Wow,it's been too long

Well several things have gone on since I last posted. First things first,I adopted a purebred Bengal kitten from a rescue group. Or what I thought was a rescue group. We were assured that he was 11 weeks old and was in good health and was up to date on his shots...Well that's not true.He had a list of issues when we took him to the vet two days after he came home with us and he was maybe six weeks old. He is doing well now and we are anxiously waiting on the next Feline Leukemia test. He's tested a weak positive the first 2 times.So we are going to do it once more and if it's still a weak positive,we'll treat him as a Leukemic kitten.

Here he was on the first day.

Then in September I went to the 2011 Heart Walk and I got to meet one of the most wonderful ladies in the world. I also got to see an old friend of mine who I haven't seen in a few years.

Katie Davis and I. Her beautiful daughter Olivia passed away this past Easter.She had HLHS.

Jami and myself. Jami is a wonderful friend who I really don't see often enough.


The other bit of news is that I've decided to go into teaching. More specifically I want to teach preschool. One of the best things about that is that I am already very close to being able to apply for my teaching permit. The other awesome thing is that this means no more math! Math is my Achillies heel. I I was a mathmetician I could have gone to med school because all of my other grades hgave always been good.

Hopefully it won't be another 3 months before I update the blog next time.

First Visit with the Adult Cardiologist

Okay so I know everyone is dying to know how the first appointment with the new cardiologist went.So here goes...

I got to the office,checked in and waited for about 10 minutes before I was taken back to an exam room. The nurse took my blood pressure which was really high in both arms (I don't think she knew WHAT she was doing) then I had to play the waiting game.Then Dr. Dinh came in and he listened to me,checked my peripheral pulses and checked my carotid artery (which is something my peds cardiologist never did) He checked my liver for enlargement.He checked my fingertips (obviously to make sure they weren't blue)

After the physical exam we talked about the Coumadin vs Prodaxa debate. He said that for now he wanted to keep me on the Coumadin because the Prodaxa isn't for those patients who have had valve issues.He is going to send a request over to Dr. Juris' office for my reccords.I mentioned that when I had a liver panel done reccently my bilirubin levels had been elevated and I asked if it had anything to do with the CHD's. He said he wasn't sure but he was going to request my lab results as well.

He said that he would have a much better idea of the care plan after he got my reccords and he was able to review them.He wants to see me in 3 months (hopefully he can wade his way through all my reccords by then) Turns out he actually worked under Dr. Laks who was my surgeon at UCLA when I was 4 1/2.So he's going to talk to Dr. Laks.

Then I had a quick visit with their Coumadin clinic and signed some paperwork for them. Then I was free to go.

I really,really liked this guy. He was through and really nice and he answered questions in a way that I could understand them.I would reccommend him to ANY Adult CHDer.

Baby/Child Loss is not a Taboo subject,so stop treating it as such

A dear friend of mine had gone to Hallmark to find a card for a family member who had lost their baby unexpectedly and she was horrified,disgusted and furious. There was practically no cards that adressed the loss of a baby. There was several that addressed the loss of a pet. When she she told me about this, I got angry. I know pets are important parts of people's lives,but to still see baby/child loss as a taboo subject is insane.

My friend inquired as to the whereabouts of the baby/child loss cards and the people who worked there,looked at her like she had three heads.That is unacceptable to her and to me. Child/baby loss is not an easy subject but it's a subject that must be talked about because it's a reality for so many families. Families already feel like an outcast when they lose a child/baby and to have the subject be taboo,just makes it worse for these families.

 The loss of a child is something that so many parents have to experience and for the friends of those people,they feel lost. They try to get a card to send the family,but there are very few options.

There needs to be the same ammount of beautiful cards for child/baby loss as there are for new babies.

May is Barth Syndrome Awareness Month!

I've gotten to know several boys with this condition and I am so lucky to call their moms my friends.I wanted to do a special blog post in their honor. Since May is Barth Syndrome Awareness Month,I decided to do it this month. I decided to profile one of the boys to give those who aren't familiar with this condition,a face and a name.

Christopher is a sweet, outgoing, funny and adorable little boy who suffers from Barth syndrome.

You would not know he had BTHS just by looking at him, as he and the other boys compensate So well to have such a multitude of issues.

You would not know the endless appointments, specialists and therapies he must go through on a regular basis. You would not know that he must have injections several times a week to make his white blood cells produce sufficiently and be able to fight infections. He must take antibiotics everyday forever.

You would not know he takes 8 oral medicines a day, some of them to help his heart function properly.

He does have more difficulty in eating and because of this, Pediasure is there to help him get enough nutrition. A true Barth’s boy, he does love his butter, salt and eggs.

