Daily Dealing with EB

My daughter, Eloise, has epidermolysis bullosa junctional. The junctional is one of the types of epidermolysis bullosa, or EB. What exactly is EB?

It is a very rare genetic connective tissue disorder that affects 1 child out of every 20,000 births. EB is actually a group of disorders that share a prominent manifestation of extremely fragile skin that blisters and tears from friction or trauma. Internal organs and bodily systems can also be seriously affected by EB. The list of secondary complications can be long and may require multiple interventions from a range of medical specialists.

The manifestations of EB, the symptoms the person suffers from, and the overall long term outcome on quality of life run the gamut from mild to devastatingly severe. This is dependent on several factors including the type and subtype of EB and the inheritance pattern (autosomal dominant versus recessive). There are many people who are diagnosed with milder forms of EB, which, while they can be extremely difficult and painful to live with, are not disfiguring or lethal. In these forms of EB blistering of the skin may be limited to the hands and feet which may not result in any scarring or loss of function. In more severe forms there is generalized blistering of the skin as well as injury to many internal organs and bodily systems. These more severe forms of EB result in disfigurement, disability and early death, usually before the age of 30. In fact, some forms of EB are lethal in the first few months of life. 

----- debra information on EB

"I have EB... I am not contagious. I wear bandages to protect my skin. I was born with it, and there is no cure. Epidermolysis Bullosa, or EB, is a rare genetic disorder. My body can't produce the protein I need to make my skin strong like yours. I get blisters very easily and sometimes they hurt. Today, there is no cure or treatment. Daily wound care, pain management, and protective bandaging are my only options." - This paragraph is on the cards that I have ordered to give to people.

My first initial thought when I heard of this disease barely a month ago was "How is that even possible? A child's skin just falls off?" But it's true in the sense of when you think of a blister. Where we have regular connective skin. Theirs is not.

Daily things for us are not the same for us. If we wear clothing that rubs us we barely notice it or it makes a red spot and that's the end of it. For her, it may not be the case. She will blister and not just red skin or tiny white pocket filled with fluid. She will get the ghastly large blisters.

Eloise does have the lesser of the types and subcategories. We are very grateful for that each day. I make sure to remind myself each day by reading the blogs of those that are not so lucky. I send encouraging words to them and let them know they are in my prayers.

We start out our day by me changing her diaper and nursing her. I make sure that we have bandage change areas in our home away from fun areas in our home. This helps her distinguish between areas that will hurt her and areas that are comforting.

After she is finished nursing I take her for a sponge bath (or Frankie does) and she is washed and we pop any new blisters we see and drain them and then bandage her. We are not allowed to wear gloves because it irritates her skin more so we just have to make sure our hands are washed well. We have small scissors and lancets to pop the blisters. After there is a puncture in the blister we let gravity help with the draining of blister. We put ointment on it and bandage of her blister. We cannot use adhesive because it makes her skin worse.

We have started learning to go about our daily normal lives. It does no good to watch her every step. She stumbles over her own feet and falls like I do. When her knees her the floor it's almost instant blisters. I take her to one of the areas and bandage her up.

As I have said before we are lucky. She can play in the sand without blistering. She can have a teddy bear. Most children with EB can not do these normal things. Some children are tube fed because eating scars their throat.

We still watch for rubbing, scratching of any kind on her. But this is what life is like for us. We live each day to the fullest now that we are over the initial shock and realizing we can't stop every blister. We say our prayers every night for those battling this that are much worse off.

If you are wandering how you can help children with EB find a cure go to Debra Take Action. 

Let Me Pencil You In

You Aren't Worth Enough For Pen.

I have had many planners over the years. There was the years when the school supplied a planner in junior high and I used it occasionally. The years in high school where I bought my own planner and it didn't work out.

Now I have found the perfect planner. I know the different ways to keep up with everything.

For work it has many advantages from me. I can keep track of when reports need to go out. Meetings can be planned out unless they are unexpected. Conferences with parents can be wrote down. Field trips that will take all day or that I need to dress a certain way for will be wrote down. Also seminars for the jobs as CEP credits.

For home life I can keep up with when I need to do deep cleaning of rooms, check fire extinguishers, smoke detectors, clean the dryer pipes, and clean the oven. When I need to rearrange furniture for special holidays. Also my new obsession (besides the planner) is getting organized. And also home projects like painting, or new pieces of furniture.

