Dear Family and Friends,

It’s been a long week of anxiously waiting for the Houston call.  We found out yesterday that Dr. Cass and his colleagues decided to move our case to the next level.  Our care coordinator, Debra, said there was mixed feedback from the team but did not have many details of how they came to the decision.  Debra called today to let me know that Dr. Cass will be submitting the petition to the Fetal Review Board this afternoon.  Our timeline for their decision is mid-to-end of next week.   I sure could use a glass of wine to help with this waiting game!

In the meantime, we are going to work on a family, ‘How to live in two different cities and keep our lives running as normal as possible’ plan.  Just in case. Todd and I are meeting next week with a Child-Life Specialist that was a part of Team Ava.  She remembers Jason and is going to counsel us on how to talk to him about his newest little brother and some of the changes that may lie ahead.  My heart is heavy for him. 

We are supposed to leave next Friday for our annual family reunion at the lake.  I hope everything works out so we can all go.  We rent the lake-side resort for the week that sits in the middle of the Ozark Mountains.  It’s absolutely beautiful and I’m in need of a big dose of family love. 

Much love to our family and friends that continue to support and pray for us.  I hope everyone has a wonderful weekend.  Happy Father’s Day to all the dads – especially the two most special ones in my life…My Todd and Grandpa Jack.

Jennifer

Todd and I got checked in with the Fetal and Maternal Center at TCH Friday morning. Everyone we were meeting with had reviewed JT’s records and even knew about Ava. It was nice to be able to start from where we left off in Dallas. Our list of appointments started with a comprehensive ultrasound. The nurse was very sensitive and sweet. She identified the left-sided hernia then started to do regular measurements of all of JT’s organs and cutest little body parts. After about 30 minutes she asked if our Dallas doctors had said anything about the right side. We told her the right side was identified as an eventration. She agreed but then measured a “good amount” of lung tissue on the right side. For the first time since we learned of JT’s CDH, I was hopeful. Hopeful that we will beat this horrible, terrible monster of a condition this time. Hopeful that JT will qualify for fetal surgery that holds a 100% survival rate at TCH. Hopeful, the way I felt when Ava had her boxing gloves on and was fighting for her life.

Then the radiologist came in to take a look. She’s the one who analyzes the pictures the nurse takes. One issue with ultrasounds is that the liver and lungs can have the same appearance on-screen. Unfortunately, the radiologist showed us a hole on the back right side of JT’s diaphragm. The lung tissue identified by the nurse was actually JT’s liver. Is there any lung tissue?, I thought, but could not find the courage to ask at that point. She called it a bi-lateral CDH. I knew from my own research that the most common were left-sided. Then there was a small percentage of right-sided hernias. I had never heard of a bi-lateral hernia before. My hope escaped from me before I could even grab it.

Our fetal MRI showed the same results. The next appointment was an echo of JT’s heart. We met the cardiologist after the scan and received some good news. JT’s heart looked normal and was functioning properly. However, his heart is being “pancaked” in between his stomach and intestines on the left side of JT’s chest and the liver on his right side. Even though JT’s heart is good now, survival depends on how much and how functional his lung tissue is after birth. One word that she used to describe the severity of his condition was dismal. I can’t get word out of my head now.

Our afternoon had been scheduled with consults with fetal surgery and the chief of maternal fetal medicine. The cardiologist let us know we would just be meeting one-on-one with Dr. Cass from fetal surgery. He turned the computer on and showed us images that had been taken throughout the day. Then came the statistics: JT’s lung volume is 11%. Severe is classified at 25% or less. Another measurement for CDH babies is the LHR (lung-to-head ratio). Severe is 1.0 or less. JT’s is zero. And at that moment I felt like that’s about the amount of hope I had left in my heart. Zero. We spoke to Dr. Cass from our hearts. We told him we wanted to try and do everything possible to help JT. He was very compassionate but also straightforward. We do not qualify for their fetal surgery because of the bi-lateral hernia. Their study parameters are only for left-sided diaphragmatic hernias. However, they can consider our case for an off protocol surgery. Dr. Cass is confident in the tracheal balloon when there is a portion of right lung available to grow. He’s only seen a few bi-lateral hernias and could not be certain the balloon surgery would work. We all decided to let Dr. Cass present JT’s condition to the rest of his team to get their expert opinion on whether or not to be considered for the balloon surgery. If they say, “yes”, then our case is presented to a non-biased panel at TCH for consideration. This is a decision Todd and I cannot do alone. We should know next week if they think we can move forward in Houston.

Our trip to Houston was not what we hoped, prayed or expected. Now that I have had a little time to let this all sink in I’m starting to feel hopeful again. I will never let it go completely. I just misplaced it for a while. I will be thankful and hopeful as we continue to take things day by day.

Thank you all for your love, support and prayers.

My mind is beginning to wrap around the reality of what’s happening.  I thought I was hugging Jason and Jackson as tight as I could  but the poor things can’t escape my embrace now.  I find myself rocking Jackson longer at bedtime and I’m having a harder time leaving him at school each day.  He’s waving bye-bye and I’m stuck at the door not wanting to go.

We received the amniocentesis and micro array analysis reports which showed all normal chromosomes and genetic makeup.  Nothing that connects JT to CDH.  The genetic nurse from Dr. Lo’s office explained that the technological advances available have yet to find a genetic link to CDH.  I agree, expect they have yet to find THE genetic link.  There has to be one but for now CDH is reported as a fetal anomaly.

