Phone Call with Dr. Lee

Earlier today I had Dr. Lee call me to try and clear up a few details about where we stand after the big let down from earlier this week.  There wasn’t much to discuss so it was a relatively quick phone call but we did get some more information on where we go from here.  I asked him how he expected things to go whenever it is that Lucas joins us.  He told me that a breathing tube will be put in almost immediately and unfortunately given the severity of Lucas’ CDH he will most likely have to go on to the ECMO machine rather quickly.  Here’s a short video that briefly discusses the ECMO process starting around the 2:45 mark.  I couldn’t find a video with a better explanation so that’s all we’ve got to go on for now.

One thing that really surprised me was that Dr. Lee said that they would most likely be able to perform the hernia repair surgery while Lucas is on ECMO.  Everything I’d read so far said that wasn’t done but there isn’t a whole lot of information on CDH so it’s kind of hard get a better game plan without speaking directly with the doctors.  I think that’s pretty good though because the quicker the organs can be moved to the right spot then the quicker the lungs have space and room to grow.  And of course there’s potential issues with going on ECMO the two main ones being bleeding in the brain, which he told me was in the 8% range, and also ECMO is a one time deal.  If he gets to the point that he can be taken off there’s pretty much no going back on.

Dr. Lee told me that the biggest thing now is to have Lynsy relax and not have Lucas come early.  And around the 34 week mark we’ll have another MRI scheduled to get a better idea of his status closer to delivery.  Hopefully things have improved a bit by then.  So now we just kind of wait around and have to do our best to not have Lucas come early!

A Big Let Down

Yesterday we had a full day of appointments at the Texas Children’s Hospital.  We started off with meeting with a nurse practitioner, Dr. Aimee Jackson.  She was super pleasant and told us what to expect for the next few months.  She said that there were a few different approaches she could use but she decided was going to talk to us as if we were going to do the FETO since that was the most likely route we were going to take.  She gave us a “pregnancy book” to answer questions we might have about being pregnant and then a folder with information about the hospital, numbers I would need, schedules of pregnancy classes I could attend, etc.  She then performed a brief physical to get basic information and beginning stats and told me that I probably felt like a science experiment, but that I was in good hands and if I ever had any questions there were many people I could call and to tell them I was “part of the fetal intervention team”…I felt like a patient VIP.

Next was our meeting with the main FETO team to discuss the next steps.  Dr. Belfort came in first with a medical student.  He went over the procedure and how I was a great candidate for it.  Then we were joined by Dr. Lee, Ms. Laura Mollett (my liaison…super awesome woman) where we all discussed the possible risks of the surgery, but the many benefits that they had seen so far and were hoping to repeat.  As we might have mentioned before, the experimental FETO procedure is only offered to those patients whose babies are in the “severe” category of CDH and are not expected to have a good chance of survival without the surgery due to the minimal lung development. They were a little concerned because Luke has about 60% of his liver in his chest, more than any of the cases they’d done so far, but they were still hopeful.  They have done ten of these procedures, but out of the ten, five matched the same profile as us.  Of those five, four were still living and according to Dr. Lee, had a “good” quality of life, even a couple years after having the surgery.  This was very promising and we felt very positive that we were making the right decision and we would be in the care of the best doctors in the country.  We spent the next 15 minutes or so discussing how the procedure would work exactly, how I would need to live in the Ronald McDonald house, come to weekly check-ups, etc. We were just waiting for the other doctors to join us so they could add anything else that was missed and we could discuss the final preparations.  Dr. Belfort left to meet Dr. Ruano who was getting the final summary on the million tests that had been performed in the last couple weeks and Dr. Lee talked with us.  