For Frankie, I can keep track of his medicines, when he has flare ups, when he has doctor appointments. I also can keep track of our date nights. And things we want to do together like different tastings and concerts. I also would love to keep track of his delivery days where I know it will just be me and Eloise since he will be out delivering. Who knows we may go with him a few times in the future!

For Eloise, of course I will keep up with everything EB related. Doctors appointments, medication, and bandages will all be on record. Keeping up track of more severe blisters in areas will also come in handy. Also for her I can keep track of milestones, first things and first things with us. Play dates I can also keep track of for her schedule. Also in my beautiful blue pen for her I can keep up activities in certain businesses for her. Like Barnes and Nobles has activities all the time for kids! Also any lessons Eloise takes.

For my fur babies I can easily keep track of appointments for them. Their birthdays, their grooming, their treatments, and their training. I love my dogs, they were my first babies so I have to keep them healthy too.

Meal planning will be a little different for my family. Our neighborhood has different dinner parties and we have friends invite us over all the time because they know Frankie and I aren't the best chefs or even cooks. I can however plan out Eloise's snacks and things of that nature.

For family things are more broad topic for the binder. I want to be able to have things written down like family portraits. Also family fun night where we do things together that isn't at home or we have a big fun night where everyone comes over and we play games and eat.

Social planning for me is quite simply I have things from my sorority. I volunteer a lot. I do things for church. Parties, showers, and teas of course are part of my schedule. Of course girls night out and in are going to be in there so we can celebrate!

Crafty things are new to me so I want to be able to write down a schedule for those types of things. For instance a list of projects I am working on that the products needs to be done by next week. Also different expos to go for scrapbooking and card making. Also keep track of certain sales and coupons too.

Pregnancy will come after November. I can keep track of appointments, symptoms, and different classes. I know there are a lot of support groups and I can write down their meeting times. Even after pregnancies I can get stickers for the baby's milestones!

Besides beauty sleep which I don't need a reminder about I know I will have to keep track of beauty appointments. From hair, nails, and waxings I can write them down and be extra happy that I remembered them and show up on time!

If you haven't already noticed, I am blogging more and more. I am starting to make sure I blog at least four to five times a week and sometimes more. I can just get a really wild and crazy hair and just blog sometimes. But I will keep ideas in my planner. Also our city does a blog meet up for the people who are serious about their blogging. I also hope to attend blogging seminars in the future and I can always write those ideas down.

Health is my last thing for me. I love to run and exercise (yes I am one of those people. Don't worry I didn't used to be one of those people.) I want to keep up with hydration, vitamins, how many miles I run each day or week. Also I can keep track of doctor's appointments, dentist appointments, dermatologist appointments, psychologist appointments (Though I am about to cancel her.), and gynecologist appointments.

Wedding is actually going to be a separate planner. I am just really anal about my wedding and there for I just can't bring myself to jot dot things in a planner I keep everything in but I will put a nice big sticker or paper block and write wedding over that square as a reminder!

Homeschooling will also have it's own planner. I am not doing the homeschooling. Frankie is doing the schooling. We have purchased a separate planner from Plum Paper Design in Atlanta, GA. It is excellent looking!

I know I mentioned this in the home section of plans but organizing has become a huge part of what I want to do. Between FlyLady and OrganizedLikeJen my world seriously looks like a five year olds closet when mom says he has to have his room clean in 5 minutes. So I am taking a few steps back and figuring out what exactly I want to accomplish and how to make my life more filling with this planner.

I have a lot ideas I want to incorporate into my planner. Here are a few from different people.

Medication List Laminated Snap in Page  - Frankie, Eloise, & myself.

Medication reminder stickers  - These are for me.

Weekly Pregnancy Stickers  - I cannot wait to use these!!!

25 Glitter shapes assorted pack - Just when I know I plan on sleeping for that time block.

Hand carved rubber stamp - Yorkie design - Dog grooming visits, food, and vet visits.

Best Friends genitals holding hands sticker  - Possible nights of conception.

Cherry Mini Stamp - I will do a cherry layout one week.

Bow Mini Stamp mint rubber - I'm going to buy six of these.... For the Sexy Six.

Owl Mini Stamp Mint rubber - I love this!

Mini Telephone Rubber Stamp Old Phone - I love this! Love it love it!

Glitter Red Washi Tape  - Looks pink to me but hey either way it's glitter and washi tape.

