A lot of thought went into this week’s challenge and Candice knew it would truly test me. We’ve been practicing and prepping for months and I’m so glad it finally happened!!
“Switch-Hit. Switch lives with me and take care of 2 kids…one with special needs awaiting transplant.” – from Candice Sheppard
Candice and I had the chance to meet when we both worked at Powers Agency. She came in a few months after me and served as one of my superiors in Account Services. We worked on a few projects together, but mostly, we built an incredible friendship. She is a great role model, confidant and friend. She has also helped coach me through my job search, served as one of the best references I’ve ever had and vented with me about our year of co-unemployment. She’s one of the most supportive people I’ve ever met.
Candice is also one of the strongest people I have ever met in my life. Shortly before she began working at Powers, Candice found out that she and her husband, Chris, would be welcoming a beautiful little bundle of joy in the spring. I was beyond excited when she shared the news and couldn’t wait to meet Baby Sheppard. On May 22, 2012, Baby Alice graced the world with her presence bringing their family to a total of five, including step-brother, Blake, and sister, Sophia. Her arrival was no small feat and came with many complications. Since then, this sweet little angel has fought, day in and day out, to survive.
Here is a little bit of the back story, taken from the “I Heart Alice” Facebook page:
Alice Mae Sheppard came into this world fighting on May 22, 2012. Upon birth, a previously undetected heart murmur was found and over the course of two days, it began to become louder and more pronounced. Ready to leave the hospital in which she was born, Alice’s parents were told to place Alice in an incubator for transport to Cincinnati Children’s Hospital Medical Center after an echocardiogram discovered she was born with three major heart defects: severe Aortic Stenosis, Mild Pulmonary Stenosis and Hypertrophic Cardiomyopathy. All of which would be fatal if left untreated. Shortly thereafter, Alice was diagnosed with a genetic mutation of her PTPN11 gene causing her to have Noonan Syndrome (NS).
Noonan syndrome (NS) is the most common syndrome you have NEVER heard of! NS is a variably expressed, multisystem disorder with an estimated prevalence of 1 in 1,000 – 2,500 births. People with NS may experience bleeding issues, congenital heart defects including hypertrophic cardiomyopathy (thickening of the heart muscle that forces the heart to work harder to pump blood and if untreated leads to cardiac arrest) and/or pulmonary valve stenosis (narrowing of the valve that controls blood flow from the heart to the lungs) lymphatic abnormalities, small stature/growth issues, feeding and gastrointestinal issues, failure to thrive, hypertelorism, learning disorders, unexplained chronic pain, chiari malformation, hypotonia, ptosis, skeletal malformations, chiari malformation, laryngomalacia, tracheomalacia, opthamology issues, orthopaedic issues, oncology issues and much, much more. Because of the variability in presentation and the need for multidisciplinary care, it is essential that the condition be identified and managed comprehensively.
Alice has endured multiple surgeries to repair her heart issues including an open heart procedure at just four weeks old on June 21, 2012. During this painfully long 9-hour surgery, all three of her defects were addressed. Her native Aortic Valve was removed and a human donor homograft piece was attached to the roots replacing her diseased valve. Her Pulmonary Valve was repaired by way of a Transannular Patch and lastly, her thick, dysplastic heart muscle was trimmed back to alleviate the pressure for the heart to more efficiently pump blood to her other vital organs. Alice recovered remarkably well, but with minor complications including a blood clot formation in the right atrium of her heart and the inability to thrive and gain weight. She returned to the hospital several times throughout the first few months of life.
Due to NS-related feeding difficulties and delayed gastrointestinal motor development, Alice was given all of her nutrition through a feeding tube. Infants with NS often display issues with eating due to sucking problems because of weak muscles in the mouth and sometimes a high-arched palate, a weak chewing action that can mean mealtimes take longer than expected and projectile vomiting during or after meals as a result of severe reflux and motility problems. Initially Alice was released home with an NG tube (Nasogastric tube): A tube that is passed through the nose and down through the nasopharynx and esophagus into the stomach. After several months with an NG tube the risks and complications such as irritation of the esophagus, sinus infections and interference with the lower esophageal sphincter which cause reflux of stomach contents, often leading to aspiration pneumonitis became evident. Not to mention… a very curious 6-month old baby can pull the tube out herself! After extensive research, GI testing and with the recommendations of Alice’s team, everyone decided it was best to switch her NG tube to a more permeant solution since she was not making little if any progress eating by mouth. Alice arrived for gastrostomy tube (G-tube) placement on November 16, 2012. This procedure was a simple and routine one requiring anesthesia and a 48-hour stay inpatient, but in true Alice fashion….things became complicated quickly after she went into her second cardiac arrest upon intubation. She was unresponsive for over 10 minutes and doctor’s feared the worse. Once they revived her heart, they began damage control putting her into an induced hypothermic state to attempt to preserve her vital organ function of the kidneys, liver and brain. She was quite literally freezing when miraculously she began to move her fingers and kick off blankets just as she normally would! As they warmed her body back up and began testing her brain function for seizure activity, the medical staff were reassured that she had not suffered any life-threating damage…to her brain or other organs. Her heart, however, took the worst of it. The function of her heart was severely decreased and to this day it has not fully recovered.
