Caregiver Poll Results

Around 2004/2005 a poll was posted on this website to ask various questions such as where they get bandages, if they have help etcetera. Here are the results of that survey.

The vast majority of people that have answered the survey stated that one of the parents is home full time and does all the bandage changes. Others tried to get the State to help, but they exceeded the income requirements (which in many states is 30k a year, no matter how many other children they have). Others tried to find daycare so that the children would have interaction with other kids, but most daycare centers did not want to accept that kind of liability. For those that either had hired help or had the state pay for the help, or had interesting tidbits about the care of their children, here’s what they said…

Here’s what some of the parents have said!

  • We have 2 nurses (for 2 RDEB children) and they both are allocated about 45 hours a week. They can do their whole bath and bandage change. The nurses are paid through the state of Ohio under the Medicaid Waiver program. The nurses go everywhere we go.
  • I have two boys with EB and live in state of NJ we also have a Medicaid waiver program and I was able to have nurses to help. They were allowed to do everything except the IV and they went everywhere with us. My youngest has a nurse to go to school with him in which the school pays for it.
  • We do have a pediatric nurse 35 hours a week that can do limited wound care. When I say limited I mean that she can apply ointments and change bandages. When it comes to draining blisters she will get one if it’s large, but she can’t do feet (they are the trickiest and most painful) and doesn’t get the smaller blisters. Insurance pays for her completely. She also goes to school with him.
  • We have a nurse from the hospital comes once a week to be sure our son has no infections, and a nurse “helper” who comes 4 times a week. They help with the bandages by holding him, I do the bandages and burst the blisters myself. They come 1 hour and a half a day for now. They are flexible. They bring all the bandages, that’s the best part, we really can’t afford those. We live in Canada.
  • We are very fortunate we have grandma to take care of our daughter 3 full days and 2 half days during the week. The other half days she is in pre-school. We pay her $200 every two weeks which is cheap but the most she would take. Our daughter needs are not severe just the mending of falling wounds and blister popping and grandma does great at this. Her preschool teacher has been notified and trained on wound care by me and I supply all of the bandages.
  • I am the sole caretaker of both my EB children. One of my girls is on SSI, and for her many needs, I receive 283 hours a month (California State max of a program called IHSS-In Home Supportive Services) I can choose to have someone else come in and help me, or I can do it all myself. At present, I do it all myself. It works out to 9.5 hours a day every day, throughout the month.
  • We took care of our daughter (JEB) ourselves. I did have friends watch my toddler (EB free) so I could do dressing changes in peace.
  • My son is 17 now. He has RDEB. I have always done the dressings myself with him helping as he got older . Now that he is pretty much a man, he doesn’t want my help. The only thing I do is put water in the tub for him and set out all his bandages and supplies. He does the rest. He is very independent!! He is truly my hero!
  • I do receive 25/week of skilled RN nursing and they are able to do bandage changes and popping blisters but my child still prefers MOM to do it. My child gets SSI and Medicaid through the state of Rhode Island and they pay for the nursing also he attends pre-k and has a CNA that rides the bus back and forth and also cares for him in the classroom, she does not do any bandaging and will take him to the school nurse if he needs any and the school department pays for that.
  • I have a LPN that comes 1 or 2 times a week to give my daughter her bath and do her dressings. She is paid for by Children’s Special Health Care Services, an extension of Medicaid in the state of Michigan. It was hard to bring her in to do this since she was 4 1/2 when she came. At first she didn’t like it at all that someone else was doing it, but now she looks forward to the nurse coming – she gives longer and more fun bathes than I do.
  • We live in the Philippines and we hired 3 certified Nurses, they each spend about 14 hours a day. They can do everything, they trained with us at the hospital so they are as familiar with dressing changes as me and my wife. We pay them about $500-800 a month.
  • Here in New Jersey the school system pays for the nurse and they hire who they want (for school). NJ Medicaid pays for the supplies.
  • Here in Florida the local hospital informed me of a special medical daycare in my city of residence. The State Program from Medicaid and Children’s Medical Services (CMS) pay for the caregiver to come to the house 35-40 hrs a week and for the bandages. I do most of the bandage changes myself at home but I personally trained her nurse on how to do dressing changes. She does full changes if they become soiled or if my child takes her bandages off.
  • My daughter has RDEB and we live in Florida, both of us work but mom only part-time. A Nurse takes care of her, we found her through our Insurance, although insurance only pays for one visit a week, and we pay for one more visit a week out of our pockets. We do dressings every day, but full blown bath and bandages every other day. Our insurance is Aetna HMO, right now they pay for supplies with a $5000.00 out of pocket then they pick up the rest, with one nursing visit per week. The nurse comes twice a week to assist me, it takes us 4 to 5 hours per dressing change. Everything the nurse does, he has to do with me. He is not comfortable doing any of it without me. He is a RN also not a LPN.
  • Our grandaughter, which we care for, has RDEB and we live in Oregon. The State pays me 20 hrs a month to care for her from (HMO) DD SERVICES. Our HMO Insurance pays for the bandages. We get whirlpool treatments 3 days a week, 20 minutes in the whirlpool.
  • We live in Washington State and in our situation both parents work, one part-time. I found our caregiver through a Girl Scout Troop. Our daughter is on SSI and they pick up the tab for the supplies we need. Our caregiver does not do any bandaging.
  • We live in Mississippi and thankfully our insurance is paying for the bulk of things at this time. We are on Medicaid. Mom does all the dressings, baths etc for our son. Before mom decided to stay home from her full time job we had a full time baby-sitter/caregiver, however the extend of care she could do as far as wound care was a quick patch up job and draining of blisters.

