Our Journey with Twin-Twin Transfusion Syndrome
This is by far the longest, most detailed, and most personal blog post that I have ever written. This online journal is usually about flowers and events and styling and tea and other pretty things, but my reason for sharing this personal aspect of my life is twofold: for one, many of our extended family members and friends have wanted to know more about what we've been through these last days (as of today it has been less than 2 weeks since this journey began for us), and this seemed the easiest way to get the information out to the people who wanted it. But more importantly, I wanted to put this record, this account of a couple's experience with Twin-Twin Transfusion Syndrome, out into the world wide web, so that other couples who are faced with a TTTS diagnosis might come across it and find comfort and hope. We personally had never heard of TTTS before receiving our diagnosis and since it's so rare (there is an 1 in a 1000 chance of TTTS in any given pregnancy since it only effects 15% of identical twin pregnancies), I'm sure we weren't the first couple to get blind-sided by the news that our identical twins could have - would have - died without intervention. If another couple receives the same diagnosis and Googles TTTS and lands here and finds some solace in knowing this story, hurrah! There is always comfort in knowing that you are not alone. We are well aware that our story thus far has resulted in best-case-scenarios again and again and again, and such is not the case for many others- yet we strongly believe in the power of human intention and the ability that we have to create our own happy results just by willing them to happen. Receiving a TTTS diagnosis can be incredibly scary, but we are here to share that it doesn't have to be a horrific process. It can actually be a beautiful experience, and surviving such an ordeal can only serve to make us more grateful than we could possibly imagine for these tiny babes who will be joining us soon!
We welcome any communication from other individuals or couples with the same diagnosis who may have questions about our experience or just need someone else to talk to. We would love to provide encouragement and support so you don't feel isolated in this process. Please, please, if you are going through this too, do not hesitate to contact us. I've also tagged the names of our medical team so that if you are wondering about any of them we can assure you that each of the specialists who brought us through this are individuals whom we would highly recommend. We really had the best of the best!
To our family and friends who have loved and supported us through every step of this journey and continue to do so, we offer our immeasurable gratitude. It takes a village- not just to raise a child, but to get through a difficult pregnancy as well. Despite it all, we know we have much to be grateful for. So much.
The image at the top of this post is of our Baby A's hand, taken from inside the womb during her fetoscopy. Magic.
And now for the saga....
About a week and a half ago, our lives were suddenly turned upside down when our identical twin baby girls (in utero) were diagnosed with Twin-Twin Transfusion Syndrome. After our first appointment with our O.B. at ten weeks where it was discovered that I was carrying twins, we were referred to a high-risk fetal specialist who was able to determine at a mere 13 weeks that our babies were sharing a placenta and were thus identical (monochorionic) twins. We were told at the time that identical twins were particularly high-risk (more so than fraternal twins), and that we would be watched closely to make sure that the babies were growing at the same pace and getting their fair share of the placenta, but we didn't really know anything beyond that.
After my latest routine appointment with my O.B., my blood work showed a spike from <0.5% risk of Down's Syndrome to 1%. The state of California offers free genetic counseling for any pregnancy that shows a risk of DS, Spina Bifida, or other birth defect, so our O.B. wanted us to go visit our fetal specialist again quickly and meet with a genetic counselor at the same time. We were able to move our scheduled appointment up by a week by visiting a collegue of our previous specialist in a new office, and so we went in to see Dr. Joseph Ouzounian in Pasadena on Tuesday, November 12th. We first met with a genetic counselor who went over our medical histories, charted our family trees, and essentially determined that we didn't carry any of the common risk factors for Down's Syndrome babies. She explained how low our risk of DS was from the blood work, but suggested that if we were concerned we could have an amniocentesis performed to determine conclusively if one or both of our babies had DS. Sammy and I had both read about that procedure and knew that it carried a risk of miscarriage, so we didn't want to move forward with it when our risk level was so low. Since we were about to have another ultrasound and see Dr. Ouzounian, the counselor suggested that we also ask him to take measurements of the babies and see if he saw any reason to be concerned about DS.
