Doctors, Families Rally for Cure for Deadly Birth Defect

Parent's Perspective News Report of Saint Louis Children's Hospital Press Conference

Wednesday, March 21, 2012

Face of CDH - Whitney Kaholumehekainani

Our journey started at week 21 gestation when my husband and I, along with our moms, went to the Dr. for the anatomy ultrasound. It’s a girl! We were beyond happy. Our moms left to tell everyone and then the Dr. talked to me and my husband. He said they see a mass in our baby’s chest or bright spot on her left lung; calling it Congenital Cystic Adnomatoid Malformation (CCAM). We were referred to a specialist, a perinatologist who confirmed it and told us all about it.
A month later, at week 25, we went back for another ultrasound and saw a different Dr. They saw fluid around the CCAM and referred us for an echocardiogram because her heart was pushed over to the left. The heart ultrasound saw that baby’s heart was developing normal and that the CCAM was pushing it. They say the CCAM is not fatal and that it would probably shrink in size and be just fine by birth.
At week 28 we had another ultrasound with different Perinatologist Dr. While doing the ultrasound, the technician saw something else in the picture so she asked for another one’s second opinion. They were being mysterious and wouldn’t say anything to us. I thought to myself that“as long as it wasn’t life-threatening it’d be okay.” They told us that we had to wait for the Dr. to come in and tell us. I remember this like it was yesterday. When the Dr. finally came in he studied the pictures and did his own ultrasound also not saying anything to us. In his office he told us it was right-sided Congenital Diaphragmatic Hernia, which is life-threatening. Not a CCAM at all. This Dr. explained it all very well to us while I cried the whole time, and I cried the rest of the day. :(
After seeing 5 different Dr.’s, only the 5thone finally gave us the true diagnosis. It’s a very rare birth defect and I was referred to the only hospital in the state that could care for us. My husband and I met with a ton of different doctors that would help care for our baby when she arrived. Of course I did tons of research on the diagnosis. The doctor’s and nurses had little hope; all the research had very little hope for a right-sided CDH baby, I almost felt hopeless. The neonatologist started talking about comfort care instead of all the scary, painful, and risky procedures to save my baby’s life. I was confused and scared. But this one time, when I went to a book store and picked up a book about infant loss, I suddenly put it back, crying, and decided that my baby would live and that I would do everything possible to help her. I had an MRI done that told us more about Whitney’s hernia. Basically, her liver and intestine were developing in the chest, so both lungs were small, one more so than the other and her heart was entirely to the left. I also got two steroid shots about 4 weeks before the birth to try to help her lungs grow. I had an Amniocentesis done to make sure there were no chromosomal abnormalities. I drove 30mins, both ways, twice a week for ultrasounds and Non-Stress Tests. I also bought a few “new baby” items to help me prepare for having a baby come home, although; they were basically telling us to buy a casket instead of a crib. I also prayed a lot. I mostly prayed for comfort and understanding and the courage to accept God’s will. This sort of thing is, understandably, extremely hard on first time parents.
When we picked Whitney’s middle name, we knew we wanted it to be Hawaiian. Thomas is part Hawaiian, so we were thinking family names. But they didn’t seem to fit. His dad told we could just make one up. So one day my husband asked me to tell him a meaning and he would research it. I said that Whitney is “Strong and Beautiful like the ocean.” Translated to Kaholumehehkainani.
I was induced at 40 weeks and after a few short hours of labor; Whitney was born on Wednesday, July 21, 2011 at 3:09PM, weighing 6lbs 10oz. I’m grateful I was able to have her vaginally and that my husband was able to cut the cord. And then they intubated her immediately. All I could do was look at the backs of several Doctors surrounding my newborn girl. I do remember one nurse watching me while I was sobbing. I wondered what she was thinking. I held Whitney’s hand for a few precious seconds before they took her away to the NICU. I continued to cry, so much that I couldn’t even talk. I wanted to tell Whitney to be strong and that I love her and that I need her. I wanted to ask her if she was okay. I was so scared for her.

