To Honor Fiona and Brigid

Our local newspaper published an article yesterday about TTTS and an event that we have coming up to raise funds for the TTTS Foundation.  It was online and also had a huge section devoted to it in the paper copy with a large photo of the family, and the headline...  Oh, the headline: 

"To Honor Fiona and Brigid"

It was bittersweet.  The editor emailed me to ask if it was okay with me to use their names like that.

I started sobbing.

Um...yes.  That, for me, is the point, really.  Thank you so very much for getting it.

Their names don't feel real to me.  They're sort of abstract, like they're not my children.  But to see them in print like that was so amazing.

I love the photo of our family because it shows that we're all still having fun together.

You can read the article here.  Please feel free to share it with others.  The goal is to make as many people aware of the cause and treatment of  TTTS as possible.  Sometimes, they might have to make their doctors aware.  I know when I asked my OBs specifically about TTTS - because I'd read about it online as soon as I learned we were having monochorionic twins (twins who shared a placenta) - and if I might be able to be seen by a perinatologist earlier for monitoring, they pooh-poohed my concerns.  Both of them (I switched after the first one dismissed my request) felt it was too rare to warrant "extraneous" ultrasounds.  I wouldn't need one before the usual 20 weeks, they said.  I even called the perinatologist to ask if I might be able to come in on my own, without an OB's referral.  They said no.

And yet, when we finally did get that ultrasound, at 19 weeks and 5 days, it was much too late.  The disease had progressed significantly and was in the late stages.

In hindsight, I wish I'd demanded it.  What would it have cost them to have me checked out early?  A form?  Probably not even that.  A computer click or two?  Women who are having multiples who share a placenta need not to feel like they are being pushy or silly for being concerned about this very real threat to their babies.  The doctors and midwives at my OBs' offices are not losing any sleep over dismissing my concerns right now.  They remain completely unfazed by it.

Yet here we are with two dead babies.

I guess this is the anger stage of my grief.  I'm not angry at God.  I'm not wondering why he did this to me or why did this have to happen to me.  I'm angry, though, that my physicians were ignorant, and that, even when I was the one who did the research and brought this disease to their attention at appointments asking to be monitored for it, they did not think it was important.  That is terribly frustrating.  And while I don't know that it would have made a difference in our outcome, it would at least have meant that everything that could have been done to stop it was done.

So now all I can do is warn others.  Be concerned.  Be pushy.  Demand.  No one loves your unborn babies like you do, and you are the only advocate they have.

Another good report

We had another appointment at CHOP yesterday to check on Brigid's progress, and we're happy to report that she's looking even better now than she was a month ago.

In fact, from the obstetrical standpoint, we have been discharged from CHOP altogether. She is growing well, looking strong and healthy, and even the chorioamniotic separation seemed to be looking better - either closing or repairing - and doesn't seem to be a major issue anymore.

Only her heart is not 100% normal, and still it's looking better than it did at our last appointment. The valves are no longer leaking at all, and the ventricles seem to be functioning better. The only remaining issues are that the wall between the ventricles seems to be slightly weakened and the right ventricle appears a little "bulbous" in shape. Her heart is slightly enlarged still, but the hope is that all of these things would continue in their current trend and would heal over time. We will have one more appointment with fetal cardiology in a month, and if things are continuing to improve, that will likely be our last visit to CHOP altogether.

It was seven weeks ago today that we learned that our twins had TTTS. That has meant seven weeks of bed rest. Seven weeks of Patrick shouldering the majority of the childcare and housework, in addition to his job.

I've had two hospital stays - the first two of my life, besides the days following the birth of our boys. We have had good news, bad news, hopes answered, hopes dashed, reassurances and disappointments. We've cried through every doctor's appointment, good or bad, and are still not feeling one hundred percent confident that we'll be adding a healthy baby Brigid to our family in the next few months, though that confidence is growing more and more.

