Participating in "Every Heart Has A Story", Hosted by Stefenie Jacks

This is the second year, we have particiaped in this event... it is a wonderful idea, to raise awareness of CHD's and to just share our story with those who may not know the whole thing! Of course, this has to be the shortened version as 4 1/2yrs. worth of happenings would be way too long for the readers... and trust me, it seemed way to long going through it all... but, it is OUR JOURNEY and first of all, I want to thank God for this journey. There has been many trials, yet this is God's way of bringing us closer to HIM! I can say, today I do feel closer to God and I hope that every year brings the same. Soooooo, here is Jilly's Journey: Jilliann Rose was born on Jan. 5th, 2007. We are not her birth parents and did not know her at this time, but this is where Jilly's journey with HLHS started. A beautiful little girl, born with so many problems... not only did she have HLHS, but also DORV and Mitral Atresia, that was just the heart defects. Jilly also had a cleft palate (not effecting her lip) and a short jaw bone (Micrognathia), and was born with severe Low Tone (Hypotonia). Jilly had her 1st open heart surgery, the Norwood, at 3 days old. Afterward she struggled with abnormal heart rythems and required pacing. Once she got through those issues, the next issue was feedings. Having a cleft palate, increases the work of sucking... having a heart defect also increases the work of sucking... and having reflux on top of that just made it more difficult. As the records from The Childrens Hospital of Philadelphia (her 1st home, although she was born next door at the University hosp. of Penn., prior to the birthing unit at CHOP being build)... anytime the speech therapist came to work with Jilly on feedings... the parents were not present. To me, that is so sad! So at some point around 3wks. old, it was decided to place a feeding tube in Jilly and perform the Nissan Fundoplication to help with the reflux. Jilly went home to NY at around 4wks. of age... and sadly was admitted to our local Hosp. a few days later. She was severly dehydrated and malnourished... This is when our family came into the picture. We were aware of the Birth parents and the situation. We had heard that little Jilly was left at the hospital, alone. I could not imagine her being alone with all she has gone through... so I went to hold her. There was something so special about her, and the next day... I came to hold her again. I FELL IN LOVE!!! I moved into the hospital that day... and just 1wk. after meeting Jilly, Child Protective Services were removing her from her Birth parents custody... I actually have a STEP-Family Connection, so CPS placed her into our custody... avoiding her becoming part of the system, she was just 6wks. old at the time. The original plan, was for us to care for Jilly while her Birth parents came and learned how to care for her... they did not participate, and we realized very quickly that it was time for us to consider her future. There was no question about it... we would never abandon this precious child, she would become a part of our family for good!!! We love her no differently than her 4 older brothers... one of which is in Heaven. Our family embraced her and loved her unconditionally, and our goal was to give Jilly the best and longest life possible... FULL OF LOVE AND SECURITY... and so starts our Journey with Jilly! Jilly was due for her 2nd open heart surgery at 6mo. old, the bi-directional glenn. She had a heart cath. prior, and then the surgery. Jilly had a very difficult time with recovery, at that time, a neuromuscular md. came and discussed her Low Tone issues, ultrasounded her muscles and found them to be abnormal, and explained that this condition causes children to recover slower. Jilly did not deal with the pain very well and had to be put on Phenobarb to calm her brain and get her through it. She did get through it and we went home a month later. We were ever so glad to be home and start enjoying life. Jilly had a very difficult 1st yr. with Stomach problems... we found ourselves traveling to CHOP over and over (a 4 to 5hr. drive each way)... but we did get through those times. Sometimes we really had to question Jilly's quality of life as she would retch and vomit up to 30 times per day... we just kept hoping and praying that she would outgrow this as none of the docs could make it better for her. At a yr. old, Jilly did improve a bit, and was able to enjoy alot of her life. Unfortunatly, it was short lived and the tummy troubles came back full force... we were life flighted via Jet to Philly, as she was really struggling and our local hospital could not get an IV into her fragile veins. We did get through this rough patch, and since we were already in Philadelphia it only made sence to go through with the cleft palate surgery that she was scheduled for. So in Feb., 2008 Jilly had her surgery to repair her palate. Right after the surgery she caught the adenovirus (a very nasty virus that causes all of your glands to swell)... it was not good on her digestive system and she started to loose massive amts. of weight. 