February 7, 2014

Paisley's Story: Part II

     I was out of recovery and in a hospital room, surrounded by my family. Everyone was so overjoyed by the news that Paisley made it.  I was still in a fog from the pain meds and was in and out of awareness. It was sometime later on that night when I finally snapped out of it. I asked the nurse if I could see her, I was thinking they would be bringing her to me in my hospital room. Sadly, when your baby is in the NICU, it doesn't work like that. I was told that I had to wait until the next day to see her. But Rusty and my mom did get to see her for a short while after delivery and I was able to see a picture of her. But still, I felt so empty not being able to hold her.  I couldn't wait for that night to be over with.  Anxious doesn't even begin to describe how badly I wanted to see her.

    Sun rise... the day was breaking outside the window.  Oh, how excited I was. I was going to get to hold my baby, snuggle with her,  kiss her sweet cheeks, count her fingers and toes, tell her how much I loved her, breathe her in, and spend my well deserved moments of baby bliss with her. I was in a lot of pain, as I refused to take more pain meds the night before. I wanted to be coherent, I needed to be alert and aware so I could get up as early as possible to see her. When the nurse came in to check on me for the morning rounds, I was up, had already taken a shower, was dressed, had my hair and makeup done and was ready, ready to get to that NICU and get my baby! The nurse was in complete shock. Apparently that's not the norm. I guess the magnitude of knowing that she made it and not knowing how much time I had was weighing heavily on my heart. I needed to get to her, I wanted to see her alive. I had read so many stories about babies who lived for a day or two before they passed, given the health conditions she had. I felt like my time was running out. Rusty got a wheel chair for me and pushed me to the NICU. 

   We were there with our daughter. Our sweet baby girl was breathing on her own. She had been on oxygen for the first 5 hours of her life, but started breathing on her own. I couldn't believe my eyes... she was nothing like I expected, nothing like the doctors had described. She wasn't deformed at all. There was no sixth finger, no clubbed feet and her head was not misshaped. Her body was a bit odd shaped and her legs were extremely bowed and folded inward toward her body, basically like she was sitting with her legs crossed. She was hooked up to several monitors and had a tube going up her nose and one coming out of her belly button, which was very scary at first.  Rusty grabbed a chair for me and I began to grab her so I could sit down with her. "Ma'am, I'm sorry, but you can't hold her" came a voice from behind me. "Why not? She's my baby." I said. The nurse explained that the UV line (umbilical vein) was not easy to insert and handling her could make it fall out. My feelings were so hurt, I began to cry. I just had a baby, my hormones were out of whack, I was in so much pain, my body was beginning to swell and all I could do was stand there next to her and hold her little hands. This was not at all how I pictured my first moments with her, another dream crushed. But through the heartache, I thanked God for her, the opportunity to be able to see her alive, the fact that she was here was such a blessing.

     Over the next couple of days, I was in and out of the NICU and my own room, trying to follow doctors orders about keeping my feet elevated and getting plenty of rest. Easier said than done when you can't take your baby with you to your room. I only left the NICU to eat and sleep in my room. Two days later,  I just so happened to walk in the NICU after shift change and Paisley's night nurse was charting her vitals and weight. When she picked her up out of the scale, she turned to me with a big grin and said, "do me a favor, hold her while I go grab a camera."  Oh my gosh, I couldn't believe it, she handed me my baby, for the first time, I was able to hold my sweet baby girl  in my arms. Paisley had been crying and the second she handed her to me, she stopped... we both just looked at each other, and it's like she knew I was her momma and she was calm.  It was the most amazing feeling ever. The nurse came back with a camera, took a picture and even printed the pics out for me. I got to hold Paisley for a while. In that time, it was like the world around me stopped. I wasn't aware of anyone or anything around me, it was just me and my baby, and I was floating.  That was the moment I had been dreaming of.

    On day 4, I was released from the hospital. I opted to stay in the NICU housing unit that night. I just couldn't go home with out her. But that night was awful. There was no way I was going to get any rest with all the loud slamming doors, uncomfortable bed and having to pump every two hours. My husband convinced me to come home the next day. It was so hard to leave her, I cried all the way home, but I knew she was in good hands. That night I spent some much needed snuggle time with my little man, Carter and slept better than I had in months. The anxiety of whether or not she would make it was no longer a constant worry, I was actually able to rest my mind and body. I needed it too. I woke up feeling so refreshed and ready to face a new day. And what a great day it was... Paisley was moved to "step down" ICU.. still in the NICU but in another section and most importantly, she was by a window to the hallway... Carter could finally see his baby sister!