Then Dr. Belfort, Dr. Ruano, and Dr. Shammy (forgot his real name, that’s what they called him) returned.   They sat down and Dr. Belfort said they had looked at the echo-cardiogram images from the week before with the main fetal cardiologist, Dr. Nancy Ayers, and the two “small holes” we had discussed as not being super important at the moment, were actually a bigger deal than any of us thought.  He said that this heart defect would actually be reason for our “exclusion” from the procedure, that there was no way they would be able to do the FETO procedure with these holes being present in Luke’s heart.  The room was silent.  We didn’t know what to say…I don’t think anyone did.  All of us had spent the last week or so planning to do the procedure and had just spent the last 20 minutes talking about how we were all glad to be doing it and how it would probably raise the chance of Luke’s survival immensely…and then in a few seconds it was all taken away.  Dr. Belfort said that it was now up to the postnatal team to do their jobs to the best of their ability, which included Dr. Lee.  Dr. Lee talked about how we would still go to monthly appointments, like a regular pregnancy, but we’d have another MRI around week 34.  The biggest thing to focus on now was to try and avoid a preterm birth, and then once Luke was born they would do their best to take care of him.

We didn’t know what to say or do other than to nod.  We just went from being very hopeful and optimistic to feeling punched in the stomach with the news that there really wasn’t a lot of hope for our baby.  You could see it in everyone’s faces, too, which made it even worse.  I flat out asked if we had much of a chance with or without the surgery, and Dr. Belfort said unfortunately not because of how much of the liver had gone up into the chest and now the risks with the heart.  He told me that there were counselors that we could talk to and that even though I was smiling (which I automatically do in tough situations in an attempt to keep my composure) he strongly suggested that we speak with them.  I wanted to punch him in the face, to be honest.  Everyone stood up, apologized, wished us luck, and then left the room except for Laura.  She stayed and asked if we had questions and then said that if we wanted to skip the other two appointments for the day we could…especially since they weren’t necessary anymore.  Then she left to make a couple phone calls to get the follow-up cardiology appointment and routine pregnancy appointment scheduled, since we hadn’t done that in anticipation of being a special case.

We thanked Laura, and left.  As we walked out of the room, all the doctors and medical student were lined up in the hallway so we had to walk through them which was so frustrating.  We drove all the way home where we spent the rest of the day doing nothing.  This is devastating and we don’t know what to do from here.  We went from being patient “VIPs” with check-ups and special numbers and hopeful surgeries to now just routine appointments and praying for a miracle.  We feel rejected and angry and frustrated, but most of all just sad and disappointed.  We know we have a great support group around us of family and friends which we sincerely appreciate, but there’s nothing that could make us feel better right now other than a phone call saying “just kidding…we’ll do the possible life-saving surgery after all”.  Hopefully in a few days we’ll bounce back because we are fighters…which means Luke is a fighter.

Echo-cardiogram and More Appointments

Today was the day for the last step in our testing (we think).  We drove to the Texas Children’s Hospital West Campus in Katy. There we met James, our technician for the day.  He explained that he would take pictures of the heart but they would be monitored by Dr. Keila Lopez, the pediatric cardiologist who would come in after the ultrasound to conference with us and tell us what she saw.  By now, ultrasounds have become commonplace so it felt like a normal routine.  We were there for about an hour and half while James took his “pictures”.  Towards the end Dr. Lopez came in and they tried to get a few more images that they needed for a full assessment.  Then we met with her to go over the echo.

She started off by saying how ironic the situation was for her to be our doctor.  We were confused, but she went on to say that just that morning she had been with a couple hundred doctors for a conference.  Their subject for the day: Lucas (of course without the name).   Dr. Lopez told us that when she opened our file she wondered why it looked familiar, but then she realized that we were the exact case they had spent all morning studying, so in her own words “she knew our fetus well”. It was very surreal for us to hear this, that this is how far our case has gone already where hundreds of doctors are studying our unborn child. It’s crazy.

Well, Dr. Lopez did an excellent job explaining what she saw.  She drew a picture of a normal heart and then used colored pens to explain to us the functions, blood flow and oxygen flow of a normal heart compared to that of baby Luke’s heart. For the most part she said that the heart looked great, which is apparently rare in CDH cases.  Yay!  She said that James was able to get images and pictures that are extremely hard to get and that she didn’t expect to have, which made it better for her to study,  Go, James!  She said the heart was in tact and had all the parts that it was supposed to have, which was also great.  Woohoo!  This was great news so far.

