Emma's Story....
Scott and I were so very excited in February when we found out that we were pregnant. After suffering a miscarriage in July 2009, we were anxiously awaiting that day when we would be able to get pregnant again. Like always, God is good all the time. He answered our prayers with a positive test in February. While we were very excited to be pregnant again, we were a little anxious about everything being ok. We decided not to tell the family until I was 13 weeks along. The only person we told was Stephanie. She was able to keep a BIG secret. Our doctor appointments went very well. The NT scan came back great and she was growing right on schedule.
We had a complete OB scan set up for May 18th. Of course, we were most excited about finding out the gender. My mom, Sharon, came up for the BIG EVENT. Well, little Miss Emma decided to be quite illusive and although they were pretty sure it was a little girl, they could not tell us 100%. They kept scanning her heart. This made me very nervous. At the conclusion of the ultrasound, the tech told us that we needed to go see Dr. Doeden. I knew something was wrong. I did not have a lot of time to think because, I was immediately taken to Dr. Doeden's office. She told us that they were seeing something wrong with the heart, and that she had scheduled us an appointment with Dr. Stanley, a perinatologist at Mercy. We jumped in the car and headed to Mercy. Within fifteen minutes we were having another ultrasound done. Dr. Stanley explained to us that our baby had a serious heart defect. He stated that he wanted a pediatric cardiologist, Dr. Ward, from Oklahoma Children's to verify what he thought he was seeing. The appointment would be in four weeks. Those were the longest four weeks of my life.
We met with Dr. Ward in June. Dr. Ward informed us that Emma has Hypoplastic Left Heart Syndrome (HLHS) (Click on the HLHS word to get a brief overview of HLHS) HLHS effects approximately 4 in 10,000 babies. Basically, Emma's left ventricle did not develop. We were devastated. Our precious little one had one of the most severe heart defects possible. Her chances were pretty good they said, about 85% of babies with HLHS survive. However, they could not tell me what her future would look like. Heart transplant?.....possible, mortality?....possible, developmental delays?..possible. Basically, we learned that this heart defect carried with it a lot of unknowns. We did learn that Oklahoma did not have a surgeon who could properly perform the surgery, so we would have to transfer to another location. Dr. Ward recommended Dallas to us.
We met with part of our Dallas team for the first time at the end of June. Scott and I were very impressed with the care we received while we were down there. They conducted a 2-hour fetal echo to look at Emma's heart. At one point, they finally got a good picture and Miss Emma put her hand directly on her heart. Little Stinker. We met with a pediatric cardiologist who confirmed the diagnosis of HLHS. Dr. Blalock also had some concerns with her foramen ovale. She told us that they would watch it because, it was not allowing a lot of blood flow.
In July, we went to Dallas again. Miss Emma is growing great and was moving around like crazy! We did find out why they were a little concerned with the foramen ovale...she does not necessarily have one...she has two little holes instead of one big one! They are calling this a restrictive atrial septum (RAS)....now for those of you who like to Google...lol....the info out there even though it is called RAS is normally referring to complete closure or highly restrictive...neither of which is Emma's issue. (or so we thought) This is not seen very often ( only about 6%) and they are a little confused on what exactly to do.... The bad news is that this increases her chances of major complications. She now only has about 20% chance of survival. The good news is that there is two little holes allowing blood flow. We also met with the OBGYN, Dr. Santiago-Munoz and had an ultrasound conducted.
