Monday, January 20, 2014

My German side!

Pork Schnitzel

I lived in Germany for a few years and I always was impressed on how Germans can live on fried food and beer and still be fit. Well, they WALK A LOT! When I lived there I tried to stay away from fried or potato's but I had my share of Riesling and cheese. I lost 10 lbs just from walking so much but my alcohol tolerance did go up a bit. Hey, I miss Germany! So today I decided to try and cook a healthy version of pork schnitzel. I had frozen a left over baget so I pulled that out. I was not sure if it would end up as a crouton when I initially froze it but I needed bread crumbs. So I pulled it out and just started crumbling. TADA...I have bread crumbs! I bought some pork on sale and just pounded it. Snitzel is a very thin pork so it was a good time to take out any anger. I made it thin! Instead of eggs, I used egg whites and breaded the pork. You do have to fry it in oil but at the end of the day, it was pretty healthy. I served it with some steam green beans, carrots and wild rice. All in LITERALLY under 30 minutes! I am so proud and it received rave reviews!


 
 
Here's to your heart!

Sunday, January 19, 2014

Bella



I am not sure if there is a formula for Mothers sometimes. I am a Mother of two and I do have a special needs child. I have so many friends with children that are special. My niece is one of them. I see post on FB and I even don't want to read them because my heart hurts. Bella's CPK's are back up and she now has a home health care nurse. She is 8.

I don't understand, will never understand why this happens. Special children are given to special parents.

Here's to your heart.

Foodie

Most people don't know but I am a foodie. I have to say that I have become somewhat OBSESSED with cooking over the past year. So much so that I have become a student at the culinary institute. What is ironic is I don't necessarily love to eat but I do like to taste. I am a heart health advocate so my obsession started with cooking every meal in the American Heart Associations Love Your Heart Go Red for Women Cookbook. Most of the recipes are pretty tasty and easy to prepare. I kind of have been feeling like Amy Adams in the "Julia" movie but on the healthier side. I think what I love most is plating. Its like cosmetics for the culinary. If it looks pretty, it makes you want to eat it!

In my quest to start over this year I treated my self with new cookware. Another one of my obsessions is Rachel Ray so I purchased the set. I did the red. I know its not Caphalon or a more expensive line but on the home cooking budget they do the job. Its kind of fun to get new cookware. My next goal is all new dishes. Of course I will stay with the red motif!

Here is my issue with a lot of dishes, SALT! I watch every cooking show and my new favorite is the "Taste", I know  I know but I do like it but why does every chef mention ADD MORE SALT. Its almost as bad as chef  Ludo who wants to ax the Vegan because he doesn't think Vegans can cook something you can't taste. Hey, I don't eat sweets or fried food but I can bake and fry with the best of them. My Mom was from Georgia. I never had a vegetable growing up that was not fried in a baby daddy fryer! My next journey is the weight watchers 15 minute superfast recipes. I'll post pictures if they are plated properly!

Here's to your heart!

Deanna P

Saturday, January 18, 2014

Avon Walk!


I have been so blessed with my family. I have spoken about Lois Lane before, my sister,
 but she is a breast cancer survivor and someone who has always been there for me...even in my darkest hours. I love her so much. As an Avon Walk Alumni, I am ready to do it again. 39 miles in two days! I do speak of heart because I am a survivor but cancer, chemo and radiation take a huge toll on your body, especially your heart. I have often said this that one cause is no better than any other but we should work together to make changes and save lives. This walk is for her. Please consider donating or joining me. Its an inspirational event and yes, I am going in for my mammogram soon! Pay attention to your body!

http://info.avonfoundation.org/site/TR/Walk/WashingtonDC?px=3841838&pg=personal&fr_id=2361

Here's to your heart and your over all health!

Deanna P

Wednesday, January 15, 2014

Matriach

I often wonder my strength. God gives you only what you can handle, right? Well he clearly has more faith in me than I do in myself.

As I petition, pray , hope for a non eventful January, well its not so good. My Grandmas' cancer is back and at 88 she is moving forward with chemo again. She had heart surgery last year and chemo 6 years ago for colon cancer. I guess I have some strong genes in my bloodline. The cancer is everywhere.

I lost my parents so she is my parent so to speak.

