make it happen.

To our surprise, it happened one day. I gave the good news to Josh on Christmas Eve 2006. He was shocked.

The pregnancy was moving along fine without any complications. However, on Monday, April 2nd, 2007, we went for our 20 week ultrasound and found out that we were having a baby girl. On Wednesday, April 4th, we found out that she has a high-risk birth defect called Congenital Diaphragmatic Hernia (CDH). God has used this time to stretch us and test our faith, but we are holding tight to His promises.

Jeremiah 29:11 “For I know the plans I have for you,” declares the LORD, “plans to prosper you and not to harm you, plans to give you hope and a future.”

The best set of doctors in Denver ended up giving our baby girl a 20% chance of survival, with no hope concerning her quality of life. Another set of doctors indicated that there was only a few weeks left to terminate the pregnancy.

After extensive research, we picked Dr. David Kays at Shands at the University of Florida, located in Gainesville, Florida to be the surgeon for our daughter upon birth. His overall survival rate for babies born with CDH is over 90%, the highest documented survival rate in the country. Based on the severity of her defect, he gave her a 70-75% chance of survival with a good quality of life.

This meant temporarily relocating to Florida from our home in Lone Tree, Colorado. With an LHR (lung-to-head ratio) of 0.8 (severe category) and all the abdominal contents in her chest cavity including her liver, we were told to expect that Ella would be in the NICU for 8-12 weeks after birth.

Within 48 hours of landing in Florida, Ella Renae West was unexpectedly born on July 24, 2007 at 7:06am at 5 lbs, 6 ounces and 19 inches long. She was approximately 3 weeks early.

Once she was born, her fight for life began. Literally every day seemed to have challenges and we were unsure if she would live. Her road to recovery was bumpy and long.

Just two days after she was born, she had her first surgery and was placed on VA ECMO (Venoarterial Extracorporeal Membrane Oxygenation). On the eighth day of ECMO, they noticed that she was twitching. After an EEG was performed, the results showed that Ella was having seizures that indicated brain damage. After approximately 3 weeks of ECMO and a few days of recovery, Dr. Kays operated and did her repair surgery on August 20, 2007.

Shortly after repair surgery, Ella became swollen and puffy. Upon investigation, they noted that her superior vena cava had collapsed and diagnosed her with SVC Syndrome. They tried ballooning the vein to open it up and the procedure was not successful. Her chest cavity was filling with more and more fluid each day. The body was not able to absorb the fluid on its own.

Along with SVC syndrome, Ella was diagnosed with Chylothorax. They placed her on a special medicine called Octreotide and placed chest tubes in her sides. At one point, she was outputting 900ccs of fluid from the chest tubes in a 24 hour period. There were 13 drips with medicine, nutrition, sedation and fluids attached to her body and running through her pic line and UAC line in her belly.

After 10 weeks of fluid issues that were not resolving, Dr. Kays ordered a CT scan. Ella ended up having 5 chest tubes in at one time (some were adult sized), including 2 placed in her back. Finally on October 17, 2007 (Day 86), Ella was chest tube free! This meant that one day later on October 18, 2007 (Day 87), we were allowed to hold Ella for the first time!

On October 23, 2007 (Day 92) at 9:15am, Ella was extubated for the first time. She was 13 weeks old when we first heard her cry. They placed her on CPAP (Continuous Positive Airway Pressure).

On November 2, 2007 (Day 102), they tried nose cannulas with oxygen flow and Ella was ventilator free for the first time! Unfortunately, in less than 24 hours, she was placed back on CPAP. Her little lungs were just not strong enough.

Twelve days later on November 14, 2007 (Day 114), they decided to let Ella try “sprinting”, where they have her on CPAP for 6 hours and vent-free on nose cannulas for 6 hours. This lasted for 4 days and Ella started to have a tough time breathing. Within 5 days on November 19, 2007 (Day 119), she was placed back on CPAP full time. The next day on November 20, 2007 (Day 120), Ella was re-intubated and placed back on a ventilator.

Each time they tried to move her to the next step, her lungs would collapse. It would take days and weeks to get her strong enough again. We were not sure what it was going to take for Ella’s lungs to recover. Admittedly, she was a mystery to Dr. Kays too.

