The Fetal Heart Team at DC Children’s Hospital provided us with great insight on our new little family’s future. Please don’t hesitate to ask us any other questions not included here!
Yesterday, Dan and I received the results of the Quadscreen – a maternal blood test that looks for AFP, hCG, Estriol, and Inhibin-A. It screens for chromosomal defects including DiGeorges Syndrome, Trisomy 18, and Down Syndrome. The results were NEGATIVE! It is a guide, not a diagnosis, so a negative result means there is a less than “normal” risk that Walter may have a chromosomal defect (less than 1 in 4,000) and not an increased risk. We will be confirming this result with an amniocentesis on Thursday, so prayers are greatly appreciated to get us, and our adorable little warrior through this safely.
1) What is Tetralogy of Fallot, exactly?
Tetralogy of Fallot (ToF), also known as “Tets” is a congenital heart defect present in about 1 in every 10,000 babies, fewer than that are identified in-utero, and even fewer require repair. There is no known cause of Tets and this anatomical defect in the heart is already formed between weeks 2 and 3 of gestation, before a mother will even know she is pregnant.
Our doctor indicated that she feels the name in itself is outdated, as the “tetra” in tetralogy implies four defects of the heart (“Fallot” is after the scientist who named the defect in the late 1800’s). These defects are identified as:
Ventricular Septal Defect
Valvar and Infundibular Pulmonary Stenosis
Right Ventricular Hypertrophy
The strikethrough seen above is precisely what our cardiologist did when she sat down to discuss Walter’s diagnosis. She explained that the Overriding Aorta (misplacement of the aorta) is an abnormality that accompanies the others, but in isolation causes no functional difficulties in the heart. She advised it is more a scientific observation than contributor to the overall defect. Right Ventricular Hypertrophy (a thickening of the heart muscle’s wall)also is not an aspect of the defect itself, but rather a result of the defect should it go uncorrected, as the right side of the heart works overtime to pass more blood through the pulmonary artery.
Ok, so we are down to two defects, which already makes me feel better.
Ventricular Septal Defect is, for all intents and purposes, a “hole” between the lower chambers of the heart. This allows very small amounts of oxygenized blood to flow back into deoxygenized blood. Surprisingly, this is the less severe of the defects. I am told how this can be fixed with a “simple patch”, and that babies and young children can go a good amount of time before needing this repaired depending on its severity. We know that Walter will need his repaired, but it is comforting nonetheless.
The second, and more detrimental of the defects is Valvar and Infundibular Pulmonary Stenosis. This is a narrowing of the valve and pulmonary artery, which carries blood from the heart to the lungs to be oxygenized. With a narrowed pulmonary artery and valve, a human’s body cannot circulate enough oxygen rich blood through the rest of the body. The result is what people refer to as “blue babies”.
2) How severe is Walter’s ToF?
This is where the good news begins. Our fetal cardiologist indicated that Tets babies are grouped as “blue” or “pink” when born. “Blue” indicates that the baby is not getting enough oxygen, where “pink” indicates that broadly speaking the baby’s oxygen intake and circulation is somewhat “normal” for the time being, despite the ToF being present.
Our cardiologist has indicated that at this time she believes very strongly that Walter will be a PINK baby!!! Walter and I will be monitored by my OB, perinatologist, and cardiologist every month until delivery for this very reason. (This means between the three doctors and other labs, appointments just about every week.) As we progress, the growth of Walter’s pulmonary artery will be measured to see what his outlook is likely to be at birth. The current prognosis does mean that Walter’s pulmonary artery is very small and will need to be corrected, but it has grown and the doctor anticipates that it will continue to grow. The likeliness of the artery catching up in growth entirely is slim to none, but we can pray for it to keep widening.
So THINK PINK: the larger Walter’s pulmonary artery and valve are at birth, the less severe his surgery, the quicker his recovery, and the lower his likelihood for subsequent surgeries.
3) How many surgeries will Walter need, and when?
This coincides with Walter’s “pink” prognosis. We know these can change (for better or for worse) as time progresses, but for now we will go off of what the cardiologist anticipates.
