Thursday, July 30, 2009

Aquarium Visit

As some of you know, Gabby loves "fishies." Her favorite stuffed toy is a multicolored fish. Her favorite bathing suit has fish on it. And she loves to watch them swim. I recently took the girls to the Seattle Aquarium. They really enjoyed it, so I bought a membership.

Our friends, Tom and Gail, have been visiting from CA. They visit us every year on their way to Canada for their summer vacation. I decided to use my guest passes to take them to see the aquarium with the girls today. A fun time was had by all.
Our aquarium is a pretty nice. As you walk in, there is a floor-to-ceiling fish tank with many, many fish swimming in it. There are little benches to sit on while you watch. (I could sit there for hours sometimes. It's very relaxing.) I call it the Wall o' Fish. It's a name Jack would have liked. I even had Tom take our picture standing in front of said wall.

Another fun exhibit is of what happens when a wave occurs. This is a really long tank with a curve at either end. At the end where the wave is generated, you can tell that the wave will happen soon. Then, the wave is formed, and it travels the entire length of the tank to the other end, and we get to see what happens to the sea life under the surface as it rides out the force of the wave underwater. It's pretty cool. We were standing at the wave generation end. Every time Gabby could see a wave was coming, she'd say something like, "It's doing it!" and jump with joy and excitement. As soon as the wave came, both girls would just scream with laughter. They did this the entire length of the tank. When they saw the wave, they laughed loudly. Some of the attendees got as much fun out of watching my girls as they did watching the fish, if not more!

They also got to play in the tidepools, but they were more interested in playing with the water than touching the starfish. We also saw lots of neat fish in tanks. Of course, Gabby always wanted me to lift her up higher so she could see the fish better. And there was the obligitory visit to the gift shop. Sarah and Gabby both got a new book each, and Tom got Gabby her very own stuffed Nemo which is now a prized posession.

I've promised Gabby a fish tank after we get back from our CA vacation next month. I think she'll really enjoy it. I already have a place picked out where we can all enjoy it.

Sarah hamming it up

Friday, July 24, 2009

She could just be short

This was the week for the visits at Children's regarding Gabby's shortness. We've seen both genetics and endocrine this week.

Our visit with genetics was very reassuring. While she does have the heart issue and some gross motor delay and short stature, they don't think that there's any kind of genetic component here. Additionally, some of Jack's relatives were shorter than me, so it could just be that she was going to be short regardless of other issues that she might have. The fact that she is cognitively on track is very reassuring and points away from a genetic problem. Relief number one.

Today we saw the endocrine folks. According to them, she's growing at a good rate. Her growth velocity is in the 10th percentile, and she's growing on her own growth curve. While it's below the 3rd precentile, she's still on the right curve. Her weight is also maintaining well. They are certain that she doesn't have a growth hormone issue. In fact, the doctor says that she is on track to be my height or a little taller. That's not what her pediatrician told me, and I asked about it, but she was sure. What they really look at is how fast she's growing, and she's doing fine in that department.

So, while Sarah is really tall for her age, Gabby is just short. It's not a problem medically. She's just going to be short like much of the rest of her family. What a relief.

Gabby's Medical Journey Part 3

As I said in the last post, Gabby came off ECMO after a week. I was finally able to hold her after she came off the ventilator a couple of days later. She remained in the ICU for about a week after she came off ECMO then went to the floor then came home 3 1/2 weeks after she was admitted. She came home on several medications and a tube feeding. The tube feedings only lasted less than a month. She needed those so she could get enough calories to heal from her surgery and grow at the same time.

At first, we had fairly frequent clinic visits. Those became fewer and fewer as time went on. She slowly recovered from what is now referred to as a "stormy course." She's on absolutely no heart medications. What we are looking at now is that she has no valve between her right ventricle and the artery that goes to her lungs. She will eventually need a valve placed there, but not yet. They don't like doing it until they must because once they "open that door" she will need the valve replaced about every 10 years or so. She will also need to have the stent that is in her left pulmonary artery removed, and the artery will need to be repaired again. The stent has been expanded as far as it will go, and it will eventually not be big enough. She will need lifelong follow-up with a cardiologist, hopefully one who specializes in adults with congenital heart defects.

Gabby has also had problems with meeting her gross motor milestones on time. That's things like crawling, walking, jumping, etc. She's been in physical therapy off and on for more than a year to address those issues. She was in therapy through the school district for a few months and made great strides as a result. When she started in Feb. or March, she couldn't jump. Now she jumps all over the place. She will probably go back in the fall. I've also had her in gymnastics through the YMCA which has been a huge help. Between the two things, she really improved. This summer I have her and Sarah enrolled in beginning swim lessons.