Patrick and I talked yesterday about how difficult this experience must be for people who do not have a faith in God. How sad to go through this with no hope for being reunited with little ones who have died, without trusting that there is a reason for this suffering - that it will make us stronger and bring us closer to each other and closer to the Lord. We have felt the prayers - of family, friends, and people we don't even know - carrying us through this difficult time. They bring us peace and comfort where we would otherwise feel overwhelmed and full of sorrow. Thank you for praying for us and for our baby girl! Thank you for doing our laundry, for cleaning our home, for mowing our lawn, for caring for our children, for sending us care packages, for bringing us meals, for checking in with us to see what we might need before you go out to do an errand. We are humbled, grateful, and so very blessed.

And we're pretty sure that we couldn't have gotten this far without your help.

Days in Waiting

It's clear to me that the Lord is trying to teach me to put each day in His hands.

After having some contractions on and off this past weekend, we asked a neighbor to come and stay with our boys once they were all in bed on Sunday night and Patrick and I headed into the hospital. It was a scary time. I was crampy and contracting fairly regularly and we were worried.

Once I was taken back to triage, they put me on the contraction monitor and could see that they were coming at a steady pace. The first order of business was to get them to stop. The nurse gave me a shot of terbutaline, which is appropriately named because it made me feel like my heart was in turbo mode, and it wasn't until after she'd administered it and left the room that I thought to ask what it might do to little Brigid's heart, which certainly doesn't need any more issues. Shaky and feeling weird from the medication, I sat there looking at Patrick and fearing that this was going to be it. We'd deliver not only our deceased baby, but our live baby at a pre-viable age and have to hold her while the doctors were unable to do anything to help. It is my biggest fear right now.

The ultrasound tech came in to measure my cervix to see if the contractions were serving to shorten it and advance labor, and from the measurements she took, it seemed that way. I started to panic a little.

I was admitted for overnight observation and Patrick went back to the house to relieve our kind neighbor since it was now almost midnight. They hooked me up to IV fluids, gave me another medication to stop the contractions called Procardia, and hooked me up to the fetal heart monitor and the contraction monitor. Then they suggested I try to get some sleep.

How?

That was Sunday night. Since then, contractions have seemed to stop and then restarted a couple of times, I've gone off my IV and then had to go back on, I've increased the frequency and then the dosage of the Procardia. I've had an ultrasound to confirm that Brigid is doing okay and that my cervix was not shortening like we'd thought. I closed my eyes for a little while this morning, and, just as I'd had a brief vision of the Lord welcoming baby Fiona into his arms a few weeks ago, I had another this morning of a nurse bringing a healthy baby girl to my bedside, and it gave me hope. Thanks to a sweet nurse who took some time this afternoon to pull up a stool beside my bed and ask me what I was thinking about or afraid of, I learned today of a baby boy who was born in this hospital at 23 weeks and is now a thriving two-year-old. She wears his photo on her name badge to remind her why she does what she does every day. She sat and asked about our story and cried with me as I recounted all we've been through in the past four weeks. She was so very reassuring and encouraging, as I've had lots of time alone in this hospital bed to think and worry about things the past few days.

It's now Wednesday night. I have had only five contractions all day, and the doctor seems to think that I should be able to go home on this medication, because, even though the dosage makes me feel very strange since they've increased it, it is working. I miss my home. I miss my boys. If you can believe it, I miss cooking and grocery shopping and running the vacuum (especially since we just got a new one and I haven't gotten to use it yet)!

We are, as we have been from the start, in waiting. That can be frustrating at times, but it's also somehow peaceful and reassuring knowing that things are not in our own hands. There are things I can do: staying in bed, drinking 200 ounces of fluid per day (!) and taking my medication. But the outcome is in the hands of our trustworthy and all powerful heavenly Father, who works all things together for our good and loves us very much. In happy times, when things are going well, it's easy to forget Him as the giver of all good things, but during these days of uncertainty, I feel myself drawing close to Him for comfort and peace. While I am often tempted to fear and worry, there are more times that I feel close to Him and confident that He is more than able to bring us a healthy baby girl.

But for now, we wait.

