November 13: Diagnostics

2:00 pm:  "Infectious Disease" is a group of specialty doctors just added to Ava's resume. They interviewed us about what lead to this event and about who or what Ava was around at home. In colaboration with Ava's regular attendings, they have ordered a bunch of new tests to try and narrow the field and rule things out. They've sent a blood sample for flow cytometry to the U of U lab to screen for cancer. They did a pro-calcitonin test which was very high- indicating a lot of inflammation consistant with this sepsis. They took blood cultures from every line in her body to culture again and to also look for fungus infection. Sepsis resulting from fungus is rare, but possible. Her white blood cell count has risen for the last three days indicating a worsening infection. She will have an abdominal ultrasound today to look for possible sources of inection in the abdomen, perhaps an absess. An absess may need to be drained. A CT scan would be better to look for infection in the abdomen but she cannot be transported with all this equipment right now. She is on two broad spectrum antibiotics so a rising white cell count raises some questions. 

Her heart meds have been turned up to support her blood pressure. I hope we'll be able to wean them again today. Her ventillator settings have been weaned a little, though, which is good. We really need to get the vent weaned enough to switch her to a conventional ventilator which will take some of the pressure off her heart. Actually any time we are able to lower vent settings, it helps take some presure off the heart. It has more to do with the pressures rather than the type of machine. 

LDH and Bilirubin are also high. 

6:20 pm 

Ava is requiring a little more support for blood pressure. The dose of heart meds she is getting is now quite high for epinephrine and vasopressin.

The ultrasound shows that Ava had a spleenic infarction. So basically her spleen died in this event of the last week +. As long as it doesn't cause problems it won't have to be surgically removed. She will be at increased risk for infections for the rest of her life. You can live without a spleen. You are just immunocompromised. Hopefully this helps explain why the white blood cell count is so high at 34. 

Ava's LDL is high and haptoglobin is low and platelets are low- constantly having to be infused. Based on these lab values and symptoms docs are now suggesting that she may have a rare genetic disease called atypical hemolytic-uremic syndrome (atypical HUS). It can be triggered by infection. It affects 1 in  500,000 people. Do not confuse it with typical HUS.  More info here. http://ghr.nlm.nih.gov/condition/atypical-hemolytic-uremic-syndrome

http://www.ncbi.nlm.nih.gov/books/NBK1367/

It will take many weeks to get genetic testing back for this, so the doctors will collaborate in the morning and may decide to start therapy just based on symptoms and lab values.   This rare genetic disease is not "why" she got sick, I think, it is just activated by the sickness. 

The hits just keep comin'.   I'm not really a fan of "today"

 She opened her eyes for Shaun and I. She looked very distressed.

 She is somewhat grabbing at things, mostly with her right hand. The left hand is kind of weighed down by ventilator tubing. 

Grandma VAn Sickle and Ivy hanging out in the Ronald McDonald Room on the 3rd floor. She said her first word yesterday- the little stinker- "dadda". She's probably going to learn to crawl and walk in this room. Good thing they like us.. I think.

 Some shiny glittery pumpkins at the Ronald McDonald house (not to be confused with the Ronald McDonald Room at the hospital). ..Note..I don't normally put my kids on public floors, but she was headed up for a bath anyway.