He has a lot of energy when you first observe him, but if you know him well, you know he does get tired more easily than others his age. Because of this, he sleeps longer than most at night.

We let Christopher guide us and he though he was slower at walking, talking and doing other normal physical things, he is very smart. Here is the story of Christopher:

Christopher Pena was born in April 2008 with non-compacted cardiomyopathy. Non-compacted cardiomyopathy is a very rare condition in which the heart muscle remains sponge-like after birth, which causes the heart to be very weak. Christopher's cardiomyopathy affects his right and left ventricles where it is very hard for his heart to pump and function correctly. They said the only fix was a heart transplant. At that time, we opted against.

Christopher was not given very long to live. Six months at most When he was 45 days old, he was admitted into hospice care where he remained for 15 months. Christopher was released from hospice when an echo cardiogram showed that his heart function had tripled! And at 18 months, his heart function was almost normal.

In February 2010, it was discovered that Barth Syndrome is the cause of Christopher's cardiomyopathy.
Barth syndrome is a rare, sex-linked genetic disorder of lipid metabolism that affects males. Typically, boys with Barth syndrome present with hypotonia (low muscle tone) and dilated cardiomyopathy (labored breathing, poor appetite, and/or slow weight gain) at or within the first few months after birth. Other important features of Barth syndrome include bacterial infections because of neutropenia (a reduction in the number of white blood cells called neutrophils), muscle weakness, fatigue, and short stature. Although most children with Barth syndrome manifest all of these characteristics, some have only one or two of these abnormalities and, as a result, often are given incorrect diagnoses. It is very rare, so much that if people would hold hands from one end of the world, all of the way around, only one of those people would be a boy with Barth syndrome.
There is no specific treatment for Barth syndrome, but each of the individual problems can be successfully controlled

Here is the website for the Barth Syndrome Foundation and their Month of May campaign to raise awareness for this rare genetic disease.

http://www.barthsyndrome.org/english/View.asp?x=1678&mp=1323

For those of you who are unfamiliar with this diease,here are some answers to qyuestions you may have.

 *What is Barth syndrome?

-Barth syndrome is a rare but serious, x-linked genetic disorder of lipid metabolism primarily affecting males around the world

*What are the signs and symptoms of Barth syndrome?

-The cardinal characteristics of this multi-system disorder consist of the following in varying degrees:

~Cardiomyopathy (dilated or hypertrophic) - A weak heart muscle usually associated with enlargement of the heart.

 ~Neutropenia (chronic, cyclic, or intermittent) - A reduction in “neutrophils”, a type of white blood cell that is most important for fighting bacterial infections.

~Muscle hypoplasia and weakness/Exercise intolerance - All muscles, including the heart, have a cellular deficiency which limits their ability to produce energy. Muscle weakness and increased exertional fatigue are characteristic findings in Barth syndrome.

~Growth Delay (often mistaken to be failure to thrive)-During childhood most affected individuals are below-average in height and weight.

~3 Methylglutaconic aciduria - An increase in an organic acid that can be measured in urine that result in abnormal mitochondria function (the “powerhouses” or primary energy producers in cells).

~Cardiolipin deficiency - A failure of Barth syndrome mitochondria to make adequate amounts of tetralinoleoyl-cardiolipin, an essential lipid (fat-like molecule) for normal mitochondrial structure and energy.

 

*What gene is affected?*

~Barth syndrome is caused by mutations in the tafazzin gene (TAZ, also called G4.5) on the X chromosome. Because males have only one X chromosome, they will have signs of Barth syndrome if that

X chromosome carries a mutated tafazzin gene.

*Who does Barth syndrome affect?*

~Barth syndrome primarily affects males.

~Females are primarily only carriers of the mutated gene that causes Barth syndrome. Females who carry an X chromosome with a tafazzin mutation are unaffected because they have a second X-chromosomes with a normal tafazzin gene that is dominant to the recessive tafazzin gene. Although it is theoretically possible for a female who carries a mutation in the Barth gene to have clinical signs of the disorder, there has been no proven affected female with a normal 46, XX female karyotype to date.

How does someone get Barth syndrome?

~Barth syndrome is an x-linked recessive genetic condition, meaning that it can be transferred from mother to son. A mother who is a carrier of Barth syndrome usually shows no signs or symptoms of the disorder herself. There is a 50% chance that a boy born to a female carrier will have Barth syndrome, while girls born to a carrier have a 50% chance of being carriers themselves. All daughters of a male with Barth syndrome will be carriers, though none of his sons will be affected. There are several known non-carrier mothers, and for this reason we believe mothers should be tested.

