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

Helpful Words for New Parents

Taken and adapted for EB from the Down Syndrome for relatives page website

I cannot stress enough how important it is for family to support the new parents at this time. This is a frightening time for the new parents and they often wonder how their family will treat the new baby. Showing how much you love them and their new baby will help to alleviate these fears; “gently” pick up the baby, fuss over the baby, play with the baby. Sometimes you may not know the right thing to say or what you say, is in fact, the wrong thing.  The following suggestions are based upon the input of many parents of children with EB and Down Syndrome.

Things NOT to say
These are the things that parents have said really upset or angered them:

“I’m sorry”, “Poor___” (either you or the baby) or any form of pity.
Pity is not what parents want or need. What they need is love and acceptance of their baby.

“God gives special parents special children” or any variation.
The new parents probably don’t feel very special right now. Also, some parents may be a little mad at God. Trying to make them feel better with words like these might be appreciated by some parents and not by others. It is best to avoid this.

“Do they know how serious it is?” or any variation.
This is a demonstration of a lack of knowledge about Epidermolysis Bullosa. Some parents may be angry and want to reply with, “How serious is it? Well, every part of his body can and will blister… he can die at anytime… is that serious enough?”

“You are handling this better than I could.”
This is an invitation for the new parents to say something like, “No, you would be wonderful.” Suddenly, the conversation has switched to you instead of the parents and their new baby. Plus, you don’t really know how the new parents are handling it, do you?

 Things TO say
These are the things parents have found comforting or made them feel good:

“Congratulations.”
They just had a baby! What better response to show that you love them and their baby than to say congratulations. It made us feel like ‘normal’ parents when someone said that to us. If the hospital allows it, a bottle of champagne could be greatly appreciated.

“He/She looks just like you.”
Or similar comments you would have said to the parents if the baby would have been born healthy. This tells the parent that you can see the child behind the disability.

Friends and family who actually ‘did’ something like read about the disability (or find information on the web!)
This really means something to the new parents. It shows love and concern for the baby.

Offer to baby-sit.
It is a fear of the new parents that their family will not accept the new baby. Especially with babies with EB, most people are afraid to hurt the child. By saying something like, “Well, when are you going to let me baby-sit?” you are showing the new parents that you want to be part of the baby’s life. This will be a great relief to them. Of course, due to the fragile nature of and EB child, you will probably never baby-sit this child, and it’s important that you do not get offended by this. But knowing that you care enough to offer means a lot.

“He/She will do fine.”
The new parents are probably pretty worried. They might not know much about EB, and they may be concerned about possible medical problems. Having a positive attitude will rub off on them. They don’t need pessimism or negativity from their loved ones.

“We’ll all learn from him/her.”
This is another good way to show that you intend on being part of their lives. After all, how can you learn from their new baby if you are ashamed of him/her? Their new child will be an opportunity to learn about love, acceptance, and respect for the disabled.

“We will always be here to help.”
Another very good way to show that you are going to be there. Let the new parents know that you intend on being part of their lives. But don’t just say it without doing nothing afterwards, actually plan how you’re going to be there for them, otherwise they are just meaningless words, and the new parent will resent it.

 FOR DOCTORS/NURSES

Disability Etiquette: Some Do’s and Don’ts-from the Parents’ Perspective
Tips to Make the Office Visit Better for Everyone

The following suggestions have been compiled from parents of children with special health care needs across the country:

DO respond positively to our children in your initial interaction. Tell us they are cute, sweet, cool, that they are wearing a neat hat. Let us know that you value them even if you can’t cure them.

DO ask questions of our children, even if the child can’t speak. Show your recognition and respect. Don’t worry; his parent will find a way to facilitate the communication.

DO look at our children when you are talking to them. Please make every effort to establish a personal connection.

DO get down to our child’s level. For example, if our child is in a wheelchair, kneeling or sitting down will help make him feel more comfortable.

DO realize that sometimes parents are very tired of telling their story over and over. Even though you may have your own personal medical interest, don’t ask if you don’t need to know. Be understanding if the parent shows frustration at telling the child’s story yet again.

DO notice and be attentive to brothers and sisters. They have grown up in a situation where their sibling, by necessity, has received lots of extra attention. They don’t understand why they shouldn’t be just as entitled to a sticker or balloon.

DO be aware that our grief and sadness may recur at any time and often does at transitional times-beginning pre-school or high school, the day of the senior prom, an anniversary of another loss. It’s never resolved entirely.

DON’T ask questions or raise issues that could be painful for us (and our child) in front of our child. If you need to understand more about the birth history, for example, find a way to talk separately with the parent. You should never assume our child doesn’t understand or doesn’t have feelings.

DON’T refer to our children by their diagnoses (e.g., “EB kid,” “Downs” kid); it’s a good habit to develop no matter with whom you’re talking.

DON’T judge parents; we are doing the best we can often under difficult circumstances. If we are impatient or rude, it may be that we are under particular stress.

DON’T be surprised if we need instructions, procedures or explanations repeated several times, especially if the information is complicated, upsetting or unexpected. We are often thinking about a million other things and we are trying to synthesize what you have to tell us at the same time.

 Silva, TJ, Sofis LA, Palfrey JS. 2000. Practicing Comprehensive Care: A Physician’s Operations Manual for Implementing a Medical Home for Children with Special Health Care Needs. Boston, MA: Institute for Community Inclusion/UAP, Boston