Monday, April 26, 2010

CHD Q & A

These some of the brilliant questions that my sister Jen has had me answer to help her get her paper for college going.. I love every question and I'm really impressed with them. Here is a peak into quite a  few of those questions..

1.Describe Natalie's condition. (or refer me the entry in your blog where you explain it in detail)
Natalie's condition is a type out of 35 known congenital heart defects called "Tricuspid Atresia". This basically means that it's a form of "Hypoplastic Right Heart Syndrome" where the right side of her heart did not form properly and was born with a single ventricle heart. Here is great definition from Denver Children's website: "Blood normally flows from the right atrium to the right ventricle through the tricuspid valve. In tricuspid atresia, the valve is replaced by a plate or membrane that does not open. The right ventricle therefore does not receive blood normally and is often small."
This condition typically has 2 other malformations that make up the "tri" part of her defect. They are called "ASD" which means "Atrial Septal Defect" and a "VSD" which means Ventricular Septal Defect. Natalie's VSD is now gone due to growth (which is amazing and does happen) but when she had her VSD there was a hole in between her heart's left and right ventricles. In actuality, this VSD helped keep her alive before her first surgery at 6 months old. Her ASD is still there and is "a hole in the wall (called the septum) that separates the left atrium and the right atrium" (via Denver Children's website). But this defect doesn't have much of an impact on her currently.

2. Describe when and how you found out about Natalie's CHD.
This is one of those stories that I find amazing because I've spoken to too many other families with either similar stories of even more nightmarish stories. It's really unbelievable.
With Natalie it was at her cardiac checkup when she was 3 weeks old. Prior to this appointment and her birth the nurses and doctors did hear a murmur. No one was too concerned since babies can be born with a mild and harmless murmur at any time. After 24 hours of being at the hospital post-delivery we were told the murmur was gone, no one could hear it. We took Natalie home and my mother in law kept commenting on Natalie's purple legs and feet. I chalked it up to her fair skin and with not being leveled enough when held and didn't worry. Days were going by with Natalie home and we noticed that she slept alot. And didn't eat much but according to her pediatrician Natalie wasn't losing weight and was "just not a big eater" and that it was Natalie's "normal". When our regular pediatrician heard Natalie's murmur a few days after being home we were given a referral to see a pediatric cardiologist at John Hopkins Hospital. "Don't worry" our doctor said.. "my son has a VSD and they took fantastic care of him. Everything will be fine."
The morning appointment couldn't have come any faster. 2 weeks after seeing our pediatrician Natalie was able to see Dr.Ravekes at a Hopkins clinic that was in Odenton, MD. The EKG showed nothing unusual and neither did her oxygen levels.. it was at about 95 if I remember correctly. So far, all signs were pointing to normal. Then the echocardiogram began and that was taking longer than I thought it would. Natalie lay there calm and was very hungry that day so she kept drinking and drinking away while the wand was slid back and forth across her check, neck, and tummy. About 45 minutes into the procedure, Dr. Ravekes pops into the small dark room, takes a long look at the monitor and says, "well, Natalie..you are trickier than I thought". I started to get sick to my stomach with anxiety, but kept my cool on the outside. They let me bring Natalie back into the exam room and at this point, James was so tired and sat there with his Gammy (grandma). Dr. Ravekes walks in about 10 minutes later and says, "well, I have some very important things to tell you". And then proceeds to show me a diagram her drew (I still have it) of Natalie's heart condition and what a regular heart is supposed to look like. Natalie's heart looked nothing like a regular heart.. I started crying but held back my tears for as long as I could. He says to me, "I'm going to give you a few minutes because I can understand why you would be upset..take a few minutes". I could barely see him walk out the door because my eyes were so blurry from the tears. I cried.
He came back and spoke very positively of how well Natalie's heart and body were coping and described her possible surgeries and that there is an 80% success rate.. the purple and blue spells to expect..that she may still be able to live a normal life and even have children later on.. and that we're in good hands.
I look down at Natalie, falling asleep in my arms with a full belly of formula, was breathing well and had red lips, and I knew right then and there that I was terrified but that she was okay. To this day though, I am still upset that I was without Rick to be there to hear the news. What can you do?