In August we met with a few other pediatric cardiologists Dr. Ikemba and Dr. Lemler. They were very pleased with the fact that Emma's heart had not changed. In fact, Dr. Lemler said there was just something about her heart that made him think they would not have to rush her immediately to the Cath Lab. That was such encouraging news to Scott and I. He said that they would schedule a C-section. Emma's heart is one that could go either way, so they hope for the best and prepare for the worst. There will be a huge team of doctors in the and around the delivery room to make sure that Emma is stable once she arrives. We met again with the OBGYN. She set the day for Emma's C-section on October 4th. They are a little concerned that Emma is slightly behind on growth and has fluid in her brain ventricles that borders and sometimes tips the normal scale. Our high risk OBGYN in Oklahoma is not too concerned about this as there has been little to no change in several weeks. However, the OBGYN is Texas wanted to do an amnio to test for potential genetic abnormalities. We refused this test because it is very risky and could cause potential spontaneous abortion or pre-term labor. We also got to meet Dr. Forbess, the pediatric cardiothoracic surgeon. He was so nice and had very positive things to say about Emma and the plan to make her heart whole. He explained the 3-surgery step process that is the most ideal, and the always-possible heart transplant procedure that is in their back pocket if they need it.
Emma was born via c-section at Parkland Hospital in Dallas, Texas on October 4th, 2010. Everything went as planned. There were two operating rooms filled with about 40 doctors and nurses. I got to see the top half of my sweet little girl's face and then she was taken into the next room, intubated, and a line put in her umbilical cord with PGE running through it to keep her PDA open. It took the team 18 minutes to get Emma out of me and into the cath lab in Children's Medical Center. Emma was A LOT sicker than they originally thought. Her atrial septum was basically intact, so by the time they got her to the cath lab, her oxygen saturation levels were in the 40s and her blood gas was 7.1 (normal is 7.35-7.45). After a successful opening of the atrial septum, Emma was taken to the CVICU to await her next surgery.
Emma ended up needing a surgery on day 2 to restrict some of the blood flow going to her lungs because her body was not getting enough blood. Dr. Guleserian put pulmonary artery bands on to help reduce blood flow. She returned once again to the CVICU to heal before the Stage 1 Norwood. At this point, they attempted to extubate Emma in hopes of letting her lungs work for the first time on their own. Emma did not have enough stamina to do this, so it was decided that they would reinutubate her. This is when things got interesting. Emma did not appreciate being reintubated and crashed on us. The team worked on her and could not figure out what was wrong. They called us back, brought up ECMO, and called in the chaplain (a person you never want to see in an emergency). We were so scared we were going to loose her. The attending had already left for the day and was called back. Dr. Guleserian and the attending, Dr. Koch came back to the hospital and worked through everything that had happened. Dr. G finally asked the RT to suction Emma. When they did, they got a mucous plug the size of the end of my pinky out of her lungs. Emma stabilized, but definitely declared herself. None of her doctors or nurses had ever seen that big of a mucous plug come out of such a little baby. This happened two days before her scheduled Norwood, but Dr. Forbess decided to go ahead with the surgery since the crash was not heart related.
Emma went in for her Norwood on October 11th, 2010. The Norwood procedure went very well and Emma did not have any major complications during the initial recovery and post sternum closure. We were very encouraged by this and had high hopes that Emma's recovery would continue to be relatively smooth. Little did we know.....
They extubated Emma again on October 17th. Emma did well at first and then started having difficulty breathing again along with some heart arrhythmias. They tried to give her every chance to hang on, including trying cipap; however, Emma continued to weaken, so they reintubated her on the 18th. Things went bad quickly this time. Due to Emma's heart being under stress from how hard she was having to work to breathe, she went into atrial tachycardia. Basically, her heart was beating extremely fast and was "fluttering" instead of really pumping, so blood was not getting where it was supposed too. They had to do chest compressions and give her 6 doses of Amiodarone. She was not responding, so they brought up the ECMO machine and even had blood pumping through it. Our little girl was slipping away and fast. Praise God, the 6th dose of Amiodarone plus an esophageal pacer worked and Emma stabilized.
Our road after this incident continued to be very rocky. Emma had recurring problems with pleural/chylous effusions this caused an excess amount of fluid in her chest cavity. Obviously this created breathing issues, which resulted in chest tube after chest tube (she has had almost 30 as of 1/29/12). Since Emma's drainage was chylous, she had to be put on a fat free formula called Tolerex. Tolerex resembles lemonade and is an elemental formula. Emma was not able to tolerate high caloric densities of this formula and was not gaining weight. Her little body was using too much energy just to maintain its temperature, so on October 21st, Emma went into a "grow box." She was only 5lbs 50zs....almost a pound under her birth weight.
