Our TTTS Story

 

27 weeks and one day . a milestone for us.. we no longer have a real due date.. just thank god for every day our babies are able to stay safe inside and grow .. ultrasounds are daily and we are monitored 24 hrs a day to make sure our babies have a heart beat .. the one sound that gives me peace💙💙

I am writing because I want to bring awareness to a condition called TTTS or twin to twin transfusion syndrome.  It is a disease of monochoronic ( one placenta) ,  diamniotic (two sacs) twins also known as identical twins. About 25% of twin pregnancies share a placenta and about 20% of those develop TTTS.   In the US it affects 7,500 babies or 3,800 pregnancies a year.  Left untreated mortality rates are as high as 80-100% .  It usually occurs in pregnancy during peak placental growth starting in week 16  proceeding to week 25, although it can happen at any time.  

The disease is a direct result of connecting blood vessels in the shared placenta.  These abnormal connections or “ anastomosis” cause blood, fluid and nutrients to unevenly be distributed between the twins.  The “donor “ twin gives his supply to the “recipient” twin.  In doing this, the twins develop a number of complications and go through stages defined by Dr Rubin Quintero and known in the medical community as the Quintero stages.  

Quintero Stage 1 is defined as polyhydraminos of 8 or more for recipient twin ( too much amniotic fluid ) and oligohydraminos of 2 or less for the donor twin (too little fluid.).  The overload of fluid puts the recipient at risk for heart failure and the lack of fluid for the donor stresses cardiac and renal function which can be irreversible.  Quintero stage 2 occurs when the bladder is no longer visible on ultrasound.  The amniotic fluid is made mostly of urine.  If the donor twin is not perfusing  his organs well, the bladder will stop working and his sac will have no amniotic fluid.  This can cause kidney disease and irreversible stress to the heart.   The twin can become “stuck” or “shrink wrapped” in his membrane.  Quintero stage 3 is the development of an abnormal Doppler or blood flow studies.  Quintero stage 4 is when hydrops or swelling of the body, brain or heart become and issue. And lastly, Quintero stage 5 occurs when one or both babies die. 

 I am one of the lucky ones who found this devastating disease before it was too late.  I am forever grateful to my angel doctors,  Dr. Ruben Quintero and Dr.  Kontopoulos  from the Maternal Fetal Institute in Miami, Dr Jesus Alverez Perez and Dr Al-Khan at Hackensack NJ, Dr. Julian De Lia , who although retired , reviewed my case and spent hours coaching me over the phone , my dear friend  Yvonne who has both a TTTS survivor and angel , Mary Salman the founder of the TTTS foundation and other twin moms who I “met” through Facebook support groups. 

TTTS is diagnosed on a simple ultrasound.  Amniotic fluid levels or the deep est vertical pocket of fluid has to be closely monitored in all single placenta pregnancies .  You should be under the care of a maternal fetal specialist who is familiar with Twin to Twin Transfusion Syndrome.  The TTTS foundation web page has a list of approved laser treatment centers in the US.  If you live in the NY | NJ area I would highly recommend Dr. Al Khan and Dr Alverez Perez’s group in Hackensack NJ. Although they are  not listed on the page, ( because they are not a laser center) they work closely with Dr Ruben Quintero , a pioneer in fetal medicine, and are experts on Twin to Twin Transfusion.  OBGYN’s are not twin pregnancy specialists.  Visits should be weekly.  The condition can occur at any time during the pregnancy and usually happens very suddenly. For me is was in 5-6  days.   There is nothing you can do to prevent it and nothing you did to cause it. There is some data on horizontal bed rest and nutrition to help the slow the disease but it has not been proven.

The treatment is selective reduction , where you terminate one twin to save the other, amnioreduction ( repeated amniocentesis where using a big needle you draw out the extra fluid) which usually needs to be repeated multiple times and can result in neurological damage  of one or both twins , membrane rupture and many other bad things, the “ wait and see approach “ where you do nothing and pray the babies don’t die  or have severe injury ( this has an 80-100% mortality rate) or a surgery called Fetoscopic selective laser ablation.  Laser ablation surgery is the preferred treatment.    It works by separating the babies circulation inside your womb.  Once separated they can no longer continue the potentially fatal donor recipient cycle.

