Friday, April 3, 2009


This is a big step for me.. I have never posted exactly what Tetralogy of Fallot (TOF) is. It's still just very hard... As Bentley's surgery approaches, I feel myself slowly falling apart again. I am so thankful for all of you who are praying for Bentley, and lifting us up. It is working. I have also received many many emails from other people who either have or know someone that has TOF. I love hearing all of your amazing journeys and it does help give me so much more hope along with everyone’s prayers. At the same time, I am terrified. When you consult with the surgeon he informs you of all the risk, and honestly it is everything you don't want to hear.. and I mean EVERYTHING. He gives your percents of the chance of each of these things happening.. the feeling is indescribable. Just know that I am so thankful for all of you, and your prayers..

Here is a diagram of a normal heart and a heart with TOF, below that is detailed information explaining TOF, and below that are Bentley's details.

(click the picture to enlarge)

What It Is?
Tetralogy of Fallot occurs in about 5 out of every 10,000 babies. There is no known reason for this. It just happens. Tetralogy of Fallot has four key features.

* Large Ventricular Septal Defect (VSD) - A VSD is a hole in the part of the septum that separates the ventricles—the lower chambers of the heart. The hole allows oxygen‑rich blood to flow from the left ventricle into the right ventricle instead of flowing into the aorta, the main artery leading out to the body.

* Pulmonary Stenosis - This is a narrowing of the pulmonary valve and the passageway through which blood flows from the right ventricle to the pulmonary arteries. Normally, oxygen-poor blood from the right ventricle flows through the pulmonary valve into the pulmonary arteries and out to the lungs to pick up oxygen. In pulmonary stenosis, the heart has to work harder than normal to pump blood, and not enough blood can get to the lungs.

* Right Ventricular Hypertrophy - This is when the right ventricle thickens because the heart has to pump harder than it should to move blood through the narrowed pulmonary valve.

* Overriding Aorta - This is a defect in the location of the aorta. In a healthy heart, the aorta is attached to the left ventricle, allowing only oxygen-rich blood to go to the body. In tetralogy of Fallot, the aorta is between the left and right ventricles, directly over the VSD. As a result, oxygen‑poor blood from the right ventricle can flow directly into the aorta instead of into the pulmonary artery to the lungs.

Together, these four defects mean that not enough blood is able to reach the lungs to get oxygen, and oxygen-poor blood flows out to the body. So TOF does not mean the heart is failing, it really means the child is suffocating. Babies and children with Tetralogy of Fallot have episodes of cyanosis (si-a-NO-sis), which is a bluish tint to the skin, lips, and fingernails. Cyanosis occurs because the oxygen level in the blood is below normal. Babies and children can also have what are called TET spells or blue spells when their oxygen levels get too low and the child may quite breathing. Ever heard of a "blue baby" if so, this is what they were referring to. For this reason, the child must not get too upset, crying can easily bring on a tet spell. Tetralogy of Fallot must be repaired with open-heart surgery, either soon after birth or later in infancy.

Complete Repair:
To do a complete repair, the surgeon closes the ventricular septal defect with a patch and opens the right ventricular outflow tract by removing some thickened muscle below the pulmonary valve, repairing or removing the pulmonary valve and enlarging the peripheral pulmonary arteries that go to both lungs. Sometimes a tube is placed between the right ventricle and the pulmonary artery.

More surgeries?
After the first complete repair, residual problems may require you to have more open-heart surgeries. It is very common for there to be 2 more open heart surgeries or possibly more.

Bentley is 7 months old and has TOF. Her first open heart surgery is scheduled for May 20 (less than 2 months), she will then have the complete repair. During the surgery they will patch the hole, and work on her pulmonary valve. Most stories I have read about or talked to other moms, their surgeon were not able to save their pulmonary valve... it all depends on the valve... how narrow it is to begin with. Bentley's cardiologist says her pulmonary valve needs some work for sure.. but he thinks he might be able to save it, which will be AMAZING, he just won’t know until he goes in during surgery. If he can save her pulmonary valve and everything goes smoothly this could very well be the only open heart surgery she will need. Please pray for this. However, if he is unable to save her pulmonary valve she will definitely need 2 more open heart surgeries as she grows. Usually they do these around 5 or 6 and again around 18 to 20.. the timing depends on the growth of the child. We have decided to go with an amazing surgeon that has achieved so much (more details on him here) so I have complete confidence in him and his team. And I know that God will be guiding them all during the surgery. But again as the days go by I slowly feel that "fear" coming back. I use to be such a strong person, but the day I gave birth to my first child, I turned into the biggest baby you will ever meet.. If you would like to read the beginning of Bentley's journey.. how we found out and more on Dr. appointments, you can here.

I just wanted to post a little about this since I haven't before. Thank you all so much for praying for Bentley and our family. I cannot even begin to tell you how much we appreciate it. Really, it just brings tears to my eyes every time I get one of your amazing comments, emails, or if I am looking at a blog and see Bentley's prayer button. It just makes my heart melt to know that you care so much. We are so grateful for that!


