We are so grateful for the vigilance of Lucy's medical team and in particular the GI fellow who thought to pursue this avenue. Dr. R admitted that he never thought to look at this because it is so rare. When talking about Lucy, rare is common in our world! It was Lucy's consistency in telling us where her belly hurt and her increased pain that lead this fellow to investigate further.
Yesterday, our day started off bright and early, ultrasound came to Lucy's room at 5:30am to do a scan of her liver, gallbladder, pancreas, and small intestine, talk about a sneak attack. Lucy hates having an ultrasound done, the combination of the element of surprise and the fentanyl patch made having the scan tolerable. That and the fact that she is one sick little girl who is unable to move on her own meant that she stayed relatively still. She couldn't roll away, which is her usual M.O. for trying to get out of an ultrasound. The ultrasound revealed that there were many areas of concern in Lucy's liver, bile ducts, and gallbladder. More specifically, Lucy's common bile duct was grossly enlarged, her gallbladder wall thickened with sediment and sludge, and her bile ducts in her liver were found to have blockages as well. There were two conflicting radiology reports that both concluded, however, that an MRCP was warranted to have a better look at things.
Last night Dr. R came and showed us the pictures from Lucy's MRI, her common bile duct looks like a funnel, wide at the top narrowing just below where the the gallbladder drains into the common bile duct. There is no visible obstruction, but something is causing the narrowing. This is a very unique and interesting finding in a kiddo Lucy's age, this just isn't something that is seen very often in pediatrics. What has caused this, we are not sure. In order to investigate the problem further Lucy is going to need to undergo a ERCP and have a stint placed in her common bile duct to help the bile duct drain into the small intestine, a procedure that is rarely performed in pediatrics, but rather more commonly in adults. Because of this Lucy is going to need to be transferred to Jefferson Hospital in Philadelphia where an adult gastroenterologist has agreed to take Lucy's case. He is "fascinated" with her case because it is not something that is seen everyday. He mentioned that there are a handful of cases in which patients with severe dysmotility of the intestinal track can also develop dysmotility of the biliary system. There is also a concern that this is a congenital abnormality, something that Lucy was born with, and overtime has developed into what it is now. We really won't know until after the ERCP.
After the ERCP, Lucy will be transferred back to DuPont for recovery. Our surgeon here at DuPont is going to have to decide if, when, and how he is going to perform a second surgery to remove her gallbladder as it is not healthy and full of slug and sediment that can drain into the common bile duct, thus causing the stint to become obstructed.
At the moment Lucy is one very sick little girl who is in a lot of pain! She most likely has colangitis, an infection of the common bile duct which explains her fevers. We started treating her with antibiotics again this morning. The fentanyl patch is working, but she is also requiring PRN (per requested need) doses of dilaudid in addition to the fentanyl, more today than yesterday. Her respiratory rate is low, so we need to be careful. Lucy's metabolic panel is a mess. Today she had yet another differential diagnosis added to her already long list of diagnoses, hypercalcemea. Despite taking the calcium out of her TPN for the past two days and increasing her fluid rate to try and flush the calcium out of her body, we have been unable to lower her calcium levels in her blood. An EKG was order and endocrinology was consulted today as we have begun the workup for this newest issue.
Now comes the very complicated task of arranging all of the details that need to be considered when doing a procedure like this for a girl as sick as Lucy, with a complex medical history. Jefferson is not a pediatric hospital and as such there are discussions being had between the doctors here at DuPont and the doctors up at Jeff, one of our biggest concerns surrounds anesthesia. We don't know when the procedure will take place, but we are hoping for sometime next week.
At the present time we are very concerned about our girl, she is not doing well. We are both relieved and worried to have an answer to her issues. Relieved that there is a treatment for what she has, and worried that she has to undergo this treatment.
This is a lot to process. We want to thank all of you for your thoughts and prayers, love and support. We could never do this alone!