My family likes to one up. I say this jokingly. I have a sister, biological. Way to much Jerry Springer there but I spoke with her last night. The last time I saw her was my Moms funeral. The same day my Grandpa died. Yep, we like to one up. So last night my sister and I spoke. She has cancer. Can we not make a call without a ping pong match?

I spent 3 hours at the hospital today. Timing check here?

I have Ischemic Optic Neuropathy. With my Cardiovascular disease, pregnancy issues, DVT  so much more, what a catch I am! I don't fit the mold. I am so blessed for my children and my life but its often a reminder, and not to seem morbid, but life can and often is short. As heart month is approaching, its important to understand your body. I had a heart attack in my 30's! It just is in my genes.

I believe in love and life. Don't forget to hug someone everyday and be thankful for the blessings in your life.

Here's to your heart.....



Tuesday, January 14, 2014

Love, Like or lust?

Gosh, doesn't this apply to 99% of our lives! Food, Fashion and relationships. Its a funny dynamic. Shoes are a good example of that and yet so are beer goggles. We want to see the fantasy but the reality is a shoe that may be oh so fab can cause you so much pain and waking up next to someone who may have looked a little different while under the influence, yet that can be scary too. Hypothetically speaking of course.


How do we REALLY decipher? What is real and what is fantasy? Every morning I stand on the scale, sometimes just on one foot in hopes my miracle number shows up. The reality is it won't happen but the fantasy is if I remove all items of clothing, including any rubber band in my hair, I just might be back to the weight I was once when I was 18 :)

This brings me to love, like or lust. I admit, I am a self sleeve wearer. That has really proven to not be such a great trait of mine! I can self destruct with the best of them. Working on that.

I think its all about love in life. Love is unconditional and should not judge. OK, we do but believe a true friend and a true love, will always see through the goggles even when they get a little bit cloudy.

Finding my tune~

Sunday, January 12, 2014

Who do we dress or undress for? :)

 
As I am engulfed in the Golden Globes, it made me wonder, who do I dress for? What is the perfect dress? I think I have always been less than perfect but always wished I had that look. The breathe taking OMG look that every guy wants and every girl wants to be. Throw in some Christian Louboutin and its a perfect night. :) Who do we dress for or should I say undress for? Lets face it, we usually dress for other women. Fashion is fun. However, I will say, some of the guys really stood out to me. I love a man in uniform or a great tux.

Ok so I am obsessed with fashion so to me award show fashions really are what the award season is about.

 http://nydn.us/1kx9Rdu

The New York Daily News (link above) showcased some beautiful dresses. I love the simple but elegant look. Lupita really stole the red carpet last night. It very much reminded me of Paltrow from a previous year. These were just some of my favorites.




 

Maybe this is why I love pageantry. I get to look for that perfect dress, which I have yet to nail but it sure is fun looking for them!

What is your perfect look? The best answer I ever received was the look that you feel like a million bucks in. Fashion is a great deal about finding your fit and your color. Perfection is perception so I will continue to look for my perfect. I am such a girl. I just love fantasy!


Finding my tune~




Sherose

Throughout the years I have learned that everyone has a story, a reason, a purpose. They can be big or small but in someway we all impact someone. Yes, there are bad situations but I think so many more good impacts. When I was in high school and college, I used to volunteer at nursing homes. I so admire ANYONE who works in geriatrics because it is tough.  Emotionally and physically. What that time taught me was to listen and you just might hear some amazing stories.

Over the years I have continued to listen and not disregard someone because their story is theirs and judge not for shoes you have not walked in. In pageants, especially platform based pageants, you really come to realize how much people are impacted by life and this creates their stories. There are so many heartbreaking illnesses that it truly can shape a person and also life events such as tragedy or abuse.  I often think abuse is over looked because it is uncomfortable. It's embarrassing to live through it and uncomfortable for people who have never lived through it to talk about it. It always makes people question your choices or how or why did you put yourself, or keep yourself in those situations.

I was fortunate to meet a wonderful and amazing lady named Laquisha Hall at the Mrs Maryland pageant about 5 years ago. She was not only brave enough to share her story but it was also her platform. It struck me in a way that yes, I felt uncomfortable because I just want to "fix" it but also because I related to it on many levels...which made me feel more uncomfortable. I think it would surprise some people to find out how many people go through abuse either in the hands of strangers or in the hands of someone you know and love. It's about control and it is terrifying.