BUT, she also said that she saw two small holes that were in important places.  With our unique case, she couldn’t predict what they would do, but she hoped that over time they would heal themselves and close up on their own.  In that case, nothing else would need to be done other than normal routine monitoring.  However, because of where these small holes are if they don’t close up, they could pose a real threat by creating uneven levels of pressure of blood traveling through the heart, which in turn would cause the heart to work harder and therefore the lungs to work harder.  The only way to fix this from happening would be for Lucas to have open heart surgery between 2 and 4 months of age.

So, overall the echo cardiogram went well and he has a strong, complete heart, but we just have to hope that those two small holes close up and fix themselves.  We asked if these small holes would prevent us from qualifying for the FETO, and she said they would not.  We would monitor them and deal with them later.

Before we left, Dr. Lopez called Laura, our liaison between all the many doctors and surgeons, to ask about setting up a follow up appointment.  That’s up in the air right now depending on everything else, but then Laura asked to speak to me. She told me that on Tuesday she had set up 4 more meetings for us.  The first appointment is with a nurse practitioner for paperwork (I think), The next appointment is to meet with the interventionist team which consists of Dr. Micheal Belfort-head of pediatric surgery at TCH, Dr. Rodrigo Ruano-CDH specialist from Brazil who’s studied the European approach using the FETO procedure, and Dr. Shammy-not sure who this is yet but we’ll find out!  We assume we’re going to discuss the FETO procedure in detail with them.  The next meeting is with a social worker, we assume to discuss living arrangements for me during the whole process.  The last appointment is with the neonatal team, to tour the NICU and go over more detail.

On our way home Laura called one more time to let us know that they had already submitted some paperwork to the FDA and that they were starting to move forward, but everything would be discussed and explained soon.  She also told us about the Ronald McDonald house, where I will most likely be staying throughout this whole process.  Some things to think about before Tuesday.

 

A Different Perspective with Dr. Hare

Today we had an another appointment with Dr. Hare.  I met Jonathan at CVS in Humble and we drove down to the Women’s Hospital.  We discussed how we didn’t know what she was going to tell us that was different from the day before because we wouldn’t get any more information until the echo-cardiogram next Friday.  Perhaps she would give us some advice from a non-surgical specialist point of view and answer some questions that we had or summarize everything so far since it was a little overwhelming.  We were just going to go and see.

On the way there, I got a phone call from the Texas Children’s scheduling department, saying that they had an opening tomorrow for the echo instead of waiting until next Friday.  She said she knew I was a teacher so she would understand if I wouldn’t be able to change it, but I assured her that I would find a way, that we were anxious to get as much information as we could as soon as we could. 

Well, as it turns out, my appointment with Dr. Hare wasn’t actually until tomorrow, but I had written it down wrong in my planner and luckily she agreed to see us anyways.  I always double and triple check appointments, but for some reason I hadn’t this time and just came when I thought it was.  If I hadn’t made arrangements with Jonathan and my school team to come on the wrong day, then the appointment with Dr. Hare would have conflicted with the new appointment we had just made with the cardiologist for tomorrow, but now we could do both.  Everything happens for a reason.

Let me start off by saying teachers have a way of suggesting things to parents without really suggesting them, i.e. “Well, I’m not a doctor so I can’t tell you if your child has ADHD, but what I do see in the classroom is….so you might want to get him/her checked out.”  That’s what Dr. Hare did. She spent about 20 seconds doing an ultrasound, I guess out of habit, and then told us that she couldn’t tell us to do the FETO surgery, but that it would raise the chances of baby Luke’s survival if successful.  We explained that we were leaning against it because of the promising chances Dr. Lee had told us without the experimental procedure.  Her response to that was that Dr. Lee should have clarified:  we did not have a 60-70% chance of survival, all cases of CDH as a whole at Texas Children’s had those chances, but we were in the small bubble of “severe” cases where the chances were much lower.  She said she wouldn’t give us an exact number because the human body is complex and our case was special, but that she thought that it might be our best chance if the echo came back fine and we qualified.  She didn’t say that exactly, but that’s what she meant in so many words.

