Dystrophic Epidermolysis Bullosa

[MMARN, BSN, RN]

I saw a show on the Discovery Health Channel about a man who had EB. I have to say, it was one of the saddest things I have ever seen. All I want to do is cry. As a nurse, how do you deal with a human being in so much pain? And, have you ever cared for anyone who has had this condition?

[medsurgnurse, RN]

I saw a show on the Discovery Health Channel about a man who had EB. I have to say, it was one of the saddest things I have ever seen. All I want to do is cry. As a nurse, how do you deal with a human being in so much pain? And, have you ever cared for anyone who has had this condition?

I thought it was very touching but not in they same way as you have said. This gentlemen's sense of humor and positive attitude should be a lesson to us all. I appreciated his preparation for his own death and he said some profound things. I work in hospice. I though it would make excellant viewing for hospice staff and all healthcare staff. I never cared for anyone with this condition but I have cared for people who are in terrible pain. Once they are on hospice we can usually get their pain controlled.

[Wickett]

I just started a home care case Friday with a 4 year old boy with this awful disease! He's been in the hospital last month, state took him from Mom, no where else for him to go. I know nothing about this illness ecept what I could find on the Web. His treatment plan is daily bath with 1/2 cup of bleach added, pop all blisters,cover with liberal amount of vaseline, apply xerform dressing everywhere, wrap in kling, and netted cover. He has sores over 99% of his little body. His meds are just Multivits, iron, zinc, pro-biotics, benadryl, motrin, prn. We did give him 5 ml of Tylenol with Codeine about 45 minutes prior to dressing change, it made him vomit. THE AMOUNT OF PAIN THIS CHILD IS IN IS IN MY OPINION CRIMINAL! When the blisters are being opened he is writhing and screaming in pain, saying " No pop-pop, no pop-pop!! I think he needs to be treated by a board-certified pediatric dermatologists ASAP! My case manager is getting him a referral for pallative care, but I have no idea how long that will take. He's losing his fingers due to the sloughing off of skin, he's got major limitation in his ROM due to skin tightening. This is the cruelest disease and I want to help this child. But I don't know what to do? Does anyone out there have any ideas!!! Anyone with burn experience? At first I didn't even know if I could handle it, but what I went through is NOTHING COMPARED TO WHAT HE LIVES WITH EVERY MOMENT!! There must be some way to make his time on this Earth less painful. Thanks for all help. Wickett

[llg, PhD, RN]

I cared for a neonate with this condition many years ago. He died at approximately 2 months of age. He had one of the most beautiful little faces I have ever seen. I used to wrap him a blanket and just rock him to try to give him some peace. Fortunately, my colleagues were able to help with my other patients so that I wouldn't have to jostle him by putting him back in his incubator.

[Wickett]

Thanks for responding to me, you have been the only one, which again shows just how rare this horrible disease is. I've been working with him for 3 weeks and after numerous phone calls to his doctors the only pain med they gave him was Naprosyn, 62.5 mg/q80/prn!!!! I thought I had convinced the foster Mom to take him into one of our Children's hospitals but she is hedging. I'm due to go back in tomorrow morning but in all conscience I can not continue to participate in his dressing change without major pain control. I feel as if this is child abuse and I refuse to be a part of it. I'll contact my case manager and social services tomorrow if I am forced to make this choice. I hate to leave him but if I stay I am just condoning this abuse! If any one has any other ideas please let me know. Wickett

[EmmaG, RN]

Thanks for responding to me, you have been the only one, which again shows just how rare this horrible disease is. I've been working with him for 3 weeks and after numerous phone calls to his doctors the only pain med they gave him was Naprosyn, 62.5 mg/q80/prn!!!! I thought I had convinced the foster Mom to take him into one of our Children's hospitals but she is hedging. I'm due to go back in tomorrow morning but in all conscience I can not continue to participate in his dressing change without major pain control. I feel as if this is child abuse and I refuse to be a part of it. I'll contact my case manager and social services tomorrow if I am forced to make this choice. I hate to leave him but if I stay I am just condoning this abuse! If any one has any other ideas please let me know. Wickett

This is horrible

Would the primary be willing to consult with a pain specialist?

At first I wondered if sustained/continuous-release pain meds could be used in peds, and perhaps a very low maintenance dose would be appropriate, but that doesn't address the acute pain experienced during the procedures. I understand your frustration. We may not be able to cure, but we DO have the ability to relieve pain. No one should have to endure that.

Thank you for being so compassionate and caring for this child.

[EmmaG, RN]

Hey. Here's a contact that could give you some direction:

DebRA's Nurse Educator

DebRA provides a Nurse Educator to serve as a point of contact to assist new parents and patients, as well as to help healthcare professionals manage the care of EB patients. This program provides general information on EB and referrals to related professionals.

