Ok… here’s the drawback of doing locum work as a generalist coming after 14 years of specialty focus practice: I may see things I haven’t seen since I was fellow! Like this case below… enjoy my stress on call! Also, great news! I am close to signing a new contract with a new hospital system to be Director of Cutaneous/Connective Malignancy and Solid Tumor Cellular Therapy. I will announce once this is official!
The Unexpected Call
It was a typical evening. I had just finished at the gym and was heading home when my phone rang. The ER physician was on the other end, telling me about a young girl with severe Hemophilia A, who had just arrived with acute appendicitis and needed urgent surgery. The surgeon was already on board, but they needed a hematologist to manage the bleeding risks.
Under normal circumstances, hematology calls are rare during a weeknight, and certainly, an urgent hemophilia case needing immediate intervention is even rarer. And you should know, it has been at least 14 years since I last managed a severe hemophilia patient during my fellowship, and now, I was being asked to guide an acute case with multiple moving parts at a new hospital setting!
Buying Time to Get It Right
I could have given recommendations over the phone, but I knew better. Hemophilia A, especially in the setting of surgery, is nuanced. What product should we use? What factor levels does he need pre-op? What’s his post-op plan? What if he has inhibitors which would make bleeding complications a much higher surgical risk? Additionally, with evolving guidelines and new therapies emerging, staying updated on optimal factor replacement strategies, extended half-life products, and potential gene therapy advancements is crucial. Understanding how these innovations integrate into perioperative care can significantly impact patient outcomes.
The patient was on Hemlibra (emicizumab) for prophylaxis, and he had Factor VIII at home, which he hadn’t taken in over a year after starting Hemlibra… cool drug! With bleeding disorders, the devil is in the details, and the risk of clotting complications with newer treatments only adds to the complexity. I hadn’t even realized before this case that emicizumab, when combined with certain blood products like activated prothrombin complex concentrates (aPCCs), carries a significant risk of thrombotic events. Additionally, over-correcting factor replacement could also lead to thrombotic complications, which is counterintuitive when treating a bleeding disorder. These nuances were an eye-opener for me, reinforcing the importance of knowing the latest guidelines and potential pitfalls in hemophilia management. I decided to drive in.
The ER Visit: A Rare Sight
When I arrived, I was met with an unusual reaction—people stopped and stared. The ER physician was almost giddy, telling the patient and his mother, “This is your lucky night—you get to meet a hematologist in person. I’ve been doing this for 20 years, and I’ve never had a hematologist come to the ER.”
I found that both funny and slightly concerning. In our field, many decisions can be made remotely, but there are times when seeing the patient, evaluating the hospital’s resources, and working directly with the team matters… specifically the pharmacist in this case as I wasn’t sure what products we had in place for these situations.
Getting the Right Plan in Place
After speaking with the patient’s father, the ER team, the surgeon, and the pharmacist, we decided on a plan:
- Loading him with Factor VIII to bring her levels to a safe range for surgery
- Monitoring for inhibitors (which could make Factor VIII replacement ineffective)
- Careful post-op factor replacement to prevent delayed bleeding
- Coordinating with his primary hematologist at the University of Colorado for continuity of care
The surgery went smoothly. By the next day, we had a clear post-op factor infusion plan with the help from our friends at the University of Colorado and the patient was recovering well, ready to be discharged within 24 hours.
Lessons from the Case
1. When in Doubt, Show Up
It would have been easy to give orders from home, but I would NOT have slept well knowing I might have missed a critical detail. Seeing the patient, reviewing the available factor products, and working directly with the ER and surgery teams made a real difference. Working closely with the pharmacist was especially valuable in navigating factor replacement and ensuring we used the safest approach. Additionally, discussing the plan verbally with inpatient nurses was crucial, as this was not their typical post-op patient, and ensuring everyone was on the same page minimized the risk of errors in factor dosing and monitoring. These collaborative efforts reinforced the importance of a well-rounded team approach in complex hematology cases.
2. Not Everything Is Textbook
Hemophilia management has changed significantly with newer agents like Hemlibra. While it reduces bleeding events, it doesn’t replace Factor VIII, especially for surgical settings. Understanding when and how to supplement factor levels is crucial. One particularly counterintuitive aspect is the real thrombotic risk associated with Hemlibra when used in combination with certain blood products, such as FEIBA (aPCCs), versus the safer alternative of activated Factor VII in the presence of an inhibitor. Recognizing these nuances is essential to avoid serious complications while ensuring effective perioperative hemostasis.
3. A Team Approach Matters
The ER team, surgeon, pharmacy, and I all played essential roles. The patient and her father felt reassured seeing that we were all actively and cohesively involved in his care. That alone can impact outcomes in a big way.
4. Learning Never Stops
Though I specialize in oncology, moments like these push me outside my comfort zone and remind me that staying engaged in different areas such as hematology matters. I reached out to old colleagues for input, did a deep dive into current literature, and came away from the case having learned something new. These moments are truly enjoyable!
Final Thoughts
99% of the time, being on call is quiet, but then there’s that 1%—the moments that challenge you, force you to adapt, and remind you why medicine is never just routine. This case reinforced the importance of being present, thinking critically, and never losing the willingness to learn.
Disclaimer:
The clinical cases and stories shared on this blog are intended for educational and informational purposes only. All patient information has been de-identified in accordance with HIPAA regulations, and identifying details may have been modified, combined, or fictionalized to protect patient privacy. Any resemblance to actual persons, living or deceased, is purely coincidental. The views expressed are those of the author and do not represent the views of any employer, institution, or healthcare system.
About the author
Dr. Sajeve Thomas is a distinguished medical professional and a compassionate guide in the field of oncology. With over a decade of dedicated experience as a board-certified medical oncologist/internal medicine specialist, Dr. Thomas has become a trusted expert in the treatment of melanoma, sarcoma, and gastrointestinal conditions. He brings a wealth of expertise to the complex and challenging world of oncology.
He is a great doctor who had managed my care when I was first diagnosed with mpnst (malignant peripheral nerve sheeth tumor) cancer of the soft nerve tissue very rare only one person a year gets it. I was very sad to learn he left but happy that he has this new adventure ahead
Amazed and happy to see humanity still exists, I glad you are a part of our family…Keep doing good…God bless.