Walter’s prognosis is the least severe of three possible surgical corrections. (These are vastly simpler than the reality of the correction, but I feel does an adequate job of explaining the idea of the surgical options.) All are open-heart surgeries:
I) One surgery, one to two months after birth to repair the hole in the bottom two chambers with a “patch”. A catheterized “balloon” will placed within the walls of the pulmonary artery forcing it to grow. There is no valve replacement needed.
II) One initial temporary surgery at birth, inserting a shunt into the pulmonary valve by way of the thigh in order to push more blood into the artery as soon as possible. This would be followed by the open-heart surgery described as (III) in a few weeks to a month post birth.
III) One initial surgery immediately following birth. Upon examination at birth, the baby’s scans would indicate that the fix cannot be postponed. At that point, a drug called prostaglanda would be used to keep the baby’s ductus arteriosus open. This is an artery that is open when all babies are born and closes in the first few days after birth. The artery is kept open in order to use it in the expansion of the pulmonary artery. In this scenario the artery is severed and widened, then patched with a piece from the ductus arteriosus. The existing valve will have to be replaced in order to fit the now larger artery. As the replacement valve will not grow with the baby’s heart, down the road, usually in mid-teens to twenties, the individual will require a replacement.
4) Where will I deliver and will it be scheduled?
We were surprised and relieved to hear that I will still be able to deliver at Mary Washington Hospital in Fredricksberg, just about 20 minutes south of us in Virginia. My doctors do plan to schedule me for induction one to two weeks before my due date mainly for coordination purposes. She indicated a c-section wouldn’t be necessary at all – so long as Walter is attached to the umbilical cord, he is just fine, so labor would not be a stress on him.
My doctor did assure me that though growing Wally big and full term is top priority, should I go into labor early, the hospital would already be well aware of everything he needs. There the NICU is run by a team of doctors from DC Children’s Hospital. They even have a video phone which can stream in real time to the head doctors at Children’s.
Immediately after Walter is born he will be examined, scanned, measured, and receive an echocardiogram. Those results will determine whether he needs to be transported immediately to Children’s for surgery, or whether he can wait to have the surgery delayed.
5) How long will Walter be in the hospital?
This, like everything else hinges on continued progress and most importantly, Walter’s post-birth assessment. But at this time, as immediate surgery is not anticipated, the cardiologist advised we would be taking our little boy home from the hospital in just a few days (perhaps 3 to 4). We can hold him, kiss him, have skin-to-skin, and nurse, just as any other little baby.
When we bring him home we will be closely monitoring his breathing, color, mannerisms, and weariness. Follow-ups will occur about every week with our pediatrician, along with plenty of correspondence and some monitoring from the cardiologist as we plan for surgery. Babies with ToF have been known to sometimes have “Tets spells” which are fainting episodes that can be serious, and indicate that surgery is needed sooner.
Surgery and recovery times vary based on severity of procedure and the age of the baby. The open-heart surgery itself can be between five to seven hours, with recovery time in hospital ranging from one to two weeks.
6) Does ToF effect brain development?
There is no known connection between Tets and decreased brain function or development. Our cardiologist explained that in-utero there is no decrease in oxygenated blood sent to the brain or other extremities, and at birth either oxygen levels are normal or supplement with additional oxygen. These little guys grow up to be just as smart as everyone else!
We have also elected to take part in a study at Children’s which will track Walter’s brain development via MRI. We will have two MRI scans in-utero and two post birth (one before and after surgery). We feel it is a great opportunity to establish a baseline for Walter, watch as his brain develops, and take part in a study that can help bring more information to ToF families.
7) Will Walter be able to play sports and be active?
Yes! The cardiologist explained that once corrected, the growing baby’s heart functions just like any other little heart. I just about fell out of my chair when the cardiologist smiled and said “Walter’s heart is stronger than anyone’s in this room.” It just didn’t seem right. But the cardiologist went on to explain that functionally speaking, Walter’s heart muscle is incredibly strong.
We tend to assess heart problems the way the understand adult heart problems: limited activities, wheezing, diseased, and malfunctioning. However, Walter’s heart is not diseased or weak – it has an anatomical defect that needs to be corrected so that it can continue to work effectively.
This was some of the most encouraging news, and gave us greater understanding as to why Tets babies have such successful outlooks. The doctor compared Walter’s heart to an athlete’s fractured leg: the leg function and muscle are not diseased or atrophied, but instead the bone within the leg needs repair to allow the entire system to work properly.
