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Case #47 The Medical Train Wreck


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#1 Mike MacKinnon

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Posted 07 February 2009 - 05:44 PM

Ok

After a hiatus d/t grad school (120 days of sheer hell left) Here we go.

You are kicked out for a respiratory "difficulty" in a local small town about 15 min from your base.

You arrive to find a man in his 40's who appears to be contracted and is only alert to name. His mom is there with him and it appears he lives with his parents who take care of him. There are volunteer FD EMTs in the room who has placed him on O2 and tell you he is 'air hungry'.

His vitals are: BP: 178/90 HR: 119 Sat: 88% on a NRM

Hx: CP, haemochromatosis, Aortic Stenosis, HTN, NIDDM, CHF, RA & severe mental retardation

Rx: atenolol, oral DM med, lasix, ativan

Allg: eggs, morphine & latex

On your eval you note he has dark skin as if it was tanned, significant contracture of the upper arms such that his hands are just below his chin/neck, he does not open his mouth much but he does not really respond to command. He is clearly gasping for air and you can hear the crackles as he is breathing.

To give a bit of a visual here is an idea of the contracture only imagine it tighter and higher up:

Posted Image


How do you proceed?

The facility that is appropriate is a 28 minute flight away.
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Mike MacKinnon MSN CRNA
WWW.NURSE-ANESTHESIA.ORG

"What gets us into trouble is not what we don't know
It's what we know for sure that just ain't so" - Mark Twain

#2 Speed

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Posted 22 February 2009 - 12:04 AM

hehe

Yes i am alive, just got really really busy over the last couple of months. A new case study will come pending the end of the one that is currently in process about PIH.


Hey Mike, did you fall on your ass in the shower, shallow grave in Mexico, or what? Do we need to send someone out on a welfare check for ya?
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Mike Williams CCEMT-P/FP-C

#3 Mike MacKinnon

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Posted 22 February 2009 - 02:14 PM

Hehe Ok case is posted :P
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Mike MacKinnon MSN CRNA
WWW.NURSE-ANESTHESIA.ORG

"What gets us into trouble is not what we don't know
It's what we know for sure that just ain't so" - Mark Twain

#4 BackcountryMedic

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Posted 22 February 2009 - 03:00 PM

Welcome back Mike!

What's his code status? DNR perhaps?
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#5 Mike MacKinnon

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Posted 22 February 2009 - 04:07 PM

hehe

you wish. :P

Welcome back Mike!

What's his code status? DNR perhaps?


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Mike MacKinnon MSN CRNA
WWW.NURSE-ANESTHESIA.ORG

"What gets us into trouble is not what we don't know
It's what we know for sure that just ain't so" - Mark Twain

#6 viking563

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Posted 22 February 2009 - 05:19 PM

Im a paramedic student, not quite in the big leagues yet but I like to give it a shot. 1st I would try and sit up as high as I get him, to keep the fluid down in the bases, I'd like to CPAP him but he's not alert enough for it, consider RSI or a nasal intubation. I start a saline lock, monitor and I would find out from his mom if the contraction is normal for him for not. As for drugs, Nirto SL, drip if we have it, maybe try Lasix. Maybe morphine but that might be on its way out of CHF treatment. How am I doing so far?
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Matt, NREMT-P

#7 Sue

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Posted 22 February 2009 - 06:27 PM

Im a paramedic student, not quite in the big leagues yet but I like to give it a shot. 1st I would try and sit up as high as I get him, to keep the fluid down in the bases, I'd like to CPAP him but he's not alert enough for it, consider RSI or a nasal intubation. I start a saline lock, monitor and I would find out from his mom if the contraction is normal for him for not. As for drugs, Nirto SL, drip if we have it, maybe try Lasix. Maybe morphine but that might be on its way out of CHF treatment. How am I doing so far?



He is allergic to Morphine, so I would not go there. Considering his contractures and inability to fully open his mouth, I would be hesitant to give him sux, let alone paralyze him.
How is his baseline mental status? I might consider CPAP and/or nasal intubation. I would also want to know where he goes for most of his treatment. I would be frugal with his fluids.
Maybe draw some blood with the IV access and I-stat him when the opportunity arises. How much Lasix does he take regularly? How is his output? Some NTG might not be a bad idea.
I am running through this off the top of my head, so forgive me if I am way off base.

