Of all the questions that concern TTTS couples, this seems to be the most important so the answer will be the most comprehensive. The treatments for TTTS pregnancies depend, in part, on when in pregnancy the twins become affected.
If the twins are mature enough to survive outside the womb (beyond 25 weeks), immediate delivery is an option for TTTS babies. However, the doctors must weigh the health effects of the prematurity on the twins versus the continued effects of the TTTS abnormalities as they try to prevent any handicaps in the survivors.
The Foundation continuously reviews the latest medical scientific reports (see section on Medical Research and Articles) to determine for TTTS couples which treatments appear to lead to the highest survival rates for the babies, the highest number of healthy survivors, and the lowest rates of prematurity for the twins.
The Foundation also recognizes that it is impossible to save all TTTS twins and completely eliminate the risk of handicap in survivors regardless of treatment because of the nature of the placental abnormalities in TTTS.
The different treatments for TTTS can be classified into those that address the connecting blood vessels in the monochorionic (shared) placenta, those that treat the symptoms, and, sadly, those that reflect a sense of hopelessness on the part of the doctors. We know that none of the treatments have been evaluated in what doctors call ‘randomized studies’ or ‘comparative trials.’
A randomized trial means taking a group of TTTS cases and only treating half, then comparing the outcomes in the treated patients to the untreated patients. Such studies may be reasonable in other diseases, but the well documented, historic TTTS death and morbidity (damage) rates calls into question the ethics of such studies.
The Foundation does not support this type of experiment for TTTS parents. A comparative trial takes TTTS cases and treats them with one of two or more available therapies to see which has the best outcomes.
Fetoscopic Placental laser Therapy: Since TTTS does not exist in identical monochorionic (MC) twins without the connecting placental blood vessels, it seems reasonable to find a way to separate the twins’ blood streams by destroying the connections.
The availability of surgical lasers in the early 1980’s led this innovative option, and the first fetoscopic laser occlusion of the connecting vessels was performed in 1988 by Dr. Julian E. De Lia at the University of Utah on a couple from Great Falls, Montana.
By linking the laser to fetoscopy (the insertion of a tiny telescope into the pregnant uterus), doctors are able to see and destroy all the connecting vessels. This operation is the only TTTS treatment that can ‘disconnect’ the twins and stop both the chronic transfusion of blood from the donor to the recipient, and the acute or sudden transfusion should one baby pass away or become suddenly ill.
This laser surgery is now performed in centers throughout the world as more and more doctors are convinced that this will lead to the best outcomes. To the best of our knowledge, placental laser surgery results in the highest numbers of healthy survivors in those patients with previable (i.e., less than 25 weeks’ gestation) onset or diagnosis of TTTS.
Reports on this therapy currently indicate the following: 80-85% survival of at least one twin, 65-70% overall twin survival, 5% or less significant handicap rates in the survivors, and a treatment to delivery average interval of close to 10 weeks.
The results may vary in different centers, and this may reflect different levels of experience or the actual surgical techniques used. The procedure involves general anesthesia for the mother, and the surgical complication rates also vary in different centers. These must be discussed with the individual doctors performing the surgery.
Therapeutic Reduction Amniocentesis: This is the ‘most widely available therapy’ and involves draining the excess amniotic fluid (polyhydramnios) by inserting a needle into the amniotic sac of the recipient.
It relieves the mother’s abdominal discomfort, and prolongs TTTS pregnancies by reducing the risk of spontaneous rupture of the membranes or premature labor from the enlarged uterus.In addition, by reducing the pressure inside the womb, it has been reported to change the nature of the transfusion in some TTTS cases.
Survival rates with amniocenteses approach 80% in some centers, but health status of surviving infants are not always reported. Recent reports (see section of Medical Research and Articles) indicate a 60-75% survival rate with amniocentesis, but 10-30% of the survivors have had neurological (brain) abnormalities when ultrasounds were performed in the nursery.
Since the babies remain connected, there is the continuing transfusion process between the twins (both chronic and acute). This may explain the higher rate of medical problems in the surviving babies than methods that disconnect the babies.
Amniotic Septostomy: This is the intentional creation of a hole in the membrane septum between the babies’ bags of water with a needle during ultrasound scanning. It allows for some of the excess amniotic fluid in the recipient’s bag of water to enter the sac of the donor who usually has no to very little amniotic fluid. In one report of 12 TTTS cases, the septostomy to delivery interval was 8.5 weeks and 83% (20/214) of the twins survived.
Medication Therapy: Several drugs have been used in TTTS cases for various purposes. These include:
Termination of the Entire Pregnancy: When pregnancy termination (induced abortion) is recommended by doctors and counselors, it is done so not as a treatment, per se, but as a reflection of their lack of faith in the available therapies for TTTS.
