TTTS (Twin to Twin Transfusion Syndrome) Awareness Month

Today marks the beginning of International TTTS Awareness Month. Each December, the TTTS Foundation seeks to promote awareness of TTTS and provide support for the families who are struggling with the diagnosis.

Twin to Twin Transfusion Syndrome (known as TTTS) is a condition unique to monochorionic twin pregnancies. During a twin pregnancy, there are several factors which contribute to the risk of TTTS. First, the babies must share a single placenta. Fraternal (dizygotic) twins will always have separate placentas (although the two placentas can fuse together) and care not at risk of developing TTTS. Identical (monozygotic) twins can have separate placentas as well (up to 30% of identical twins do have separate placentas) and many doctors will mistakenly diagnosis these pairs as being fraternal twins.

The most common scenario is for identical twins to share a placenta but each baby remains in its own separate amniotic sac. This is called monochorionic/diamniotic (mo/di) because the babies share a chorion (the outer layer of sac) but are separated by an inner dividing membrane-the amnion. The most dangerous scenario is twins who are monochorionic/monoamniotic (or mo/mo) because these twins are inside the same amniotic sac and are at risk of umbilical cord entanglement. Fortunately, mo/mo twins are rare-only around 1% of twins present this way. More commonly, the identical twins are mo/di and these twins are at risk of developing TTTS.

(My belly at 35 weeks pregnant with the first set of twins)

TTTS is a disease of the placenta in which there are abnormal blood vessels which connect the umbilical cords of each baby, enabling them to "share" blood. When the "sharing" becomes unequal, one baby receives too much blood flow and the other does not receive enough. The baby who does not receive enough is known as the "donor" and the baby receiving too much is the "recipient." Obviously, this type of unequal blood flow can harm both babies if left untreated.

There are two different types of TTTS. The most serious type is chronic TTTS, which occurs between 12 and 26 weeks gestation, before the babies are big enough to be born. The second type is acute TTTS which occurs later in pregnancy, or even during labor. Acute TTTS can be resolved with immediate delivery of the babies but chronic TTTS can require treatments such as laser surgery to separate the connecting blood vessels.

TTTS can strike any monochorionic twin pregnancy without warning. Of all monochorionic twin pregnancies, about 15% will go on to develop TTTS. There are about 4,500 cases of TTTS in the US each year.

(Sarah & Leila, just a couple of hours old)

When Leila and Sarah were born in 2006, I had no idea what TTTS was or even whether they were at risk for it. Because I didn't have an ultrasound until 22 weeks (that was when I found out I was carrying twins!) it was impossible to tell whether the girls shared a placenta or whether two single placentas had fused. There is a narrow window of time in which doctors are able to easily tell whether twins are monochorionic by looking for that thin dividing membrane and this is quite early in pregnancy. I was well past that point and the girls were big and crowded inside me, making it impossible for anyone to know whether they were monochorionic.

It wasn't until after their birth that we knew something was wrong. I had wonderful midwives present for the birth and Leila and Sarah were born on the floor of my bedroom. There were no complications other than a loop of cord in front of Leila's head that had to be pushed aside and Sarah's hand trying to come out before her head. The girls were both 7 lbs, 6 oz and healthy. However, acute TTTS had struck sometime in my final days (or hours) of pregnancy.

Because of the unequal blood sharing, the recipient baby in a TTTS case becomes overloaded by the extra blood and suffer from heart failure. The donor suffers from lack of nutrients and poor growth. There are warning signs such as discordant growth and discordant amniotic fluid levels. Doctors also look at the way the umbilical cords are attached to the placenta and how many vessels are in the cords.

One of my girls had a single umbilical artery but there were no other factors to indicate TTTS was developing. After their birth their pediatrician was alarmed at hoe red Sarah's skin looked. Blood tests revealed that Sarah was polycythemic as a result of acute TTTS and that left her skin red for months. Leila (the donor) was anemic and looked pale and thin in comparison to her twin. There were no lasting effects of the TTTS and we were blessed that everything turned out just fine.