Over the next few months, Emma continued to stay in this endless cycle. On and off of feeds/TPN and lipids, not gaining weight, unable to extubate, large amounts of drainage from her chest cavity, thick and large amounts of secretions (she would plug in ET tube 3 more times), extreme GI issues including abdominal distension, and the development of kidney stones.
Due to Emma not making much progress, she was taken to the Cath lab on December 6th to assess her heart function. Her squeeze was good, but her left pulmonary artery was stenosed and the relaxation of her heart was poor, so they began the process of listing her for transplant. December also brought another RARE diagnosis for an infant: PLE (protein losing enteropathy). On January 4th, 2011 Emma was officially listed 1A for transplant.
On February 11th, they took Emma into the Cath Lab again to reevaluate her heart. Dr. Nugent was REALLY glad they went to the cath lab because Emma's left pulmonary artery was almost completely closed off. Her left lung was not getting very much blood flow. He was able to get in opened up to some degree, which actually allowed blood flow to go to her left lung. This was a turning point for Emma. This cath procedure along with a decision from one of the CVICU docs to stop holding feeds turned Emma's recovery around. Emma got stronger, gained weight, and stopped having effusions. The doctors were able to pull chest tubes and begin once again to wean her off of the vent.
On March 4th, 2011, Emma was successfully extubated FINALLY! After 5 months of being intubated Emma and her lungs were strong enough to handle being off of the vent. You can imagine the lovin' Emma received since she was not able to be held much while intubated. They extubated Emma to 20 liters of Vapatherm. It sounded like a jet engine shooting up Emma's nose, but it worked. We weaned slowly (like 1 liter every other day) in order to let Emma's lungs properly heal and catch up. As we weaned on Emma's oxygen, we moved her feeding tube from her duodenum to her stomach. Emma was also able to switch from Tolerex to breast milk.
A big milestone was reached in April when we got to move to the 8th floor (cardiac recovery floor). Emma was able to wean off of oxygen. We got to leave the hospital at the end of April and go to an apartment in Dallas. The team approached us the day we were leaving the hospital and informed us of how happy they were with Emma's progress. Since Emma was doing so well, they wanted to electively make Emma status 7 on the transplant list since it seemed clinically her heart was better. The plan was to bring Emma back to the hospital at the end of May, wean her off of Milrinone and assess her heart in the cath lab. We had to go back to the hospital because Emma was having 12 plus bloody diapers a day. We were readmitted into the hospital. GI determined that Emma had a milk protein allergy. They changed her to Neocate and it worked wonders for Emma.
Emma did great weaning off her Milrinone. The cath in May did show improvement in Emma's heart function. Dr. Nugent opened up her LPA more and proved once again that her pulmonary veins were not stenosed (her pulmonary veins have been questioned for a long time, they look stenosed via echo, but not in the cath...controversy...yes). The decision was to wait as long as possible, but to go ahead and leave her status 7 and do the Stage 2 bidirectional Glenn.
We went back to the apartment and concentrated on development and staying healthy. Dr. Forbess wanted Emma left on a small amount of oxygen until her Glenn. Emma grew great and looked great despite being such an old untouched sano shunt. We were really enjoying being out of the hospital and only had to go back a couple of times for slight fevers. Time neared for Emma's scheduled Glenn (end of August). In true Emma style, she got a raging eye infection and pneumonia a few days before her surgery date (how she got pneumonia we still don't know since we allowed no visitors a week before). After a hospital stay to treat the eye infection and pneumonia we were sent back to the apartment to wait. Emma was getting blue. We had to increase her oxygen up to 2 liters. She was set to have her Glenn on September 19th.