2 wks. into this, it was decided that Jilly would need IV feedings and a Broviak (central line) was placed in her Left leg. Sadly, not 24hrs. later... on March 1st, 2008 Jilly suffered a Stroke to the right frontal lobe of her brain. She then went into a seizure that lasted an hour... the team of doctors were in there working very hard to keep her alive. It was said to us by the head cardiologist after the fact... that Jilly scared him, he did not think he was going to be able to keep her heart beating... BUT HE DID, and Jilly survived her 1st Stroke. A week after the Stroke, Jilly started to wake up a bit... she cried all the time and seemed like she was in pain! We were sent next door to CHOP, to The Seashore House to recover and participate in and intensive rehab. program where you would spend 6hrs./day in therapy. We spent a total of 4months in Philly, and slowly we started to see Jilly regain all that she lost. She had to start over, learning to hold her head up and make eye contact and everything you would expect of a newborn to learn. We went home, and continued with therapy there... and Jilly continued to improve monthly. Eventually, life got easier and Jilly got stronger and smarter... life seemed almost normal in fact. The next plan was to wait for the next heart surgery (the Fontan), until it was apparent that Jilly's body needed it. Looking for low oxygen levels and decreased energy, and watching her as she became bluer and bluer, due to lack of oxygen in her body. Jilly is such a fighter, it did not matter how blue she was... she just kept working towards normal development. It took time, but eventually Jilly even started to walk... It was Christmas time... 2009. We were so excited and proud of Jilly and her accomplisments! One lasting affect from her Stroke was her lack of Speech... Jilly only had a couple of words she could say verbally... but Jilly knew over 100 signs, and was very good at learning new signs daily!!! We knew, we were growing closer and closer to needing the next open heart surgery... we did not look forward to it, and yet we looked forward to what is called the Fontan Circulation Completion... it is the goal of a single ventricle CHD... to have that surgery... the 3rd and final surgery.... HOPEFULLY!!! 2010 came along, and the surgery was being scheduled... At first, the date was in January, but because of the new outbreak of swine flu... it was decided to keep Jilly away from the hospital. So again, in April, we were scheduled for the Fontan... Just prior to the surgery... Jilly was at the very best she had ever been physically and cognitivly. Her receptive skills, her ability to understand was tested as that of a 6yr old. That was a huge accomplishment with all Jilly had gone through in her short life. We are so proud of our Jilly!!! April, 2010... The 1st attempt at the Fontan. Jilly headed off to surgery, Dr. Gaynor headed of to get it done. We were waiting in the parent lounge for our 1st hourly update... when suddenly a nurse came running in and pulled us to a conference room and told us Dr. Gaynor needed to speak with us. OUR HEARTS DROPPED!!! What was wrong? Was she dying? We waited a few min. and then came Dr. Gaynor all dressed up in his OR gear... he asked us to sit, we were trembling! Then he said: do'nt worry, she is ok...... PHEW....... Then he said: BUT.... and our hearts sank again.... "But, there is a change of plans... so drastic, that I felt I needed to come tell you first"... we were glued to his eyes, wondering what this could be that would bring the surgeon out to talk with us after already opening Jilly up for surgery... Well, Jilly had a history of a bulging Aorta... we knew it was large, in fact our Cardiologist, Dr. Donner would say that it was "the size of NY city"... joking of course, but the largest he had ever seen on an echo or heart cath. Well, Dr. Gaynor got in there and saw that it was indeed large, but "larger than NY city"!!! There was no option but to replace her aorta... it had not been done on a child that small, not the entire thing replaced... but it has on adults for 30 to 40yrs. and the surgeon was confident that we could do this on Jilly too. There was