Intake for this service is available via phone or email Monday - Friday 9 a.m. to 5 p.m. Eastern Time. Contact Information:

DebRA Nurse Phone: [phone]

DebRA Nurse Email: [email protected]

The DebRA Nurse Educator

Geri Kelly-Mancuso, RN Ms. Mancuso has extensive pediatrics experience as a school nurse and nurse at South Bronx Children's Health Center. Prior to becoming a DebRA Nurse Educator, she was the Clinical Nurse Manager of the Hope Center at St. John's Riverside Hospital in Yonkers NY, where she managed the facility's outpatient AIDS clinic. Ms. Mancuso speaks Spanish and helps DebRA meet the growing demand for outreach to Spanish-speaking families. Ms. Mancuso earned a bachelor's degree in nursing from Hunter College in New York City.

In 2007, The DebRA Nurse Educator Program is made possible through the generosity of The Fund for the Poor, Integra Foundation, National Rehab, NYBOT Futures and Options for Kids, NYMEX Charitable Foundation and other DebRA supporters.

http://www.debra.org/modules.php?op=modload&name=News&file=article&sid=4&mode=thread&order=0&thold=0

http://www.debra.org/modules.php?op=modload&name=News&file=article&sid=8

Good luck, hun.

[RNAnnjeh, MSN, CNS]

It is a terrible condition. My only experience was with a babe in the NICU. We were making a plan to transfer him to our peds medical unit, and the first med on his list was for pain management. Our palliative care team was managing his pain meds and they did a fantastic job. The transfer to medicine never happened; the celesital transfer came first.

[Wickett]

Hi,

I told the foster Mom about the debra site, she's done nothing with it. His current docs need to give referrals for pain team to step in, EVERYNONE keeps passing the buck on this little guy. Makes me very angry!!! At this point, I agree his pain can be managed, but no one wants to be the one, not sure if it's ignorance or fear of using narcotics. Whatever, it's totally unacceptable and if I go in at 8AM tomorrow and nothing has changed my first phone call will be to his primary to alert her to the fact that my next phone call is to social services for physical and emotional abuse, as well as neglect. If foster Mom won't take him to Children's Er immediately I will call an ambulance and send him myself. Enough is enough!! I'm angry at myself for letting everyone talk me into, " they're working on it", nonsense. I am confident my notes will support whatever steps are needed to get this little guy treated in the appropriate way. In additon to all of this, his main language is Spanish and no counselor has spoken to him about where his mommy, siblings, grandparents are, and why he's living in a new place. I was told that this is also "in the works". The reason I became a nurse was to help people, not participate in their pain. Thank you for caring about our little guy. Please keep me in your prayers tomorrow for the strength to help this child, because I know that I am going to be going up against people with way more initials after their name than I have.

Wickett

[EmmaG, RN]

If he is in foster care, have you contacted his case manager? Have you tried the debra contacts for advice? It sounds as if you are doing an amazing job. That little boy is very lucky to have you to advocate and care for him *hugs*

[EmmaG, RN]

I was playing with our little guy, and telling my daughter about this poor child.... and after re-reading your posts, I am sooo angry.

I'd be tempted to call in the local media, HIPAA be damned.

[Nursebarebari]

I just started a home care case Friday with a 4 year old boy with this awful disease! He's been in the hospital last month, state took him from Mom, no where else for him to go. I know nothing about this illness ecept what I could find on the Web. His treatment plan is daily bath with 1/2 cup of bleach added, pop all blisters,cover with liberal amount of vaseline, apply xerform dressing everywhere, wrap in kling, and netted cover. He has sores over 99% of his little body. His meds are just Multivits, iron, zinc, pro-biotics, benadryl, motrin, prn. We did give him 5 ml of Tylenol with Codeine about 45 minutes prior to dressing change, it made him vomit. THE AMOUNT OF PAIN THIS CHILD IS IN IS IN MY OPINION CRIMINAL! When the blisters are being opened he is writhing and screaming in pain, saying " No pop-pop, no pop-pop!! I think he needs to be treated by a board-certified pediatric dermatologists ASAP! My case manager is getting him a referral for pallative care, but I have no idea how long that will take. He's losing his fingers due to the sloughing off of skin, he's got major limitation in his ROM due to skin tightening. This is the cruelest disease and I want to help this child. But I don't know what to do? Does anyone out there have any ideas!!! Anyone with burn experience? At first I didn't even know if I could handle it, but what I went through is NOTHING COMPARED TO WHAT HE LIVES WITH EVERY MOMENT!! There must be some way to make his time on this Earth less painful. Thanks for all help. Wickett

WOW, I am tearing as I write this because it is so heartbreaking to see a child go through something like what you described. I don't know anything about this and can not give you any advise on what to do. But just keep trying to get him the max pain med he can get. I wish him all the best.