My take-away from the Debra’s PCC in Orlando – Part 5

Here is more information about several talks from the conference from July 31, 2012:

Anesthesia for your child with Epidermolysis Bullosa & Interdisciplinary EB care

These two talks, which I will bunch up together simply because I don’t remember a whole lot from them separately, were done by Dr. Eric Wittkugel & Dr. Anne Lucky, both from Cincinnati’s Children Hospital. The only two notes I wrote down were to, one, look up ‘Mepitac‘, which is a tape that can be used for EB patients (does it work for RDEB? They say it does, I am always a little weary of it, but I am willing to try it) and, two, the duration of anesthesia is not a problem, there is no risk in case a patient needs to be ‘under’ longer for multiple procedures.

Other than that, I will let the slides do the talking, hopefully they will help someone. Remember to click on the slides to view a bigger version…

All of these slides are from the Anesthesia presentation, I found THIS PDF document online from Stanford with more info about Anesthesia that relates to EB.

Here is the videos of these presentations courtesy of Debra:

Dr. Lucky’s presentation is available ONLINE at this link HERE! (you will need a Google account to view, but it’s FREE) Basically, Interdisciplinary EB care means having everyone that is an expert of EB, from the dermatologist to the GI, hand surgeons and more under one roof.

Following these two talks there was a Physical and Occupational panel Q&A.  One question that comes to mind was from a therapist asking if she could use coban with the finger wrapping between web spaces and them stating coban was just too harsh on RDEB skin (which I agree-when Coban touches Nicky’s skin it’s blister city, so I only use it on top of bandages). My two questions were if there were any oils they could recommend to soften scar tissue and what exercises they could recommend to improve bone density since Nicky can hardly walk. The first question about the oil, they did not have any recommendations, except for just massaging the hand, which is hard to do when it’s full of wounds. As per the bone density question, she stated that at least standing is important, so Nicky has been standing as much as he can since I told him, bless his heart! Another suggestion I got is to get a vibration platform, that it could be rented etc. By talking to other parents and doing a little web search , I found out that Coconut Oil, Vitamin E, and I am trying this Argan Oil, all soften scar tissue. I soak his hand in these oils before rewrapping them.

Here is the video of the presentation, courtesy of Debra of America:

Here is the presentation for OT & PT at the 2010 Conference:

Medical and Integrative Approaches to the Management of Pain and Itch in EB

This talk was probably one of the most important of the entire conference. It was given by Dr. John Saroyan & Dr. Traci Stein. I mostly took photos of the slides because they were SO IMPORTANT and I did not want to miss a beat.

This talked about the various drugs that can be used depending of what form of EB, drugs used to subside itching and the issues with constipation.
So, once again, I will let the slides do the talking. Remember to click on the slides to view a bigger version…

If anyone has any info I might have missed, please leave a comment below (in the Facebook comment section).  THANK YOU!

Update August 15th, 2012: I found more notes about this latest talk about Pain Management that I think are worth sharing. The Doctor spoke about different ways to reduce pain, such as trance inducing activities, relaxation techniques, pleasant scents… for more information she stated to contact her at her website: http://www.drtracistein.com/

Here is the video of the presentation, courtesy of Debra:

GO TO PART 6 –>>

Links to — > Part 1Part 2 – Part 3 – Part 4

Mom to 3 boys, 1 in heaven, 2 on earth. My first son Alex (whose demise is most likely EB related) was stillborn at full term. After a miscarriage, I had my second son Nicky, who has the Recessive Dystrophic form of Epidermolysis Bullosa. My youngest son, Connor, is 100% healthy, and I never, ever take it for granted. I am an author, photographer, graphic artist, webmaster, blogger and more.

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Hand Wrapping Instructions

By Lorraine Spaulding

The following illustrations are an example of a hand wrapping technique found to be helpful in the protection and preservation of RDEB involved hands. The instructions are for very small hands and some adjustments probably need to be made for larger hands. Lorraine also have some suggestions if anyone is having trouble with the thumb pulling in.

Please note: these instructions may seem overwhelming at first, but after a little practice and as your child gets used to the process, it becomes a worthwhile routine.

Keep in mind the positive results and don’t get discouraged!

