JASON EMIL GUTIERREZ born with HLHS with a intact atrial septum. Half the heart....twice the fight
Baby Jason’s Heart Journey
Being Pregnant is supposed to be one of the happiest times in a woman’s life. Imagine being worried, sad, stressed and scared while another life is moving and kicking inside of you. At 21 weeks we not only found out that we were having a baby boy but also that something looked “different” about his heart. It was very obvious while performing the ultra sound, the doctor was seriously concerned. That day I left my OB’s office in tears. We later found out that my son’s Mitral Valve was restricted or closed. The cardiologist then proceeded to tell us that this can lead to something called Hypoplastic Left Heart Syndrome. This is where only half of his heart develops. I sat there is shock thinking to myself, “How does anyone live with only half of a heart?” Not only was I devastated that my son would have to undergo one open heart surgery but a total of at least three. I had no idea what to think or do with myself. I was that stereotypical person who would hear birth defects in general and think of no good pregnant girls who partied all the time and did drugs. Now I was in the same position and did none of those things while I was pregnant. I had never heard about Congenital Heart Defects (CHD) for I had no heart problems in my family history nor knew anyone who had ever experienced something so dramatic but I blamed myself for what was wrong with my baby. I felt lost and helpless. The doctor informed us that without surgical intervention, this defect is fatal. He then went on to tell us that while he was in the womb he was healthy and safe, but the moment he is born he will be fighting for his life. I went on with my pregnancy and tried my hardest to stay positive even though I knew in the back of my mind that my first son may not live to see his first birthday. Even with this news my boyfriend and I decided to give our baby every chance to live and decided to do the surgeries.
At 25 weeks we visited Children’s Hospital in Aurora, Colorado to discuss our son's birth plan. While we were there we met many doctors, nurses, social workers, and cardiologists. They also gave us a tour of the CICU (Cardiac Intensive Care Unit). Seeing the babies hooked up to so many machines and tubes was hard. I couldn’t prepare myself to see my own flesh and blood in that same position. It made my stomach turn just thinking about it. While we were there the cardiologist wanted to do an echo to see exactly what the baby’s status was and once again the doctors concern stabbed me like a thousand knives. After 45 long minutes of measuring heart waves and blood flow I felt sick to my stomach. The sack of rocks in my gut was right because the cardiologist proceeded to inform us that our baby was going to be a lot sicker than we had planned. I didn’t think that anything could get any worse but to my surprise my son would not only be 1 in 100 but 1 in 100,000 babies born with Hypoplastic Left Heart Syndrome with an Intact Atrial Septum. This meant that immediately after birth he would be stabilized and rushed to the Cath Lab to balloon his Atrial Septum open. We had a short period of time to do this before it becomes fatal for our baby. The septum being closed doesn’t allow the blood flow to enter his lungs so it was crucial for them to get it open as soon as possible. They also insisted that I give birth by caesarian in the Maternal/Fetal ward at Children’s Hospital to ensure that the baby could get to the Cath Lab before it was too late. Before we left they told us that his chances of survival dropped with the intact septum diagnosis, just more bad news to add to my overfilled plate. We met with the Cath Tech that would perform my baby’s first procedure. He said that within the last seven years he worked for Children’s Hospital he had only preformed about four of these procedures and that this diagnosis was very rare. They also suggested that we move to the Denver area temporarily at about 36 weeks to ensure I don’t go into labor so far away. The next time I would see these people my son’s life would be in their hands and I would be living at Ronald McDonald house in Aurora Colorado, 200 miles away.
On July 22, 2011 at 8:33AM Jason Emil Gutierrez entered this world. He weighed 9Lbs 3Oz and was 20.8 Inches long. The doctors were amazed at how big he was and even made some smart comments about it. He came out not breathing though so the joking around didn’t last very long. He was intubated and rushed to the Cath Lab immediately. While waiting for news on his status I can’t even describe the anxiety and helplessness I felt. Many family members were with us to show support but it was still very intense and scary. It would be about eight hours later before I even got to meet my own son. We finally got news that Jason’s septum was ballooned and lasered open. He was as doctors say, “critically stable”. When I got to lay eyes on my son I felt like the happiest person in the world. The sight of him intubated, hooked up to machines and tubes coming out from every angle of his body was disturbing but he still looked like a angel laying there to me. Just the strength that he gave off amazed everyone who seen him. He had already impacted so many lives and he was only hours old.
