Conclusive MRI Results

First, we were completely surprised today to get a wonderful gift from Ashley (Maxton's Mom). Ashley, you are so creative and to think about us with this gift was incredible. Thank you so much for this special gift and for also thinking about Hollie! Here is a picture:

It's been a long day, but overall we feel a good day. We didn't learn anything surprising and we are just fine with that. We hit the road about 9:00 and had barely made it out of the neighborhood when we got the call about the MRI machine not working. We don't have a lot of luck with these machines since the one in Houston went down when we were scheduled also. We got called back about 11:30 and the actual test took about an hour. Stef was so uncomfortable throughout the entire experience, but she stayed strong and made it through the procedure.

Dr. Twickler met with us a shortly after to discuss what she found. I have to say she is the nicest lady and just incredibly thorough. She really is an authority on fetal imaging and I feel lucky that we are close enough to have been able to get her opinion. She was just terrific to work with. She was able to get fantastic, noise free images that I believe were much easier to read than what we had in Houston. The first item up for discussion was the liver position. Our gut was right, it is "up" (in the chest - not where it is supposed to be). The good thing is there is only 2.8% of the liver up and she really had to search for it. It's barely remarkable and much lower than 20% which is one of the predictors for ECMO and mortality (less than 20% is good). Finally have that mystery solved!

Another measurement of conflicting importance is Total Fetal Lung Volume and then the Observed to Expected Total Fetal Lung Volume %. Our situation with twins presents a somewhat unique situation because we are able to see what Kamryn's lungs measure and then compare them directly with where they should be by measuring Brooke's. The total volume of both Kamryn's lungs is 20cc's while Brooke's total lung volume is 81cc's. Brooke is a week ahead of Kamryn, so if you back that up a week, Kamryn should have 80cc's if all where normal. Basically, Kamryn has 25% of the lung volume that Brooke has. That's not so good since some of the studies have suggested lower survival rates with that low of a %.

At the same time, Dr. Twickler measured Kamryn's LHR (lung to head ratio) and the result was 3.3 which is extremely close to what Dr. Zaretsky has found between 3.0 - 3.4. Anything higher than 1.4 is considered good. We are in fantastic shape with LHR.

*** I go into detail about how these are calculated in my document in the upper left hand corner of the blog so I will spare everyone the details.

Total Fetal Lung Volume measures the volume or space of the lungs while LHR measures the area in just a single plane. They are measuring the lungs in two totally different ways and we are basically getting conflicting results. Dr. Twickler didn't put nearly as much weight on the measurements as she did on the liver position and we are in good shape there.

The bottom line is none of this tells us how much hypertension will be present in her lungs and ultimately how this will all play out. Stef asked afterwards what my gut was telling me and I do believe Kamryn situation is going to be a challenge to get through with many ups and downs, but I believe we have a "good" scenario and she is going to survive. Kamryn has one heck of a fight ahead of her, but she can win this. Here is one of the images of Kamryn.

Overall, today was a good day and I know Stef and I are both ready. As ready as we will ever be I suppose. Thanks for all the continued support - we honestly can't thank you all enough!

Shawn

p.s. - Scott, I do NOT watch American Idol like your comment suggests :)

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Maternal Fetal Medicine Appointment

We had our appointment today with Dr. Zaretsky and it went really well. I think we both would be losing our mind at this point without Dr. Z, Leah, and Sharon. Leah is so good to us when she is doing the sonogram and just has amazing patience with all the questions we ask her. After visiting Houston, we realize we are once again blessed with our local healthcare professionals.

The appointment was pretty uneventful (we like that!). The twins weigh 1.15 (Brooke) and 1.11 (Kamryn) which is actually on par with a singleton pregnancy. This correlation will likely diminish a bit once we get to 28 weeks and then we should see them fall off that curve a bit. Based on the measurements, there is a 14% gap between their growth which is totally normal and we are right on track.

Amniotic fluid is measuring around 6 to 7. I forget what the measurement is of 6 to 7, but we know it is within normal limits as long as it is below 8. Heartrates were 134 (Kamryn) and 138 (Brooke). We were a little concerned about the 134 with Kamryn, but soon found out that is within normal range so all is well there.

We talked with Dr. Z about liver position and if a % of liver being up would be a predictor of needing ECMO (Heart/Lung bypass machine) and mortality. My feeling is that it had too be and while Kamryn's liver is up, 15% didn't seem that much. We talked about a number of studies that have recently come out and one specifically that indicated a much better prognosis if less than 20% of the liver was in the chest cavity. Another study talked about a very promising predictor of outcome being Predicted Percentage of Lung Volume using MRI measurements. I think they are a fantastic resource for CDH families and will give them a framework to ask questions. One of the most frustrating aspects of this condition is the difficulty that the healthcare community has in predicting mortality and morbidity in these babies. For every article I find that talks about Lung to Head Ratio being a good predictor there is another article or study that shows it isn't in their pool of babies. As all of you can relate, when you or a family member finds out about a condition you just want to know what the outcome is or what to expect. In the beginning of this journey I would get so frustrated because I felt Doctor's weren't giving us the truth about the prognosis. After many months and a lot of research, we finally understand that they simply don't know and I can tell just how frustrated they are that there are no solid predictors. They honestly won't know until Kamryn is born. These newer studies I talked about show there may be some promising new indicators out there. I have posted the three studies at the top of the page under resources.

