By Carla Bickel, mother of Alexa Bickel
When Frederick Sherman, MD, director of Fetal and Perinatal Cardiology at Children’s Hospital of Pittsburgh of UPMC and Magee Women’s Hospital of UPMC, said the words “I’m sorry,” it really hit me how serious the situation was. People don’t say those words if everything is okay, especially not doctors. I was 20 weeks pregnant with twins when we found out about Alexa’s congenital heart defects. The defects had been discovered during a routine anatomy ultrasound. Suddenly, we were being ushered in to have an echocardiogram done on her heart. She had been diagnosed as having Transposition of the Great Arteries, Pulmonary Stenosis, and Atrioventricular Septal Defect. We knew that day that she would need surgeries. When I think back to that day, it still feels overwhelming. To be honest, a health issue is the last thing I expected to learn that day. It is true that you never think it will happen to you. The truth is that heart defects that occur during fetal development affect 1 in 100 births.
On Jan. 26, 2011, we met with several doctors at Magee and then planned to head to Children’s to meet with the surgeon that would be operating on our daughter’s heart. Our last meeting at Magee was with a neonatologist. Certain genetic abnormalities are associated with an increased chance of having heart defects. If she had a chromosomal abnormality, it could be one where she died within the first week of life. We felt that the chances of miscarriage having an amniocentesis were way too high to risk, so we wouldn’t know until after she was born if she had an abnormality. Because of the unknown, the neonatologist walked us through how hospice care worked and what our options would be, just in case. She explained to us that they would take Alexa as soon as she was born and prepare her for transfer to Children’s. If the circumstances were dire, she warned that I may not even be able to see her before she leaves. The drive to Children’s was the longest drive of my life. I felt like I was hanging on by a thread.
As we waited for the surgeon, I had tears welling up. I felt like all he had to do was walk in and I would completely lose it. When Victor Morell, MD, chief of the Division of Pediatric Cardiothoracic Surgery at the Heart Institute at Children’s, walked in, the absolute opposite happened. For the first time since we learned the news, I felt like things really would be ok. He said that Alexa’s defects are severe, but not the most severe that they had ever seen. He advised that she would need three surgeries in the first three years of her life. He gave us statistics. He drew pictures. He wrote everything down. He told us that it would be emotional and difficult at times, but that it wouldn’t be horrible. He was confident and kind. He gave us hope. We trusted him immediately.
In April, I had been scheduled for a C-section at 39 weeks pregnant. Luckily, Alexa was well enough that I got to hold her for five minutes before she was transferred. Once she arrived at Children’s, she was given a room in the Cardiac Intensive Care Unit (CICU). My husband reported back that the nurses there were excited to meet her and were very loving towards her. It was so very hard to be separated from my newborn, but it was a huge comfort to know that she was being doted on. They even made a little bow for her hair. The doctors did an echocardiogram and confirmed that her defects were exactly as diagnosed when I was 20 weeks pregnant, when her entire body was 6 inches long. I don’t know how small that makes her heart, but I am always amazed when I think about it.
On April 29, at 2 weeks of age, Alexa underwent her first surgery. The surgery was the Blalock–Taussig shunt procedure. When all babies are born, their aorta and pulmonary arteries are connected by a blood vessel called the ductus arteriosus. The vessel allows oxygen-rich blood to mix with oxygen-poor blood. The vessel naturally closes after birth. Because of her defects, her oxygen-rich blood would always pump back to the lungs for more oxygen rather than to the body. Likewise, her oxygen-poor blood would never reach the lungs for oxygen. With the ductus arteriosus closing, there would not be enough mixing. Her body would not get enough oxygen. Dr. Morell and his team had to create a shunt to keep the blood mixing.
Nothing can prepare you for the sight of your child after surgery. There were wires and tubes everywhere. She was so swollen. She had a central line in her neck that was stitched in so that they could deliver medications quickly in case of emergency. The incision on her chest was half of her torso, since she was so tiny, not yet even six pounds. It was terrifying. I will never in my entire life forget how amazing the CICU nurse was. She explained everything. She showed us how they can tell if she is or is not in pain and that they would be sure she would be kept comfortable.
Five days later, we brought Alexa home for the first time.
I’d be lying if I said that the next 5 months were anything but emotional and difficult. When we were released, it was with a pulse oximeter that we had to keep her attached to around the clock. It monitored the oxygen level in her blood. Being in between her first and second surgery meant she was at a greater risk for sudden death. We were advised to keep her home as much as possible and to avoid crowds. We had a nurse come in two times a week to take vitals and do weight checks. We had appointments with the Cardiology group every other week. A few days after initially being released, Alexa was readmitted to tweak her medications due to decreased heart function.
