(An HLHS Survivor)
By Linda Testa
Zachary’s story begins before he was ever born, back in the summer of 2007. It was about 18-19 weeks into the pregnancy with Zachary and had an ultrasound done as routine but without the routine findings.
It was a Friday morning and the doctor’s office called to tell me that I needed to see a perinatologist because of some findings on the ultrasound and that the doctor would call me later to discuss things in more detail. Therefore, the call came and we were told that they had seen an echogenic focus on the ultrasound on Zachary’s heart and so our journey began.
At 20 weeks, we saw our perinatologist and another ultrasound was performed at which time the doctors suspected that there could be a serious cardiac issue and we were set up to meet with the pediatric cardiologist for a fetal echocardiogram for 22 weeks. At 22 weeks into the pregnancy, we met with the perinatologist, pediatric cardiologist and the first of many fetal echocardiograms were performed and Zachary was officially diagnosed with Hypoplastic Left Heart Syndrome or HLHS a severe and potentially life threatening congenital heart defect. At this time, we were asked as to whether or not we wished to continue to pregnancy or to terminate. We explained that it was not our decision if this precious little boy we chose to name Zachary would live or die and that this was god’s decision.
We met with the perinatologist and the pediatric cardiologist on several different occasions throughout the remainder of the pregnancy and continued to monitor Zachary’s heart function, growth rate and overall condition. At 28 weeks into the pregnancy, I began having contractions and was placed on modified bed rest for Zachary’s safety and mine. We continued to have regular check-ups with the perinatologist and pediatric cardiologist and were scheduled for tours of the NICU and PICU and to meet with the cardiothoracic surgeon to begin planning for Zachary has continued medical care. At 32 weeks, we were told that Zachary would have to undergo several open-heart procedures at various stages of his life with the first being shortly after birth and the risk factors involved could include death.
We met with the perinatologist again at 34 weeks and were scheduled to have an amniocentesis to check lung maturity on January 21, which would have been 37 weeks and just on the edge of full term.
Zachary decided that he wanted to decided his own entrance date into this world; I started contracting on January 15 and was sent home from our local hospital on complete bed rest, as they believed the contractions had slowed with the aid of IV fluids for rehydration and the rest. However, to our surprise on January 16, 2008 while I was taking a nap at 3:30 in the afternoon I was awakened with severe contractions at 3 minutes apart. We notified the perinatologist in Las Vegas and were instructed to get to the hospital immediately. Zachary was delivered via Caesarean Section at 10:57pm, weighed in at 6 lbs.’ 8 oz., and was 18.5 inches long. He was taken to the NICU and had to be placed on a mechanical ventilator to assist with his breathing as his lungs were not matured enough to handle breathing on his own and in conjunction with his heart defect it was too much of a strain on his little body. An echocardiogram was performed and it was discovered that even though he had Hypoplastic Left Heart Syndrome it was a rare version and he had his own small left ventricle that was larger than the normal child with HLHS and a decision as to whether or not to try for a two-ventricle repair could be done. Zachary had been on medications for his heart to keep fetal circulation intact or the patent ductus arteriosus open to assist in circulating the oxygen to his body were stopped the next morning. Zachary seemed to be doing well and was tolerating things on his own and had even been taken off the ventilator. Everyone was hopeful and thanking god for this little miracle and then we were hit with a devastating blow and Zachary went into congestive heart failure and respiratory failure and had to be placed back on the medications and the ventilator and the cardiologist were called and Zachary was rushed to the cardiac catheterization lab for his first of many procedures called a balloon atrial septotomy. They used the balloon that is used in adults to open up blockages in cardiac arteries and pulled it through the atrial wall to create a hole to help circulate oxygen through his body and decrease pressures on his pulmonary arteries. Zachary was then transferred to the Pediatric Intensive Care Unit and was continued on the medications to keep the fetal circulatory system open. And then came the next blow that we were not prepared for and he was diagnosed with RSV, a respiratory virus that can be severe enough in an otherwise healthy infant let alone one that was already compromised with premature lungs and a congenital heart defect. His doctors continued to monitor his heart and lungs, continued the medications and continued to discuss plans for his first of many surgeries that had now had to be postponed until a later date at which time his body was in a more optimal stage for surgery.
