Resources

There are many resources to assist with various things needed by EB families or other families in need. Here’s a list of some we’ve found, please let us know if there are others we might have missed by entering the link or links in the Facebook plug-in below this post! Some of these links are simply link of interest.

  1. Medicare list of contacts
  2. Children’s Defense Fund – The Children’s Defense Fund Leave No Child Behind® mission is to ensure every child a Healthy Start, a Head Start, a Fair Start, a Safe Start and a Moral Start in life and successful passage to adulthood with the help of caring families and communities.
  3. Coalition of Advancement of Medical Research – The Coalition for the Advancement of Medical Research (CAMR) is the nation’s leading bipartisan pro-cures coalition. Comprised of 100 nationally recognized patient organizations, universities, scientific societies, and foundations, CAMR’s advocacy and education outreach focuses on research toward developing better treatments and cures for individuals with life-threatening illnesses and disorders.
  4. Insure Kids Now! – Your child or teen may qualify for no-cost or low-cost health insurance coverage through Medicaid and the Children’s Health Insurance Program (CHIP). Many parents may also be eligible.
  5. Centers for Medicare & Medicaid Services – CMS Provides Health Coverage for 100 Million People through Medicare, Medicaid, and the Children’s Health Insurance Program. And with health insurance reforms and health care exchanges, we are improving health care and ensuring coverage for all Americans.
  6. Habitat For Humanity – Habitat’s vision: a world where everyone has a decent place to live.
  7. WAP: Weatherization Assistance Program – The U.S. Department of Energy’s Weatherization Assistance Program (WAP) reduces heating and cooling costs for low-income families, particularly for the elderly, people with disabilities, and children, by improving the energy efficiency of their homes while ensuring their health and safety.
  8. Pro Bono & Public Service
  9. Help from Disability.gov
  10. State Pharmaceutical Assistance Programs – Includes seniors, disabled, uninsured and others.
  11. Patient/Drug Assistance Programs:
    Partnership for Prescription Assistance – www.pparx.org
    RxAssist: Accessing Pharmaceutical Patient Assistance Programs – www.rxassist.org
    RxHope: The Heart of the Pharmaceutical Industry – www.rxhope.com
    Free Medicine – http://freemedicine.com/
    TogetherRX – http://www.togetherrxaccess.com
  12. The National Financial Resources Guidebook for Patients: A State by State Directory of information for patients seeking financial relief for a broad range of needs including housing, utilities, food, transportation to medical treatment, and children’s resources.
  13. Receive Help for Food, Bills, Clothing etc… http://modestneeds.org/
  14. Your local Salvation Army http://www.salvationarmy.org
  15. American Public Human Services Association – Internet site that list social service agencies by state
  16. Feeding Children Better – Do you find yourself having to choose between rent and food? Not eligible for food stamps? This site lists food banks throughout the USA if you click on the “get help” button.
  17. Giving Board – Whether you are in need yourself or wanting to help others, this is the place to be! Giving isn’t limited to monetary help. Words of encouragement, prayers and the ability to give others hope are equally important. We invite you to become part of our “family”, whether it is as a lurker or by chatting and letting us get to know you better.
  18. Feeding America – This website will give a listing of food banks in your state. It is very helpful, even if your income is too high for federal food help the local food banks will help.
  19. Administration for Children and Families – The Administration for Children & Families (ACF) is a division of the Department of Health & Human Services. ACF promotes the economic and social well-being of families, children, individuals and communities.
  20. Newborns in Need Inc. – Help with baby clothes and other items.
  21. Telephone bill assistance info – Provides helpful information for US citizens about applying for grants for lowering your phone bill. Must be low income.
  22. Women Children Family Service Charities of America – WCFS is a coalition comprised of many of America’s finest charitable organizations dedicated to meeting the needs of women, children, and families in America and around the world. Our members provide real solutions to real problems, and act as a force for change with law and policy makers.
  23. Angel Tree – Helps Families at Christmas who may have a spouse in Prison

How to find Individual State Programs

These programs help low-income families (usually) or/and children with special needs.