Overall, she's doing very well, especially considering what she went through as a baby. I keep hearing from her daycare that she's really smart, and I can see it as well. She can keep herself quietly occupied playing by herself for a long time, especially for a 3 year old. We're fortunate that she hasn't had any problems beyond the gross motor stuff, and she adapts to that and is making improvements. I am so thankful for that.

Saturday, July 18, 2009

Gabby's Medical Journey Part 2

Gabby had her corrective surgery for her tetralogy of Fallot (ToF) on 1/5/06. Sending her off to surgery was one of the hardest things I've had to do, but I knew that she needed the surgery in order to live. The wait seemed to take forever. There was another family there that was waiting for their son to go in for his corrective heart surgery. They had the same surgeon. They told us that the case before theirs was taking longer than expected. Our comment to them was, "That's our kid that's the first case." This was not a great feeling. Finally the doctor came out of surgery. He took us into a conference room to discuss what was done. I don't remember the conversation in all of its detail, but parts of it really stick out in my mind. He went over what he'd done in surgery. I asked whether or not he was able to save her pulmonary valve, which is the valve that's between the right ventricle and the artery going to the lungs. His comment was, "Oh, I destroyed that." Jack never forgot that comment and how he seemed to just take it as part of doing the surgery. He told us that when they were measuring her outflow tract, the 2 surgeons looked at each other across the table and asked each other how she was doing so well with the defect she had. The tract is supposed to measure 10 mm, and hers measured all of 2 to 3 mm. He also told us that "She's going to get sicker in the next 12 to 24 hours. I don't know how sick she's going to get, but she's going to get sicker." He then went over some of the tools that they had when this happened. His words turned out to be horribly prophetic.

After they got Gabby back to her room in the ICU and settled in, we were finally allowed to go in and see her. She was so swollen, I almost passed out. It was tough to see. I remember her nurse apologizing for her IV lines being such a mess, "but we've had more important things to worry about," she said. To which I replied, "Like what?" She answered, "We're having a hard time controlling her blood pressure." (To any parent whose child is going through this, if a nurse says this to you, stick around!) It had been a long day, it didn't seem like we could do much there, and Jack had to get Sarah out of daycare before they closed. We decided to head home in our respective vehicles. We told them where we were headed. In hindsight, I wish I'd stayed close, but no long term damage was done.

A quick diversion: At the time, we were living in the town of Redmond. For those who aren't familiar with the Seattle area, Redmond is located at the other end of a freeway that starts in Seattle called Hwy 520. Part of Hwy 520 consists of a floating bridge that goes across Lake Washington. Lake Washington dominates the Seattle area and is miles long and 1 or 2 miles wide. It's between Children's Hospital & the town of Redmond. There's the Seattle side and what's known as the eastside. I'd guess that the 520 bridge is a mile or 2 long, and there's nowhere to go if there's a problem.

So, I was headed home in my car. Jack was on his way to get Sarah. Then my pager from the ICU beeped. I was stuck in commute traffic on the 520 bridge, driving in the rain, when I was paged. I immediately called the ICU on my cell phone. The guy tells me to pull over after I told him that I was driving. "I can't! I'm in the middle of the 520 bridge!" I tell him. He says, "Pull over as soon as you can." About this time, panic is beginning to set in. I demand some sort of explanation, and he tells me that they need me to come back to the hospital. "Why?! What's happened to my baby?!" I demanded. He got one of the nurses on the phone for me. She told me that Gabby had coded, they'd needed to do brief CPR, they had her back, but they needed to start her on ECMO. Yikes! This was my worst nightmare. I'd heard horror stories about ECMO. Basically, it's a bypass machine that sits by the bed. The patient's blood has to be really thinned while they're on it, and that can cause all kinds of complications. I was terrified. I got back to the hospital in record time. Took the first exit, turned around and went back. I half ran back to the ICU. Different docs came out who I recognized, and I again found myself in that little conference room. The other surgeon who had been in on Gabby's surgery exlained everything to me. The only other option was to beat her heart with drugs, and they figured that her recovery would take even longer that way. While it was scary, of course I consented.

The doctors then turned her ICU room into a mini operating room and put her on ECMO right there. Pretty amazing, huh? I waited until they were done and had her cleaned up. (I later found out that the nurse who came on to take care of her while this was going on had no room to enter in what had been a large room because it was so packed with staff trying to save my child's life.) I went in to see her after they were done. It was even more tubes. (At the most, I counted a total of 12 lines going into her little body.) With all of the equipment in the room plus an ICU nurse and ECMO tech, it was pretty crowded. But I was told that they were able to stop some of the medications as soon as they started ECMO. The theory was that it would give the right side of her heart a chance to rest. ECMO (extracorporeal membranous oxygenation to use the full term) takes blood from the right side of the heart and takes the place of the lungs, then puts it back into the left side of the heart to be circulated to the rest of the body. It's used sometimes on really premature infants who have really immature lungs.

