EB Awareness Week 2013- day 5 & 6

I didn't have time to blog yesterday because I was busy working on my final blog post.  And I wasn't sure I would get time today either but while doing the dishes my thoughts started to wonder and decided to write about what its like as an adult with EB and be an EB parent.  And thought my intent is NOT to offended anyone with my writing, I know it's very likely that some or many maybe be offended by this post, so if you are an EB parent and offend easily, it might be best not to read this:-)


With the onset of National EB Awareness Week many EB parents have shared their child's birth story and how shocking and heartbreaking it was that their child was born with EB.  While I do have three stories of my own, they are not very similar to the many I have read this week.  There was no shock that my child was born with EB; we knew it was a possibility.  I was not horrified on how much skin my child was missing; I already knew what EB looked like.  While I was saddened they had inherited EB with from me and I knew things wouldn't be easy, I didn't think even for a second their life or mine for that matter was over because they were born with EB. 


As an adult with EB, I do understand how painful baths are;  As an EB parent I also know how necessary they are and do what I can to make it fun and as painless as possible.

As an adult with EB I understand how painful wound care is emotionally and physically and sometimes there is no amount of pain medicine to take away the pain; As an EB parent I try to make it as comfortable as possible and let the child have some control...even if that means it takes hours to get it all done.


As an adult with EB I understand how damaging itching is to the skin but i also know its like an addiction and very difficult to stop;  As an EB parent I try various ways to relieves the itching but also know my child is going to itch some days to the point of bleeding and there isn't anything I can so to stop it.


As an adult with EB I understand how humiliating is it to spend hours at the doctors office having even inch of my skin document on film or worse sitting in just my underwear while dozen of doctors and medical student parade thru the exam room to see what EB looks like;  As a EB parent I do not allow my child to be put on display at the doctors office or hospital.  We only do what is necessary to stay healthy and while I am all for educating and teaching it can be done with exploiting my child.

As an adult with EB I know what its like to be turned into guinea pig and subjected to "experimental" treatments involving vitamin supplements medication or other procedures all in the name of science.  As an EB parents I will wait until my child are old enough to decide from themselves if they want to participate in such things.

As an adult with EB while I was not taught to hide their EB (though some with EB were) it also wasn't something openly shared either.  Maybe it was because it was a different day and age and the technology available today wasn't available then.  As an EB parent my kids know they have EB; they know other kids their age with EB.  They are encourage to tell people about it if they want to but I don't force them too.  I suggest ways they can deal with questions from strangers but also tell them its ok to tell people its none of their business.

As an adult with EB we did not have the internet or social media when I was growing up. My parents did not post pictures of my wounds for the world to see or express how much they hated EB for their friends, family, co-workers and eventually for me to read when I got my own Facebook account;  As an EB parent i never try to hide the fact that my kids have EB and I do post things and pictures about my kids' EB but I am very cautious about what I say and about the pictures I post.  I try think about how they would feel someday if they were to read something I posted before I post it. 

As an adult with EB I know how hard it is to only have few friends and be made fun of at school.  As an EB parent I teach my children that in order to have friends you have to be a good friend.  If people really want to be your friend, they will care less you have EB and if they do care or make fun of you for it, then they don't deserve to be your friend. And while there will be those people who will tease and make fun of you,  you will also find some great friends just like I did!

©Garrett's House 2021

EB Awareness Week 2013: Remembering the EB Angels

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©Garrett's House 2021

EB Awareness Week 2013- day 4

Today I will be sharing some pictures that show the reality of having EB and being a toddler :-)


These are Joey's feet.  All the wounds you see are self inflicted from him just itching his feet:( 


Even with oral and topical medication to help reduce the itching sometimes there is nothing we can do.  And if you have ever tried to reason with a 2 year old you know how hard that is:-)



WARNING graphic pictures below

 

 

 

©Garrett's House 2021 

EB Awareness Week 2013- day 3

Today has been one of those days where I had every intention of getting a lot accomplished but unfortunately life had other plans and I got very little done. I wasn't able to write the post I was intending to write about so instead I invite you to re-read three posts I did last year for EB Awareness Week.

1) How do I know so much about EB

2) The "Happy" Part of my Job

3) The "Sad' Part of my Job

 

 

 

©Garrett's House 2021 

EB awareness week 2013- day 2

There are many kids with EB who have a g-tube in their stomach to make eating much easier.  Joey got one when he was 3 weeks old.  Most kids with g-tubes are on a feeding pump over night.  The pump runs at a slow feed for about 12 hours delivering a set amount of formula to the person.  We found out quick that Joey could not tolerate pump feedings so we switched to bolus feeds which means every 3-4 hours we would attach a special tube to his g-tube and pour his baby formula into his belly.  It was the same concept as feeding a baby every 3-4 hours but instead of drinking it by mouth we would just use the tube.  