At this time, she had worked up to full feeds of Portagen via an NJ tube and Dr. Kays felt that she had a bad case of reflux. An upper GI test confirmed that Ella had moderate to severe reflux and was likely aspirating small amounts of food into her lungs. This was his theory as to why her lungs were not healing and getting better.

Since she was already intubated, Dr. Kays decided to proceed with another surgery: a nissen and g-tube on November 21, 2007 (Day 121). Ella spent her first Thanksgiving without any food due to her surgery!

After this surgery, Ella seemed to be improving each day. Although she was still in need of the level 3 NICU, they were running out of room. So on Monday, December 10, 2007 (Day 140), they moved her down to the level 2 NICU (not as intensive of care). Two days later on December 12, 2007 (Day 142), they decided to try Ella vent-free on nose cannulas with a flow of 500ml.

Although it was difficult, we made another big decision on December 13, 2007 (Day 143). We felt that it would be the best to commit to staying in Gainesville for several more months. At this time, we had no idea how much longer Ella would be in the hospital.

When we started to think about the consequences of going home to Denver, there were not a lot of things that made sense. First, it was winter and not a good time to travel or transition Ella while it was bitter cold. Second, she was having such a rough time during this hospital stay and we were unsure about the altitude and what effect that would have on her fragile lungs. Third, we needed more time to find good doctors in Denver. Staying close by Dr. Kays and the team that helped her to this point seemed to make a lot of sense. Plus, Dr. Kays had a pediatrician to refer us to during our short duration. Dr. Kathy Sarantos was not only an excellent physician, but she had a daughter with CDH too. Once Josh received permission to continue telecommuting from his employer, it seemed to be the best decision for Ella. Selfishly, we wanted to go home but knew in our hearts that it was best to stay put for however long it felt right.

Surprisingly, the week of December 17, 2007 (Day 147) was the first time that discharge was even mentioned (a new nurse practitioner took over Ella’s case and she was motivated to get her home). In preparation, they gave her a blood transfusion to increase her hematocrit (the proportion of blood volume that makes up red blood cells), switched her diuretics from her central line to giving it to her via her g-tube, her first set of vaccinations, an MRI, a follow-up echo, etc.

By the end of the week, Ella was not doing well. On December 22, 2007 (Day 152), she had a fever and it was hard for her to breathe. She was having lots of episodes where she would hold her breath and turn blue/purple and was not consolable. Since there were so many changes, it was tough to distinguish the problem. To be safe, they ran a bunch of tests to start ruling things out: took a blood gas, 2 CBCs, chest xray, etc. It was the weekend and the holiday, so Dr, Kays was not in town nor the nurse practitioner that ordered all the changes. The nurse practitioner on shift decided to double her oxygen to 1 liter and added 2 more diuretics, just to maintain her stats.

Once Dr. Kays returned from the holidays, he was not happy that the diuretics were modified while he was out of town. Instead of discharge, the NICU staff began to focus on simply getting Ella back to where she was stable on 500ccs of oxygen.

By December 27, 2007 (Day 157), switching from IV diuretics was not going well. They kept trying this because her central line had been in place since November 21, 2007 and a CT scan on January 5, 2008 (Day 163) showed that her IVC (inferior vena cava) was partially occluded. Her SVC (superior vena cava) was already completely occluded, so having her IVC occluded was not a favorable situation. Elevated temperatures, heart rate higher, irritated and not consolable were some of her symptoms at this point. The best guess is that maybe she had a virus.

As we headed into 2008, it seemed that the two main challenges were finding a proper formula (she was on Portagen which is not a complete nutritional supplement for her age; not intended for long term use), and switching her diuretics from IV to oral without any compromise in her respiratory status. On January 7, 2008 (Day 165), Ella had an EEG done (Electroencephalography (EEG) is the measurement of electrical activity produced by the brain as recorded from electrodes placed on the scalp). They wanted to know how her brain was functioning considering the results of her recent MRI in December ’07. With both tests, they could give us a better idea of what it meant.

Ella did not feel well during the early weeks of January and they could not put their finger on it. By January 9, 2008 (Day 167), Ella was laboring to breathe and it seems like her switch of diuretics from IV to oral was not working. But on January 10, 2008 (Day 168), they finally decided to remove her central line. On the one hand, you wanted the central line to stay because receiving her diuretics this way seemed to help her so much. Yet, the CT scan showed that the IVC was partially occluded. Leaving it in longer was not going to help that situation.