 

*How is Barth syndrome diagnosed?*

The diagnosis of Barth syndrome should be considered for any child or adult found to have any one of its four cardinal clinical characteristics, and evaluation for the other diagnostic criteria should be undertaken by obtaining the following studies:

~Quantitative urine organic acid analysis, including quantification of 3-methylglutaconic acid

~Cardiolipin analysis of muscle, platelets or cultured cells

~Complete blood count and differential

~Echocardiogram

~DNA sequence analysis (genetic testing) of the tafazzin gene (TAZ, also called G4.5) which falls on the long q arm of the X chromosome; Xq28.

~Please see the “Diagnosis of Barth syndrome” webpage for further information on diagnosing Barth syndrome as presented by Dr. Richard Kelly MD, Ph.D.

~For more details about these tests and a listing of approved labs, please refer to http://www.genetests.com.

 *Why is an early diagnosis critical?*

o Early and accurate diagnosis is key to survival for affected individuals. Historically, boys died of heart failure or infection by three years of age, but today, with improved diagnosis, treatment, and management, the survival rate and future of these boys is much brighter.

~Congestive heart failure

~Risk of serious arrhythmia, including sudden death

~Serious bacterial infections

~Gross motor and/or fine motor delay

~Growth delay

~Exercise intolerance, lack of stamina

~Frequent diarrhea

~Recurrent aphthous ulcers

~Hypoglycemia, including fasting hypoglycemia in the newborn period

~Osteoporosis

~Chronic headache and body aches, especially during puberty

~Extreme fatigue

~Feeding problems

~Mild learning disabilities

 *Incidence and Prevalence*

~To date, there are no good studies of the population or birth incidence of Barth syndrome; however probably fewer than 10 new Barth infants are identified each year in the United States, which suggests an incidence of only 1 in every 300,000 – 400,000 births. Currently there are fewer than 500 individuals within our registry from around the world.

~Barth syndrome occurs in many different ethnic groups and does not appear to be more common or have originated in any one group.

~Dr. Peter Barth of The Netherlands published the first comprehensive description of Barth syndrome in 1981 and again in 1983.

~Dr. Richard Kelley at the Kennedy Krieger Institute at John Hopkins published a further study on Barth syndrome in 1991.

~In 1996, the specific genetic location of the Barth gene on the X-chromosome was identified. (Bione, et al 1996

~There is no specific cure for Barth syndrome at this time, but the BSF is funding research for interested scientists and physicians in hopes to further their understanding of the metabolic and biochemical abnormalities seen in this disease.

~There are no specific treatments for Barth syndrome. Not all patients exhibit all of the symptoms at any one time, therefore heart symptoms, infections, and nutrition problems are treated as they arise. Careful attention and monitoring for symptoms is advised.

All of this information is used only for awareness purposes.It is not intended to be used as medical advice.

*How do you treat Barth syndrome?*

*Is there a cure for Barth syndrome?*

*When was the Barth gene identified?*

*When was Barth syndrome first described?*

*Ethnic Incidence*

*Are there other possible clinical*

*What are the major clinical problems?*

*Can females get Barth syndrome? *

Advice from Heart Parents to new Heart Parents

Start therapy now. ;)

Don't sweat the small stuff :)

My advice comes and goes but I would suggest asking many questions, searching for every piece of information on your childs heart

doctors don't have the last say in the outcome!

to filter out the bad stories.. focus on the positives, take notes (don't be afraid to ask tons of questions even if you think you already asked them) and get all the facts that you can grab. Get support. And lastly, trust your gut..

To make a note of a memory everyday. Live and love with all your heart and don't be afraid to ask for help.

To never feel alone, you are not the only parents starting out in the CHD world.

All of the above!! ALL OF IT!!!

I agree a lot with dawn. Every case is different just go day by day and don’t try to compare your child with another, it will drive you nuts! believe me.

That you have been given a gift because you are strong enough to handle it.......that gift is the realization of how precious every single minute of every single day really, truly is!!

I know its overwhelming, but you are stronger than you realize.

You are not alone and enjoy every moment and take pictures even with tubes and wires etc.

To never give up hope and reach out to those around you for help. You are alot stronger then you ever realized you could be and that strength will come through when you least expect it to and you think you can't go on. Find other in your community with CHD's to talk to as no one knows better what your going through then another CHD family.

BREATHE!!! Sleep when you can and remember that there is NEVER a stupid question asked. It is ok to cry, no one will think any less of you. You have a family with this community.