(My ginormous belly just days before Nathan & Ryan were born)

With my next pregnancy, I suspected right away that I carrying twins again. Everyone assured me that I was crazy, and I tried to believe them. Finally, I went for an ultrasound at 9 weeks and found out that I was indeed carrying a second set of twins. On top of that news, I learned that they were also monochorionic twins.

I ended up seeing the same OB for backup care that I had seen with the girls. He was very knowledgeable about TTTS and was amazed that I was carrying a second set of identical twins. Because the odds of having two sets of identical twins were already high, he assured me that the odds of having two sets of twins with TTTS would be astronomical. Indeed, when I consulted with a leading expert in TTTS research, he also reassured me that it would be almost unheard of to develop TTTS again. He knew of only 2 cases of that happening.

And then, in an incredible twist of fate, TTTS struck again. After a long pregnancy full of conservative monitoring, acute TTTS reared its ugly head. This time, things were different. And scary.

Just days from my due date, I started to have an incredible pain in my groin/hip/leg. I thought for sure that it was a baby pressing on a nerve and tried everything to fix it. When all failed, I ended up in the hospital. A recent ultrasound (notoriously inaccurate in the third trimester) showed that both babies were the same size and were big and healthy. Despite the ultrasound results, things were not well. There was a significant growth discordance and Baby B was in distress. When they were watching me overnight, Baby B's heart rate tanked. His heart rate had been having slow decels down to the 70s & 80s, but suddenly it dropped to 50 and then disappeared. That was it. They put me out and I had an emergency cesarean. When I woke up I had no idea what had happened.

(Nathan & Ryan at a few days old)

Ryan (Baby B, my donor) was resuscitated at birth and was thin and pale, but healthy. He weighed 6 lbs, 6 oz. Nathan (my recipient) was 7lbs, 9 oz and was red and plump. Examination of the placenta revealed an unequal sharing and Ryan's umbilical cord had been small and thin.

That is the scary part of TTTS. It can strike anyone and can come on without warning. Close monitoring is important, but having an educated care provider is key. I can't even tell you how many mothers of twins have told me that their doctor said their twins can't be identical because they have separate amniotic sacs. The TTTS Foundation distributes a (free) wonderful handbook for parents and their care providers that will tell you everything you need to know about TTTS.

I consider myself extremely blessed to have my six sweet children. I know that many cases of TTTS are severe and end in the loss of one, or both, babies. The TTTS Foundation is working to stop this through raising awareness of TTTS risk factors and treatments. I'm fortunate to have two healthy sets of TTTS survivors, but many families are not so lucky.

You can help the TTTS Foundation by making a donation or by drawing attention to TTTS. Blog about it, tweet about it, talk about it!

The Birth

On Wednesday morning, September 3rd, I began having pain near my hip. It seemed to get worse as the morning wore on and by 11am I was very uncomfortable. An hour later I called Jason and asked him to come home. I was in tears and he knew that something was definitely wrong.

I quickly decided that I needed to do something because the tricks I was trying to relieve the pain were not working. I was using my TENS unit, a heating pad, ice packs, getting in the tub, laying in an inverted position, and anything else I thought might encourage the babies to move.

By 1:30pm I was on my way to the chiropractor. He was so sweet and came back early from lunch just to see me. He tried to adjust me but it did not help. He could tell that I needed help and walked me out to the van to tell Jason to take me to the hospital.

I arrived at the ER just before 3pm and was quickly ushered upstairs to L&D. I was in so much pain that I could not think straight but kept assuring them I was not in labor. Nobody believed me. I let them monitor the babies and took a Percocet to relieve the pain. Unfortunately, the pain continued and the Percocet only made me sleepy.

Eventually I decided to go home. I was still in pain but wanted the freedom to move and planned to get into the birth pool and see if that brought relief. At 8pm I left the hospital after signing out AMA.

