|Cadence “Cady” P – June 15, 1996- January 31, 2017
My sweet Cadence was born on Saturday, June 15, 1996 at 12:39 AM. It was a long, hard labor for both of us, and she had a little trouble transitioning. She was sent to WVU Children’s hospital in those first hours, for observation, and came back home to me (still recovering from the cesarean that got her here) a few days later. I did not get to hold this precious blue eyed child until she was 4 days old, but once I did, I vowed nothing would separate us again. Two weeks later, PA reared its ugly head and sent Cadence back to WVU for 32 days. These were harrowing times, no one caring for Cady had ever seen anything like it, and it was a very long time before we realized what we were dealing with. I had just graduated with dual degrees from college literally two weeks before Cady was born, and my strength just happened to be in organic chemistry. The learning curve caring for Cady and understanding her disease was steep, but my background allowed me to understand it deeply and assume her care. Over the years, despite PA, Cadence had a very full and happy life. I refused to let this monster steal a second of our lives and was determined to give Cady everything within my power to ensure a wonderful life for her. She loved to travel, she has literally been from the East Coast to the West Coast of the United States. She had more friends than can be counted. She laughed and played every waking minute of her life. She was so full of joy and laughter. Over the last weekend of January, Cadence’s belly began to hurt and she had trouble having bowel movements. Ileus was common for Cady and we’d weathered several in the last few years. She was managing Saturday, but something changed Sunday. Her pain became more distracting to her, and she asked to go to the hospital. She always knew when it was time. Once at the local ER, ileus was seen on xray and an NG tube was placed to relieve the pressure on her stomach. She was immediately relieved, and was once again smiling and happy. She was loaded into an ambulance for transport to WVU for observation. That was the last time I’d see her smile.
Two hours later, upon arrival at WVU, Cadence crashed and had to be rushed to emergency surgery. Surgeons discovered not an ileus, but a volvulous, where the bowel twists and cuts off the blood supply to the gut. Without blood, her gut began to die off, and surgeons removed 8-10 inches of bowel trying to save her. Over the next few hours, it was touch and go, but Cadence was so strong, she actually was managing to get better. In the end, however, the sepsis proved to be too much, she never regained her blood pressure, and that let to further death of her gut. We were given the option to stop these extraordinary measures and give her a peaceful passing. As I stood at the foot of Cady’s bed, rubbing her feet (because that’s all she ever wanted when she didn’t feel good), the rest of the world slipped away and it was once again just she and I. I closed my eyes as I held her feet and listened to her heart tell my heart, “No, Mommy. I just want to play. I don’t want to do this anymore.” And so, as the last act as her advocate, as her protector and mother, I gave her what she wanted, and returned her to her Creator. We stopped the drips and in just a few minutes, her heart stopped. At 3:35 pm on Tuesday, January 31, 2017, my sweet Cadence threw her arms around her Creator’s neck and laughed in utter joy. No more g-tubes, no more nausea, no more belly aches, no more fatigue. Cadence now only knows joy and my heart sings to know this. I never did anything so grand as to deserve the gift that God bestowed on me by giving her to me. She was a blessing from beginning to end, and continues to be so.
PAF Fall 2016 Newsletter
I’m not sure where to start with this update, it seems that Cady
has been around forever,that this life has been going on since the beginning of time, and yet I can’t believe I’m 43 and Cady has hit 20 years old.. Back in 1996 when she was born, I was told that she wouldn’t see two months, let alone 20 years. Those times, vivid as they are, seem so far away. And yet, how could two decades have passed?
For those of you new to the PAF (at least since our last update) I’ll just recap from the beginning. Cadence was born on June 15, 1996 in a small hospital here in northern WV. Everything seemed fine the first two weeks, until one day Cady would not wake up for me. I rushed her into the local ER and things only became more grave from there. She was sent out to WVU Children’s Hospital, but with a pretty clear warning that she wouldn’t make the hour and half journey. Well, she made it, but things continued to be pretty bad for a while. That summer we spent 32 days at Children’s, much of that time being on a tightrope of whether she’d make it to see another day. Hers was the first case of its kind they’d seen at WVU, and the medical staff there had pulled out all the stops to keep her alive. After 32 days, we knew what ballpark we were in, if not which disease we were dealing with, and she was stable enough to come home. During her time those 32 days, fibroblasts were grown and sent away for assay. The result came back after Cady was home about two weeks.. She had Propionic Acidemia. She was the first child ever born in WV to survive to diagnosis. To this day, 20 years later, she is still the only survivor born here.