Note from the webmaster: this is a graphic intense page. I recommend waiting for it to load and then print it out.

WATCH THE VIDEO OF THIS DEMONSTRATION AT THE STANFORD WEBSITE:

http://dermatology.stanford.edu/gsdc/eb_clinic/eb-videos.html

All illustrations are of the left hand.

Step 1 is an optional application of Vaseline Gauze to the palm of the hand. Use a 3″ X 9″ piece of Vaseline Gauze. Cut about 2″ off one end, then slit lengthwise about a 4″ length, about 1″ down from the top edge.

Wrap around hand with 1″ strip between thumb and index finger.

This provides some resistance from retracting.

Fingertips can be left exposed to some degree, so that tactile sensation can be experienced.

At this point it may be necessary to start a 2nd roll of 1″ gauze. Make one overlap and continue.

Views 10-19 Front of Hand

Spaces in palm bandaging will be covered by criss/cross bandaging of web spaces.

Web spacing can be randomly done. The point is to cover any open areas of the palm while

gently pulling a wrap between each web space

Be very careful to never wrap or pull too tight!

Be sure to have slight tension in web spaces.

Suggest wrapping additional 1/2 roll of bulky bandage around arm from the wrist to the upper arm. (Held by Tube Gauze) Provides padding of forearm and elbow.

Wrapping Hands & Arms The ABC Way

By Sheri Coil – with  the assistance of Brandi

The following is the way we wrap the hands (and arms while we are so near) to reduce the mittening deformity that RDEB causes.

After cleansing and debriding I use a lot of Alwyn cream or what ever else I have used before it came along.  The open wound or skinless areas get Mepital first, then Vaseline gauze.

We have had excellent luck with the Mepital preventing sticking bandages. The oozy serum stuff can drain right through it to be absorbed by outer gauze.  And the creams and ointments can get back to the wounds.  The  Vaseline puts up a further barrier to sticking.

The gauze starts on the top of the hand.  I use Kendall conform 1″.  You can see the Mepital under the Vaseline gauze.  Please note that the wounds on this arm are all self inflicted from scratching. The areas she doesn’t scratch are very clean.  I used this area to demo on because it needs the works as far as under gauzes

The gauze then goes around the thumb a couple of times, until it is wrapped and then  around the hand, over the top and around the first finger,  around it til wrapped, and around the hand to the next finger…..

After the fingers are wrapped, I go around the hand and come up through each web space.   Two going around the first way and then  an extra half wrap around the wrist to make the last one cross over the first two.  Clear as mud, huh?  Ggg

Then I wrap a two inch Kendall  Conform bandage over the wrist, around the hand one loop and then through the thumb space and on around the arm on up Coming back down the arm, I pad the armpit, elbow and anywhere bad and roll the bandage right over it.  I use Kerlix for that padding, cut to what I need.  I just roll the bandage on.

No pulling, but not loose.  It just needs to support the skin, not cut off circulation or cause mis-shaping over time.

I  use tube gauze over everything.  I do not like to use tape at all.

Here she is, all ready to go! 🙂 Our Dancing Queen 🙂

Here is the Video of the wrapping technique recorded in January 1998

Physical Therapy

I put this page together with the aid of a little booklet distributed by DEBRA UK called “A guideline to physiotherapy for parents of Children with Dystrophic Epidermolysis Bullosa”.

As the booklet states, this page is most useful for parents who have a child with Recessive Dystrophic Epidermolysis Bullosa.

The importance of movement

Most people move their joints fully every day and don’t hold them stiffly unless they are hurt-in EB there is often blistering (with scarring) of the skin near and around the joints which can stop them from moving fully. This, in turn, makes the joints stiff. This is why movement of the joints is vital to help prevent movement from becoming permanently lost.

We are all guilty of trying to prevent our loved ones from being hurt. It is particularly difficult for parents of EB children not to wrap them up in cotton wool and stop them from being adventurous. But mobility is absolutely necessary for these children. They need to keep their joints mobile to prepare them for all of life’s activities.
 

