Valtrex Voyage - Epidermolysis Bullosa Simplex

Day 15 - I need to push it.

noreply@blogger.com (Joan Q. Public) — Thu, 16 Apr 2009 15:06:00 +0000

I spent the day yesterday, again in my ratty old mock crocs, sockless. I forgot to put on my pedometer, so I didn't count steps. It was cold and dreary yesterday!

I walked a short distance down the street (Pugnacious pup in tow, sporting his new yellow harness) to pay my water bill. It was cold, and as I said, not a long way - but I got no burn and no blisters. It occurred to me that night (I left the shoes on even when I didn't have to) that my feet are not sweating the same way they once did. In the past, these crocs have gotten all icky/slippery FAST on the inside due to excessive sweating. Even though they are well ventilated, they are plastic, essentially; and that's why, before recently, I always wore them with socks.

I still haven't gotten into doing any real, pointed walking yet. Time constraints have interfered with my plans for that big time. I must get on it - the JDRF walk is coming up fast, and if I can't make the distance I want it to be because of my skin - not my total lack of conditioning!! Heh.

Perhaps the good weather will be more conducive to my getting up and out. It'll be cool this week, but sunny - perfect time to get going. Onward!

Day 13, 14 - Yuck.

noreply@blogger.com (Joan Q. Public) — Wed, 15 Apr 2009 00:03:00 +0000

I say yuck, not because of anything nasty going on with my skin - but because of the weather. The teasing sunshine is mocking me, winking through the clouds in between the chilly drizzles. Supposedly, there'll be sun tomorrow. Oh purty please!

Dampness usually makes my skin blister more. I'm having a hard time thinking of a way to describe how my feet feel today - I didn't record steps yesterday because I barely walked at all. Today I've done about 430 steps so far, in mock crocs with no socks (heh).

I have a little soreness - like the beginnings of blisters - on the ends of my third and fourth toe on my right foot. It's not going anwhere worth taking a picture of; only slightly red, slightly sore. The rest of the skin on my feet is in good shape today. Onward!

Walk for the Cure - JDRF. Oh yes, I'm going to do it.

noreply@blogger.com (Joan Q. Public) — Mon, 13 Apr 2009 14:34:00 +0000

A dear friend of mine has a child affected by Juvenile Diabetes. She put out the call for walkers to join her family on a 5k Walk for the Cure. I thought to myself WELL, that will be 30 days from the beginning of the Valtrex dosage - if it becomes really effective by then, then that would be the ultimate test... what if the girl who's never been able to do a walk like this can actually do it?

What could that mean for future efforts for EB fundraising?

I have officially joined the walk. Call me nuts if you will - maybe I am; I have to spend the next few weeks just building some stamina - heh. My page for the walk is here:

Support the Walk for the Cure!

Please donate if you can - or at least comment as a cheerleader. ;)

Easter Weekend - Day 11, 12

noreply@blogger.com (Joan Q. Public) — Mon, 13 Apr 2009 14:29:00 +0000

Saturday: 1895 steps.
Shoes & Socks: Nike tennis shoes and thin nylon socks.

I took the young'un to an art show on Saturday, and walked around quite a bit. After the art show, I grocery shopped at a small store, then went home and did some cleaning. By the time I left shopping, I was feeling a good bit of 'burn'. That night and the next day, my feet were still sore - but no visible blisters; I really thought I would be able to take some pictures!

Sunday: 607 steps
Shoes & Socks: Mock crocs w/cotton socks

Sore this fine sunny Easter Sunday, but no visible blisters. Is very odd - this is the stage where I really expected to see some flat blisters under calluses or some bubbling up on my toes, but nothing. Stayed sore most of the day, through egg hunting and socializing - but not crippling.

I may have been too ambitious in my fundraising walk pledge, but we'll see how it goes! I will address this in a new post....Onward!

Day 10 - and, I must be nuts.

noreply@blogger.com (Joan Q. Public) — Sat, 11 Apr 2009 04:04:00 +0000

565 steps today - in mock crocs with no socks. Not much walking - and no new blisters.

Hmm... I feel a poem coming on!

Mock crocks without her socks
Counting steps and watching clocks
...Walking helps with writer's block.
Hoping hard that Valtrex rocks!