First, Do NOT look up anything online. You have no idea how old that info is!!!
　
Never stop Believing, and take care of yourself,
　
Cry when you need to cry, scream when you need to scream and laugh when you need to laugh...Its ok to be angry and find a doctor you trust, hold on tight and lean on your support system

I agree with all but also write everything down also its over before you know it and your child is just another child. Don't worry about the future.

Never give up hope, try and stay positive, live each moment like it is your last, and always remember you know what is best for your child. The doctors, therapists and you all work together as a TEAM! You could not do it with out them and that can not do it without you!

What is the hardest part about being a heart parent? (Part 2)

Even 9.5yrs into this and watching my girl live like the rest of her friends it is sitting here waiting for the other shoe to drop, without not really waiting. Make sense?

There are 2 things that are really hard for me. First one is not knowing his future, knowing that the oldest HLHSer is really not all that old, knowing that we could lose him at anytime. Second is knowing there is nothing I can do to take his pain away or to make him better, I can only be there to comfort him.

The constant fear that Jace might die. I have horrific nightmares on a regular basis. It's constant anxiety for me bc of it. He is my everything and my purpose for living. The thought of losing him is gut wrenching

Not knowing what to expect. And not being able to take her place whenever she is in pain or has to go thru something rough
Besides the obvious of not knowing what will happen and how long he will be with me. The hardest part is watching him go through the pain, stress, and anxiety and not being able to make it stop for him.

The unknown!

Watching your child grow up and not knowing how long you have them.

Worrying if anything will happen when he's at school and I not make it in time, his impending surgeries.. going thru the wait and signing papers with complications that may or may not happen..I hate the signing part..I do want to thank God that he's almost 11 on the 25th!!

knowing when it's time to say goodbye and trying to live after saying goodbye

Fear of the future, the unknown.

all of the above...

For me it seems to be harder when he is doing well because you start taking it for granted that he is doing well. This really hit me when he was fine at 9 am on April 20th and by 3 pm he had had 3 strokes. When the look sick or act sick its ...easy to make the most you have with them cause you know you could lose them anytime. But when they are doing good its easy to forget they are sick and you think well I at least have a while left losing him isn't an immediate threat when it is always an immediate threat

After dealing with this for almost 22 years...it never gets easier...every hospitalization, every illness, everything just weighs on you. Just enjoy every moment they're here.

Wondering what else can happen...the "WHAT IF" or "WHAT NEXT"

Saying goodbye.

Knowing that you can't do anything to help.

Not being able to take it for them.....watching them in pain.....seeing a cry but not hearing anything.....watching a monitor fearing the next minute....not having any control over the situation......It is a long hard road and when it is bad it is awful but I will walk with him every step of the way because the good helps to ease the fear of those bad days!!!!

The unknown but knowing the possiblilities...

Not knowing what the futures holds...and knowing that even though my Olivia looks perfectly fine on the outside...but she's working ten times harder than all of us healthly folks on the inside.

And watching my husband be so afraid to get close or even feel the baby move cause he didn't want it to be too hard if our little guy didn't make it.

There are a lot for me...Not knowing what the future holds for him, worrying every night at bedtime or everyday at nap time...Watching him go through so much with the iv's, labs, x-rays, etc,etc,etc...It's extremely hard right now for me knowing he just had surgery 2 months ago & he isn't progressing as well as they hoped right now...

Thinking I might lose another baby, and being helpless to help him even though I would have given my life for him to never feel an ounce of pain.

The uncertainess... The fear of him not having a tomorrow. Having to smile and be happy sometimes when my heart is breaking. There are many happy moments too, however with an instant everything changes!

In a nutshell, being a heart parent. Never expected to be one but if wishes were horses, it'd be me with the uncertain future & trials, not my wholly innocent baby. Just one of those burdens you can't truly care for those you love, proof you can't be a supermom & protect them from everything. It is terribly humbling. And I don't do humble well. ;)

What is the hardest thing about being a heart parent? (Part 1)

I don't have the answers to that because I have never seen it from that side. So I had to ask the heart parents I know and here are their answers.

Going to bed every night wondering if your child is going to wake up the next morning. Other than that... hmmmm it's all gravy! ... Remember laughter is the best medicine.

The hardest part for me right now that Bianca is doing good is seeing other heart babies and heart parents and other heart adults, hurt...it breaks my heart when my CHD family is hurting.

Never knowing what's around the corner
Worrying about her future and how her health is gonna be when she gets older.
I agree with both of them.
I often check Legend to make sure he's breathing. Also everytime i see a baby doing bad or pass and see as of right now legends doing good it makes me feel a little guilty. I feel like if i put a post about him im bragging. But the main thing is not knowing. He still has 2 more surgerys and the ones coming up in a few months and you never know afterwards just hope all goes smoothly

The uncertainty, for sure.....not knowing what the future holds.

