This is an offshoot of another thread that strayed far off its path.
I will be posting questions that spring from situations I see on my quaternary level PICU to test your knowledge of critical care topics that apply to children. As we know, children are not small adults and the reasons they are admitted to ICU are very different from those of adults. For example, PICUs see virtually NO life-style related comorbidities such as COPD from a 2 pack-per-day cigarette habit, cardiovascular disease from a 10 Big-Mac-per-week habit or end-stage cirrhosis from a 6-drink-per-day habit. Congenital anomalies, trauma (accidental and non-accidental), metabolic disorders, ingestions and respiratory infections are our biggest offenders.
Question #1:
Name the 4 main treatments for JET (junctional ectopic tachycardia).
It's not cheating EK! The UAC--if you're shooting for a high-lying--should have the tip between T6 and T9. The UVC just needs to be above the diaphraghm. (This helps you confirm that you're not in the liver). And EK, I learned just the opposite. The UAC is the one that swoops downward and then back up on x-ray, following the umbilical artery. You can check out this link and look at Cases 5 and 11 to see a nice example of what we mean.
Specializes in NICU, PICU, PCVICU and peds oncology.
Sweet! Next kiddie I have with umbilical lines, I'm scoping films!!!
I'll field the next question, but I have a few things to do right away so check back in a few hours for the next puzzle. (Unless somebody beats me to it!)
It's not cheating EK! The UAC--if you're shooting for a high-lying--should have the tip between T6 and T9. The UVC just needs to be above the diaphraghm. (This helps you confirm that you're not in the liver). And EK, I learned just the opposite. The UAC is the one that swoops downward and then back up on x-ray, following the umbilical artery. You can check out this link and look at Cases 5 and 11 to see a nice example of what we mean.
I'm taking STABLE tomorrow so I actually knew that last one!! Sorry I don't have any scenarios to add. If I can think of anything from the adult ICU that would be applicable, I'll add it!
Specializes in NICU, PICU, PCVICU and peds oncology.
Okay, sorry for the delay folks... I've been working on a Powerpoint on bullying in the workplace and have been a bit busy...
Your patient is an 8 year old boy who has been on your unit for 10 days receiving treatment for severe H1N1 pneumonia. This child has been critically ill and appears not to respond to aggressive treatment for MODS. He continues to run a temperature around 39C that is unresponsive to acetaminophen and ibuprofen. His CBC reveals hemoglobin 7.8, hematocrit 0.23, white count 1.7 with decreases across all cell lines, and platelet count 23. Other lab results include fibrinogen 0.9, aPTT 78 sec, INR 3.1, bilirubin 64 and serum ferritin 11,300 mcg/L. Physical exam reveals mild jaundice, multiple ecchymoses, splenomegaly, a waxy, scaly rash along his hairline, generalized lymphadenopathy and decreased LOC.
!. What other diagnostic tests can you anticipate?
2. What is the diagnosis?
3. How will this be treated?
4. Any guess as to prognosis?
This is a tough one, but we've seen it in several patients in the last 5 years and it seems to be becoming more frequent as time goes by.
I would think we might get an abdominal ultrasound or CT to evaluate the liver and spleen. And given the pancytopenia, perhaps a bone marrow biopsy? I would think we would want some liver labs, BUN, and creatinine.
I have an idea here, actually. Sparked by a patient I saw in the PICU during nursing school. Is it valid to guess if I might have had to use Google to remember the name? And to see if the clinical picture really fit like I remembered it? :)
Specializes in NICU, PICU, PCVICU and peds oncology.
Abdominal ultrasound shows non-specific enlargement of both liver and spleen. Liver labs are all moderately abnormal although bilirubin has now climbed to 147, BUN and creatinine are mildly elevated but not significantly so. Bone marrow biopsy shows increased macrophage activity.
Nobody said you guys couldn't look things up... As far as I'm concerned it's far better to know where to find pertinent information than to try and store it all in your head!
Specializes in NICU, PICU, PCVICU and peds oncology.
Okay, so you've got the other diagnostic tests and the diagnosis, sort of... it's post-viral secondary hemophagocytic lymphohistiocytosis. What about treatment and prognosis? Keep going...
We've seen at least four cases of this in the past three years, and it's a very rare disease. After the first case was confirmed, it increased our index of suspicion for it and now we're catching it earlier, which is a good thing.
Specializes in NICU, PICU, PCVICU and peds oncology.
No takers... hmm...
Treatment is based on risk of mortality (which is often very high). High risk patients will be treated with etoposide (VP-16) and corticosteroids. Low risk patients will get cyclosporine A (either Sandimmune or Neoral) and/or corticosteroids. If the onset of the disease is caught within hours (and let's face it, the odds of that are pretty slim) some patients will recover following IVIG administration. Some patients proceed to bone marrow transplantation.
Overall prognosis is not great, with a mean 5 year survival rate of about 22% in the chemotherapy-only group and 66% for those receiving BMT. The success or failure of allogenic BMT is the biggest prognostic indicator. Untreated it is invariably fatal within 2-6 months. The problem is that the disease is usually identified late in the course and by then it's too late. Patients with this are at extremely high risk of death early on. We've had one survivor so far out of the four I can think of, and it's still early days there.
My son was diagnosed with a related disease, then called histiocytosis X and now known as Langerhans cell histiocytosis, when he was 26 months old; he had been symptomatic for about a year at diagnosis and was by then at Stage IV. He was treated with vinblastine and corticosteroids, methotrexate, more vinblastine, radiation, etoposide, more vinblastine, alfa interferon and finally, cyclosporine A. He was a pioneer guinea pig in the current treatment of the histiocytoses and has been disease-free (touch wood!!) since 1992... the year I entered nursing school.
guess I've still got it backwards
littleneoRN
459 Posts
It's not cheating EK! The UAC--if you're shooting for a high-lying--should have the tip between T6 and T9. The UVC just needs to be above the diaphraghm. (This helps you confirm that you're not in the liver). And EK, I learned just the opposite. The UAC is the one that swoops downward and then back up on x-ray, following the umbilical artery. You can check out this link and look at Cases 5 and 11 to see a nice example of what we mean.
http://www.hawaii.edu/medicine/pediatrics/neoxray/neoxray.html
The rule of thumb for a UAC is that insertion depth is approximately the baby's weight in kg x 5 + 9.
Who's got the next question? :)