Not quite out of the woods yet

In fact, I'm thinking we're actually going to be living IN the woods for a while. We can pitch a tent, keep the food up high in a bear box, and sing songs around the campfire. Care to join us?

We had our follow up appointment at CHOP today, one week after my surgery, and while we hoped to see leaps and bounds of improvement in baby Brigid, what we actually saw was not too different from the scan we had the day after the surgery. Her ventricles in her heart (the bottom two chambers that do the actual "pumping" work) are thickened and a little weakened from having had to handle so much excess blood, and her valves are not closing as well as they should for the same reason. The cardiology team will follow me weekly in hopes of seeing improvement, and there is a medication that I can take that can help her heart (which is amazing to me), if it does not improve well enough over time. She looked beautiful, though, and was moving all over. I've been feeling her kick and move so much more since they removed the excess fluid and it's been a reassuring feeling that's reminding me every day that we just need to take things one day at a time. Every day that I carry her and feel those kicks is a blessing.

The scan also showed something that we knew was a possible effect of the surgery. That is, that my amniotic sac has detached from the chorion it is usually fused to, and fluid has gotten behind it. This does not really affect the baby at all, I am told; she has enough fluid for herself. The risk is that it gives me a greater potential for premature labor, and so I am to stay on bed rest for the entire remainder of the pregnancy. The longer this period is, of course, the better. This is quite daunting, but again, something we'll have to handle one day at a time.

We are so grateful for meals, childcare and offers of helping with chores and errands that have been pouring in. It is incredibly humbling to have someone over to your home for the first time and to watch them vacuum your living room while you're sitting on the couch. Patrick and I like to be self-sufficient, both individually and within our family. "No thanks - we can handle it!" But this may be part of the lesson that the Lord is trying to teach us - to humble ourselves and ask others to help us, because the reality is, we don't have another choice right now.

Bittersweet

To say that we have had an emotional week would be the understatement of the decade.

Patrick and I had an appointment last Wednesday, the 9th, to see our twins for the first time since my 9 week ultrasound, pictured in the last post. We knew we were having identical twins and couldn't wait to find out if we were adding two little girls or two little boys to our family. What we learned from the ultrasound, however, would change our lives.

The twins looked beautiful to me. Both were moving around and it looked at one point like they were facing each other, each with their arms reaching out to the other. The technician was kind, but a bit tight-lipped, skirting around some questions I had and spending a lot of time looking at "Twin A's" heart in particular. When she spent significantly less time on "Twin B," I became suspicious that something might be wrong. The doctor came in shortly after and told us that our babies were very sick and in danger. They needed immediate intervention if they were to have any hope of survival. He diagnosed us with something called Twin to Twin Transfusion Syndrome (TTTS), and said he would like us to be seen at Children's Hospital of Philadelphia as quickly as possible.

TTTS occurs when there are abnormal, unbalanced vascular connections between twins who share a placenta. The arteries of one twin (the "donor") pump into the veins of the other (the "recipient") and both twins are in danger. What I experienced physically was a huge increase in the size of my uterus over a short period of time that I did not know was not normal for twins. I was very large and very uncomfortable. This happens when, in an effort to process all the extra blood, the recipient urinates much more frequently than normal and makes excess amniotic fluid. The donor twin, however, had very little fluid and was sort of "stuck" to the side of my uterus. She did, however, have a visible bladder, which was a good sign.

Patrick and I spent a lot of time crying and went home feeling a little numb. I was sad that we came away from the appointment learning about this terrible syndrome and yet still not knowing whether the babies were girls or boys. We also had no ultrasound photos of our babies. It was a very empty feeling. I called the doctor the next day and asked him if he was able to see the gender of the babies, and he told us they were girls. It felt good to learn that and we tried optimistically to imagine our family with two little girls in it.

We reached out to the TTTS Foundation, letting them know what we learned and asking for information and help. Mary, the founder, was wonderful and supportive and called to answer our questions and to offer us hope. She suggested I remain on bedrest until our appointment at CHOP on the following Monday and mentioned to be aware of an even greater increase in fluid. She also suggested that we name our babies so that we didn't have to just keep referring to them as "donor" and "recipient" or "Baby A" and "Baby B." We decided to name our donor baby Fiona, which means "fair," and our recipient baby Brigid, which means "strong."