The Twin to Twin Transfusion Syndrome Foundation is a pro-twin and, therefore, a pro-life organization. We are in the business of hope, and there is always hope. Certain doctors refuse to believe that parents would risk any health problems in their surviving TTTS babies, and would terminate all TTTS cases despite the fact that the majority of twins survive and are normal regardless of treatment used.
Some physicians accomplish this end by ‘passive neglect.’ This is where there are signs of significant TTTS on ultrasound, and the doctor asks the patient to return weeks later instead of one week hoping the pregnancy ends in the meantime.
The babies can live, please give the parents the opportunity to save their babies. They all deserve the ‘right to try.’
Selective Termination of One Baby by Various Methods: The termination of one twin (with the hope that the pregnancy will continue for the other) is undertaken for various reasons.
Perhaps one reason is when a severe birth defect baby, known as an acardiac MC twin (here an identical twin is deformed and does not have a formed heart), is kept ‘alive’ by the normally formed twin who pumps blood to the acardiac twin through the placental blood vessel connections. The normal ‘pump’ twin may go into heart failure due to the strain.
The various techniques to separate these twins include ligation of the umbilical cord with suture, or the cauterization of the cord with laser or electric current. Methods must be used that occlude the umbilical cord or major fetal vessels in the deformed baby to avoid death or damage (especially of the brain) to the other normal baby as a result of an acute transfusion through the connections.
Sadly, some doctors use this technique in TTTS when they either fail at attempted laser surgery, or when they feel that one twin may have a poor outlook. We have seen babies in heart failure with hydrops, a chosen baby for cord ligation, have laser surgery and live and be completely healthy.
With few exceptions the pregnant woman’s health status is virtually ignored once the twins are determined to have TTTS. Both the doctors and couples focus on the ultrasound findings and condition of the babies. This booklet is to help you understand ways that mothers can immediately help even right after you have diagnosed them.
There are things that mothers can do to benefit the babies and themselves right away, which may play a role in the outcome of TTTS pregnancies. These are related to changes in diet and activity (see section on Nutrition and Bedrest).
It seems that most women with TTTS at mid-pregnancy are found to be malnourished. Anemia, low blood protein, decreased calorie intake and dehydration are common findings. Many women with multiple gestations have morning sickness or poor appetites that may be worse than that seen with only one baby. It may be frustrating and upsetting not to be able to eat well.
This below normal intake of nutrients, combined with the needs of twin babies and some of the mother’s changes in TTTS (e.g., a womb that is more than twice the normal size for the time of pregnancy) may all contribute to the development of malnutrition. There are two unusual circumstances in TTTS that could make a mother’s weight go up despite decreasing your dietary intake. These cause inaccuracies in determinations of nutritional status when one weighs themselves on a scale:
The Foundation has explored a number of ways for women to help solve this problem and found that the most efficient way to recover the losses, and prepare for the rest of the pregnancy, is to take liquid dietary supplements (e.g., Boost, Carnation Instant Breakfast, Ensure Plus, and others that are soy based) sipped slowly, continuously throughout the day in addition to whatever you can eat at meals.
If the morning sickness is still present, encourage your patient to try your best with the supplements and liquids until it passes. The sensation of thirst is also common, and seems unusual in the face of excess body water.
We recommend soy milk or athletic drinks (e.g., Gatorade.) to provide more than just water the mom and your babies.
The benefits of best rest (or lying on a couch or the floor) in multiple gestation has always been debated by doctors. However, TTTS is a high-risk complication in which all possible beneficial remedies should be utilized to improve outcomes.
Horizontal rest should be used. Horizontal rest (on the mom’s side) will help blood flow and oxygen to the womb, and aid in removing some of the excess edema fluid in patient’s body.
Work is problematic for many women affected by TTTS. Studies have shown a definite relationship of physically demanding work to adverse outcome in pregnancy. Given the high-risk nature of a TTTS multiple pregnancy, we recommend a leave of absence for the duration, especially if the patient is undergoing one of the treatments outlined above.
Twin to twin transfusion syndrome affects 15% of monochorionic identical twin pregnancies, but at least 20% when you include all the losses before 20 weeks not included in the statistics. We estimate 4,330 cases annually in the U.S. (most definitely much higher) so you are not alone. It occurs from abnormalities in the twins’ shared placenta that occur spontaneously and, as far as we know, cannot be prevented.
The outlook for twins with TTTS was hopeless over 30 years ago, but now we have the ability to diagnose the condition early (with ultrasound scans) and implement treatments that will ultimately lead to most of the twins surviving and being healthy. TTTS is still regarded, though, as one of the most challenging problems in modern obstetrics. For you, you have been forced into a high risk world that no one ever talks about or that you could ever be prepared for.
We strongly recommend that you create a medical plan of action from the moment your twins are determined to be monochorionic, because the expertise and interest in TTTS varies widely among doctors.
