Depression

Tweet I've been vocal about living with depression but I don't think I've ever really posted about what it's like when I'm depressed. Thankfully, through medication, I don't have to deal with depression very often. 

However, I'm dealing with depression right now. I hate it. I hate what it does to me and I hate what I, in turn, do to everyone around me when I'm depressed. 

Yes, I'm on Zoloft and my life is so much better because of it. However, it's not perfect. Everyday is not some miraculously wonderful stress-free day. Depression still slaps me in the face at inopportune moments...Zoloft just helps it happen less often. 

Right now, I want to run away by myself without anyone being able to find me until I'm ready to be found. 

No, I'm not going to do it. Even at my worst, I'm still rational. I have two wonderful kids who depend on me and need me. That fact never leaves my thoughts, although I often wonder if things would be different without antidepressants. It scares me enough to know, without a doubt, that I will continue to treat my depression with medication for as long as I live.

Right now, everything is overwhelming. Everything is too much and I want to walk away from my responsibilities. I want to turn in on myself and ignore the rest of the world until I feel like myself again.

I know that I can't do that, regardless of how much I want to and I am grateful for enough clarity to realize it. 

Right now, I want to be alone. It literally exhausts me to have a simple conversation with someone. Chitchat requires more energy than I have available to expend.

Right now, I'm numb. I don't connect with other people the way I should when I'm depressed. I don't feel appropriate emotions, positive or negative, when I'm depressed. I view life happening around me like an observer & not a participant.

I unintentionally hurt people when I'm depressed. I know it. I see it happening and I am powerless to stop it. Feeling powerless is part of my depression. Being powerless is one of my biggest fears and depression is good at using that against me.

I used to feel like this all of the time. Now, it just hits me every once in a while but sometimes I think it's harder on this side. 

I know what it feels like to enjoy life now. I know what it feels like to wake up in the morning aware of the fact that I am blessed in so many ways.

I know what it feels like to be happy and I know exactly what I'm missing.

24 Weeks - a comparison

Tweet In my last post, I didn't really want to do a comparison shot between my pregnancies with Klaw & Scream. I changed my mind.  I'm just over 24 weeks pregnant with Scream and I decided to just go for it. I think it's neat to compare the two pregnancies. I'm a little bigger this time around, but I feel like my body & belly is shaped almost exactly the same with the two boys.

Thankfully, everything is going well. I'm still pukey, but I have accepted that this is just how I do pregnancy. Scream seems healthy and is very active in the womb. Very. Active.

I've been reading some new (to me) pregnancy & birthing books. I tend to be interested in the books discussing the socio-economic/cultural aspects of the American birthing system and books that affirm our bodies' abilities to give birth without intervention, barring emergencies.

On the one hand, it's absolutely amazing how much we are able to do with modern medicine. So many lives, both babies & mothers, can be saved now that would have been lost decades ago. On the flipside, it seems so counter-intuitive to prescribe these amazing life-saving interventions to women who are capable of having a medically uneventful birth.

No, I'm not getting into a debate over which model (midwifery or medical) is better. I have my opinions about what appeals most to me but I won't begrudge another woman the decisions she makes or the healthcare provider she trusts & is most comfortable with leading up to & during birth.

Personally, I like to keep my feet in both worlds by birthing in a hospital with both a midwife & a doula. That way, someone else has to clean up the mess and my dogs aren't all up in my business. Balance.

What is some of the best advice you have heard regarding pregnancy &/or childbirth?

For the Love of Kathryn (TTTS)

Tweet

Today, a dear friend of mine, Alexa, is sharing the story of her experience with Twin-to-Twin Transfusion Syndrome (TTTS).

 Alexa and I met while we were both living in Charleston, SC and became fast friends sharing a love of laughter, good food, and, well, shenanigans. We met through a mutual friend, eventually lived in the same apartment complex, and she helped me get a job with her at a tiny little French restaurant. Later, I moved on to outdoor education and running a canoeing program while Alexa learned about four different languages in her spare time (I may be exaggerating, but not by much) and joining the military. Years passed and we have reconnected on another level as mothers.  Alexa's experience with TTTS was the first time I had ever heard of the syndrome and I find her story informative and her strength inspiring.

For the Love of Kathryn

Did you know that TTTS kills more babies than SIDS?  TWICE as many babies!  Was your first question after you read that “What the heck is TTTS???”  A year ago, that would have been my reaction.  Yet I bet everyone is familiar with SIDS.  Well, I am altogether too down and personal with TTTS because it killed my daughter.  

TTTS is Twin-to-Twin-Transfusion Syndrome.  If you EVER know of anyone pregnant with identical twins, you need to tell them to stop, do not pass go, and head directly to the TTTS Foundation webpage.  (Contact information at the end of this post.)   And don’t allow them to be like I was.  My thought was “What can some woman in Wisconsin do to help my situation??”  So I never made that call to Mary at the TTTS Foundation.  And that will haunt me forever. Turns out, she has saved a LOT of babies. 

I was stupid.  Ignorance is not always bliss.  In my case, ignorance is a lifetime of pain and loss.  Earlier intervention, more ultrasounds, knowing the signs and symptoms, knowing the treatment options (since even many OBs do not!) can help change the outcome for babies.