Babies

Babies should be put on their stomach to play (PLAY ONLY NOT SLEEP!!!). This is a good position to learn movement, and it helps prevent tightness developing at the hips and knees

Contractures

Because of scar tissue at the joints, muscles and other soft tissues shorten around the joint which in time can permanently lose some of its movement. If this continues, they can worsen and may make certain activities difficult or even impossible. If you notice your child being unable to fully move any of his joints, it is a good idea to encourage him to move to its limit and then repeat asking him to go as far as possible each time. When done early, he can get back to normal, avoiding further stiffness.

Walking

It is important for your child to walk a short distance each day (if old enough) usually regardless of blisters on the feet. When they are very sore, encourage the use of tricycles or other activity toy to move independently.

Swimming

This is a lovely form of exercise. By encouraging your child to participate early he will be allowed to compete on equal terms with his peers. Make sure to go armed with plenty of moisturizers for afterwards.

When to exercise

As soon as little stiffness is noticed it is a good idea to begin. Exercises are best done several times a day if there is stiffness, if not, allowing the child to be generally active is good.

Which exercises are most important  

  • All children with EB should be encouraged to lie on their stomach daily.
  • Mouth exercises are of benefit to people with RDEB as most have some tightness. They should be done each day with the care of teeth.
  • Hand exercises should start immediately. 

Exercises

Hips

Hips often stiffen, particularly if a child sits for much of his time. Prone lying each day helps stretch them out. Lie on front. Lift right leg straight behind. Repeat with left leg.

OR This same exercise can be done standing holding on to something for support-such as a chair.

Knees

Knees too can lose flexibility and if bent for long periods can result in an inability to straighten fully. Prone lying will help to prevent this. Lying on back- Tighten muscles hard and pull feet “up” at same time count to 5 slowly.
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then Straighten one knee fully and tighten. Lift that leg into the air, then lower. Repeat with other leg. (NEVER do both legs together as this can lead to back problems)

OR Straighten one leg when sitting on a chair and hold for the count of 5. Repeat with other leg. Make sure the chair has a back and that your child does not cheat by leaning back (holding on to the front of the chair with his hands may stop this)

then Bend right knee up taking the heel toward the bottom. Repeat with left knee. This exercise can be done lying on one side if for some reason your child cannot lie on his back.

Mouth

Most children with RDEB develop tightness of the mouth stopping them from opening it fully. The tongue can become immobile too. This makes dental care more difficult. It is important to combine these exercises with daily teeth cleaning (which is so essential). Stick the tongue out as far as possible and move it up and down and side to side. then Open the mouth as wide as possible (saying “eee”).
If your child is sensible he can gently pull the corners sideways with a finger at each side to give a gentle stretch. then Open the mouth as wide as possible to form an
“o”

if your child’s mouth cracks, use a little moisturizer or a lip balm before starting exercises.

Neck

If the neck stiffens, these exercises help stop this from happening. Turn head to right fully and then to left fully keeping shoulders facing forwards.

then With face looking straight ahead, take the left ear towards the left shoulder-Repeat to right.

then Look up to the ceiling and then down to the floor.

Shoulders

Because clothing frequently causes blisters and dressings are difficult to keep in place, children often avoid reaching with their arms above their heads as it pulls or clothes rub. This can lead to permanent tightness. These exercises are to be encouraged when reduced movement is first noticed. Lift arms out to side up to shoulder level and then to ears and lower.

then Lift arms forwards and straight above head with elbows straight.

Elbows

Elbows too can become a little difficult to fully straighten or palms turn up. Bend elbows and touch shoulders, then straighten with PALMS UP. Check that the wrists can move fully up and down.

Hands

RDEB sufferers experience particular problems with joints of the fingers and hands. Regularly check that your child’s fingers will fully straighten. This can be done by putting them flat on a table. If they have become a little bent or are being always held bent start daily exercises. The webs between the fingers can begin to creep up towards the tips in this condition, exercises cannot stop the webbing, but dressings and hand wrappings seems to help. With the fingers a little apart, straighten the fingers fully. Check that they are completely straight on a flat surface.

Feet

Blisters on the feet can discourage your child from walking. Bandages should not be too tight over the tips of the toes so that they can grow properly. Ensure that your child’s ankles are not stiff as a result of habitually walking on its heels or toes to avoid blistered areas. If tightness is noted, this exercise should help. Pull feet up and then push down.