If you have any hints or tips for other parents regarding help or how you get bandages, please leave a comment below! Thank you!

Helping Kids Cope

CLICK HERE for the Spanish version

Epidermolysis Bullosa, EB, is a very difficult condition to live with, even with the mildest forms.

These are things of outmost importance in helping your child coping with EB:

  •  Accept the EB and accept your child for who he is.
    This sounds simple, but it’s not. There is a certain degree of loss and mourning when a child is not born healthy, and it takes parents different amounts of time to learn to accept things how they are. This may take months or years, depending on the circumstances. The amount of time will depend on many factors… such as if this is your first child… or last, your age, and who you are and your upbringing. But, remember, the more accepting you are of your child’s EB, the less of a ‘big deal’ you make of things, the happier and comfortable your child will be.
  •  Remember, your child is #1, EB is to be treated separately and “secondly”.
    This is also easier said than done, especially when the children are small and there is so much care involved. But it’s important for the kids to grow up not thinking EB is all they are, because their little brains are working just fine, and they can do so much! Remember, most EB kids are straight-A students!! Skin care is important, as their health and life depends on it, but it is important for their mental well being, to help them be children, and you need to help them enjoy all the things in life a normal child enjoys, to play, to laugh, and to learn, with all the potential and need for joy as any other child.
  •  Don’t be afraid to consider your child ‘disabled’.
    This is one of those things no Dr will ever tell you, and parents are afraid to consider, but it’s the truth. Go ahead, get a wheelchair (it will save your child’s feet on long walks), get SSI (for financial help if needed), apply for help (Dept. Of Developmental Disabilities), get disabled plates (for extra wide spaces to get in and out of the car), it will make your child’s life much easier.
  •  Teach your child to explain EB
    There will come a time where you might be a breath away and not hear someone ask if your child got burned or has chicken pox. But your child heard and wants to answer. What is he going to say? This will happen often once he/she enters school, so, better be prepared. They can say something as simple as ‘I was born with a skin disorder’, or they can even hand out cards that explain what EB is (my favorite Option). Whatever you decide, make sure to instill in your child the notion that EB is not ‘who’ they are, just something they have.

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Coping with Baths/Bandage Changes

Bath and bandage change times are hardly fun times for either the patient nor the caregiver. The kids are scared of the ‘upcoming’ pain and they scream and holler in discontent. My son even hyperventilated-always did. He will do just about anything to get out of it, even take a nap (which he will always resist in taking otherwise).