Our story is written.  But I can try to help change someone else’s story.  That is why I am dedicated to raising awareness.   So to do just that, I’d like to share some of our personal journey.

September 28, 2011.  It was approximately 1:30 p.m.  My husband and I watched the ultrasound screen in amazement.  Identical twin girls!  Even though we found out very early, at just six and a half weeks, that we were pregnant with twins, we still were reeling from the idea!

This being our third pregnancy, we were experienced with the 20 week ultrasound, but I wanted my husband to be there anyway.  I knew he’d regret it if he never got to see how the awesomeness of the two babies interacting in the womb.

Thank the Lord he was by my side that day.  

The ultrasound tech seemed different to me that day.  There was something about the way she was telling me the information.  She was very matter of fact, and seemed to be in a hurry.  I commented on how big these girls were going to be when she told us Baby A already weighed 15.8 oz at 20 weeks and 3 days.  And how odd that Baby B only weighed an estimated 8 oz…

Then, the words that forever changed my world.  “See this black area in Baby A’s belly?  This is fluid.”  As soon as the words came from her mouth, tears started streaming down my face.  My husband looked at me and laughed.  “Don’t start imagining scenarios!”

“This is bad hon,” I told him.

And it didn’t take much longer before he understood the degree.

The ultrasound technician hurried through the rest of her exam and told us that we had TTTS and the Doctor would tell us more about it.  As we sat with the doctor, she told us the basics – when twins share a placenta (called monochorionic-diamniotic or mono/di twins – one placenta, two sacs) there is a high risk that they will not share fluids from the placenta equally.  One twin winds up “donating” fluids and the other receives too much.  This is why there was such a size difference in the babies.  The “black” area in the belly indicated hydrops fetalis – or a build-up of fluid in the baby’s abdomen, indicating heart failure.  

Not all babies with TTTS develop hydrops… the craziest thing about TTTS is how varied the outcomes can be.  Single loss, double loss, double survivors, some with severe disabilities and those that have no disabilities at all.  And then, because many of them are born so premature, they are faced with all of the challenges of severe prematurity.  There is no certainty about anything with this disease of the placenta.  But one thing is certain – you can’t fight something you know nothing about!  So learn, be aware, and share that awareness!  You never know when someone in your life may be impacted by this!

The card below gives some very key points and information.  

The details of the next few minutes, hours, days, weeks would fill a novel for me to write.  If you are interested in knowing what transpired in the months to follow, please come visit my blog “No Holding Back”.  Luckily our story doesn’t end completely dismally.  Our donor baby, Tiny as I refer to her, although only 1lb10oz at birth, has overcome all odds and is a perfectly healthy and very happy baby!

Thanks to Dana for allowing me to use her Blog as another opportunity to spread awareness!

An Interview with Colby Wren #KnowAboutMito

Tweet Last fall, Colby Wren hit the front page of  "mito" news when he was interviewed by several news outlets, including the Atlanta Journal Constitution and CNN. He was a baseball player for Georgia Tech, is the son of Braves Manager, Frank Wren, and lives with mitochondrial disease. When I read an article and found him on twitter, I immediately sent him a tweet thanking him for going public with his struggles.

We are big sports fans in this house and I have mentioned our hopes that Klaw will be the best pitcher ever recruited by the Chicago Cubs. When we got the VLCAD Deficiency diagnosis, we were afraid that Klaw would no longer have the option to pursue sports even if he desired it.

Over the past year, I've had the opportunity to get to know Colby better and I'm glad that he can be a role model for Klaw and other kids with mitochondrial disease. Colby's story highlights how different mitochondrial disease an cbe for each person affected and it serves as a source of hope and determination.

Colby took the time to answer a few questions for me to feature on this blog during Mitochondrial Disease Awareness Week:

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1 - Thankfully, many infants & children are being diagnosed with mitochondrial disease. You weren't diagnosed until you were high school. What were some of your thoughts when you got the diagnosis?

Some of my thoughts were how am I going to have to change my life/lifestyle and what am I going to have to give up or start doing differently.

2 - As an athlete, you put your body through more stress than the average person. When you add mito to the equation, you have to take even more precautions to maintain your health and stamina. Describe a typical day with how you prepare yourself for baseball and how you recover.

Well, now I am a student coach at Georgia Tech so I have had to change up my routine. My old routine was wake up for a 6 AM workout and then hope and pray that I had enough time for a nap before my next class. Practice from 3:00-6:30 and do it all over again. Things that helped me and my body recover were proper nutrition, knowing my personal limits in workouts and exercise, and as much sleep as possible throughout the day.

3 - Looking back on your younger self and knowing what you have learned about mito, what were some of the biggest warning signs that are clear with 20/20 hindsight? 

Well my gastrointestinal problems have been apparent since I came home from the hospital. (bad reflux and couldn't hold down food as a new born) I would always get sick always throw up, but since I never truly played a sport that was extremely high intensity until I was in 4th grade I didn't see a lot of the more intense signals and problems occur.

4 - What advice do have for parents of mito kids who are athletically inclined, to the best of their abilities? What should we pay attention to when our children are playing or exercising that a child may ignore because they are having fun?

This is my personal opinion and knowing that each child is different here are some basics that can be applied to a larger spectrum.