Friday night into Saturday, I felt the increase in fluid that Mary was referring to. I had much more pressure under my right rib, felt a little lightheaded, and was really having a hard time breathing. I knew I'd been having some anxiety attacks from the upset of our diagnosis, but this was something more. On Saturday morning, we decided it was best to go into the hospital. Unfortunately, this was also the day of my little sister's wedding. My parents came to get the boys to bring them to the wedding and Patrick and I went into the hospital.

I was seen by the OB on call and we learned that our perinatologist was in his office and would be able to do an ultrasound to see what had changed since Wednesday. What he saw was actually an improvement. Our donor twin appeared to be moving around more and to have some more fluid in her amniotic sac. This was encouraging, but because she was moving more, he was also able to see her umbilical cord better and to determine that it looked rather fragile. It was a velamentous insertion into the placenta, which means that rather than inserting directly into the mass of the placenta, the vascular connections of the cord extended off the very edge of the placenta and went along the surface of the sac's membrane before connecting to the baby. This could be contributing to baby Fiona's poor growth, as she was much smaller than her twin sister.

We went home slightly more hopeful and anxious for our appointment at CHOP, where we could get a better idea of what we were up against and determine a plan of action.

Monday's appointment at CHOP consisted of hours of testing. Ultrasound echocardiograms for the babies, genetic counseling, more anatomical ultrasounds and finally a consultation with the doctor. We could see that little Fiona had no more fluid around her and her bladder was no longer visible. It was evident that her heart was struggling from the syndrome, as was Brigid's, which was overworked and straining. The tests were overwhelming and exhausting, but the staff and technicians were all very sympathetic and encouraging to us. One even printed out some photos of the two girls for us, which were both sad and beautiful. I put them in a folder and have not been able to look at them since. Seeing those babies struggling in there and just wanting to reach in and help them or hold them was so frustrating and upsetting.

During our consultation with the doctor at the end of the day, we learned that not only did we have TTTS, but that it was complicated by something called Intrauterine Growth Restriction (IUGR), which is what happens when a baby has only a small portion of the placenta, as our little Fiona did. The doctor suggested that she actually only had less than 10% and that she was already showing indications of brain damage as a result of the lack of blood supply. He told us what our options were: we could do nothing, in which case our babies had less than a 5% chance of survival; we could terminate the pregnancy altogether, which was not an option for us; we could "selectively terminate" Fiona since she was already so severely damaged, giving Brigid a better chance at survival, which was also not an option; or we could have a surgery to stop the abnormal blood flow between the two babies. This surgery was going to be the best chance at saving both of them, and for us, it was the only option. The doctor said he'd like to do the surgery the next day because of the urgency of the situation, and told us that if Fiona did not survive until then, not only could they not do the surgery, but it was likely that Brigid would die as well. If Fiona survived and if the surgery was able to help her, he warned us that she would likely be born with severe neurological problems, including cerebral palsy or worse.

That was one of the most difficult nights of my life.

Sleep did not come easily. I prayed for my babies. I prayed for the doctors. I prayed that Fiona would survive until the surgery and even beyond the surgery. I slept fitfully and woke very early in hopes of feeling some kicks that would give me an indication that the babies made it through the night. Once I did, I prayed they could hold on for a few more hours until the surgery.

We returned to CHOP and they got me ready for the surgery. Just before I went to the OR, they did one last ultrasound to determine that both babies had indeed made it through the night, and they had! The procedure is an amazing one: the doctors insert a 4mm wide instrument into the uterus that has both a camera and a laser on it. They map out all of the connections between the babies on the surface of the placenta and determine which ones are the unbalanced (vein to artery) ones. Then they use the laser to cauterize all of these connections to ensure that each baby is only getting her own flow of blood to and from the placenta. I was only under a twilight sedation, so I did have some awareness of what was happening, but I didn't really feel any pain, just some discomfort. After the surgery, they also took out some of the excess amniotic fluid from Brigid's sac. They removed 2.5 liters and my stomach was half its size! It was remarkably quick - only about an hour and a half - and the doctors were so encouraging and compassionate. I went back to my room and slept for most of the afternoon. They kept me overnight and would do another ultrasound after 24 hours to see how the babies were responding.

