Congenital Diaphragmatic Hernia Awareness

CDH Awareness Day 2011 copy

Ava at 30 minutes old. She was immediately put on a ventilator. It helped her breath. She didn’t take even one breath on her own.

On Ava’s second day of life her little body started doing "hick-up like" contractions. She was hooked up to a seizure machine to check for abnormal brain activity. We learned her brain was just fine. We were very relieved.

Pulmonary hypertension strikes these babies and it is SO difficult to control. Here is Ava at 9 days old. Her Hypertension was severe and she had to be on the Oscillating Ventilator and Nitric Oxide gas. This machine shook her entire body.

Pulmonary hypertension begins when tiny arteries in your lungs, called pulmonary arteries, and capillaries become narrowed, blocked or destroyed. This makes it harder for blood to flow through your lungs, which raises pressure within the arteries in your lungs. As the pressure builds, your heart's lower right chamber (right ventricle) must work harder to pump blood through your lungs, eventually causing your heart muscle to weaken and eventually fail completely.

Ava right before her surgery. Dr. Adzick made an horizontal 4 inch incision on her left side under her rib cage. Her stomach, large and small intestines, spleen, and part of her liver were take out of her left lung area and placed in their proper places in her belly. He then placed a Gortex patch to simulate a diaphragm. Surgery took two and a half hours. All was done in her NICU bed.

While our little newborn fought so hard at birth, we took the opportunity to be joyful over the normal, father and daughter similarities of their hands. "Find Joy in the Moment, no matter if it is hard."

Ava was 18 days old and still on the ventilator. Daddy was always close by watching over her. She was very aware of his presence at all times!

This was a wonderful day. I held my baby for the VERY FIRST time! She was 18 days old. We had to be very careful as she was STILL on the ventilator. Brian thought it was too soon but Taryn our nurse was quite sure Ava was ready. The emotions were quite overwhelming!

Brian held Ava for the first time at 22 days old. He was very nervous. She loved it! THAT was the start of her being Daddy's Little girl!

One of the very first time we saw Ava's eyes. What a Joyful day! Tears of joy...

We came in to the sun shining in our baby this day. She was still on the ventilator but was wide away. Doctor told us the real healing had finally begun and she was over the big hump. Praise God!

This birth defect played a large roll in our other children's lives as well. Lexis and Emeline had to be away from Brian and I for three months. It was SO tough. We were in Philly fighting for Ava's life while they were in Iowa with family. They sure are strong girls :)

Ava was on the ventilator for 29 days. The day she was extubated she was showing ALL the right signs that she was fully ready. My exact words and prayers at the time they took it out. "I believe. I believe. I believe." I knew God was there helping her. The VERY next day she was also taken off oxygen. She didn't need it! How GREAT is God?

After Ava came off the ventilator she had difficulties sucking and swallowing, thus causing feeding issues. She tried SO hard. She would suck but then become very tired and fall back to sleep. Her favorite thing to do was snuggle with me after eating.

The Ronald McDonald House was our Home away from Home. We would stay here for a total of 4 months. We met so many wonderful families along the way. Thank God for the RMH of Southern New Jersey.

Ava getting her very first breast milk bottle. FINALLY she was eating! The feeding battle just beginning...

She hated the scale. You can see here her repair scar on the left side. What a strong willed girl she is!

Ava looking great but still battling feeding issues at Children's Hospital of Philadelphia at 37 days old.

Removing the PICC line was a great day. However the other IV's took over... just a little longer Ava.

Brian and I had to take several class in order to bring Ava home. We had to take CPR and a NG feeding tube class on how to put it in her nose and take it out. We had to take over her medicines care while still in the NICU. Going home meant lots of hard work for Mommy and Daddy. We loved and welcomed the challenge!

Ava had to do the "car seat test" before we could leave CHOP. Funny thing was our trip home was 16 hours. I believe she passed it after 3 hours. THAT was a LONG drive home.

Dr. Adzick, Ava's surgeon from Children's Hospital of Philadelphia stops by her NICU bed to tell her good bye. We couldn't believe we were FINALLY after 47 days going home.

We left CHOP on October 16, 2006. We were finally bringing our newborn home. The girls were SO excited. We would take the trip home in two days. We had a full car and GRATEFUL hearts. What a Miracle we had been given!

FINALLY we were home with Lexis, Emeline and Ava. They were so happy to see us. It was very hard to hold back the tears. This was the first time meeting their baby sister. We had been away from them for 3 LONG months. The Joy we felt....

She has delays once being home. She didn’t crawl until 8 months She didn’t walk until 15 month. She loved being home and she wasn’t afraid of anything! This is one of my FAVORITE pictures of her taken at 8 months old. She was such a Miracle to us and all that knew her.

Ava did very well after discharge from the NICU. Then when she was 25 months our lives came crashing down yet again. On October 3, 2008 Ava became deathly ill. I saw it in her eyes. I took her to the doctor 3 times that day and no one listened to me. Ava's patch had failed and she was again hanging onto life. She was life flight to a bigger hospital. I couldn't believe my eyes as the helicopter lifted my baby up into the air.

The Gortex patch that saved Ava at birth had some how gotten digested in her small bowel causing not only for her to reherniate but caused a TOTAL bowel obstruction. After the 7 hour emergency surgery, Ava was hanging onto life.

About a week after her emergency surgery her bowels became badly infected. Her stomach had to be opened, kept open and drained. (Her stomach would remain open for 4 months.) Her body swelled to 3 times her size. She fought so hard. Almost impossible to see your child this way.

Ava got very depressed in the hospital. Her smile and zip for life was gone. I became very concerned and asked how I could take her home. The doctors told us they normally did not let a child go home like this but since they knew I could handle it all they decided to let us. I became Ava nurse. Ava was finally discharged from the hospital after 47 days. (Quite ironic since it was 47 days that we were also in the NICU.) She came home with an open abdomen, central line and many IV antibiotics and lots of medications.

Ava went through a total of SIX major abdominal surgeries and SEVEN minor surgeries. She's a fighter in every sense of the word. I find my strength from her. Oh how God has blessed us.

Ava's CDH has brought her many things. It has changed her. She is a very thankful, polite little girl who LOVES life. She enjoys to play with her sisters and be with her family. CDH is only a small part of our little girl. Her strength is AWE inspiring to all who meet her. We love you Ava!

I asked Ava one day to lift her shirt and show my Mother how great her tummy looked. She surprsied me by what she says next. "Grandma look at all my cracks!" She followed them with her finger and smiled big. I LOVE that!

We are so lucky Ava survived all her CDH battles. We learn to savor life and appreciate EVERY day that is given to us. This is a good reminder to all of us. I'm STILL amazed that she is alive today.

I look back on her CDH Journey and Marvel at How Great God has been to us. Many are not as fortunate. The technology has gotten better but still only half of all babies born with this birth defect survive.

AWARENESS is so important! It is key. I'm not sure CDH will ever be wiped out completely but I hope someday it will be considered rare. Today it is far too common in my opinion.

~Terri L. Helmick