1. Know your body and your personal limitations with energy. 

2. Be very careful about heat and hydration. The hotter the days the more water needed and the less activity should be accomplished. 

3. Slowly work into athletics or exercise. Never just jump into things until you have specifically talked with your doctor/physician about what he thinks should be your boundaries and work from there.

4. There is a big difference between pushing yourself physically and being stupid. There are times when a little extra push or energy is okay, but then again it is up to the individual. DON”T try to impress people and end up hurting yourself I have done that many times and it never ends up doing me any good in the long run.

5 - What advice would you give a kid like Klaw who will likely face similar issues that you have dealt with should he pursue athletics?

I would give any kid with the ability and well-being to play athletics to pursue them and just enjoy every bit of them. There are some kids who just hate sports, but if you have the passion or the itch you just have to go for it. Keeping yourself active is not only good health wise it is also good for you energy levels. Whatever you can physically do can help your body and have fun at the same time. It teaches life skills and personal skills that cannot be taught by a book, but by just experiencing it first hand.

6 - Can you give some info in the foundation you work with and how others can get involved?

The Foundation for Mitochondrial Medicine supports the development of the most promising mitochondrial disease research and treatments of the many forms of mitochondrial disease. Formed in 2005 and renamed in 2010, our Atlanta-based non-profit organization financially supports treatment based research. From functional brain MRI studies on cognitive fatigue to testing of new drug compounds, including the first FDA-approved drug treatments that began in early 2010, FMM focuses on supporting Institutional Review Board (IRB) or FDA approved studies. Stated simply: we're funding the cures.

The foundation's stewards and founders are parents of patients and medical experts. We were created to accelerate the development of the most viable mitochondrial disease treatments and therapies.

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To read more about Colby Wren's story, check out these articles:
Son of Braves Manager Battles Genetic Disease (AJC)
Human Factor: College Athlete Battles Mitochondrial Disease (CNN)

Light a Light for Mito #KnowAboutMito

Tweet Each Wednesday during Mitochondrial Disease Awareness Week is set aside to remember the lives that have been lost to mitochondrial disease.

MitoAction

Mitochondrial disease runs the gamut from fairly manageable disorders that have little effect on overall life expectancy to disorders that still carry an overall fatal prognosis. For Klaw, his first year of life was the most tenuous but management of his VLCADD has been successful. He is expected to lead a fairly typical life with continued health management and regular observation of his liver and heart functions, barring any surprises or major illnesses which could throw him into a metabolic crisis.

For others, there is a steady decline in the function on major health systems.  Many children with mitochondrial disease do not survive to reach adulthood. For some adults, a typical, productive life can suddenly take a downturn to debilitating symptoms with little to no hope for improvement.

If you or someone you know has lost the battle to mitochondrial disease, please take moment to light a candle in their memory.

VLCADD Friendly Convenience Foods #KnowAboutMito

Tweet People regularly ask me how we manage Klaw's diet in relation to his VLCADD. He is currently limited to 6g fat per day. Most toddlers should get 40-50g fat per day. As you can imagine, this can make it difficult for us to just pick up something at the grocery store or at a restaurant for him to eat.

In order for Klaw to eat "fun" meals that incorporate a variety of flavors and foods, we pretty much have to cook at home. It's the only way we can guarantee the amount of fat in each serving. However, sometimes I just don't feel like measuring out each ingredient and using MyFitnessPal or another online recipe app to do the math and determine the fat/carbs/protein in each serving.

Sometimes, I just want to grab something, read the label, & hand it to my kid. Chris & I have found a few favorite "convenience" foods that we keep around the house that are low to no fat and that Klaw loves.

Every (non allergic) kid loves a good PB&J, right? Well, not when one serving of peanut butter has more fat than you can eat in a day.  Thankfully, Trader Joe's carries Better 'n Peanut Butter. It has 1g fat per tablespoon. We use that with sprouted whole grain bread, which is high in dietary fiber, high in protein, & low in fat. Our favorite sprouted grain bread is from, again Trader Joe's. Sprouted Flourless Whole Wheat Berry Bread has no fat at all. So, Klaw gets a full PB&J for a whopping 1g/fat.

We don't just shop at Trader Joe's, I promise. Our local Food Lion & Farm Fresh carries many items that are VLCADD friendly, too. Happy Baby Organic Puffs are fat free, taste like cardboard, and great for road trips. Happy Baby also makes some fat-free yogurt drops, Happy Yogis, in a variety of flavors, too. Yes, these snack items are pretty much devoid of nutrients but sometimes non-staining convenience wins. (These yogurt drops do not stain, in our experience!)

Most stores, including Walmart, are carrying the GoGo Squeez apple sauce packets. Apple sauce is, you guessed it, fat free. Yes, it's full of sugar but Klaw burns energy from sugar pretty efficiently. He's a VERY active toddler and he can't burn fat for energy. As long as he's going, going, going, the sugar in applesauce is not a big issue. A few other brands of squeezable sauces are also fat-free, but the GoGo Squeez brand seems to be pretty ubiquitous while we have to make special trips to Babies R Us or Target for the others.

