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Day 21 of 30 Days of Gratitude: I am grateful, grateful, grateful for Dr. Emani and his incredible skill to give Isabelle a ‘perfect repair’.

From the first time I shook his hand I knew that this man was going to be our surgeon. The mere fact that he called my husband back after he left a message with Dr. Emani’s admin that he wanted to make an appointment for us to talk about him possibly doing her surgery- that SAME DAY- amazed me. We met him and as he went over the various scenerios, he was very confident in the images he had seen of the echocardiograms that it would go well. He encouraged us to ask questions and of course (thanks to my Heart Mamas) I had a few. What are the chances for chylous effusions? Do you clip one of the sinus nodes? Could that affect her rhythm? I was told she had good rhythym and NO ONE was messing with her rhythym. 

He backed me up in the CICU when I told the attending I wanted her extubated after she was breathing room air. I knew that the odds of her having an oral aversion were high and would only get higher if they kept that tube in. The opinion was “Why not keep in a few days? She’s having surgery on Monday.” Me, “Get it out, I want it out.” Dr. Emani: “There’s no need for her to be intubated, we have a few days and I am comfortable with it coming out.” Attending: 0, Me: 1. 

The day of her Norwood was the most emotional day of my life. I knew about the preparation, that she was going to be on bypass and that her aortic arch had to be reconstructed. I knew the shunt would be put in. What I didn’t know is whether her heart would start beating after being ‘shut-off’ momentarily. I wasn’t feeling well almost a week out from the C-section and I saw Dr. Emani as I was leaving the restroom. I knew he was coming to talk to us so I practically ran him over trying to get into the family waiting room. He took us into the consultation area and  apologized for taking so long. I’m sorry, what was that?

Apparently her arch was so small, he had to create a new one. Technically, she has two arches like McDonalds but only one of them works. He explained that it took a while for it to be stitched correctly and then went on to go over details of the surgery. He told us he used a Sano shunt and how he used a newer way to attach it to the heart muscle without causing too much damage. Went on to explain the conduit, and why he chose the size he did. He was very pleased with how well everything started back up again and was able to close her chest. I asked him what size her arch was, because I had seen research that suggested a tiny arch didn’t have great outcomes. “She had the smallest aortic arch I have ever seen.” Alrighty then.

He then went on to say he found something that he didn’t expect while he was in there. There was some tissue in the wrong place cutting off her subclavian artery. Basically she had some duct tissue cutting off the artery that feeds the left arm. He could have left it alone but one of the side effects is having one arm longer than the other.  He decided that he would just create a new section of artery and fixed it up. The surgery took a total of 5 hours. I was expecting more like 8. And this guy is apologizing to us for taking too long. Really?

Our cardiologist is one of a few who read echoes for patients. She couldn’t wait to tell us how incredibly perfect Isabelle’s repair was. She had never seen a repair that was as well done as that one. Other doctors have said that to us too, but I was stunned to hear that from someone who reads echoes all day. Every time we had a clinic visit, Chris and I would hear how great her heart sounded. Now our job was to fatten her up. 

When it came time for her second surgery, we had an informal meeting by her bedside about the next steps. She would have a cath, and then we would schedule the surgery. Her cath was incredible. Seeing her heart illuminated as the dye ran through was magical. I wish I had images from it. Dr. Emani thought they looked great and she had a few collateral vessles but nothing major. We were good to go.

Once again I handed her over to him and once again she came back to us. Her shunt was taken down, various pipes were attached to other pipes, and now all we had to do was to recover and go home. We were out of the CICU the next day and went home after a week. Everyone was happy with how everything looked and we couldn’t wait to go home and start living. After a few days home we noticed a red bump on the base of her incision. Long story short, we ended up at the ER with a staph infection in her incision. 

When I saw Dr. Emani this time he was standing by her bedside shaking his head. He was upset at himself for not remembering that her body doesn’t like the dissolvable stitches that he used both on her Norwood and then on the Glenn. He promised he would fix her scar when he did the Fontan. He wanted to see everything to make sure she was healing. He knew everything that was going on. We ended up going home and then coming back after a night of profusely vomiting. Turned out she was having a reaction to the antibiotic. Were we ever going home? This is the moment, by the way, when I began to wonder if we were going to be able to  leave. 