As time went on, she wasn’t eating enough to be gaining weight like she should so her food was fortified to make it more calorie dense. Eventually, she stopped eating enough to gain and she was admitted to Children’s in July to get a nasogastric feeding tube (NG-tube). This is a tube that we had to change once a week or when it was pulled out that we put in through her nose down to her stomach. It was unpleasant, at best, for her. We literally had to hold her down while she screamed. When we fed her, we put her food in a syringe that had to hang higher than her stomach so that gravity would help it drain in. In order to get as much food in to her as possible, we had to continue to feed her as a newborn; every three hours. Unfortunately, after adding the feeding tube, she vomited all of the time. She was admitted just a few days after she received the tube to do a swallow study to make sure her food wasn’t getting in to her lungs. It was important that she be a good, strong weight for her second surgery, so even though she vomited with the tube, it still helped her to gain weight. She would vomit 4-5 times a day, every day.
We had been referred to the Feeding Clinic at Children’s to discuss her vomiting. Sylvia Choi, MD, felt that her stomach wasn’t pushing the food through at a normal pace, so she started Alexa on a medicine that would help her stomach pump, essentially by cramping. She also gave us a feeding pump so that we could put the food in her stomach slower. We still fed her every 3 hours around the clock and because the tube could possibly slip out of her stomach and in to her lungs, we had to stay awake for the hour each time that the pump ran. The vomiting decreased to 2-3 times per day. I know that almost always she just didn’t feel well, but she was gaining, and her second surgery was approaching. By mid-August, she was completely tube-fed and had developed severe oral aversions.
In September, Alexa turned 5 months old. We took her to see a pediatric gastroenterologist. The doctor we saw said she couldn’t address the vomiting until Alexa had a permanent feeding tube called a gastrostomy tube (G-tube). The G-tube would be inserted directly in to her abdomen and her food would be delivered right to her stomach. Before we could really address the tube situation, we had to be past her heart surgery.
On September 16, the doctors did a heart catheterization to prepare for surgery. To get to her heart, the doctor went in through the artery in her leg. As can happen after a catheterization, her pulse in her leg got very low. She had to stay overnight and was put on a blood thinner to keep blood circulating in her leg. Just two days later, we were back in the hospital as she had caught a stomach bug and getting dehydrated could have been life-threatening.
At her follow up cardiology appointment, we set the date for her next heart surgery – October 25.
I can’t tell you how excited we were that we had reached the highly anticipated second surgery in October. The irony of us looking forward to open heart surgery is not lost on me. Admittedly, it was unnerving that the surgeon would be stopping Alexa’s heart and she would be put on Extracorporeal Membrane Oxygenation (ECMO). My child’s heart would not be beating. Even now when I think about it, it freaks me out.
We were unsure which surgery she would have: the Nikaidoh procedure or the first step of the Fontan procedure known as the Bi-directional Glen. The Nikaidoh has a more difficult recovery, but results in a more normal structure, and therefore she likely would not need further surgery. If she had the Glen, the surgeon would change the way her blood flowed in her body. The blood from the upper half of her body would flow directly to her lungs for oxygen due to gravity. If she had the Glen, she would someday need a heart transplant. She would either have a surgery that made this nightmare essentially go away or she would have a surgery which meant a third surgery between the age of 2-3, potential intervention as she got older, and a transplant when her heart was too tired.
I carried her back to the operating room and stayed with her until she was asleep. As I headed to meet my husband in the surgery waiting room, it occurred to me that this would be the third time we waited in Alexa’s 6 month short life. When the nurse called to let us know that Alexa was almost done and would be transferred to the CICU, she assured us everything went well. However, she also told us that they had to do the Glen. I was devastated. Gone were the thoughts of Alexa having a normal life and back was the reality of a heart transplant. Someday my little girl’s heart will be discarded; thrown away. Dr. Morell came in to talk to us and I had clearly been crying. He was very reassuring again that everything would be okay. Alexa had come through with flying colors. Even though I was disappointed that Alexa’s anatomy didn’t qualify for the Nikaidoh, I think it is important to note that most heart surgeons don’t do it. Had we been at any other hospital, it wouldn’t have even been considered.
Alexa was released six days later. She actually was ready to go home after four, but we decided to try a nasojejunal tube (N-J tube) to see if that would reduce the vomiting. The N-J tube went down Alexa’s nose and in to her intestine. There are two major drawbacks of the N-J versus the NG. The first is that my husband and I could not put it in. Any time it was pulled out, we would have to take her in to Interventional Radiology for them to replace it. The second was that since intestines do not expand like the stomach, she would have to be on a constant drip of food. This included while she was sleeping which meant that she was sleeping with tubing in her crib. We did what we could to keep her as safe as possible, but it was not an ideal situation. I was terrified she would strangle herself.
Fortunately, her G-tube surgery had been scheduled for December 5, so it was only for a little over a month. It was very difficult living with the N-J tube. I was not sad to see it go. We headed to the hospital that morning for another surgery and snapped one last picture with the tube taped to her little cheek. I love that picture. It truly signifies moving forward. We had previously spoken with Kevin Mollen, MD, the pediatric surgeon who would be performing the G-tube surgery, and we all decided to go ahead with a procedure called a Nissen Fundoplication. Alexa had been suffering with severe reflux for months. It was controlled with medicine. In a Nissen, the top of the stomach is wrapped around the esophagus. This means the acid can’t get out of the stomach. It also means that Alexa would be unable to vomit.