I think I am rambling again........sorry. Stephanie turned to me the other day and with all the confidence in the world said, "Kamryn is going to make it." I believe she will also and I can't wait to tell her somewhere down the road when she is older that she sure scared the heck out of mommy and daddy. We are looking forward to that day.

Friday April 3rd is our next big day when we get to meet with the Doctors at Children's here in Dallas. We are really looking forward to it and getting our plan together for delivery.

Thanks to all of you who give us support each day and keep us grounded. We would be lost without you all!

Shawn and Stephanie

P.S. Kamryn says "hi" to everyone in the picture below.

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Houston Appointments

That was a long day!! Overall, we aren't sure that we are all that impressed with what we found and literally feel a little deflated. Honestly, what we found out today is pretty good news with all things considered. It's just not great news and it's hard to keep yourself from holding out hope for that amazing news. Here's how the day went:

7:50am - Started the ultrasound and the technician was just not what we hoped for. We have been spoiled in Dallas with very personable and compassionate people doing our ultrasounds. It was 2 hours of almost no communication as the procedure was conducted. It was 10:00 and we were still doing the ultrasound and hadn't met with the doctor. We became a little nervous because our MRI was scheduled to start at 10:00. We finished up around 10:15 and met with the Doctor for about 5 minutes where he told us everything looked fine except the CDH and he couldn't tell if the liver was up or down. We rushed to get to the MRI.

10:30am - We showed up for the MRI and checked in. Around 11:15 they came out to tell us the MRI machine we were scheduled for was down and someone was working on it. They were trying to re-schedule us. We explained we needed to investigate plan B since we traveled in and around 11:30 we started the MRI which "would take about 30-40 minutes". Half way through the MRI they came in to tell Stef that they needed to start over. The band they were using around her stomach was too small and wouldn't cover both babies. They changed the band and after being in the MRI machine for an hour and a half, it was finally over. Unfortunately, it was now 1:00 and that's when we were supposed to meet with Neonatology. It was just one mess after another. No time for lunch so we shot over to pickup the copies of the MRI and then to the next doctor appt.

1:30pm - We met with the Neonatology doctor who didn't have our MRI results and had briefly reviewed our chart. We didn't talk about our specific situation, just general CDH procedures. Unfortunately, the meeting was choppy as the doctor stepped out to take calls on 3 different occasions during our meeting and then a fourth interruption to move us to another conference room. We just didn't get much out of that appointment so we wrapped it up knowing we were supposed to be over at the NICU at 2:00 for that tour and it was already 2:15.

2:30pm - Toured the L&D area and NICU. This really was the highlight of the trip. It was difficult seeing all of those babies in the Level III NICU, but the facility was incredible. They have 1:1 baby to nurse ratios and you could tell it was run very well. The Labor and Delivery area was literally right next door so transport would be no problem at all. 3:00pm - We grabbed some lunch and had a chance to talk about the day before our last appointment at 4:00.

4:00pm - We showed up to see the pediatric surgeon and were seen around 4:30. He had reviewed our results from the day and discussed CDH in general with us before getting to our specific situation. It was obvious that he had quite a bit of experience handling these types of cases which was reassuring. Here's what we found out:

Brooke is still doing fantastic!!
Kamryn:
LHR (lung head ratio) is 1.4 (less than 1.0 = severe, 1.0-1.4 = moderate, greater than 1.4 = best case scenario)
Liver up (estimated 15% of liver up) - this was a bit of a blow
80% chance of survival
50% chance she will need ECMO
Estimate 6-8 weeks in NICU (this was purely a guess and I pushed him to give us his gut feeling)
Unable to estimate % of diaphragm missing or undeveloped, but believes we will need a patch for repair
Imaging Doctor didn't have time to calculate volumes so they will send to us soon

I had somewhat convinced myself that with her LHR at or above 1.4 and most of the liver down, survival would be approaching 95-100%. I definitely convinced myself that ECMO was a remote possibility. In his opinion, it is more severe than what I had talked myself into and it was a little deflating.

It's frustrating as I once again have to remind myself that at the end of the day, it's really anyone's guess as to what will actually happen. Babies with indicators much better than this still struggle significantly while babies with far worse indicators come through remarkably well. We simply won't know the severity of the situation until they are born no matter how many tests are conducted or what numbers they give us.

What we do need is to feel like we are making the best decision for Kamryn and Brooke with regards to where we deliver and we could only do that by comparing facilities and Doctors. With our Dallas appointment coming up on April 3rd, we will be able to make that comparison and feel good about our decision.

I continue to be amazed with how strong Stef is. She was such a trooper today through all the tests while I knew just how uncomfortable she really was. She's been through so much and somehow gets up everyday with her gameface on and is ready to tackle anything thrown her way. I'm proud of you Stef and thankful to have you by my side through this journey. You really are amazing and I love you so very much!

We are both exhausted and ready for some sleep before the drive back home tomorrow. Talk to you all soon!

Shawn
“I delight to do your will, O my God; your law is within my heart.” – Psalm 40:8

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