I returned home and again tried to relieve the pain by encouraging the babies to turn. I was sure that one of the babies was compressing a nerve. I was praying for guidance and that God would relieve my pain. The kids were worried about me and the girls were trying to console me by kissing my belly and rubbing my back.

Around 11pm I called my friend to see if she could drive me back to the hospital. I was in tears as I told Jason that I'd be having a hospital birth for sure now. He didn't want me to go at first and kept asking if I was sure that I wanted to go back. I told him that the pain was unbearable and that I needed to go.

After getting back to L&D they again hooked me up to the monitors and this time I agreed to an IV and a cervical check. I was 1cm dilated. (I told them I wasn't in labor!) They gave me a shot of Demerol and I was able to relax enough to sit in the bed. It was around this time that we noticed Baby B (Ryan) having some slight decels in his heart rate. They weren't major and he only had 2 or 3 but it did take a while for his heart rate to recover.

Eventually I fell asleep and awoke around 3am to find another friend in my hospital room. She camped out there with me overnight and was there when things really got crazy. Baby B's decels became more frequent and more severe. Sometimes they dipped to the 90s and then to the 70s. They would take a minute to recover. I got a dose of morphine for the continuing nerve pain.

By 6:30am I was very concerned. I pretty much knew that a vaginal delivery was out of the question. If Baby B couldn't stand the stress now, there was no way he would survive labor. I asked when the OBs would be doing rounds and found out that my OB was coming in that morning. He was not scheduled to work so it was a pleasant surprise. I would obviously prefer the care of a good midwife, but I have had a good relationship with this OB throughout this pregnancy and my pregnancy with the girls. If anyone was going to operate on me, I wanted it to be him.

Around 7am I called Jason and told him to pack a bag and come to the hospital. I called my friend to come care for the kids. I called my mom and let her know that I'd be having the babies that day. As I'm sitting there making phone calls, Baby B's heart rate plummets. It dropped down to 50 bpm before disappearing. My room filled with activity as everyone rushed to have me flip onto hands & knees, gave me more oxygen, and tried to stimulate the baby.

That was it. I knew it was time. I cried as I asked about the details of the cesarean. My OB was en route to the hospital. (He told me today that he drove 80 mph and ran a red light trying to get to me.) The nurses quickly prepped me as I called Jason again and told him I was going to the OR now.

Within minutes I was rushed down the hall and into a cold, sterile OR. The doctors made a quick attempt at a spinal because I had specifically requested to be awake and not restrained so that I could hold my babies after they were delivered. The spinal did not take and there was no time to try again so I was put out with general anesthesia. My last memory was watching them put up the blue sheet for the sterile field.

Nathan was born at 8:08 am and Ryan followed at 8:09 am. Jason was waiting for them outside when they brought them to the nursery.

I woke up in recovery. Jason and my friend were there. I think that the hospital lactation consultant (a friend) was also there judging by the pictures. I have no memories of it, but apparently I nursed the babies for the first time. I also called my mom (which I don't remember) and the nurses brought me my placenta to bring home.

My only memory is when they wheeled me to my postpartum room. As we rolled down the hallway they played the lullaby song (twice) for the boys. Then I was left in my room to get to know these new little boys who had just rocked my world.

As I sit here, 5 days later, I'm still struggling to process what happened. I'm definitely grateful for the cesarean and I know that I made the right choice. I'm grieving the loss of my homebirth and staring at the empty birth pool was a painful reminder so Jason deflated it yesterday. That just made it seem even more real. No more babies? No more births for me? I can't beloieve it.

Today I had my staples removed. I'm feeling good aside from some wicked back pain from the spinal attempt. I ran to get groceries today and I'm feeling capable of moving without my insides falling out on the floor.