8) Does ToF raise my chances of pre-term labor?
Thankfully, no. Walter is perfectly happy and comfy with me doing all the work for him, and we’d like him to grow as much as possible before meeting the outside world.
9) Do I have restrictions with my pregnancy, or any changes to make?
The short answer is not really. As my pregnancy is already healthy, I’ve been advised to continue what I’ve been doing. My only changes are to limit my activity some to lower the risks of pre-term labor: walks are okay, but jogs or exercise significantly raising my heart rate should be nixed. As my due date draws nearer, there is a slight possibility of being placed on bed rest if I get any Braxton-Hicks contractions or indications of pre-term labor.
I also won’t be able to take some of the pre-baby travel trips I was hoping: so no stops in Michigan or flights to California. It’s disappointing to miss out on seeing friends and family, but we agree that it isn’t wise to travel so far from our dear medical team.
10) Am I going back to work?
Staying home with our children has always been a priority to Dan and to me. Thankfully, we have been able to save and plan for our future, which will allow me to be home with Wally. I had initially wanted to put off my decision until further down the road, just to make sure that everything was set and my options were open.
With Walter’s diagnosis, I know that returning just around or shortly after my new baby boy undergoes open-heart surgery is absolutely out of the question. Plus, with my vastly increased and lengthy appointment schedule, it will be difficult to keep up the demands and strenuous hours asked of me as a part of the “Management Development Program” at my work. For this reason, I’ll be foregoing an upcoming promotion and resigning from the management program. It looks as if I will be able to stay in my current position (without the extra tasks) for as long as I wish to continue work leading up to delivery.
11) How long will you be in Quantico, and where will you be next?
Life is unpredictable with the military, and it seems as soon as plans are made, they are quickly once again changed. However, current forecasts indicate that we will likely be in Quantico until March/April of 2015. Dan finishes training at The Basic School (TBS) the last week of September. Quickly, Walter will be in our arms, and Dan will be able to take some leave to be with us. With ramp-up and other duties on hand, he will be likely starting his specialty school in January, at the fruition of which, we will be moved to a Permanent Change of Station (PCS). Where that is exactly, we don’t know, but we can rule out some possibilities.
The social worker at Children’s informed me that Walter will qualify as part of the military’s “Exceptional Family Member Program” (EFMPs). Before Dan can receive PCS orders moving us as a family, Walter’s medical team will have to fill out paperwork indicating exactly what medical doctors Walter will need access to and how often he will need them. The Marines will be unable to move us anywhere Walter cannot be provided with necessary medical care. This means that locations such as Japan, Europe, and Alaska are out of the question – so we will likely be stateside.
12) Is ToF likely to repeat itself in future children of ours?
The chance of any couple having a child with a congenital heart defect is about 1%, with about 10% of those CHD babies/children needing some kind of repair. After a couple has one child with a CHD, their risk of one occurring in a subsequent child doubles to 2%. Right now, we are taking things as they come, and focused on getting Walter fixed, recovered, and healthy – but we do still hope and plan to continue to grow our family down the road.
13) When will Walter be baptized?
We are planning on having Walter baptized in the hospital soon after birth, before he may have any Treps spells or need his surgery. It comforts us to know that before facing his trials in the coming weeks, he will already be a child of Christ and have received his first sacrament.
If timings of surgery and recovery accommodate, we hope to have a joint-ceremony with family and godparents in late December in order to celebrate our blessings within the Church. We have not yet finalized any information in that regard, but we have plenty of time, and will make sure that those interested are aware.
14) What are the names of our doctors and which Tricare plan are we on?
This answer will be a post in itself. We adore our doctors, and want to recommend them all – from our OBGYN to the specialists. Also, I’ve learned a good amount about Tricare, and hope our experiences may help other families as well!
These answers gave us so much hope – as time goes on we will update on Walter’s progress as well as any changes to his prognosis. Right now we pray for PINK.
We have been so humbled by the love, prayers, and intentions being made for our sweet boy and have been deeply moved by the wisdom and comfort those words can bring. We cannot begin to express our gratitude nor thank you for the powerful force you have raised beside us, but please know that it does not go unappreciated or unnoticed.