His doctors consulted with physicians from other prestigious hospitals and pediatric surgeons to discuss treatment options. We relied on our faith and trust in god to help us make the right decisions and to touch Zachary’s body and begin the healing process to help him gain strength for his surgery. At 3 weeks old, the decision was made by his doctors and me to do a relatively new procedure called the hybrid procedure, which gave him a 50% chance of survival versus the 5% chance he was, facing with the traditional Norwood procedure which is used traditionally to repair a child’s heart with HLHS. Therefore, the next morning Zachary was taken to the cardiac catheterization lab once again and a stent was placed to keep open his PDA or patent ductus arteriosus followed 2 days later on February 7 with an open procedure to place bands around his pulmonary arteries to restrict blood flow and decrease his risk of congestive heart failure again. He returned from surgery with his chest open and covered by a sterile dressing with which it remained for 3 days at which time the swelling in his body and heart had gone down enough to close his chest safely and began weaning him off the ventilator. At 4 weeks old, he was breathing on his own and now began the task of teaching him how to take a bottle and tolerate eating without burning more calories than he was getting. He had a temporary feeding tube through his nose and was given formula this way when he was not able to take enough from the bottle and his weight was monitored very closely and there was a discussion as to have a feeding tube placed or not for home tube feedings. His father and I were both opposed to this at that time and he was taken home at 6 weeks with a temporary nasogastric feeding tube, which I would take care of since I was and am an ICU nurse. The feeding tube was removed the next week after his follow up appointment with his pediatric cardiologist as it was determined he was taking enough food via oral feedings from his bottle and gaining weight. All seemed well and everyone was pleased with his progress, but one week later, he was back in the hospital with congestive heart failure and required medications to help him maintain an adequate fluid balance with which he was taken home on called Lasix. He continued to do well and at 3 months old, he was scheduled for a cardiac catheterization to evaluate for the next stage in his surgical repair, and yes, if you are counting this is his third cardiac catheterization in as many months. It was determined at this time that we would need to wait for a few more months and repeat the heart catheterization to proceed with the next stage in his surgical repair.
At 6 months old in June of 2008 Zachary was once again taken to the cardiac catheterization lab and images were taken of his heart and cardiac pressures monitored and it was determined that he was ready for his Hybrid Stage 2 which would involve ligation of the PDA and removal of the pulmonary band and to have a Glenn Shunt and a Dames-Kaye-Stansel procedure performed. His surgery was scheduled for July and Zachary went home to wait for his next procedure. In July he was once again taken to the operating room, his Hybrid Stage 2 was completed, and he was once again in the familiar PICU on a ventilator. Everything appeared to be going well with the exception of his blood counts were high in regards to he was now diagnosed with polycythemia a condition in which his hemoglobin and hematocrit was too high and they needed to draw off extra blood. He also developed a blood clot that was lying in the pericardial sac that lines the heart around the insertion site of his special IV line called a direct RA line. He was weaned of the ventilator within a few days and all appeared to be going right on schedule as planned, but Zachary decided to throw a curve ball as all of his doctors and nurses have decided to call it affectionately as he had become septic and was placed back on the ventilator. He was diagnosed with klebsiella pneumoniae and aceinetobacter in his lungs, his right ventricle was beginning to shut down in relation to the infection, and the thoughts begin to roam that he may require a heart transplant to survive this. He was started on multiple antibiotics once again and we continued to monitor him for the next three days to make a determination as to whether or not he would need to be transferred and be evaluated for a possible heart transplant. Through continued prayers from family and friends and through continued faith and treatment by his doctors Zachary made a turnaround for the better within 3 days and was once again weaned off the ventilator and went home after 3 and half weeks. At eleven months old, we noticed that Zachary was not moving the left side of his body and he was scheduled for an MRI of his brain at which time it was discovered he had a stroke and therapy began.