Sue
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Sue Toberman, RN

#8 ST RN/PM

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Posted 22 February 2009 - 07:38 PM

OK. So, this guy is in his 40s, and severely mentally retarded, so any self-reporting of the symptoms of the onset of Hemochromatosis (HCTS for ease in written discussion) probably didn't happen. If the parents were medically savvy and noticed abnormalities such as hair loss, skin color changes or abdominal pain/joint pain, and got the patient to a physician that checked a ferritin level, then his HCTS may have not been misdiagnosed/untreated like many of the cases.
If this has been untreated/undetected/misdiagnosed, then there could be MI, Cirrhosis, Cancers, Liver failure (think fluid overload)......
Reading a bit about HCTS, there are often arrhythmias/MIs that occur. Sequestration/buildup of iron in the myocardium/coronarys leads to MI.
What is this patient's 12 lead EKG? His CHF, Aortic Stenosis and HTN are going to make for a high afterload and increased workload. Perhaps Hypertrophic Cardiomyopathy....(sounds like HOCM to me...takes Atenolol, has CHF, outflow obstruction due to iron deposits sounds intuitive. An echo would tell us what we already suspect.
TX: Try non-invasive ventilation... agree with CPAP and NTG drip....sit him up, if CPAP fails, nasal intubation with sedation. We have a pressure to play with, and the course of further treatment depends on his response to the initial treatment. Reducing the workload of the heart and allowing diastolic fill time is the goal of medical management when he is not in extremis.....so if this is an MI, a lil Lopressor may not hurt.
As far as intubation, he has predictors of a difficult airway....as he is probably stiff everywhere due to inactivity and HCTS....so externally....wanna look at the mouth, Mallampati is probably difficult to assess, 3-3-2 as well, and neck mobility is probably limited. I would shy away from RSI. If nasal intubation fails, maybe an "awake" intubation if he fails to respond to CPAP.
At this point, audible crackles, air hunger, hypoxia, tachycardia, hypertensive (im sure his parents keep him compliant with his antihypertensives.... but maybe not), looks to be in failure....pulmonary edema......what are lung sounds.....are there crackles bilaterally? Is there a fever? This sounds like a great case.....keep it comin.... and WELCOME BACK MIKE! Steve
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Steve T. RN, PM

#9 viking563

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Posted 22 February 2009 - 07:39 PM

Maybe morphine but that might be on its way out of CHF treatment.

whoops! Slipped my mind
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Matt, NREMT-P

#10 Speed

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Posted 22 February 2009 - 08:45 PM

Welcome back Mikey,

[quote]You arrive to find a man in his 40's who appears to be contracted and is only alert to name. His mom is there with him and it appears he lives with his parents who take care of him. There are volunteer FD EMTs in the room who has placed him on O2 and tell you he is 'air hungry'.

His vitals are: BP: 178/90 HR: 119 Sat: 88% on a NRM[/quote]

OK, 40 and this bad off, obviously none of this happened overnight. I'm guessing he's probably on hospice so we should have a pretty good history, and yes, the first thing I'd want to know as well is his DNR status or advanced directives. What kind of "contractures" are these? I see the RA, but I'd expect to see more deformity if it's bad enough to fix the joints in a contracture. His skin has some obvious pathology, looks like a type of scleraderma, ketoids, or papules of some sort, so I'm wondering about an influence on the neuro system (viral/bact) for resp. drive or a mechanical hindrance (muscle, joints, or skin). If they're associated at all? The high iron, low clearance kinda points to why his skin is dark and may be causing a toxic build up or reaction so you have to wonder if this "change" may be toxic by chance. It would be nice to have labs. But, anyway... ABC. Listen to his lungs and rule out the need for diuresis (valve and htn probably has some P pulmonale and/or PPH) as a cause for resp failure and treat it if need be. I'd go ahead and intubate him since he's non-verbal and no help to me, he needs it pretty bad. If the contractures have affected the diaphragm and inter-costal muscles and it's not just "tight skin"(like a burn) but muscular as well you're probably going to have to "play" with the settings because of restricted excursion (limited volume / protective pressures). We know his liver is shot, so if it's toxic we have to consider the kidneys as well, so what's his output been, what does it look like?