Bandage change time is probably the only time EB kids get to release their frustrations, using it as an excuse to complain and cry about the whole situation, the powerless feeling of the condition, perhaps how they are treated by society etc. This, however, is not healthy. For kids with RDEB it’s a known fact that hollering and screaming hurts the throat-my son always throws up after a fit.
So, what do we, as parents, do to help the kids cope?

These are some suggestions that came forth from adult EBers.

  •  Diverting their attention AWAY from the wounds and the pain during the bath and/or bandage changes really work, such as watching a particular video, having someone else play with them or read them a story, or have them fiddle with a particular toy. Making a game out of bandage changes has worked with some kids too.
  •  IF your child loves a particular food, try to reserve it for bandage changes. My son *loves* M&Ms and Skittles, so I do not give them to him except during those times where he really needs the distraction. It does not always work, but it’s worth a try.
  •  Try to involve your child in his care as early as possible… the sooner the better. Whether that is letting them take the bandages off, having them hand you a needle or gauze, have them decide what limb to start with, etc. Basically, anything you can do to give them “a say” in what’s going on.
  •  Teach your child breathing techniques. Breathing helps control pain (and I know this one from having gone through 2 labors, lol), and once your child gets the hang of it, it will become second nature.
  •  Have your child pick a soft toy, they can beat, bite, punch, pull or whatever they need to do to channel their pain.
  •  Try saying things like, “I know it hurts.” or “this is going to hurt.” or, “Mommy does not like seeing you in pain but, we need to do this.” Be honest. And tell the child what you are doing as you are doing it. Instead of using the word “I” use the word “We” so, that the child hears he/she is part of what is going on and not just having this stuff done to them. Like, instead of saying “I need to pop this blister.”, say “We need to pop this blister.”. And so forth…

Helpful websites:

If you have any other helpful hints to share, please leave a comment below. Thank You!

Hints From Grandma Shirley

Grandma Shirley holding Nicky

Shirley, grandma and caregiver of Jennifer, an RDEB patient, shares her hints and tips and her knowledge about EB care with us.

Constipation

Hi all, I found this article in a pamphlet put out by dEBra International in their Nutrition section. Thought it might be helpful:

Constipation in babies and young children is often the result of an inadequate fluid intake, due to a reduced intake of feeds and/or increased requirements in hot weather. An EB sufferer with extensive blistering may have fluid requirements considerably above normal.
Constipation can be aggravated by iron supplements. It may also occur for no apparent reason. The frequency with which the bowels are opened is less important that the degree of discomfort felt. Provided the motions are soft and painlessly passed, it is not essential that the bowels are opened every day.
For an EB baby, straining to pass even a moderately bulky motion may cause pain and blistering of the delicate skin around the anus. Fear of pain on passing further motions can quickly lead to withholding the motion and before long a vicious cycle is set up as he becomes more constipated and appetite is reduced.
Because regular bowel movements also depend on a regular intake of food, a poor appetite and irregular feeds can lead to harder, drier motions.

The importance of preventing constipation cannot be overstated. Try to ensure a generous fluid intake i.e. at least 150 ml per kg ( 2 – 3 ounces per lb.) per 24 hours, for young babies who are not receiving fluid from foods. If your baby refuses plain, cooled, boiled water, offer well-diluted fresh fruit juice (i.e. 1 teaspoon juice diluted with 100 ml water) or give ready-to-feed baby juice diluted with an equal volume of water.
If extra fluid makes no difference to the constipation, try adding a teaspoonful of sugar to all baby feeds for several days. Alternatively, try giving the diluted juice from a tin (can) of prunes or the water in which dried prunes have been stewed. Once you baby is taking solids, try to include fruit and vegetable puree daily. From about nine months, offer wholegrain cereals such as Weetabix (English cereal – don’t know what the American version is), and from 10 – 12 months, include baked beans and sweet corn. The fiber in these foods, combined with adequate fluid (about 100 ml per kg. (1 – 2 oz. per lb), will help to keep the motions soft and they will be more comfortably passed. Unprocessed bran should not be given. If constipation persists despite these measures, a gentle laxative may be required. It is important to give this regularly as a preventative measure rather than waiting until he is very constipated. Discuss this with your doctor also.