The time went slowly and, knowing the babies experienced the same sedation I did, I didn't feel many kicks to let me know they were okay. It was frightening and yet we felt a peace, knowing that we did everything that we possibly could do and the rest was in God's hands. We knew he loved our babies even more than we did. For a few minutes, I closed my eyes and I had a vision of Jesus welcoming our little Fiona into heaven, and then the technician came in to do an ultrasound.

I watched the technician's face, looking for any sign of what she was seeing on the screen, but couldn't tell. Then she went to get the doctor and his face was a little easier to read. He seemed encouraged and then dismayed. "Did we lose the donor," I asked. "Yes," he said, compassionately. He showed me the screen and told me that Brigid looked to be showing some improvement already, but he also showed me that little Fiona's heart was no longer beating. The range of emotions was difficult to process. Sorrow over the loss of our little one, thankfulness that she was able to hold on until the surgery, encouragement that Brigid seemed to be doing better, fear that there was a chance that we could still lose Brigid in the days and weeks to come. Patrick and I held each other and cried. It was so terribly bittersweet. I asked the doctor what would happen to Fiona, and he said that she will just stay until Brigid is ready to be born. Thinking about what that day will be like is terribly overwhelming and for now, I try not to.

I had to stay in the hospital for a little longer to finish my dosing of post-surgery antibiotics, and then we went home. I am on fairly strict bed rest now (only up to use the bathroom and shower), hoping and praying that our little Brigid gets stronger. Having our boys here is like medicine for our aching hearts. We have been so overwhelmed by the outpouring of love and support from our friends - offering childcare, bringing meals, helping with groceries. It is very hard for my Type A controlling personality to sit on the couch and watch others run the house or to delegate to people who are here and asking how they can help, but I think I'm going to have to get better at it for at least the next two or three weeks.

The whole experience has been surreal. We were not trying to have another baby right now, but we learned that we were. Then we learned that we were having twins. We learned they were identical and that felt special. Identical twins is something that happens to other people, not to me, but here we were. Then we learned that we had this syndrome and I was going to have in utero surgery and that, too, seemed surreal. Losing a twin makes it feel like that specialness has been taken away. The dressing them alike, comments from strangers - all that has been taken away and it feels like a void even though they haven't even been born yet. It's only the second time I get to use the "twins" tag on a blog post, and yet, it's the last.

I will be going back to CHOP on Tuesday for a follow-up appointment and to determine how well Brigid is improving. Knowing that she has almost the entire placenta is both sad and encouraging. Having to tell the boys what happened was very difficult, and I knew Jack would take it very hard. He was so excited about having twins, and he was especially thrilled to learn that they were girls. His innocent questions have been so hard to answer, and he is acting out in a way that shows he is still having a hard time processing everything.

I'm praying that God will heal Jack's little heart. I'm praying that Patrick will have the patience and stamina to do his work and to take on more of the housekeeping and childcare as well. I'm praying that little Brigid's heart would continue to show improvement and that she would grow strong and healthy enough for a full term pregnancy. And I'm praying that our hearts would heal as well and that our faith would be strengthened. We feel the sting of our loss but are trying to stay positive and encouraged by Brigid's improvement, trusting that her future rests in God's hands as well.

It's hard not to dwell on the what ifs. What if we had caught the TTTS sooner? What if we go on to lose Brigid too? We are hopeful. We are prayerful. We are anxious. But we know that God is sovereign and He is good, and His ways are not our ways. We are also encouraged to trust that one day, we will get to meet our little Fiona in heaven - and so will her twin sister and her big brothers.