It's hard to explain my childhood. I really don't remember a lot of it. We moved a lot and there was always tension at the house. I was always academically inclined but I spent a lot of time isolated. This is why skating became my escape. If I could hear the music, it was a peacefulness and beautiful place. I dreamed of getting out, sleeping trough the night and not under the bed and just hearing that music everyday. I ran away 6 times in high school. My friend and I drove from Florida to Canada. Yep, Canada. I was not promiscuous and did not do drugs, I just wanted peace. Because I was academically inclined I mange to graduate HS on time after missing so much time ( I was in honors classes and had some pretty amazing teachers).

I try to shut a lot of it out but the reality is that my past makes me who I am. I don't want to relive it, cast blame or victimize myself but it was my reality.  It's always interesting to me that the man I was the most afraid of is also the one that encouraged my dreams. He was a dreamer too. This was the level we connected on.

We were watching " Lifestyles of the Rich and Famous" one day and they had a segment of the American Academy of Dramatic Arts. He dared me to audition. I applied and audition and much to my complete surprise was excepted. I think I watched Fame and flash dance way to many times. Throw in some Dirty Dancing and you can see were my head was.

He gave me a lot of independence but yet controlled. I had a lovely Ford Escort Wagon, yes a very sexy car, as I embarked on driving from Florida to California by myself to chase the dream. My car was pretty full but not organized and I had enough money for a few hotels and enough tuna cans for food. Let the dream begin.

Florida is a long state, period. Even from Tampa its 6 hours to the state line especially when getting on 10. I drove with my cassette tapes blaring and my hopes of Hollywood. I have always been a drinker and no not always alcohol:) but diet coke so I always have to go to the bathroom. I have been checked time and time but you will rarely see me without a beverage in my hand. Another story for another day but my Dad once left me on 20 while driving to Arizona because I had to make another stop. Hey when you have to go you have to go!

I stopped at a rest stop in Louisiana. It was not really populated but my Grandpa was a truck driver and I just was never afraid of stops. I still remember what I was wearing. I remember the glasses. I came out and there was a white car blocking mine in. He presented himself as a cop. He was dressed the part.

He started asking me questions and said there was some drug thing so he had to search my car. He opened up my back door and my pink bunny fell out.  My parents when I was younger had given me a pink bunny and a teddy bear. To this day, they are in my car everyday. I don't recall a lot of what happened. I survived and I don't know why. He was put to death in 2006.

I just blocked so much of this until last year. I had a neighbor who took advantage of a situation while my husband was deployed and I had a friend in distress. I had truly forgotten of sorts but just made me a mess. Andy was called in Afghanistan because of the attack and I just feel I was too liberal in my allowing him in my home to help her.

I was a mess last year but I allowed myself that luxury. Shame on me.

NO ONE should ever make you feel the victim, or take away who you are or who you will be. If they do, they win and frankly its about control. I could not over power but I survived. If you allow yourself to be the victim, they win. Be you and never apologize for what you cant control and embrace what you can. Be the rose. We all will find our time to bloom. Thank you Laquisha for giving me the courage to share.

Friday, January 10, 2014

Taking a deep breath!

It was 5 years ago that my amazing son was born. We were given grave prognosis at times. Carter has chronic lung disease and we were told he may be on a trach for life. We were also told he would be blind. It's been a journey but today I made a call I never thought I would be making. Please pick up our oxygen tanks because he doesn't need them anymore. I did reserve the condenser but Carter has not needed supplemental O2 for a year and sees with his ever so fashionable glasses! 2014 is looking good! His sister thinks so too!





Say Something......

Music is such a big part of me. Even in my darkest hours or brightest light of the day, it's music that gets me through. It's a language that is universal. When I was a little girl, I was a skater. My family did not have resources for lessons but I skated everyday for 6-8 hours a day. I taught myself how to do a double axle and had Olympic dreams. Keep in mind I also was in a neighborhood pageant and was crowned with a Burger King crown so I have always been a bit far reaching in my dreams.

My skating was more about the music. It was my escape. When the weather was really bad and I could not go outside, I could still hear the music as I looked outside.