Pasta & marinara sauce is a quick & easy VLCADD friendly meal we frequently cook. However, I don't always want pasta. Gerber Graduates Pasta Pickups make two flavors, chicken & carrot and turkey & vegetable, which are low fat, easy to heat up, and Klaw loves both flavors. We do limit how often he can have these but it makes for a quick, convenient lunch or dinner option. Especially if Chris & I want to have something high fat like pizza or, well, just cheese for dinner.

Pediasure Sidekicks Clear is a "juice" drink box for toddlers. It's completely loaded with the full spectrum of vitamins & protein. It doesn't contain dyes. Granted, it doesn't contain juice, either. However, Klaw pulls the toddler pickiness every once in a while and this drink provides a quick, convenient way to make sure he ingests calories and protein when he refuses to eat a meal. I don't know of any other fat & dye free drinks that also offer protein, so this is the best I could find. Granted, I have no issues giving him Gatorade Prime on occasion, if the situation and energy expenditure warrants it.

We plan for the unexpected each trip each trip, whether it be traffic delays or just an overexertion by Klaw at play. We always have some of these convenience foods tucked away in Klaw's bookbag (our diaper bag) in case he needs a snack or if we just decide we want to go to a restaurant to eat. We can't take it for granted that we'll be able to find something for him to eat on the fly.

Do you have an "convenience" foods you keep on hand for your kids? What are they?

Mitchondrial Disease Awareness Week 2012

Tweet Mitochondrial Disease Awareness Week is September 16-22, 2012. I've planned a week of posts that describe different aspects of mitochondrial disease and how it affects us in more ways than most people realize.

United Mitochondrial Disease Foundation

There are events taking place all over the globe to raise money and awareness for mitochondrial disease, the numbers of which are approaching, if not surpassing, the number of children afflicted with cancer. However, mitochondrial disease is not limited to children. It can affect people of all ages and in many different ways. As research continues, we are finding more connections to mitochondrial dysfunction in illnesses/disorders like Alzheimer's Disease, diabetes, autism, and many more.

If you don't think mitochondrial disease affects you, think again. Every 30 minutes a child is born with mitochondrial disease that will be diagnosed by the age of 10. Each year, more and more adults are diagnosed with mitochondrial disease. As the body of research grows, more and more diseases are showing connections to mitochondrial dysfunction. Chances are, you or someone you know is affected by mitochondrial disease.

Please take this week to learn more about mitochondrial disease and spread awareness to others. The more we learn about mitochondrial disease, the better for all of us.

Please check out last year's Mitochondrial Disease Awareness Week posts.

Do you work out?

Tweet Why, yes, yes I do.

I won't come out & say that I love it or have had some huge change of heart and suddenly hate my slovenly nature...but I do love the results.

Until very recently, I weighed more than I weighed the day I gave birth to Klaw...before I gave birth.

I have lost 12 pounds, slowly but surely. I have gained muscle mass. My clothes look better & I'm not embarrassed to wear a swimsuit.

I have lost inches in my hips & waist my chest, too, but I don't want to measure that. (I have 1.2 mil bras of different sizes, so I just switch out when necessary)

Klaw has enjoyed his hour or two at the Y, although it resulted in both of us getting sick in April. I was out for the count for about 2 weeks. Klaw had to be hospitalized for a weekend because we had GI issues in addition to upper respiratory issues.

I can honestly say not being able to stay at the hospital with your child is possibly one of the worst the things to go through. Chris was with him the whole time, thankfully. He had to take leave the following week because I was in no condition to care for myself or a toddler.

However, we are back on track. Klaw gets to play with other kids and he loves it. The staff at the local Y seems to be awesome and there is not as much turnover as I experienced when I worked in daycare.

I'm working on strength training, jogging (which I hate), & swimming (which I love).

For as long as I held out, I'm glad we finally joined the Y. I haven't felt this healthy in a long time.

I haven't felt like showing any skin in public in a long time. If you knew me 10+ years ago, you'd realize what a big deal that is...(right, Wesleyannes?)

Are there changes you need to make in your life? What's holding you back?

I'm sick, y'all.

Tweet And I hate it.

In fact, I hate almost everything right now. I am a MISERABLE sick person.

I have bookmarked a site to check for drug interactions because I am taking as many OTC medications & herbal concoctions as I (mostly safely) can in the hopes that something eradicates the gunk that is inside of me.

Yes...the Neti pot is getting a LOT of action. It ain't pretty, folks. I'm pretty sure it would get me banned for life from YouTube if I vlogged it.

Please send thoughts and prayers to Chris & Klaw as they continue to deal with me. Also, feel free to ask the powers that be for a Dora or Team UmiZoomi marathon.

Yes, the TV is Klaw's babysitter right now.

It also seems like a LOT of people are currently sick, but I, admittedly, have tunnel vision right now.

I hope you all get better soon. If you haven't been sick, I hope this plague skips you & your family.

this post brought to you by caffeine, sudafed, mucinex, Advil, & Benadryl

When bloggers disappear...

Tweet Where do they go?

I've been mainly going to the local Y & working my butt off. Literally.

I even have the blisters to prove it.

Chris & I created accounts on MyFitnessPal so that we could track our food & exercise to help us both get back in shape.

As much as I would love to lose weight, I realize and accept that my body is just different since having Klaw. My hips are wider & my booty actually sticks out now.

I'm okay with this.

I'm not okay with my lack of energy and poor muscle tone. That's what I'm hoping to "fix" more than anything else.