     Over the next few days I worked with the hospital's on staff therapist to help Paisley drink from a bottle. We had been informed somewhere in the span of the first few days that she had a cleft palette. I didn't understand what that meant, I'd only ever heard of or seen cleft lips. Several doctors tried to explain why it was preventing her from drinking and I just didn't get it... I kept thinking she would eventually get the hang of it. The therapist best described it... imagine drinking out of a straw with a hole in it, you'll get a little bit through the straw but you'll never achieve full suction. Aha... an explanation that I could relate to! We tried several special nipples made specifically for cleft lips and palettes but she wasn't taking to any of them. I even tried breastfeeding and that didn't work either.  She was in the Baptist East NICU for 7 days... 7 days of seeing my baby in a large open space with no privacy, pulling a curtain around me to pump every two hours and not being able to relax. It was day 7 when the physical therapist recommended that I push the doctors to get her transferred to Lebonheur Children's Hospital. Luckily, Lebonheur had room for her and we were transferred that day. At Lebonheur she had her own room with privacy and comfortable chairs, perfect for snuggle time, bonding and naps together. I was able to be somewhere quiet with my baby girl and just relax. It wasn't home but it was the next best thing.

    The next day we met the speech therapy team at Lebonheur and began working with them every day. Paisley was finally drinking from the Pigeon nipple... a specialty nipple for clefts. But she wasn't drinking much.  Some feedings she would drink 1ml, others only 5cc's. It was very inconsistent. In the meantime, we started getting results in from all her blood work, MRI's, CT scans and X-rays.  We were seeing all sorts of different doctors. She had her therapy team, which consisted of speech, occupational and physical, a geneticists, an orthopedic, a neurologist, a neurosurgeon, an ENT, an ophthalmologist and a dentist.  There was a lot going on and it was very hard to keep up with it all. Her geneticist was telling me that she suspected Paisley had a rare form of dwarfism called Kniest Dysplasia, but the blood work came back inconclusive. It was clear that something wasn't right with her bones and shape, so I gave consent for all of Paisley's images to be sent to Cedar Sinai in CA, The National Skeletal Dysplasia Registry. They have top notch researchers and her geneticist was confident that they would be able to diagnose her. While I wanted answers, mostly I just wanted to bring my baby home. The pediatric doctors made their rounds every morning and I would ask them all the time "When can I take her home?" The answer was always the same, "When she can drink her feedings consistently with out using the pump." At the rate she was going, I felt like that could take forever.  Already, nearly a month had gone by and there was still no improvement in the amount she was drinking. The speech therapists started talking about g-tubes. At first I wasn't open to the idea of my baby having to be fed via a tube in her belly. It seemed so scary and I was still holding on to hope that she would miraculously start drinking her bottles. A nurse put it in prospective for me one day when she explained that she had seen babies in the NICU up to 5 months before for the same issues Paisley was having. She flat out told me that the longer I put off the g-tube, the longer we would be there. That was all I needed to hear to get on board the g-tube train! Not to mention, I was exhausted. I was spending 8 to 10 hours a day at the hospital, coming home and still keeping up with my mommy duties. I was on maternity leave, a time when you're supposed to relax, let your body heal, spend quality time with your newborn and enjoy the break from work. But this was far from any of that.... since the day I had her, I never got a chance to relax. There was no down time, only go time. Work would have been less stressful than the life I was living.

    I began telling the doctors that I was open to the idea of a g-tube. They were happy to know that I was no longer in denial about Paisley's progress, but in a last ditch effort to avoid the g-tube, they decided to try to have her fitted for an obturator. (a mouth piece that would fit inside her cleft palette to close the hole so she could get suction to drink from a bottle) Unfortunately, she aspirated on a piece of the molding they used to cast her mouth and the process had to be redone. And even though the second time around, it worked and the mouth piece fit, she still couldn't drink anymore than she had been. It was disappointing. I had high hopes but alas it was time to get the g-tube ball in motion. That day came on May 23rd. Aside from seeing her hooked up to a ventilator and being intubated after surgery, the sight of a foreign object sticking out of her belly was overwhelming. I felt so guilty. I knew it meant we'd go home sooner, but the reality of having to feed my baby through a tube really hit hard. I was heartbroken and scared. I was not cut out to deal with these sorts of things... or so I thought. A few days later, I was being taught how to hook up her feeds over the pump and how to bolus feed her. It was a lot to learn in a short span of time, but you'd be amazed how quickly you catch on when your baby's livelihood depends on it. Then, like no time had passed at all, Day 49 arrived, May 29, 2013..... a day that felt like a thousand Christmas mornings wrapped up in a big pretty bow, we got to bring our sweet Paisley Grace home with us.  Oh, how I cried, but for the first time, it was tears of joy! Nothing in the world can compare to the feeling that was in my heart that day. It was purely seventh heaven.