We did, and since that time we have been in for a PEG removal and Mic-Key button placed. We have had a tear duct opened and her neurological development assessed. When I saw Dr. Emani walk into the cafeteria, you KNOW I had to talk to him. I drive my husband nuts. He calls me an ‘Emani Groupie’. Yeah. I admit it. I am enthralled with the man who saved our daughter’s life and gave her such an incredible chance at life. When I brought Isabelle over to see him today, she gave him a big smile and he was thrilled with how great she looked. He asked me how everything went the other day. I was thinking maybe he was referring to her tear duct surgery. Nope. He knew about her cardio-neurodevelopment assessment and wanted to know how she did. The man knows everything about his patients and is concerned enough to ask. Love it. I said to him “I don’t know what you did in there, but she has more energy than I could have imagined!” He laughed and said “Good! That’s good!” It is good, and we owe it all to him. 

When you are first told that your child needs surgical intervention in order to live, once you get over the shock, you want the best surgeon doing that surgical intervention. When I am told by numerous people how great Isabelle looks, how pink she looks, I know that he is responsible. One of the esteemed cardiologists on the floor enjoyed using her as a teaching moment. She pointed out to the med students how difficult it was to see her cyanosis. When I asked her why that was she said to me “That is what happens when you get a perfect repair.” 

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Successful snapshot of Isabelle’s happy personality!

Thank you, Dr. Ram Emani. Dr. ‘MacGuyver’. You have given us a child with boundless energy, a matching smile to that energy, and a chance to enjoy a relatively normal life. She can walk. She tries to run, we aren’t there yet. She goes up the stairs and down the stairs. She would do that all day if I let her. Isabelle is thriving in a way we didn’t expect, but are so grateful for. LHS

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Day 20 of 30 Days of Gratitude: I am Grateful to Dr. Lightdale

There was no question after Isabelle’s first few weeks at the hospital that we were in the market for some feeding issues. Not that we were looking for them, I had hoped that I would be able to nurse and have that bonding post-op. Nope. She didn’t have the stamina to nurse for the amount of time she needed to in order to get calories. The rate she burned calories astounded me. She would lose grams when I was sure she was going to gain. She kept vomiting. She had no interest in the bottle after 4 minutes. I would weigh her in the morning, hoping the numbers would go up. When they did I was elated- this means we’re ok! When they didn’t, I would panic and go over every intake, add up the volume, and make myself crazy. 

After four months of ‘failure to thrive’ and her inability to keep food down consistently, we were referred to Dr. Jenifer Lightdale. Isabelle’s NG tube had been pulled out so many times we had to come up with a different solution that was less invasive to her nose. She needed to start gaining weight so we could make it to her Glenn. At the pace she was going, it would be months and we didn’t have that long to wait. 

Dr. Lightdale examined her, asked us questions and halfway through the visit, Izzie got hungry. I had some breastmilk and gave her a bottle. Dr. Lightdale was interested in seeing how she ate. After watching Isabelle for a few minutes, she told us her thoughts. First thought: Isabelle had learned that she satisfied her hunger with the first round of drinking. After that, she didn’t need to eat anymore. She had trained herself to eat enough to be satisfied, and by four months, it is too late to try to teach them how to suck a whole bottle. Second thought: NG tube had to go. She recommended a G tube such as the PEG. Third and final thought: moments after Izzie ate, she began to cry and pulled her legs up. I couldn’t keep her snuggled to my shoulder as she writhed in obvious pain. The verdict: she was sensitive to certain proteins in the breast milk such as soy, eggs and dairy. WHAT? Not only will my daughter never drink a whole bottle but she needs a feeding tube placed in her stomach and I can’t have dairy, eggs, or soy until further notice. 

I had to give up all of those things if I wanted to continue pumping. I had already pumped quite a stash of milk was  devastated that we couldn’t use what I so lovingly squeezed out of my body. Four times a day. Sometimes more. Dr. Lightdale suggested we change her formula to a hypoallergenic powder called Elecare (aka The Most Disgusting Stuff on Earth). It would take about wo weeks for my milk to be completely hypoallergenic but we could still give her what I was producing at that time. I cried all the way home. 

She called me on my cell and just wanted to make sure I was okay. She told me she knew her instructions were overwhelming. She knew it was going to be hard to give up all of those things, and appreciated my efforts. She had given me suggestions on what to eat before we left the office and she asked me if I was okay. Dr. Lightdale was very confident this would work. She couldn’t have been more right. 

When Isabelle pulled her NG out for the last time in December, we decided that after she was treated for dehydration, we would schedule her PEG placement. Twelve hours after being admitted I was told I could give her just plain breast milk in case her stomach couldn’t tolerate the 28 calorie formula we had her at. I gave her a bottle with breast milk in it. She drank the whole thing. I was stunned. She did it again 4 hours later, this time with Dr. Lightdale sitting next to me. Dr. Lightdale was delighted. “She proved me wrong, she can drink a whole bottle! Let’s see how tonight goes and we will talk tomorrow. Isabelle took all of her required intake that night by mouth. I couldn’t wait to see how much she had gained the next morning.

