Twin-to-twin transfusion syndrome (TTTS):
A story of successful prenatal treatment
Life-Saving Baby Doctors—The Story of My TTTS Twin Girls
Have
you ever heard it said that we appreciate something more when we’ve nearly lost it? As I type, I listen to the giggles and baby
chatter of our beautiful twin babies and intensely feel the truth of this statement. I would like to share our story of our
babies’ journey into this world and how they were saved from an almost-certain death
before birth by a very special doctor.
It
all began on a sunny morning in late July last year. I had planned to arrive late to my
investment job because I had to stop by the perinatologist's office at California Pacific
Medical Center in San Francisco for what I thought would be a routine ultrasound. My husband Spencer was out of town on a business
trip, so I was alone for the visit. 16 ½
weeks pregnant with our identical twins, I still had intense morning sickness that lasted
all day, but as this was my first pregnancy, I didn’t know that this was abnormal,
and was just thrilled at being pregnant so I didn’t mind the side effects. At my
appointment, the ultrasound technician very professionally went about the business of
scanning and measuring our babies. She then left the room and conferred with our
perinatologist, Dr. Carl Otto. He then came in after reviewing the ultrasound pictures
with a grim, drawn look. "I'm so sorry to tell you, but your babies are suffering
from Twin-to-Twin Transfusion Syndrome," he said. I sat,
stunned, not knowing what it was, but presumed it was serious. He provided me with
devastating information and statistics that confirmed my fears.
Twin-to-Twin
Transfusion Syndrome (TTTS) is a disease that strikes about 10% of all identical twin
pregnancies. In the US, it affects over 7,500 babies—or 3,800 pregnancies—each
year. This serious condition occurs when twins share a single placenta (or afterbirth),
which contains blood vessels connecting the twins’ blood streams. In a sense they are like conjoined (or Siamese)
twins, but are connected in the placenta instead of their bodies. One baby (the recipient) may get too much blood,
while the other (the donor) is losing blood through the abnormal connections. The recipient may die in heart failure from a
cardiovascular system overload, and subsequent over-production of quarts of amniotic
fluid. The donor may die from the loss of blood, and tends to have very little amniotic
fluid. While the twins begin development totally normal, the placenta abnormalities cause
their subsequent death or serious birth defects. The
loss rate may be as high as 80 to 100 percent for twins who develop TTTS at mid-pregnancy
(the second trimester). TTTS babies may die in the uterus or at birth from prematurity.
More than half those who survive suffer from many serious birth defects including cerebral
palsy.
There
were several courses of treatment that Dr. Otto discussed with me that day. The first
option was termination of the pregnancy, either directly through abortion or by letting
nature take its course with inevitable miscarriage. This was absolutely not an option for
us. The second was reducing the excess amniotic fluid that developed around the recipient
baby, done via an amniocenteses. At a 50%
chance of surviving twins, this procedure might balance the amount of amniotic fluid
surrounding each baby and hopefully spur the placenta into regularity once again. The
third was an in-utero surgery called fetoscopic laser occlusion of the connecting vessels,
pioneered by an obstetrician named Dr. Julian E. De Lia.
Because of the situation’s urgency, Dr. Otto recommended an
amniocentesis within the next hour, and then recommended I call Dr. De Lia later to learn
more about his surgery.
Terrified
and panicked, I called my husband Spencer from the doctor's office. We agreed to do the
amnioreduction right away, but the next plane would not bring him home until evening. So I called my cousin Ben, who rushed over from
work to sit with me through the procedure. The
period between the diagnosis and seeing my cousin was the most agonizing and lonely time
of my entire life. I sat in the doctor’s office lobby by myself and cried openly. I felt hopeless and helpless, wanting to save the
babies who were struggling for life inside of me but not knowing how. During this time, an
elderly woman approached me, sat down and gave me a long, silent hug. To this day I think
she was an angel here on earth to reassure me and keep me strong before the procedure. Dr. Otto took 2 liters of fluid from my uterus
that day, and ordered me to remain at bed rest until my appointment 4 days later.
I
left a message that very day for Dr. De Lia. He didn't know anything about me and really
didn't owe me anything—he only knew me through the voicemail I left, choking on my
own words as I tried to get through a message asking for information from his office. He
called me from his home in Milwaukee late that same night, and spoke to Spencer and me for
nearly an hour about TTTS, its treatment, and the efforts we ourselves could take to
combat our helplessness in battling this deadly disease. He suggested horizontal rest and
nutritional supplements (such as Boost or Ensure) as ways to help the status of the twins. Besides the laser surgery, his research of TTTS
also found that the majority of TTTS mothers are severely anemic and low in blood
proteins. He then told us more about the placental laser procedure.