  1. There's not much we bloggie friend can do to help, but we WILL pray. And not just the week or the day of the surgery, but long before the time comes. And we will pray even after that. Our God moves mountains and parted the red sea for some really stubborn, rebellious people. But He did it because He LOVED them. And He loves us too. I'll be praying for COMPLETE healing and the wildest of blessings & miracles. Hang in there, you're in good Hands :-)

  2. Dani (Daniella) is right....

    Our GOD moves mountains....

    I can totally imagine the fear and heartache you are experiencing... but God says 352 times in the bible, one for every day of the year...TO NOT BE AFRIAD, DO NOT FEAR!!!

    HIS plan is already written.... and although as peaceful that can be, the not knowing can me terrifying for a mom of a child. I KNOW THIS!

    We surround you will love, hope and prayer!!!

  3. I googled TOF the other day b/c I didn't know exactly what it was, and everything you posted was exactly what I read; I'm glad you posted all this information--it helps those like me who don't know what it is understand more. You said you weren't very strong, but I would disagree, I think you are very strong and I know that God will continue to make you stronger and stronger every day, as well as Bentley...he is right along with ya'll on this journey; I am praying for Bentley as well as your strength many times a day...our God is amazing and I know that he will guide ya'll through this!!!!!

    Lots of love and prayers,

  4. Thank you for posting about TOF and filling us on.... I know it really took a lot of you to to post about... you may say you are not a strong women but I know you are!! I can tell bye reading this post and many others! U are stronger than you give yourself credit... You are the glue that holds your family together... I have been praying and will continue to pray for you, that God gives you strenghth to stay strong today and everyday... and will be praying for your whole family and for a successful and amazing and complete healing surgery for Bentley.... Faith Hill sings a song that I love and I shared it with another blogger the other day and thought Id share it with you...

    Better Days

    Hard times are fallin on you
    Even when you smile I see the hurt come through
    And I know it feels like its never gonna end
    You say nothins been right for a long time
    And every step you take is an uphill climb
    I see youre reachin out
    So let me tell you friend that

    Better days are comin around
    I know you feel like
    The whole worlds gone and let you down but
    Better days theyre comin for you
    I know they will
    cause Ill be right here makin sure they do

    And if your sky aint lookin the fairest
    There aint no reason to be embarrassed
    Aint nobody in the world aint felt
    The rain from time to time
    And just when you think its all over and done
    The night get easier one by one
    So take my hand and hold on tight
    And soon youre gonna find that

    Better days are comin around
    I know you feel like
    The whole worlds gone and let you down but
    Better days theyre comin for you
    I know they will
    cause Ill be right here makin sure they do

    I know what its like
    To have to face the fight
    But I wont let you stand alone, no

    Better days are comin for you
    I know it feels like
    The whole worlds gone and let you down but
    Better days theyre comin for you
    I know they will
    cause Ill be right here makin sure they do

    Oh, better days
    I know, I know, I know
    The whole worlds gone and let you down
    Better days are comin for you
    I know they will

    love and prayers and HUGS

  5. We are praying for ya'll!!! I think that you are strong, everyone has times of weakness but that does not mean you are not strong. Ya'll just enjoy that sweet baby and know that God has his reasons for this!!! Girl ya'll are a wonderful family and I have Faith that everything will be just fine :)

  6. I never knew TOF was so complex!! Such a great description of it!! You've already mastered it and Bentley hasn't even had surgery yet. Just wait to see how much you know afterwards.

    You are an incredible mom! We are praying for Bentley as her upcoming surgery approaches. Did they give you any indication as to the average recovery time?

    Keep us posted.

  7. Thank you for stepping out of your comfort zone and educating us on TOF. Now we'll all know exactly what you are talking about when surgery day comes.

    Personally, I think God sent me to your blog to love on Bentely and your entire family, to introduce me to a world that I knew nothing about. You are a role model of strength and unconditional love. You'll never know how much your story and Bentley's life has changed perspective in my own.

    I will always and forever be thinking about, praying for, and loving on Bentley.

    Thank you again.

    Analiza and The Bernat Boys

  8. Oh hon..I seriously can not imagine what you are going through..I hope it helps knowing that you have people that care about and love your little girl and your entire family. I pray for your little girl every night and want you to know that I'm here if you ever need to talk.

  9. Crystal, Thank you for sharing all that info on TOF. I had been wondering what it was and not had the chance to google it. I can only imagine how you are feeling with that date approaching. I believe all your bloggin buds will be sharing thoughts that day with you.

    Bentley is just beautiful and your other kids too. I enjoyed seeing the creek/mud fight. There were a couple pics ( I think #4 and 5) that would look awesome in a frame.

