Hello all - 

I can't seem to upload my Word document here (if someone knows how, please let me know!) so here is the protocol I use. I'd rather upload that because it's much easier to read:

SCLS Protocol for Jeffrey Zielinski

 

WARNING:

Jeffrey is a good judge of his symptoms and is educated about his disease.

Normal Values:

HCT: 38-46

BP:  110-120/70-80

PULSE: 60-90

Plasma Volume (at 45ml/kg body weight) = 4500ml

 

DX OF IMPENDING SHOCK OR SHOCK

Elevated BP = impending shock

HCT: usually >50

BP:  90-110/60-70

PULSE: 110-140, persistently. Also decreased UOP, despite approx 1L of PO fluids at home.

 

Low or absent BP=shock

HCT>70

SBP<90

PULSE: >130 and thready

 

CLINICAL SIGNS:

Initially Jeff may be alert, anxious, may have cold, clammy extremities.  He will often complain of arm/leg fatigue, lower lumbar pain, increased nasal congestion and facial/neck fullness. May lead to progressive anxiety, leading to confusion, and livido reticularis (mottling) of his skin in severe leaks only.

 

IMMEDIATE MANAGEMENT

1. Establish secure IV lines in EACH arm. Use a #18 or larger angiocath. Secure with armbands and tape.

2. DO NOT use CVP lines unless you suspect volume overload. Otherwise, it is inaccurate.

3. If pt confused and unable to use urinal, place Foley catheter (ask patient first).

4. Obtain blood samples PRIOR to administration of albumin.

            - PT/PTT (blue top)

            - Electrolytes, BUN, Cr, CPK, LDH, LFT's (red top)

            - Venous hematocrit STAT (purple top), first calculated, then manual.

            - CBC, albumin level. Albumin will trend downwards during leak; will slowly normalize after administration of albumin.

5. Keep IVF (NS) at 150-200cc/hr ONLY UNTIL ALBUMIN IS AVAILABLE.  Excessive fluid administration with saline will lead to muscle engorgement and possible compartment syndrome.

6. Increase rate of IVF (wide open) if pt with SBP <80 only if mental status changes are present and no albumin available.

7. He should be placed on telemetry for his entire length of stay.

SPECIFIC TREATMENT: RE-EXPANSION OF INTRAVASCULAR VOLUME WITH COLLOID OF HIGH ONCOTIC PRESSURE

Initial Phase:  Rapid restoration of normal plasma volume

Total Time:  approx 2 hrs for total infusion

DO NOT leave patient if pt in shock

1. Re-expand blood volume with albumin only - discontinue saline.

- 2 units (1000 ml total, 1 unit = 500 ml) 5% albumin in one arm

- 4 units (200 ml total, 1 unit = 50 ml) of 25% albumin (SPA) in the other arm.

-The RATE should be approx 1 UNIT/HR for 5% albumin and 2 UNITS/HR for 25% albumin in each arm SIMULTANEOUSLY (approx 500cc/hr into one arm and 100cc/hr into the other arm)

2. Warm IV bottles if possible to prevent hypothermia and chilling from rapid infusion. Albumin can be infused at room temperature.

3. Monitor serial response by SERIAL VENOUS HEMATOCRITS.

- send first post-Rx sample immediately after combined albumin infusion (6 total units).

4.  Excessive re-hdyration may lead to pulmonary edema. Follow O2 saturation closely.  Jeff has normal EF by ECHO on 12/19/01.

 

Hematocrit post-treatment therapy recommendations:

If HCT 20-50 then DISCONTINUE ALBUMIN and recheck serial hct, albumin q4hrs and supportive mgmt                             

IF HCT 50-60, speak to patient about condition, status of his UOP and back pain then recheck HCT ONE HOUR later. If >55, and SBP <90, administer: 1 unit of 5% (500 ml) and 2 units of 25% (100 ml total) over one hour. If <55, may consider following clinically.

IF HCT >60 then repeat initial mgmt: 2 units (1000ml) 5% and 4 units (200ml) 25% in each arm over 2 hrs. Recheck labs as per protocol after combined infusion of albumin.

Thanks for posting this Jeff, and in its own dedicated Discussion Forum space!  I'm glad to know that this albumin-replacement regimen has worked for you, and that you've let Dr. Druey know about it. I wish that merely being injected with replacement albumin during an episode of SCLS worked for most of us too!

And just so that everybody realizes, this site was purposely designed such that nobody can upload anything -- or otherwise we would look like a rare-disease version of Facebook, with an abundance of photos of people's favorite pets, religious images, cartoons, videos, political statements, documents protected by copyrights, commercials, pornography and spam.

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What all of us can do to exchange files, instead, is message the RareShare member you want to get something from, or send something to, letting them know what your email address is, so that they can upload whatever to an email message and send it to you that way, or else you can send them something once they reply to your email with their email address.  You don't need to create or use a Discussion Forum topic for that purpose; just click on the green "[envelope] Message" button and off you go!

Thanks again for sharing this, Jeff.

Ah - understood, Arturo. No problem at all and I can certainly send the word doc to those that request it - would just need an email address if so. 

Please know that this protocol is NOT a replacement for IVIG - I only use it when I experience an episode and I am headed to the ED. It is a stopgap ONLY but it was all I had for 18 years. It may not work for all but if it prevents worse outcomes for some - or even 1 - then that's great. 
FYI I am in Rochester, NY but the protocol was developed at Beth Israel Deaconess Med Center in Boston, where I was originally diagnosed. It helps to have this protocol available in my electronic medical record so when I do go to the ED, triage can quickly see that I need immediate care. 

Thanks so much for sharing this Jeff! My husband's hemotologist thinks it's wonderful, and has created a similar document for my husband. 

He made some minor changes based on how doctors work in the emergency room.

He said "The hematocrit is not as useful to diagnose, treat, or manage shock as it can take over an hour to return.  If we were to include this metric on the triage panel, it might delay necessary treatment.  For comparison, the blood pressure and pulse rate take 15 seconds to measure."

So he removed the HCT references in the baseline values and DX of impending shock sections of the document. He included HCT monitoring as your doctor did for immediate management, specific treatement, and post treatment recommendation.

Thanks again for such valuable information.

To all my best wishes for your health and safety during this global crisis.

Rebekah Sellers

Hi Rebekah - that's great, anything that helps is fantastic. Happy to answer other questions you have about using the protocol.
One note, my hematocrit level is always drawn almost immediately (along with blood pressure and pulse) but ED staff does not wait for results to start the albumin administration. They are putting IV's in and starting the albumin as soon as it is ordered and delivered and then crit number comes back eventually.

- Jeff

Makes sense, thanks again Jeff 

Jeff will you email a copy to me please

 

Thanks shannon. shannoncdowell@gmail.com 

Jeff could you please email me a copy also  sarah-foreman@hotmail.com

 

I have the original protocol printed out but will add this. Thanks so much