So she stayed like that for a few days. She was medicated so she wouldn't move, and I don't think she remembers anything or felt anything thanks to the morphine and ativan that she got in pretty good doses. She didn't even open her eyes. I'd come for hours twice a day, stand at the head of her ICU bed and pet her head for hours. It was the only place that wasn't punctured. And I loved the way her wavy hair felt under my hand. Before her surgery, when I'd wash it, it would feel so springy. Kind of reminded me of her father's hair.

They tried to take her off ECMO after a few days, and she failed miserably. They couldn't understand why. So, to find out why, they took her to the catheterization lab on ECMO. This was a major production. It took something like 6 people to safely transport her and the equipment. I called it the Gabby train. When I saw them taking her like that, that was one of the times that I fell apart because I realized how sick my little girl really was. Well, remember that trouble spot in her left pulmonary artery that I talked about in Part 1? It was a trouble spot again. For some reason, her left pulmonary artery (LPA) had collapsed, resulting in not enough blood getting to her lungs, which is why she failed coming off ECMO. They placed a stent, like they'd put in an adult's coronary artery, in her LPA. After that, she slowly started getting better. About 6 days after putting her on ECMO, they tried to take her off again, and it was really close. I've never seen a surgeon agonize over a decision the way her surgeon, Dr. Cohen, did that day. She was so on the fence in terms of coming off. Then one lab value went up showing that she was under some stress, so they decided to keep her on for one more day. The next day, she sailed through the trial of coming off, and they removed her from ECMO that day, a week after her corrective surgery.

To be continued

Gabby's Medical Journey Part 1

Gabby's heart defect, tetralogy of Fallot, was diagnosed during a "routine" 20-week prenatal ultrasound. My chances of having a baby with a genetic defect went from 1 in 84 to 1 in 3 instantly. We had the amniocentesis done a few days later, and we found out that other than the heart issue, everything looked fine. By the time she was born, I'd had a total of 8 ultrasounds, including at least 3 by her pediatric cardiologist. The funniest one was when he had to get her to move to get a better look at her heart. As he was re-scanning my belly to look at her heart, he started to laugh. I asked what was so funny, and he answered, "She just shook her fist at me!" That's my Gabby.

For those who don't know, here's a brief explanation of tetralogy of Fallot (abbreviated ToF). Tetralogy means 4 things. In this case, it's an aorta that goes into both the left and right ventricles (it's supposed to only go into the left one), a narrowed outflow tract in the right ventricle, which goes to the lungs (resulting in reduced flow to the lungs), an opening between the 2 ventricles, and overgrowth of the muscle in the right ventricle because it's working so hard to pump blood through the narrowed outflow tract. In Gabby's case, she also had an opening between the 2 atria, but it was kind of minor compared to everything else. What all of this causes is circulation of blood that is not oxygen rich. Not enough blood is getting to the lungs, and if the baby cries (and at other times), that outflow tract narrows even more, causing the oxygen saturation to drop even more since the body is circulating deoxygenated blood. At least blood is able to circulate thanks to the opening between the ventricles. There is a lot of shunting that goes on, which means that blood is forced from the right side of the heart to the left side. All of this results in what are called "tet spells" where the baby looks more blue than before or even loses consciousness due to lower oxygen levels in the blood. You have to try to not let the baby cry much, which can be challenging. This is the defect that a lot of "blue babies" have. They're the babies that look fine when they're first born, then a week or 2 later, they start looking blue. Wikipedia has a pretty good explanation, complete with illustrations. I'm just glad that she was diagnosed before birth so we were prepared.

Gabby was fine when she was first born. She had to be on bilirubin lights for jaundice for several days, just like her sister. She looked great at her first heart check-up. Then she started having tet spells. They were kind of scary. Diaper changes were challenging. We took her back to the clinic, and they started her on medication. During the first visit where her saturations dropped, I saw them drop into the 50's then come back up. The medication didn't help much. She was hospitalized the first time just before she turned 1 month old. She had her first heart cath during that stay. They found a narrowing in the left pulmonary artery, which goes to the left lung. They took a rotor blade to it and expanded it with a balloon in the hopes that it would last her until she was 4 months old, the goal age for her corrective surgery. It was not to be.

