Hot Seat #63: 18 yo M with HbSS and Altered Mental Status
Posted on: October 14, 2015, by : Astrid Sarvis
Astrid Sarvis MD, Children’s National Health System
The Case
18 yo M HbSS, Protein S deficiency, Asthma and Chronic Headaches presenting with 2 days of worsening bilateral ankle pain. His symptoms began the day prior to arrival with no obvious triggering event. He was using 10mg oxycodone which helped, but the pain progressively worsened. He reported a pain score of 6-7/10 on initial assessment. He is otherwise reporting good PO without vomiting. No diarrhea. Good UOP. No fever, chest pain or breathing difficulty. Usually has pain crisis in his legs and back.
Pertinent history of acute chest and aplastic crisis several years ago. He currently has avascular necrosis of L hip and shoulder (likely needing surgery in the future).
ROS: Remainder as per HPI.
Additional PmHx: Chiari I malformation (stable). NO history of stroke. NO prior PICU Admissions.
FmHx: Noncontributory.
Physical Exam: VS: T 37.5 (oral)/ HR 82/ RR 18/ BP 132/62 / Sats 97% on RA
General: Alert and oriented. No distress. Well-mannered.
Skin: No rash. No appreciable jaundice.
Eyes: + Scleral icterus.
Ears, nose, mouth and throat: Oral mucosa moist. No appreciable jaundice. No pharyngeal erythema or exudate.
Gastrointestinal: Soft. Minimally tender to deep palpation on the R. No guarding or rebound. No appreciable HSM. Non-distended. Normal bowel sounds.
Back: No CVA tenderness.
Lymphatics: No lymphadenopathy
MSK: Pain in bilateral ankles limiting ambulation, FROM at ankles with moderate pain with passive and active movement. No swelling or redness. No bony tenderness. Otherwise with FROM and grossly normal strength throughout
Neuro: Awake alert and oriented x 4. No focal deficits.
Remainder of exam within normal limits.
You give morphine, toradol and NS bolus.
Labs results showed CBC: WBC 6.6/ Hb 9.2/ Hct 25/ plt 123; 58 seg/ 26 lymph, 9 mono, 1 eos; retic 13; BMP: Na 139/ K 4.0/ Cl 104/ HCO3 23/ BUN 10/ Crt 0.64/ Gluc 75.
The patient reports 5/10 pain after the 1st dose of morphine so the dose was repeated with almost complete resolution of ankle pain. However the nurse tells you the mom seems really worried about the patient’s “yellow eyes” and is requesting “liver tests.” LFTs are added to blood-in-lab.
You re-evaluate the patient 30 mins later and notice that the patient seems more confused, is randomly repeating phrases and intermittently not oriented to place and idea. The remainder of the patient’s neurologic exam including speech, vision and strength are normal. Mom then says that the patient developed “yellow eyes suddenly yesterday” which is the “main reason” she brought him in.
LFTs are still pending and the lab is backed up.
Questions for you:
Update:
LFTs return: Total bili 20, conjugated 12.7, AST 109, ALT 110, Alk phos 207, Alb 4.3, Tprotein 8.0.
How would you approach this case? Please share your opinions by clicking on “What do you think?” below.
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Direct hyperbili in sickle cell disease makes me think of sludge/cholestasis. but I am surprised that the AlkPhos is normal here. So I would definitely get more information including PT/PTT, GGT, abd US, direct coombs, NH3, lipase, CK, LDH, and urinalysis.
But then you have to decide if we can tie these things together (AMS, hyperbili, leg pain) or if they are true and unrelated. VOC and cholestasis are certainly common in sickle cell. Hard to ignore stroke here so I would absolutely get neuroimaging while waiting for more laboratory information….assuming we didn’t think his new symptoms were medication related (from morphine).
Most of the things I would worry about in this picture i would think would cause transaminase elevation, liver dysfunction, or at the least moderate abdominal pain, such as: iron overload from chronic transfusions, hepatitis, sclerosing cholangitis, cholecystitis, gall stone pancreatitis, etc. He certainly can get an acute hemolytic anemia but his Hb is normal, especially for a HbSS kid.
In the absence of other interesting labs and with a normal head CT, I would probably chalk this up to a VOC with routine GB sludging….as long as his mental status returns to normal. Actigall please. and some IVF. If this shows signs of progression to intrahepatic obstruction with liver dysfunction or hepatic crisis, that may be a sign to get that gall bladder out.
I’m sure Dewesh with tie this together in less than three notes. but I’m voting for true true and unrelated.
The only way I can tie together the acute mental status changes (presuming it’s not narcosis) with the liver dysfunction and h/o Protein S deficiency is Budd-Chiari syndrome, but this typically presents with abdominal pain and/or ascitis, which this patient did not have. I think the NH3 level is a good thing to check to assess if the mental status changes are secondary to liver failure. Protein S deficiency may also lead to thrombosis in the vasculature (typically dural sinuses) of the head, which could present with acute mental status changes. So, I’d make sure they do CT-angiography with the head CT.
With the expert comments above, I have little to add beyond
– If daytime, would consider a stat MRI. He’s older, seems cooperative though altered, and neuro/Hem may appreciate that more to get the best idea….Of course, if it’s Friday night at 0200, different story….
– I’d like to use this chance to remind folks that our pathways are also great teaching tools, and though we can often use them mindlessly, to me the point is to create a minimum standard of care, not follow them blindly. I’d make sure to go through with the resident or nurse how/why we are using the pathway (everyone always appreciates education – and you have enough understanding to share it) and when it’s limited: AMS, jaundice are not common so not addressed as opposed to not addressed so not important.
– I’d also talk to Hem/Neuro early on (like even before/as I order the neuroimaging). Neuro has been absolutely fabulous about helping us sort out strokes and plan the ideal imaging, evaluation and management (in my opinion).
Can’t wait to hear/see the results…..