Emily's Story

by Amy Dini

On Thanksgiving of 1997 I gave birth to a beautiful 6lb 13 oz little girl, Emily Suzanne Dini. She made a slightly early appearance, but nothing that anyone had any concerns about. Everything was perfect, I had gotten the ok from the insurance company that we could stay and relax for 48 hours instead of 24, She scored 9/9 on her apgar scores, and was already nursing just fine. Our family came to visit that evening with the exception of my mom and our other daughter Taylor. She was just getting over the flu and we decided we didnt want to expose Emily. She slept in our room that night and life was wonderful!

The following morning our trusted peditrician, Dr Munoz came in to do his evaluation. They took her to the nursery which was a little strange to me, but I just assumed that policy had changed. After an hour I sent my husband, Tim to find out what was going on! They said that Dr Munoz thought that he heard a murmur and although none of the other doctors had, he was insisting that someone from Cardiology come and look at her. This didn't alarm me too much since Taylor has always had a murmur and it is believed to be innocent. Several hours later, Dr Munoz came back into the room and said that Emily had been taken to do an Echocardiogram of her heart "just to make sure" and try not to worry.

The next thing I know is that they are calling me from the NICU and telling me that they are flying her to Riley Hospital for Children in Indianapolis ( 2 hrs away) as soon as possible. The cardiologist came in and told us that Emily had a terrible defect called Truncus and that she was born with out any pulmonary arteries. They said that there was no surgery that could save her, but they were sending her to Riley to make sure. They were not even sure that she would survive the transport. We finally got to see her and it was probably the worse thing I had ever seen up to that point. She was in a little isolette and hooked up to numerous tubes and wires to keep her alive... I just couldnt believe them! How could my little baby who was so perfect be so sick?? We had her baptized right there and then left so we could be there when she flew into Riley since they wouldnt let me ride in the helicopter with her. We had nothing with us, never got to see our other daughter before we left and Thank the Lord that I had a normal delivery and not a C-section! We arrived at Riley and the staff there was nothing less than wonderful! They got Emily in her room and stabilized as quickly as they could and I was able to see her again in no time! It was several hours until a cardiologist could come up and see her and do her own echo. Dr Farrell did Emily's Echocardiogram and is a wonderful lady..she gave us the first piece of good news we had gotten all day. She said that Emily did indeed have pulmonary arteries, but they were very very tiny. She also had a rather large VSD and she had a Patent ductus Arteriousis that they were using medication to keep open so that she would stay alive. She also had some major collolaterall arteries that were branching off to her lungs that were helping with the blood flow. Poor Dr Farrell couldnt believe that we were happy at this point. We fully understood the severity of Emilys defects, but at least we had hope and Emily had a chance, however slim it might be!

The plan was to sit and wait as long as we could and let Emily get bigger and stronger. I got to hold her the following day once and then the next day twice. I would hold her for hours until I just couldnt move and she loved it!!! We were there at her bedside 99% of the time, just talking and singing to her! Her oxygen saturations started off being about 68% for many days, then started dropping off to the 50's. Dr. Cordes (her pediatric cardiologist) decided to do a heart catherization to see exactly what they were dealing with. He came in and explained everything to us and then had us sign some consent papers stating that we understood that there was a 1% chance of death. That was horrible! It was probably the first realization that this wasnt going to be easy! The next morning as we walked down that hallway I knew that there was a chance that we might never see our child again! I had never felt such pain up to that point...

The Cath lasted about 31/2 hrs and Emily did wonderfully. It was decided that her only option was a central shunt placed to connect her main pulmonary artery to her aorta in order to increase the blood flow to the right and left pulmonary arteries so that they would hopefully grow. They were going to wait and hope that she could get a little bigger, but that night her sats dropped down in the upper 30's and it looked like her bowels had started to shut down. They decided to do the surgery on Dec 9 when she was just 12 days old. Dr Turrentine, her Surgeon and Dr Cordes sat down with us and told us that she had less than a 50% chance of surviving this surgery. The nurses told us that it was very dangerous and that babies don't do well with it....But it was our only hope!! I spent most of that day like many others crying and the compassion that Dr Cordes showed was wonderful...