Dr. Ouzounian and his sonographer spent a lot of time looking closely at our little babies, and Sammy and I were able to watch the ultrasound on a monitor, where it was pointed out to us pretty early on that there was a big size discrepancy between the babies. There had been a one-week size discrepancy at our very first O.B. appointment at 10 weeks, but that discrepancy had now increased to more than a two-week difference. It was quite clear to the naked eye that Baby A was significantly larger than Baby B. We asked Dr. Ouzounian if he saw any cause for concern for Down's Syndrome in the measurements he took and he said no, but he did see another cause for concern: apart from the difference in size, he could tell that Baby A had a lot of amniotic fluid to move around in, while Baby B seemed "shrink wrapped" in her amniotic sac with practically no fluid, and that she wasn't moving at all. He said this was the first sign of a disease called Twin-Twin Transfusion Syndrome. He said that he would be sending us to a fetal surgeon immediately to verify his findings, but that if the twins did indeed have TTTS, the disease would be fatal without intervention. He managed to still be incredibly encouraging while delivering this shocking news-- also explaining that we could undergo a fetal surgery that uses lasers to sever the blood vessels on the placenta that the babies were sharing, therefore allowing them to each receive their own individual blood supplies instead of funneling blood back and forth to each other without ever actually receiving what they needed. He said that the surgery had a very high success rate and that "we would take care of it!" He tried calling his collegue, Dr. Ramen Chmait (the fetal surgeon) while we were in his office, but Dr. Chmait was in surgery at the time. As we left Dr. Ouzounian's office, he assured us that someone from Dr. Chmait's office would call us within a few hours and that they would get us in for a screening either later that afternoon or the next morning.
Sure enough, by mid-afternoon I received a call from Arlyn, one of the nurses who worked with Dr. Chmait. She explained a little more about TTTS to me and encouraged me to peruse her office's website to find out even more of the details of the disease. She also explained that if we met the perimeters for the surgery we could be scheduled for surgery within a day because this is such an aggressive disease and the sooner it's cured via surgery, the better. Arlyn was calm, answered all my questions, and seemed very positive about the whole process. We scheduled an appointment for first thing the following morning.
On the morning of Wednesday, November 13th, Samuel and I arrived at Dr. Chmait's office in the Doctor's Tower at Hollywood Presbyterian Hospital. We were half an hour late to our appointment as I'd decided to use my phone's GPS for directions in navigating traffic and in my state of extreme anxiety I hadn't noticed until we were practically downtown that the GPS had mapped us to South Vermont instead of North Vermont. I think that morning drive was my most nervous moment in this entire experience-- I didn't have enough information yet, didn't know just how sick my babies were or if we'd be candidates for surgery or if our insurance would pay for surgery or... well... everything was unknown at that point. But from the moment we stepped into Dr. Chmait's office I felt all the anxiety leave me. We were greeted by Kris, another of Dr. Chamit's nurses. In retrospect, I don't think we would have gotten through all of this without Kris and Arlyn- they were both the most calming influences imaginable- so positive, so reassuring- it was like we knew instantly that they were on our side, that they were there to make this process easier, and that they would be there for us whenever we needed them. As we waited to go in for our ultrasound, we noticed that an entire wall of the waiting room was covered with birth announcement photos of identical twins and triplets who had obviously been saved from TTTS by Dr. Chmait and his team. It was an astonishing sight. Even though we still had no idea where we stood, I think that just seeing all of those dozens and dozens of precious LIVING little babies gave us immense courage. Katie, Dr. Chmait's sonographer, spent nearly two hours taking tons of pictures and measurements of our little babies using both external and internal ultrasounds. Kris stayed with us in the room for most of that time, talking to us, calming us, answering questions and explaining as much as she could about what our next steps might be. When Katie had finished, Dr. Chmait came in to do another ultrasound and look at Katie's findings, but because we'd arrived late he needed to rush off to another meeting. He told us to go get some breakfast and he'd meet us back in the office in an hour to explain everything to us.
After we ate in the hospital cafeteria, we went back to the waiting room a little early in anticipation of Dr. Chmait's return. Another couple was coming out from having been with Katie, and I heard them say that they didn't meet the criteria for the surgery. I wondered a little anxiously if we would, but then I heard Dolores, the Admin Assistant, on the phone asking my insurance for authorization for surgery. I knew then and there that we'd be proceeding. I figured she wouldn't be calling for insurance authorization if Dr. Chmait wasn't planning to tell me that I needed the surgery. Even though I still had no idea what stage of the disease our babies were at or when we'd be scheduled for surgery, I knew it was going to happen, and I felt immense relief.