I spoke with a nurse coordinator from Texas Children’s in Houston last week.  We are scheduled for a day long set of appointments this Friday.  JT will have another comprehensive ultrasound and MRI.  We will have consults with the pediatric surgeon and MFM and also be counseled on the fetal surgery itself.  The doctors will take measurements and estimate JT’s lung volume.  We are also meeting with a Child Life Specialist.  When the nurse first asked me if we would like to speak to one my heart starting breaking for Jason.  He’s so excited about JT.  When we told him we’re having another baby he responded, “O yeah, I want ninety nine hundred thousand more brothers and sisters.”  Jason kisses my tummy at night and already adds JT to our conversations.

The nurse gave us a brief description of what would happen if we qualify and decide to move forward with the tracheal occlusion surgery.  Sometime between 26-29 weeks the surgery would take place.  (I’m 22 weeks now).  While the balloon is in JT’s trachea I have to stay within 10 miles of the hospital.  I would go for weekly checkups on the progress of his lung development.  The balloon would be removed around 34 weeks.  We can choose to deliver at Texas Children’s or come home to Dallas and deliver with our team of doctors and surgeons here.

I keep thinking of Ava and how she fought so hard for her life.  Those awful, terrible feelings of helplessness and fear that I have put way back in my mind have crept back into my daily thoughts.    A dear friend told me yesterday to take things day by day.  “One foot in front of the other and what you need will be provided.”  I’m going to take that advice and keep praying, hoping and trusting that God will take care of us, especially little JT.

I want to thank our family and friends that have reached out to us with love and in prayer and support.  We are grateful and could not even think about going down this road without you.

Much love,

Jennifer

Details of the tracheal occlusion CDH study:

http://clinicaltrials.gov/ct2/show/NCT00881660

Details of a leading doctor in CDH research at Texas Children’s:

http://www.texaschildrens.org/About-Us/News/Dr–Ruano-joins-Texas-Children-s-Fetal-Center/

Dear Family and Friends,

As some of you know we’re expecting a baby boy, James Todd II, in early October! Jackson just turned one and has been such a blessing this past year. Jason is an awesome big brother and when we learned Jackson was going to be a big brother, too, we were overwhelmed with joy. I’ve learned this past year that I love every second of motherhood. Sleepless nights, continuous feedings, life-changing everything – bring it on. I love it. I knew I wanted another baby almost immediately (it took 9 months to convince Todd!).

When it came time for our 18 week sonogram my mom said as we walked into Dr. Lo’s office, “Let’s go see that diaphragm.” The same nurse who showed us Jackson’s diaphragm looked around for 30 minutes or so. JT was facing in so it was difficult getting good images. Eventually, she started taking pictures and letting us know what she was seeing. “Head circumference is good, he weighs a whopping 9 ounces, kidney’s are here.” A few more minutes went by and she turned off the monitor and said, “I’m concerned we have another diaphragmatic hernia.”

I burst into tears and thought I was having a horrible nightmare. We had a 1-2% chance of this happening again. It rarely happens and so far isolated CDH cases have not been found to be a genetic or chromosomal abnormality. I now think otherwise and had an amniocentesis last week to find out more chromosomal detail of little JT. (Ava had not other chromosomal abnormalities).

We were able to have a fetal MRI that same day. It was all too familiar. Same imaging center, same waiting room and same procedure as we did with Ava. Dr. Twickler consulted with us after she analyzed the images. She remembered us and Ava. Her expert opinion confirmed a left-sided CDH. JT’s bowels, stomach and partial liver have already herniated into his chest cavity. She saw “minimal to no lung” on the left side. She also saw an eventration of the liver on the right side. There’s not an identifiable hernia (or hole) but the diaphragm seems to be weak and bulging up. We left the appointment hopeless, confused and completely in shock.

Fast forward 2 weeks to today. Our Dallas team of doctors have consulted with a team of doctors from Texas Children’s Hospital in Houston. Texas Children’s has a specialized group of doctors and surgeons that concentrate in fetal care and treatment plans specifically on CDH babies. We met with our Dallas team today and were presented with several options. Houston wants another MRI done at their hospital in 2-4 weeks to evaluate JT”s potential lung growth. If they estimate that JT’s lungs will be developed at a rate of 25% or less we would qualify for their CDH in utero surgery, a tracheal occlusion. They only perform this surgery on the most severe cases. Houston is 1 of 3 hospitals in the US that is FDA approved for this procedure. They’ve performed it 6 times in Houston but the surgeon leading this team has done over 60. Four of those babies went home with mom and dad and the other two are recent births and still in the hospital. I like those odds. The surgery would be performed at 26-28 weeks. A balloon would be placed in JT”s throat in order to block fluid from leaving his lungs. (I breathe for JT now until birth while his lungs grow). The fluid helps expand the lungs at a more rapid rate helping fight for space taken up by the herniated organs. Another surgery will be performed to remove the balloon before birth around 36 weeks. The tracheal occlusion surgery is one option, only if we qualify. (We also have to have normal results from the amnio because this study is only for isolated CDH cases).

With or without the tracheal occlusion surgery we have the option to deliver in Dallas with Team Ava. Depending on Houston’s MRI results we can choose an aggressive treatment plan as we did with Ava or we can choose comfort care. I’m fairly certain we have some of the biggest decisions we’ll ever make in our lives ahead of us. I’m praying for clarity and peace.

We will keep everyone updated on JT’s progess as we embark on a new CDH journey.

Much love,

Jennifer