Our whole world changed yet again in those few minutes.  We are under the impression that without the surgery, we have a slim to none chance of Lucas surviving and are now learning more towards doing the FETO.  The odds might not be improved by great numbers, but any extra chance we can give to him are worth it and that’s what we are going to do.  Now to wait for the echo results.

A Full Day of Appointments

Yesterday we had a really busy day that started bright and early.  Today was the day we’d hopefully find out a lot more about where Lucas stands with his CDH and get a game plan lined out.  First up was the ultrasound where they did an hour long check on just about everything you can imagine to look at on a baby.  Including even counting fingers and toes to make sure everything else was right.  There wasn’t a whole lot to report from here since the sonographer was just getting pictures and we knew there was a meeting with the pediatric surgeon later in the day.

After the ultrasound we got ushered over to the radiology? department where they performed a fetal MRI.  Lynsy was obviously not looking forward to that since it’s a tiny space that she had to be in but it needed to be done.  *Sidenote, I’ve never seen a normal size MRI machine but this one was supposed to be one of the larger ones since they deal with pregnant women.  Well the large one is still pretty tiny.

After getting her set up on the table for the MRI the tech was kind enough to give her some ear plugs, eye mask and headphones and even played Lynsy’s music of choice.  I was able to be in the room with her in case she needed me but luckily Lucas cooperated and didn’t move too much during the scans and the tech was able to finish in about 45 min.

The MRI was over around 1045/11 and the next appointment wasn’t until 3 pm so we decided we’d get some lunch.  We figured we’d test out the cafeteria at the hospital since that will probably end up being the easiest option in the future and I have to say the food was pretty good.  I don’t remember the lady’s name but she saw us wandering around and gave us the rundown on the cafeteria.  It was so nice of her to take the time to deal with us.

Since we still had time to kill until the meeting with the pediatric surgeon Lynsy and I went on a walk up to the Houston Museum of Natural Science and to Hermann Park.  There wasn’t enough time to really do much but it beat just sitting around the hospital and waiting.  Plus we got to see an iguana that lives in the butterfly center when a volunteer brought him outside to sunbathe.  It was pretty cool to see an iguana that large up close and personal.

We headed back to the hospital to go meet with the pediatric surgeon, Dr. Timothy Lee.  Unfortunately it was bad news that he was delivering.  Lucas has about 40-50% of his liver up in his chest as well as his stomach and some of his intestines.  The lung to head ratio (LHR)  of 0.9 places Lucas in the “severe” group of CDH cases.  It’s not the most severe case that Texas Children’s has seen, but it’s still one of the worse ones and is very serious.

Dr. Lee told us about the fetal endotracheal occlusion (FETO) as an option to try and get the lungs to “grow”.  If Lucas meets the criteria for the procedure, then he would be only the 11th operation of this kind performed at TCH which is only one of two hospitals in all of North America that is approved by the FDA to do this procedure.  So it’s very much experimental, at least in the US (apparently it’s a common procedure in Europe, but not here due to all the restrictions we have).  What they do for the FETO is while in utero they take a tiny camera on attached to a cable with a balloon attached to the end.  They’ll have a small incision to get the camera inside and then direct it towards Lucas’ mouth and insert the balloon into his trachea and blow the balloon up.  The balloon will hopefully force the lungs to grow through increased pressure inside the lungs since they are fighting for space with the organs that shouldn’t be up there.  After about 6 weeks they would perform another procedure to pop and remove the balloon.

Here’s a video explaining the FETO procedure.

We asked Dr. Lee where Lucas stood at the time and he told us it’s about 60-70% chance without doing the FETO but with it being experimental we couldn’t get a lot of information about what kind of improvement the FETO would give.  We’re leaning towards not doing the FETO unless we can get a better idea of what Lucas’ chances will be.  If we’re looking at improvement in the 5% range by doing the FETO it seems a bit too risky given the problems that the FETO could cause.