When we finally gathered in Dr. Chmait's office with Kris, Katie and Dolores, Dr. Chmait explained in extreme detail what Twin-Twin Transfusion Syndrome was and used visual aids to show us the blood vessels on the placenta and how the babies were sharing blood vessels and transferring blood back and forth to each other instead of just getting their own supplies. He explained how the surgery would work: he would make a small incision in my stomach and insert a small metal tube (called a trocar). Then he would put a tiny scope in through the trocar all the way into Baby A's (our recipient baby) amniotic sac. He would use the scope to map out the blood vessels on the placenta and determine which vessels were going from me to each baby directly, and which were getting "crossed" (so to speak) between the babies. He'd then use a laser to occlude (or sever) the crossed blood vessels. He'd also be able to remove some of Baby A's excess amniotic fluid.
Dr. Chmait told us that there were five different "stages" of TTTS and that our babies were still just at Stage I. He said that many of the fetal surgeons who perform this surgery (of which there are only about a dozen here in the States and less than 50 in the world!) won't proceed with it until Stage III or IV, but that he takes an aggressive approach and prefers to move forward as soon as it's safe. Unfortunately, in our case, it was too early for the procedure to be safe. Apparently at about 18 weeks the babies' membranes will fully attach to the uterine wall, but as I was only 17 weeks and 5 days along ("on the cusp"), Dr. Chmait felt there was a higher risk of miscarriage at that point than there was a risk to the babies if we just waited a few days. I would pass 18 weeks by the following Monday, so we made a game-plan: I was to take it easy for the next few days and return on Monday morning for another screening to see if the babies' membranes had attached and if they'd gotten any sicker from the disease. We were of course presented with our other options for dealing with TTTS, but since all of them included letting one or both of the babies die, Sammy and I both agreed that surgery was our only option.
I can't say the next few days weren't very difficult, I found myself in tears many times thinking of my baby girls. I don't think I ever really let myself think that either of them would die-- I tend to be extremely optimistic and I have never really doubted that these little girls would come into this world and be healthy and perfect. But it was heartbreaking to think of how sick they were and hard not to worry that they were getting worse and worse while we had to wait for my body to do what it needed to do.
On this past Monday morning, November 18th, Sammy and I went back to Dr. Chmait's office and that morning was a bit of a blur. Katie did our scans and then Dr. Chmait came in to tell us that the babies had progressed to Stage II of the disease, meaning that Baby B's (our donor baby) bladder was no longer visible via ultrasound. I was at 18 weeks, 4 days, so Dr. Chmait felt that it was safe to proceed with the fetoscopy. We were scheduled for surgery at 10:30 the following morning, and then walked over to the hospital with Kris to go though the hospital admittance procedures, have my blood drawn, etc. Then we went over to Children's Hospital. Sammy headed back to work at that point, but I went to visit a fetal cardiologist who performed an echocardiogram (basically another ultrasound) where he checked each of the babies' hearts in minute detail. He was able to assure me that neither baby was experiencing the heart trauma that he commonly sees in TTTS babies, their hearts were both fully intact and fully functioning. He mentioned how lucky we were to have caught the TTTS so early, and he also had nothing but good things to say about Dr. Chmait. I was already confident that we were dealing with the best team of fetal specialists possible, but it is always nice to hear it from someone else: "you couldn't be in better hands."
On Tuesday, November 19th, we arrived at the hospital with my overnight bag, ready to get the show on the road! I checked in around 8:30am and was taken to a room where I was prepped for surgery. I had to remove all my clothes and put on a hospital gown, booties, and a cap to cover my hair. A nurse came in to put an I.V. into my left hand, and she started a drip of fluids. I hadn't been permitted to eat since the night before, and carrying twins usually makes me quite hungry every 2-3 hours, so I was feeling famished and a little light-headed, but the fluid drip began to make me feel better. Another woman came in to run an echocardiogram on my heart. The procedure went surprisingly quickly, and I was pronounced to have a "perfect heart." Next, the O.R. nurse came to introduce herself to me, as did the anesthesiologist, who asked about my medication allergies and explained what he'd be giving me for surgery. Dr. Chmait was performing the TTTS surgery on another woman right before me, and that surgery went a little long, so it wasn't until after 11 that the nurses came back to get me. I said goodbye to Sammy and asked him to go get me some food from our favorite Greek restaurant since I was convinced I'd be ready to eat a horse by the time I got out of surgery (which turned out to not be the case, but oh well, it gave Sammy something to do while I was in the O.R.).