I wasn’t able to see her for quite a few hours afterwards because I had gotten an epidural. My whole family was with me but I didn’t want them to see her before me. Eventually I did see her and so did our families. Even though my husband and I were exhausted, we took turns taking every one back one at a time because we didn’t know if Whit would make it through the night. From then on, it has been a roller coaster for everyone, especially Whitney. The next morning, I signed papers for Whitney to be put on the ECMO machine. My husband wasn’t there but I had already decided to do everything possible to save her life. That afternoon, before she was even one day old, they hooked her up to that heart/lung bypass machine. I was discharged but they let us stay in a special room for parents of critically ill babies. Whitney was able to be weaned off ECMO at six days old. The neonatologist told us“these babies have maybe a 20% chance to survive.” I whispered... So there’s a chance. Somehow, I just knew she would survive. On day seven they performed the CDH repair surgery. They repaired her right side with a large gortex patch. They said that they were confident that Whitney would heal very well. I always asked Whitney to promise me that she’d be “okay.” I know it was asking a lot of her and I feel bad about it, but anytime there was a big hurdle, I asked her. I don’t want to list all the details of every little medical thing but like I said previously, it’s a roller coaster, and not a fun one at that! I was always asking Whitney if she was okay. I called the NICU all the time and just asked if she was okay. I just needed her to be okay, and they always told me that she was “critical but stable.” I know that she was never alone and it helped to picture Heavenly Father holding her in his arms. 
I spent as much time as I could by her side. Holding her hands, singing to her, reading to her, praying with her, and crying with her. Thinking back on it I wish I would have spent even more time with her. But all the nurses there said that my husband and I and our family were there more often than any other parents. So after 2 weeks, they kicked us out of the room at the hospital. We lived 30 minutes away and that was way too far for us, but we didn’t qualify for the Ronald McDonald house. Luckily we had a good friend who lived nearby the Hospital. We stayed with her for the next few weeks until we were satisfied that Whitney would survive.
I held my daughter for the first time at 15 days old. The first time I heard her voice was when Whitney was 3 weeks and 2 days old when she was ex-tubated. The first time that I was certain she was going to be okay was when she could breathe on her own; she was off of oxygen, if only for a couple days. The first time I was able to feed her. Her first bath. All of these things were miracles. It took about 1 ½ months to move from the NICU to the Annex, where babies are just working on feedings and getting ready to go home! So, to make a really long story short, Whitney came home when she was about 2 months old, a total of 56 days in the hospital. She came home dependent on oxygen and a monitor. We made a huge banner and hung it on the house. A lot of family members were there to same this special moment with us. 

And a new adventure began! She struggled with eating for a really long time. At 5 months old she needed a feeding tube. That also has been a roller coaster ride with many ups and downs. Whitney was gagging, vomiting, and had oral aversions. Within Whitney’s first year of life she needed many tests and procedures. She is weaned of oxygen and is on Silenafil with a Cardiac Cath to balloon open her left pulmonary artery. She has a G-tube and is completely dependent to survive. She is pretty small for her age but is growing steadily! She is on prevacid and bethanechol for GERD. She’s needed x-rays, Modified Barium Swallow Studies, Upper GI’s, Endoscopey’s, echo’s, and synagis shots. She continues to make progress with feeding therapy, speech therapy, and occupational therapy. She graduated Physical therapy by one year of age! Even though Whitney has some special needs, she is a very smart and happy little girl! We are so grateful for her and thank God every day. I knew it wouldn’t be easy, but it is worth it!

We agree wholeheartedly! 

1 comment:

Rachel Starchman said...

Beautifully written Alicia! I love my Whitney K, I get to be her adopted Aunt. She is so amazing and strong. She is the smartest little girl I know. CDH babies hold a special place in my heart because of Whit, and CDH Mamas are like no other. They are strong, faithful, and just plain amazing. Alicia and Thomas are wonderful examples to all around them, their faith in Heavenly Father and the love that they surround Whitney with is awesome. I'm so proud to be Alicias best friend!!