And this is where our new journey begins….
Alice is listed for full organ replacement. She is in need of a new heart. One must understand, that all of the surgeries have been and will continue to be palliative, meaning that they seek to relieve the symptoms of her disease without ever providing a cure. Simply put, there is NO CURE FOR HER DISEASE.
So, now that you’ve got the background on Alice’s situation, you can understand why this challenge is so different from any I’ve had before. There is literally a small person’s life in my hands and I was BEYOND terrified that I’d screw it up some how.
Candice and I have spent time over the past few months practicing and prepping for this week. I have learned how to administer Alice’s medicine, read up on infant CPR, learned how the machines work and familiarized myself with all necessary information. I have also spent time with Alice as she has a level of discomfort with “stranger danger” and making her as familiar with me as possible was really our only hope. On previous visits, Alice would begin crying the moment Candice left the room and it was just me and her. But this time, I was greeted with a big, beautiful smile the second I walked in to see her.
Alice also has a big sister named Sophia. Soph is 3 (going on 13) and is a ball of energy. She is incredibly smart, incredibly sweet and loves her little sister like crazy. She is currently in “word police” mode so I had to be VERY careful about what I said. For the record, God and Shoot are off the table. She picks out her own clothes, loves shoes, and doesn’t hesitate to provide you with instruction if she thinks you’re doing something wrong. But it’s all soooo adorable. She reminds me sooo much of Candice.
I would be responsible for these two for the day while Candice and I traded lives. Now, I thought about booking a Pure Barre class, or having her spend time with Gus, but this was a challenge for me, not Candice. I decided that instead of all of those things, I’d give her a break. I also knew that if I didn’t give her a reason to, she might not leave the house. I booked her a massage at Mitchell’s and sent her off for a day of relaxation and personal time. I got this.
Alice is hooked up to a G tube through her abdomen. This is a blessing because before it ran through her nose and down her throat. She would rip it out from time to time. This one goes directly through her belly and doesn’t seem to be as uncomfortable. She also has the largest accessory I’ve ever seen for a baby. This lovely wheeled pole follows her around, with the exception of four-hour breaks between feedings, every where she goes.
Candice and I went over the meds right when I got there. She receives three different medications via syringe through her G tube and they go directly into her stomach. She gets blood pressure medicine, acid reflux medicine and heart rate monitoring medicine to ease the every day stress required for her heart to function. She also receives a flush of water to push everything through her system.
The next step is to clamp up Alice’s feeding tube. If you do not properly clamp these before opening or closing a port, both the contents of Alice’s stomach and the food entering it will pour out through the tube. I did pretty well the first time, but learned that the hard way upon closing her back up. Whoops.
So…Clamps closed. Syringe Inserted. Clamps Open. Push Syringe. Clamps closed. Remove Syringe. Repeat until all medicine has been administered and tubes have been flushed.
Sophia was coloring and having some breakfast so Alice joined her at the table for some rice Krispies. I’m so excited that she can finally enjoy solid food, within reason.
Her monitor beeped and she could be removed from her feeding tube around 12:30. From the moment of that beep, Alice gets a 4 hour break where she can roam free from wires. At this point, Candice usually takes them to the park, for a walk, runs errands, etc., but she has not always had this opportunity. For awhile, Alice needed to be connected almost all the time. The family had spent the previous day at King’s Island for Andy Dalton’s King for a Day and the girls were wiped out. Alice went down for a nap and Sophia and I prepared to play outside on the swing set. I braided her hair and got her dressed and ready to go.
I carried the video monitor with me, but it really didn’t provide me with much comfort. You can’t tell if a baby is breathing through a small video screen.
Sophia showed me the ropes around the new play set. She’s so cute! I was terrified she’d fall and hurt herself while doing pretty much everything. I’m going to have to work on that before I have children of my own or they’ll all end up encased in bubble wrap for extra protection.
Sophia wanted head in after awhile, deciding it was “too hot to play outside” (debatable). We watched some alphabet movies, made lunch and Alice joined us for a “picnic” in front of the TV.
At this point, I’m thinking the day is going realllllly well. Everyone is full, happy and safe. Sophia went down for a nap and I spent some time with Alice, cleaning up and playing with her toys.