Unfortunately Lucas’ situation has gotten worse and we’re still in waiting mode.  We have an appointment with Dr. Hare (perinatoligist) this afternoon and can hopefully get more information from her but nothing can be decided until we get the results of the echocardiogram for Lucas which is scheduled for next Friday.  So it’s more hurry up and wait.

Amnio results are in and TCH appointments scheduled

We got some good news today when the geneticist called back with the results from the amnio.  Everything came back normal with no abnormalities.  It’s what the geneticist and we expected since there wasn’t any family history and the first test already showed that Down/T13/T18 weren’t an issue but it was a big relief to get confirmation of that.

Earlier this week we also got a call from Texas Children’s Hospital to schedule some more imaging and meetings.  The appointment will be next Tuesday, August 26th and will be a full day.  We’ve got a more in depth ultrasound and a fetal MRI and a meeting with a pediatric surgeon.  There’s also an echocardiogram scheduled for September 5th to take an in depth look at Luke’s heart.

Now we wait, yet again.

A Sense of Normalcy

Ever since finding out that we were expecting a baby our plan was to have a gender reveal party where it’s a surprise to us and to our friends and family.  Well yesterday was the big day and after the emotions of the past week or so it was great to have a sense of normalcy and take our minds off of the situation.

I was so glad to be able to be home for the party and share in this awesome news with everyone that could join us.  In true fashion Lynsy and I were running around with our heads cut off because she had to be a couch potato for 24 hours after the amnio from Thursday.  Everyone started showing up and we were both excited to see everyone.  For those that we didn’t really get to speak with I apologize but your presence was very much appreciated.

In case you missed it, IT’S A BOY!!! And thankfully that was the case because we hadn’t settled on a name for a girl.  In late December we’ll be welcoming Lucas (Luke) Dean to our family.

 

Meeting with Dr. Hare

Getting the news of our baby’s CDH condition was quite a shock and the first two days were rough.  There was so much that we didn’t know about our baby’s situation and that made it so difficult.  Were we on the moderate side or the severe side?  So we waited for the call from the specialist and the next appointment.

The specialist’s name was Dr. Hare and we had no idea what to expect.  Although based on the internet she seemed to be a really straightforward, no-BS person but she knew what she was doing.  Imagine Christina Yang from Grey’s Anatomy and you have a good idea of our new doctor’s personality.  We didn’t really want someone to coddle us but the reviews did worry us a bit.  The important thing is that she will do what’s best for our baby. 

The meeting with Dr. Hare was good but also bad thanks to the confirmation of CDH.  We learned that our baby’s liver was partially up in the chest cavity due to the hernia.  The liver had pushed the lungs and heart over.  In our research about CDH the liver being up seemed to indicate more severe cases but the liver was also usually accompanied with the stomach and intestines which luckily was not a situation we were in.

We spoke with a geneticist to discuss doing an amniocentesis to rule out over 150 chromosomal/genetic issues such as Down syndrome, Trisomy 13 and Trisomy 18.  We decided to move forward with the amnio despite the risk of a miscarriage (approx. 1 in 300) because if our baby’s situation worsened and it required a FETO (fetal endotracheal occlusion) the doctors would require an amnio to rule out other issues before they performed the FETO.  Most cases don’t require this experimental surgery so it probably won’t apply to us, but getting the amnio would move us closer to an answer by ruling out the other issues so we decided, why wait another week for the results when we can get it done now and get more information.

So now we wait again.  That seems to be the theme of dealing with this.  We’ll get the amnio results back in about a week and should have some more testing done this time at Texas Children’s Hospital the week after next.