Eye Abrasions

Here is the information I have learned from Jennifer’s experience with eye abrasions. Please discuss it with your doctor.

Jennifer has had eye “blisters” for many years. Before she came with me they were untreated and she has ended up with scar tissue on her left eye cornea. If this isn’t treated it can cause blindness. I took her to an optomologist who just happen to be familiar with EB, as he worked at St. Jude’s hospital and said there were children there who had EB. We were very lucky to have him. He did a thorough exam of Jenn’s eyes. This is what he told us:
EBer’s have a great tendency to “dry eyes”, as they are mucous membranes. He prescribed “Isopto Homatropine”. This is a drop that you put in the affected eye, and it dialates the pupil and relaxes the eye taking away the pain, and giving it a chance to rest and heal. They should be in a dark room as the pupil is dialated and light will cause damage. About five – ten minutes later eye OINTMENT should be put into the eye to keep them very moist. We use Refresh Plus Eye Ointment. The dosage – one drop three to four times a day – and the ointment as often as needed, even if it is every hour. After the eye begins to get better, use the eye drops, not the Isopto, again we use Refresh or Celluvisc Lubricant Eye Drops (I like this better – it is a little thicker) as often as necessary. When Jenn is on the computer I keep a box of Celluvisc right next to the PC and remind her often to use them, even if the eye feels good.
While she is going through the initial beginning problem it is VERY painful, swollen, red and VERY feverish. I put a few ice cubes in a clean white face rag and she holds that on her eye. This keeps the fever down and she uses MANY ice cubes, as they melt fast from the fever. Both of us dread this problem, as it is so painful for them.
I have no problem keeping her in a dark room, as she usually stays in bed for the three days, and sleeps…. Dr. Tether said the reason this happens is that EB will cause very dry eyes. When they sleep and go through the REM eye movements, their eyes blink and that scratches the eye lid and cornea, which cause the blisters. Also, as we all know they sleep with their eyes open slightly. Also, I keep Jenn’s hair back with a twister, especially at night, cause a little piece of hair that can get in the eye will also irritate the eye and cause a blister.

Prenatal Diagnose

There is now a test that can be done to see if the baby has EB. Then if he/she does, you will have to make a decision that of course none of us even want to think of – to abort or not. You will need to contact Dr. Angela Christiano – email addy: amc65@columbia.edu to get the details. She is the Dr. who invented this test. She is also very active in research for EB at a hospital in Philadelphia, Pa. It has been done and it works. There is another EB mom who decided to have more children and had this test done. Fortunately the baby did not have EB and all went well. Good luck.

Infections and Wound Dressings

A friend, Nancy has twins, 21 yrs, Katie and Kelley and shared this with me. Since it is for the good of all I want to share it also.

Regarding infections: Nancy suggested it is better to rotate topical antibiotics – gentamycin for about 3 wks to a month, then bactroban, and areas with a lot of discharge/seepage silvadene. She feels it confuses the bad bacteria, and I agree.
Some of the antibiotics you may want to mention to your Dr. are: Cipro, Cephalexin, Rifampin, Sulfa, Minocyclene, Augmentin, Vancomyacin. Cipro does not work for Jennifer. Some of these may not work on your EBer. A bath soak with epsom salts – half a quart size container to a tub of water. When there is a very stubborn infection that does not want to respond, try a vinegar bath – about a gallon of vinegar in a VERY full tub of water. Thanks to Nancy for this info.