Within 3 days he was extubated and we were able to hold and cuddle him. I have never been so scared to have a baby in my arms as I was that day. He was just so fragile and I was so afraid that I would hurt him. We learned fast how much people take for granted. Even things that people think are routine, we weren’t able to do at first. Things like Holding, playing, feeding and changing his diaper felt like a privilege. We were able to begin doing these little things for our little guy just in time for his first surgery. Then it would all be taken away yet again for Jason’s own safety.
On July 27, 2011 at about 7:00AM I had to hand my newborn son over to the nurse so that he could undergo his first open heart surgery (Norwood). This was so hard for me to do considering that he just came into the world, and I was afraid that I would never see him alive again. The thought of my helpless babies perfect chest being cut open was horrifying. But knowing that this was the only way that my son would live, I did it. At that moment I realized that Jason had already influenced my life in more ways than I had thought. I was stronger than I thought possible, all because of my love for my baby.
9 long hours and many updates passed before Jason was off of the bypass machine and stabilized. Dr. Max Mitchell (Jason’s surgeon) came out of the OR and his first words were, “well I have good news and not so good news”. These are words that every mother in my situation dreads. He proceeded to tell us that Jason was doing fine but the surgery didn’t go as good as he would have liked. His Pulmonary Artery was smaller than the shunt and the shunt didn’t fit onto it as well as he would have liked to see. This could cause problems for Jason in the future or may not so at that point Dr. Mitchell didn’t see any point in altering it. By the time we got to see Jason nothing could prepare us for what our eyes would see. Jason was not only swollen and blue but his heart had so much swelling from the surgery that the surgeon was unable to close his chest in the OR. To be honest my son looked dead. The only thing that eased our minds was the sight of his little mended heart beating underneath a thin white cloth sewed over Jason’s open chest. I just sat at my son’s side and cried. I wanted to switch places with him so badly and prayed that it was all a dream but when I opened my eyes I knew that this is my reality.
During the next month or so Jason would overcome many tough obstacles. He would get his chest closed, get his drainage tubes taken out, come off the ventilator, his oxygen weaned down, and get his staples taken out. His biggest challenges included overcoming a infection caused by I.V’s, Arterial lines and Pic lines, and eating and controlling his breathing. The “Normal” baby breaths in the 30’s while Jason was breathing in the 70’s and even 100’s at times. We battled with many nurses about his oxygen and flow because no matter what they did he was a fast breather and no one knew why. We later found out that he suffers from something called PIG (Pulmonary Interstital Glycogenesis) or in normal people terms, underdeveloped lungs. This should get better with time but Jason would come home with ¼ Leter of Oxygen. Eating was also a huge hurdle for Jason. Unlike some heart babies he did eat by mouth somewhat, but he couldn’t eat nearly enough and what he did eat, he overworked himself. This means that the effort it took to eat burned off most the calories he should have been getting. Because weight gain is such a huge priority for heart kids we decided to get Jason a G-tube (feeding tube) and Nissen to help him with nutrition and aspiration. The doctors insured us that this was just temporary and eventually he will be able to eat on his own and it would be able to get taken out. They also said that once Jason got this, he should be able to go home sooner. This was so exciting because at this point we had already been in Denver for 2 months and in the hospital a little over a month. I could hardly wait to get out of there. The CICU was beginning to feel like home but I wanted to go to the real home, with no doctors, nurses or machines beeping at us every minute.
