Mary Elizabeth’s Story
After 2 years of trying to conceive we were delighted to be pregnant with our miracle baby. The over-the-moon feeling came with a huge snag in it when we were told at 20 weeks gestation that our baby had what they called then, “Cystic Hygroma” also known more currently as a Lymphatic Malformation (LM). This diagnosis was a bit overwhelming. Looking it up, you read so much – especially with the term “cystic hygroma”. I didn’t even know if my baby would live – and I thought it might be linked to a chromosome abnormality of some sort. All I did know is the child had a growth on the side of her face under her neck that was the size of a golf ball and graduated to maybe the size of a tennis ball towards the end. At the time it was devastating news… and I had no idea of the true extent of the condition. I really thought it would be removed and that’d be all.
Towards the end of my pregnancy I was told it was growing and I needed to be sent to Westchester Medical Center in NY for a high-risk OB and from that point we proceeded to meet with other doctors including an ENT. They were great doctors, they seemed to know much more. And we trusted in that. At 37w6d – May 20th, 2005 we had scheduled an Exit Procedure (specialized surgical delivery procedure used to deliver babies who have airway compression, which is basically an extension of a c-section, where the baby remains attached to the umbilical cord/placenta while the doctor establishes an airway so the baby can breathe). They knew the baby’s airway would be compromised because they had sent us in for a fetal MRI which showed the mass more in depth and that the baby’s tongue was enlarged as well. In addition to this, they did not want to have to make the c-section cut any larger than it needed to be. So, while in the OR and on the table they used an amnio needle to drain the cysts from the baby while still inside of me. This was done before any drugs were administered and lasted for about 20 or so minutes of twisting and shifting the needle to get the right spot. Needless to say, it became painful and felt like 20 hours. After that was done they put me under general anesthesia for the exit procedure due to the seriousness of it.
Having said that, I – Jessica the mother, had missed the birth of my baby since by the time I woke up she was already whisked away to the NICU. As I became more aware my husband kept repeating to me that I had a baby girl (as I did not know the gender previously). We were both feeling that over-the-moon feeling again. When the drugs wore off though, I was overwhelmed by what lied ahead. My husband began to show me photos of my new little girl, Mary Elizabeth. And then, one by one my family members got to see her – but I didn’t – until 12 hours later and even then it was difficult as I had been recovering from a c-section and she was up high in a little incubator. The next 30 days of her life were extremely stressful. We lived at the hospital (there was a Ronald McDonald house IN the hospital a few floors up and we were grateful for that). She was on a ventilator for a week and had a CT-scan, and then at 7 days old she had her first surgery. It was about 7 hours long and the hardest day of my life. She couldn’t breathe on her own so they needed to place a tracheostomy tube in her neck, but her mass was larger than I had imagined as it stretched down to her chest and there was no way they could trach her with that there. So, they did a debulking surgery and trached her that day. The recovery was difficult, she was so very swollen and had a hard time/fought the ventilator etc. They gave her a muscle relaxant drug/paralyzing agent which basically made her lay there for a few days until they could get her breathing controlled. It was a long few days for us. But eventually they got her off and she was doing better and even came off the vent.
As mentioned, her tongue was larger than normal and post-surgery became even more swelled. There was no way she could be breastfed or even suck on a bottle, so she was fed by a feeding tube through her nose. This was not ideal to bring her home and do, so the doctors said she needed a gastrostomy-tube placed. And at 3 weeks old she went under anesthesia once again to have that done. We learned very quickly, that our new titles of mom & dad would include us becoming little nurses as well as we learned how to care for our daughter.
After 30 days we finally went home and felt like we were stealing our own baby. We pushed forward and were determined to care for her as best we could and did great. Only a month later however we were in the OR because of a high fever, redness to her cyst area and swelling. The local doctors didn’t know what to do or what it was. They did give her antibiotics which helped clear it up but the swelling didn’t subside. So, we felt it was a set-back, but today we know her LM was infected and she would’ve benefited from steroids in addition to those antibiotics. In addition to that little hurdle, we noticed her large protruding tongue was just bleeding and oozing a little more each day. We were worried about all this loss of blood, we were disheartened by this, the fact that it was always dried out and crusted over, and generally having no real true hope for it. Our doctors gave us little information, said she’d grow into it. But it was too large to imagine that!! So at 3 months old we said something had to be done, and thus they admitted it’s out of their expertise and referred us to Dr. Berenstein, an interventional radiologist who they thought could simply cauterize the area or something.
We remember our first meeting with Dr. Berenstein because it was a very long wait in the waiting room, our daughter was fussy and we were at our wits end with the unknown. But upon walking in to his office, we knew we were with a whole ‘nother league of doctors. He immediately called Dr. Waner over from wherever he was (whether it was in the hospital or his nearby office) and within minutes the two of them were bouncing off information to each-other and seemed extremely confident in her case and what they were to do. There was hope in that room, hope that we needed. No, this wasn’t a condition that I thought might be taken care of in a year… but it was a condition that they were educated in, weren’t doubting they could help or handle – they were going to tackle this obstacle with the confidence and the knowledge that was needed.
From that day forward Mary Elizabeth proceeded to have surgeries upon surgeries that included tongue reductions, quite a few sclerotherapy procedures, debulking in the chin a few times, her right parotid removed as well as some of the LM on the right side of her face debulked a couple times, dental work, steroid injections, coblation and several (to say the least) CO2 laserings to her tongue, airway, gums, cheeks, and mouth. Between those and some MRIs and other small things to be done, she’s been under anesthesia over 40 times in her 6 years of life. I would be here all day long if I tried to explain what happened during each of those surgeries. It was sometimes quite the rollercoaster ride. But, to look back on it- even though I’m not sure how we did it- we did and because of it she has improved leaps and bounds. And even during the hard parts, we had the support of the NY team all along the way who was always answering our emails or calls with questions. We no longer were in the dark. And with that our knowledge about the condition has grown so much. Because of the success of the surgeries, her appearance and quality of life has dramatically changed. And we are happy to report that at 4 ½ years old she had her gtube removed (though she started eating at 1 yrs old and was improving all the while). In addition, the most memorable day was November 30th, 2010 at 5 ½ years old when her trach was finally removed. Had it not been for the perseverance of her doctors she may still have that trach in place and have the airway obstruction.
The big picture is- that we have a happy, beautiful, confident & smart little girl who has overcome so many challenges. won’t lie and say the journey was easy – it wasn’t – but most of the hard stuff she won’t remember. She has a few things left to do, some that has to wait but all in all she has come far. I would encourage anyone going through something like this to get the best doctors out there, for that is key and secondly, to join online support groups which has also helped us tremendously in this journey.
– Jessica & Andrew