I have lost 6 pounds along the way, which means I finally weigh less than I did the day I gave birth...before I gave birth.

Also? The Submarine Birthday Ball is coming up and I bought a smoking hot dress that accentuates my curves. Spanx helps to contain the extra fabulosity. I don't mind the help!

(also, I'm checking out a Blogger app to see how it does for posting on the fly, so I have no idea what this post is actually going to look like published!)

Have you ever reached your breaking point where your health is concerned? Did you have to learn to accept changes to your body due to age, illness, or pregnancy?

Great Strides For Cystic Fibrosis 2012

Tweet My friend, Darby, has posted before on Really, What Were We Thinking about her son, Brady.  As we approach the 2012 Great Strides Walk for Cystic Fibrosis, I wanted Darby to have the opportunity to update all of us on how Brady is doing and, hopefully, help raise awareness and money again for this important cause.  To learn more about Brady and his family, check out Darby's blog, Keep on going...no matter what!

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Cystic Fibrosis...it's a progressive disease with no cure and an average life expectancy of 37 years old...CF is a genetic disease that causes mucus to build up in the lungs and digestive system resulting in chronic lung infections.  98% of all CF patients are pancreatic insufficient meaning they are unable to digest the fat in their food.  CF affects the respiratory system as well as the digestive system...it's a life threatening disease that affects more than 30,000 people in the United States.

Jeff, Brady, & Darby at the Great Strides Walk 2011

Our 3.5 year old son, Brady, has Cystic Fibrosis.  His days are filled with breathing treatments, chest physiotherapy, medicines, and g-tube feedings.  He spends each day fighting to stay healthy, and he spends a lot of time in the hospital each year, with his first hospitalization at only 5 months old. 

Brady will fight this disease for his entire life.  As a lot of people take for granted the ability to breathe, that's something he will always struggle with and be grateful for...he is blessed with each breath he has.

We are walking in the 2012 Great Strides Walk in May this year, and we ask everyone to either join our team in Virginia or to find a walk in your area and walk for Brady and the other 30,000 people fighting this disease.

Please donate to this cause so our child, and many other children, may have a chance at a longer life...come out and walk at a Great Strides walk near you...don't take for granted the breath you have.

Click here to make a donation to Great Strides

VLCADD Update #KnowAboutMito

Tweet We recently returned from an appointment with Dr. Vockley at the Children's Hospital of Pittsburgh. Dr. Vockley first met Klaw when he was about 8 months old and wanted a follow up by the time Klaw turned two, earlier if anything crazy happened that was VLCADD-related.

Thankfully, Klaw's VLCAD deficiency has been well managed through diet & supplements and our primary geneticist, Dr. Proud, did not see a reason to visit with Dr. Vockley any earlier.

First of all, it took three people, but we finally got a head measurement for Klaw.  This has been a bit of an issue for the past, oh, year. He is currently in the ~75%ile all the way around in height, weight, & head circumference. (35" tall & 29.5lbs)

Dr. Vockley does believe we dodged a bullet by the early diagnosis & intervention through the expanded newborn screening in Virginia. VLCAD deficiency can involve the heart, liver, & muscle tissue. Infants who are not diagnosed early are at a high risk of death during the first year of life.

Klaw's blood sugar is well-controlled. Even with the illnesses we've been able to manage at home, his caloric intake has remained high enough to prevent hypoglycemia, which is always a concern during any illness.  Our biggest day to day concern now is preventing rhabdomyolysis, which is when the body destroys muscle tissue. The dangers of rhabdomyolysis are not as acute as low blood sugar, but there are many long-term issues if he has multiple episodes over the course of time.  Again, we are trying to stay one step ahead of the game with his diet.

Naturally, Chris asked about how this will affect Klaw's participation in sports as he gets older (he's still hoping for that Cubs gig...). Dr. Vockley assured us he has many older adolescent patients with VLCADD who are active in a variety of sports. We'll just have to be vigilant about keeping Klaw hydrated & ingesting calories. Basically, while everyone else is drinking water or watered down gatorade...Klaw will be drinking the stuff straight because he'll have a higher need for sugar & electrolytes.

There is a new database/research registry being formed at the Children's Hospital of Pittsburgh, so Chris & I elected to have Klaw's records submitted. It's not directly tied to his identity; each case file is given a number. However, if the information can help people learn more about VLCADD, it is worth it.

Dr. Vockley was very pleased with Klaw's progress so far and future visits with him are at our discretion.

Staying Busy

Tweet Well, I just got back from Blissdom 2012 and I'm about to get in the car & head to Pittsburgh with Klaw for a genetics appointment.  Needless to say, between washing clothes & re-packing, I'm a touch busy which means no new blog posts of much worth.

I had a great time at Blissdom and had the opportunity to meet some wonderful people. And nap.  I have quite a few things to share with y'all but it's too much to type up in the 10 minutes I have at my laptop.

Klaw's appointment with Dr. Vockley and the genetics team at Pittsburgh Children's Hospital is just routine. I have no idea if we'll glean any new information or if he will recommend any changes to Dr. Proud (or primary geneticists). I am going to give permission for Klaw's genetic information to be included in a new database for this family of disorders. It's mostly anonymous, each person is assigned a number. I really think it's important to share as much information as possible with researchers. If his information helps find a better treatment or, even better, a cure, it will be worth it.