When you click on the link for YOUR State’s Department of Public Health, search for (if it’s not immediately evident) ‘Children Health’ or ‘Children Medical’ services.

If the NAME of the State Program and information it is known, that information will be below the state and link.

Another resource for all states is your local Social Security Office. If a child can be declared disabled, they would be covered by the local or state Medicaid program.

**************

State and Local Health Departments

Want a link added? Do you have the NAME or any information about a Children’s State program that is not listed? Are any of these links old or outdated? PLEASE LET US KNOW!!! Just fill out the SURVEY below and I will edit the list.

THANK YOU!

Fecal Hoarding and Constipation

Written by an Anonymous RDEB Patient

I have had these issues since birth and have tried a myriad of medications that have been prescribed or suggested by prior physicians. The list includes mineral oil, senna products (liquid and herbal tea) ex-lax, milk of magnesia, dulcolax, docusate sodium, Metamucil, Miralax, Benefiber and many others that I don’t remember the names of. Ultimately, none of them worked in a manner that was consistent, or the side effects were to severe so outweighed the benefits, or they didn’t help at all.

For anyone who may not understand why constipation and fecal hoarding is such a prevalent problem for EB patients it is because our skin does not have the same elasticity that others do. Therefore, during a bowel movement, the skin of the rectum/anal area is stretched which causes blistering, tearing, splitting, shearing and detaching of many layers of skin. This is EXCRUCIATINGLY painful.

Generally, what happens is the fear and anxiety of having a bowel movement causes a behavioral habit called fecal hoarding which is when a patient deliberately avoids going to the bathroom when the body is trying to naturally expel the matter in an attempt to avoid the pain. What happens then is the matter becomes impacted and the intestines become stretched as more and more matter becomes impacted, thereby causing the size of the feces to become too large to be expelled naturally, as the skin simply would not be able to accommodate its passing. Because of the impaction, the matter becomes very hard and gas builds up in the body, causing painful cramping, as the gas cannot be released from the body, as it has no room to move past the matter.

At this point, the only thing that is going to alleviate the impaction is a series of laxatives, stool softeners and enemas. This is a painful and humiliating process that can sometimes take a few days to complete. The best way to keep this from happening is to make sure that you are ingesting enough fiber and water to allow the fecal matter to be soft so there is less pain when having a bowel movement.

What can also happen is some of the matter can become fluid and is able to slip past the harder matter so there are small bowel movements that are generally very messy but do help relieve some of the painful pressure of the matter that is bearing down on the inside of the intestines and rectum. This can give a false sense of security as one may think that they are having a bowel movement regularly; however, the case may be that only a small amount is being released during these loose movements.

It is a trial and error process of trying different medications or fiber supplements. Either they don’t help, or they help a little but not enough or you spend all your time running back and forth to the bathroom, an sometimes not quite making it there in time, which is humiliating for a person of any age.

When I was in my late 20’s I decided to try something different as I was frustrated by the lack of control I had over my bowel’s and it angered me that this skin disorder was making such a simple action so complicated. I decided to try a more natural approach of simply increasing my fiber and liquid intake. I began eating prunes and drinking more water every day, and it worked! After a day or two, I began having regular bowel movements that were very soft, and while it was still painful, it was not as painful or traumatic as I had been enduring.

After a few weeks, I got tired of prunes so I started looking for other foods or products that would have the same effect. The other benefit of eating foods high in fiber was that there were no side effects like when I was taking the medications.

Please remember that I am simply relaying information that helped ME. I strongly recommend discussing any diet change with your or your child’s doctor before making any serious changes. Also, continue taking medications until discussing any changes with the prescribing physician.

Below is a list of high fiber fruits and veggies and snacks

Please remember that FRESH fruits and veggies have much more fiber, vitamins and minerals than canned.