[quote]Hx: CP, haemochromatosis, Aortic Stenosis, HTN, NIDDM, CHF, RA & severe mental retardation[/quote]

ECG, FSBS, heart tones

[quote]On your eval you note he has dark skin as if it was tanned, significant contracture of the upper arms such that his hands are just below his chin/neck, he does not open his mouth much but he does not really respond to command. He is clearly gasping for air and you can hear the crackles as he is breathing.[/quote]

Ok, there ya go, give Lasix. See how that works and keep Dobutamine in the back of your head. So, no reason to stick around, get your primary intact (tube & IV) and go.
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Mike Williams CCEMT-P/FP-C

#11 LWTRF14

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Posted 22 February 2009 - 11:46 PM

Can we get mom to answer a few things before we get knee deep in procedures........ What were the events leading up to this...I know he is severely challenged, but lets rule out as much as possible.... Besides the breathing, is there anything else she sees different about her son, such as his arm positioning qnd his skin color always the way it is presenting and if not how long ago did it get like that? How has his urine output been(does he have a foley)? What is his temperature?

Thanks
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Earl F Bakke III, NR-EMT-P, CC-EMT-P, PNCCT

#12 EMT-I2RN

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Posted 23 February 2009 - 04:30 PM

Alright, this is my first response to case study, and I'm only an EMT-I, starting RN school in April, so go easy on me hehe! First off, I would ensure my crew and I were wearing nitrile gloves, keeping in mind the patients Ax to latex. I'd like to put the patient in a semi-fowler position and see if that helps his respiratory distress/O2 Sat. I'd want to put a PIV in, NS @ a TKO rate, give him some Lasix, 40mg IV slowly, if he is on a lot of Lasix, then I think we could give him 60mg or 80mg IV, don't quote me on that though. I'd like to see what his temp is. I'd like a 12 lead EKG as well. If the 12 lead is not indicative of anything that nitro could exacerbate, I'd like to start him on a nitro drip, 5mcg/min.

Intubation is out of my scope of practice in Nevada, so if you guys could help me out a lil with that, that be great. The issue with him not opening his mouth is an issue, but if we drop him with 3mg of Versed, and 100 of Succs, won't that make it so we can open his mouth easily? If we're not comfortable with the intubation, then couldn't we Combitube him? Thanks in advance guys. Your knowledge amazes me.

How'd I do?
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#13 MSDeltaFlt

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Posted 23 February 2009 - 08:40 PM

Ok

After a hiatus d/t grad school (120 days of sheer hell left) Here we go.

You are kicked out for a respiratory "difficulty" in a local small town about 15 min from your base.

You arrive to find a man in his 40's who appears to be contracted and is only alert to name. His mom is there with him and it appears he lives with his parents who take care of him. There are volunteer FD EMTs in the room who has placed him on O2 and tell you he is 'air hungry'.

His vitals are: BP: 178/90 HR: 119 Sat: 88% on a NRM

Hx: CP, haemochromatosis, Aortic Stenosis, HTN, NIDDM, CHF, RA & severe mental retardation

Rx: atenolol, oral DM med, lasix, ativan

Allg: eggs, morphine & latex

On your eval you note he has dark skin as if it was tanned, significant contracture of the upper arms such that his hands are just below his chin/neck, he does not open his mouth much but he does not really respond to command. He is clearly gasping for air and you can hear the crackles as he is breathing.

To give a bit of a visual here is an idea of the contracture only imagine it tighter and higher up:

Posted Image
How do you proceed?

The facility that is appropriate is a 28 minute flight away.


First off, is this his normal mentation?

Secondly, is everything we have latex free?

Third, with audible rales, this man is drowning regardless. So we are stuck on "B" for right now. The RT in me wants to go aggressive and NT suction him right now. However, must revert back to the above question first.

Also, what's temp? And since he has contractures, I'll bet he might have a Foley. What does the UO look like if he has any? There's a good chance he's got either MRSA and/or VRE so lets be careful here.
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Mike Hester, RRT/NRP/FP-C
Courage is resistance to fear, mastery of fear - not absence of fear -- Mark Twain

#14 5263rn

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Posted 24 February 2009 - 01:59 AM

Like other posts, I would ask about the patient's baseline status. Make sure this is not a DNR. Get IV access and get an accucheck. Get him on the monitor. Avoid any latex.

These patients are sometimes given routing vaccines. This doesn't sound like an allergic reaction, but I would ask about recent flu vaccine due to his egg allergy.
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#15 MedicNurse

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Posted 24 February 2009 - 02:29 AM

Count me in.