Think about lyrics. Aren't there some that you would really love to say to someone but don't have the nerve. Music is the excuse and a blessing. Always listen to the lyrics and hear the melody. So many stories, so much truth.

Finding my tune

Thursday, January 09, 2014

Angels

Do you believe in angels?

I have really gone back and forth on this for sometime. I know the reality and I also understand the fantasy. There is a certain something to believing but in actuality for me, I kind of do. I look back on my life and the vast amount of situations I have been in and someone has been looking after me. It's a strange thing, especially when it comes to dreams. What prompts them?

This holiday season came with so many weird dreams. My grandpa, Benjamin Lee, died many years back at Christmas. I was working at HSN at the time, overnights, so I slept a lot in my living room chair. I woke up, it was maybe 2am and I swear he was leaning on my kitchen table staring at me. I still remember that. He was a great man.

Since my parents passed, I wonder sometimes. I do feel they are with me and maybe have yelled at me a time or two.

Ok at the possibility of sounding crazy, I am choosing to believe in angels. God has always put people in my life that have saved me, changed me and just made me a better person.

There are angels amoung us for sure.

Wednesday, January 08, 2014

FAST. Stroke Warning Signs





January has been a rough month for me for the past 5 years. I don't know what it is but my body just goes haywire every time a new year begins. It may be the stress after the holiday or just that time of year but it always is gloomy. I am working on changing that this year and so far so good. It was a year ago January 18 that I had a stroke and ended up in the ICU for a week. I had a heart attack and surgery in 2010 so I really did not see this coming. For me I have bad jeans, literally. My parents both died at 54 and 56 so that genetic component has been a bear for me. On top of that my mom was adopted so understanding fully my family history comes with some challenges.

I have to say it was scarier than my heart attack. After my heart attack, I was in the ICU awaiting surgery but I felt fine. My troponin levels keep going up but I felt fine. The stroke was very different. I had had a few TIA's ( pay attention to warning signs) but ignored the obvious. When I had the stroke, I lost my ability to talk, my vision went crazy and I just felt out of control of my own outcome. I was so scared more for my family.

Throughout the year I received therapy. I admit I still have not been the best in my recovery and I still limp and my vision has taken a toll. I am so blessed to be ok. The good and the bad is through my heart disease, stroke, bruising, raynauds syndrome, multiple pregnancy losses, prematurity and  eye disease, they think they found the cause which is vascular. Can this be fixed, no but managed yes.

The reason for this post is because I was stupid but thanks to a great friend, Juliana Mckee and my family, I survived BUT the important thing to remember is to act FAST if you think you are having a stroke and to realize that 911 can be your friend. Hey, I did get a new door out of it but I am forever blessed and grateful for true friends.

I am young, I didn't fit any mold for Cardiovascular Disease or stroke. No one is immune so please learn more at www.heart.org!

More info:

Stroke can and often does occur without warning, and its effects can be devastating: Stroke is the fourth-leading cause of death in the United States and a leading cause of disability. For people experiencing the symptoms of stroke time is of the essence. The symptoms of stroke need to be recognized quickly and immediate action needs to be taken. Learn all about F.A.S.T., which is the acronym being promoted through the AHA/ASA's Together to End Stroke initiative as an easy way to remember the sudden signs of stroke. The F stands for face drooping, the A for arm weakness and the S for speech difficulty, all of which are major symptoms of stroke. The T stands for time, which emphasizes the severity of the situation and indicates the need to immediately call 911.

Tuesday, January 07, 2014

The Lollipop Kids Foundation

I have been involved with a lot of organizations and to be honest, it is life circumstances that have introduced me to them. Since the birth of my son, 5 years ago, we have been blessed to have resources to obtain great healthcare, supplies and medical equipment. Most people who have children with disabilities do not have these resources. They are costly and most are not covered fully by insurance. I wanted to donated Carters equipment as he was outgrowing them and was guided to an amazing organization called the Lollipop Kids Foundation. http://lollipopkidsfoundation.org. It was started by a lady name Debbie Sahlin who knows first hand the struggles that families go through. This is a non-profit that I am proud to work with and I hope you will take the time to visit the website and learn more. Volunteers, support and donations are always needed. We all can make a difference in someone life everyday. To me, it starts with the children. I love my little man (and his sister:)  and so proud of his journey. As a Mom and I think most can understand this, I will do whatever I can to help him hit those milestones.  He has already exceeded expectations and will continue to do so. Its foundations like the Lollipop that changes lives and gives the children the chance to thrive.