I have been aware of all the risks since day one and have been under the care of a MFM.  Early in my pregnancy I went to another hospital in NYC.  There , I was told from weeks 14 to 18  that our babies likely have an “underlying genetic condition “ being expressed differently between them.  I was told twin A had a significant heart defect (which included a dilated aorta) and a recessed jaw. They also called the flow in the ductus venosis reverse ( a sign of severe blood flow problems). Twin B was found to have a posterior fossa cyst or brain cyst and , according to Them , likely Dandy Walker Syndrome, ( a devastating neurologic disease that results in severe disability.). They suggested I meet with a neurologist so I could understand how poor the quality of life would be for my boy when I refused termination.  When I asked about an MRI they didn’t seem to think it was necessary but agreed to do it at 20 weeks. Twin B was also very small (twin a in the 90% for weight and b in <3% for weight) and has only a 2 vessel umbilical cord. Many babies even singletons thrive with a  2 vessel cord but they used this information to further suggest genetic defect and termination.   The attachment of the umbilical cord to the placenta for baby b was marginal and hanging off the side.   They again encouraged termination because he could “go at any time” and that would potentially cause demise of baby A.  At every visit I felt as though they saw that as my only option.  I was told at one visit “most Mother’s at this point would just start fresh.” It was a nightmare.

My symptoms started becoming significant at this point. I had severe  shortness of breath and began to have significant abdominal tightness.  My dear friend who lost her baby to TTTS earned me about this many times.  My amniotic fluid levels were so close to TTTS around 3 and 12.  The last few visits I had there they would tell me how many things were wrong ( still with no diagnostic tests other than early ultrasound.) and when I asked about my TTTS symptoms I was  told, “laser surgery is only for people with healthy babies  and you don’t have healthy babies.”   That was when I decided I needed to see someone else.  I was heartbroken and would cry each week.  I knew termination wasn’t an option for me.  But I did question myself, how could Columbia be this wrong about both twins.   I did know enough medically that they should have waiting for an echocardiogram or Britain MRI before recommending “starting fresh.”  There were so many things that they had found and all supported the story that there was something very wrong.  In my heart I still couldn’t believe it.  Palm Sunday weekend James took the girls to our families and I just shut down.  I needed a plan.

I knew of Dr Quintero and had already reached out to his office.  They had all my records and I had even spoke with him a few times  directly about my case and situation.  He is that doctor.. one that heals not only your body but also your heart and mind.  He would return my calls before I was even a patient.  I posted in one of my support groups late Sunday night what I was told and was asking for help.  An angel mother wrote back to me and gave me Dr. Quintero’s cell phone number . ( see her message in my pictures). She told me to call him right away.   It was 9:30 Sunday night.  I could not believe that I could just call a world renound doctor on his cell at 9:30 when I wasn’t even his patient.  Desperately, I called.  He spent about an hour on the phone with me , reassuring me, and explaining very simply, “statistically your babies are ok.”   He said I will not sugar coat anything , in a professional but very kind voice , but we need to do tests to prove if your babies are healthy .  Do not do any procedures and come as soon as possible.  I trusted him.  Maybe it was because of the thousands of posts in my support group of Mother’s just like me posting how he saved their babies and is a miracle worker.  Maybe it was his pristine resume and the fact that he even invented the tools to do the surgery… I don’t know why but I knew when we go there we will have an answer and I will trust him. We chose to visit him at the fetal institute in Miami.  We are blessed to have tremendous support form our families and my parents moved in to watch my girls and keep their life as uninterrupted as possible.   Once there He immediately performed his own ultrasound on me.  He spent over an hour checking every little area he could.  He told me I did not yet have ttts and he didn’t see anything with baby a’s heart or jaw.  He did see the cyst and wanted more testing.  He also wanted more testing on the heart to confirm.  I saw a neurologist and had a fetal brain MRI.  He also arranged for us to meet a pediatric cardiologist and have a fetal echocardiogram. The neurologist diagnosed the brain cyst as a Blake’s pouch.  A small embryonic reminant that is benign and will likely disappear at 30 weeks.  The cardiologist did not see any issues with my babies heart.  He also confirmed the flow in ductus venosis was normal.  He advised that although baby b was small he was in stage one SIUGR and had normal flow in his umbilical Doppler.  He advised that In stage 1 the risk of fetal demise is very low and I did termination should not even been discussed or considered. I cried because I could not believe it.  My babies might be ok. After weeks of visits thinking they were deformed and had severe problems .. we were discovering they were ok!!! He was concerned about the fluid and although it was not quite TTTS at our first visit with him he suggested we come back in a week and he would then be able to tell which direction we were headed.  We returned a week later and we still did not meet criteria for TTTS.  We did not need surgery! He did however biopsy a piece of my placenta to rule out any genetic defects.  The next few weeks waiting for those results were tourture.  After our experience at Columbia I had a hard time accepting everything could be ok.