Once an infant with a g-tube they typically switch from formula to Pedisure or other high calorie ready made formula.  However there are some in the EB community (and general g-tube community for that matter) use a blended diet vs Pedisure or some other ready-made formula.  A blended diet is basically normal food that regular people eat just blended up so it is liquified enough to go thru a g-tube.   Not many in the EB community use a blended diet but there are a few of us:)  Blended diets are very controversial in the medical community but I am not going to start a debate.  Instead I will share our recipes we use for Joey's blended diet.  

The main ingredient we use is Kefir (it is a yogurt drink)

32oz Kefir  (560 calories 44g protein)

Next I use one 16oz serving of a fruit and one 16oz serving of a veggie.  I mix and match each time depending on what is available at the store and/or on sale.  Here are the fruits and veggies we have tried.

16oz servings:

FRUITS
3 large apples diced (288 calories 16.2g fiber 1.6g protein)
2 mangos diced (214 calories 6g fiber 3% iron)
3 large pears diced (288 calories 15g fiber 6% iron)
3 large peaches diced (114 calories 5.5g fiber 3% iron)
3 large bananas chopped (315 calories 9g fiber 3g protein)
1 pint of Blue Berries (194 calories, 8.2g Fiber 2.52g protein, 5% Iron)

VEGGIES
1 large sweet potato diced (480 calories 6g fiber 3g protein)
1 bag baby carrots chopped (159 calories 6g fiber 3g protein)
2 bunches kale (68 calories 3.9g fiber 6.6g protein)
2 Avocados (454 calories 12.9 fiber 4.2g protein)



GRAINS- I recently started experimenting with using a grain...this is what I have tried thus far.  I don't use it every time though

1 1/3 cups cooked minute rice multi grain medley (brown rice, red rice, wild rice, quinoa)
320 calories 4g fiber 8g protein 16% zinc 8% iron


I blend the fruits, veggies and Kefir with 8oz prune juice (180 calories 3g fiber 4% iron 4% zinc)

Once this is all blended it makes about 72 oz of blended food.  I then divide it into two separate 36oz containers. I then add the following to EACH container:


3 tablespoon Happy Bellies Organic Baby Oatmeal (60 calories 2g fiber 10% zinc 80% iron.)

1 tablespoon olive oil or coconut oil (120 calories)

1 tray (1.5oz) of Benecalorie (330 calories.)

Each container comes out to around 1300 calories each.  He gets 7-8oz servings 4 times a day depending on how much he each on his own by mouth.  I make his blended food about 3 times a week.  

In addition to the blended diet he also gets 7oz of 30cal/oz Elecare Jr. Formula and 120 cc of free water per day.

The doctor wants him between 1800-2000 calories a day when possible:)  He needs those calories for wound healing, energy (which he had a TON of) and to gain/grow.    

There are many blended diet recipes out there and this is just an example of what we use!  Yes this is a lot of work, but I think Joey is healthier because of it!

 

©Garrett's House 2021 

EB Awareness Week 2013- day 1

Today is the first day of international EB awareness week!  It runs every year from October 25-October 31. In years past, I have done a blog post every day on some EB related topic. This year will be no different!!  I should have planned little better on topics but with 4 kids and a job it's hard to do that sometimes! 

This first post will be a reintroduction of our family. Though our story is scattered through out this blog and on our family blog: Four Busy Bees and Two Butterflies, I know people don't always have time to search thru archives.  So I will do a little introduction of our family:-)

I am Sara, I am 36 and I was born with Epidermolysis Bullosa Simplex Dowling Meara or EBS-DM for short. I have a defect in my Keratin 5 gene meaning my EB is genetic and is not contagious.  But it also means it will never go away even there are times I have no visible blisters or wounds.  I have been married for almost 13 years to a great guy named Joe:)

Our oldest is Sami and she is 11.  She too was born with EBS-DM. She is a typical 11 year old who enjoying singing, acting and drawing.  She goes to a regular school and has many friends. 

Next came Garrett and he too was born with EBS-DM.  Sadly he developed some complications during his 2nd week of life and passed away when he was 12 days old.  Garrett's House was created in memory of him to further educate the world about EB and to help the families of other EB babies.  This coming December Garrett will celebrate his 9th birthday in heaven and his 9th angel day as well. 

Summer was born 15 month later.  She is now 7 years old and loves to read and play on the computer. She does not have EB!

19 months later came Gracie who is 5 and just started kindergarten this year.  She has many friends and loves to play with her dolls.  She too does not have EB!

Then came Joey.  He was born in March of 2011. He was born with EB and was the most severe of my three:(  He was born with no skin from the knees down on both legs along with some missing skin on his wrists.  When he was 10 days old he started having breathing problems and was admitted to the PICU.  Within hours of arriving to the hospital, he went into respiratory failure and spent 7 days on a ventilator and almost needed a trach...but thankfully the blisters and swelling had gone done enough where he didn't need a trach.  But it was discovered that he had severe acid reflux.  The reflux was causing him to aspirate when he swallowed. Which in turned caused damage to the back of his throat spreading into his upper airway. (which is why he stopped breathing) He could no longer safely eat by mouth and required a feeding tube to be surgical  placed in his stomach. 

