Liver Transplant
Patient Presentation/Diagnosis:
In the first day of life, full-term baby girl K.B. was admitted to the NICU with the following symptoms: seizures, hypoglycemia, lethargy, poor PO feeding and vomiting, temperature and respiratory instability. A myriad of tests were performed, and at two days of life, K.B. was diagnosed with a rare genetic defect causing a urea cycle disorder. The baby girl was unable to process nitrogen properly leading to a rise in nitrogen levels and a consequent buildup of ammonia in her bloodstream and around her brain. She was placed on the national liver transplant list immediately, but a matching organ was not becoming available. Once placed on the transplant list, K.B. and her parents waited for the organ. The parents reported subtle changes that indicated a rise in her ammonia – she would nap longer, or be fussy. The parents decided they would not be able to live with themselves if something happened to K.B. while waiting for an anonymous donation – the father agreed to donate a part of his liver. The transplant was successful and K.B. was admitted to the PICU postoperatively.
Treatment:
While in the PICU, the patient was intubated, paralyzed, sedated and closely monitored for hemodynamic instability and postoperative complications including bleeding and infection. Immediate critical lab results: PLTs:29, PT:21.4, PTT: 66.4, INR: 1.9, Fibrinogen: 123, Total Bili: 6, Direct Bili: 0.6, AST: 701, ALT: 1024, Phos: 5.8, serum amylase: 17, serum lipase: 37, serum ammonia: 100, GGT: 37. Pt was placed on a heparin and insulin drip that were titrated according to lab values. Transplant labs were sent every 4 hours for the first two days, liver ultrasounds performed daily, and patient remained intubated for several weeks due to fluid overload. The patient also required frequent blood transfusions and albumin and Lasix® for post-operative edema. Anti-rejection medications were started immediately, along with a myriad of anti-infective medications per protocol.
Outcome:
Pt has had a complicated course including a hepatic artery bleed and consequent thrombosis formation and biliary obstruction/cholangitis (AST: 2,715, ALT: 4,070, LDH: 3148 before thrombectomy was performed). She had a thrombectomy and biliary drain placed to deal with the complications. K.B. continues to come in and out of the hospital based on lab results from her outpatient transplant appointments. There is a chance that the patient will eventually need a new liver due to these complications along with signs of chronic rejection.