Dr Quintero then suggested I follow up with Dr Al Khan at Hackensack and Dr Alverez Perez.  He assured me he would be in contact with them and Could even log on remotely to the computers to see all my ultrasounds and reports and arranged for us to meet with them.   When we returned home Dr Quintero continued to remain in contact with us.  We had a great experience following up with the team at Hackensack and were getting good news every week.  Once again we were in the hands of highly skilled doctors who also cared. I have everyone’s cell number and again was encouraged to call at any time for anything!

My nightmare came true when we were diagnosed at 26 weeks with TTTs .  My intention is not to speak badly of Columbia but rather to inform anyone  going through this.  This was my experience and although that facility was not right for me .. it may be good for someone else.  In my support group, many woman have a similar experience at CHOP and Columbia and terminate very quickly.  We did not go to chop because it is usually very similar opionion to Columbia according to Mary who runs the TTTS Foundation and the thousands of woman in my support groups.  Ultrasound early on is not the best diagnostic tool. It is good but hasn’t limitations.  If issues arise request echos or MRI to better evaluate your babies tiny body .  Listen to your heart … do not feel pressure to terminate and if your doctor does not care about your babies please run and find a new one!

At Hackensack ,  I see my doctor and speak to him at every visit.   Its more than a tech doing an ultrasound.  At the fetal institute Dr Quintero does all his own ultrasounds.  At 26 weeks, we had our routine visit .. babies fluid levels were 4 and 3 ( stage one ttts is 8 and 2) and they were healthy.  We were told we could schedule appointments every 2 weeks now but I insisted on weekly visits knowing how quickly and deadly ttts could be.  I also remembered the symptoms and felt them developing as well as some contractions.  The dr agreed.  We did find out the results of our Cvs placental biopsy and micro array and everything was normal.  A few days later , the contractions and  increased and my belly seemed very uneven, right and painful. I also had significant shortness of breath.  All are  signs and symptoms of this terrible disease.  We went to the hospital and were diagnosed with stage 3 TTTS.  Baby b was shrink wrapped so tight inside his membrane you could not even see his profile.  He had no visiable bladder and no fluid.  Baby a ,our recipient , had a fluid level of 12 ( 8 or more is required to dx ttts) and his heart was becoming stressed from the volume overload.  He was in danger of heart failure, hydrops and demise.

We had to make a decision.. we could deliver our babies at 27 weeks or we could have a life saving surgery and give them time to heal and grow.  I have an anterior placenta and that alone makes it very difficult to perform laser however Dr Ruben Quintero developed the tools used and technique to perform the surgery successfully. We walked in tears to Dr Alverez Perez’s office.  He had already spoken to Dr Quintero who agreed at this time surgery would be our best option.   I had trust in Dr Quintero as well as Dr Alverez Perez.   He is revolutionary in fetal medicine and both he and Dr Alverez Perez were confident that would give my babies the best chance.  I was willing to do anything to save them! We knew if we delivered now the babies faced a questionable fate. Baby b had decreased blood to all his organs and baby  a had  too much and was suffering from volume overload. Also baby b was about half the size of baby a and although we were at 27 weeks baby b was measuring closer to 25 weeks. 