3. What prenatal tests did you have? Did you have a first trimester fetal echocardiogram? During what months of pregnancy did you have ultrasound(s) done?
Other than the usual round of testing that a regular pregnancy (compared to a high risk pregnancy) includes, I had nothing unusual or different happen. I don't even remember having a first trimester anything. Not even an ultrasound. The ob/gyn I had for the first 20 weeks of my pregnancy told me that because my previous pregnancy and birth went so well, there was no reason to have a 1st trimester ultrasound. I still don't know why I didn't push for one anyway. During the 2nd trimester, at some time around my 5th month I finally had my first ultrasound. This was during the time that I had just transferred to a new ob/gyn who I wanted to give a try. She was highly recommended by a good friend of mine and I kind of liked the idea of trying someone new. I had the ultrasound and found out that Natalie was a Natalie. They told me that everything looked great but they couldn't get clear enough pictures of the heart and that I'll probably come back again soon to do a more in-depth look after my doctor takes a look at the results.
When I went in about a week after the ultrasound my doctor says that everything looked great. Boy was I confused. I asked her if the heart pictures came out clear enough and she said yes.
I was weirded out but unfortunately, let it be. All I kept thinking was "everything's probably fine anyway". That was the only ultrasound I ever had while pregnant.

4. What tests do you feel might have detected Natalie's CHD?
For sure a 2nd ultrasound. I shoulda coulda woulda but either way I should have put my foot down and demanded another ultrasound. If they had found something peculiar I probably would have then been sent to have a fetal echocardiogram at Hopkins. In this day in age, some necessary surgeries are being performed while the baby is still in utero.

5. Did Natalie have a pulse oximetry done in the nursery?
Nope. But to be honest, even if she had I don't think anything would have been detected. Natalie was tricky like that for the first month or so. Her O2 levels were always higher than expected for a baby with a CHD.

6. What are the limitations (if any) on Natalie's life, both presently and in the future?
Right now, there are none. Right now, Natalie has visits from a physical therapist where she just graduated from receiving physical therapy biweekly to monthly visits for some gross motor delays from her long recovery from ages 6 months to 8 months. From those few months of recovering and having a hard time getting her mobile, Natalie's thigh, ankle and foot muscles need extra therapy. She didn't officially begin walking until she was about 21 months old. For the future, Natalie, just like now, will set the bar on her own limitations when it comes to daily activities. As for being the next football captain, soccer goalie, or wrestler, those things aren't safe for her. Any sort of contact sports could really hurt her heart. Other than that, she is encouraged to get plenty of exercise and will take a rest if she needs one. Pretty amazing, eh?

8. What changes do you want to see implemented in the possible detection of fetal CHDs?
Overall, just more awareness. More discussion. More research. Less of scaring soon-to-be new moms but more of just letting them know of things..signs..of things they can look out for while they bring their baby home. Letting everyone know the statistics of CHDs and how too common it is. How too many kids are dying because of it. There needs to be some form of screening process before children come home from the hospital. Whether it be the use of pulse oximetry or an echocardiogram, I'm not yet sure as to what will be the most cost effective and effective for detection, but certainly something needs to change. Too many kids are going home undetected and dying without notice in their parents arms within days. I still wonder how many undetected CHD kids were labeled as passing away from "SIDS".

9. Do you feel Natalie was born with her CHD for a purpose?
Absolutely. As her cardiologist from Hopkins once said, "Natalie's body was made for this". She always coped well even when situations like her open heart surgeries made her even more fragile. She's always pulled through with flying colors. I don't want Natalie to always focus on her heart condition because I want her to blend in with the crowd and be a normal kid. I want her to keep being brave and take (safe) risks. As she gets older I'll let her decide if she wants to be active in spreading awareness, CHD organizations, or even do walks to help fund research and assist families ( I can't say "cure" since there is no cure). But I'll let her decide if she wants to do all of that. I think she was born to cope with her defect and to make me a better mom.