The surgery went very well. Dr. Mollen’s team taught us how to care for her tube. During our stay, they worked with us to slowly increase her feeds. Since she had been getting fed via a drip to her intestine, we didn’t just want to overload her stomach. Until the standard post-surgery follow up in January, she had to stay hooked up to a farrell bag 24 hours a day. The farrell bag was a way her stomach could empty if the pressure was too much. It had to hang above her stomach at all times. It was difficult to be out and about with, but I was relieved her stomach had a way to empty if she needed to vomit. Alexa was discharged from the hospital on December 8. It had been her eighth stay in 8 months of life.
Three weeks after her G-tube surgery and the Nissen procedure, Alexa’s stomach started backing up in to her bag. Then she started spitting up, which should have been impossible. Eventually she started vomiting again. We were absolutely beside ourselves. The earliest we could get in to see a pediatric gastroenterologist was January 12. It felt like years.
Jeffrey Rudolph, MD, was worth the wait. To sum up his involvement in our lives, I will simply say he saved my sanity. When I first met Dr. Rudolph, I literally begged him to help us. I told him that I felt like I had failed Alexa when the first pediatric gastroenterologist we saw told us she couldn’t do anything. I teared up as I told him that none of us could handle the vomiting anymore, especially Alexa. We discussed her history and I could tell right away that he got it. He knew something needed to change. He advised that we would make small changes at a time and that it wouldn’t happen overnight, but that she wouldn’t live her whole life vomiting. He told me to call anytime I needed to.
And boy did I call. A lot. I knew I was being that crazy parent but I didn’t care. And luckily, neither did Dr. Rudolph or his nurse Erin. They were amazing. Dr. Rudolph would return my calls and really listen to what was going on. He didn’t rush to get off of the phone; we had real conversations. He ordered some tests and confirmed his suspicion that Alexa had Gastroparesis, also known as delayed gastric emptying. We also confirmed that her Nissen had become non-functioning. We slowly made changes and Alexa stopped vomiting. It was in March of 2012 when she finally had relief. She vomited daily for 8 months. I can’t imagine. Even now thinking about it makes my heart ache for her.
Even though Alexa had been receiving early intervention services to combat her oral aversions since 5 months old, she still wasn’t eating by her first birthday in April. I don’t blame her. She never felt well. I wouldn’t eat either. She also wasn’t gaining as well as would be ideal. We were at the absolute minimum intake. Any time we tried to increase just a little, she would vomit. Even though she was no longer vomiting, I didn’t feel like we were at our final solution.
I started doing research on the Internet and learned of what is called the Blenderized Diet among the feeding tube community. Essentially, you blend real food to a liquid state and give that through the feeding tube instead of formula. I read of the amazing effects that the diet had for other tube fed children and adults. Since food is heavier than formula, it passes through the stomach better. Also, bodies are designed to digest food after a certain age. My husband and I agreed that this would be the best next step for Alexa.
Unfortunately, I had read a lot of stories about how about 80% of GI doctors were not on board with the new diet. A lot of the parents from around the world that I spoke to did a lot of fighting about it with their child’s doctors and eventually had to do it without the GI doctor’s support. When I called Dr. Rudolph to discuss, I hoped that he would be supportive but knew we’d go ahead and try it either way. To my very pleasant surprise, not only was he 100% on board, he also knew of a study that was done that he mailed to me. In the study, there was a recipe that had been tested to completely meet nutritional needs. We talked about how to switch her over and he planned to call to see how it went.
It was now June 4. Alexa was just shy of 14 months. I was terrified to switch her over. In the past, even if we tried to increase a formula feed by 10 mL (1/3 of an ounce), she would vomit. We decided to do it very slowly and switch one feed at a time. I gave her the first blenderized feed and she didn’t throw up. I decided to give her the next feed as blenderized since she did so well with the first and she still didn’t vomit. She was completely switched over in just a few days and did not vomit one time. She was getting about 100 more calories and we eventually bumped that up to 300 more calories a day. It was crazy.
At this point, her heart was very stable and her feeding issues absolutely consumed our
lives. The blenderized diet is the best thing that ever could have happened for Alexa. She became more active and we could tell she felt better than she ever had. We started the diet on June 4. By June 16, she had started eating puffs. Just 7 months later, she is now 90% weaned from her feeding tube. We anticipate her being completely weaned by mid-February. True to his word, Dr. Rudolph called and followed up several times throughout the process. He also was very supportive about weaning from the tube.
Alexa is now 21 months old. I don’t know how, but she is a typical toddler. As I type this, she is naming the colors of a puzzle and claps for her twin brother when he fits the piece in. She will have another surgery between the age of 2-3, but I don’t feel anxious about it. I know I am biased, but I truly believe Dr. Morell is the best cardiothoracic surgeon there is and his team is just as great. I know she is in good hands. We have a follow-up appointment with Dr. Rudolph in March. I can’t wait to tell him she is 100% weaned from the tube. I know he’ll celebrate with us. I literally owe my daughter’s life and quality of life to the great doctors at Children’s Hospital of Pittsburgh of UPMC.