To be honest with you.... The hardest thing for me is treating Lilli like a normal little girl. Letting her climb and fall, letting her run and play.

The constant fear and anxiety - that pretty much means everything! Sorry, probably not the answer you were looking for ;)

I worry sooo much about Binaca's future and what God has planned for her and her lil heart! I agree with everyone! And if I haven't said it, we are so glad and blessed to have a great supportive CHD family!

For me

This past week and weekend sucked big time. First I fell on my left side and landed hard on my ribs. That fractured at least one of them.So I spent my Spring Break in pain and loopy on pain meds.So not the way I wanted to spend my week off. Then there was that whole issue with Jenna Evans which I will not go into. My heart hurts for Holly and I hope she knows she's got the support of a ton of people.

Then last night came. I was really not looking forward to going back to school so I was relaxing and watching the Lakers/Hornets game when I got the news.Sweet beautiful Olivia Davis had died. I had been Facebook friends with her parents before she wa even born and it broke my heart to hear that she had lost her fight. on the first day of my vacation I had gone to Target. I was going to get her something small but I couldn't find anything I liked.

So when I got the news last night I was hit with a sickening realization. I wasn't meant to find something for her because she wasn't going to make it. My stomach lurched,the tears streamed down my face.I developed a lump in my throat that made swallowing so hard.I reached out to some very dear friends who understood what I was feeling completely. Ladies you know who you are.

 I felt guilty. Stanford was not far away and I should have made the trip down there to meet them and to see her. Sleep didn't come for a long time last night. I kept seeing her in my mind ever time I closed my eyes and the waves of sadness kept hitting me.

I can't live like this. I can't allow every loss to absolutely devastate me. I need to take some me time away from Facebook and away from these amazing families who I adore so much. I need to do this for me so I take care of the one life I have. The one life I was allowed to live. I won't go into the survivors guilt issue because I've already talked about that. I'll just say that it hurts and it makes me feel guilty when another child loses his or her life because of CHD.I know I shouldn't feel guilty and I'm working on that.

If you want my number just ask and I'll give it to you.That's the best way to contact me while I take this much needed hiatus.

Jealousy and Joy

First of all let me preface this with the statement that I am so excited for her. She deserves this as do so many other women in our CHD community,With that said,I'm jealous and really hurt. I'm supposedly a good friend of hers and yet I had no idea that she was expecting until today.I'm hurt that she didn't tell me herself and that I had to find out from other friends.

It hurts knowing that I will never be able to carry a child because my cardiologist has already told me that it's much too dangerous.I want to get married and have a family someday and that day doesn't seem to be coming any closer. I have already made the choice to either use a surrogate or find some way to adopt.

It's hard knowing so many women in our community are expecting.Especially someone who's a CHD Survivor like me. That hits so close to home.I don't know if I'll ever get that chance to be a mom and yet I cannot help but be happy for all of these women in our community.Especially for my fellow CHD Survivor.

I don't want to be the jealous angry friend because I am truly excited for her. I want to be beside her every step of the way in spirit because I can't be there in person.I want to be supportive and loving.I want to be a good friend despite the baby envy I am having.I really hope that I can get past this feeling of envy and intense jealousy.

To her I'll simply say congratulations. I am so happy for you.

Why

I know I said that the next blog post would be about PTSD in CHDers and CHD Parents but I have to write this. Why am I still alive? What is my purpose in life? Not easy questions but ones that come up in my head sometimes.

I may not have done bad things to my body but I have coded multiple times and yet each time it happened,I came back.I don't understand why I was allowed to live and so many children have lost their battles. These musings happen more often as I have become a part of the CHD community on Facebook.I have bonded with parents and Adult CHDers.I watch some CHD parents lose their children and it makes me wonder why I was spared and why my family was spared the pain of losing me.

I know I fought hard to live but I don't think I fought any harder than these kids who are losing their lives. I know my family fought for me,but I also know that these Angel Parents fought hard for their children as well.I watch these babies and children struggle to survive and it makes me wonder why was I able to beat the odds and some kids arent?

I went through the trials of not having an appetite and getting poked and proded and having countless caths and 5 surgeries and twice yearly cardiology appointments and more.

When I was 12 I was so sick,they weren't even sure that I'd make it off the operating table after my emergency aortic valve replacement.And yet I did. I was in a medically induced coma for a month and developed a nasty pressure sore on my tailbone because I couldn't be moved without my sats dropping dramatically.When I was released from the hospital six weeks after I was admitted,I weighed 42 pounds. I was skin and bones and my hair was thin and falling out.

Yet I made it...