Okay, that's over!

Now, for the nuts part. I've signed on to do a 5k walk on May 2. An ubertest. Possibly overly ambitious. Details tomorrow!

Day 8, 9

noreply@blogger.com (Joan Q. Public) — Thu, 09 Apr 2009 23:09:00 +0000

I'm encouraged - I traipsed WalMart yesterday when I went to pick up my pedometer, and did a lot of extra walking - just because. Had particularly lousy shoes on yesterday; broken down purple Kangaroos. I managed to twist my ankle up pretty good within five minutes of putting them on, but it wasn't sore enough to keep me down. By the time I finished window shopping and NOT coming home with doughnuts (hooray for me!) my ankle was complaining and I had the 'burn' on the bottoms of both feet; but there were no new blisters. It was a cool day here in Tennessee yesterday, so I'm still reserving judgment on the efficacy of the Valtrex.

I have fired up the pedometer and it works great. I have, however, been chained to my desk again, and so at this hour have only recorded 313 steps for day 9. I'm surprised my butt hasn't blistered in my chair, nor my fingertips from typing. The evening's still young, though!

Onward!

Ugly Blog

noreply@blogger.com (Joan Q. Public) — Wed, 08 Apr 2009 01:22:00 +0000

I know there're some blogging aesthetes reading. Help! What can I do to make this page easier on the eyes?

Day 6, 7

noreply@blogger.com (Joan Q. Public) — Wed, 08 Apr 2009 01:16:00 +0000

Wildly unexciting news at the moment! Nothing new to report - except my pedometer finally arrived, I just have to go pick it up. The icky weather should be done by tomorrow, and then I'll be counting steps. I'm excited!

The pedometer I chose:

Omron Premium Pedometer

Onward!

Days 3, 4, 5

noreply@blogger.com (Joan Q. Public) — Sun, 05 Apr 2009 13:41:00 +0000

Sorry for not updating daily these past few days; I'm having mad internet connection troubles.

Things I have noticed the last few days: The dry mouth issue seems to be abating. I still wake up parched, but not having thirst-attacks so often anymore!

My face has cleared up - that hormone-induced little acne breakout I mentioned is gone, and far faster than anticipated. I also didn't get any blisters on my face this time around - which often happens during my period. I notice my skin is a bit drier than usual; I have been using a good deal of moisturizer on areas of skin that don't normally blister - arms, legs, shoulders.

Here's a weird little thing - I have some small scars on my right hand and right calf from rambunctious pet play; not old enough to fade but fresh enough to still see them - they appear to be changing color. Could be my imagination - but healing wounds like that for me usually stay pink for a long long time; I'm very fair skinned. All the little bumps and healed scratches now seem to be a bit more pigmented; not darker, exactly, just a different color - more of a tan/brown. As I said - I could just be nuts, but I thought it was worth mentioning. I'll keep an eye on that as days go by.

I've been obsessively cleaning house this weekend, which usually means broom blisters. I have only a tiny one on the palm of my right hand so far. My feet are holding steady; still a little sore from the shopping excursion, but no new blistering yet. I haven't really taxed the feet since then; and usually do not experience much if any blistering when I'm running around in stocking feet, which is what I've done all weekend.

Price paid for snazzy pedometer: $26 plus way too many waiting days for shipping. Argh! I'm really anxious to start walking with a purpose, counting steps and posting those results. Onward!

Day 3 - Just chillin'.

noreply@blogger.com (Joan Q. Public) — Fri, 03 Apr 2009 00:28:00 +0000

Welcome, Facebook friends! Glad to see you reading - jump in and comment or question anytime.

Nothing terribly specific to report today. The dry mouth seems to not be as severe today, but I have been drinking lots of fluids. I am less footsore today; the blister 'burn' seems to have subsided. I have been off my feet today and yesterday playing catch-up on desk work, so I figured the ouch would subside fairly quickly. My shopping blisters are a bit red and swollen, but no further fluid filled.

I am experiencing some blistering between folds of skin at the top of my right thigh, not sure which item of clothing perpetrated that lovely little situation, but if I figure it out I will surely burn it. I usually don't get that kind of chafing blister pattern until June or July when the weather's a lot warmer.

Onward!

