Wednesday, February 10, 2010

Hypoplastic Left Heart Syndrome - HLHS {CHD week}

(Joshua - 4 months pre-Glenn)
While there are many congenital heart defects, Joshua unfortunately has one of the most severe, if not the most severe. He has hypoplastic left heart syndrome, or HLHS. I still remember when the doctor who diagnosed him uttered those words & thinking to myself, "huh? Was that English?" Then a moment later I realized that incredibly long name would become a regular in our vocabulary. I don't often think back to the day when we found out because it was really tough. The short version goes something like this; I got a phone call from genetics the morning after our 20 week ultrasound. The woman wanted us to come back in immediately for more testing because it "appeared that a portion of the baby's heart looked smaller than the other side."
HLHS is as simple as that (if that is simple). Joshua's left heart is severely underdeveloped. Therefore, his heart required surgery at birth in order to support life.  Other options included transplant, pallative care and termination. We chose to have Joshua undergo the Norwood procedure, as we felt that it offered the best shot at life. The Norwood procedure consists of 3 stage surgical intervention. The Norwood at birth, the Glenn at a few months old (Joshua was 4 months) and the Fontan during the toddler years (or there about). The result of the procedure allows the heart to function with a single ventricle; the right ventricle in Joshua's case. It's actually pretty fascinating the way his body functions right now. The blood flows passively to the lungs from his upper body & then goes to the heart where the right ventricle then pumps the oxygenated blood to the body. After he has his Fontan the blood flow from his lower body will also be directed to the lungs & then to the heart, allowing him to function with higher O2 levels than his current state, which is at around 80-85%. If you know Joshua, you may have noticed that he looks a little bit blue. After he has his Fontan his O2 levels should be in the high 90's, and hopefully his lips will be pinker!

We have mixed emotions about sending our little guy back into surgery. While we know that it's for the best, it's awfully hard to imagine going through recovery again, this time with a boy who can very clearly communicate with us. When he was a baby I told myself time & time again that he wouldn't remember all that he had been through. It'll be harder to convince myself of that this time.
When he had his previous 2 surgeries he went into each of them in poor health. He had tough recoveries that left us in the hospital for 6 weeks each time. When he has his Fontan, I assume he will be in good health. He's enjoyed good health for nearly 2 years & I have faith that he will remain strong heading into the Fontan, which should theoretically make for a faster recovery.
Until recent years, there has been no hope for children born with this disease. We are so very fortunate that Joshua was born at a time where success rates are improved and real hope exists. With a few survivors now in their 20's, there isn't enough data to conclusively say how long Joshua's heart will provide him life. His heart will eventually fail him and he will need a heart transplant. We have no idea when that will be. It could be next year, it could be when he's 20, or 30, we simply don't know.
Having a child with HLHS has taught us many lessons. Lessons that I may not have learned otherwise. I try to see the good in the situation and truthfully while I wish I could take away the pain & suffering, I wouldn't change Joshua. He is a remarkable boy, because of what he's been through. He has the ability to overcome obstacles that others couldn't, and he doesn't even notice them. If I possessed a fraction of his determination, I'm pretty sure I could walk on water.  Obviously, that miracle isn't happening any time soon, but it's okay, because he is our miracle.


Herlehy Family said...

I am so happy Joshua is doing so well now...he is such a darling little miracle!

Lindsay said...

I can't believe that it is approaching that time again. It does make sense about him being so much healthier now that hopefully he will get an easier recovery. He definitely deserves that after those other two recoveries. By the way, I sure am glad to have you as a fellow heart mom, too. You have always had the best outlook on Josh's condition and it has helped me to find a similar sense of contentment with Marin's situation.

Dina said...

Joshua is an amazing little boy and you're an amazing mom.


Cortney said...

Hi There,

Thank you very much for posting this blog. I find the story very inspirational and am very happy to see that Joshua is thriving. The reason I write to you is because I am an Occupational Therapy student, and one of our assignments this semester is to present to our class a presentation on hypoplastic left and right heart syndrome - we would love to include a personal story. would it be ok if we asked you some questions, and possibly share some of the information on your blog with our classmates? thank you very much. Have a great day. All the best. Cortney

allison said...

I would be happpy for you to share about Joshua with your classmates. YOu can also e-mail me with any questions you may have:
Thanks for your comment:)

MelissaErinNiaJosh said...

Joshua is an amazing beautiful boy who has amazing support from yourself and family. I wish you all the best for the future and i will keep him close to my heart as i can imagine what you are going through. Very hard having to fight medical issues esp when it is your child. Good luck xxxx

Cortney said...


I just read your post - thank you so much for your willingness to answer our questions. My group members and I are working hard on our project now, and trying to come up with the best questions to ask you. Stay tuned for an e-mail. It will come from Again, thank you so much.

Cortney said...

Hi again, well, we have certianly developed alot of questions for you... but the e-mail keeps popping back as undeliverable.

we are really sorry to send you the e-mail this way, but we can't figure out how else to ask you the questions.

so, here is the email. Perhaps .

Hi Allison,
This is Cortney (and Chiko and Linda), the OT students. Thank you again for your willingness to talk about Joshua’s story. The pictures you posted are adorable, and your family is beautiful. We cannot imagine the strength that it took for all of you to persevere.
We are so excited to get your thoughts and words of advice. While a good grade would be nice, we are most concerned with how to make ourselves good practitioners. We hope that by talking with you, we will be able to educate our classmates about HLHS and also educate them on the best way to interact with the family. We tried to develop the best questions to ask you, but please forgive us if we have to e-mail you a second time. We also apologize if we have asked the same questions that 4967453048 people have asked you.
• When your family first found out about Joshua’s condition, how did you react to the information?
• What were your main apprehensions when Joshua came out of surgery?
• What was the most helpful information (resources) you received?
• What was the least helpful information (resources) you received?
• Did you receive OT services? If so, what were they?
• Did Joshua have a feeding tube? And if he did, how did you introduce solid foods to Joshua?
• What challenges did Joshua have after surgery?
• Does Joshua have any physical limitations right now?
• It appears as though Joshua is around 3 or 4 according to the pictures, is he currently in pre-school or pre-K? If so, how has he adapted to the school environment? Do you notice anything you might want to improve?
• Do you notice any learning limitations?
• What are your goals for Joshua now?
• Does he have any goals?
• As health professionals, what are a couple of the top things you would like us to do?
• Is there anything that you would like us to know that we didn’t ask?

Thank you so much for helping to advance our education, we are looking forward to your responses.
All the best for you and your family,
Cortney, Chiko and Linda

TateFamily said...

Thanks for sharing Joshua's story. 4 weeks ago my husband and I found out that our little baby boy im carrying has HLHS :(. Its been hard for me to come on the internet and not google about it cause im was scared to find out what i would see but tonight I thought why not, then I found your blog. Im so glad that your little boy is doing awesome sure makes me feel good. Thanks so much for sharing.

Ashley Schroeder said...

Is this account still active? I have so many questions for you and your family! I am about 24 weeks pregnant and my husband and I recently found out that our baby has HLHS. It has been really hard just to process it all. If you still keep this blog updated I would very much appreciate an email back to myself or to my husband
Myrl ironworker8*

jela mostak said...

Hy I m from Croatia.I Have son name Imbra. He have hlhs. He was haveing two op hearth and dyafragma. Can you send me email