Unfortunately by January 12, 2008 (Day 169), Ella hit a wall. When we called in the morning to check on her, she had a brand new nurse. We asked how she was doing and she told us: she was on 1 liter of oxygen with a heart rate of 207, saturations at 86. Immediately, we headed to the hospital and when we looked at her, we knew something was very wrong. Then, she had an episode: she couldn’t catch her breath and required blow by oxygen just to bring her back up and calm her. After spending one month in the NICU 2, she was transferred back to the highest level of intensive care in the NICU 3 and was up to 3 liters of oxygen to maintain her stats.

Once Ella spent 2 days back in the NICU 3, her oxygen requirement had gotten as low as 800ml. Although it seemed that the reason that she started to do better: they had given her an IV dose of Lasix. Once the IV dose of Lasix wore off 48 hours later, Ella began to have respiratory distress again and was requiring more oxygen to keep her saturations in a good place. Her diuretic needs were really quite a challenge to the medical staff. They had never seen such a phenomena before: the method of administering diuretics causing such drastic changes in respiratory status, even with an increase in dosage of diuretics.

On January 15th (Day 176), we received some tough news. The results of Ella’s echocardiogram from the week prior revealed that there were no signs of pulmonary hypertension. We thought that was a bit miraculous, but figured anything was possible on this journey. On this day, they informed us that she does have moderate to severe pulmonary hypertension.

Although this was not the best news, they started administering sildenafil (Viagra) to help her heart and lungs work better together and increase her pulse saturations. The medicine was supposed to take effect in 3-4 days before any improvement would be noticed. Also, they increased the Bumex to 1.0mg total and only administering it twice per day, at 11am and 11pm, when her tummy is less likely to be full (hoping this helps with absorption). Within 24 hours, Ella was responding like a champ!

By January 19th (Day 180), Ella had looked the best she had ever looked since birth! At this point, it finally seemed like they had discovered the right meds for our baby girl. Although there were lots of tests/discharge items to be complete, it looked like Ella might be getting a discharge date.

On January 25th (Day 186), one day after Ella turned 6 months old, we were discharged from Shands. They wanted to take blood labs before discharge, per Dr. Kays. When they went to draw blood, they were not able to get enough to detect all her levels and electrolytes. Rather than try sticking her again and getting her upset, they let us leave the building.

After the first couple of days at home, Ella started to display some signs of distress – she was having a hard time breathing, even at 500ccs of oxygen. We could tell because her saturations were in the low to mid 90s and her heart rate was between 155 and 180 while she was sleeping. When she left the NICU last week, her saturations were 100 and her resting heart rate was 115 and even lower. When you looked at her, you could see that her breathing was more labored. When we thought about it harder, it explained a lot of her fussy behavior the past 2 days. Something told us that things were not right and heading in a direction that could be bad. So on January 30th, 2008 (Day 191), we headed back to the hospital via the ER, as Dr. Kays requested.

We had a horrible experience re-entering into the hospital via the ER. And since Ella had been exposed to the outside world, we were not readmitted back in the NICU with the staff that knew Ella for the past 6 months. Instead, we were placed in a shared room on the pediatric floor. There was no lock down or security, so we literally had to stay at the hospital 24/7 with Ella. We were in the hospital setting for 9 more days. It took testing different formulas and increasing diuretics to get Ella in a place where she was strong enough to leave (ended up with Alimentum formula and doubling her diuretic to 2mg total of Bumex, 1.0mg twice per day). Thankfully on the afternoon of February 7th, 2008 (Day 199), we left the hospital.

Since that time, Ella has been progressing slowly but in the right direction. This blog is a record of our thoughts and experiences while going through pregnancy, birth, 6+ months in the NICU, our journey back home to Colorado, and the new challenges Ella has faced while being back in Colorado including hospital time, surgeries and additional medical complications. Each day is a challenge, but we are so thankful to have Ella Renae in our lives. She is a true gift from God.

Please feel free to post comments, or contact us directly if you have any questions or concerns. And above all else, if you or someone you know receives a diagnosis of CDH for their baby, just know that there is hope. Never give up.