When I was 19 I had my aortic valve replaced again. This time I was only hospitalized for 8 days. That was a record for me.I sailed through that surgery and recovery.


Sometimes life makes absolutely no sense at all.

Nightmares

I never really had nightmares as a child.At least not ones that were connected in any way to my CHD's.When I was recovering from surgeries at ages 12 and 19 I had what were called "Fentanyl Nightmares" They were vivd and horrific.My grandmother said that when I was on Fentanyl I even scared her.

When I was 12,one of the nightmares I had involved two of my classmates who were following this faceless guy down a dark alley and I remember screaming at them not to go.I said "Don't go with him.Please!" But they went anyway and I saw their limp and bloodied bodies a short time later.

When I was 19,these dreams were even scarier. One nightmare in particular scared the living hell out of me.I was lying face down in a doorway with the taste of blood in my mouth and this girl screaming at me "You sick bitch,you raped my sister."I was so scared and I remember waking up in a cold sweat and I was shaking so badly I couldn't stop.

So heart parents if your kids are ever on Fentanyl after surgery,you should expect them to have this type of reaction.Scary vivd nightmares that scare even you.You should also know that these won't last and once Fentanyl is completely out of your child's system he or she will go back to normal.

At a Standstill

This isn't CHD related at all but it's something I've been thinking about a lot lately.After conversing with and old friend on Facebook last night I was startled to realize how little my life has changed in the last 5 years.Sure I may have moved,but that's it. I am still at the same Community College I haven't had a date in 8 years I still haven't moved out on my own.

I'm watching all of my old friends from grade school and high school get married and have kids and I'm jealous. I know it sounds crazy,but I need something to change. I usually hate change but I really need it.I need a big change.When is it going to be my turn to have a major earth-shattering change like falling in love and getting married.

I feel like my life is at a standstill and unless I make some decisions about my life,it's going to continue to be at a standstill.I have to make a career choice and I have to get back to going on dates.

Please think of me and pray that I get answers because I could really use some help right about now.

Something needs to change and soon.

Raw Emotions

I blog to get my feelings out and right now I am over everything that happened over the past few days. I can't focus on the anger of others. I can only focus on how I react to things. I am stubborn and tenacious,but I am also a person.

I wish more than anything that I owned a private plane so I could go meet so many people on a moments notice.Hearing the news that Olivia Davis is once again struggling scares me. I have never met her family but I feel like I should be there. Stanford is not a really long way from where I live. I hurt when these kids hurt. I hurt when their parents hurt.I am heartbroken when things don't go well. Maybe I am too overinvolved or maybe it's just because I view these people as part of my family.

Many of these people I have never met and will likely never meet but I have a bond with these people.I cry when a CHDer dies whether or not I knew them even a little.I am happy when a child goes home from the hospital or celebrates a birthday.What many parents would consider small milestones such as birthdays or crawling or walking,heart parents treat it differently. I treat it differently.

These kids are a part of my life. They are my heart sisters and brothers and they inspire me to keep going. They inspire me to keep living my life. When I'm down,knowing that they are out there looking up to me,reminds me to keep moving forward.They remind me not to feel so scared by changes. They remind me that I am here for a reason. I am here to fight for awareness for them. I am here to fight for accuracy.I am here to love them and love their families as deeply as their families love me.

CHD Parents and Survivors thank you so much for your constant love and support.I am truly blessed to have you all in my life.I love you all more than you'll ever know.

Respect

We don't always agree with each other's opinions,but the most important thing is to remember is we have to respect each other. Over the past 24 hours I have been disrespected frequently and told what to do about this whole situation.I have been told to back down and leave well enough alone but I cannot do that.I will not allow someone to tell me what to do.If I didn't think this was the right thing to do,I sure as hell would not have done it.

I am doing this for the HLHS families.For Survivors and for Angels and some people don't seem to realize that. I got messages and FB posts telling me that I was wasting my time and that I was fighting a losing battle with a network.

Then I had a small circle of heart moms who stood beside me while I stood my ground and fought for what I believed was right.These 5 women were just the women who I have forged a bond with.
-Krystal Van Ryssel
-Stacey Lihn
-Tina Snel
-Stephanie Rastelli
-Zoe Lee
Without these ladies in particular cheering me on I don't want to think about how much worse yesterday could have been.I am so greatful to them. I also recieved support from HLHS moms I have never even met on Facebook.

So even if you do not agree with my decision to go up against the network,please be respectful.

Respect goes a long way.

Done

I have been fighting all day to get ABC to see the gross errors that they made last night.I have gotten people angry at me and telling me to leave the hell enough alone and then when people on Private Practice's FB page made stupid statement I was told to let it go. Which I cannot do.All I am trying to do is be the voice of so many HLHSers and I have lost my inspiration to do it because of all the crap I have been through today.

