Thursday, April 16, 2015

April 16 - Dear Nicholas Sparks (Guest Blogger Jessica Morgan)

Dear Mr. Sparks,

Joe and I recently brought home our baby girl Kaylaya Arissa. She was born with what is known as a Congenital Diaphragmatic Hernia. She had a right-sided hernia and her liver was up in her chest where her lungs should have been growing, her chances of survival early on were lower than 50%.

We found out at 18 weeks that something was wrong and that her heart was displaced. On Halloween last year we got the diagnosis of CDH and were told that her liver, at that point, was as far up as her shoulder. CDH is more common in boys and more common on the left side. With Arissa being a girl and being right-sided, she was determined to be different I guess. Typically right-sided hernias are said to be more severe because the liver slips up into the chest rather than the stomach and bowels, the liver is a dense organ and it's said to more difficult for the lungs to grow. It took us completely by surprise when we were given the option to terminate the pregnancy, it was just something that we had never even considered. Both of us said no without even looking at each other. We decided then that our job was to give her the best fighting chance possible.

We went on a search and found a surgeon, Dr. Kays, in Florida where it was said he had a 92% success rate rather than the national 50% given to these babies. We were told that he tends to be pretty optimistic when predicting the chances of survival and at our consult with him he gave our baby girl a 40%. She had an LHR (lung to head ratio) of .58 and typically in studies babies under a 1.0 LHR are considered severe and babies under a .6 LHR are considered a non-survivor. Luckily with all of that against her Arissa had an isolated case of CDH and she was in absolute perfect health besides the fact that her diaphragm didn't form, though we weren't sure of that until after she was born.

So I relocated to Florida a little over a month before she was born, since there is a risk of preterm labor with DH babies. She was born on February 21st and within hours was put on VA ECMO (a machine that works as the heart and lungs, it pumped all of her blood out of her body and through a machine that oxygenates her blood and circulates it back into her body, understandably it's a very critical time where almost anything can happen). Dr. Kays decided that her condition was so severe with so much of her liver in her chest, and so little room for her lungs to expand that he wanted to do the surgery while on ECMO when she was only 5 days old, this was a concern because she was on blood thinners to try to prevent blood clots forming in the ECMO machine where they could break loose and send a blood clot through her system. Dr. Kays put a Gore-Tex patch in to serve as her diaphragm now, only about 2-3% of her own diaphragm had a chance to grow, so it is a large patch to make up for the other 98%. It is sewn to some muscles and her ribcage on the right side where there was nothing else to sew the patch to. Her own diaphragm will continue to grow and by the time she's an adult it will only be a small percent of her diaphragm rather than 98% of her diaphragm.

After the surgery Dr. Kays told us that over 75% of her liver was in her chest. She had a small left lung that had been trying to grow between her ribcage and her heart and he described her right lung as a "pancake". After a long two weeks on ECMO blood clots had started to form in the 'wrong' places and the circuit needed to be changed so they decided to try her off ECMO completely because she had done so well when they stopped the machine after a previous blood clot scare. We were told the next day that there was a 90% chance of her going back on ECMO. Her heart was having trouble pumping blood to her lungs because the pressure in her lungs was too high and blood was backing up in her liver. Dr. Kays worked his magic with some fine tuning, flipped her on her belly with the cannulas still in her neck, chest tube, breathing tube and IVs and Arissa never took another step back. She had tolerated everything from day one with no desats and from then on she was on her way home, we weren't always sure but I guess she was.

She was soon off the oscillator and onto a vent. After 2 weeks she was able to go to a CPAP. I was able to hold her for the first time when she was 1 month and 1 day old. She spent 6 days on the CPAP then went to just a nasal cannula. One day later at 5 weeks old she was moved down from NICU III to NICU II.

We spent the next 25 days in NICU II working on her feedings without too many problems, the main concern was her weight gain. With her being a right-sided DH, so much of her liver had gone up into her chest that there wasn't much room for anything else to fit so the rest of her organs stayed down for the most part only a small portion of her bowel had slipped up during the last few weeks of the pregnancy. This being the case she doesn't have as many problems eating as they'd expect from a left-sided DH.
She does use more calories than other babies eating because she works harder to breath with her lungs being so small so her weight gain has been slow going. She's 8lbs 6oz now at 13 weeks so we have some catching up to do. It ended up being a total of 61 days in the hospital before we took her home, and 91 days away from home for me. It has been a LONG pregnancy and first few months of her life, but I wouldn't change it for the world. She's amazing and has gone through more than most people go through in a lifetime.

I'll be the first to admit that Baby Girl has an attitude, but I'm sure that's what got her through this and I promised the Lord that if she got through this I'd be willing to put up with anything she had to give me. There were times when I thought we might be asking too much of her, especially when we had to hold her down through the breakthrough pain, but to the moms out there that are going through the same thing right now, she's one of the happiest babies now, after it all, that I've ever seen. With the way she fought I knew she wanted to be here just as much as we wanted her here and there was no way I'd ever have given up on her. For some reason she was meant to fight this battle and Joe and I were meant to be there for her while she did it. I felt from the very first day we found out that she had CDH that she was chosen for me, I don't know if I've done anything special for her but I know that she's changed my entire outlook on life, I've always believed in miracles but never thought I'd be given one.

We're home now, and she's on a very small level of oxygen just until her lungs finish healing. She doesn't really need it but Dr. Kays thinks that it can only do good now. To be quite honest I'm not sure how much she actually gets, she's a Houdini with that nasal cannula, at this point I'm just short of wrapping her head in medical tape to keep it on. Her lungs will continue to grow throughout childhood so she's going to live a completely normal life.

Written by Arissa's mom, Jessica Morgan (MD, USA)

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