Day 2 - Post-shopping

noreply@blogger.com (Joan Q. Public) — Wed, 01 Apr 2009 18:43:00 +0000

Here is an image of my left foot this afternoon. I walked for about two hours yesterday in Hanes plain cotton socks and a pair of mock Crocs. The blisters weren't quite as bad as I expected. They are mostly flat and stingy, and under calloused areas - which means they'll probably hurt more tomorrow than they do today. I strongly believe it is too early to credit the Valtrex with the blistering not being as many or as severe as anticipated; it was a cool day yesterday and I wasn't moving around very fast. I have circled the areas where the blisters are the most painful. I shall try to get rid of some calluses for future shots. Discovered the hard way that I need a better camera; closeup shots I tried to take did not resolve well.

Physical things I have noticed today: My mouth is still dry. I woke up with a medium-severity headache that went away with a single dose of ibuprofen. I'm having a mild rashy acne-looking breakout on the right side of my face, but due to the day of the month, that is probably unrelated.

I will probably not be walking any more today, as I have desk work to do for the rest of the afternoon and evening. Onward!

Official Day One!

noreply@blogger.com (Joan Q. Public) — Tue, 31 Mar 2009 15:07:00 +0000

I took my first 500mg dose of Valtrex with breakfast this morning. Based on others' experiences, my understanding is that it may take as much as 30 days to notice positive effects, so my initial posts may be a bit boring!

I am traveling out of town for a sushi and shopping voyage today; after which I am sure my feet will be decimated. I don't have my pedometer in hand yet - wish I could count steps today! c'est la vie.

The only noticeable side effect 4 hours after my first dose is that my mouth is really dry. I didn't drink much yesterday or this morning, so this may be unrelated, but it's unusual for me to feel so parched *after* breakfast, water and toothbrushing.

Onward! More later.

Scrip has arrived!

noreply@blogger.com (Joan Q. Public) — Mon, 30 Mar 2009 18:40:00 +0000

The long awaited lil' bottle of meds has arrived. Tomorrow we begin the voyage!

I would like to caution anyone reading to please not take Valtrex or begin any experimental treatment for EB without the advice and monitoring of your physician. That's what I'm doing. I am not a medical professional - and while I am happy to provide information about my personal experiences, I cannot advise or direct anyone with regard to their own treatment. You are, however, welcome to forward my blog or print any posts/pictures to share with your doctor(s) to help begin your own discussion about Valtrex and EBS..

That said, here's a link to the Valtrex home page for more information.

Also, please read the complete text of uses, dosage, warnings and contraindications at RXlist.

Photos of EB Simplex damage

noreply@blogger.com (Joan Q. Public) — Mon, 30 Mar 2009 15:47:00 +0000

Pictures of EB Simplex affected patients. These were collected from web references, they aren't my body parts. Photos for my personal experiment are coming soon!

General Information about Epidermolysis Bullosa

noreply@blogger.com (Joan Q. Public) — Mon, 30 Mar 2009 00:05:00 +0000

What is Epidermolysis Bullosa (EB)?

A child with Epidermolysis Bullosa (EB) is afflicted with a type of inherited skin disorder that causes blisters after even the mildest trauma. Most commonly, EB causes blisters on the skin, but EB can also affect the mouth, esophagus, lungs, muscles, eyes, nails and teeth. Depending on the type of EB, the effects of the disease can be mild, disabling, or life threatening. EB is never contagious because it is a genetic disease.

There are three types of EB, all caused by a different genetic mutation: EB simplex (EBS), Junctional EB (JEB) and Dystrophic EB (DEB). EB simplex is the mildest and most common form of EB, while Junctional EB and Dystrophic EB are relatively less common and affect the patient more severely.

What are some symptoms of EB?

Blisters on the skin, usually beginning in infancy
Blisters or sores on the mucous membranes (mouth, throat, respiratory tract)
Thickened skin on the palms of the hands or soles of the feet
Thickening or absence of fingernails/toenails
Anemia (more common in severely affected individuals)

How common is EB?

Based on statistics collected through the National Epidermolysis Bullosa Registry, EB is estimated to occur in 20 newborns per 1 million live births in the United States. The exact number of persons with EB is unclear, but estimates suggest that 25,000 - 50,000 people in the United States have EB.

How is EB treated?