Emma had her Glenn on September 19th. The doctors were nervous about this procedure because Emma is essentially has one lung. The only hiccup Emma gave us prior to surgery was that her broviac line stopped working the day before her surgery (the only reason it was left in was for the surgery). The Glenn went smoothly and Emma only had to be on the bypass
for about 45 mins. Her recovery post Glenn was up and down. Emma ran a high fever and had a lot of secretions in her lungs that she could not cough up. She was able to be extubated that same day, but required a lot of support via vapatherm. After getting over the initial rough days, Emma did quite well. However, our old nemesis, chylous effusions came back. So, once again Emma was put on Tolerex. Emma conquered a big milestone. She went to the OR and had her nonworking broviac line removed as well as a chest tube put in without having to be intubated.
We moved to the 8th floor and continued to make good progress. Emma was chest tube free and only requiring a small amount of oxygen. We celebrated Emma's first birthday at the hospital then got to go back to the apartment on the 8th of October. The plan was for Emma to stay in Dallas for a couple of weeks and then go HOME, for the first time, to Oklahoma. Emma never listens to plans, and in less than 24 hours we were back in the hospital with an increase in breathing and oxygen. Emma had reaccumulated fluid. Emma had to have a chest tube placed and was back in the ICU. It was decided that since we were back in the hospital we would go ahead and look at her heart via the cath lab.
Emma went to the cath lab on October 25th. Dr. Nugent opened up her LPA some more, coiled some collaterals, and plugged a collateral that was bigger than her Superior Vena Cava. Dr. Nugent was very optimistic that these fixes would help with her pleural effusions and her lower saturation levels. They were able to extubate her quickly and she looked quite good. However, things started going south several hours later. Emma began running a high fever, swelling, and turning purple in her extremities. Scott and I were very concerned. That next night Emma got very sick. Her blood gas was 6.9, Co2 of >130 and O2 sat of 10. They intubated Emma and things looked like they would turn around for a few minutes....then Emma's sats dropped and would not come up. Emma was bagged for over 2 hours. They finally got an echo and Emma's heart was barely functioning. As a last ditch effort, they hooked Emma up to an oscillating vent (first Glenn they have ever done this too). You could not feel nor doplar pulses in any extremity. Emma had 6 liters of extra fluid on her and her blood pressure was too low to use diuretics. She had to go back on epi and dopa. They told us she would not live through the night. Scary! Emma was paralyzed and sedated for about a week. She slowly got better and her heart function returned to normal. After about a month, she was able to move up to the 8th floor again.
We moved to the 8th floor and basically were just trying to wean on oxygen and stop draining from our chest cavity. However, after only 24 hours on the 8th floor, Emma caught an undetectable virus, ran a high fever and ended up back in the CVICU intubated.
It took about another month to recover and Emma spent another Halloween, Thanksgiving, and Christmas in the hospital.
We are now back at the apartment in Dallas. Due to Emma’s rough course, we are moving to Dallas so we can be close to CMC. Emma is trying to get caught us on developmental issues. She sees PT, OT, and ST twice a week each. We are also seeing the cardiologist once a week to monitor labs and x-ray since Emma is still on a high dose of diuretics. Emma is on a very low oxygen setting. She is even able to come off of it for periods of time during the day. Our newest hurdle is at Emma’s last Echo at the end of January, Emma had a very mild decrease in heart function. We are hoping it improves soon.
Issues we are still dealing with/ needing to address:
1. Emma is dependent on her NG tube (might need g tube in the future)
2. Emma has eye muscle issues (getting better, but still there).
3. Developmental issues (Emma’s muscles and bones are weak from so much back time in the hospital).
4. Lung pressures (hopefully will be low so she can go Fontan route).
5. Dentist visits every 3 months for fluoride.
6. Kidney stones still visible on ultrasound.
Emma has spent 12 of her 15 months in the hospital. She loves her CMC heart family. We have been truly blessed to have so many great doctors, nurses, surgeons, respiratory therapists, techs, and other staff in our lives.
Emma is a fighter. She has already defied the odds. She is the light of our lives and brings us so much joy. She many only have half of a heart, but she makes our heart whole. We truly enjoy every day with her and try not to take any day for granted.
God is Good! All the Time! God is Good!
