Everyday Chaos

Happy Belated Anniversary!

Two years ago we were telling Victoria that the reason she needed brain surgery was because her brain was too big to fit into her skull. Yep-That came back to bite us but we were completely new to the whole let's explain brain surgery to a 6 year old thing. Although two years out and part of me would still stick with that explanation.

So where are we after two years? In my opinion still in a really good place. Is Victoria headache free? Nope. She still deals with the occasional headache. Sometimes we know why sometimes we don't. Does she still have leg pain, insomnia, exhaustion, swallowing problems, or eye issues? Nope. Does she still have the occasional bouts with brain fog, memory lapses and neck pain? Yes, but they are so infrequent that we just note them and move on.

Two years out and Victoria is finally talking about brain surgery, her scar, her memories and her feelings about surgery. In my mind that is progress. After two years Victoria is more aware of her body and tends to listen to her body instead of ignoring warning signs. That is a huge step for an 8 year old.

At the end of the day my little Chiari Warrior has her life back and she is enjoying every minute.

Where are Mom and Dad after two years? Hmmmm .... We are in a good place. Will we ever be able to relax and forget about Chiari? No we don't have the luxury of forgetting but we are learning to let go and trust Victoria and everything she knows. Hard lessons to learn to let go and not hover ... To trust her, her doctors and ourselves ... To let her just be a kid and try not to base every decision on her medical condition. The best I can tell you is that we are making progress in our areas-we aren't perfect but we are learning to appreciate what we have been given, count our blessings and live in the present.

Happy Belated Anniversary Victoria!!! Here is to many, many, many more outstanding decompression anniversaries!!

 

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Thank You ...

To those who serve our Country or who have served our Country - Thank You.  To those who have given their lives in service to our Country - Thank You.  To the families who have lost a loved one in service to our Country - Thank You.  To the families who are waiting for a loved one to come home - Thank You.

 

Photo Shoot

We started out the Memorial Day weekend with a photo shoot with the kids.  Alex is graduating from 8th grade in a few short weeks and we needed graduation pictures.  So, I started my day with coffee on the front porch with a friend and then spent several hours with the kids getting their photos taken.  It was so much fun that I am pretty sure we will do another session at the end of the summer and then again in the Fall and maybe during the Holiday season.  Yep, I had that much fun.  Not sure the kids will want that many repeat sessions but too bad.  I was surprised at how even the boys got into the photo shoot.  We knew Victoria wasn't going to be an issue, but the boys are never fans of getting dressed nicely once let alone two or three times.  They got to pick out their outfits so maybe that helped and they also got to pick out how they posed and where they posed.  So at the end of the day things went smoothly and here is a sneak peak of what we got accomplished today.  This is just a smattering of the photos we got from today.  The only down side to a photo shoot - going through all of the shots and making choices.

Happy Mothers Day!!!

Today was one of the best Mothers Day ever (as Victoria would say). It was a beautiful day spent with my husband and children tinkering in my gardens. For me there is nothing better. I hope today was special for all the Moms out there.

 

I found the story below on Pinterest and for me it sums up not just being a woman, a wife but a mother as well.

 

A little boy asked his mother, “Why are you crying?” “Because I’m a woman,” she told him. “I don’t understand,” he said. His Mom just hugged him and said, “And you never will.” Later the little boy asked his father, “Why does mother seem to cry for no reason?” “All women cry for no reason,” was all his dad could say. The little boy grew up and became a man, still wondering why women cry. Finally he put in a call to God. When God got on the phone, he asked, “God, why do women cry so easily?” God said, “When I made the woman she had to be special. I made her shoulders strong enough to carry the weight of the world, yet gentle enough to give comfort. I gave her an inner strength to endure childbirth and the rejection that many times comes from her children. I gave her a hardness that allows her to keep going when everyone else gives up, and take care of her family through sickness and fatigue without complaining. I gave her the sensitivity to love her children under any and all circumstances, even when her child has hurt her very badly. I gave her strength to carry her husband through his faults and fashioned her from his rib to protect his heart. I gave her wisdom to know that a good husband never hurts his wife, but sometimes tests her strengths and her resolve to stand beside him unfalteringly. And finally, I gave her a tear to shed. This is hers exclusively to use whenever it is needed.” “You see my son,” said God, “the beauty of a woman is not in the clothes she wears, the figure that she carries, or the way she combs her hair. The beauty of a woman must be seen in her eyes, because that is the doorway to her heart - the place where love resides.” Author: Unknown

Happy Mothers Day!

 

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Happy Nurses Appreciation Week

Having a chronic illness puts you in right smack in the middle of the medical community. You deal with doctors, nurses, techs, office staff on a regular basis. You deal with the less than stellar members of each profession and you recognize and are eternally thankful for the talented and dedicated members of the medical world. This post is to thank those nurses who over the years have provided not just medical care, but compassion, empathy, and understanding.