Well, that's where we are right now. Hopefully, next week I'll be able to sit down and share some exciting news with y'all.

CHOP Denies Transplant to "Mentally Retarded" #TeamAmelia

Tweet I hope that statement alone is enough to piss you off.

It sure as hell pissed me off.

The Children's Hospital of Philadelphia's transplant team has denied a kidney transplant to a child because she is "mentally retarded." (those are THEIR words, not mine). Take a few minutes to read what happened when Amelia's parents faced the transplant team and were told her developmental delays made her life less worthy.

"I begin to shake. My whole body trembles and he begins to tell me how she will never be able to get on the waiting list because she is mentally retarded."

 According to Amelia's parents, "Mental Retardation" was listed as one of several contraindications for a transplant along with illnesses such as HIV and Hepatitis C. Amelia does not have HIV or any of the other disqualifying diseases (this has been confirmed by her mother). She is cognitively delayed and this transplant team has decided that her life is less worthy than the lives of other children.

Put yourself in their position. Imagine being told your mentally delayed, developmentally delayed child, your child with a rare genetic disorder is ineligible for a transplant because of something they can't control.  This isn't a smoker who refuses to quit in need of a lung transplant. This isn't an alcoholic who refuses to get help in need of a liver transplant.  This isn't a person in a "persistently vegetative state" being kept alive by machines.

This is a child with a disorder whose kidneys are failing and will die without a transplant.

I keep thinking about kids like Klaw. VLCADD can affect the heart & the liver. What if he one day requires a liver or heart transplant? Would he be denied because his condition cannot be cured? Will he be safe solely because he's not "mentally retarded?" Would he be placed ahead of someone else because he is cognitively typical and a transplant team deems his life as more valuable than another child with cognitive delays?

Do you want to take action?  Sign the petition at Change.org.

CHOP: Special needs individuals deserve life-saving transplants

Share these posts & the petition every where you can. Tweet your support with the #TeamAmelia hashtag.  Blog about #TeamAmelia and the fight for the rights of special needs individuals.

CHOP's response to allegations that they denied Amelia's transplant based on "cognitive ability."

UPDATE :: 30 JAN 2012

How to Use a Neti Pot #VlogTalk

Tweet I promised friends on Twitter & Facebook that I would vlog myself using a neti pot if enough people expressed interest in it.  I never defined "enough" because, honestly, I was gonna do it anyways.

I apologize for the wind noise, but, really, it's the visual you want, right?

I generally use 1/4-1/2 teaspoon of non-iodized salt in warm water. Many people prefer a mix of non-iodized salt and baking soda because it adjust the ph of the solution and may be more comfortable.  Some people use water at room temperature, but I find that room temp water is not comfortable at all.

My own little testimony is that I have "dainty" nasal passages. I did not make that up, a former ENT specialist told me that.  It's the only "dainty" thing about me, so don't judge.  She also told me that I would continue to be plagued by sinus infections if I didn't have surgery or find something else that helped...like a neti pot.

Thankfully, the neti pot has worked. I still get sinus infections but it only gets bad enough to warrant antibiotics maybe once a year. I'm able to push through with a neti pot & pseudoephedrine combo and feel better in a matter of days instead of weeks.

Run a Marathon, Give Birth, Who Cares?

Tweet Evidently, a lot of people do.


 photo credit

I wasn't one of them until, Liz, a twitter friend & blogger at a belle, a bean, & a chicago dog, posted an innocent question on the twitter:

Everyone keeps praising the pregnant woman who ran the #ChicagoMarathon. Anyone think that wasn't such a great thing for her to do?

Some of the responses she got made me realize how many people are coming down hard on this mom for her "reckless" and "irresponsible" decision to "run" a marathon at 39 weeks of pregnancy. Not just, "I wouldn't do it" or "I don't think she should have done it" but people seriously calling her "reckless," "irresponsible," "selfish," and "risky."

In case you didn't catch the news coverage, Amber Miller got clearance from her doctor & support from her husband to run half the marathon & walk half the marathon at 39 weeks of pregnancy.  Obviously, she didn't just up & decide to run a marathon.  She is in excellent physical condition and had no contraindications to continuing her physical activity during pregnancy.

I'd like to point out that my Google news search for "chicago marathon birth" on 10 OCT 2011 turned up more articles about the winner of the marathon & the firefighter who died than it did for the woman who gave birth.  She was mentioned in a few articles, but only rarely mentioned in the titles.

Why is this a big deal?  I wasn't in condition to run a marathon BEFORE I got pregnant, so it would be asinine for me to do so while pregnant.  Personally, I think running when you aren't being chased by a knife-wielding maniac is pretty crazy, but I'm a lazy sloth. Other women have continued to train for marathons while pregnant, so this isn't anything new.  The key point is that your activity level during pregnancy is individual to you.  I was walking 3 miles a day towards the end of my pregnancy with no issues.  Some women are on modified bed rest.  I am not going to judge another woman's pregnancy needs based on my own.