 Carter


 

Mission

Children with disabilities have the right to be given opportunities that allow them to live to their fullest potential. Too often, parents raising these children feel isolated socially and burdened financially, emotionally and physically. They are forced to make hard decisions and sacrifice substantially in areas that most could not imagine. The Lollipop Kids Foundation exists to combat social stigmas, ease the financial burden placed on these families, offer emotional support and provide a brief respite so that every child with a disability has access to a hope and a future.
Guiding Principles
Love
Love encourages and inspires. Children with disabilities encounter prejudice at every turn. Love empathizes and resists the desire to look away. The Lollipop Kids Foundation believes that love discovers a passion within that fosters understanding and creates a desire to help.
Faith
Faith implies complete trust. Every child with a disability must have faith in himself or herself. Faith strengthens self-esteem and confidence. The Lollipop Kids Foundation believes by building the faith children with disabilities have in themselves and their environment, we empower them to prosper.
Hope
Without love and faith, there is no hope. Hope sustains and motivates, it enriches outcomes. Hope helps each one of us say no to despair and yes to endurance. The Lollipop Kids Foundation wants to give each child with a disability the gift of hope.

Monday, January 06, 2014

A disability does not make you disabled

Judgment is hard. Its hard to receive but yet so free to be given. I see it everyday. It was hard for me to accept that I have a child with special needs. As I compare, even as a Mother, its hard not to sit back and judge myself. What did I do? How did I cause this? The worst for me is what others think or say. Its been hard and I wont lie. I sit back and look at other children, including my own, and just wonder why. With Carter it was 165 days in the NICU and 3 years of question with his chronic lung disease and Cerebral Palsy. He is still gtube fed but is eating. This has to do with his lungs but after 3 years of not being allowed to eat, eating has also become a challenge. I wish I had this problem.

Carter turned five on January 2nd and pardon the crazy Mom bragging but is amazing. He breathes room air, is physically beautiful and can trash my house with the best of any 5 year old. He is truly amazing.

This brings me to judgment. I am somewhat jaded because we fought so hard for Carter after 4 previous losses. I use to walk in the mall with him and have STRANGERS question why he still has a binky or may have been leaning over to one side. (he does not do this anymore) However, I just reflected back on an amazing person in my life when I wanted to yell at them and say, "YOU DON'T KNOW MY LIFE". I did want to follow up with some creative vocalizations but people can and have been stupid. They look, they judge, they assume. Yes, on any given Sunday, I have had my babies in a bar:) Football and we walk but sometime you just need to take a walk.

  The one thing I always say to my son is that you may have some disabilities but you are far from disabled. I will always believe and never judge. After all, you don't know his life.

Sunday, December 15, 2013

2014, hello maybe?

I have learned in my years that expect the unexpected. 2013 has been an interesting year. I use to say 13 was my lucky number, maybe not! I can't say it was completely unlucky. My husband came home from Afghanistan after his 10th deployment. When he was gone, much like car problems, everything happened. I have recovered from my stroke and my family got me through. I think every week someone was here. My eyes are going. Then in September, we had a miracle. I love my family.

One of my highlights was visiting my family in SC over thanksgiving. The last time I saw many of them was my Daddy's funeral and unfortunately because my Grandpa passed the same night as my Mom in 2004, ironic, no one could make it. Past the little pity I just had, we Pembertons rallied. I think it's in our genes. I am so proud of my cousins, Uncle, Nephew, Brother and Memaw. It's the land of Doctors, Nurses, Businessmen/women , military and  I frankly felt a little unaccomplished. So proud. We Moms rock too! My Aunt Robyn has done so much for the family and her children reflect everything that she gives.


So what's for me in 2014? Let's plan for a great January first. I grew a lot this year, all over :(, but I am starting over. Fresh.





Putting this out there...If I did anything to hurt you, I am sorry. I am so not perfect. Yes, my apologies have been blatantly turned down even when I have no clue what I am apologizing for but it's life. I am still sorry.

