November 9th Scarlett was admitted to the ER. She had been vomiting off and on for about 10 days prior. She would throw up a couple times then be fine for a day or two then throw up again. We thought she had turned the corner that afternoon when she had an episode of extreme pain to the point she looked like she was having a seizure. We took her to the ER along with every other parent in Birmingham who had just finished watching the Alabama / LSU game and we would wait. Once we were back they started an iv and found that she had blood in her urine. They assumed that she may have a kidney stone and ordered a CT Scan. Shortly after they read the results the doctor informed us “it’s her pancreas and we need another CT or MRI with Contrast Dye”.13 hours in the ER they finally said it was not her kidneys they couldn’t figure out the blood in her urine and that she was being admitted and we would talk with an attending once she was in the room.
You could have knocked us over with a dust bunny when they came in and just laid it all out. Scarlett has acute on chronic pancreatitis. 2/3rds of her organ is dead and the last 1/3 has calcifications and we don’t know how long the remaining tissue will continue to live. She will be diabetic soon. She has a collection of fluid with necrotic matter in the fluid and a necrotic wall of tissue blocking the fluid and is at a higher risk for infection. The doctor explained they don’t see this here but would consult with UAB. They then launched into about 500 questions. I have to say the sensitivity training was lacking as Scarlett laid there trying to process everything they were saying.
Chronic pancreatitis in kids is extremely rare. It is primarly caused by a variety of genetic mutations but can also be caused by auto immune condition. Within the genetic type there are numerous gene mutations. We later discovered that she has a genetic mutation of the PRSS1 gene. This mutation causes a digestive enzyme that breaks down protein to activate in the pancreas rather than the small bowel. The pancreas is made of protein and the enzymes overtime break down the organ which produces it. This gene mutation is also one related to Pancreatic Cancer.
We now know that she was misdiagnosed with acute episodes for years. In January of 2018 she had a bad episode after a GI bug. While at Childrens they measured her lipase and amylase and said that likely the GI virus has made the pancreas very angry. They diagnosed her with acute pancreatitis. What they should have done from there is an ultrasound or some form of imaging. Five days later we were in a motor vehicle accident and it likely triggered events for the next 4 1/2 months. We were chasing symptoms of pain, vomiting, bile reflux and bile vomiting, back pain and weight loss. We were told time and time again it was a variety of gi issues, reflux or muscular. I asked numerous times if it was her pancreas but it was usually dismissed. We can go through health records all the way back to age four with visits to the ER, walk-in Urgent Clinics as well as our pediatricians with complaints of severe belly pain and vomiting and the same answers were given. This is very common with Chronic Pancreatitis children.
The GI Team at Children’s decided to refer us to Cincinnati Children’s Hospitals Pancreatic department. She was scheduled 12/16 for a packed week of appointments with their TPAIT team. It was a really tough visit. With every specialist we learned that the disease had progressed rapidly and she was worse than we realized. She had an ERCP on 12/23 which was considered to be a failure. The goal was to remove several stones that had accumulated in the pancreatic duct, implant a stent or two to alleviate some of the necrotic fluid on her pancreas. They couldn’t access the pancreatic duct due to the amount of inflammation and scar tissue. The stones were not accessible b/c there were so many and some embedded in the tissue. They implanted two stents which were not successful. The surgeon explained there was very little organ left and basically she was a mess.
Fast forward from 12/23 to 1/5 she had 1 good day and a lot of bad days. She has had daily pain, a visits to the ER and now her liver enzymes are off the charts. After daily calls and emails to our pediatrician and her team in Cincy they finally ran bloodwork and performed an ultrasound on Thursday. They called Friday evening after hours and told us we needed to get her back to Cincy within 24 hours. Kelly arrived with her Saturday where she was finally admitted. Her Enzymes and liver toxicity is higher than it was Thursday and they are proceeding with an MRCP today to determine if there is a blockage in the bile duct preventing drainage or if there is a viral component to or something.
In the meantime we have to get her liver well in order to get ready for the TPAIT Procedure in February. This is where they will remove her pancreas, gallbladder, appendix, spleen and part of her small intesting. They will take the remaining islet cells from the pancreas and transplant them into her liver (if the liver is healthy enough). This will require a 2 month stay and be a long healing process. I will try and break up additional information into smaller entries. https://care.cincinnatichildrens.org/pancreas/tpiat?gclid=EAIaIQobChMI-ML-x47t5gIVC18NCh3z1QI8EAAYASAAEgJ_RfD_BwE
Specifically please pray for wisdoms for her doctors as they try and determine the cause of her liver issues. Please pray for a clear diagnosis and treatment plan to heal the liver. Please pray for her physical and mental strength. Please pray for our family as a whole as we divide up and conquer to try and manage and support our entire family.
1/7/20 – Success! Dr. Lin was able to access the Bile duct, clear the blockage and found the opportunity to access a side pancreatic branch. He was able to implant two additional stents and even grab a couple small stones. Her enzymes have declined steadily and she is coming home.
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.