And then this happened:
Alice began crying out of nowhere. A lot of crying. If I put her down or stopped moving – crying. I had a flash back to babysitting Belle when this exact thing happened. The only difference there is that feeding her eventually made her stop crying. I couldn’t just feed Alice. She’s on a schedule and there is a machine that feeds her with a set dose at a set rate. We were still an hour away from when feeding time could begin again. Do you know how hard it is to unload a dishwasher with a crying baby on your hip!? They say sometimes you’re supposed to just let a baby cry, but it doesn’t feel right when it’s not your kid. So essentially, I never “just let them cry” as I do not have children of my own. Eventually, after pacing around the room and talking to her softly, I got her calmed down. She napped on me while I watched Orange is the New Black. (You were right, Candice. I’m hooked.)
She woke up after about a half hour when it was time for her to feed again. Candice left me very thorough instructions and walked me through the process before she left, but I was still nervous. I had to clear out the rate and dose and input them to the new numbers. It took me a minute, but I figured it out. I also remembered to clamp and unclamp the tubes properly this time, thank God.
I put her down for another nap with her feeding tube and she slept for almost the entire feed. Sophia and I played memory and then began making dinner so it would be ready when Chris and Candice got home.
Sophia was VERY helpful. It was actually very nice having someone to talk to that understood what I was saying and could respond. She is so very polite. She knew the answers to the questions I had about Alice so I actually only had to call Candice once for something little. We laughed a lot and it made my day each of the 50 times Sophia told me she loved me. Heart. Melted. After a nice family dinner I headed home and crashed, completely exhausted, but inspired. That little miracle has beaten the odds, literally died TWICE and pushed through SO much. And she’s only 15 months old!
I was a difficult kid. The ADHD made it nearly impossible for me to regulate my volume, temper, anxiety levels, thought processes, patience- basically everything that keeps a kid composed in any way, shape or form. I used to fear whether or not I’d be able to handle it if my children were born the same way. I never even considered the other possibilities that could happen until I met Miss Alice.
She is making so much progress every single day! She has biweekly speech therapy and physical therapy at Cincinnati Children’s Hospital Medical Center. Speech therapy consists of a therapist teaching her how to swallow, drink from a cup, taste new flavors, and work on sensory issues and aversions to food taste and texture.
Physical therapy is generally working with sounds right now. It is a series of cognitive and physical development through activities, like playing with toys and stretching, that encourage fine and gross motor skills. Developmentally, from a physical standpoint, she is that of half her age. She can sit up and skate, but cannot crawl or bear much weight on her legs and is nowhere close to walking. Many kids with Noonan Syndrome have this issue due to early surgeries and problems developing as well as low muscle tone which is associated with the disease.
She also goes to the hospital for a 24 hour inpatient stay once a month called IVIG. This is a blood byproduct that is given to her via a 12 hour IV infusion. As the product goes into her body it attempts to find and destroy any of her built up foreign antibodies. She has an astronomically high number of these foreign antibodies due to the fact that she was given a human donor aortic valve at four weeks old as well as about 10 blood transfusions from her various surgical procedures. The problem with finding a new heart for her is that she cannot accept a heart to which her body has built up antibodies against. This could lead to serious rejection issues. More importantly, if her antibody levels do not come down then they will not be able to find a negative crossmatch heart for her. We have to find a positive size and blood type as it is. If they can’t get these things under control, there is a good chance that Alice’s body will reject the transplant she is given, thus defeating the purpose.
I mean. CAN YOU IMAGINE?!
I learned a lot about myself. I am more patient than I thought I was. I’m not sure if that’s something I’ve learned in the last few months since I babysat Belle or if it’s just a sense of comfort and confidence I’ve developed, but I am pretty proud of myself. Both kids survived. No major issues. Brilliant. Obviously, I only had the girls for a day. I didn’t have the full experience of countless hospital stays, extensive surgeries, watching the heart monitor and listening to the doctors explain what they think might happen. I don’t have the endless medical bills or biweekly trips to Children’s for therapy. I also didn’t have to do daily chores while making sure Alice doesn’t miss a feeding or dose of medication. That being said, I also don’t get to wake up to that little miracle to remind me how lucky I am, every single day. A few days have passed since I watched the girls and this has really stuck with me. I cannot get her out of my head. I can’t help, but think about all the time that I’ve spent with her and how inspiring she really is. It’s kept me up at night that there is literally nothing I can possibly do to change the situation and I’m not even family. I am however honored to say that she is no longer afraid of me 🙂 Little victories, right?
I have asked Candice to contribute some of her thoughts on raising Alice, as there is no way I can possibly explain it through her eyes. Careful, it’s a tear-jerker.