On August 31, 2011 at 8:00AM Jason was on the schedule to go back to the OR. In my mind this was easy because had had already been through the worst part. We were on the home stretch now and we only had one more step to go. When the G-Tube surgery was done and Jason was being extubated a nurse ran into our room and told the respiratory therapists not to extubate because the cardiologist had to perform an echo and he had to be still. I don’t think my heart has ever dropped so fast in my life. I couldn’t imagine anything going wrong now. There were four cardiologists surrounding my son asking mind boggling questions like, “What is this? What way is the blood flowing here? Why is there red and blue blood there?” These questions are what they are supposed to be answering, not asking. I just sat there wide eyed. Dr. Yunisai (Jason’s main cardiologist in Denver) eventually looked over at me and noticed that I was seriously confused and concerned. What he then told me was devastating. My son had a Pseudoaneursym around his heart. The cause was unknown and at this point Jason’s life was in serious danger until they can operate. This was a huge set back to our plans of going home. Dr. Mitchell (surgeon) was concerned enough to put Jason on the schedule for the next morning. I asked him what Jason’s chances were to make it out of this emergency surgery. His response to me was that this was more risky than the Norwood and at this point all I could do is pray to God that he makes it out of surgery. All I could do was put it in God’s hands, stare at my son and cry. I once again had to hand my now one month old baby over to a nurse, only this time was ten times harder than the last 2 times. Surgery lasted 8 hours and included most of the cardiac surgeon team at Denver Childrens. As Dr. Mitchell walked down the long hallway to update us on the outcome of the surgery and I could see a glow about him and this time he had a smile on his face. He told us that surgery went well and Jason was doing great. Not only did he fix the Pseudoanurysm but was also able to fix the shunt and Pulmonary Artery issue he had with the last open heart surgery. He said that the Pseudoanurysm was caused by a leaky patch. He informed us that this rarely happened but that it is always a risk. For the first time he was very optimistic about Jason’s recovery which was a relief. I don’t know if I could have handled anymore bad news without completely breaking down. During the next 2 weeks Jason made massive progress. He not only looked better but he could eat better and his breathing slightly slowed down. We retired out of the CICU and up to the 9th floor where we could begin discharge requirements. We were discharged on September 15, 2011. By the time we were able to leave I was under the impression that the doctors didn’t expect Jason to ever leave the hospital alive. One of the doctors even made a comment like, “He is doing so well and you have a real success story on your hands.” Boy was that a relief.
I was so happy to be able to hold and take care of my son without nurses, doctors, wires, and beeping monitors surrounding us. A couple short months later, On December 03, 2011 we returned to Children’s Hospital for Jason’s cath and Glen Operation (2nd scheduled open heart surgery). I was so scared about having to do this whole hospital thing over again but I prepared myself to do it only to be let down. It’s a real confusing thing to want your son to be able to go under the knife but I longed for that because the series of 3 surgeries is the only way for my baby to have a decent semi long life. After testing pressures and looking for restrictions in his already battered heart they found that Jason’s Pulmonary Arteries were too narrow to move on the Glen. They told me that right now if Jason were to get his Surgery he would die on the table. They ballooned these arteries to get little growth and scheduled us to go back in a month to balloon again and hopefully get some progress. After hoping and praying for growth we went back many times between December and April only to get let down over and over again. Our Denver team wanted us to go back for a complex surgery that included complex Cath procedures and replacing his Sano Shunt together because Jason was beginning to outgrow his Sano shunt that he had. They would have to do this procedure close to every year as Jason grows. His surgical team is pessimistic that Jason will ever be eligible for the Glen Procedure.. The Surgery that they wanted to do was another risky procedure where the doctors couldn't tell me statistics or success rates. Jason would paving the way for other kids that are in a similar positions. This didn't sit quite right with me if there was another better option out there for him.
After many months of hopeless second opinions I began to give up hope that anyone out there could give my son a fighting chance at a long life. Finally God answered our prayers and we got news that our surgeon in Denver was referring us to Dr. Hanley at Packard Children’s Hospital in Stanford, CA. He invented a procedure called “Unifocalization” and he is optimistic that this could help Jason’s Pulmonary artery problem. His specific recommendations read like this, “My specific recommendation for Jason Gutierrez is that he should be scheduled for elective pulmonary artery reconstruction to address the peripheral stenosses in both his left and right pulmonary artery systems. At the time of the reconstruction, I would place and central aortic to pulmonary shunt, and then obtain follow up catheterization data at approximately three months following the PA reconstruction to determine his candidacy for a Glenn Procedure.” After little time Anthony and I decided that this was the best decision for Jason and I made his appointment for his 3rd open heart surgery.
We made it to Stanford CA on September 11th 2012. Jason had pre-operation appointments all day that consisted of blood draws, x-rays, listening to heart rhythm and measuring heart waves. By the end of the day Jason was worn out and tired. His little arms and bottoms of his feet were already covered with bruises from being poked and prodded all day. He was absolutely miserable. He was old enough now where he knows what is going on and his attitude shows it. It seems like he is handling everything well until you set him on the hard bench with the thin sheet of paper over it. The moment he touches that paper he knows something is going to happen and begins to sob with anxiety with worry in his eyes. He looks at me like I am doing something wrong… Like I am supposed to be his hero and I am not fulfilling my duty at his superwoman mommy. It’s just heart breaking. I knew that this surgery was going to be hard but I never imagined feeling like a helpless hole of nothing. Who am I if I can’t even take the worry away from my one and half year old?
