🌹Piper Rose, my Leap Year Baby🌹
My daughter Piper was born via c-section at 39 weeks right at the start of the COVID madness. 2/29/2020 at 2:29pm I had a normal and healthy pregnancy, but her birth was anything but normal. When the doctor pulled her out of my womb, her lower body was completely raw. The hospital staff was completely shocked and horrified. No one had ever seen anything like it. As they attempted to clear her airway, blood started pouring out. She was whisked away straight to the NICU before I could even see her.
The doctor stitched me up as quickly as he could as I laid there sobbing. I was alone in the delivery room, and had no idea what was going on with my baby girl.
Once I went to recovery, all the nurses were quiet around me, none one would tell me how my baby was doing; I was terrified she was dead,
Eventually, the neonatologist came into recovery to speak to me. A nurse came with him as well. I was sitting there with tears streaming down my face, afraid of the news I was about to receive.
They had good news and bad news. The good: Piper was alive and they think they knew what was wrong. The nurse had seen it before, but only once in her 25 years career. The bad: they thought Piper had a rare skin condition called Epidermolysis Bullosa or EB for short. I had never heard of it before, but it sounded very scary.
The doctor explained she had very fragile skin and would need to be wrapped in protective bandages to prevent infection. I asked if Piper would get better; the doctor said he didn’t know. Some get better, some get worse, some die. And worst of all, there was no cure :(
They said I could see her as soon as I felt up to moving. I just laid there in shock at the news I just received. I was full of so many emotions. I was all alone and had no idea if my baby would live or die.
A few hours later I felt I could move into a wheel chair and a nurse wheeled me up to the NICU. There was my baby, in this incubator covered in bandages and wires. But she was beautiful with her Fuzzy blonde hair and blue eyes. She was a whopping 9 pounds, 9 oz and was 21 inches long. Aside from the skin issue, she was perfectly healthy. They wouldn’t let me hold her just let, so I sat there rubbing her head telling her how much I loved her.
The next few days, there were so many ups and downs. They sent off blood for genetic testing to confirm her diagnosis. The doctors began researching EB to learn how to best care for Piper. I had also enlisted my best friend, Ava, to research EB. I couldn’t bring myself to look on-line for fear of what I might learn. Ava found so much on line about EB; some good, some bad, some terrifying. She got us a list of the correct bandages and ointments to use, links to videos on how to bandage an EB baby, the best clothes to buy, diapers to wear and bottles to use. She found support groups online with so many helpful people also going through the same thing.
Over the days I learned how to carefully bandage my baby to protect her skin from getting worse or getting an infection. She struggled to breastfeed, so she was fed with a special bottle. At first she started out eating amazing, but slowly started to struggle when she was around 10 days old, her breathing began to get “noisy” but no one knew why. Around that time, her doctor learned about some hospitals that had an EB clinic. He contacted one of them and spoke to the dermatologist there. She had dealt with EB for many, many years. She gave the doctors some good tips and suggestions but feared Piper had the worst form of EB, Junctional Hertliz, based on his description of her.
A couple of days later Piper stopped eating altogether and a feeding tube had to be placed down her nose into her stomach. It was so hard to watch my baby fight for her life. I felt she was fading right before my eyes.
When she was 2 weeks old, we still had no answers. The unknown of COVID was delaying everything. The doctor felt she might receive better care at a hospital with an EB clinic. I was all for anything to help my baby get better. He contacted the nearest hospital with an BB clinic, but because of the uncertainty of COVID, they weren’t accepting transfers. The dermatologist however, offered to watch a bandage change via video conference before deciding to override the transfer request.
The next day, everything was set up and the doctor at the EB clinic watched me carefully unwrap, clean and rewrap my fragile baby. Afterwards, we had a meeting and the dermatologist felt her hospital couldn’t provide any better care than Piper was already receiving. She said transferring her would be risky and she might not survive the transfer. She also said that in her many years of experience of treating EB patients, she felt Piper had the Junctional Hertliz form of EB, and she likely wouldn’t live past four months. I asked about having a feeding tube placed, only to be told she might not survive the surgery. It felt like no matter what we did, she was going to die anyway.
