Patient Stories
Twin-Twin Transfusion Syndrome / TTTS
Following Fetal Surgery and 'Ups and Downs' in First Months Sophia and Olivia Little Are Home with Their Parents
It was a lot to cope with all at once. At her very first prenatal visit to confirm the results of her home pregnancy test, Heather Little learned that, yes, she was pregnant, and she was having twins. She was also alerted to the possibility of Twin-Twin Transfusion Syndrome (TTTS).
Heather and her husband, Mike, were both 33 years old at the time. It was Heather's first pregnancy. The date was August 13, 2005.
Heather's OB/GYN, Sarah Shores, MD, referred her to a specialist, E. Mike Vlastos, MD, who ordered another ultrasound. The ultrasound confirmed that Heather was carrying monochorionic diamniotic twins. The identical twins shared a single placenta with connecting blood vessels, although each twin had her own amniotic sac—enclosed in a membrane and surrounded by amniotic fluid, the fluid that surrounds and protects the fetus and helps provides the environment for proper development.
Twin-Twin Transfusion Syndrome / TTTS occurs in about 10% to 20% of monochorionic diamniotic pregnancies. In Twin-Twin Transfusion Syndrome / TTTS, the exchange of blood through the connecting blood vessels on the shared placenta is unbalanced. Blood from one twin (known as the "donor" twin) is transfused disproportionately to the other twin (known as the "recipient"). As a result, the donor twin has reduced blood volume, which can lead to slower growth and less amniotic fluid. The recipient twin gets too much blood which can lead to heart failure and other complications.
TTTS Confirmed at 16 Weeks
Dr. Vlastos continued to follow Heather closely and when she was 16 weeks pregnant, confirmed Twin-Twin Transfusion Syndrome / TTTS. Heather and Mike learned more about TTTS from the website hosted by the Fetal Care Center of Cincinnati, from Dr. Vlastos, and from other sources. They learned that in cases such as theirs, when TTTS develops before 20 weeks of pregnancy, without treatment there was only a 25% chance that either twin would survive.
Dr. Vlastos contacted the contacted the Fetal Care Center of Cincinnati and soon afterwards, Mike and Heather Little and her mother, Connie Hoffman, drove the six hours from the Littles' home in St. Louis, Missouri, to the Fetal Care Center. They left on a Sunday so they could be sure of being on time for their first appointment very early Monday morning.
Team Meeting and Difficult Decision
The rounds of appointments started at 6:30 am and lasted more than 12 hours. The fetal care team was "extremely accommodating. I can't praise the fetal care center staff enough," Heather reported. Along with another ultrasound, "I had everything from an echocardiogram to an MRI," as the team looked every facet of Twin-Twin Transfusion Syndrome / TTTS affecting the two fetuses and checked for any other abnormalities.
Later that day, sometime after 6 pm, Mike, Heather, and her mother met with Timothy Crombleholme, MD, FACS, FAAP, Director, and William Polzin, MD, Associate Director, of the Fetal Care Center of Cincinnati, as well as with other members of the fetal care team. "There were no problems as far as the babies' hearts were concerned," Heather recalled, but there were other problems that would require Heather and Mike to make a tough decision.
The decision was whether to first try amnioreduction—removal of excess amniotic fluid from the recipient twin—or to have laser surgery to coagulate or block the blood vessel connections between the fetuses. Because amnioreduction does not treat the cause of TTTS, but only one of its effects, the fluid is likely to accumulate again, which would require another amnioreduction. Some studies have shown a better outcome with laser surgery, known as selective fetoscopic laser photocoagulation, although it has not been shown conclusively that laser surgery reduces the incidence of neurological and other developmental problems. A large study sponsored by the National Institute of Child Health and Human Development at several medical centers, led by Cincinnati Children's Hospital Medical Center, is rigorously comparing amnioreduction and selective fetoscopic laser photocoagulation in patients with severe TTTS, but the results are not yet available.
