Rylee’s journey with Tetraology of Fallot

When my husband, Jason and I decided to have a third child, we never expected to have one with medical issues. We had two boys, who were perfectly healthy pregnancies and deliveries, and never expected anything less with our third. Little did we know that there was something else in the cards for us.

My pregnancy with Rylee was by far my most difficult. I suffered from severe heartburn, and lost weight in my first trimester because I was so sick and unable to keep much down other than water and bread. Gradually, the pregnancy got better, but in my last trimester, we found out that she was breech and that my blood pressure was up. It was not significantly high, but because I had never had issues with blood pressure before I was put on bed rest. Rylee was delivered via c-section on February 7, 2007. She weighed 7 lbs 10 ozs and was 18 inches long. When I heard her cry for the first time, I thought her voice was a little hoarse sounding, but I have a naturally scratchy voice so maybe she was going to have a voice like mine. When she was brought into the room, after I was brought out of recovery, I noticed that her hands and feet were really purple. I asked the nurse about it, and was told that they are the last thing to get circulation and that everything was fine. As the day progressed, and I noticed that her hands and feet weren’t really getting any better and that she wasn’t eating well or urinating, my “mommy sense” kicked in and I really thought something was wrong. Again I was told that everything was fine and not to worry. I tried to relax.

I let them take Rylee to the nursery that night, since I was unable to get out of bed without assistance and take care of her. The following morning, my husband went to get me a cup of coffee and I was alone in my room. There was a knock at the door, and a nurse practitioner came in and introduced herself as the Neonatologist nurse practitioner. She said that overnight Rylee’s breathing had become labored and her oxygen saturation levels were low. She then told me that they thought that she had a heart defect. I heard nothing past heart. I just burst into tears. This couldn’t be happening to my family. We had done everything right, and the pregnancy was more difficult than my first two, but not enough to think that something bad was going to happen. When my husband returned to find me sobbing, I told him he needed to call the nursery because they thought something was wrong with her heart, and that’s all I could say over and over, “It’s her heart. It’s her heart.” The actual Neonatologist came in after my husband called the nursery, and explained everything in more detail. He said that he thought she had a congenital heart defect known as Tetraology of Fallot, and he drew us a detailed picture showing us what the problems were with her heart. He had an echocardiogram team come from Driscoll Children’s Hospital to verify. Once they verified that it was indeed TOF, she was transported to Driscoll in Corpus Christi, TX, less than 24 hours after she had been born. I wanted to curl up and die when they came in to take her away. My child was going to another hospital three hours away, and I couldn’t even get out of bed to go with her. I was terrified not knowing what was going to happen to her, and the fact that I couldn’t go made it ten times worse. What if she didn’t make it and I was stuck here in this hospital bed? It was the hardest thing I had to do, but I knew it had to be done. Jason was going home to gather some things, and then heading to Driscoll to be with her and my family was coming to the hospital to be with me. That night was horrifying and I was unable to sleep or stay calm. They finally gave me something to help me relax, and I was able to sleep.

The following morning, my doctor came in and told me that he was so sorry that this had happened to us, and that no one had seen this coming. I still struggle with this because during my pregnancy, I had 8 ultrasounds. One of them was to specifically look at the heart, and nothing was ever detected. We were completely blindsided. My doctor told me that he was reluctantly going to let me go, even though I really needed to stay another day to recover. I told him that I was going even if I had to sign out against medical authority. I went home to gather my things to head to Corpus and to see my precious boys before I left. My boys were 5 and 3 when Rylee was born, and they had never spent more than one night away from me and my husband. It was important to me that I let them know that things were okay and that Mommy was fine. Telling them good-bye that day was so hard. I didn’t know if their sister was going to come home, and when they asked about her I didn’t know what to say. I told them she was sick and that she was going to be in a special hospital for sick babies, but we would have to wait and pray. When I got to the hospital in Corpus Christi, many members of our family were already there with my husband. Rylee was in the NICU (Neonatal Intensive Care Unit). Jason had already spoken to the cardiologist and the cardiothoracic surgeon. I wanted to see Rylee. That is all I was worried about was seeing her. I went into the NICU and saw her twice before they took her off for her first heart surgery. She was barely two days old and already had to face a surgery.