To the ER nurses who have taken the time to learn our story. You took time that I know you didn't have to ask questions, to understand and to make a difference during ER visits which are always stressful. Thank you for taking the time and treating my children like individuals and not just another box to check off to get through your shift.

For the nurse who understood why I wouldn't leave my 4 1/2 year old to go get a cup of coffee from downstairs and brought me a cup from the nurses station. I know you said it was not what anyone ever wanted to drink - for me it was the best cup of coffee I have ever had because it was given with kindness and understanding. You didn't have to take pity on me and help me out but you did - thank you.

To the nurse who blocked a lab tech from Victoria's room at "oh dear God it's early" and prevented the tech from taking a blood draw because it was the first night Victoria had actually slept through the night. Instead you took the vial and did the blood draw through the IV line. Thank you for your understanding, awareness and compassion. Your actions let a very sick, tired little girl continue to rest and it also meant a very tired Mommy got some much needed sleep as well.

To the OR nurses who were awake and cheerful on June 15, 2011. You made us laugh away the fears of brain surgery. You reassured us and you kept us informed. Thank you for taking those extra steps not because of your training but because of your caring spirits.

To the nurse in ICU who made the connection between the issues with EDS and let the valium take effect before taking additional actions. Your intelligence and quick thinking helped to prevent additional actions that would have been uncomfortable right after surgery.

To the nurses who helped us figure out creative ways to get a little girl to take her medications after surgery. You went above and beyond the call of duty in thinking through the problem and coming up with new solutions when other approaches failed. Thank you for your determination and dedication.

To the nurses who came back after their shifts to check on Victoria because they were so worried about her. Thank you for your honesty and your obvious relief when you saw her progress - I knew I wasn't alone in my fear and concerns.

To the nurses who held my hand and kept me calm as my blood pressure continued to plummet during a miscarriage. Thank you for making sure I made it home again. Thank you for letting me know when things were starting to level out. Thank you for your protectiveness and for taking over advocating for me when I could no longer do it for myself. Your dedication, compassion, wisdom and understanding helped me through a very difficult situation.

To the nurses who let me vent my frustration over standard of care issues. You took my tirade at the events of the day which did not occur on your shift with professionalism. Thank you for letting me vent and for understanding the frustration of the situation.

To all of my friends who are nurses and who have spent hours answering questions and brainstorming with me to find answers. Your knowledge and caring always humbles me.

So to all the nurses out there who have touched our lives, who may touch our lives or who are dedicated to their jobs - thank you for your service, your compassion, and your dedication.

 

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Nick's Appointment With Dr. Francomano

Friday we headed to Baltimore for Nick's yearly appointment with Dr. Francomano. It was a very productive appointment. Overall, we are managing Nick's obvious EDS symptoms quite well. Of course EDS has obvious symptoms and not so obvious symptoms. Add in conditions that tend to go hand in hand with EDS and you end up spending quite a bit of time figuring out which condition is responsible for which symptom.

Right now Nick still has intermittent periods high intracranial pressure and cervical cranial instability. We will continue to treat the headaches caused by both with Advil, Diamox, an Aspen collar and Promethazine. We will continue to strengthen Nick's neck muscles through PT at home. We will also continue to strengthen his core muscles through exercise and PT at home.

We discussed the endocrine visit to Children's National Medical Center which was an absolute waste of our time and the requisite co-pay. I explained my thoughts on metabolic syndrome and insulin resistance and how it fits really well into my family's medical history. Dr. Francomano agreed that there are most likely underlying endocrine issues stemming from Nick's underlying conditions. She ordered some base line tests to hopefully give us an idea of what we need to do next. At this point we will continue using the modified diet that we started about 6 months ago we will also ramp up the exercise routine as soon as Nick is over yet another respiratory infection.

I will be scheduling the blood work this week. It will be an early morning appointment since we are running a fasting glucose and a morning cortisol test. Dr. Francomano also ordered two other tests. We discussed additional tests but decided to start with these for now. Once we have the results for these four tests we will decide how we want to proceed.

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Victoria's First Holy Communion

WOW!  What a great weekend.  Super busy but a spectacular weekend.

Victoria made her First Holy Communion on Saturday.  Victoria enjoyed her special day and had a great time at her party with close friends.  Here are a few pictures from yesterday.  I will post more later.

Congratulations Victoria!  We are so very proud of you!

Today

Today we raise our own EDS awareness by heading to Baltimore to see Dr. Francomano. This is the first of three appointments this month. Nick is the first one up to bat this year. We have quite a few things that we need to discuss. I don't feel as prepared as I would like to be for this appointment - but I have a bit of a safety net since we have two more appointments this month. I will update after the appointment. So right now fingers crossed that we find a way to get a few more answers about Nick's issues. The goal for today is to figure out how to proceed with the metabolic syndrome and/or insulin resistance issues Nick is dealing with. Based on my research those are most likely stemming from his high intracranial pressure messing with his pituitary. Yes, EDS and Chiari issues are never ending.