Today Joey is 2 1/2 and is ALL boy! His skin is doing much better but he is still wrapped from his chest to his ankles for his own protection. He is able to eat by mouth now but 80% of his daily nutrition is still thru his g-tube. He loves Mickey Mouse, Sponge Bob, riding on his sister's scooters and swinging on his swing.  He loves to play with cars and climb on anything thing he can reach! 


My hope that this blog helps spread awareness and educate those about EB and to make the journey of those with EB a little easier to navigate!!

 

 

 

©Garrett's House 2021 

The Butterfly Network Here to Help 2013

©Garrett's House 2021

Epidermolysis Bullosa Awareness 2013 Video

©Garrett's House 2021

Books on EB

Introducing two recently released books about EB!

 

 

 

 

 

Everyone Belongs

By Dennis Vanasse M.Ed.

 

 

 

 

 

Courage Under Wraps

Photographic Documentary of Nicholas Zahorcak Living with Epidermolysis Bullosa

By Jodi Champagne

 

 

 

 

Cardiomyopathy and severe forms of EB

*author note..I understand these types of topics are tough to talk about.  It not something people want to think about and hope if they ignore it, the problem will go away.  I am not trying to make people feel uncomfortable.  I just want to get this information out there so EB families have the information and tools they need to make the best decisions they can for their health or for the health of their child.


There are many complications of severe forms of EB that can arise at any time.  One of them is called Cardiomyopathy- which is a disease that weakens and enlarges your heart muscle.

While I have no personal experience with this condition, I do have some friends with EB who have or had this complication and I have been reading up on it to learn more about what they are going thru.  Cardiomyopathy seems to be a silent, and in many cases, a deadly complication of EB.  In the past week I have discovered that many with EB have no idea this is a complication that could affect them, let alone know the symptoms of it. 

The three types of cardiomyopathy are:

Dilated cardiomyopathy. This is the most common type of cardiomyopathy. In this disorder, the pumping ability of your heart's main pumping chamber — the left ventricle — becomes less forceful. The left ventricle becomes enlarged (dilated) and can't effectively pump blood out of the heart. Although this type can affect people of all ages, it occurs most often in middle-aged people and is more likely to affect men. Some people with dilated cardiomyopathy have a family history of the condition.

Hypertrophic cardiomyopathy. This type involves abnormal growth or thickening of your heart muscle, particularly affecting the muscle of your heart's main pumping chamber. As thickening occurs, the heart tends to stiffen and the size of the pumping chamber may shrink, interfering with your heart's ability to deliver blood to your body. Hypertrophic cardiomyopathy can develop at any age, but the condition tends to be more severe if it becomes apparent during childhood. Most affected people have a family history of the disease, and some genetic mutations have been linked to hypertrophic cardiomyopathy.

Restrictive cardiomyopathy. The heart muscle in people with restrictive cardiomyopathy becomes rigid and less elastic, meaning the heart can't properly expand and fill with blood between heartbeats. While restrictive cardiomyopathy can occur at any age, it most often tends to affect older people. It's the least common type of cardiomyopathy and can occur for no known reason (idiopathic). The condition may also be caused by diseases elsewhere in the body that affect the heart.  

Per the Mayo Clinic's web site here are some symptoms of Cardiomyopathy

Some people who develop cardiomyopathy have no signs and symptoms during the early stages of the disease. But as the condition advances, signs and symptoms usually appear. Cardiomyopathy symptoms may include: 

            Breathlessness with exertion or even at rest

            Swelling of the legs, ankles and feet

            Bloating of the abdomen due to fluid buildup

           Fatigue

            Irregular heartbeats that feel rapid, pounding or fluttering

           Dizziness, lightheartedness and fainting

No matter what type of cardiomyopathy you have, signs and symptoms tend to get worse unless treated. In certain people, this worsening happens quickly, while in others, cardiomyopathy may not worsen for a long time.

During my research of articles about cardiomyopathy and EB, I found the following articles that I found to be very interesting and informative.  I hope that those with severe EB or have a child with severe EB will take time to educate themselves about this complication and talk with their doctors to determine their risks and what tests should be do to check for the issue. Being proactive is the best thing you can do when it comes to complications of EB! Especially for complications that can be silent until its too late. 

The Cardiomyopathy of Iron Deficiency

Anemia of Chronic Disease

The risk of cardiomyopathy in inherited epidermolysis bullosa

Dilated cardiomyopathy in epidermolysis bullosa: a retrospective, multicenter study

 

 

 

**NEW** A recent study linking EB and heart issues 

©Garrett's House 2021