WELCOME BACK Mike Mac - time flies when you are doing time :lol: Wishing you all the best - you are going to be a wicked awesome CRNA!! The end is getting close! (for school and maybe this patient)


First Impression - these may not be all that accurate in the cruel light of hindsight! :P

SICK. Congenital challenges of cerebral palsy, mental retardation, cardiac structure disease - add NIDDM, HTN, RA - the hemochromatosis is a likely recent discovery (males usually present after age 30, but may have been diagnosed earlier) all point to a high likelihood of badness. Regardless of the mental issues or possible mobility issues - I'll also bet on obesity and poor diet.

Patient appears obtunded - is this baseline?

Baseline - is this patient mobile, degree he is able to participate in self care or is he total care and bedfast?

Immediate questions:
Onset of symptoms (suddenly, hours, days)
When was the hemochromatosis diagnosed? Treated and how often/last treatment (last phlebotomy, if ever)? Known extent of damage to liver? Renal failure?
Fever? Does he have indwelling medi-port or foley cath? Productive cough?
Prior cardiac evaluation? Extent of cardiomyopathy? Best ejection fraction? Prior MI?
Oral intake? Medication compliance? Swallowing problems - any choking? Recent foods (lots citrus, eggs, avocado, bananas, salty or cured foods, etc.)?

Airway - open but tenuous. LOC decreased. Patient clearly unable to protect.
Breathing - is the rate and depth adequate? Sat's are not acceptable even with O2. I'm going to assist respirations via BVM/O2 and prepare to secure an airway via ETT (have many options ready). CPAP may work - but, I'm thinking that a seal will be difficult to impossible and etiology is not clear. Old fashioned ventilation/maximize oxygenation are still in style.
Circulation - Rhythm? Do a quick 12 lead. Immediate venous access - quick large bore access, EJ, central line or IO if needed - get a blood glucose? Does the blood seem "thick"? NS to KVO.

Additional Assessment ?'s -
Auscultate lungs, heart and abdomen? abdominal assessment-distention/palpable liver/ascites present? well cared for-hygenine? height? weight? rashes? color of palms? clubbing of fingers? peripheral circulation? peripheral/dependent edema?

Differentials/Concerns -
Decreased LOC, probable difficult airway (mouth opening, suspect anatomy, probable poor dentition), skin color is "tannish bronze-metallic" indicative of hemochromatosis (can cause MI and CHF exacerbation) possible pulmonary edema from onset history? possible aspiration pneumonia, consider contractures and pad/support voids in "package" for transport.

Assess, assist respiration/ventilation/oxygenation, IV access and KVO NS, chemstick?, 12 lead - determine rhythm, treat if indicated, NIPB q 10 min.
Secure airway - Nasal ETT would be my first choice, if unable to secure I'd commit to RSI and be prepared for King Airway placement or cric if all else fails.
Will consider - lasix, nitro gtt, sedate/control any pain, maximize CO as needed

After airway is secure and IV access - we get ready to fly away now.

:blink:
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#16 Speed

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Posted 25 February 2009 - 02:10 PM

Alright, this is my first response to case study, and I'm only an EMT-I, starting RN school in April, so go easy on me hehe! First off, I would ensure my crew and I were wearing nitrile gloves, keeping in mind the patients Ax to latex. I'd like to put the patient in a semi-fowler position and see if that helps his respiratory distress/O2 Sat. I'd want to put a PIV in, NS @ a TKO rate, give him some Lasix, 40mg IV slowly, if he is on a lot of Lasix, then I think we could give him 60mg or 80mg IV, don't quote me on that though. I'd like to see what his temp is. I'd like a 12 lead EKG as well. If the 12 lead is not indicative of anything that nitro could exacerbate, I'd like to start him on a nitro drip, 5mcg/min.

Intubation is out of my scope of practice in Nevada, so if you guys could help me out a lil with that, that be great. The issue with him not opening his mouth is an issue, but if we drop him with 3mg of Versed, and 100 of Succs, won't that make it so we can open his mouth easily? If we're not comfortable with the intubation, then couldn't we Combitube him? Thanks in advance guys. Your knowledge amazes me.

How'd I do?