I was scared to fly in so much pain and with my contractions.  I was given one dose of procardia to help quite the contractions and a steroid shot to help my babies lungs develope in cas I went into labor.  Dr Quintero was texting us at midnight on the plane to set up a time to meet in his office.  Within 12 hrs of our diagnosis,  we were having surgery.  A camera is inserted into the womb through a small incision the size of a staple.  A laser is then used to separate the blood flow and break the donor recipient cycle. I watched and couldn’t help tear as I saw their tiny bodies on the screen next to a laser beam.  My babies vessels were not typical .. they were very very large about 5x the size.  Dr Quintero said it is very rare and he has only seen about 20 cases.  He advised that if he lasered the vessels and separated them , as they do in the traditional surgery, we would have lost them both.  He performed a procedure called Supraselective laser surgery and only ablated some small vesselsto balance their blood supply.  Like a artist, he traced each vessel and only laserwd the ones that were “extra” or throwing off the balance not touching the larger ones.  He told me my boys are so very connected.  I have no doubt that if I had this procedure anywhere else they would have ablated every vessel as you usually do and both of my babies would not have survived. He could not promise complete recovery but he felt confident this would get the boys to a more sustainable gestational age.

Today, I am so very happy to report our boys are doing well and thriving.  It is a miracle.  They both have even fluid levels and are not showing any signs of cardiac distress.  Baby b also has a visible bladder on ultrasound and is no longer stuck to his membrane.  Dr Quintero is hopeful that we will be able to continue many more weeks into our pregnancy and give the boys the best chance possible.  Our post op course has been more complicated than most but we are getting there!! 

We have already made it one extra week thanks to the surgery.  We are counting days now and every 24 hours increases their chances of survival .  One NICU nurse told me 1 day in the womb saves 5 days in the NICU.  I truly believe our babies are still alive because of Dr Quintero, Dr Al Khan, Dr Alverez Perez  and the mothers of survivors who dedicate themselves to educating other twin moms. Even as a medical professional, I found personal experience from other mothers on Facebook support groups to be one of my main resources.  Their knowledge and support is endless. I would also like to praise the medical staff in both the ICU and labor and delivery at Wellington Hospiatl.  Dr Quintero’s office is in Maimi but he operates in Wellington.  Every nurse, especially Sam , JB , Charlotte and Rebecca the director went above and beyond for me and my husband! As we left Dr Quintero in Miami he said to us please keep  my cell number and know you can call me anytime FOREVER💙💙

I am hoping the story of my sons TTTS journey brings some awareness to this and if you hear of a friend pregnant with identical twins you remember and take a little time to share.  That’s what my dear friend did for me and it helped save my boys.  Use Facebook groups and find foundations.  There is nothing like the love a mother who has suffered a loss .. the passion and drive these woman and men have is unbelievable and they will help educate you and connect you to the medical leaders.  Find someone that has the knowledge but also cares about you and your babies.  I have that in both Dr Al Khan, Dr Alverez Perez and Dr Quintero.  I feel so blessed to have a team that is skilled beyond compare but who also are compasionate and supportive and always available to me for any questions I have.  I always knew I was doing everything I could possibly do for my boys. 

Do not settle. 

Many mothers go undiagnosed .. others are sent to a mfm too late .. there are many stories of moms who terminate because they are not educated of the options.. we are lucky and so very blessed to have been given this chance.  

I believe in the miracles and angels .. I know we have many. .. after 7 different mfm, 3 different pediatric cardiologists, 2 brain mris and a week stay in the intensive care unit.. we can say we are carrying healthy babies.. I am so lucky to have had 2 perfect pregnancies .. for many this is all they know.. my family..  your help and endless love, I could never thank you enough .. especially my angel mother who taught me to how to be a mom .. from the very first heart beat.. and my husband my true rock.. who supported my every decision and has not left my side you are my everything and I pray our boys are as strong as you are  and they continue to be as strong as the men they are named after .. Peter & Maurice 💙💙

 

 

 

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