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#16985 - 04/24/07 10:47 PM Ideas for Infant care
snackboy Offline
Member

Registered: 04/24/07
Posts: 9
Hello all,

Does anyone have any ideas for infant care for my son who has EHK (inherited from me)? He is now 7 days old and still in the NICU being monitored for weigth gain and pain management during his dressing changes. Right now we, at the suggestion the pediatric dermotologist, using Aquaphor on Telfa to cover any openings wrapped with gauze. We change his dressing once a day, and during this time, even though it only takes an hour, he is screaming at the top of his lungs.

Morphine was prescribed at first, but that knocked him out for the day. So then we went to Tylenol, but that was not enough to take the "edge" off, so now we are back to morphine, trying to figure out the appropriate dosage.

Does anyone have any ideas? I have EHK myself and currently I manage it through Soriatane. The details are a little sketchy from when I was born 38 years ago, so right now I am at a loss. Any ideas or experiences would be greatly appreciated.
_________________________
It's only temporary.

snackboy (EHK - NPS ?)

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#16986 - 04/25/07 07:59 PM Re: Ideas for Infant care
Curtise Offline
Member

Registered: 05/16/06
Posts: 115
Loc: Sheffield, UK
Hi, my daughter Nina is now 16 months and has EHK. This came out of the blue to our family, and she was actually born at home, so we transferred to the hospital and she was in the Neonatal unit for 10 days.

Nina had a lot of blisters and large raw areas on her skin at that time. She was on IV morphine for the first week or so, then it was reduced and she was off it altogether by the time we came home, although with hindsight, I think we should have continued with a more rigorous approach to pain relief, I think we underestimated how uncomfortable she was.

She certainly screamed when we changed her, every time - and that continued for some time. Our regime at that time was a bath with bath oil/emollient every other day (it soon became daily) followed by lots of emollient. We used a 50%/50% liquid paraffin /soft white paraffin mix, which is probably roughly an equivalent to Aquaphor. We used Mepitel silicone dressing on raw areas, and Tubifast on her limbs and torso to keep the emollient on her skin as long as possible, and to prevent irritation from clothes. That's a kind of stockinette tubular retention bandage which you cut from a roll - when Nina got bigger, we used garments made out of the same fabric, still do actually.

We did the whole "greasing up" (for want of a more pleasant phrase...) routine about 4 times a day, but soon found that was just too hard to maintain, and so distressing for us all, so we stuck at 3, which we still do - morning, lunchtime and bedtime.

Funny, it seems a world away, thinking of those early days! I came to this board seeking help and support and felt like a total novice, but 16 months in, I guess we're settled into a routine. I think Nina's distress at change times was probably the worst thing, it felt like we were torturing her, but that did eventually improve. I hope Kimberly, Evan's mommy, sees you post and replies, as she may well be able to advise you more precisely about pain medication.

Lots of luck, and congratulations on the arrival of your little boy!
Best wishes,
Curtise

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#16987 - 04/27/07 03:16 PM Re: Ideas for Infant care
Evan's Mommy Offline
Member

Registered: 08/11/05
Posts: 165
Loc: Portland, ME 04106
Congratulations on the arrival of your son. Our 22 month old son, Evan, has palmar plantar EHK. If you have any time or access to a computer in the NICU, do some searches through these boards on any topics that you have questions. I found that very helpful was Evan was first born.

Evan's care has always involved putting a very small amount of chlorine bleach in his bath water to control infection (1 capful for a baby bathtub). This has been amazingly effective in preventing infections. This idea was given to me by another EHK mom, but was approved by Evan's dermatologist. For dressings, we used plain 3 x9 and 3 x18 inch vaseline gauzes and held it on with surgilast (elastic netting - ask the nurses). As Evan got older, the need for dressing has diminished. We also distracted him with sugar water squeezed into his mounth with a syringe. Up until about two weeks ago, Evan was on morphine. He had it once a day right before his bath/treatment and dressing change. Our dermatologist was never supportive of our use of morphine. Evan's pediatrician and a pallative care doc did support opur decision to medicate. We just recently weaned him because his changes are quicker and he seems more angry than in pain when we have to stabilize him to remove dead skin or lance blisters. However, we truly believe that the limited amount of pain relief that we gave to Evan has enhanced his ability to develop. I would not hesitate to give him a dose, if I knew that we needed to work at his skin and cause duress for more than 30 minutes. I would have to look back through Evan's notebooks to check on his dose schedule, but at 22 months old he was on 4 mg once a day.

Right now is probably a very scary time for you and your family. The NICU is an awful place to be. One piece of advice that another EHK mom gave to me was that my son would not remember any of this awful time. Another thing that the other caregivers will tell you is that is does get easier over time.

Feeding wise, EHK children have a very difficult time gaining weight becuase their skin eats up so many calories. Feed him often and early, but do not expect him to gain quickly, as much as you'd love him to,

Please fel free to call me @ 207. 799. 0520 or e-mail me at kimmayone @juno.com. I would be happy to listen and or support you and your son in anyway that I can.
Best Wishes,
Kimberly

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#16988 - 05/01/07 06:26 AM Re: Ideas for Infant care
mumofcalista Offline
Member

Registered: 04/12/06
Posts: 38
Loc: Gold Coast, Queensland, Austra...
Hello & congratulations!
At 18 months old, the newborn days in ICU are fairly fresh in my mind still, but I dont think that Calista has the severity of EHK that many members seem to have (are there degrees?). Anyway, for what its worth, Calista was kept totally naked in a humidicrib set at high humidity and 'basted' with 50/50 paraffin until the initial lesions had healed. No dressings to change, just paraffin over lesions. She was only given paracetamol when she seemed distressed, and not bathed at all for the first 2 weeks. I would also agree that this does get easier, and you are in front already, as you have know how he feels. However, now is a very traumatic time and you are in my prayers.

Rebecca

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#16989 - 05/02/07 02:29 PM Re: Ideas for Infant care
snackboy Offline
Member

Registered: 04/24/07
Posts: 9
Thanks to everyone that has posted. Its very helpful to know that other people (as unfortunate as it is) have gone through similiar experiences.

David came home on Saturday and is doing well. For the EHK, we have been using Aquaphor pasted onto the Telfa over raw skin and wrapped in gauze. For his diaper, we use Aquaphor as well. Pain is managed with morphine once per day (.4mg/2ml[?]). David was doing really well with no (large) open areas until today. We think he had a gas pocket or something and while keeping us up all night he wound up kicking his knee and thigh raw. Also, there were blisters that formed which were clear. I am assuming that this is part of the process that we don't always see. I know that if I have a constant irritation, from shoes or elastic, I will blister in the same manner.

My mother did say that my skin began to "crust" over (keratinize may be the more appropriate term) at around 6 months. Any ideas or experiences to add.

The pediatricians have upped David's calorie intake to 24 calories via EnfaCare. I hope we can move away from that since he seems to be gaining well now - after two weeks he is above his birth weight.


Again, thanks for the congratulations and support.
_________________________
It's only temporary.

snackboy (EHK - NPS ?)

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