NO option, of doing the Fontan, the Aorta was in the way... and he could not do both the Aorta and the Fontan because that would cause too much bleeding and she would not survive. So the only option was to do the Aorta surgery now, and come back later for the Fontan. It was clear, there was no choice... so we just said OK, and off Dr. Gaynor went to do his amazing work... guided by God of course!!! The surgery went well, and the only complication was that Jilly's tummy troubles were stirred up. We spent another 3 months in the hospital and Jilly ended up having 3 abdominal surgeries and numerous complications... but she did get through it all. A very weak, underweight child... but strong enough to fight. We went home in June, and worked on building her back up knowing that the Fontan would be attempted again before long. Jilly also required Oxygen 24hrs./day at that point... the thinking was, that she just needed that Fontan surgery to bring up her oxygen levels. Due to this, it was decided that Jilly needed to go forward and have the Fontan in 2010. We were scheduled for Octover, 2010 for the Fontan attempt... again!!! Jilly had grown nicely, and gained alot of her strength back that she had lost from last hospitalization. She even had to learn to walk all over again... but she did. So now, open heart surgery #4... The Fontan. We were so hopeful that this would be the final surgery... yes, she had 1 extra surgery, but we got through it ok. On Oct. 26 we were scheduled, we were bumped to the 27th after Dr. Gaynor had gone through a very tough day. That was ok, we wanted him nice and rested... he performed the surgery and everything went as "textbook"... wow, we finally had the Fontan. We felt like we had jumped all of the tallest hurdles and we were on our way to the finish line. Unfortunatly, this was not the case.... Jilly was loosing enormous amts. of fluid through her chest tubes, she had fluid resusitation two different days... she did seem to pick up for a couple days, then seemed to be going back down hill again. None of the docs knew why... I suspected something was wrong on that Tue. and shared it with the docs... on Wed. they agreed with me that something wasn't right... and then that Thursday... Nov. 4th, 2010... the day started with a bang, things were going down hill... Jilly's body was so cold to the touch, her color was horribly blue, her sats were dropping... she was alert at times, but others she was not. The bedside tests began... a very special intensivist started checking Jilly every 15min., feeling like something was brewing... they were looking for infections somewhere... she was sweating profusly and her body was getting colder and colder. A heart cath. was scheduled for the next am., with the feeling that there could be some issue with the heart. But she continued to decline, and then her heart function became serious/ heart failure... she was rushed off to the cath. lab, in hopes of finding something easy they could fix in there.... that was not the case... what they found, was that the new conduit that is used to hook the lower bodies blood flow to the lungs had become completly full of numerous blood clots.... she had a complete blockage, and only a couple of minute collateral vessels had formed to let little to no blood flow through. Dr. Gaynor was out of the country at this time... he had left on monday, stopping in to see us as he was headed out the door... he told Jilly to behave, and that he did not trust her and that he would be checking on her constantly... So we needed the Surgeon on-call to do the emergency surgery... THE FONTAN TAKEDOWN... As they rushed Jilly from the Cath. lab to the OR and was prepping and opening her for the surgery... the cath. doc came to explain to me what was happening... it was not good. This man puts it all out on the table... Jilly needds the Fontan Takedown and most do not survive the situation that brings them to this surgery. He explained that I needed to make a choice... either they go in and try to remove the clost and fix the Fontan Circulation... WHICH WILL ALLOW THE CLOTS TO GO ALL THROUG HER BODY AND DESTROY EVERY ORGAN... HEART/BRAIN/LUNGS/KIDNEYS/LIVER/ETC... OR... we can attempt the Fontan Takedown and hope that she survives and they can contain the clots and remove them all at once. Dr. Fuller would be our surgeon of the night, we had no time to wait for Dr. Gaynor to come home. He was fully aware, and supported these choices... it was very clear to us... we had NO choice... the FONTAN TAKEDOWN IT WAS!!! Jilly survived the situation and the surgery by the grace of God... But, after looking at the clots in the OR... it was seen that they