  10. I came across your blog and had to stop and read about your precious Bentley. What a blessing she must be to your family! My third and youngest also has tetralogy of fallot, had his repair at 8 weeks and it never slowed him down. He is turning 3 in July. I will be praying that Bentley also has that best possible outcome. It is great that she is well enough that you can wait as long as May for her surgery. Of course, what could be better than a miracle?

  11. Awww....Crystal..I can't even imagine what you and your family is going through. I can't say I understand but I can say that we will be praying you, your family, Bentley, the Doctor's and the nurses. What a very difficult thing...I'm so sorry that Miss Bentley has to go through this but I know she is strong. Our God is good and he is in control...Bentley is one of his children....he will lay his hands on her...I just know it. We will continue to keep you guys in our prayers and I will pass the prayer chain on. Let me know if there is anything I can do!


  12. I know that you, as a mom, a believer, a wife, a blogger, are a strong and beautiful person. I want you also to know ... that you are entitled. You are entitled to feel however it is that you feel. You are entitled to be scared and to cry. I know that God works miracles... you have read Ky's story.... you know that my miracle is sleeping in the next room. I know that your miracle is sleeping in your next room also... but we are all human. God does not expect us to be perfect. If we were perfect He would not have sent His only son to die for us.

    Through the entire time with Kyleigh's journey (almost 4 years now) I have never physically uttered the words "I don't know if she will make it"... because I never once thought she wouldn't! I don't know if it was blind faith or ignorance but whatever it was it got me through.

    The night BEFORE the OHS #1 was the worst for me. I held onto Ky until Troy pried her from my arms and MADE me go to sleep... I was holding her in the rocking chair, Tachy monitor, feeding tube and all! Troy and I laid in bed that night and cried together. We mourned for the first 6 months of her life that were stolen from her (Ky was REALLY ill... barely alive by the time we had her surgery). We cried for the next day and the 13 hours of pure He!! that we were about to experience. We cried out of fear of the unknown and we begged God, through snot and tears to hold us closer than ever before.

    After the tears subsided the peace set in and we have never looked back. Not one moment were we ever afraid again. Even through our entire month in the CICU, the multiple emergency intubations, extubations, no rhythms, pacering, fevers, staph infections, and so on. Stressed, upset, totally out of sorts... yes. Even with the SECOND OHS... still peace.

    So... my prayer for you... is that He will wash over you with that perfect peace that washed over us. That our Heavenly Father will give you grace and strength. That He will not only allow you to see this through but that you will ride on HIS shoulders all the way. I am praying that he will carry you and Bentley. That He will bless the lives of her doctors, bless the hands of anyone who touches her beautiful little body, bless the hearts and minds of everyone who will be caring for her.

    On a lighter and almost comical note... don't worry about percentages. Ky's nickname is 5% Kyleigh for a REASON. If ever the chance of something happening is less than 5% it will happen to her. She has made it through more 5%'s than anyone I know. Its a running joke now with all of our surgeons.... I mean really... who goes in for open heart surgery and comes out with pig lungs? MY KID! So... there's your laugh for the day!

    Many hugs and prayers.

  13. I just wanted to let you know that miracles do happen. I was diagnosed with VSD at only a few days old. The doctors determined that surgery was the only way that I would be healed. Surgery was scheduled when I was about 4 so that I would be ready to start school.God had another plan. Before surgery, the Dr. did an X-ray. God chose to heal me himself. Since then, I have been monitored yearly, but have never had any issues with the VSD. I am now 24 years old and recently got married. GOD IS THE MIRACLE WORKER! I am praying for your sweet girl.

  14. Hi I have never heard of TOF. And I'm really glad you posted about. I can't even emagine what you and your family must be feeling.

    I am praying hard for Bentley, and that God will give you & your family some peace in this trying time. Trust in God sweetie, He's cares and is with you:)


  15. I'm coming over from Hannah's blog. I'm sooo sorry about all you've been through and continue to go through. Your family is definitely in my thoughts and prayers. (((HUGS)))

  16. i just happened to come accross your page when i was searching congenital heart defects. my daughter was also born with CHD. she's only 7 months old and she's already had 4 surgeries and is waiting for another one. i know if God can help my little girl, he will help yours too! i will keep your little girl in my prayers =)

  17. my son had t.o.f he had his repair done in oct.07 it was the worst & best day of our lives, worst because of what baby chandler was going through,and best because it was finaly over,no more fears of waking up to a empty crib,or doctor's bad reports,of what they think is wrong verse what they was just finaly over,i give credit to where credit is do,an that's to my lord and saviour JESUS CHRIST.''the name above'' Pulmonary Stenosis,Right Ventricular Hypertrophy,Overriding Aorta, Large Ventricular Septal Defect, Tetralogy of Fallot is just 'JESUS'

  18. Hi, my wife was one of the first children to be saved by the Blalock/Taussig shunt for "blue babies" Her operation was in 1946 and she has her second corrective surgery in 1965 when the heart lung machine was in place. She is 69 this year and is a living testament to how God can work miracles.


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