and joy in the expectations of the plans God has for her. We know that God has a plan for her life and that this physical life is only transient.

(Beth holding Liv for the 1st time at 2 days old)

Sunday, August 17, 2014

Baby Liv

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.”
Psalm 139:14