I think you're going down the right road. With this guys stenosis I would go low and slow with the diuresis and nitrates. You want to make sure he's got volume behind the blockage (look at his skin and output), then start slowly with the Lasix. Same goes for nitro. His pressure could drop like a rock with the blocked output. I would avoid hypotension or increasing his rate because he has the obstruction. My end-point for the nitro would be low-normotensive. This would be somewhat diagnostic to "test the waters" with his AS. If after diuresis and nitro the edema was still sticking around I might try a trial of Dobutamine, again low and slow to see if increased filling or "taking a bigger bite" would push that stuff on through, but you've got to be ready to go back a few steps if he deteriorates. Of course if you've got CPAP it works great too. I'm feeling that we've got to differentiate whether the resp. failure is more from the edema or tetany, maybe both. With only a 28 minute transport time we probably wouldn't get too far into tweaking hemodynamics and I don't know that I'd want to get too aggressive without better equipment, maybe a PA cath and echo.
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Mike Williams CCEMT-P/FP-C

#17 JLP

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Posted 25 February 2009 - 02:30 PM

The issue with him not opening his mouth is an issue, but if we drop him with 3mg of Versed, and 100 of Succs, won't that make it so we can open his mouth easily? If we're not comfortable with the intubation, then couldn't we Combitube him? Thanks in advance guys.

How'd I do?
[/quote]

Good thoughts but I would not use SUX without further info - first, long-term contractures are often permanent and will not relax with SUX, also patients whose immobility is neuro based or due to muscular dystrophies will have excessive K release to SUX. Probably would use SUX at all, go awake with topical anesthetic and sedation. Coming from a facility, I would sooner use anesthesia for a guided intubation (i.e. bronch) than a non-ETT airway device like a Combi.

I'd like a cortisol assay - hemachromosiderosis also causes adrenal damage, and the tan may be melanin due to Addison's. May need solu-medrol or hydrocortisone IV
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#18 Speed

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Posted 25 February 2009 - 03:52 PM

patients whose immobility is neuro based or due to muscular dystrophies will have excessive K release to SUX


True
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Mike Williams CCEMT-P/FP-C

#19 EMT-I2RN

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Posted 25 February 2009 - 03:58 PM

The issue with him not opening his mouth is an issue, but if we drop him with 3mg of Versed, and 100 of Succs, won't that make it so we can open his mouth easily? If we're not comfortable with the intubation, then couldn't we Combitube him? Thanks in advance guys.

How'd I do?
Good thoughts but I would not use SUX without further info - first, long-term contractures are often permanent and will not relax with SUX, also patients whose immobility is neuro based or due to muscular dystrophies will have excessive K release to SUX. Probably would use SUX at all, go awake with topical anesthetic and sedation. Coming from a facility, I would sooner use anesthesia for a guided intubation (i.e. bronch) than a non-ETT airway device like a Combi.

I'd like a cortisol assay - hemachromosiderosis also causes adrenal damage, and the tan may be melanin due to Addison's. May need solu-medrol or hydrocortisone IV



Sorry if this makes me look like a moron, but what's "bronch"? Thanks for all the pointers. This forum and of all you guys are an amazing resource!
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#20 Medic09

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Posted 25 February 2009 - 04:53 PM

Like others, I want some short, quick info from Mom if she knows it. After that, I want to scoot pretty soon.

Why is he on a beta blocker? Lasix?

Are the contractures new?

Does he normally have some level of respiratory compromise?

Are there changes in mentation?

Has he had phlebotomy and/or transfusions for his primary disease? Other recent illness or procedures/treatments?

As previously stated, I'm stuck on B of ABC. I could live with 88% for a short flight, if pt. is otherwise stable. Clearly he'll be a hard tube, and maybe we can just bag assist or CPAP him. I'm more concerned with what's going on dynamically?

Thinking out loud:

The skin colour sounds consistent with the Hemochromatosis.

RA can cause contractures; but this seems pretty severe for that? I don't *think* iron overload does this; but does iron overload cause what causes this? Is there another, more recent cause?

How's his WOB? Is there reasonable chest expansion?

What does a little more physical exam show? JVD? Indications of hepatic involvement?

Does he appear to be in pain?

Is this respiratory or cardiac in origin?

Start with the usual IV, O2, monitor (EKG certainly). Positioning. His BP looks like it would support some NTG or Lasix.
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Mordechai Y. Scher
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It's all about kind, competent patient care; and getting home safely to tell about it.