For those of you anticipating Apligraf surgery. Jennifer had this done, and it worked about 70 % – better than nothing – right. Of course it will slough off as normal skin does, and then the bad gene will produce more bad skin – a catch 22. Here are some other options I have learned about:
We are planning to use one of these new options when the next skin graft is necessary – which appears to be soon – on her chest… : Oasis, Cook Manufacturing Company, Andy Cron, General Manager, 800-468-1379, EXT 3456, Fred Roemer, V.P.,800-457-4448 EXT 204. Mention Gary Cummings from Winfield Labs referred you – I will tell you about that in a minute.
There is a Silicon Gel Sheeting called Duo Dress manufactured to put over scar tissue to make it more soft and pliable, and possibly invisible. The only information I have regarding this is: Mark Dillon, President, BioMed Sciences, www.Silon.com I am not too familiar with this. It was mentioned to me by Gary Cummings and I have not had a chance to research this as yet. When I do, I will get back to all of you.
Of course there is the Ortec product called CCS (Composite Culture Skin), which FDA has just approved for use in the U.S. That is the product I am leaning toward for the next graft Jenn has.
I have been introduced to a wound contact dressing called N-terface this summer. I find it as good in a lot of ways as Mepitel, and cheaper. I am not saying Mepitel is not the best, it is just when you can’t afford it you take the second choice. They make several products and if you contact Gary Cummings, President, Winfield Laboratories, 800-527-4616. They make the N-terface and also Breakaway, which is a pad to go over the N-terface to absorb the excess seepage. Gary will send you samples I am sure. Please tell him I referred you. They are also working on a “vest” to cover a much larger area for those who need it. It is new, being introduced at the next meeting in California next week or two. He is sending me a sample. It is manufactured in China and is VERY inexpensive. These products work for Jennifer very well.
I just feel it important to share this with you all – it may work for your EBer also. Please let me know…. As most of you know Jennifer is just ending the Accutane Study at Chapel Hill – our last visit is Oct. 1 – 3. 2000. The Study will end in November. The results will follow a bit of time later as some are not finished for sometime. It does appear that FDA will approve the 5 year study. Soon as I can I will post from the results from the start of the Study in March to the end.

Blisters on the bottom

For Blisters on the bottom I suggest using a product called MEPILEX. It does have a sticky side which goes onto the wound. It will not stick to the wound, but will around the “good” skin. It comes off very easily with no damage. It will absorb the “seepage”, and can stay on until it is completely saturated. If it becomes saturated it will come off by itself. Instead of wrapping between the legs like a “vest”, we now use HYAFIX. It is the only type of tape we can use. It will come off very easily if you take a 4 x 4 (non-sterile), saturate it will alcohol and ease the tape off. The little bit of alcohol will not hurt the skin, just don’t get it on the wound. Place the MEPILEX on the wound and surrounding area about 1″, place about 2 – 4 x 4’s (sterile) over that, take the HYAFIX tape and criss cross it over the 4 x 4’s, just enough onto the “good” skin to hold it in place. Leave it on until the seepage comes onto the 4 x 4’s. You can also put your topical antibiotics onto the MEPILEX. Just keep it away from the outer edges so it will stick to the skin. If you put the anti close to the edges the MEPILEX will slide off the wound. The MEPILEX will act like a cushion and protect the wound. Try it, you’ll like it… 🙂 Also, for those of us who do not use MEPILEX, or prefer to use something else: in the past we have also used VISCOPASTE (this is gauze saturated with zinc oxide, and also MEPITEL. In this case, we cover the MEPITEL and/or VISCOPASTE with a sort of sponge called ALLEVYN, before we put on the 4 x 4’s. This absorbs the exudate. This is also a good method. The VISCOPASTE was especially effective. Zinc is good. There is another product called BREAKAWAY, that has a built in padding. It is also good. The diaper will also hold the bandage in place. Also, if it gets wet or dirty, just change the 4 x 4’s, that is of course if the MEPILEX is dirty also. Gee, I don’t mean to insult your intelligence.. Well I hope this helps. When Jenn was a baby and until she came with me in 1997, she had this problem. Now she has the scars to prove it… We do keep a nursing ointment (we call it BLUE GOO) on the scars if they look like they are starting to breakdown, and it works…. another thing that we have found as far as this is concerned has to do with bedding because it can be quite uncomfortable to sleep. We have found that babies sleep better on a sheepskin blanket.