Prunes  – Peaches  – Cantaloupe  – Watermelon  – All green veggies
– Plums – Nectarines – Grapes – Grapefruit – Honey Dew Melon –
Apricots – Apples – Pears – Oranges – Mandarin – Oranges

  1. Salad (darker lettuce and baby spinach has more fiber, vitamins and minerals than iceberg lettuce)
  2. Pretty much ALL forms of BEANS are high in fiber (they are effective although they do create more gas)
  3. Whole wheat bread (Brownberry whole wheat has 3 grams per slice and is the best tasting whole wheat I’ve found…It has a sweet taste and is yummy : )
  4. Weight watcher brand muffins and dessert cakes (generally found in the bread aisle)
  5. Slim fast brand muffin bars
  6. Polaner all fruit with added fiber
  7. Shredded wheat cereals (Fiber One brand has a shredded wheat cereal with a higher fiber content than regular brands)
  8. Oatmeal (several brands have many flavors with more fiber or added fiber)
  9. Fiber One pop tarts
  10. Fiber One brownies
  11. Boost Plus supplement shakes

For infants and those that are not crawling or walking, it is helpful to massage the abdomen as well as pushing the legs up towards the abdomen area (as if riding a bicycle). This helps move the fecal matter through the intestines. If the matter is firm or hard it can hurt as it moves through the intestines (cramping) as the tissue is sensitive. It best to limit milk intake as it does not provide as much hydration as water or juices. You can water milk down or add ice to it and add a touch of sweetner to make it taste better. I recommend using stevia or Agave Nectar sweeteners as they are all natural, do not raise blood sugar levels or cause chemical reactions (mood swings, hyperactivity etc…)

It is imperative that you increase your water intake (fluid) if you increase your fiber intake as the body pulls the water into the intestines to allow the fecal matter to soften. If you don’t drink enough water then your body is not hydrated enough to complete this process and the matter will become harder.

Below is a photo of the rectum of an RDEB patient. It depicts the trauma caused to the rectal area by the stretching of the skin from passing fecal matter. There is a permanent tear in the rectal wall from repeated ripping of the tissue during bowel movements.

 

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….

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

Medical Care for EB patients

Bathing

Bathing can be a good way to cleanse wounds because you can add different things to the bath water. Some patients bathe while others shower or do a combination of both. These are some of the things people use in their baths to help with infections:

Aveeno Oatmeal bath – Aveeno Daily Moisturizing Bath with natural colloidal oatmeal is a natural cleanser and helps with dry itchy skin. This product needs to touch the skin, it will not help if the patient takes a bath with the bandages on.

Domeboro – Domeboro astringent solution helps dry out oozing, infected wounds. If you don’t want to dry out all of your wounds you can make a compress only for certain wounds.

Bleach – Use one capful for a normal size tub.

Chlorine – Hot Tubs/Spas are wonderful also and used often because of the helpful effects of chlorine.

Vinegar – I am not sure of the suggested amount to add to a bath so please consult a physician first.

Special Bleach and Vinegar Combination – I am also not sure of the suggested amount, and you should be very careful with this method, however I’ve heard it works wonderfully for infections.

Showering

Some patients cannot shower because it may be too painful to have the water hitting open sores. Some things that are helpful when showering are:

Shower chairs – There are different kinds of shower chairs, some with backs, some without, some are padded, some are not etc. They are useful so you don’t have to worry about having to stand up the whole time. They’re easy to clean. You can put a soft towel on it for extra padding also.

Adjustable shower head – This is a nice thing to have because it allows you to adjust the pressure of the water and how much you want to come out. Some even have a setting that mists you. They can be expensive depending on the one you choose but worth it.

Loofah – This may be easier to use than a bar of soap or constantly squeezing out liquid soap. You can just lather it up and gently wash areas or just squeeze soapy water over sores.

Preparing for a bath/shower

It’s easiest in most cases to set out all of the bandages, ointments etc. that you will be using before you bathe so it is ready for you when you get out. If you have long hair it’s best to put it up to avoid it sticking to wounds. A shower cap works well if you’re not planning to wash your hair.

Bathing – If you’re bathing, prepare bath with any solutions if necessary. You may want to put a soft towel on the bottom of the tub to sit on. A loofah also comes in handy for gently washing healed areas and you can also use it to squeeze water over sores to rinse.