Jason’s Open heart surgery was scheduled on September 13th 2012. For the 3rd time in Jason’s life I cuddled Jason as long as I could before the anesthesia team came to remove Jason from my arms and lay him on a cold flat table while they operated on his little heart. I almost fell to the floor in grief as they turned the corner and I couldn’t see my son anymore, not knowing if that was the last time I would see him breathing. 12 ½ Hours later a tall man with gray hair and a sports coat came into talk to us about the surgery. Dr.Hanley, Jason’s Angel, He was so soft spoken and to the point, I could tell just by his mannerisms how kind he was. Jason was unstable during the first hours of surgery but stabled out eventually. He used 8 patches on his pulmonary arteries to make them bigger and have better blood flow. Jason was on the Bypass machine for 4 hours straight while they did this procedure. He moved his shunt connecting it from aorta directly to the pulmonary arteries mimicking the ductis that saved Jason’s life early on. Jason had a hard time coming off the bypass and they team had to turn it off then back on a few times before Jason was ready to be off completely. He proceeded to tell us that he was unable to proceed onto Jason’s 2nd stage surgery (GLEN) due to him being on bypass so long. While on bypass the machine makes your lungs wet and mucky, and really does a toll on the function of the lungs. Dr. Hanley didn’t want to chance doing a Glen on Jason while his lungs were unhealthy and it failing. He insisted on giving the lungs time to re-coop and become healthy and strong again before attempting a GLEN. He did insure us that although nothing is certain, Jason’s pressures and blood flow leaving the Operating Room were acceptable for a GLEN and that he wanted us back in about 3 months to do a CATH and hopefully progress onto the GLEN.
I was really upset and let down that Jason had to have another extra open heart surgery but thankful all at the same time. Emotions rushed me. I thought about how Jason will be up to at least 5 surgeries when we are past the 3rd stage. 3 staged surgeries and 2 extra surgeries and that’s only if he does well and doesn’t need anymore which is highly unlikely. Than I thought how much will he go through until it’s too much and he just stops fighting? I just couldn’t and still can’t imagine burying my son. Our reality is that eventually this disease will kill Jason. It’s not a IF it’s a WHEN. I had negative after negative thoughts rolling through my head. I really had a hard time controlling them. On the other hand I was so thankful that Dr. Hanley was able to do something… anything that can get Jason that much closer to the goal. That much closer to a chance at a long life… kindergarten, high school, college maybe even marriage. That’s what I want to imagine for Jason’s future. Not this. Not a life full of hospitals, I.V.’s tests and surgeries.
Walking into the CICU (Cardiac Intensive Care Unit) many things were familiar for me and I had little questions. But Jason was hooked up to a machine much like a Ventilator but different. Jason’s entire body shook and the machine wasn’t showing big breaths but many little tiny breaths. He was hooked up to a Oscillator. A step up from a Ventilator. and for more critical kids. Because they did such extensive work on Jason’s lungs they didn’t want Jason to take big deep breaths right away but little short breaths so that they could give his lungs time to heal. Made sense to me, but It was very unnerving to watch my sweet boy lay on a bed, motionless, naked and his whole body trembling from a machine doing all the breathing for him. He was like this for a little over 5 days, they then put him on the common ventilator for a few more days. We never left his side, although he never moved, or blinked we wanted him to feel our presence with him all the times just in case he was in there somewhere. It was a little over a week later when he was finally extubated (Taken off the Ventilator). He sat up within a hour and started playing drums on a calculator with the nurse but never smiled. He was worn out and in pain but was still a joy. He battled obstacles this time around that included fighting a infection and viral infection that he contracted from the hospital, keeping oxygen saturations up and blood pressures down. He battled withdrawal again which was the cause to his discomfort, and fussiness. But did well and we were out of the hospital a little over a month later. The nurses grew to love Jason and called him the Gerber Baby. The CICU nurses and step down nurses fought over where Jason would stay because both wanted him on their unit. When we got to bring Jason home he was on many extra medications but he was like night and day. He began to crawl shortly after and had much more energy. He was a completely different child physically.