The
technique uses a small 3.5millimeter scope to identify, and then use the laser to
coagulate the connecting vessels between the twins. The twins become separate and the
passage of blood from one to the other stops. The surgery is performed in
"pre-viable" pregnancies (less than 25 weeks), where delivery of the TTTS twins
is not an option. Dr. De Lia told us that for now, my best bet was to drink my Boosts, stay on bed rest,
and continue the amniocenteses if necessary. Our twins would only be candidates for the
laser surgery if they met certain "criteria", which included severe size
differences between them or evidence of impending heart failure. We ended that call with
the hope we'd never meet, because we prayed the simple measures would correct the
condition and make surgery unnecessary. Meanwhile,
he suggested we contact Mary Slaman-Forsythe, the director and founder of the Twin to twin
Transfusion Syndrome Foundation in Cleveland. We
did so and Mary immediately sent us a comprehensive book on the history, placental
abnormalities, treatments, and success stories in TTTS. So although I was nearly
hysterical with panic and grief, I had enough lucidity to gather the necessary facts,
speak with Mary and with Dr. De Lia, and make the decision with my husband that if
necessary, I would undergo this surgery. As
fate would have it, my twin brother Christian and I were born, 29 years earlier, at the
very same hospital from which Dr. De Lia operates-- St. Joseph Regional Medical Center in
Milwaukee, Wisconsin. This fact only solidified my decision, as I felt this was somehow in
the plan.
One
agonizing week later, and after a second amnioreduction of 1 more liter of fluid, the TTTS
appeared to be getting worse. The twins' size
difference was greater, our recipient’s heart was starting to thicken, and Dr. Otto
felt the future bleak for the twins without Dr. De Lia’s laser surgery. Mary and the TTTS Foundation arranged for us to
fly complementary on Milwaukee’s Midwest Airlines.
They have a program called ‘Miracle Miles,’ where frequent fliers
donate mileage so that families with sick children can fly to leading medical centers for
treatment.
I
underwent the laser surgery on August 14th, 2002, at only 18 1/2 weeks of
pregnancy. In the operating room, Dr. De Lia keeps prayer cards of Saints Gerard and Jude,
the patron Saints of pregnancy and hopeless causes. Just before the surgery, we said a
prayer together and asked St. Gerard to help our babies stay strong. I drifted off under
the anesthesia, knowing that I was in the hands of a faith-filled surgeon who is totally
dedicated to saving little twin babies with TTTS.
The
surgery which Dr. De Lia pioneered is a miracle to thousands of otherwise hopeless
expectant parents. In 90% of the cases where
the operation is performed, at least one twin survives, and in 68% both twins live. Less
than two percent of the survivors have any disabilities or birth defects, compared to up
to 30% with some other treatments. Dr. De Lia began his TTTS research in 1983, and five
years later the first placental laser surgery was performed
October 3, 1988, at the University of Utah Health Sciences Center in Slat Lake City. The
learning curve for the procedure was completed in 1994. Although other centers in the U.S.
now perform this surgery, I found it strange that none of these surgeons studied under Dr.
De Lia. Sadly, they are putting TTTS babies
through redundant learning curves, which result in lower survival rates and more handicaps
in survivors. For me, I never questioned my decision to ask Dr. De Lia to perform the
surgery as opposed to one of these other centers, as I felt most confident in the surgeon
who created the procedure, and I thought his experience offered me the best shot at
keeping my babies and me safe and alive.
I
sat waiting, following the surgery, hoping of course for the best outcome but still
preparing for the worst as I had been since that July day. I was unable to return to work,
as I was ordered to strict bedrest for the remainder of my pregnancy, which hopefully
would last another 10 weeks, when they would be at 28 weeks’ gestational age, a big
milestone in the eyes of medical professionals. I
felt understandably superstitious about having baby showers and doing even the most basic
preparatory work for what I desperately hoped would be our impending two arrivals. Also
during this time my husband's mother passed away and so he flew to Kansas for 3 weeks just
a month after my surgery (I of course couldn't go). I was so fortunate to have many
friends and family members flying out to San Francisco to care for me while I sat, in bed,
thinking and dreaming about these babies, and aware of their every twist and turn inside
of me.
On
December 15th, 2002, over eighteen weeks after their in-utero surgery, our
beautiful little angels Sophie and Sara were born....small of course, but otherwise
healthy and perfect. As I rub noses with
these two tiny and delightful faces and hear their squeaks and babble, I am again
overwhelmed with appreciation of what we have almost lost.
I feel absolutely gutted at the prospects of where we'd be if we had not
found out soon enough, or if we had decided to terminate the pregnancy, or if Dr. De Lia
hadn’t dedicated his life’s work to curing the seemingly incurable. The world
would be deprived of the rays of light that are our girls, who are the most resilient,
joyful and delightful babies I've ever known. We
are so thankful to our physicians: Dr. Otto who diagnosed the disease early and was
informed enough to give us a chance to help them; and Dr. De Lia, whose years of study and
caring hands performed the surgery that saved our babies’ lives. Our babies would have had no chance without treatment, or if we had
opted to terminate the pregnancy. His surgery, which is starting to be offered in centers
throughout the world, offers incredible hope for a healthy life for two or more lives, as
yet unborn but certainly not untested. These two men are our friends for life. We are also so thankful to the Twin to Twin
Transfusion Syndrome Foundation for increasing awareness of the disease, for giving us
support and encouragement, and for arranging our flight across to Milwaukee. Every August 14th, , on the anniversary
of our babies’ life-saving surgery, we will celebrate a little mini-milestone for the
girls- the day they were given a second chance at living and thriving as they do today.
August 20, 2003
email: kof@dodgeandcox.com
Other sources of information on TTTS:
www.TTTSfoundation.org The Twin to Twin Transfusion Syndrome Foundation
www.TTTSMD.org
Twin to Twin Transfusion Syndrome