We need more CHD Awareness but last night was not the way to go. Sure it was only a show but as a medical show you have to be accurate. You have to make sure that you get things right. Last night was a travesty.

I don't want to fight anymore. I can't fight for the kids if I am getting flack for it. I will be standing my ground on this issue,but I am angry and disappointed that people have doubted my choices and decisions all day. It's hurtful and after today I suppose I know who my real friends are.

Last night

First let me say that I know not everyone takes my stance on this,but if you are a medical drama and you are talking about something as serious as a heart defect take the time to get the facts right before you put it on your show.I know Private Practice has a medical team and I am extremely disappointed that they clearly didn't consult the team. Or if they did,the medical team was clueless about HLHS.

This was only part of the letter I sent to ABC.

Congential Heart Defects happen in 1 out of 100 people and Hypoplastic Left Heart Syndrome is the defect in 1% of these cases.
However Hypoplastic Left Heart Syndrome is not a death sentance. There are options for the family.There was no reason to paint it as doom and gloom.If Hypoplastic Left Heart Syndrome was truly a death sentance like was stated on the show than many of the children and adults I know who have Hypoplastic Left Heart Syndrome would be dead.And they aren't.Get the facts correct.

This was what I posted on the ABC boards which I never do.

I have been watching Private Practice from the beginning and this is the first time I was truly angry by an episode.Congential Heart Defects happen in 1 out of 100 people and Hypoplastic Left Heart Syndrome is the defect in 1% of these cases.

However Hypoplastic Left Heart Syndrome is not a death sentance. There are options for the family.There was no reason to paint it as doom and gloom.If Hypoplastic Left Heart Syndrome was truly a death sentance like was stated on the show than many of the children and adults I know who have Hypoplastic Left Heart Syndrome would be dead.And they aren't.Get the facts right.

There are options that the show didn't even talk about(Or if they did talk about them we didn't see it and that's just as bad).

With Hypoplastic Left Heart Syndrome they generally go for the 3 stage operation.

1. Norwood at about a week old depending on the heart function

2. Glenn about about 6 to 8 months depending on the heart function

3. Fontan at about 3-4 years depending on the heart function

Some kids are not eligible for the 3 stage operation so they opt for a heart transplant.The problem with that is that there is a shortage of infant hearts available.Some patients have the three stage operation but still need a transplant in their teen years.

I know Private Practice has a team of physicians that they consult for storylines and maybe they need to find a pediatric cardiologist willing to help in cases like this.

Mothers who are pregnant with a Hypoplastic Left Heart baby need accurate information. There is hope for these babies even if the show failed to show that.

I truly hope that the letter and the postings will get to the right people. I'm still pretty steamed about the misinformation and I can only hope that the moms who are pregnant with HLHS babies don't lose hope because of this. There is always hope,

Understanding

I didn't truly understand my CHD until about 3 or 4 years ago. That was partly my fault because I didn't really want to know. I didn't ask questions of anyone.In fact I always let my mom or grandmother ask them for me.And yet in other non-medical parts of my life I did ask questions and I was very assertive.Part of it was the fault of my family. They made the mistake by not explaining things to me from an early age in very clear terms. They micromanaged my medical care from the beginning and it wasn't until right before my last surgery that they relinquished control at least somewhat.

I had been in control of taking my medications and keeping an eye on my heart rate from the time I was about 10 or 11. Everything else was things I didn't care to be involved in. That was the mistake I made.I should have been more assertive and done the questioning myself. I remember the last time I was hospitalized for arrhythmias,was the first time I asked a question.I asked my cardiologist and my surgeon (who came to see me for no reason at all) if the arrhythmia could be caused by stress.One doctor said no and the other one said yes absolutely.

I recieved a book in 2008 called It's My Heart which detailed a lot of CHD's and finally I was able to understand mine and why they called it Double Outlet Right Ventricle Tetralogy of Fallot Type. I had always known that in some ways DORV was like ToF,but I never understood how. Now I understand that it's because DORV shares 2 of the 4 ToF defects.

Parents make sure you tell your children early what exactly is wrong with their heart so they understand it earlier than I did.Obviously tailor the explainations to be age appropriate,but nevertheless be honest with them from the beginning.

A Normal CHDer

The other day I was having a conversation via text messages with another Adult CHDer who was nervous about me meeting her because she did not live like a normal CHDer. I told her that it didn't matter and that I was not a judgemental person.

