OB Case

[milrisome]

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Here's a recent OB case I ran into...let's see what everyone thinks.

23 year old, primip, full term, with known history of both von Willebrand's syndrome with significantly low vWF, and Ehlers-Danlos. To demonstrate, she grabs a piece of skin and stretches it 5 inches away from her body. No other significant findings on H&P. She requests labor analgesia. What do you do?

Also, what if she needs a c-section?

 

[urgewrx]

You could discuss pros and cons of an epidural in this pt at length(pregnant hyper coagulable vs vWF deificiency), order labs like PT PTT, TEG, factor level, bleeding time.... give DDAVP (if it's one of the responsive ones) or even transfuse cryo empirically..... BUT the botton line is ANALGESIA is an ELECTIVE procedure.

For me it is a pretty easy scenario "Tough sht lady, you are going to have a good ol' painful delivery, just like most of the women in the rest of the world." Try some IV stuff, maybe even try a PCA but THAT'S IT.

Tube down the throat for c/s. Difficult airway too?> Awake FOB

Bleeding to much?> transfuse as necessary.

 

[Noyac]

Here's a recent OB case I ran into...let's see what everyone thinks.

23 year old, primip, full term, with known history of both von Willebrand's syndrome with significantly low vWF, and Ehlers-Danlos. To demonstrate, she grabs a piece of skin and stretches it 5 inches away from her body. No other significant findings on H&P. She requests labor analgesia. What do you do?

Also, what if she needs a c-section?

I would probably not put an epidural in her for labor. I would however do a spinal if she got desmopressin prior to surgery to control surgical bleeding.

 

[VentdependenT]

If you got time buzz heme and have em come by and check a VWFactor level or percentage or whatever (cant recall). DDAVP for sure. Have cryo ready or recomb factor 8 (if available).

Since she may get products have blood bank make sure they get Rh - stuff if they can (remember cryo is pooled and I'm not sure about alloimmunization with the stuff since its spun down).

If the levels of VWF come back normal, and the patient has no bleeding hx, I would give a spinal after DDAVP like Noyac.

Otherwise only other stuff I'd be concerned about is aortic and mitral valve pathology. With a normal physical I suppose I wouldn't be too worried.

 

[Planktonmd]

This woman has 2 problems that complicate neuraxial anesthesia:
1- Ehlers- Danlos syndrome: Causes significant fragility of blood vessels and might precipitate bleeding.
2- Von willebrand: causing platelet dysfunction.

So:
My answer would be: Sorry, No Spinal and no epidural.

 

[Noyac]

This woman has 2 problems that complicate neuraxial anesthesia:
1- Ehlers- Danlos syndrome: Causes significant fragility of blood vessels and might precipitate bleeding.
2- Von willebrand: causing platelet dysfunction.

So:
My answer would be: Sorry, No Spinal and no epidural.

Nobody can fault that answer.

 

[milrisome]

This woman has 2 problems that complicate neuraxial anesthesia:
1- Ehlers- Danlos syndrome: Causes significant fragility of blood vessels and might precipitate bleeding.
2- Von willebrand: causing platelet dysfunction.

So:
My answer would be: Sorry, No Spinal and no epidural.

I think this is the right way to handle it too. Turns out OB was worried about bleeding too; Heme had seen the pt and was giving DDAVP. They thought this would bring the vWF levels back to normal temporarily (I think 6 hours). But with the combo of friable blood vessels and potential coagulopathy I took a pass on the epidural. Stoleting recommends against regional for pts with Ehlers-Danlos.

For C/S also I think no regional, but caution with the airway as it's more likely to bleed. Of course get T&C for RBCs and cryo. Interestingly, Ehlers-Danlos has increased incidence of pneumo, so watch out for that.

Good call Vent, check for MR and prophylax if necessary.

👍

 

[milrisome]

Anybody tried Remi PCA for this type of situation?

 

[Noyac]

Anybody tried Remi PCA for this type of situation?

WHAT?😱

 

[milrisome]

WHAT?😱

Sure! check out this reference: A&A 2005;100:233–8

They had good analgesia, pain scores of 3/10, minimal fetal depression, no problems with apgar scores.

It makes sense, doesn't it? A rapid acting narcotic, titrated to pain, that goes away almost as soon as you turn it off? It's got to be better than loading someone with a long acting narcotic that builds up in the fetus.

Having said that, I have no experience with it.

 

[Noyac]

Sure! check out this reference: A&A 2005;100:233–8

They had good analgesia, pain scores of 3/10, minimal fetal depression, no problems with apgar scores.

It makes sense, doesn't it? A rapid acting narcotic, titrated to pain, that goes away almost as soon as you turn it off? It's got to be better than loading someone with a long acting narcotic that builds up in the fetus.

Having said that, I have no experience with it.

I'd love it if they started using something that got them to stop calling me for epidurals.

 

[milrisome]

I'd love it if they started using something that got them to stop calling me for epidurals.

Yeah, especially at 3 am. Notice in that study only 10% of women requested epidural while getting remi pca...maybe epidurals for labor are a thing of the past...

 

[dhb]

Anybody tried Remi PCA for this type of situation?

We have a remi protocol but i haven't seen anybody use it yet

 

[milrisome]

We have a remi protocol but i haven't seen anybody use it yet

What's your protocol (if you don't mind me asking)?

 

[dhb]

What's your protocol (if you don't mind me asking)?

Well i looked for it but i couldn't find it and since i've never used it... i'll post it once i get a hand on it