It is considered a ‘rare disease’ so most physicians see only a few cases a year if at all. You have to be your babies’ advocate. It is important to have consultations with a high-risk obstetrician (perinatologist or maternal-fetal medicine specialist), to have frequent ultrasound scans, and be aware of the TTTS warning signs.
Do not sit back and listen to doctors or nurses who chalk everything up to it being ‘just twins’. Begin to educate yourself on the syndrome and the treatment options so if things get worse, or if an immediate decision regarding treatment is necessary, you can choose what you feel is the best for you and your babies. Follow that inner voice inside you, and trust it.
Sadly, some of our TTTS parents have had to fight for their babies all the way with their doctors and insurance companies. You want to know in your heart, now and for the rest of your life, that you did everything possible to save your babies.
The Foundation is a pro-twin and pro-life organization that is here to help you and your babies in your fight every step of the way, with educational materials, emotional support, and professional referrals. We are here for you.
Nutritional supplementation, evaluation of the uterine cervix and bed rest in the mother with a multiple gestation are extremely important. Specific need for cervical assessment by ultrasound, increased protein and calories and bed rest may be even more important with a diagnosis of twin to twin transfusion syndrome (TTTS).
Recognition of these maternal issues will lead to better outcomes for the twins. In TTTS, it is the polyhydramnios (excess amniotic fluid) which most often leads to the loss of the pregnancy.
This common symptom may be managed effectively by reduction amniocentesis. However, the over-expansion of uterus, especially when the diagnosis of TTTS is made late and the uterus is massively enlarged, may damage the uterine cervix (it becomes abnormally too short to keep the babies inside).
These TTTS mothers are then at increased risk for premature rupture of the membranes, and labor and delivery of immature to very premature babies. This variable may also be responsible for losses that here-to-fore were thought to be complications of the various treatments (e.g., laser surgery, reduction amniocentesis).
The ultrasound machine can provide important information regarding abnormal cervical changes, before they can be detected by vaginal examination alone. Studies have shown that the shorter the cervix in twin gestation at mid-pregnancy, the more likely a premature delivery (see Skentou et al., attached).
If severe shortening is detected early, the physician may stitch the cervix (cerclage procedure) to provide the necessary strength to reach term. Cervical assessment and cerclage placement will improve the outcomes (survival, duration of pregnancy, healthy survivors) that were reported for treatments in the last decade.
For instance, it may explain why 25% of TTTS cases treated by laser surgery in one center (see paper by De Lia et al., in Treatments section), had no surviving babies. Since cervical assessment with ultrasound has now become routine in their center, 20-30% of TTTS cases either come with a cerclage in place, have it placed there after the laser, or return home to have it placed. The cervix should be checked frequently by ultrasound in TTTS.
Bed or horizontal rest is another means to reduce pressure on the cervix in multiple gestation, with or without TTTS. Having the mother lie on her side, while on a couch, bed, recliner, lawn chair, or floor with her kids, helps reduce pressure on the cervix.
Horizontal rest also improves blood flow to the mother’s uterus (where the TTTS twins may be struggling due to the abnormalities in the placenta) and kidneys (where the excess water in the mother is eliminated).
We highly recommend that parents call the National Sidelines Organization and order one of their packets on bedrest. We have enclosed some of their information for you to read in the meantime.
Dr. Julian De Lia has investigated the nutritional aspects of TTTS. He recommends that his patients drink 3 cans per day of either Boost or Ensure High Protein in addition to their meals. Dr. De Lia explains, “TTTS patients at mid-pregnancy have severe hypoproteinemia and anemia.
These maternal metabolic parameters may influence fetal TTTS characteristics and explain maternal sensitivity to intravenous fluid in complicated multiple pregnancies in general. We believe our data are sufficient to support the use of nutritional supplements in the management of early (monochorionic twins with subtle growth and amniotic fluid differences) and severe TTTS regardless of specific invasive therapy used (i.e., fetoscopic placental laser surgery, reduction amniocentesis, septostomy, etc.)
We at The TTTS Foundation suggest nutritional supplementation and have seen nutritional therapy benefit many women. They report less fatigue and more energy, and on occasion, the fetal signs of TTTS improve (less polyhydramnios) obviating the need for invasive therapy. Mothers also feel immediate satisfaction knowing that that there is something they can do right away to help their babies.
Twin to twin transfusion syndrome is still being described by some as enigmatic and poorly understood. The recognition of the above maternal variables is exciting and new. We have no doubt that adding these to the TTTS paradigm will improve outcomes of TTTS babies regardless of therapy, whether directed at the placenta or the symptoms.
Nutritional supplementation, ultrasound assessment of the cervix (with cerclage placement when necessary), and horizontal rest are within the scope of any physician.