I believe in this Christmas season in love! laughter! faith and hope. I pray for us all great health in 2014.

Time to celebrate. Let the New Year begin!



Tuesday, December 03, 2013

My life long wish

The holidays are always filled with emotions. I am beyond blessed that we spent Thanksgiving with 18 members of my family.  This included my Memaw.  Dec 18 is her birthday and Dec 22 would have been my Moms.

This made me think of my favorite Christmas movie, " Miracle on 34th Street". Yes, before "Hostages" Dillon McDermott was so nice. Lol. ( I also have all the earlier versions!) 

So I have made my own Christmas list.  I also love Amy Grant so I am borrowing my Grownup. Christmas list from her

Love
Laughter
Acceptance
Understanding
Forgiveness
Understanding 

And maybe we van throw in a Gibson, some 3 carrot ( lol) earrings and three chords and the truth for good measure.

Happy Happy Happy Holidays! 

Be blessed

D




Sunday, November 10, 2013

Heart Walk 2013



I have been so blessed to work for the American Heart Association and volunteer for them also. 13 years and many events later, I volunteered at Heart walk yesterday! The great part about being a survivor is when develop a family within it. You're the Cure is an arm of that. A rather hefty one that does so much on the hill so I was thrilled to see Jennifer Witten yesterday. I also got to catch up with one of my favorite people, fellow survivor and amazing fundraiser, Senator Frank Shore. He calls me his QUEEN of Hearts. How can I not love that!

The GWR Heart walk raised close to 1.3 million this year and the walk on the National Mall was amazing. I was really excited for the puppy depot!

Beautiful day, I survived the Metro  and got to enjoy catching up with some very special people.

Every step matters!

Here's to your heart!

Sunday, November 03, 2013

Mrs Maryland America 2014

 I have done pageants for a while and I always find them to be a point of self discovery. Sometimes, not always good but I learn a lot each time and mostly about myself. I competed in Mrs Maryland America 2014 last weekend and I had a great time. I went back and forth on whether or not I should but at the end of the day I just decided to make a girls weekend out of it so I did. On Friday, Cara Cole, another military spouse, and I hit the road and checked into the hotel. Jessica, another contestant also shared our room and it reminded me a bit of a college day slumber party. We all three have children so it was Mom's night out!

Orientation was fun and we gave out our contestants gifts. I loved all of mine and a common theme was the gift of wine and some homemade(thank you Alyssa!). Thank you to SKINN Cosmetics for supplying my gifts of beauty!!!! All of the ladies received a sponsored gift by SKINN. (www.skinncosmetics.com).

We stayed up after orientation, rehearsal and dinner until 1am in the morning just talking! We may have cracked into some wine too. It was so much fun. The next day was rehearsal, interview and finals. All of the ladies rocked it and when top 5 was called, I was honored to be among them!

I placed second runner up, photogenic winner and interview winner. I was so happy and still am! Congrats to Zereana Jess Huff, your new Mrs Maryland America 2014. She is an Ovarian Cancer Survivor, a beautiful lady and will rock it at Mrs America.

I have to say the ladies were ALL just amazing. Thank you for the smiles!

My heart smiles because of you~

Deanna Maria
 

Thursday, October 17, 2013

Legacy

Legacy precedes us. Some in greatness and some in shame. We cant shape those before us but sometimes we sit in admiration. I am a Pemberton. What does that mean? I am not sure at times except we don't invest well. We are all very smart. A history of doctors, chemist and amazing people.

So as history is told, and before anyone gets confused my family lost it all, but yes we are the Pemberton's that created Coca Cola. I personally lived in Atlanta until 7th grade. OK so it was a drug when it was created but I am still addicted.