Glitter Blue Rhombus Washi Tape - So perfectly perfect glitter and diamonds.

Little One, Girl - 3 packs of mini stamps - I just love these!

Toddler in a Bath - For cute reminds about picking up bath items!

Miniature Cookies Paper Clips - OMG I LOVE THESE! So freaking dang cute and perfect

For Rectal Use Only' stickers. - Enema reminders and more.

tiny sticker for your schedule - I love these little things

More Tea Please

I wanted more time with Eloise and I got it. Frankie was delivering furniture he had made over the week to their owners today. She was nursing as Frankie left this morning. She did want some solid food afterwards. We had mini banana pancake muffins. We watched PBJ Otter on YouTube. She really enjoyed it and so did I.

Eloise and I cleaned up a little and she was a huge help. I gave her a bath and brushed her hair. To the normal person this doesn't seem like a big deal. But to me it was huge because her skin is so delicate and I am still caution of what I am doing.

I boiled her new plastic tea set to make sure the germs were off of it. Then I dried hers. I got my set out from Teavana the Butterfly Cast Iron Teapot. I decided to have Black Dragon Pearls Black Tea. A favorite of mine. For her "tea" I poured some organic apple juice in t here. I made use the sandwiches above and also some lunch sandwiches too. We had a small picnic on our floating bed on the patio. 

After having our small picnic on the patio we cuddled up for some reading, nursing and a nap. After that I made us both some more tea. Real for me and apple juice for her of course. We explored Aunt Odette's garden for a bit. Discovering that there are some butterflies which made me very excited. 

I decided to make her a sensory bin. I look at what I had and discovered I had plenty of oats for making oatmeal; so in the box they went. I gave her two different sized cups and a spoon. It has now been two hours of nonstop play for her. She pours, scoops, moves, etc. I have been fascinated watching her. 

I did move the box inside so I could start on making dinner and writing this blog. That hasn't hindered her at all. So for our first day alone, I believe it has been amazing. 

Milk on the Go

A month ago Frankie and I discussed what rooms to turn into a playroom and nursery. Three weeks ago I was planning to have children next year. Two weeks ago my heart found my child. This week? I hold my daughter in my arms.

True, life comes with unexpected turns. But most of all? My daughter is breastfed. I haven't had milk in my breasts for five years since I had my own daughter.

I began research and listening to doctors about breastfeeding. My fiance, Frankie, and I did a lot of talking to family members and friends about breastfeeding. Many of our friends and family are breastfeeding or have. I went to my mother who breastfed but also is a retired pediatrician. She helped me get my head together and also start.

Massages and suctions on a schedule were how I started. I pretty much was reading about the condition my daughter has while I was massaging my breasts. My mom ordered me domperidone from New Zealand, I believe. I have not really been on that. I have been working with herbs and vitamins and that is working just fine at the moment building up my breasts. I also have a zStarter Supplemental Nursing System, Supplemental Nursing System™ (SNS™) and Symphony® Breastpump.

Push and shove things started happening and I have Eloise already. She nurses when she first wakes up, lunch and at night. I make very, very little milk at this point in time and she is on donated breast milk. I have had several people tell me to put her on formula but a very low formula. We may try that at lunch in the near future until my milk comes in fully.

The only problems we have faced so far have been easily fixable. One problem was sliding the supplemental tubing in her mouth while she is latched onto me. We got over that one real quick. She became used to it faster than I thought she would. With her having EB nutrients are a big deal and breast milk has a lot of benefits for her over formula. The second problem was my back. I didn't really want to use a nursing pillow. We were given one as a gift so I used it and it does help. What I had to learn quickly was that I cannot just sit however I like. I must elevate my feet to nurse comfortably for me and her.

 I love SNS system but I believe in the future while my milk is coming that I will use Lact Aid. It's a lot like it the SNS system except it's not as uncomfortable. The SNS isn't exactly uncomfortable it's just bulky bottle there. The other is a high grade bag full with the same tubes as the SNS.

What matters is that my daughter gets the nutrients she deserves. I love breastfeeding her. And as for Frankie's bonding time with her. He bonds with her while she is nursing too! When I asked him how this was the response, "I get to hold you and give you support. And as I have my head resting against yours I can stroke her cheek and see the love between our little family." Another reason why I love him.

We are planning on trying to conceive as soon as we are married in November. I am sure then I will have plenty of breast milk for all the children I have.

