Abbey has a CPA1 genetic mutation which causes her to have chronic pancreatitis. She has been selected by the Cincinnati Children’s Pancreatic team to have a TP-IAT on 8/1/23. This is a major surgery that is giving our family some relief after 2 years of uncertainty with Abbey’s health. Here is her story…

As an infant Abbey was what doctors called a happy spitter, meaning she would spit up after every feeding. We knew this wasn’t just a baby spitting up as she would have full evacuation after most meals. She was also the most challenging baby of the three always having “colic” and oh my, her cry was and still is the loudest cry most have ever heard. She would gain weight but not very fast and was always and still is very tiny for her age ranging between the 3rd-5th percentile. We also noticed that every time she would get a cold it would take forever for her to get better. We were in and out of our PCP (Primary Care Provider) office more times than we can count. Many of those times would result in just break down crying during the visit because we were at such a loss.

Around 10 months old she caught a virus and after going to our 4th visit to our doctor in a week, we decided to run labs and check her pancreatic levels. Our doctor didn’t think anything would come back but checked because we had just recently learned that our oldest daughter, Olivia, has 3 genetic mutations that cause chronic pancreatitis, SPINk1, CPA1, and CF. Lo and behold we get a call about 4 hours later from the doctor’s office telling us we need to go to the ER immediately for admission due to extremely high lipase levels. At 10 months old Abbey had her first hospital stay for pancreatitis. During this visit we were visited by so many doctors and students all in disbelief because “We have never seen an infant with pancreatitis.” Y’all that is never something a parent wants to hear from a person that they are entrusting with their care.

We also found out that Abbey has FPIES (Food Protein Induced Enterocolitis Syndrome), and her allergy is to poultry. Now when I say she has FPIES it is an assumed diagnosis as Abs has never been well enough for us to have a challenge study conducted. So, with the FPIES now on the table we took all forms of poultry out of hers and mom’s diet (breastfeeding). We started to see more weight gain and Abbey start to grow! We thought maybe she was turning a corner and we finally figured things out. We had genetic testing done through our GI (gastroenterologist) because of the pancreatitis diagnosis and Olivia’s mutations. When the tests came back our GI was kind of puzzled as Abbey only had one mutation CPA1. We were all very confused and didn’t know how this specific mutation was going to affect her in the future. We started tracking all of Abbey’s symptoms learning that every time Abs would get a virus with inflammatory markers in it, the inflammation would target her pancreas. She has a constant high lipase and amylase level which always freak out doctors when they see her labs come back. Abbey’s non-symptomatic levels average around 4-6 times the high on any scale she has been tested on. For most pancreatitis cases you are diagnosed when levels are 4x the max. Abbey therefore has been suffering in pain since she was born explaining all her symptoms before her diagnosis.

At the beginning of her journey, she was being admitted with flare ups once every 8-12 weeks. Fast forward to October 2022, things started to get more challenging and frequent. She caught parainfluenza which caused her to be admitted 3 times in 10 days, pancreatitis, sepsis, and pneumonia. This visit is when momma bear started to come out in a furry when all the student doctors were ignoring symptoms I was bringing up then sending us home resulting in us coming back 4 hours later and needing oxygen plus having 105+ temperatures. Needless-to-say after October we no longer see students, residents, or fellows, we only see the attending physician. After our October stay Abbey never seemed to get back to her true self. Our visits started every 4-6 weeks and were getting more severe with every visit. We added many specialists to her care team just to see if she had something we weren’t catching. Everything would always come back fine or inconclusive. We were at a complete loss and feeling defeated that there was nothing we could do to help Abbey. Finally, after a follow up GI visit we agreed to reach out to the Pancreatic Center at Cincinnati Children’s Hospital. They immediately replied saying they were interested in reviewing Abbey’s history to give a second opinion. We received a call two days later requesting intake information and requests to get her extensive medical history. We were told there were two options for the visit: a second opinion consult or a full TP-IAT (Total Pancreatectomy with Islet Auto Transplantation) evaluation. Since we were having to travel so far to start, we opted for the full TPIAT evaluation. Not thinking we were at that point, but since we were there might as well.

We traveled there at the end of February/beginning of March of 2023 and wow just wow! Everything and everyone were so different from what we are used to here. They knew what we were talking about, they could tell us all of Abbey’s symptoms without us having to tell them. They didn’t think we were crazy when we told them about Abbey’s everyday pain. We learned that Abbey’s condition is a very real and debilitating condition, and she will never grow out of it. In fact, it will just continue to get worse since the pancreas does not self-heal; it is just continuing to shrink in healthy cells, eventually killing it. Since Abbey’s condition is related to genetics the TPIAT will be something she, at some point, will have to have if we want her to have any hopes of a pain-free future. While in Cincinnati we also had an ERCP (Endoscopic Retrograde Cholangiopancreatography) where they placed a stent in one of her pancreatic ducts in hopes that we would be able to allow more flow in and out of the pancreas giving us more time between attacks and, hopefully, allow more time till a TPIAT would need to be done.

While back home and the stent in place Abbey was great! Complaining of little pain, eating ALL the food and getting back into the happy, funny, smart little girl she is. We removed the stent on the 14th after being in the maximum allowed time of 6 weeks with new hope. This hope came crashing down after just a few days. Abbey was hospitalized with her highest lipase levels yet (1480 on a scale of 0-60). With this, her Cincinnati team put her name back on the table for discussion. Before a decision could be made, she was hospitalized yet again, just 7 days after being discharged. Her lipase levels hit a personal high of 1540, and she spent 3 days on morphine and oxy to get through the pain. Abbey was moved to a ‘yes’ for the surgery and the planning mode started. She had an NJ feeding tube placed on Thursday 5/4 and it would need to slow feed into her small intestine until her surgery in August.

We have teamed up with COTA as we feel they have our family’s best interest in mind. COTA is a nonprofit organization and has been fundraising specifically for transplant children for many years. Our Community Coordinator is Ramia Whitecotton. We are so thankful to have her on our side, helping us raise funds for COTA in honor of Unique Abbey to assist with transplant-related expenses.

The Children’s Organ Transplant Association (COTA) helps children and young adults who need a life-saving transplant by providing fundraising assistance and family support. COTA is the nation’s only fundraising organization solely dedicated to raising life-saving dollars in honor of transplant-needy children and young adults. 100% of each contribution made to COTA in honor of our patients helps meet transplant-related expenses. COTA’s services are free to our families, and gifts to COTA are tax deductible to the fullest extent of the law.