Jennifer’s Experience w/Anemia

Jennifer had been going to Chapel Hill, NC to the EB Registry as she was on the pilot study for Accutane. We go every three months via Angel Flights. They do thorough lab reports on her each visit, and she is seen by Madeline Weiner and Dr. Jo-David Fine. In June, 2001 we all noticed that Jenn’s RBC (red blood count) was really dropping. At that time her Hematocrit was about 26, which is low. Originally it had been at 32, which was do-able. Normally the Hematocrit should be between 35 – 45. However, in EBers trends are very important. For example, when Jennifer’s Hematocrit was at 32, she did very well, but the Hematologist wanted it higher as there is a “leak in her bucket”. Will explain that later.

We got in touch with her doctor here in Indialantic, Fl. Doctor suggested we have her kidneys checked. Kidneys produce the erythropoietin which stimulates the bone marrow to make the red cells. The kidney doctor did a thorough exam and found the kidneys are functioning properly but we needed to see if they were producing the Erythropoietine. He referred us to a Hematologist and we saw him sometime in July. When they drew blood the first time her Hematocrit was down to 23. Very bad. The Hematologist ordered a “mid-Line” and she was given intravenous iron (ferritin) every day for two weeks. A mid-line is actually a line that is introduced through the blood vessel and goes up the arm to the shoulder area. A pic-line is the same thing, except it goes past the shoulder area and down close to the heart. With a pic-line you also need an x-ray to be sure the line is not too close to the heart. This is all done on a out-patient basis. You should not have them draw any blood from the mid/pic-line as it will clog up the line and cause a blood clot. This is not a blood transfusion. However, a blood transfusion works for some EBer’s; in this case it was not what Jennifer needed. This was done at the I.V. Therapy Lab. At the end of two weeks they drew blood again, and the Red Blood Count (RBC) had gone up, slightly, but not enough. The Hematologist then prescribed Procrit Injections (Erythropoietin) to be given – I believe this was done once a week, with blood drawn every two weeks. Procrit is a pharmacological erythopoietin and is usually used in patients with depressed bone marrow function. This is not the usual cause of anemia in EB patients and a hematological work-up should be done prior to treatment with this drug. Other causes of anemia will not be corrected with Procrit and there are risks involved with this treatment. Jennifer’s RBC and Hematocrit was coming up and the doctor suggested we continue once a week with the Procrit Injection, with blood drawn once a month to keep checking the hematocrit and rbc. Jennifer’s hematocrit is now at 37.5, which is good. There is a pronounced difference in Jenn’s energy level and her skin is really looking good. Jennifer still gets Procrit every Friday, and we see the Hematologist and have blood drawn once a month. A great description of why Jennifer may need the Procreit for a long time – maybe not every week – but every two weeks or once a month – picture a large bucket full of liquid, with a tiny pin-hole leak in the bottom of the bucket. If you do not take measurements to keep the bucket full, eventually you will empty the bucket. EBers bleed constantly – as their wounds always have some seepage, being blood or some other liquid, usually blood. So it is very important to keep that “bucket full”. 🙂

As we all know, each EBer is different. This was extremely beneficial for Jennifer and that is why we want to pass it on. It may be a good thing for your EBer. Anemia is very prominent in EB and it is so important to keep up their Red Blood Count and Hematocrit.

Anemia is caused by a various number of different causes. First you must establish what is the reason for the Anemia through a Hematologist, and then follow the advise of that doctor.

Cradle Cap

We soak the scalp for 15 minutes with Acidic Solution, and it consist of 8 O.Z of saline and 2 O.Z of White Vinegar. And then we put the Olive Oil on the scalp and below is how we do that.

We bought Olive Oil and I part Jenn’s hair where I can see the areas that are dry and I take a 4×4 sterile pad and puts some Olive Oil on it and rub it GENTLY to massage the Olive oil in her head & hair. We put it in my hair every day. If you want it to work leave the Olive oil in the hair for about One day to Two days.. Check for Order, this will give you an idea when to wash the hair or if you can leave it for another day.. If you smell an order you NEED TO WASH hair, and then put the Olive Oil in the hair again.. The scabs that form with come off, but you need to help me a little bit.. If it doesn’t come off will just a LITTLE force then leave it and put Olive Oil on it.. This method has worked for me and I am sure that it will hopefully work for you all.

Love to all