Butterfly Kisses

If nothing ever changed there would be no butterflies.

My life looking back and seeing pictures, it's easy to see how people thought nothing was wrong. I looked like a rebellious teenager having a wonderful time and living it. Teenage pregnancy, emotional stage, smoke and drank. Only it wasn't that and it's not that for many.

Yes there are the people who cut or even go as far to say the cut themselves for attention. For those of us who have actually been hospitalized for cutting it pisses us off because we are a true cry for help. You have people thinking we are all just cut or are cutting for attention and that's not the case. 

I was raped. Not once not even just twice but for years by a family member. He threatened to kill me if I told anyone. He actually jump started my cutting because he cut my skin. I was scared to death but honestly I started to associate that with feeling calm because when he wasn't doing that he really was hurting me. I started cutting when I started to panic about someone finding out what he had done.

Then I ended up pregnant and I hit severe depression. I carried full term miraculously. I gave birth to a little girl that was honestly doomed from the day she was born. I get frustrated with people, even people close to me that say to me "At least you got to hold her and hear her cry." Excuse me but were you in the delivery room when they yanked her from me? No you weren't, so sorry to be rude, but not sorry; SHUT THE HELL UP! I never got to hold her. I was banned from seeing her and surrendered her to adoption. It was a full surrender because I knew where she was for the next five years. I only have four pictures of her. 

During those years I had a severe issue of cutting. The smoking was second in the worst category. Drinking was as prevalent as the cutting and smoking was during that time. I would party sure but I didn't find comfort in drinking. I still don't. The only alcohol beverage I find comforting is rose champagne and wine. 

Cutting placed me into the hospital twice, both of those times were before I moved. When I moved things got slightly better. Only because I wasn't being constantly ridicule and shamed for having a baby and cutting. Slowly, I realized how alone I was and the cutting got worse.

Last time that almost ended me in the hospital was December 1st, 2012. I didn't end up in the hospital. It's the day that changed my life, forever. Drinking and cutting was it  for me the night of November 30, 2012. I guess I stopped responding to my best friend because from my drowsy disposition. I fell asleep only to be woken up by my front door being kicked backwards off it's hinges onto the floor of my apartment.

I was snatched up and carried into the bathroom. Forced to feel hot water and soap into the places I had cut. I was then held in a lap with alcohol being poured into my wounds. I realized it was my best friend and her boyfriend, Odette and Chris.

After Chris bandaged me up I noticed someone else was picking up in my apartment. My heart about sunk into my pelvic when I realized it was Frankie, Chris's brother. He wanted to talk to me. He told me how sad it made him that I wanted to cut my beautiful body. I watched in amazement as he drew a beautiful detailed butterfly on my arm.

He explained to me that if I cut again the butterfly would die. I looked at the beautiful butterfly not wanting it to die because it was beautiful. I hugged him and it made him blush. He explained to me more about the Butterfly Project. "The person that cuts either draws a better or has someone else draw a butterfly where they cut or whatever. If you cut or self harm then you kill the butterfly But the ones drawn by other people are special. Even just one cut kills all the butterflies no matter how many you have."

They had plans to go ride the Polar Express at the Railroad Museum. So I was put into some extra Christmas pajamas that Odette had and got in the car. The ride there was quiet compared to the screaming I had done of the wee hours that morning. Frankie had managed to write a sweet note on Facebook to me for different ideas instead of cutting to do.

After that butterflies became very dear to me. But my views on butterflies have drastically changed in the last two weeks. It means even more to me now.

I talked at a conference about how not matter how scarred my skin was I was still beautiful. It was then my life changed a second time forever. A little girl about six and half wanted to talk to me about her skin condition and how I really spoke to her. She wanted to meet me. I drove back up there. This girl was an orphan and has a skin condition called epidermolysis bullosa.

I arrived and the little girl excitedly told me she was getting adopted. We talked about her what it felt like to her and how bad she had the disease since there are different forms of it. They are called butterfly children because of their skin being so thin like a butterfly's wings.

Frankie had went with me but had left and wandered off on a trip to the restroom. I finished up my talk and went to go find him. I discovered him playing peek a boo with a blue eyed and bouncing curls tiny little girl. It was over in an instant.

I now sit with this precious little girl who is a suffers from EB as well craddled against me. We are finalizing paperwork right now. I cannot believe that butterflies have changed my life so. And for this reason I will always love them.