It was strange to be wheeled from the warm, stuffy prep room down the hallway into the cold, sterile operating room, but I remember thinking, "it looks just like it does on T.V." Kris, Arlyn, Katie, the O.R. nurse, and the anesthesiologist were all there, and they were talking and laughing and just made me feel totally at-ease. I mean, they put a catheter in my uretha, turned me on my side so that my backside was completely exposed, strapped my legs down, strapped my arms down... I was basically naked and tied down on a board in the middle of a freezing cold room, and yet I didn't feel self-conscious or anxious at all. Everyone talked to me and explained what they were doing and why as they did it, so there weren't any ugly surprises. The anesthesiologist began a series of medications into my I.V., the first one made my arm very cold, but the second one burned and my face began to itch like crazy. The anesthesiologist explained to me after surgery that when that happened he gave me something else to counteract the mild reaction I was having, and that it was a medication that made me much sleepier than how he had intended me to be. There was a moment when the medications all hit and suddenly everything around me seemed to be happening in slow motion. Dr. Chmait came in and he and the nurses prepped my body- I couldn't see them since there was already a screen separating my face from the rest of my body, but I felt it as they rubbed me all over with an antiseptic and numbed my stomach where the incision would be made. Dr. Chmait told me that he was going to push the trocar in (the metal tubing) on the count of three. As he counted, I'm fairly certain I unconsciously tensed my stomach muscles preparing for impact, and impact is what I felt. It was painful, and I felt a little deflated when Dr. Chmait made the announcement that the trocar hadn't gone in and he needed to try again. There was another countdown and yet again, no luck. I'm not exactly sure if it was the third try or the fourth that finally worked, but I do remember hearing Dr. Chmait saying something about my stomach muscles and then asking me if I worked out. I said "yes, I run," and on that final try I knew the trocar finally went in. The pressure I felt was incredibly uncomfortable, but honestly that was the worst part of the surgery. Once the trocar was in place, Dr. Chmait put the scope through it and suddenly I saw one of my baby's hands! There was a monitor right by my face so that I could watch and see what Dr. Chmait would see with the scope, and I literally gasped in awe-- I'd seen some pretty incredible 3-D images of my daughters up until that point, but I really was not prepared for the sight of a fully formed hand with little fingers and fingernails! The scope moved around and I watched as Dr. Chmait explored the placenta. I was having trouble keeping my eyes open and I think I drifted in and out of consciousness several times, I would sometimes wake up and see a little hand or foot, but when I saw the placenta I just didn't have it in me to keep my eyes open and I would drift off again. I understood a little of what was happening: Dr. Chmait had only found one major blood vessel to occlude, he said something about the fact that it looked like Baby B did have enough of her own blood vessels (there had been earlier concern that she might not), and I remember him remarking on the color difference between Baby A (the recipient baby), who was very red, and Baby B (the donor baby), who was white as a ghost. But mostly I remember towards the end of the surgery that I was beginning to feel the trocar (or the scope? or the laser?) as it was being moved around and I was feeling a little concerned that we needed to wrap this up or I'd need more medication. But within minutes, we were done!
I could tell that everyone was really pleased with the outcome of the surgery but as I was unstrapped and moved to a gurney and wheeled to the recovery ward, I really couldn't focus on what was happening around me. I felt even more sleepy than I'd felt in the operating room and I just couldn't keep my eyes open for more than a few seconds at a time. The nurses in recovery got me hooked up to a device that would moniter me for contractions- even the ones that I might not be able to feel, but I remember thinking, "pshaw! I won't have any contractions!" And I didn't. Sammy joined me and he seemed really pleased- Dr. Chmait had already visited him and said that the surgery was a success and our babies were cured of Twin-Twin Transfusion Syndrome. He'd given Sammy several photos that he'd taken of the babies from inside my womb - colorful pictures of Baby A's healthy hands and feet, and sad pictures of Baby B's feet, pale white and shrink-wrapped in her own amniotic sac. We talked about how Baby B will now be able to grow bigger and healthy like her sister, and then I fell back asleep.