Most people understand difference between discussing/debating a controversial issue (which I greatly enjoy).  However, when I see words like "irresponsible" and "selfish" coming up repeatedly, I tend to think it's no longer a discussion but a judgment.  It would be very irresponsible for ME to run a marathon ever at 39 weeks, but it's not irresponsible for everyone, as evidenced by Amber Miller.

Guess what?  I have a few confessions:

1- I ate sushi while pregnant.
2- I cleaned the litter box while pregnant.
3- I had half a Guinness while pregnant.
4- I ate soft cheeses while pregnant.
5- I took zoloft while pregnant.
6- I did not get the seasonal flu or swine flu vaccine while pregnant.
7- I slept on my back more than once while pregnant.
8- I went to a party, wore heels, & met Donald Rumsfeld the night before I went into labor.
9- I labored at home for several hours after my water broke.
10- I hired a midwife and not an OB.

For each of these things (okay, maybe not meeting Rumsfeld, I just like to throw that in whenever I get the chance), I was told by at least one person that I was being selfish and putting my baby at risk.  I never asked for anyone's opinion, but my growing belly made some people think they needed to tell me exactly how irresponsible and negligent I was being.

Guess what? I have the right to make decisions concerning my own body.  Just because a woman is pregnant does not suddenly mean that she loses the right to make her own decisions. Random members of society don't get to make these decisions for me based on what they think I should do.  In fact, unless I'm declared mentally incompetent, no one gets to make decisions for me.

This kind of thinking worries me because it can lead to pregnant women being forced into birth interventions, surgery, or tests without their consent because someone else deems the mother's right of refusal invalid due to her being pregnant.  It can throw us back decades with women's rights.

the New Jersey appellate court found that V.M. and B.G. had abused and neglected their child, based on the fact that the mother, V.M., refused to consent to a cesarean section and behaved erratically while in labor. The mother gave birth vaginally without incident, and the baby was "in good medical condition." Then she was never returned to her parents, and the judge in the case approved a plan to terminate their parental rights and give custody of the child to foster parents.

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Melissa Ann Rowland stands charged of murder by the State of Utah forfailing to permit a timely cesarean section that could have saved one of her two babies from in utero death.  Her case demonstrates the classic maternal-fetal conflict: Mrs. Rowland refused to consent to a procedure that had substantial risks for her and no benefits, but which could have saved her stillborn child’s life.  The charges represent the farthest extent of state action in favor of fetal rights over maternal autonomy and proceed against a jumbled background of contradictory precedents.

These were just two examples and the facts surrounding the cases are murky but the charges were brought against these women due to their refusal to do what someone else decided was best for them.

This leaves a really bad taste in my mouth.  It tastes like paternalism & misogyny.

I'm going to step off my soap box now but I'd love to hear your thoughts on this. Do you think women lose their autonomy upon getting pregnant? Should doctors/caregivers/loved ones have the authority to overrule a pregnant woman's decisions regarding her body?

Mitochondrial Disease Awareness Week

Tweet

Mitochondrial Disease Awareness Week is just around the corner & I am in the process of lining up several guest bloggers to share their experiences living with MITO. I hope that it will be an informative and thought provoking week. I still have spots available for the week and, if possible, I'd like to feature MITO bloggers during the following weeks. If you are interested in submitting a post, please contact me at ReallyWhatWereWeThinking (at) gmail (dot) com.

I'd also love to hear from all of you what you'd like to know about Mitochondrial Disease. I've discussed & described Klaw's life with VLCADD in several posts, but the fatty-acid oxidation disorders are just one branch in the family of mitochondrial disorders. Please leave your questions in the comments or email them to me at ReallyWhatWereWeThinking (at) gmail (dot) com. There is no question too small, long, or personal.

So, folks, what would you like us to cover in the coming weeks about mitochondrial disorders? Has your life been touched by mitochondrial disease in some way?

VLCADD, the heat, & Klaw

Tweet I have been very nervous about exposing Klaw to too much heat, thanks to his VLCAD deficiency (very long-chain acyl-COA dehydrogenase defiency).  Any situation that causes his metabolism to crank up can potentially be dangerous.   Since the VLCAD deficiency prevents his body from metabolizing very long-chain fats into energy, it's extra important to make sure he remains hydrated and has enough carbohydrates and proteins in his system to burn for energy.

To facilitate keeping him cool in the hot summer south, we have annual memberships at both the Children's Museum of Virginia (Portsmouth) and the Virginia Living Museum (Newport News).  Both are great spots for kids and families and provide ample indoor space for Klaw to run.  Both places have lots of buttons to push, too.

I grew up running around on the beach all summer long and it's something I want Klaw to experience, too.  However, I've been fearful to attempt it, especially during the week when it would most likely just be the two of us.  Yes, his health has been good overall.  Even his hospitalizations were not caused by his VLCAD deficiency, even though VLCADD was the reason hospitalization was absolutely required.

It's still scary.

It's scary to have to always worry that your child might get too hot, go into metabolic crisis, and die.  I know that sounds melodramatic but it's what is always in the back of my mind.

However, I can't and I won't live in fear - at least, I won't let him live in fear.  He's a toddler and he needs to have fun.  We just need to prepare for any possibility and continue to stay one step ahead of his VLCADD.