Things were kinda woogy for the first two years, but she stabilized out and we led a pretty uneventful life until she was 12 years old. Then in the summer of 2008, she became ill and the rules seemed to change. We had no idea why she kept decompensating. It took what seemed to be forever figuring out how to treat these decompensations. During the winter of 2009, she spiraled so badly that it really looked like we’d lose her. She made it through that episode, but lost most of her hearing to the wild electrolyte swings and crazy blood chemistry. Finally, her GI specialist got her started on a flagyl regimen and that seemed to help. It didn’t stop the decompensations, but it did seem to fight them back a little.
In 2012, Cady became the Children’s Miracle Network Champion Child representing the state of West Virginia. It was a busy year, with many ups and downs. That was the year she turned 16, and also the time her doctors decided to look at her growth. They did a bone age study and at 16 years old, Cady’s bones were aged at 144 months (exactly 12 years). Her plates hadn’t closed yet, and there was some discussion about putting her on growth hormone to get more growth out of her before they fused. We tried it, and she absolutely flourished. The reaction was immediate and dramatic. Her appetite increased, she had more energy, better muscle tone.. it seemed growth hormone was a wonder drug for her. But she took to it a little too well and it swelled her brain in a condition called pseudotumor cerebrii. The pressure in her skull was crushing her optic nerve, threatening to blind her. I couldn’t stand the idea of that.. this disease was picking my baby apart, first her hearing, now her sight. We stopped the growth hormone and put her in the ICU. She had to be started on Diamox, which is a cerebral diuretic. The problem with Diamox is that it causes hyperammonemia and bicarb wasting at the level of the kidneys (you all know what that means.. high ammonia and low pH. Yay, just what we need!) It was a dance with the devil, balancing the need to quickly get the swelling down in her brain against causing a metabolic crisis. Fortunately, it was a dance that we won. No more growth hormone for Cady. Ever. But she kept her sight intact and didn’t suffer much more than a metabolic hiccup.
That same autumn, she won Homecoming Princess, voted by her sophomore class. Her classmates had grown up with her since the time they were all four years old. They are very fond and very protective of Cady. She won by unanimous decision.
The last few years, up until this past late Spring, Cady continued to be unstable. I tried different things with her diet, and that would work for a while, but we’d always end up back at square one. We began alternating flagyl therapy with augmentin, which also seemed to help. I was never a fan of giving her flagyl all the time, due to it’s black box warning and the fact that I’d like to figure out WHY she’s decompensating and stop it instead of just trying to treat it all the time. It appears I’ve finally hit on the the right combination, for Cady, at least. Cady had been orally fed for years, but in light of the fact that she was on the pump with her decompensations about as much as she was off, I decided to put her on the pump exclusively and give her system a rest. I also could find nothing but unproven theories regarding what was going on in her gut, causing these cascades so frequently, but what I did find was that her symptoms seemed to be more consistent with imbalance of gut flora versus over growth. I changed the supplement regimen that she’d been on for years and began using ID Life, which uses a specialized, individualized health assessment to generate a personalized recommendation. There were probiotics in her recommendation, something that her doctors had not wanted her on in the past. These supplements are also pharmaceutical grade, unlike the unregulated, unverified supplements on store shelves everywhere. So, I took the plunge and started her on the new supplementation. All I can say is, WOW. She is stable again, happy, growing like a weed and the healthiest she’s been in a decade.
She also attended prom this year, her Senior year. She was once again the darling of her classmates’ eye, as they voted her Prom Queen. A couple of weeks later, and in defiance of every odd levied against her when she was two weeks old, she graduated high school.. with honors.
I can not convey how very proud I am of her, of how blessed I feel to have walked this journey with her, nor how humbled I am that I was chosen to be this very special child’s mother. She makes me proud every single day. – Leslie