“In a normal day I use Alice’s breaktime to go to the YMCA to swim, the Zoo, the park, meet with other Mom’s who will accommodate our crazy schedule. It often sounds like the BEST JOB EVER. I have been home with my kids (not including multiple hospital stays) for 15 months. But…you have to understand that we are “living it up” this summer since we spent last summer hospitalized and Alice’s health can really become compromised during flu season, which spans from October – April! A simple cold can and did land in her in the hospital and lead to a respiratory/cardiac arrest during a routine procedure last year! Also next summer (it is so unknown) we may be back at Children’s again post-op from Transplant. The future is so unpredictable so one thing Alice has taught me is you have to live every day to the fullest and never take little moments for granted. Chris and I have focused more on her quality of life than anything and that is subjective…yes. However, we know that Alice thrives at home surrounded by family. This environment provides lots of natural light, a nice comfortable crib as opposed to a hospital bed and we can hold her all day when we can. Hospital life is not like that. The CICU rooms are probably the cleanest I have ever seen in my life, but you just feel dirty there. The air is dry and cold. There is a tiny window that sometimes you don’t even feel like opening since you are stuck, missing out on fresh air and sunshine with your baby who deserves to see and feel that. Nature doesn’t surround you, but rather beeping sounds and alarms for heart monitors and oxygen and children that have coded in the room next door.
And quality of life also means being able to eat and move. These are two things Alice cannot do. It didn’t seem quite as hard to accept that she was “behind” even 4-5 months ago. She still seemed average size and despite all she had been through, was really doing quite well! Now that she is 15 months, I feel pangs of hurt that she looks half her age and cannot even crawl – let alone stand or take steps. Nor can she enjoy one of life’s simple pleasures – food! She has so much trouble eating and drinking and it’s frustrating to put that spoon up to her mouth 1,000 times and never really see her be capable of finishing even a quarter of a baby food jar. But that is also what makes her “little victories,” as we call them, so special. Even big sister Sophia gets in on the action. She cheers her on right along with us while she helps to try to feed her and show her how to drink from a sippy cup. It’s amazing.
What I really have come to appreciate with Alice is how unknown all of our futures are. I am sure you can relate. One day things seem like they couldn’t be better and one doctor’s appointment can turn your world upside down. It is universally true that people are so lucky to have their health. There are so many things in the world that money can buy, but it can’t buy health and happiness. It rolls off the tongue so easily when talking about the impending birth of a child – “I will be happy so long as it’s healthy.” I don’t think I ever knew what I was saying. I got caught up in the gender game, too – hoping for this ….wanting that. You never consider what that means until your baby isn’t healthy. The thought of saying goodbye to your child at a young age is excruciating and knowing it’s a real possibility will keep you up at night. I haven’t slept in 15 months and I mean that. My mind races. I have had panic attacks for the first time in my life. I have cried myself to sleep on a weekly basis. The hardest part is waiting. Waiting for bad news – waiting for good –-waiting for a life changing surgery – waiting for that phone call – waiting in a waiting room – waiting to say hello again – waiting to say goodbye. But what is so different for me – for you? None of us know what the future holds- nobody. Life is so precious and I am so happy that Alice reminded me of that. You have to LIVE. I suppose I thought I was living before, but now I know that I wasn’t because I didn’t appreciate life in the same way. And some days I am truly just surviving…others I am conquering the universe. I never said it was easy or glorious, but it is real life and I will never regret a single day I spent living for my girls and for myself. And I have a lot of help. One thing that Alice does best is bring people together. People have come out of the woodwork to write, call, cook, visit, support and love us. People I never imagined. One little being who cannot say more than two words did that! She resurrected friendships and connected me with moms all over the world on the NS Support Group on Facebook. If I could take all of the pain away from her – of course I would. If she didn’t have to be sick of course I would want that for her, but then she wouldn’t be Alice. She is just Alice. The girl has died twice in year and came home in 3 weeks each time. She shouldn’t be gaining weight and thriving, but she is. She wouldn’t be waiting for a heart at home on limited medication and no oxygen, but she is. She shouldn’t possibly be this happy, but she IS! Expect her to throw you the change-up. Every. Single. Time.
If you’re interested in contributing to the I Heart Alice Fund, donations can be made here: https://www.paypal.com/cgi-bin/webscr?cmd=_s-xclick&hosted_button_id=ELV5UF8UKL9KJ
If you’d prefer to send a check, it can be contributed to the I Heart Alice Mae Foundation through WesBanco.
If you’d like to reach out to The Sheppard family personally, contact me and I’ll provide you with their information.
Candice and her family have worked tirelessly to research, discuss and prepare for every possible scenario. I have had many conversations with her regarding Alice and what could happen down the road. Every single time we speak about it I am completely amazed. I walk away with the same exact thought. “I do not know how she does it.” She keeps a positive attitude and makes sacrifices every day to make sure that her children are in the best possible hands. She doesn’t let the circumstances change her outlook on life and she doesn’t ever seem to break. Sure, they’ve cried and grieved over the possibility that Alice might not make it, but I truly feel that their unconditional love is saving this child every single day. That’s why Alice Mae Sheppard is my favorite little miracle.