That was devastating news to hear. The thought of my baby girl dying was more than I could bear. I spent the next few days holding my baby girl, trying to decide what was best for her. I wanted her to live, but what kind of life would she have??? Would she be in pain everyday of her life?. Would she ever walk? Eat without a feeding tube? Here I was a brand new parent, all alone holding my fragile baby girl, feeling totally lost.
Over the next few days, Piper started to decline. Blood work showed she was anemic and needed a blood transfusion. Two days later her breathing got worse and she spiked a fever; more blood work was done, and it was discovered she had a blood infection. Her doctor called the EB clinic for advice on how to best treat the infection, and the doctor asked to speak to me. She said I could choose to treat the infection, and Piper might survive, or I could decide to make her comfortable and let her go. It was an awful decision to have to make.
I sat there in the NICU rocking my baby girl while trying to decide what to do. But I never got to make the decision; at 2:50am on a Thursday, while holding my baby in my arms, she looked up at me and smiled, closed her eyes and took her last breath. I waited too long to decide. She was 21 days old.
The autopsy revealed that her epiglottis was severely inflamed and it closed off the opening in the back of the throat that leads to the upper airway. There was evidence of silent reflux, mucus plugs and swelling in her upper airways. The NICU doctor didn’t know what any of that meant in terms of being related to EB or if it would have been treatable. I later learned what it all meant and And the what if’s still haunt me to this day.
The day of Piper’s funeral, her genetic results came back, but I couldn’t bring myself to open them. I put them in a safe place and tried to move on with my life. Opening them wouldn’t change anything and I worried the results would make me question every decision I had made in those 3 weeks.
On Piper's first birthday, I decided to open the results. Much to my shock, Piper didn’t have Junctional Hertliz EB like the doctor at the EB clinic suspected. She didn’t even have Junctional EB. The results listed her as having generalized severe EB simplex. I was completely floored. I had no idea what any of this meant, so I began to research the hell out of EB and specifically, EB simplex. I couldn’t believe what I was reading. Most with EB simplex survived. I joined social media groups and read other people’s experiences. I researched her specific Keratin 5 mutation. I spent hours on-line looking for any information I could on her mutation, c. 1421c>t, trying to understand what it meant, and why it caused EB.
I found another EB family with the same mutation. The mom has EB, and 3 of her kids were also born with EB. Sadly she too has an EB angel. Knowing it wasn’t just Piper, made me feel less alone, but it still sucked.
I met with a genetics doctor to help understand how Piper got EB. She explained how one gets EB simplex, and recommended I be tested. It was discovered I didn’t have the mutation. I tracked down her “father” and demanded he be tested. He didn’t have the mutation either. The doctor explained that her EB was caused by a spontaneous mutation. A fluke of nature. I learned that silent reflux was common in EB and totally treatable. My baby could have lived. The more I researched, the more angry I got. I was so, so angry, why Piper?? Why me?? How could the doctor have been wrong? Why did I listen to her?? My baby should have lived. I was more angry at that point, than I had been anytime in the past year. I felt like I had failed my baby. I blindly trusted the doctors because that is what we are taught to do. But the doctors was wrong, so very, very wrong.
The genetics doctor explained the odds of having another baby with EB would be very, very small. But I didn’t want another baby, I wanted Piper. I wanted my baby in my arms.
This entire experience has opened my eyes to so much in this world. It has also caused me to trust doctors a lot less. I am currently going to nursing school to be a NICU nurse. I hope I never get an EB baby in my NICU, but if I ever do, I’ll give him or her the best care I can. Everything I do is for Piper. I don’t want her death to be in vain.
Thank you to Garrett’s House for allowing me to share my story. I miss my baby every single day and I wake up everyday to keep her memory alive.
❤
Lynnea, Piper’s mommy.
