[Sweeper933]

I'll try to make this all as brief as possible...
I've been taking cary of this baby all week who was born at 25 2/7 weeks. Soon after birth, it was suspected that he had epidermolysis bullosa - the skin from his knees down was already peeling off... Upon getting more of a family history, it was discovered that parents had a daughter born at 27 weeks in Pakistan who died "from and infection in her legs because all the skin fell off". It was also discovered that these parents are first cousins (don't even get me started on that one... I know it's a cultural thing, but still!)
Anyway, this little guy has gotten progressively worse. He has never had any urine output - ever. Abdominal US revealed a whole ton of things: high grade partial pyloric stenosis, areas of decompressed bowel loops, bilat. hyperechoic kidneys with poor corticomedully comp/ wit renal dysplasia. no urine found in bladder. Bilateral ureteral stensosis or atresia... In other words, his kidneys are not formed right, so if he is making any urine at all - it has nowhere to go. Dermatology has taken a skin biopsy to confirm the epidermolysis bullosa diagnosis.
As I've been doing my research on all of this, as well as talking to the docs, we've determined that he most likely suffers from the junctional form of the disease - which is why his kidneys and such are all so severly affected. Our docs have now had several talks with the parents about all of this, and so has dermatology and genetics. However - the parents still want everything possible done for him - they are hoping for a miracle.
So my question after all of this, is what can we do for him to make him comfortable in the mean time?? He's in a giraffe bed w/ the humidity set at about 80%. We have him laying on Telfa, as he seemed to not stick to it nearly as much as anything else. The skin from his knees down on both legs is constantly peeling off, as is both wrists, fingers, groin area. We are applying liberal amounts of Aquaphor (as we would do with any baby this size...). His belly is huge (from a build up of tons of fluid in the periteneal space), so we are adjusting his vent settings accordingly. We are treating his acidosis/high potassium levels (>8) accordingly.... He is on a Fentanyl drip of 3mcg/kg/hr, with addional bolusis ordered prn.
All of the information I have found about this deals w/ term kids... I havn't been able to find anything regarding preemies w/ this disease. Is there anything else we should be doing for his skin/comfort level???
(Sorry for writing such a long post... for a little guy he sure is complicated!)

[prmenrs]

It might help to consult the Ethics committee! I hope your facility has one.

The only thing I could find is this citation, which deals more w/the genetic issue than care issues:

http://www.ncbi.nlm.nih.gov/entrez/q...&dopt=Abstract

Sometimes, I've seen the Burn Service consulted on patients w/skin problems like this. You do need to do Reverse Isolation--sterile linen, gowns, gloves, the works. There is absolutely NO barrier to infection. Liberal use of pain meds--as you are doing. You could use the sterile clear plastic barrier sheets/drape for him to lay on. And to make a little "blanket" to tuck around him--containment kinda thing.

All I can do is offer support for you--it's a seemingly no win situation. I'm sorry.

[NiteRocker]

You are to be commended for your good care. Such a hard situation. Medical knowledge and parent wishes can be far from the same.

Like my manager asked me recently, "What are you doing for yourself? You've done so much for the baby tonight, now what are you going to do for yourself?"

I was once given a smooth rock from a chaplain. She said hold it, talk to it, grieve with it until you can let go. This helped me. I had a candle and my rock. Then I buried it in a house plant, where it still is.

[Sweeper933]

Originally Posted by prmenrs

It might help to consult the Ethics committee! I hope your facility has one.


Sometimes, I've seen the Burn Service consulted on patients w/skin problems like this. You do need to do Reverse Isolation--sterile linen, gowns, gloves, the works. There is absolutely NO barrier to infection. Liberal use of pain meds--as you are doing. You could use the sterile clear plastic barrier sheets/drape for him to lay on. And to make a little "blanket" to tuck around him--containment kinda thing.

All I can do is offer support for you--it's a seemingly no win situation. I'm sorry.