Mom, KriKri (my niece), and I decided to go to Jekyll Island for a beach trip.  I packed a cooler full of water, Gatorade Prime, cut fruit, jarred food, & applesauce.  Yes, I gave Klaw Gatorade.  This particular one is full of glucose, sucrose, & b vitamins.  Although most moms of toddlers would like to avoid that much sugar with their toddlers, it's exactly the high carbohydrate solution that could help prevent a VLCADD related metabolic crisis.

Mom rented an umbrella, which is smart for anyone but it was a really good idea for Klaw so that he could have a shady spot to stay cool and out of the sun.  The water temperature was perfect and, thanks to some haze and a decent breeze, it wasn't too hot.

After he got over the initial 20 minute screamfest driving over there, we had a blast.  Klaw spent time digging in the sand under the umbrella and playing in the water.  I got a good reminder as to why I do not want a pool at my house when he dove face first into the ocean without hesitation. KriKri was a great big cousin and played with him the whole time.

I made sure he had his Gatorade available and that he snacked on some applesauce while we were there.  It was actually really difficult and I felt like he wasn't getting quite enough fluids or food for me to be comfortable.  He ate almost a pint of fresh fruit when we stopped for sandwiches on the way back & finished his Gatorade, so I'm afraid my instincts were right.  I'm not really sure what I can do because he just didn't want to be distracted from fun in order to stop & eat.  I know (hope) this will be easier when he's older and can understand why he has to stop playing and eat or drink.

However, he was no worse for the wear by the time we got home and he was worn out!

Have you ever had to make a toddler eat or drink?  Do you have any tips you can share?  Do any of my more experienced Mito Moms (mitochondrial disorders) have other tips for surviving the heat with our kids' special needs?

VLCAD Deficiency Update

Tweet Klaw had an appointment with his geneticist, Dr. Proud, and the 12 month well-check with his pediatrician this week. Our big boy is 25lbs and just over 31" in height.  His weight has been holding steady for several months now, so he's long & lean.  He is also ridiculously strong...but that's just a personal observation of mine.

Klaw recently had pneumonia...and didn't have to be hospitalized. This is a pretty huge milestone, even though it's not in any development charts. None of his previous hospitalizations have been for issues caused by VLCADD, but his VLCADD is what made hospitalization necessary. The average child would have been treated at home for the same illnesses.

Dr. Proud told me, and I quote, "You can calm down now, Mom."  Now that Klaw is a year old, he has enough glycogen reserves to sleep through the night & go longer between feedings, in general.  Now, Klaw has been sleeping through the night since he was born.  It sounds awesome, but it's not when your kid has an FaOD.  Just because he was sleeping through the night didn't mean we were.  These past few days have been the first days since his birth that he didn't require at least one feeding during the night.  A feeding he slept soundly through almost every single time.  Lucky kid.

Anyways, back to VLCADD:

Taking Great Strides for Brady

Tweet  This year, we are participating in a Great Strides Walk for Cystic Fibrosis as part of "Brady's Bunch."  Brady is the son of our friends, Jeff & Darby, and he has Cystic Fibrosis.  We met Jeff & Darby through the Navy when our husbands served aboard the USS New Mexico together.

Darby maintains their blog Keep On Going...No Matter What!  They hope to get the word out about Cystic Fibrosis and some of Brady's other medical conditions just like I hope to spread the word about VLCAD.

I asked Darby to write a guest post for our blog because I've learned so much about Cystic Fibrosis through her & Brady.  I've also learned a lot about about how to keep my head above water while working through Klaw's genetic disorder.  Enjoy!

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Brady is the happiest little boy you'll ever meet. He doesn't have a
care in the world...he loves his life, just the way it is, and longs
for nothing! Don't you wish to long for nothing one day? I sure do:)

Even though he's made to do 2 breathing treatments, 2 chest therapy
treatments, take 10 medicines, and do multiple g-tube feeds each day,
he is just the happiest thing ever! He looks forward to his breathing
& chest treatments as they are "fun" for him. He even fake coughs
when he sees the equipment! Why is all this necessary for Brady?

He has Cystic Fibrosis... what is Cystic Fibrosis?

We feel honored that the Lord chose us to be Brady's parents, to take
care of him, to raise him, and to love him. We wouldn't want him to
be any other way than he is...regardless of how long he lives.
Currently the life expectancy is about 37 years old, and Brady's 2.5
years old right now. We have plenty of time to show the world how
much we love our son and plenty of time to get the word out about
Cystic Fibrosis, which is a progressive disease with no current cure.
Brady will spend his entire life fighting to breathe...what will you
spend your life doing? Personally, I'm an avid runner, as running
clears my own lungs of whatever is in them, and running will clear
Brady's lungs of all the bacteria/infections in them on a regular
basis. Running is the best form of "natural" treatment for Cystic
Fibrosis patients, and we can't wait until Brady can learn to
run...walking is first on our list though...
he's almost there, but not quite.

Here's a quick example of why running is so important for CF
patients...take a minute and check this out...

Every spring is the Great Strides Walk for Cystic Fibrosis...this will
be our second year walking as last year Brady was in the hospital on
the day of the walk so we weren't able to go.

Check out our Great Strides Page for more information on the walk.

We want CF to stand for Cure Found!

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If you would like to make a donation to help find a cure for Cystic Fibrosis,

I've added Darby's Great Strides widget to my sidebar.