Removing bandages – Some prefer to remove all bandages before getting in the tub, while some prefer to soak first to allow their bandages to be removed easier. Some also bathe with bandages on and remove them and re-bandage one at a time afterwards to avoid any trauma to the skin.

Blister Popping – It is definitely important to cut and drain any blisters, however wait until after the shower/bath because the blister fills up with water while bathing and that can make it larger and cause pain.

Soaps – Soaps aren’t necessary, however you can use mild, non-drying soaps such as Dove.

Washing Hair – Some prefer to wash their hair separately, on a non-bath/shower day. If the patient is prone to a lot of FLAKES, a shampoo/conditioner such as Head and Shoulder works wonders.

When You’re Finished

Getting in and out – It is important to have hand rails or something or someone for support to grab onto when getting in and out of the tub. Non-slip rugs and mats are important also.

Drying off – Remember to pat dry, do not rub. The bigger and softer the towel the better. Make sure the towel you are using is free of hair and tiny fuzz balls etc. because they can get stuck in wounds. You can also use a hair dryer or small electric heater to help dry off. Some use an electric heater to keep warm during bandages.

*****

With Recessive Dystrophic EB, some type of surgery or procedure is inevitable, whether it be a G-tube placement, hand surgery to release fingers, esophageal dilatation, dental surgery, blood draws, transfusions, etc. It all can be scary especially when most doctors and nurses do not know the specific things you can and can not do when treating an EB patient. The following is a checklist to remember some things to tell the doctors, and suggestions on how to do things in an EB friendly way without causing trauma to the skin.

Blood Pressure and Temperature

Blood Pressure – A child sized blood pressure cuff may be needed. The blood pressure cuff should NOT be placed directly on the skin. Put something soft underneath such as your sleeve, a washcloth, webril or cast padding. Also make sure they do not put it on too tight.

Taking Temperature – Some patient take their temperature under the tongue however many with RDEB have fused tongues so it must be taken another way. If you do get yours taken under the tongue I would make sure to not let them place the thermometer themselves to avoid them poking you too hard. You can also have them use an ear thermometer, but remind them to be very gentle, sometimes they push down on the ear too hard. You can also get it done under the arm with a regular thermometer, although that can tend to rub or stick to the skin.

Blood Draw and IV Placement

Before Drawing Blood – First make sure the person drawing the blood knows that absolutely no adhesive can be used on the skin so this means no band-aid afterwards. Also tell them to be very gentle when touching the skin because the skin can literally come off. Don’t be afraid to tell them when they’re being to rough! Tell them to gently dab the alcohol on the skin, do not wipe.

Drawing Blood – A tourniquet can be used ONLY if a soft material such as your sleeve, a washcloth or webril is wrapped around the arm underneath it. Often times a family member may be asked to gently squeeze the arm instead of using a tourniquet. Ask for a butterfly needle if they aren’t already using one, it is the smallest they have.

Removing Needle – Have them use a small piece of gauze over the site while removing the needle, then you can apply some pressure with the gauze until any bleeding has stopped.

Before IV Placement – Again, make sure they know that absolutely no adhesives can be used on the skin. If possible, ask for a 24 gauge needle, I believe it is the smallest IV they have but they can’t always use that one. Get materials ready to secure IV once it is in. Tell them to gently dab the alcohol on the skin, do not wipe.

Securing IV – There are different ways to secure an IV without adhesive. It’s up to you. First have them put a small piece of gauze underneath the IV so it doesn’t get pushed down onto the skin. I usually bring my own Conform wrap and they wrap around semi-tightly and tape the gauze to itself to secure it. Remember to tell them tape can be used only if it does not touch the skin. Another thing you can use is Coban. It sticks to itself but not the skin and can be used to secure an IV. The IV can still come loose so avoid too much movement or bending of the arm (assuming that’s where the IV is)

Removing IV – Gently unwrap or cut away gauze and make sure someone is holding the IV in place so it doesn’t jiggle too much and cause discomfort. Once it’s all unwrapped they can place a piece of gauze over the site and pull out the IV while you apply some pressure with the gauze until any bleeding stops.