We returned to CA January 2013 for Pre-GLEN CATH and his second Staged surgery (GLEN). Jason did well this time around during his Pre-Op appointment and although he cried while he got poked and prodded he did not move and let the nurses take multiple blood draws. During his Echo he fell asleep and let the tech get all the pictures they needed of his heart. The CATH showed that after all the hard work Jason was FINALLY eligible for his GLEN, but while in the CATH lab Jason spiked a major fever. While recovering he got diagnosed with the FLU and we were inpatient for a week and a half. They sent us home only to return in 3-4 months for his GLEN. Since the FLU is a respiratory virus it was unsafe to have a GLEN because Jason's lungs had to be in the best possible shape. Although we didn't have the surgery I could feel weight lifted off my shoulders just hearing that he was eligible and still beating the odds with every heartbeat of his precious little heart. For the first time in over a year I was beginning to see the light and Jasons future at the end of the tunnel.
We got on the plane with high hopes in May 2013. Jason underwent his GLEN and did well and was extubated the same day as surgery for the first time ever. We had a long stay this time due to him developing a Cylus infusion. We spent many weeks in the step down unit with draining chest tubes and a new fat free diet. We spent most of our days taking Jason on wagon rides to look at all the colorful paintings on the 3rd floor, going to the library picking new fun books, and going to play when the play area was open. Jason loved to go through the Animal bin and by the end of our stay he could name and tell you the sound of every single animal in there. He also began to love the music sessions and making loud noises, he became quite the musician. Not only was he more energetic but from trucking all around the hospital he began to walk all on his own. Under a month of being in the hospital and he was starting to walk!!!! I really do have a warrior!!! We were Discharged in June 2013, and although he was on a fat free diet for a few more months, Jason had a lot of weight gain.
Even with Jason’s complex HLHS He is a normal 2 1/2 year old. He is doing well considering the severity and complex circumstances he faces every day. We have made many trips to the ER for things that concerned me and have even been life flighted because Jason had a cold and made his already low oxygen saturations even lower. He gets short of breath easily and sweats more than other kids. But regardless of all of his hurdles he is not far behind other kids and is actually a bit more advanced cognitively. He eats orally and is beginning to really progress with his eating habits and abilities. Although he is still behind on his physical capabilities, he is showing improvement every single day. He is the most pleasant baby I have ever been around because he is always laughing and smiling. Surprisingly, he is genuinely a good little guy hardly ever crying or fussing and always uses his manners. He loves singing and dancing. He can sing his ABC's almost perfect and can even count to 10 by himself. If you didn’t see his scar you would never even know the hardships and battles he has already fought in his little life. Every mile stone Jason reaches is such a good moment for our family. Jason still has a long road ahead of him, full of catheterizations, surgeries, cardiologists, medications, and eventually a Transplant. HLHS is not curable but only manageable. We do this to buy time, we are buying time until he needs a transplant or until his heart fails. But I have faith that my angel will continue to shock doctors with his strength and will to live. Even through all the ups, downs and hurdles to come I will continue to thank God for my son and lessons he continues to teach me. I would not change anything about my little fighter. As he grows older I will teach him to be proud of his scar because it has made him strong. My son beat the odds against him with the help of his entire team. I am so proud of be such an amazing and inspiring persons mother.
As heart moms we deal with battles everyday full of hospitals, doctors, medications and surgeries. We can operate oxygen tanks, feeding tubes, machines, and pulseoxcimiters like the back of our hands. We have face masks, Sanitizer and notes on our doors explaining proper hand washing techniques. Were overly cautious, protective, and are aware of all the germs around us that can make our babies sick. We deal with daily stress including stress that the typical mother can’t even relate to. Including the fact that at any point we could lose our child to Congenital Heart Defects. We have all thought about what we would do if our child dies and always have it in the back of our minds that it’s always a possibility because it happens every day to other mothers just like us. We don’t take little things for granted like our child eating a simple meal or walking all the way through the store without having to sit down to take a break. We wake up in the middle of the night just to check in on our child to make sure that they are still breathing. We deal with things every day that some parents can’t even imagine and will never be able to understand. But what we need to remember that we are not superwoman and we can and will break down through all the ups and downs that CHD throws at us and that is ok. We will get through this because god chose us to be Heart mommies and God only gives us what we can handle. God picked you for a reason, even if u can't see the reason yet!
Written By, Mary Kincaid