Many patients with mild forms of EB require little or no treatment. In fact, people with the most common form of EB, the Weber-Cockayne Subtype of EB Simplex rarely seek medical help. However, patients with severe forms of EB require hours of daily intense care that is usually provided by their families. The care given to these patients is similar to the care provided for burn victims; blisters are wrapped in dressings and the dressings are changed daily. Currently, treatment for severe forms of EB is focused on promoting wound healing, preventing infection, protecting the skin from trauma, decreasing blister occurrence, minimizing deformities, alleviating pain, attending to nutritional needs, and providing psychological support for the family. With the more severe forms of EB, it is often necessary to have several physicians involved in the care of the patient, including a dermatologist, a primary care doctor, dentist, hand surgeon, gastrointestinal specialist, and pain specialist. Additional help is provided by nutritionists, physical and occupational therapists, social workers, and other health care professionals. This multidisciplinary approach is needed for children with severe EB.

Forms of EB:

EB simplex

There are many types of EB Simplex and most are caused by an autosomal dominant gene mutation that leads to a defective keratin protein. Keratin proteins function as the scaffolding for the skin, and so when this scaffolding is not formed correctly, the skin is more likely to fall apart and form blisters. EB Simplex can be split up into two main types, Generalized and Localized. In Generalized EB Simplex, blistering occurs all over the body, and in the more common Localized EB Simplex blistering only occurs in areas that receive the most trauma, usually the hands and feet.

Junctional EB

Junctional EB is an autosomal recessive condition that is caused by mutations in the genes that code for collagen17 or laminin-5. Collagen17 and laminin-5 are proteins that help anchor the skin together. Without them the skin separates easily, causing blisters. There are many types of Junctional EB, and all of them cause widespread blistering. Some forms of Junctional EB improve as the patient gets older, while a rare form of Junctional EB can be fatal in infancy. Because this disease is autosomal recessive, parents can carry the genetic mutations and be completely healthy. Afflicted patients receive one copy of the abnormal gene from each parent (see "How is EB inherited?" below).

Dystrophic EB

Dystrophic EB is caused by a mutation in the collagen7 gene and can be dominant or recessive. The collagen gene codes for the collagen7 protein that anchors the deeper layer of skin, called the dermis, to the epidermis, or superficial layer of skin. When an individual has an abnormal collagen protein, the skin is fragile and separates easily, forming blisters. Both dominant and recessive forms of Dystrophic EB cause scarring. An individual with dominant Dystrophic EB generally experiences mild to moderate blistering of the skin, but only a small amount of blistering of the mouth, esophagus, and GI tract. This type rarely causes pseudosyndactyly, a deformity of the hands or feet caused by the fusion of the fingers or toes. The recessive form of Dystrophic EB is the most severe, chronic type of EB. Blistering begins at birth or shortly afterwards. Much of the skin is covered in blisters and there is extensive internal blistering. Children can develop deformities caused by the recurrent scarring of the fingers and toes (pseudosyndactyly) and the hands and arms become fixed in stiff positions (contractures). It is painfully difficult for a child with recessive Dystrophic EB to ingest food due to the internal blistering that occurs in the mouth, esophagus, and gastrointestinal tract.

How is EB inherited?

EB is a disease that is passed to a child from the mother and/or father's chromosomes. All of the genes that cause EB are autosomal, meaning the genes are X and Y chromosomes. You can think about the process of inheriting genes by imagining chromosomes as two sets of instruction manuals that contain information on how to make everything in your body. One instruction manual is from your mother and the other manual is from your father. Genes on chromosomes are like pages or sections of the instruction manual that focus on a specific part of the body, how to make hair or what color to make it.

In individuals with EB, some of the pages of one or both instruction manuals are missing or incorrect. These pages should contain the information on how to make the proteins that hold the skin together. Faulty instructions lead to proteins that are either absent or abnormal. Without these proteins, the skin is not as strong as it should be. Depending on the form of EB, either one or two sets of bad instructions are needed to produce disease.

Autosomal Dominant Inheritance:

When only one abnormal gene is needed to produce disease, that disease is autosomal dominant. The abnormal gene is able to disrupt the function of the normal gene, causing disease. Most forms of EB Simplex and Dominant Dystrophic EB are autosomal dominant.