I've been known to say that the cath procedure gave us a less-worried Christmas. On New Year's Day, her 2-month birthday, she had her most serious tet spell to date. She woke up from a nap in the early afternoon crying. She was in her carseat in the stroller at the end of the table. We had just finished having lunch. (We had also found out that she had a problem with reflux. This did NOT help matters.) Anyway, Jack picked her up and started flying her through the air in an attempt to get her to calm down. Then she went kind of gray. Jack said, "I don't know if she's asleep or unconscious." I was immediately on the phone to get a hold of the cardiologist on call. I had Jack flick the bottoms of her feet to see how unresponsive she was. It made her cry. That was better to hear than nothing at that point. When the cardiologist heard her cry, she had me calm her down (breastfeeding rocks for this) & transport her to Children's since we were only 20 minutes away and she was more conscious than she'd been. I came very close to calling 911 that day, though.

When we arrived at the ER less than an hour later, I was amazed at how quickly we were ushered in. They were expecting us and knew that she needed immediate attention. As soon as we got to an ER room, they had her hooked up and monitored. She was fine for awhile. But they still wanted to admit her for observation at that time, but they weren't sure if it would be the floor or the ICU. We also had Sarah to care for, and she wasn't quite 2 yet. Keeping her occupied wasn't easy, so Jack took her home since we didn't have a ready babysitter to call at the time. As I was sitting there in the ER with Gabby later, I saw her oxygen sats start to drop again. I quit looking when it hit about 40. All I could think to do was pick her up and hold her with the thought that if she was dying, at least she'd die in my arms. (The cardiologist said that the lowest she saw was 38.) This was not good. She bought herself a bed in the ICU that evening.

One of the options that the cardiologist said we had was to maybe sedate her and put her on a ventilator so they could better regulate her oxygen levels. But it was an option, not a treatment plan just yet. So, when we got to the ICU just after 8 that evening, I noticed a bunch of medical personnel in scrubs gathered at the door to the ICU. Being a nurse, I knew that something was up. I asked, "What's going on?" The doctor said, "We're going to intubate her." At that point, I put my hands up and said, "Wait. I was told that this was a possibility, not definite. I haven't signed a consent for this. I need to talk to my husband." Now, I wasn't being foolish here. I was watching the monitor and saw that her saturations were in the 80's and had been there for awhile. I wanted to be sure that this was really necessary. They were willing to wait, so it was obviously not an emergency. I called Jack. He wanted to know what her regular cardiologist, Dr. Lewin, thought of this. The guys in the ICU got him on the phone for me which really surprised me. Dr. Lewin said that he'd trust this doc with his child's life, and if he said this was what needed to be done, then it probably was. So, I told them that it was ok to intubate her and put her on a ventilator, but I wanted to nurse her first. The doc then asked, "When did she eat last?" I replied, "About 8:00 in the ER." He told me that she couldn't eat for 4 hours beforehand, which was even more proof that this wasn't an emergency. So, we waited until midnight when the same bunch of folks in green scrubs gathered at her door. I looked at her monitor, and it was at 88, which is where it had been since we'd been admitted. I asked the docs, "Her sats have been in the 80's since we got here. Is this really necessary?" I was then told that the fellow was checking with the attending for that very reason. She wound up not having a tube down her throat until her surgery 4 days letter. The lesson here is to always advocate for yourself or your child if you don't understand why something is being done.

To be continued

Friday, July 17, 2009

The New Gabby Blog!

I have decided to start this blog as a place to talk about any health issues that Gabby might have. As many of you know, she was born with a congenital heart defect called tetralogy of Fallot on 11/1/05. In addition to her heart issues, she's been delayed on almost all of her gross motor milestones, and she's very small. (At the age of 43 months, she was the size of a 27 month old.) In the next 16 days, she has 3 appointments at Children's Hospital of Seattle. The first 2 (7/21 & 7/24) are to evaluate her short stature in both the genetics and endocrine departments. Then she has a cardiology follow-up complete with chest x-ray, EKG & echocardiogram on 8/3. At this time, she has cardiology appointments every 6 months. I have no idea how often, if at all, we will follow-up with the other departments.

I intend for my next posting to be about what has happened with Gabby thus far. It's been a long and winding road, but she's been doing well for some while now, which, is, of course, the good news.

And other postings will talk about life in between medical stuff. Fortunately for us, the medical stuff doesn't happen anywhere near as often as it used to. While she may have some medical issues, she is a bright, energetic, smart kid. Plus she has a sister, Sarah, who is 20 months older than her and who also has her own adventures! I'll talk about them here, too.