were something that started a few days ago, most likely the Tue. when I felt something wasn't right... and it was believed that the clots appeared to tell them that she had become fully blocked 12hrs. prior to the Takedown surgery... I did not know you could look at clots and know how many hours old they are... learned something new. So, the expectancy of recovery was not good for Jilly!!! Major Brain injury was the thoughts of the entire cardiac team. In fact, later I found out that every nurse had heard... "Jilly is going to die"... thankfully I did not hear those words at that time!!! About 2 1/2 days later, Jilly was extubated... and she did not know how to breathe correctly... her chest was jumping up and down, her abdomen was see-sawing all over the place... the thoughts were that she may have a parylyzed diaphram... they wanted to intubate her again... I had that one special doc and several respiratory therapist friends who talked about c-pap and how it can be used for these situations... the docs were very hesitant... I felt for some reason, that if Jilly was intubated that she would die. Her blood gasses very worsening every 15min. and something had to be done. This special intensivist talked the other docs into trying c-pap 1st... we were given 15min. and if the blood gasses worsened, she would be intubated... 15 slow min. went by, another blood gas checked, and it had stabalized... so were given another 15min. and so on... it worked, the c-pap was controling her breathing and her blood gasses eventually improved!!! It still did not get easier though... Jilly started waking up, and she could not control any movements... her left side did not move at all, and her right side was having uncontroled movements called "CHORIA"... That is when Neuro. and the Stroke Team were called in, then Neuro-muscular docs too... It was decided that it was impossible to take Jilly to have a MRI of her brain, due to her breathing... she would have to be bagged, to go anywhere... they did do a fluroscopy of the diaphram and that was only a few min. of having to bag her... she could not breath on her own. So, we were told... based on clinical signs and symptoms... Jilly has suffered brain damage... possibly a stroke again, possibly due to hypoxia (lack of oxygen) possible damage to grey matter of brain, possibly to white matter of brain... sometimes a scan cannot even pick up these different damages like a stroke is picked up... but all in all, the treatment is the same regardless of the damage to the brain... REHABILITATION!!! We did this before, it did not make it easier to accept... in fact, it seemed harder so unfair that Jilly had already learned everything twice in her life and now again!!! And that is what was needed... a couple days later, Jilly could not use either side of her body. We were told after a EMG with the neuro-musc. docs that she may have a neuropathy...??? All we could do is start rehab. and continue c-pap for her breathing. We could not even see her face, she could not see us... the mask covered her eyes because they are not made well for the little ones... when we did try to get close, she could not focus, she could not make eye contact, she could not move her body much at all. It was horrible!!! But, Jilly overcame these things... it took months, but she has regained alot of what she lost that day... It is now, August, 2011... not quite a year... and Jilly is walking and talking and learning it all over again. Yes, somethings are different for example retaining her sign language like she used to... but, believe it or not.... some things are better!!! Odly, Jilly came out of this talking... seriously talking... new sounds, new words, and now even sentances... all new since this brain damage!!! God works in mysterious ways!!!! God has a plan for Jilly... I don't know what it is, but I do know that my job is to keep fighting WITH Jilly, until God calls her home... and I know, that absolutly NOONE on that cardiac floor thought Jilly would live another day, and if by chance she did live... they all expected her to be a "vegetable"... Thankfully, God is our Physician and he has control.... he breathed life into Jilly, that day of Nov. 4th, 2010... and I praise him for the Storms and the Miracles!!! My advice to any one traveling these CHD journey's is..... Never loose Hope, as long as they are with us... we have to keep fighting, just as our children are!!!! God has a plan for each of our children. And yes, one day he will call them Home to be with Him... but today, as long as they are still fighting.........................

WE FIGHT WITH THEM!!!

Joy Kimble, mommy to Jilly (HLHS WARRIOR/STROKE SURVIVOR)