Life Can Change in an Instant

Starting at the beginning of this year we didn’t exactly start trying to have a child but we stopped not trying.  It finally felt like the time to start working towards adding to our family.  On Easter Sunday, as we got ready to relax after a long day visiting family, Lynsy surprised me by showing me her positive pregnancy test.  Even though this announcement was good news, the gravity of the change didn’t really sink in until one day in May. Around the middle of May we had an appointment scheduled and luckily things worked out with my work schedule that I was able to go with Lynsy to that appointment.  We got the confirmation that we were, indeed, expecting and it was a huge emotional roller coaster.

Just finding out news that I’m now fully responsible for the care and well-being of another person is a bit overwhelming and changes your perspective on things.  Coupling the emotions of that news with the issues my grandfather was going through at the time made it kind of bittersweet.  My grandfather had just recently fallen and broken his left hip and left shoulder.  That was a truly wild ride because we didn’t feel that the time was right to share our good news with my family because the focus really needed to be on the well-being of my grandfather who was in bad shape.

The doctor even came in and essentially told the family that my grandfather most likely wouldn’t make it through the weekend, let alone the surgery needed to repair the bones that were broken.  I mentioned before how my grandfather is my biggest inspiration and it’s truly amazing how strong that man is.  He’s had multiple heart surgeries starting about 40 years ago, as well as a back surgery or two and now he’s having to deal with a broken hip at the ripe old age of 93.  After a lot of back and forth between the doctors and my family, the decision was finally made to try and fix the bones as soon as he was stable enough to attempt the surgery, even though the odds of him surviving the surgery was probably in the 10% range due to his weak heart.  It amazes me to find out how medical technology has changed and how amazing the doctors and surgeons are.  The surgeon that performed the procedure recommended that we only fix the hip to limit the time that my grandfather was under anesthesia which made perfect sense given his age and risk.  He was able to insert a rod and screws through two inch long incisions and did all of this about 10 minutes when the surgery was projected to take about 20-30 minutes.  My grandfather is still recovering and I don’t ever expect him to walk again but getting to still have him around is amazing.  Did I mention that he’s strong?  Sometimes stubbornness is a good thing.

Anyways, back to my original story.  Finding out that Lynsy was indeed pregnant was amazing and scary all at the same time and sent all sorts of thoughts through my head.  Will I be a good father?  How do I raise our child?  Are we really ready financially and emotionally to go through a pregnancy and at least 18 years?

My biggest fear with the pregnancy is that besides being there for conception, pretty much everything else is out of my hands.  Sure I can help out a bit more around the house to help her out, but she has to carry our child all by herself.  I can’t lighten her load and take the baby off her hands…errr…stomach for a few hours.  It’s essentially up to her and the miracle that is child birth.

Fast forward to last week when my wife went to the doctor to get the anatomy ultrasound.  Unfortunately I wasn’t able to go to the appointment with her because I was actually heading back to work that same day.  The plan was to have the doctors find out the gender and they would put the information in an envelope and we’d find out at our gender reveal party.  I got a call from my wife with her crying rather hysterically and I knew something had to be wrong.  She finally calmed down enough to explain to me what had happened.  Our child has a congenital diaphragmatic hernia, or CDH.  Essentially what that is is that the diaphragm doesn’t completely form which allows some of the abdominal organs up into the chest cavity.  The lungs possibly not having space to fully develop is the biggest issue with CDH.  I immediately turned my car around and went back home to spend time with my wife and try to learn more about what it is that is affecting our child.

It’s pretty rare with about 1 in 2,500 babies being affected with about a 50% survival rate after birth.  The worst part about it is that there’s essentially no known cause for CDH.  Best case scenario is 2-3 days in the NICU followed by one surgery and then a few days in the NICU for recovery.  That was absolutely heartbreaking to learn that our child will be needing at least one surgery within the first few days of its life when neither my wife or I have had a major surgery in our 61 combined years.  But the great thing is that there’s some absolutely amazing hospitals in Houston.

We’re both excited to be expanding our family in late December, at least that’s the projected due date.  But we’re also both terrified of the unknown since most of the action and issues with CDH don’t occur until after the birth.  For now we’re taking it a day at a time; some better than others but never without hope.