Her first surgery was a coarctation of the aorta repair. Her aorta is narrower than it should be, and also had an obstruction in it. This is the reason that she wasn’t going to the bathroom like she should because she was getting inadequate blood flow to her lower body. Dr. Mark Bielefeld went in through her left shoulder, on her back, and cut the obstruction out of her aorta, and then reattached it. After surgery, she was placed in the PICU (Pediatric Intensive Care Unit). When we were allowed in to see her, I was totally unprepared even though the nurses had told us that there were lots of tubes and things. I walked to her bedside, and burst into tears. You could hardly see her for all the tubes and cords going into her small body. Things started to go dark around me, and one of the nurses grabbed me and forced me to look at her while repeating over and over again, “She’s okay. It looks worse than it is. She’s okay.” Thank goodness for Joann. I don’t know what I would have done without her. She calmed me down, and kept me from passing out. Despite this, I still couldn’t look at Rylee like that. I am haunted by the fact that as her mother, I couldn’t even look at her. It broke my heart to walk out of there feeling that I had failed her as a parent. I should have been able to look at her for hours and hours, but I couldn’t even stay for five minutes. I sobbed and sobbed trying to come to terms with all of this.

Rylee recovered from that surgery really quickly, and by the next morning had almost all the tubes removed. Now, I could face her more calmly. I sat by her side for hours that day just touching her little body, and holding her hand. We knew that she was going to need open heart surgery to close her large VSD (ventricular septal defect), but the plan was to do it at three months, so she had time to grow. Rylee was released from the hospital just four days after her surgery. We were so excited to bring her home! She still had a very weak cry, and would tire very easily while eating. She also turned almost black when she would get very upset because of the large VSD she didn’t get fully oxygenated blood. TOF babies are often called “blue babies.” On top of her large VSD, she had a double outlet right ventricle (DORV). She also has an Atrial Septal Defect (ASD). She came home on a diuretic regiment but no cardiac drugs. One week after she was released, we took her back to the hospital for a follow-up appointment, and I mentioned that her cry was still weak and she tired easily while eating. Dr. Bielefeld ordered a swallow study to see if she was aspirating fluid into her lungs while eating. We found out that she was, and they thickened her formula with cereal. Unfortunately, the amount of cereal that we had to add to keep her from aspirating also made it even more difficult for her to eat. She could eat maybe one ounce before she was completely exhausted. I was keeping in close touch with the speech pathologist at Driscoll who had done the swallow study, and I called her to voice my concerns a little over a week later, and she asked that I bring Rylee in for another evaluation. My mother and I took her to Corpus Christi again, and while she was there, they decided to put her in the hospital for observation because she was losing weight rapidly. My little angel had only been home for fifteen days and here we were already back in the hospital. Rylee cried all night the night that they put her in the hospital. She was completely inconsolable, and nothing they gave her would calm her down. She also wouldn’t eat for them. The next morning, while the doctors were rounding, I told the nurse that I thought something was seriously wrong. Rylee’s color looked off, and she was really struggling to breathe. At first, I thought it was because she was so exhausted from not sleeping all night, but in my heart I knew it was something much more. When the cardiologist walked into the room to look at her, he listened to her and said, “Call the PICU now.” My heart dropped, and I started to panic. They took her down the PICU immediately, and I went right in with her. They analyzed her condition, and told me that she was in heart and respiratory failure, and that she needed to be intubated to take some of the pressure of breathing off her heart. I stepped out so that they could do what needed to be done. When I was allowed back in, I was shocked by her appearance. She had to be given a paralytic in order for them to intubate her, and seeing her not moving and her little eyes not closed all the way, was heart wrenching. The doctors all told us that she was going to have to have open heart surgery immediately.