A person with a dominant form of EB has a 50% chance of passing the disease onto their children each pregnancy.

A person may have a dominant form of EB and not have an affected parent. In these cases, a new mutation in the egg or sperm of one parent occurred.

Autosomal Recessive Inheritance:

When two abnormal genes are needed to produce disease, that disease is autosomal recessive. All forms of Junctional EB and Recessive Dystrophic EB are autosomal recessive. A person can have one abnormal copy of the gene for the disease be completely healthy. This individual is called a carrier. If an individual that carries one recessive gene for the disease has a child with another carrier for the same disease, their child may be afflicted.

When two carriers for a disease produce a child, there is a 25% chance the child will have the disease, a 50% chance the child will be a carrier, and a 25% chance the child will have completely normal genes. This information is most useful to parents who have had one child with a recessive form of EB in determining their risk of having another affected child in the future.

Tell me what you want

noreply@blogger.com (Joan Q. Public) — Sun, 29 Mar 2009 03:13:00 +0000

Now that I've posted the link to this blog around a few places, I'd like you folks reading to let me know what you hope to get out of this blog. The process of documenting this sort of experiment is new to me. What pieces of information would be the most helpful?

I'm still champing at the bit waiting for the Valtrex to arrive. I have purchased a snazzy pedometer and am planning a regular walking routine in order to 'push it' with the blisters. I will be posting about brands and styles of footwear I'm using, and posting pictures of my feet and/or hands or other areas of skin affected by the EB. I will post results of doctor visits and lab test results if they are significant.

Anything else you would like to see?

Waiting to see.

noreply@blogger.com (Joan Q. Public) — Fri, 27 Mar 2009 07:20:00 +0000

This is the beginning of a voyage; one that I hope puts me on my feet. I mean that literally.

I have a hereditary skin condition known as Epidermolysis Bullosa Simplex, Weber-Cockayne type. For those unfamiliar with this rare condition, it is a genetic malfunction that causes localized blistering of the skin in the presence of heat, friction, moisture or all of the above. It's passed on as a dominant trait. My dad has it, and so does my son. We've known nothing else since birth. The blisters can be very painful. We have generally accepted, many years hence, that there is no cure or effective treatment for this condition.

Recently, I got excited about a post I read on the Community Forum at http://www.debra.org, the research organization dedicated to Epidermolysis Bullosa. A gentleman in Lawrenceburg, Tennessee (my home state, incidentally) had been using Valtrex for some time now; it seems he has the same subtype of EBS that my family does. He reported dramatic positive results from this unlikely little pill after as little as 30 days.

Of course you know - I had to try.

I talked to my good doctor last Tuesday about trying the Valtrex, and he was very positive and supportive. Many years ago, when I was a new patient, he said to me in regard to the skin condition: "Honestly, I don't know anything about it - and you seem to be very well informed. If you ever run across any way that I can help, then I'll be happy to." True to his word, he has prescribed me the Valtrex and set up for regular bloodwork to be sure to monitor kidneys, liver and bone marrow. He said that it wouldn't hurt me if it didn't work - and if it DOES work, it could change everything.

I'll be 36 years old tomorrow, and I'm reaching a point where the blisters, in combination with too many hours working in chairs and not enough hours seeking out a proper diet, have pushed on to the point where my age and my weight are beginning to catch up with one another in a bad way. I've always been heavy; and I am not the heaviest now as I have ever been - but safe to say any medico would label me 'morbidly obese' or the slightly less clinical 'supersized'. I am a strong and confident person; I'm bright and effective. I'm pretty spry for a fat chick - despite the blisters and the extra weight, I don't have bone, joint or mobility issues. Let's get real, though - eventually, the pounds and the lack of exercise will take its toll. There will be serious consequences in some part of this big ol' body that I'd really rather not face.

Make no mistake - I am a size acceptance supporter extraordinaire. I have no desire to be rail thin or tan and oiled musclebound. I do, however, want to remain strong - and feel good.

So here we go. My Valtrex prescription, 3 months' worth, is en route from my insurance company's mailorder pharmacy, and should arrive within the next few days. I intend this blog as a record of my results, and plan to count steps, post pictures, report side effects, and generally provide as much information as I can to anyone interested in my grand experiment.