After an hour and a half with no sign of contractions or complications, I was moved upstairs to a private room in the maternity ward on the 7th floor. I slept most of the remainder of the day, and apart from staying lucid during visits from my sister, Dr. Chmait, and Kris, I don't remember much. I know that my nurses checked my temperature and blood pressure regularly, put antibiotics into my I.V., asked me if I was feeling pain or contractions, brought me dinner and snacks and juice and water and finally removed my catheter... but otherwise, I slept. One of the nurses set up a bed for Sammy in my room and the last thing I remember that night was seeing him sitting on his bed watching television.
I awoke early the morning after surgery when the night nurse came to check me around 6am. I woke Sammy up shortly thereafter, and we ate breakfast together. Our room had a beautiful view of the Silverlake hills, and although it was overcast out, I remember thinking that it was a gorgeous day full of possibility and I knew that we would have good news about our little girls. Sammy helped me to sponge bathe and dress, and then we waited. It wasn't long before Kris came over with a wheelchair and took us back to Dr. Chmait's office in the Doctor's Tower. We went in again with Katie for another round of ultrasounds and I wanted to burst into tears when she told us that both of our babies still had heartbeats. I hadn't expected to hear anything else, but somehow that confirmation was the most wonderful news I'd ever heard. Sammy and I held hands tightly as the ultrasounds continued, and when Katie was finished, we sat on the sofa together and Dr. Chmait came in. He told us again that the surgery was successful and the babies were now "cured" of TTTS. He said that they had both survived the night, the most critical period of time following surgery, and that Baby B's bladder was once again visible on the ultrasound. He told us that she definitely wasn't out of the woods, but he seemed optimistic that she had a good chance of making it. He had other things to tell us about the pregnancy that were issues we needed to be aware of, but those were unrelated to the TTTS. Essentially, Dr. Chmait's role had come to an end and he was handing us back to Dr. Ouzounian, our fetal specialist, for weekly follow-up visits to monitor the progress of our babies as they recovered from the surgery. We had hugs all around with all of Dr. Chmait's incredible staff before we went back to the hospital to be discharged, and I hope they all knew just how grateful we were to them for everything. Everything.
I've been home now for three days, resting, laying low, trying to allow my incision to heal- and more importantly, those angry stomach muscles around my incision. Sammy has been my rock. He has literally doted on me since the moment we got confirmation that I was pregnant, but his care of me throughout the surgery and since has been above and beyond. I have often thought of how very grateful I am that we are not young newlyweds, but instead a strong couple of nearly 11 years, because weathering a storm like this is no easy task and I just can't imagine doing it with anyone else by my side but my long-time, long-term partner in crime. I don't think Sammy has wavered once in his positivity and optimism in the outcome of this pregnancy. He is convinced that his daughters will arrive healthy and happy, and nothing can shake his conviction. I have needed nothing less from him than this stalwart conviction, because his strength of belief has given me the strength I have needed to push through all of this believing too that everything is going to work out.
Today I felt our Baby A move for the first time. I have faith that I will be feeling Baby B move very soon. On Tuesday we visit Dr. Ouzounian for our first post-surgery appointment, and we both trust that Baby B will be happily situated in a full sac of amniotic fluid, growing rapidly and turning pinker. Our journey isn't over yet-- in a sense it has just begun. But our babies have survived Twin-Twin Transfusion Syndrome and we have every reason to believe that in a few months we'll be holding them in our arms, our healthy, happy, tiny little baby girls.
I know some people would prefer to focus on the realities of the situation: in our case, that Baby B has a long road ahead of her and that the statistics say she might not survive. I guess some people would rather stay "realistic" so that they won't risk disappointment. But our own perspective is to focus on the good: in our personal experience, when we focus on the positive, on the outcome we WANT to happen, it does. If things don't work out the way we expect, we'll deal with that then. But for now, we have to believe that all of this-- catching the disease so early, best-case-outcomes every step of the way-- it can't be for nothing. There is a reason. And we choose to believe that the reason is that our daughters will not just survive, but thrive.