Rylee had her open heart surgery on her one month birthday, and the real journey began. She came out of that surgery with her chest open, and for someone who had had so much trouble the first surgery, this didn’t even faze me at all. I could see her heart beating inside her chest, because the bandage they covered it with was skin colored, and almost translucent. It was surreal. She had to be externally paced for a few days after surgery, but that was quickly taken off and she was on the road to recovery, or so we thought. Rylee had many, many hiccups following her open heart surgery that resulted in her being in the hospital for almost four months. Rylee developed a chylothorax, a leak in her lymphatic system that caused her chest to fill up fluid. She had a chest tube inserted to drain the fluid, and was placed on a different formula that went straight to the kidneys to be broken down rather than through the lymph system. The chylothorax was found only after she had failed an extubation attempt. It was great to get to see her face without tape holding a tube in her mouth, although short lived. My husband had to get back to the boys and to work, so I was left all alone at the hospital with Rylee. I spent everyday there all day, only breaking to eat and use the restroom. I spent twelve hours a day at her bedside. The first few weeks were really rough. Rylee would have spells where she would turn purple and almost stop breathing, and the respiratory therapist would have to bag her back up. She had to be on several different narcotics to keep her calm. I would count down the tubes everyday, and keep track of how many we had lost. Every day that we lost an IV or tube was a good day for me. Slowly, I was beginning to see Rylee under the maze of wires. Rylee was getting stronger everyday, and there was talk of once again extubating her. I was excited for them to try again. I was asked to leave while they extubated, so I was waiting in the hall. One of the residents that I had become very close to came out, and I knew that something was wrong. She led me into a private consultation room, where I collapsed in tears. She told me that Rylee was just struggling to hard and that they had to almost immediately re-intubate. I was supposed to come home to go to my six week checkup the next day, and I couldn’t bear the thought of leaving her alone. I went in to see her again, and she was paralyzed again and sleeping, so I went to my room to gather myself. I was hysterical at this point, but I held it together until I got to my room. I made it through the door, and I collapsed to the floor in tears. I couldn’t stop crying. I was so scared! I thought this was going to be easier. I had seen so many other babies in the PICU have heart surgery, and recover and go home. Why wasn’t this happening for Rylee? My husband convinced me to come home that night, and spend the night at home with my family before my doctor’s appointment. As much as I didn’t want to leave Rylee, I knew that I had to go for my own sanity. It felt so good to be home with my boys, but I knew that Rylee needed me more than they did right now.

At my doctor’s appointment, he asked me how the baby was doing and I burst into tears. It seemed like all I did lately was cry, and I am not a crier by any means. I asked him to give me some medication to help me deal with all the emotions that I was having, and he told me that that was a very good idea because once Rylee actually came home, my postpartum might begin. He thought that everything I was dealing with now was keeping my postpartum from actually beginning, and that once things settled down I might crash.
Armed with a prescription for myself, I headed back to Driscoll. Rylee had an uneventful night with me gone, which was a huge relief. She was moved out of the most critical spot in the PICU where she had been for almost six weeks to a different bed. It was a relief to know that she had come down from such critical status. Things were calm for a while, no major changes and they tried to extubate again, and she failed again. They did some further testing to try and determine why she was having such hard time breathing on her own. They did a heart cath, a fluoroscope on her diaphragm and many, many chest x-rays. They moved her again from the bed she was in to the back of the unit. Everyone says it’s the last step before you leave completely, but that didn’t hold true for Rylee. We spent another five weeks in the PICU. They decided to give Rylee a g-tube for feeding, so that she could get adequate nutrition and gain weight. This surgery was done laproscopically, but she ended up with a perforation in her stomach that resulted in her having to be cut open the day after to repair it. I wanted to know why she was unable to breathe on her own. The doctors did a bronchoscopy, and discovered that Rylee had a lung disease known as bronchomalacia. Her bronchial passages were floppy, and wouldn’t stay open without the pressure support of the ventilator. It was decided that we would attempt another extubation. We extubated, and everything seem to be going well. I was at Rylee’s side, watching her sleep, and she woke up screaming and her oxygen sats plummeted. The doctors came in, and tried to put her on CPAP but she was too far gone for it to be successful, so once again she was intubated. After much thought and discussion with the doctors, we decided that we would give her some time to gain weight before attempting again. She was only 8 lbs at this time. The only thing that could help her bronchs was time for them to harden on their own, but the doctors thought she could breathe on her own despite the floppy airway because she had done it before. Now, it was a waiting game.