After talking to the OB I have figured out a few things. First of all, my nerve pain was just that-nerve pain. The severe pain was caused by my obturator nerve and was unrelated to the babies' conditions. I firmly believe that the pain was God's way of telling me to go to the hospital. I wasn't in labor and had my next OB appointment scheduled for today. There is no way Ryan would have survived another week-or even another day. God got me to the hospital so that Ryan would live. I am 100% sure of it.

Ryan's umbilical cord was very thin and had almost no Wharton's Jelly. He had stopped growing as well as he had been and although there were no signs of it on ultrasound, the beginnings of acute TTTS were at play. There were no complications (unlike the girls where one had anemia and one had polycythemia) but Ryan did need to be resuscitated after his birth. My uterus looked great and there were no issues with the placenta-no accreta or previa. We are all healthy and doing well!

I'm sad that I couldn't give these boys the peaceful birth that they deserved. But I'm incredibly grateful that they are both here in my arms where they belong.

Ultrasound Results

Today was the big ultrasound to determine chorionicity of the twins. They are monochorionic, diamniotic. Basically, this means that they are monozygotic (identical) and share a placenta.

For those who are unfamiliar with twinning I'll explain a few things. Twins are either monozygotic or dizygotic. Monozygotic (MZ) twins are often referred to as identical and are the result of one egg splitting after fertilization. Dizygotic (DZ) twins are often referred to as fraternal and are the result of two separate eggs being fertilized.

Dizygotic twins will always have separate placentas, amnions, and chorions although sometimes the two placentas can "fuse" into what appears to be one placenta. The amnion is the inner sac (or membrane) and the chorion is the outer sac. Many people do not realize that the membranes are made up of two separate layers. DZ twins will always have separate amnions and chorions because they result from 2 separate eggs. DZ twinning can run in families and can be increased because of fertility treatments.

Monozygotic twins are trickier. About 1/3 of all twins are MZ and it is unknown what causes MZ twinning. MZ twins can have separate placentas, amnions, and chorions like DZ twins. About 1/2 of MZ twins are dichorionic, diamniotic (DCDA.) About 60% of MZ twins are monochorionic, diamniotic (MCDA) and these twins share a placenta and are at risk for Twin to Twin Transfusion Syndrome (TTTS.) Only 1-2% of MZ twins will be monochorionic, monoamniotic (MCMA) and this is the most dangerous type of twinning because of the risk of cord entanglement.

It's amazing how little some medical professionals know about twinning. The ultrasound technician today did not know what a chorion was and had to call in a doctor. She said she has NEVER seen a monochorionic twin pregnancy before. This is totally wrong because she is the same technician who performed my first u/s with the girls. She told me then that they were fraternal because they were in separate amniotic sacs! She has been giving out bad information to who knows how many people. I think I set her straight today.

Anyway, these twins are again at risk of TTTS. The girls developed acute TTTS at term. We don't know when it happened but fortunately God was watching over us and everything was fine. S was the recipient twin and so she had a very high red blood cell count for about 8 weeks after birth. She would have received a transfusion if we had been in the hospital. L was the donor and was anemic. S has always been chubbier and healthier-looking than her sister. The differences are noticeable even today.

I'm a little worried about TTTS but mostly wondering how difficult this pregnancy will become. I am now "high-risk" and with that comes a probable referral to that doctor I saw last time and wanted to avoid this time. I'm sure he'll be thrilled to see me again! This also means frequent ultrasounds and monitoring because there is a greater chance of growth discordance and of course TTTS. So much for an uneventful pregnancy!

Oh, and one last note. I decided that I will go ahead and find out the gender of these babies. I didn't plan to find out but if something does happen, I want to name them now and not later. I can't explain it but I just feel compelled to know so I'm going with that.

(And yes, I am very excited, thrilled, and can't wait to meet these babies! I'm just also feeling overwhelmed by the possibility of complications. Please don't think that I'm not completely grateful for this blessing because I really am but my head is still spinning from all of the developments in the past few weeks.)