I wish I had that genius gene but as I always talk so negative at times, we did make history so lets have a Coke and a smile:)

Friday, September 06, 2013

Bella's Story


Bella

 


Isabella Lillian Linz

Born April 19, 2006

At Portsmouth Naval Hospital

 

Bella became very ill her second of life and was given a diagnosis

of VLCAD by the Virginia State Newborn Screen

 

For more information contact:

Carrie & Tom Linz

814-573-1636

Carrielinz@hotmail.com

 

A muscle biopsy determined Bella has VLCAD-C the most severe form of VLCAD

Very Long Chain Acyl-CoA Dehydrogenase Deficiency - VLCAD

VLCAD deficiency is a rare genetic inherited fatty-acid oxidation disorder that is characterized by cardiomyopathy, fatty liver, skeletal myopathy, pericardial effusions, ventricular arrhythmias, and sudden death. The condition prevents the body from converting certain fats to energy, particularly during periods without food (fasting). People with this condition have inadequate levels of an enzyme that metabolizes a group of fats called very long-chain fatty acids. These children are also at risk of serious complications such as liver abnormalities and life-threatening heart problems.

1.
Avoid going a long time without food
Children with VLCAD must eat frequently to maintain their energy level and prevent a metabolic crisis. Bella requires a G-tube to receive calories throughout the night while sleeping.
2.
Diet
A low fat, high carbohydrate, high protein diet is recommended. Carbohydrates give the body many types of sugar that can be used as energy. Most food in the diet should be carbohydrates (bread, pasta, fruit, etc.) and protein (lean meat and low-fat dairy foods).
3.
MCT oil and L-carnitine
Medium Chain Triglyceride oil (MCT oil) is often used as part of the food plan for people with VLCAD. This special oil has medium chain fatty acids that can be used in small amounts for energy.  Some children may be helped by taking L-carnitine. This is a safe and natural substance that helps the body make energy. Bella is currently participating in
a FDA trial of Triheptanoin. Triheptanoin is a triglyceride that is composed of three seven-carbon fatty acids. These odd-carbon fatty acids are able to provide anaplerotic substrates for the TCA cycle.
4.
Call your doctor at the start of any illness
Immediate medical assistance should be sought if the following symptoms are observed:
poor appetite        low energy        vomiting            Reddish-brown urine        a fever       muscle aches & weakness
Children with VLCAD need to eat extra starchy food and drink more fluids during any illness - even if they may not feel hungry - or they could develop hypoglycemia or a metabolic crisis. When they become sick, children with VLCAD often need to be treated in the hospital to prevent serious health problems.
5.
Avoid prolonged exercise or exertion.
Long periods of exercise can also trigger symptoms.
If muscle symptoms happen, prompt treatment is needed to prevent kidney damage. Children with muscle symptoms should:
  • drink fluids right away
  • eat something starchy or sugary
  • get to a hospital for treatment
VLCAD Children can commonly experience episodes of illness called metabolic “crises”, periods when their bodies become toxic, and break down muscle.  Once started, metabolic crises usually cannot be reversed w/o medical intervention.  Early signs of crises:
Extreme lethargy, Behavior changes/Irritable moods & poor appetite.
Bella is prone to Rhabdomyolysis , a condition in which muscle tissue breaks down rapidly.  Breakdown products of muscle cells are released into the bloodstream; some of these, such as the protein myoglobin, are harmful to the kidneys and may lead to kidney failure.  Some symptoms may include muscle pains, vomiting and confusion.   The mainstay of treatment is generous quantities of intravenous fluids.
Bella also struggles with increased CPK.  Creatine phosphokinase (CPK) is an enzyme that can be found in the heart, brain and skeletal muscle. A high level of CPK is not a good sign and is an indicator of heart attacks, strokes and other serious diseases. A level above 250 is considered abnormal. Her highest CPK was over 69,000.
The 2013 has been a difficult year for Bella.  She has been in the hospital 8 times already this year.  Bella enjoys being as active as her body allows, but unfortunately her specialists have had to put her on limited activity.  Although Bella qualified for States in Gymnastics in early 2013, due to her VLCAD and increased hospital stays, the doctors were not able to let her compete and have told Bella as of now she can no longer participate in Gymnastics.  Bella is scheduled to return to Oregon Health and Science University Hospital for more research testing.  Doctors are hoping to learn valuable information from her to benefit children in the future.  After her stay in Oregon, Bella will return to Pittsburgh Children’s hospital for more testing and an opportunity to participate in another form of treatment.
 
 
 
 
 
 

 

 

                                                                

Additional Research or Information:

www.fodsupport.org

www.gemssforschools.org/conditions/vlcad

www.newbornscreening.info/Parents/fattyaciddisorders/VLCAD