The morning came and I again realized that she may not come back alive... Probably the worst and the best day of my life all rolled into one! Emilys surgery started off rough! The nurse who came in to give us periodic updates, told us that her heart had stopped before they could even get all the "lines" in, but they had revived her... basically told us not to expect her to survive. We were devestated, all these thought of her dying overwhelmed us for the next hours... I have never prayed so hard before! About 5 hours later Dr Turrentine came in to tell us that Emily had survived!!!! I could have kissed him then and there! He warned us that the next 96 hours were just as critical if not more so than surgery, but I didnt care... I felt as if I were there I could help her if something went wrong! Miraclously, Emily went through her post op experience with flying colors... we were home in a record 9 days and just in time for Christmas!! Even her Doctors were calling her a miracle baby!! Although we knew very well that this may not even work, at least we were spared some more time with our precious little one!!

We spent alot of quiet time over the holidays just being a "normal" family and then in Feb, they decided to schedule a cath to see if the shunt was doing its job and if Emilys PA's had grown. This cath I was a little worried about since they felt that the shunt had narrowed, and were planning a balloon angioplasty which has a slightly higher risk! She came through this again with flying colors and they didnt have to do the angioplasty... she was up and eating about 2 hrs afterward! We had a reprieve for a couple of months and then it was on to phase 2... all of this time Emily had been growing and eating much better than expected and she was developing just like a normal baby, laughing and talking and rolling over! On April 6 we handed her laughing over to the anestheisologist for her second surgery... She was 4 1/2 months old! Dr Turrentine was not even sure which surgery would be preformed. They were either going to replace her central shunt with a bigger one if her PA's werent big enough or if they were, he would connect her Pulmonary artery to her Right ventricle with a patch made out of gortex (same material as her shunt) and put in a valve. We were hoping for the latter since that would be more of a corrective procedure and would save her a surgery! Once again God smiled down on us because 13 very LONG hours later Emily came out of the OR with a new valve connecting her ventricle and pulmonary artery. This time Emily had a bit of trouble post operatively. Her blood had to learn to go in a completely different direction and so she had a little bit of trouble with her pressures and some arrythmias. Her oxygen levels also were not as high as they would have liked so they placed her in an experimental study with Nitric Oxide to dialate her pulmonary beds..She was closely watched and then was sent home almost 3 weeks after surgery. Since that time Emily has done wonderfully. She has had 2 PC appts and an echo and EKG. Her pulmonary arteries are growing and everything looks good so far! They are planning another cath around the end of October and then maybe the final stage of "repair" in the spring or summer (the VSD closure). Emily will always have to have surgeries as she grows because she will outgrow the outflow tract patch that they put in during her second surgery. Hopefully by the time she outgrows it the first time (3-5yrs old) they will have come up with a material that will grow with her...new advances are made everyday! Five years ago they could not have done anything for my daughter, so who is to say what can be done in another 5 yrs!! Overall we have ridden quite a rollercoaster with "little Em" She is our special miracle from God and are just grateful for everyday that we have together! Her quality of life is that of a normal baby and she has developed just as well as any other child. To see her you would never know what she has gone through, although you can definately tell she is a fighter with a VERY strong stubborn streak!!! We are getting very excited about seeing her first birthday soon and pray that God grants us many many many more years together!

Dr Turrentine and Emily

Just an update on Emily...She is a little over a year old now and still doing wonderfully. She had her third cath in October and did fine, although a little grumpy...it looks as if we will do another cath in April and if all goes well, Dr Turrentine will do her final repair in the summer!! We are looking forward to the day when we hear that we wont probably have another surgery for several years!! Emily is as normal as any baby could be...she just took her first steps tonite!! She is our little miracle from God!! We treasure every minute that we have with our children now and have learned not to take any thing for granted!!

April 6, 1999. Emily is still doing wonderfully, more trouble than ever! She had an unfortunate stay in the hospital recently due to dehydration, but has recovered and is now eating EVERYTHING in site

January 15, 2000 Emily had her third open heart with Dr Turrentine on Sept 28, 1999. It was a really long and detailed surgery for poor Dr T, but Emily did GREAT!! She was home in exactly one week and Dr Turrentine was smiling the whole time! I have to say that they did something too her, because she has more energy than ever imaginable! We also received some WONDERFUL news.....Emily should not have to have any more open heart surgery!! A far cry from hearing that we would have to have surgery every 3-5 years until she was grown. We could never be more thankful to God and all the Drs at Riley who have taken such great care of our little "miracle" not to mention all our wonderful family and friends!! We couldnt have done this all with out your support!