 

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What is EDS?

O.K. I will admit the photo caption is a bit of an over simplification of EDS - but I like it. EDS is a lifelong, chronic illness that effects every system in the body. And though you may look good on the outside the inside is another story completely.

EDS is a connective tissue disorder. There are various forms of EDS, but all forms of EDS involve a genetic alteration at the molecular level which causes abnormalities, (weakened) defective connective tissue within the collagen itself.

Connective tissue is found throughout your body. A major component of connective tissue is collagen. For people with EDS, our problems stem from faulty collagen.

Back in November, 2011 I wrote about EDS. Here is what I wrote:

I have been looking for a way to explain the importance of collagen for quite awhile and I finally found a fantastic explanation at wiseGeek.

Collagen is a type of protein. Fibrous in nature, it connects and supports other bodily tissues, such as skin, bone, tendons, muscles, and cartilage. It also supports the internal organs and is even present in teeth.

There are more than 25 types of collagens that naturally occur in the body. Collagen is one of the most plentiful proteins present in the bodies of mammals, including humans. In fact, it makes up about 25 percent of the total amount of proteins in the body.

Some people refer to collagen as the glue that holds the body together. Without it, the body would, quite literally, fall apart.

Possessing great tensile strength, collagen functions in a manner that is very different from many other types of proteins. For example, it can be found both inside and outside of cells. Collagen fibers are important in contributing to the external structure of cells. However, they are present on the inside of some cells as well.

Collagen works hand-in-hand with elastin in supporting the body’s tissues. Basically, it gives body tissues form and provides firmness and strength; elastin gives the same body tissues much needed flexibility. This combination of collagen and elastin is very important in many parts of the body, including, but not limited to, the lungs, bones, and tendons. Even the blood vessels rely on both collagen and elastin.

Often, collagen is discussed in relation to the skin. It works with keratin to provide the skin with strength, flexibility, and resilience. As people age, however, collagen degradation occurs, leading to wrinkles.

Since collagens are so important within the body, it stands to reason that collagen deficiencies can be problematic. In fact, there are some genetic diseases that are associated with collagen deficiencies.

Collagen is a basic building block in human bodies.

With EDS our foundation is built upon faulty building blocks.

If the foundation of a house isn't sound or built on solid ground then the entire structure is at risk.

That is how it is for us.

The effects of EDS can be felt each day in many different ways.

All we are trying to do is to keep from coming unglued.

 

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Zebras, Zebras and Zebras - Oh My!

It is May 1 and that means it is EDS Awareness Month. I never really know how to deal with this month. I am torn and pulled in several directions.

First, I know how important it is to make more people understand EDS and to be more aware of all of the issues associated with EDS. I really want the awareness to spread to the medical community. As I have said on more than one occasion I want the luxury of walking into any ER or doctor's office and have the medical professionals not just know how to spell Ehlers Danlos Syndrome but truly understand all of the systems impacted by EDS.

But let's be honest I hate EDS. I would love the luxury of not dealing with it on a daily basis. There are so many days that make me want to hide my head in the sand or put my fingers in my ears and say "I can't hear you!" If it was just me with EDS I would probably slip too easily into denial. However, I have three beautiful children with EDS and therefore I put on my big girl panties everyday and I deal.

Then there is the part of me that is still so tired of talking - of repeating the same things over and over and over again. I feel like rinse, wash, rinse, repeat was a mantra created just for me.

I realize that awareness and understanding will make my life and the kids lives easier. I know that we are so far from having a full grasp of all of the problems caused by EDS and therefore awareness, research and understanding are so vitally important to making things better. But seriously why do I need to do this? Why can't the medical professionals just do their jobs? So many whys and honestly too many private whine fests to admit to.

So which way am I going to go? Well despite my current bent towards whining, I am going with my true personality - the one that will slap you upside the head if I have to spend too much of my precious time on rinse, wash, rinse and repeat. The fighter in me (or maybe it is the stubborn mule) refuses to accept the ignorance and others desire for silence. I want my voice to be heard, more for my kids than for me, but I will be heard.

Today marks Day 1 of 31 days dedicated to EDS awareness. Thirty-one days out of three hundred and sixty five seems rather insignificant given how many lives EDS has wreaked havoc with or sent down the Rabbit Hole. But it is not the number of days dedicated to awareness but what we do with those days.

Keep coming back this month because I will be talking most days about EDS and how it effects our lives. Some of the conversations will be factual, some will be rants and some may be uncomfortable, but conversations about EDS need to happen. I will be linking to other blogs as well, there are quite a few eloquent zebras out there spreading the word about EDs and sharing their knowledge.

 

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