Before Surgery

Anesthesiologist – If at all possible, speak to the anesthesiologist before the surgery to go over the Dos and Don’ts.

Eyes – Since the eyes are very sensitive to begin with and anesthesia can cause them to dry out even more, it is important to remember to put a lubricant in the eyes before hand and ask them to reapply more a few times during the surgery. Usually any gel type lubricant works. Also obviously remind them that the eyes can not be taped shut! A soft, damp cloth or vaseline gauze can be placed over eyes instead. If “blow by” oxygen is used, avoid having it blow across the eyes.

Lips – You may also want to put some ointment on the lips and if they’re working in the mouth for dental surgery or dilatation you may want to remind the doctors to apply more throughout the surgery to avoid blistering.

Bedding/Moving – Move by lifting, NOT sliding onto OR bed. To make the hospital bed and operating table softer, egg crate or sheepskin can be used. Use them as a hammock to lift onto another bed.

Versed – A drug called Versed can be given before surgery to essentially make you forget everything that happens. This is used mostly for children who are very upset, scared and anxious about the surgery and getting put to sleep. I’ve had this once and it did make me forget ever getting put to sleep, however the effects last even after surgery and most people will wake and up go back to sleep continuously for several hours until it wears off. It also makes you a bit “loopy”. But it is a lifesaver in some cases to ease the fear and anxiety.

Anti-Nausia – Medication is usually given to help reduce nausea and the chance of throwing up after surgery, although it may still be comman to be nauseous or throw up afterward.

Miscellaneous

Instruments, Gloves and Face Masks – All instruments placed into the mouth should first be generously lubricated with a water based lubricant. Gloves should be lubricated with vaseline whenever possible. Face masks should also be lubricated.

Heart Monitors/Leads/Probes – If monitors are needed FIRST cut off the adhesive portion of ECG leads, probes, pulse ox monitors etc. The monitors can then be secured using webril, koban or any other type of gauze or tucked under netting. Pulse ox monitors can also be clipped onto thumb or toe.

After Surgery

Eye Abrasions – Even when precautions are taken, it can be comman to wake up from surgery with an eye abrasion. You may want to have some eye medication on hand and you may want to have a patch or cloth to lay on the eye or wrap gauze around the head to keep a patch on. Keep the room dark if possible.

Rebandaging – If any bandages were taken off during surgery and not put back on you may want to have some bandages ready to rebandage right after surgery.

Cleaning Up – With dental/mouth surgery there may be dried blood etc. around the mouth. A washcloth wet with warm water can be used to gently clean it off. Ointment may need to be applied to the lips also.

Throwing Up – Anti-nausea medicine can be given before surgery but it is very comman to still be nauseous and even still throw up. So having something nearby at all times to throw up into is important.

Specific Procedures/Surgeries

Barium Swallow – Be sure to pad the table with blankets or sheepskin etc. You can use a straw to drink the barium.

Dental Surgery – Because RDEB patients generally have very small mouth openings, dental surgeries can be difficult. Make sure all instruments going into the mouth are generously lubricated and the mouth and lips should be continually lubricated throughout the surgery. If a lot of teeth need to be pulled or worked on, it may be best to do it in stages (multiple surgeries).

Blood Transfusion – Blood transfusions are generally a simple procedure although they can take several hours depending on how much you are recieving. Read the IV Placement information above for more information.

Iron Transfusion – Premedicating with Benadryl may be important, they will do this at the hospital. Reactions of iching, hives or swelling is not too uncomman when getting an iron transfusion. Be sure to tell the doctor of these kind of reactions. Read the IV Placement portion above for more information.

Epo Shots – Epo shots can be given the same way as anyone else. Usually they are given on the thigh or stomach. Instead of wiping the alcohol onto the skin, gently dab.

EKG – Since the sticky monitors can not be plced directly on the skin, for an EKG you can have them first cut pieces of gauze, such as Conform, and get them wet. Apply them to the areas the monitors will be placed and put the monitors directly onto the wet gauze, making sure the sticky part does not touch the skin. Someone may need to hold each monitor in place. Dry gauze will not work, they need to be damp.