8 comments:

NikkiAWardell said...

What a great blog post. You can visit Paul Cardall at www.livingforeden.com. He also has tricuspid atresia and just recently had a successful heart transplant and is 37-years-old! He has a very inspirational story! I never had a maternal fetal ultrasound either, and my daughter's CHD wasn't diagnosed in utero. Although, her murmur was LOUD and at three days old she was diagnosed with Tetralogy. Her condition would have been picked up with a pulse ox test had her murmur not been so obvious. Her normal sats before surgery were around 85-87 and her sats when crying or exerting plunged to around 60-65 and sometimes lower. Your blog is great and I am excited to have found it! Hope you'll visit us at www.perfectbrokenhearts.wordpress.com

NikkiAWardell said...

Just noticed you were friends with Paul on facebook....you must know about him!!!:)

Tiffany Strickland said...

Hey Dawn,
As you know Kylie has the same CHD as Natalie ... and that question would be the same =)

2. Describe when and how you found out about Kylie's CHD.
It was during my 20 week ultrasound. It was taking longer than expected and I thank god that I had some one who was on the more experienced side because she detected that there was a problem right away! After my ultrasound I was sent home (it was done in another office, Not my OB) and not more than 20 minutes of being home I got a phone call from my OB telling me that they found something on my ultrasound and to come in right away. Trying to be as calm as he could he explained to me that Kylie appeared to have some sort of heart defect but wasn't able to give me an exact diagnosis. I was then sent of to Winnie Palmer hospital (where Kylie was born) for another ulatrasound and testing. It was then that I was told the even more horrible news and about Kylie's condition. I wish I could have gone back to give this particular doctor a piece of my mind because one of my options was to "terminate" the pregnancy if I should choose. AT nearly 20 weeks! That was just not an option.

What prenatal tests did you have? Did you have a first trimester fetal echocardiogram? During what months of pregnancy did you have ultrasound(s) done?

With more tests, blood work, and an amino nothing came back as to tell us why this happened. Just the "unlucky" winner :(
I am however glad though that I had time to prepare for her birth and would have much rather found out earlier than have the possibility of taking her home with a worse outcome! I am very thankful for that day.

5. Did Kylie have a pulse oximetry done in the nursery?

Knowing what to expect with Kylie after her birth I was able to hold her for a few short minutes before they wisked her away to the NICU. They came back and told me that they were going to transfer her to Arnold Palmer Hospital since she wasn't doing well (very blue with low sats) so that she could have around the clock care from a more advanced set of doctors in the PICU. At ten days old Kylie was well enough to come home until her first surgery at 20 days old.

6. What are the limitations (if any) on Kylie's life, both presently and in the future?

Kylie is actually doing really well. I have heard other heart children that have to have therapists and be on medication. Kylie has done so well that this time she is the "lucky" winner! And like you said Dawn, she won't be a soccer goalie but she will be able to run around like any other child with rest when she needs. Which I have already noticed, that she gets out of breath and 15 months from running around like an animal but Kylie has not yet had her Fontan. To be scheduled within the next year.

What changes do you want to see implemented in the possible detection of fetal CHDs?

A simple placement of a pulse ox monitor on their little toe while they are being checked over doesn't seem so difficult to me! And if there seems to be something fishy about that then the further testing to save that child's life!!

. Do you feel Kylie was born with her CHD for a purpose?

I believe everything happens for a reason and God has put her here to be a little survivor and show us just how tough she is!! She is kicking butt =)

I really loved reading this and I LOVE reading your blogs Dawn! Keep them coming!!!!

LOVE TO OUR CHD BABIES!! <3
Tiffany (Kylie's Mommy)

Sarah said...

Wow, Dawn. Amazing Q&A. You are one tough momma. What a blessing it must be to look at her beautiful face every day and know that she is the miracle that she is.

Dawn B said...

Nikki- SO happy you found us!! I love connecting more with wonderful CHD families! Bookmarked your blog and can't wait to add it to my page. The first thing I noticed that I loved on your website is the header.. really really pretty! I was wondering, also how your daughter is doing now and how old she is!