At 15 months old, Zachary had regained all his function in his left side and began walking and the decision was made that at 17 months we would once again schedule a cardiac catheterization and discusses his third and final stage in his repair for his HLHS. The cardiac catheterization went as planned and Zachary was scheduled for surgery in September of 2009 prior to RSV season. Zachary’s surgery was done on September 22, 2009 a Fenestrated Fontan as it is called and he was once again in the PICU on the ventilator, but something was horribly wrong this time I could feel it in my heart and the nurse in me could see it as I watched the doctors and nurses adjusting medications and ventilator settings throughout the night and at 4am I called a friend of mine to be by my side. In addition, my darling husband could rest to be by my side later on throughout the night. In addition, in hopes she would tell me I was just letting my mind as a mother playing tricks on me.
His surgeon came at around 6:30 am and thought that Zachary was just playing games with us yet again and they continued to adjust medications and monitor his heart, and at 6:30am his cardiologist arrived and performed and echocardiogram at which time they were unable to detect the fenestration and his surgeon was called back to the PICU for another evaluation. Zachary was very sick at this time and the decision was made that they needed to open his chest back up at the bedside there in the PICU and the surgery team was called emergently to Zachary’s room. Upon opening his chest, it was discovered that the fenestration had clotted off and that they needed to perform a revision of his surgery and he was taken to the operating room. The revision of the fenestration was performed and a peritoneal dialysis catheter was placed as Zachary’s kidneys had begun failing and it looked as though he might require dialysis. Once again, my family and I relied on our faith and prayers and the dialysis catheter was never used except to drain fluid from his belly related to his liver also failing for a short period. After a week and a half on the ventilator, Zachary was finally weaned off and placed on oxygen via a nasal cannula to meet his oxygen requirement. After two and half weeks, we were able to take the little troublemaker home with oxygen of course. He remained home for 6 months at which time he was once again taken back to the cardiac catheterization lab to close off the fenestration. Surprise Zachary once again through his doctors his notorious curve ball and with the aide of his blood thinners had dissolved the clot in the first fenestration and he now had two, which had never before been seen. Zachary’s surgeon was then consulted and the decision was made to close one fenestration and then decided if it would be possible to close the second one at the same time. The decision was made that it was safe to close both fenestrations and Zachary left the hospital the next day with what are known as Amplatzer Closure devices in place, we affectionately call them his cuff links or space ships when looking at them on his echocardiograms. Zachary did well and then he began to complain of back pain late in the summer of 2010 and after many trips to the ER and pediatricians office by January of 2011 just 12 days prior to his 3rd birthday his pediatrician sent him once again to the ER where he instructed them to perform an ultrasound of his gallbladder . On January 6, 2011, Zachary was taken once again to the operating room for guess what gallstones and his gall bladder was removed. His 3rd birthday came without any difficulties, Zachary was growing and gaining weight without incident and his heart was working wonderfully. He played on a soccer team that summer and then in May of 2011 being a typical boy we were playing and he tried to do a flip and fell hitting the back of his head on our hard wood floors. He fell about 3 feet to the ground and I knew instantly that even though he didn’t lose consciousness that something was wrong and we rushed him to the ER. Zachary walked into the ER on his own two feet and talked to the doctors they were going to do a CT scan just because when Zachary stopped talking, wasn’t focusing on anyone or anything and began to have a seizure that lasted for 15 minutes. Zachary was rushed to CT scan and it was determined that he had a subdural hematoma or bleeding in his brain. He was transferred to a higher level of care and a neuro-surgeon was consulted for possible surgery. At 6 am the next morning Zachary woke up, looked at everyone in the room, and proceeded to tell us how hungry he was and that he just wanted some gummi bears. Zachary is now a happy and very healthy 4 year old, his doctors are very pleased with his progress. His heart continues to stay strong with each follow-up echocardiogram every 3 months, his last cardiac catheterization showed no complications and there is no indications at this time to say he will ever need a heart transplant. Zachary continues to defy the odd and exceed all expectations of his doctors, as he is an advanced belt in taekwondo and is excelling in his pre-k classes. Zachary continues to inspire me every day in everything I do and I thank god that I never gave up hope and continue to believe in god and prayer as I get to see his miracle work walk around in front of me on a daily basis and that little miracle tells me every day how much he loves me.