The Ethics committee is a good idea, hadn't thought of that yet. I'm sure that if the parents continue to want this whole situation drawn out, we will be consulting w/ the Ethicc committee. We are using some sterile things on him; sterile gloves when handling him, and the Telfa he's laying on is also sterile when we lay him down on it. Anything plastic like you mentioned, would only stick to his skin even more. By using the bendy under the linens, we do sort of have him in a nest...
thanks!

[EricEnfermero]

I'm sorry to hear about this little guy's issues. I have yet to come across an EB patient, but it can't be fun.

[Jolie]

Bless you all for providing such good care to this precious baby.

I don't mean to sound crude, but I just can't imagine that he will survive very long. His poor renal function, fluid and electrolyte status, severely compromised respiratory status, and potential for infection seem insurmountable for such a tiny preemie.

When his parents say they want everything done, what do they mean? Dialysis and kidney transplantation are clearly not options due to his prematurity and size, and it seems highly unlikely that he will survive long enough to grow and gain sufficient weight to become a candidate.

Infection control and comfort measures seem the most important priorities now, but they won't slow the decline of his renal status. How terribly sad.

Please take good care of yourself and the baby's parents.

[BittyBabyGrower]

I've taken care of 2 of these kids in my 20 some years We used strict sterile technique for everything on these kids. We found that they grew fungus on their skin very easily also, most likely because of the high humidity and Aquafor combo we used on them. Both of those kids lived for about 3-4 months then they died from overwhelming sepsis...it was awful.

Are they giving adequete pain meds? Those poor babies have all their nerve endings exposed all the time and the tiniest touch makes them miserable. We kept them well dosed on drips and PRNS. Also, make sure you are using lambs wool or eggcrate on the bed, or gel pads.

Those kids break your heart...you sound like you are doing an awesome job though!

[prmenrs]

You really need to have sterile processing sterilize his linens--baby blankets, etc.

I think his chances are absolutely nil, and whatever it takes to provide comfort is the goal.

The parents (and the whole family) need some serious genetic counseling.

As BBG said, you are doing an awesome job!

[janfrn]

I'm right there with the recommendations for totally sterile handling and adequate-plus analgesia for this babe... no touching without a nice bolus of something narcotic and strong, and with sterile gloves. (Can't quite figure out the application of sterile gloves inside an isolette... would need someone to open the portholes I guess...) But I also agree that he WILL become septic no matter how scrupulously technique is maintained and he will die, either from sepsis or from hyperkalemia. I honestly don't know which way is worse. Sepsis will entail much suffering on everyone's part, but especially his. Fluid boluses won't do much for his skin integrity and inotropes will only make his gut more ischemic. Antimicrobials would be useful, sort of, but they can't work fast enough or be given in high enough doses to keep this kid from growing everything there is to grow. Hyperkalemic arrest, in the absence of a DNR, would mean CPR... dang! I hate when that happens! And that would lead to worsening acidosis, an even higher K+, PAIN BEYOND DESCRIPTION... and for what? The ethics committee would not side with the family on this case, I'm sure. I wish we were able to somehow avoid the whole keeping-the-child-"alive"-because-the-parents-can't-let-them-die scenario altogether, but this isn't a perfect world. These kids tie me up in knots. Maybe if there was a way to translocate the pain and suffering onto the parents they'd get the idea.

Now, the people who are providing care for this poor wee scrap need to be cared for just as much as he does. I hope your unit social worker and chaplain are available to you whenever you need them and that you have an outlet for all the sorrow and frustration your'e feeling. Of course, you always have your allnurses family to turn to. Please take at least as good care of yourself as you are giving this baby.

[fergus51]

I'm sorry to hear this story. I've cared for one baby with EB in my time as a NICU nurse and it was by far the worst experience I've had. All you can do is sterile technique and pain meds. I doubt the parents will ever agree to withdraw (I've seen this over and over when dealing with parents from that part of the world), so all you can do is what you're doing.