"It was up to us to decide," Heather recalled. They weighed what they learned about the risks and benefits of both choices and called Dr. Vlastos. "He told me that he thought it was pretty serious and that I should do whatever I could," Heather said.
"We decided to go ahead with the surgery. It was a very hard decision."
'We Made a Good Decision'
That Thursday, Heather underwent fetal surgery at Cincinnati Children's to selectively sever blood vessel connections between the twins. She had an epidural for the procedure, which took 1 ½ to 2 hours. Dr. Crombleholme told her that he disconnected 11 blood vessels between the twins. "So it was a very aggressive form of Twin-Twin Transfusion Syndrome / TTTS, and we made a good decision," Heather said.
A few hours after the surgery, Heather was transferred by ambulance to Good Samaritan Hospital in Cincinnati, where she spent night. She recuperated for a few more days in a hotel. "The only pain was from the epidural itself," she recalled.
Heather, her husband, mother, and father, David Hoffman, who had flown in before the surgery, returned home on Monday. Heather remembers it was Halloween. She was told she should not have to return to the Fetal Care Center for the remainder of her pregnancy.
Throughout that time, Heather stayed on modified bed rest until delivery was and "followed extremely closely" by Dr. Vlastos. "He was the most incredible doctor," Heather recalled. "He always cared, answered every question, had all the time in the world just for you, and was really one of the reasons we have two healthy girls."
The Babies Are Born
Although she had had contractions the weekend before, Heather delivered her twin baby girls on January 27, 2006, at St. Johns Mercy Medical Center in St. Louis. As expected, she had a caesarean section.
"Dr. Crombleholme had told me that making it to 30 weeks of pregnancy is a victory and I made it to 31," Heather proudly reported.
She also proudly reported that the fruits of that victory, her twin baby girls, weighed in at 3 lbs, 15 oz, and 15 ½ inches for Olivia, who had been the "recipient" twin in utero, and 2 lbs, 9 oz, and 13 inches for Sophia, who had been the "donor" twin.
Although Heather left the hospital after four days, the girls had to spend almost two months in the hospital's neonatal intensive care unit (NICU). That was tough. She advises new mothers facing the same possibility to "mentally prepare yourself that your babies won't be coming home with you."
"It's very hard being a parent," when your children are in NICU, Heather acknowledged. Sophia and Olivia experienced "a lot of ups and downs. They went backwards for a while," and although Heather and Mike knew that was to be expected, that didn't make it any easier to handle.
'Take Nothing for Granted'
On March 22, Sophia and Olivia went home. They had monitors to check for any breathing and heart rates, but by four months were being weaned off the monitors. Heather trusted that the girls were medically ready to do without the monitors and admitted that the reluctance to let go was "a parent issue."
By late July 2006, Olivia was 16 lbs (considered normal for a baby that age). Sophia was 11 lbs and 5 oz.
They were still experiencing some acid reflux and were taking child doses of lansoprazole (Prevacid). They were also receiving physical therapy at home to help with range of motion, rolling over, and sitting. Their mother said that they are "healthy girls."
Heather's advice to parents facing similar situation is to "get as much information as you can and do whatever you can." She also said, "Don't wait. I know that there are many doctors that want to wait things out with Twin-Twin Transfusion Syndrome / TTTS. I truly believe we have two healthy girls because Dr. Vlastos sent us to Ohio and we acted quickly."
Although Heather used the internet to get information about her visit to the Fetal Care Center of Cincinnati and about TTTS, including patient stories such as this one, she wished she also had been able to talk to someone who had personal experience with TTTS. "Ask your doctor if there is someone who would be willing to talk to you," she advised. She has expressed her willingness to talk to expectant parents faced with a diagnosis of TTTS.
She also recommends that expectant parents "get and use as much family support as you can."
"Take nothing for granted," she says. "My husband and I prayed a lot."