Then I got to thinking.Is there such a thing as a "normal" CHDer. We all have different complications and restrictions.We don't all have the exact same diagnosis and we don't all have the same cardiologist who imposes the exact same limits on us. Some of us have pulmonary issues in conjunction with our cardiac issues.Some of us have skeletal-musuclar issues as well.Some CHDers have strokes and some don't. And some of us use a wheelchair to get around.Not because of our cardiac issues . but because of other secondary issues. Every single CHDer that I know is different in diagnosis or restrictions or in complications.

Some of us adult CHDers aren't as careful with our bodies as we should be.Some of us take unecessary risks in order to feel "normal" We know we should be more careful but some of us honestly don't talk about our cardiac issues outside of the online communities that we are a part of.

CHD kids learn at a very young age that they are different than other kids their age. These kids see more doctors,endure more pokes and procedures than healthy kids do.They often don't like talking about it to their "healthy" friends because they don't want to seem different.

So not only are us CHDers not "normal" because of everything we have had to go through. Each of us CHDers has had a unique journey whether it's because of the diagnoses or the complications we have endured or even the restrictions that have been placed upon us.

Fear of the unknown

Next week I'll be making an appointment with a new cardiologist. I am scared to death to do this. I have been with my pediatric cardiologist since I was 2,so for 23 years.He knows me and my medical history better than anyone else does (except my family) Having to transition to an adult cardiologist is nerve-wracking. I have an excellent relationship and rapport with my pediatric cardiologist and I don't know if I'll have that same thing with the new guy.

I know that this has to be done because my pediatric cardiologist isn't well educated in the issues surrounding aldults with complex CHD's.But the idea of leaving him and the office staff who I have grown to love and appreciate (especially my favorite Echo tech Penny) scares me to no end.

I wonder many things about the new guy.I wonder if we'll develop a good patient/doctor relationship.I wonder if we'll have a good rapport and I wonder if I'll trust him.I wonder what kinds of tests/procedures he'll want to run.I wonder if he'll want to do a cath so he has a better idea of my cardiac function.

Life is scary.Change is scary. But in this case it is a necessary change.That said I don't think I'll be able to relax until I have met the new guy and can see if we mesh well together as a doctor/patient relationship.

Musings

I never knew this family,but today I cannot stop thinking about them.Nicole DiCarlo and her family and friends are celebrating the life of her sweet boy Travis Gary DiCarlo who passed away at only 16 months of age. I cannot imagine the pain that this family is experiencing and I wish more than anything that I would take their pain away.

No one deserves to have to deal with this. No one deserves to lose a child. I wish that parents didn't have to experience this. I know that people say life isn't fair,but this certainly isn't.It's not fair that a mother should have to bury their child.It's not fair that a mother should outlive their child.

But then again nothing about the CHD  world is fair. It's not fair that 3 year olds see more doctors than kids their own age.It's not fair that they are subjected to surgeries and caths and pokes.It's not fair that normal for these kids is a lifetime of health concerns.

Knowing other kids who are going through the same things is a huge morale booster. I didn't have such a big group of other CHDers when I was little but nowadays there is a bigger circle because of the advancements in medicine.More CHDers are living longer and developing friendships with other CHDers through heart camps and support groups.

My Letter to my CHD Family who I have met on Facebook

Dear CHD Family

 First of all, I want to say thank you. Thank you for allowing me to share in your successes and failures,joys and sorrows.Trust has always been a big issue for me so being allowed in to so many people's lives so willingly feels amazing. Knowing that people care about me is a great feeling. Especially given that the majority of you I have never met and yet you still trust me.

I thought I was done being in the CHD community. I wondered why I should even bother.I didn't think I had anything to offer to CHD parents. I figured I was done with surgeries and other invasive procedures so I didn't think I could get anything out of being involved in the CHD community.But I was wrong.My job now is to offer support to those who are just beginning their heart journey. I want to be there,emotionally,physically for those families who are embarking on both the scary and unknown.

I also have to thank several of the CHD Angel parents who have opened their hearts and have loved me so much despite the pain they are experiencing every day.They are truly inspirational to me and I can only hope I can be half the woman that these women are. I can only hope I find the kind of partner that many of these Angel Mommies have.

Thank you to the parents for allowing me to share in your children's journey's.Thank you Angel parents for sharing your angels with me. I know I have been told that CHD parents are blessed to know me,but they are wrong. I am blessed to know them. Their friendship and their love has meant more to me than they'll ever know.

Thank you to my fellow adult CHDer's. I love you all so much. You are my sisters and brothers. We share a kind of bond that our heart healthy siblings cannot understand though they may try to.

Thank you all for your love.Thank you all for your friendship and thank you all for your support

<3

Alexia