We came to day 60 of being in the hospital, and things were not going very well. Rylee was having fits, dropping her sats, and being cranky. We were supposed to extubate the next day, but I was too uneasy thinking of it. I spoke to one of the doctors, who ordered all kinds of tests to see if we could determine what was making Rylee act this way. I t was determined that some of her sedation meds had been weaned too drastically and she was going through withdrawal symptoms. They adjusted the meds again, and she seemed to rest a little easier. This would be our last extubation attempt, and if this did not work Rylee would have to have a tracheostomy. I struggled and struggled with the decision that I had to make. Did I let them try to extubate one more time and watch her fail, or did I just call it quits and sign the consent for the tracheostomy surgery? My husband told me that I knew what was best for her and he would follow whatever decision that I made. No one knew her better than I did. I went in with the intention of letting them try one last time, but I watched her struggling to keep her sats up after they weaned her vent settings a little bit and I knew in my heart of hearts that she wasn’t going to be able to do it. I asked for the consent for surgery because I knew it was what was right for you. No matter how hard the decision was for me to make, I know it was the right choice.

Rylee had her tracheostomy on her three month birthday, and day 66 in the hospital. She looked so beautiful when she came out of surgery. Her little face was angelic. She took to a pacifier right away, which was a shock considering she hadn’t had a bottle or anything in her mouth for months. The doctors anticipate another week in the PICU, then we would go up to the floor and then home. It seemed so unreal. We had to learn how to take care of the trach, and change it. It was overwhelming, all the information that we had to absorb in order to bring her home. We are going to take it one day at a time, and trust in our abilities as parents to make this work. Rylee moved up to the floor, and spent two weeks up there while we learned all that we needed to know, and got all her medical equipment and nursing for home lined out. Rylee was finally released from hospital on June 11, 2007. It was a short stay at home though because the medical supply company we chose changed a vital piece of her equipment when she got home, and it caused her lungs to become oversaturated and we ended up back in the hospital on June 13th. We fired that medical company, and got another one on board and went home again on June 18th. We went to Driscoll for a follow-up appointment on June 27th, and Rylee was put back in the hospital for a low heart rate. I didn’t think we were ever going to go home and stay home for longer than two weeks. Rylee ended up in the PICU, and had her third heart surgery to have a pacemaker placed on July 2, 2007. The doctors still have no idea what caused her to have third degree heart block, but the pacemaker was the only answer. It was also discovered at this time that Rylee had another chylothorax, so it was back to the special formula for her again. Rylee came home again on July 10th. We would have to wait and see how long we got to stay home this time.

Rylee was able to stay home with our family from this point on, except for a few overnight stays for outpatient procedures. She worked her way up from being on the vent all day when she first came home to off the vent all day while awake, and was finally able to come off the vent completely in November 2008. She turned two in February and started walking shortly thereafter. She is still behind in some of her daily activities and receives occupational, speech and physical therapy several times a week to help her overcome this. She still eats via g-tube at night, but is taking all her daily formula by mouth, and has recently started to take Stage 2 baby food by mouth three times daily. She had her trach removed in September of 2009. She talks all the time now, and is beginning to put sentences together. Overall, Rylee is a walking, talking, dancing bundle of joy that we consider our miracle baby. She has overcome so much in her little bitty life that you can’t help but feel blessed to know her. She has made our family complete, and stronger because of all that we had to struggle through to keep her with us. CHD’s affect too many children daily, and awareness and research needs to improve. Education is key to helping families deal with this. Rylee, her brothers, her father and I thank you for allowing us to share her story with you.

There are many things that parents of CHD babies have to deal with that no one else can begin to understand, but telling your story is the only way that you can help people try to understand. They will never know the feeling you feel when your child turns blue and stops breathing, or how scary it is to sit at their bedside as they struggle to survive with the chaos of an ICU around you. I learned more from being in the PICU for that short time with Rylee then I had learned in almost 30 years of life. There are certain things that still haunt me. I saw some many parents lose their children to CHDs while Rylee was there, and the sound that a mother makes when she loses her child often resounds in my nightmares. I hope that educating others, and raising awareness and research will keep other families from having to endure the hardships that my family has faced.

To all the parents of children with CHds, you are the strongest, most wonderful people. I have much respect for each and every one of you, and an acute understanding of what you are going through. Keep your head high, pray hard, and love your little one like no one else.