Tiffany- thanks for reading!
Wow.. I had NO IDEA until now that they asked you to terminate. Oh my god. I mean..I just can't even begin to imagine the horror in being asked to do that.
And now look at her.. thriving and exceeding the odds. That baby girl was meant to be here (as if you didn't already know that..lol) so happy you shared your answers. I learned so much from you. And so happy that you knew about her condition before she was born!
Sarah- hey, now..you have a beautiful miracle as well! Every time I see new photos of her I feel so happy. She's so beautiful. Talk about miracles.. thanks so much!!!

annamarie said...

Awesome post! I don't remember if we are Facebook friends, but I post at Facebook.com/1in100. So here I am reading this as I sit in a Health IT hearing in Washington DC. My meetings tomorrow are all on pulse ox screening - although today there has been much talk about the Minnesota pilot as well. Basically, screening is coming down the pike. It has been nominated for universal screening with the national committee (HHS) and is in the external workgroup phase right now. I expect a vote in September - but will be here again in May to make sure we are moving ahead as expected. Funny thing - my best friend, and our heart baby Eve's godmother, was also in DC this week. She is a CRNA (anesthetist) at Mayo - one of the colleagues she was lobbying with out here is married to a man named Nick Zerwas. I met his 10 years ago as a high schooler when he was honored at an American Heart Assn gala. He has underwent 10 heart surgeries - and I am sure he was diagnosed with tricuspid atresia after birth. He is amazing...and I can't believe I "accidentally" met his wife here. I don't believe in coincidences any more! Anyway, here's a book he authored: http://www.deforestpress.com/item.asp?ItemID=42

I'll be hoping to connect with you as things progress on the advocacy front! Annamarie (mom to Eve, mitral valve defects, WPW, SVT, enlarged heart, 2 heart surgeries 2009)

SteveC said...

I'm a 43 year old man living with Tricuspid Atresia, and doing fairly well! CHDs are nasty in that rwo people can have the same defect, but totally different symptoms. Paul Cardall had to have a heart transplant; I'm still getting along on my original equipment.

I was born in 1966, back in the stone ages when Fetal Electrocardiography hadn't even been thought of and the only "prenatal testing" for heart defects was a good pediatrician. My first surgery was at Johns Hopkins in 1967. I never had Hypoplastic Right Heart Syndrome - that term hadn't even been thought of back then, I "only" have TA.

Natalie can live a good long, useful life, but she is going to need to be under a cardiologist's care for all of her days. And you aren't getting her an "average" cardio - you need, you want, you DESERVE the very best available. That very well may mean you have to travel to see him/her, but the results will be worth the effort. Encourage Natalie to exercise as much as she can, even if all she does is get out and walk every day. Her heart muscle is damaged, but she can keep it strong through exercise. Eat good foods and stay away from too much salt, that will help too.

She may need some more surgery in the future, once again, you want the BEST surgeon available. When she is old enough, encourage her to learn her medications and learn about her heart.

They used to tell kids like me that we were "fixed" after our surgeries... time has proven that isn't true. We still have problems as we age, like everyone does, but having a defective heart can cause an entirely different set of problems. We need to keep those doctor's appointments no matter what. Years from now Natalie will be dragging her boyfriend/husband to the doctor's office with her.

I don't know if we will ever cure, or eliminate heart defects - we may just find better ways to live with them. But there are a lot of treatments coming that will just positively startle you. A doctor at Wake Forest University in North Carolina is growing blood vessels and bladders in a lab, they are 100% compatible for transplant. No rejection - ever! Heart valves are being replaced through Catherization rather than open heart surgery, there are stem cell advances, and there is even a way to create or enlarge an ASD without cutting the skin - the future will be amazing! Natalie will benefit from some of these new procedures, I am sure!

Feel free to contact me if you need to (Wildcat - 3 - at - Gmail - Dot - Com) or drop by my blog, "Adventures of a Funky Heart!"
http://tricuspid.wordpress.com/

All the best;

Steve

Stefenie said...

Thanks for joining the blog event on my blog Dawn and sharing Natalie's story!