She lost 30 grams. I was speechless. In true interstage fashion, she ate a lot and it burned off faster than her body could store it away. We scheduled surgery for that Thursday and began mapping out NPO protocol. After months of trying to get her to eat my daughter was getting a feeding tube. Did I do everything I could for her? Was this a failure on my part? Should we have held out just a little longer? Looking at her today I know what the answer is. 

Izzie gained steadily in preparation for the Stage 2 surgery and although she threw up more often than not, we were able to fatten her up enough. I stayed on the diet and found that I liked the coconut butter spreads and my body appreciated the non-dairy lifestyle I had adopted. I felt great and just had way too much peanut butter. All natural peanut butter, since the commercial brands have soy in them. By the way, soy is in EVERYTHING. From bread, to spaghetti sauce. Check the labels! 

The irony of the feeding tube is that it gave us more freedom. We were free from the nightmare of interstage weight gain issues. Free from measuring everything in calories such as “That cry lasted a good five minutes so that was 80 calories just wasted.” Free from worrying every day. Well, worrying about her weight gain anyway. We were free to give her solids and after her glenn we were free to give her just breast milk. No more Elecare, no more Polycose- just milk. And she started to drink from bottles again. We stopped using the tube for feeding in March. It was taken out in August. 

Our daughter is a chunky, lovey, baby who loves food and signs for ‘eat’ all the time. We are trying to get her say “food” but hey- at least we know what she wants part of the time! I was able to get off the diet after she was 10 months old and showed no sign of distress with the Before Diet milk. She is allergic to eggs right now, and there is a chance she will outgrow that eventually. When I think of how remarkable Isabelle is, I think of everyone responsible for helping her where she is right now and Dr. Lightdale is in the top three on my list of BCH’s AMAZING DOCTORS. Image

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Day 19 of 30 Days of Gratitude: I am grateful for the Cardio-Neurodevelopment Program at Children’s

I remember taking psychological exams, stacking blocks, looking at patterns and being asked lots of questions. After the tests were over, my parents would be told that I had something called Attention Deficit Disorder. It was a relatively new diagnosis and very few medications (if any) were prescribed for it at the time. An ed plan was designed and I made it through school. End of story. That was my neurological experience. Basically, I remembered the blocks. 

We brought our assessments and paperwork, and were instructed to wait in the lobby of Children’s (which has completely changed, by the way). Izzie is old enough to notice the musical sculpture outside Au Bon Pain and CVS. She loved looking at the fish and I had fun watching her point at each fish and laughing. I know what these doctors are going to say. They are going to tell me she should be speaking more. She should be understanding more. And she isn’t. I watch her delight as the fish swim by her fingers on the glass. Time to head up and play with some blocks!

She saw the toy car in the hallway and made a bee-line for it. We had to go into the boring office instead and she voiced her displeasure of being denied the car. We went over the paperwork, went to get some coffee and waited for the psychologist. A nice woman was waiting for us with a little desk and lots of toys. And notebooks. She asked us questions, asked Izzie questions, and as we went through each part of the assessment, we could see how much she had grown in the past few months. It was phenomenal to see her do tasks I didn’t expect her to be able to do. 

She can feed a doll, point to certain objects she knows, figure out how to get cheerios out of a bottle, and stack blocks like a champ. She can’t, however, say more than our names, or follow more than one step. Her expressive language is behind, which is what I had been thinking. She should be speaking more than a few words, and be able to make sounds that mimic names of objects. Just a new focal point for Early Intervention to follow, I guess.

The experience was such a mixed bag. At one point I wondered about the limitations I put on her without realizing it. Then I see what limitations she has and think about my other two (both were speaking at least two word sentences by this age). I think about my heart mama friends, whose children can’t sit up without support, walk independently, or share in our joy of going up and down the stairs after her. She truly is remarkable for someone who has has two open-heart surgeries and compromised circulation. One interesting fact we heard while we were there- at 15 months she already has a hand preference, and they think it has something to do with the circulation being different than a heart healthy child. Apparently, children who have gone through what she has tend to show hand preference early. [Cliff Claven moment over]

I am so grateful they have this set up for these kids, and I am glad we are participating. I think knowing where she is at comparatively to other cardiac kids is important for me to truly understand how fortunate we are. Yes, being alive is a good sign right there but she is doing more than living, she is thriving. I feel so relieved that her gross-motor skills are at an 17-month level. That rocks. Talking is overrated.