     You'd think my stresses would be over, I could be home and relax now, right? Wrong! Upon her release, we were given a book. The book had all the info pertaining to her health, care instructions for her g-tube, numbers to call for her medical supplies, names and address of her team of Dr.'s and a calendar of all her follow up appts with all 12 of her Dr's. Yes, you read that right.....12. And it began the very next day, May 30, first Dr. appt with her new pediatrician. I was barely home with her for 12 hours and had to get up and go... with all of her tube feeding supplies. It's one thing to feed a baby in a Dr.'s office, it's quite another to feed a baby through a tube in a Dr.'s office. Not that I cared what people thought, but the stares, the questions and even the disgusted looks on some people's faces... I realized then that my new reality was not going to be easy. And it wasn't. Soon after coming home, she began to get sick after every tube feeding. Her ability to feed orally had improved a bit but she was still getting sick. I was constantly cleaning up messes. I'm sure many of you can relate to the reflux and projectile vomiting issues.... no fun and extremely tiring.
      The month of June flew by. My employer had been kind enough to grant me an extra 4 weeks of leave so I could be home with Paisley. But the time had come to go back to work. I started back on July 1st. I was happy to get back to a sense of normalcy and routine. The preschool Carter attended so graciously accepted Paisley with her g-tube. I had to teach them how to feed her but they were willing to learn and happy to do it. Trying to work while managing all of Paisley's appt's soon became more than I could handle. I was lucky to get 20 hours most weeks. Then we finally had the follow up appointment with her neurosurgeon, the game changer. We were informed of Paisley's congenital cervical spinal stenosis and told that she would one day need spinal surgery. They explained that her neck needed to be handled very carefully because any sudden movement could cause permanent paralysis. WHAT? Why was I just now hearing about this? We had her home for nearly 3 months and had no clue about this. Granted, she was still a baby, so naturally we were already supporting her neck and head, but still, I was outraged that we were not informed. Immediately I knew that I couldn't risk her staying at daycare. In the nursery there are some babies who are learning to crawl and pulling up on things, all it would take is a simple fall and Paisley could have suffered serious damage. Another devastating blow, another change in my life, my routine.... and how would we  manage financially? Every detail of our lives was up in the air. But we took a leap of Faith. I felt that this was God's gift to me, he was creating the perfect storm for me to be able to stay home with my babies. And so, on Sept 20th, I put in my last shift as a Business Systems Specialist, the very thing I'd prided myself on for 8 years, my identity up to that point, the job that I shared with my second family, my coworkers, and the place where I grew from a young carefree lady into a woman with a family and values. It was hard but it was necessary and ultimately Paisley's health was my main focus.

   Fast forward 5 months... here we are, February 2014.  The results from Cedar Sinai came back and confirmed our geneticist findings, Paisley has officially been diagnosed with Kniest Dysplasia. A rare form of dwarfism caused by a mutation in the COL2A1 Gene. This form of dwarfism affects the collagen of the eyes and joints. Some children with this disorder experience severe arthritis by the age of 3, some lose a little to all vision and hearing and most have cleft palettes. There are several other symptoms of the syndrome, of which Paisley has.
You can learn more about Kniest Dysplasia at www.ksginfo.org/info.html
In other updates,we don't use her g-tube anymore, although she still has it. Her nutritionist switched her to a lactose free formula back in September and she started drinking her bottles with no problem. However, she can not get rid of the tube until after her cleft palette repair surgery. We're hoping that will be in 2014 to avoid major speech delays and eating/chewing issues, but her neurosurgeon must give the ok. We may need to undergo the spinal surgery first, then do the cleft repair. Only time will tell. In the meantime, we are going to enjoy every minute of her... she's such a blessing and such a joy to be around, always has a smile and giggle to share. I can't wait to continue sharing her story and milestones. Thanks to all who have read this and prayed for my sweet angel.

I've learned a lot in the past year. I've been humbled beyond belief and blessed beyond measure. My heart has been broken and my world has been shattered, but through it, I learned the true meaning of Faith, Letting go and letting God. I've learned to grateful for the troubles I have. No matter how bad a situation seems, it could always be worse. I share all of this with you because I hope that Paisley's Story will be a blessing for someone else one day. Maybe you're that mother that was just told to terminate your pregnancy, or maybe you're that mother who has just been told that your baby has a form of skeletal dysplasia, or maybe you're the friend or family member trying desperately to find that glimmer of hope your friend or loved one needs to hear.....either way, I hope you find inspiration and hope here in my blog. God Bless!