HPLS - Left heart syndrome

[Berry1]

Tegam, thank you for your kind words.

Nicole, I have been really feeling for you and hope you are holding up ok. In terms of 'questioning whether you will come to a place of peace with your decision' I understand what you mean. In my situation, I wasn't so much 'at peace' because making that decision as a women who desperately wants that baby goes against your natural instincts, but I did come to a place where I knew it was the best thing to do for her. What really helped me cope afterwards was knowing that before we made the decision, we saw every specialist we could, did all the tests we could and explored every option and took into account the 'solid facts'. Also, getting support & understanding of my decision from those closest to me was really important, as I needed them to reassure me after the event. My husband was very rationale about the decision as well, it took me longer to get there, but we did it together, which is vitally important.

It's good that you are taking this week to think & talk about it and to see a psch, all really good steps. If you have any questions at all, please do ask. Obviously I can only offer advise from one perspective, but there are some great ladies here who can certainly give advise from the other, which is important. Hugs to you this week

[MistyFying]

Nicolabee I'm sorry you're in the midst of this right now

(installation of a stint - no heart bypass or reconstruction of aorta)

this is what my son had done when he was born. He had the stint at 3 days, and then underwent bypass surgery at 3 months to close some major holes in his heart. He recovered very quickly from the open heart surgery and was home in 10 days.

You asked what his prognosis is - I go back to his cardiologist once a year to check the repairs are growing with his body (the stint is synthetic). The cardiologist does an echo, takes blood pressure, checks circulation, height, weight etc to ensure everything is ok. He will only have to have more surgery if one of the repairs fails (they think this is very unlikely). He was in hospital for 3 months when he was born. We lived approx 70 mins from the hospital and I travelled up and back each day and my husband went in every night after work for a visit, and we had an 18 month old at home to look after. The 18 month old hasn't shown any real after-effects of the turmoil during that period of her life (just to reassure you that your other kids will be fine - don't let that weigh too heavily on your mind when making your decision).

I know just how awful it is to know there may be something wrong with your baby

[Nicolabee]

Berry1, you certainly sound as if you exhausted all possibilities before making your heartbreaking decision. That is what I wish to achieve, but still feel like I have a long way to go.

MistyFying, by what they have told us, it sounds as if your son's situation is different to ours - they are talking the stint, then the same 2 operations as for HLHS finishing with the Fontan, and all the long term issues of it being palliative and not a "fix". Is this the same path that you are on, or is it different? (as this sounds a lot more positive than that put forward to us)

I do wonder if what they see now in their ultrasounds is what they would see in another 4 weeks, let alone at birth? When we asked this they were quite adamant that it wont change.

DH and I have major issues at the moment with the thought of termination and burying a child whose life we will have brought to an end. Can we live with ourselves, and is that our right if there is a viable option for a somewhat healthy life, even if it does come with risks?

We still need to explore these options further. I am going to try and make contact with the cardiologist again at the RCH to answer our questions.

Thankyou everyone for your support and the sharing of your experiences, we really appreciate it.

Nicole

[nev]

i;m so sorry to hear about your little girl

[Nicolabee]

Yesterday was Laura's due date. It's surprising sometimes, how you do find the strength to soldier on.

I would like to share the next part of our story, for those who may be searching for information as I was; as to possible outcomes.

We consented to Laura having an autopsy to confirm the diagnosis, this was quite scary, as I was petrified they would come back and say, "sorry, we got that a bit wrong, not quite as a bad as we thought". But that was not the case.

Firstly I would like to say, we had the support of the Fetal Management Unit at the Royal Womens Hospital in Melbourne. They were fantastic and couldn't do enough for us, in providing information to assist us in making decisions (would never once tell us what we should do - and we asked!), to helping explain results, and sometimes answer the same questions. They have all the specialists down one corridor. We spoke to Neonatologists, cardiologists, geneticists, psychologists, social workers, obstetricians and their fantastic coordinator a midwife (and Mum) during our many visits.

We now know that (as a result of a new DNA array test) that Laura was missing some DNA, not chromosomes, but small peaces of DNA. These particular DNA are linked in with the heart. Laura's case so far is unique, however some similar cases (missing less DNA than Laura) show children with significant brain damage; delayed speech, lack of muscle control to name a few. She was also extremely small - less than the 5th percentile for her gestation.

In summary Laura had three major issues - severe growth restriction; the DNA deletion; and the Hypolplastic Right Heart. The geneticist explained to us that each one these issues was potentially fatal on its own, let alone stacked upon each other. It appears that each of these issues were interdependent, not isolated occurrences.

Had we not made the choice we did - when we did; we were probably going to be faced with a similar dilemma in another couple of weeks due to her restricted growth, to deliver early (c section) or allow her to die in utero (due to the placenta not feeding her - it was equivalent to that of a 14 week at 20 weeks).

At the same time; for heart surgery to be successful, we were constantly told, she would need to be full term and a good weight. On top of this, apparently, given the genetic condition, cardiologists often refuse to operate due to the poor chance of recovery.

We received many answers from the geneticist, from this new test (and testing my DH and I) they could tell us that Laura's condition was not inherited (which also means our son and daughter will not be impacted by this) and if we decide to try again, we are back in with everyone else at our age when it comes to risk. One other answer was that this occurred at conception - so there is nothing I could have done to have caused this.

While yesterday I mourned the loss of my daughter, and the dreams that we had for extending our family; some of the guilt has been lifted, as realistically I know that had we elected to proceed; Laura would never have made it to term; not even close.

I hope my story is not over; we have decided to try again. We will still go through extensive testing (more for peace of mind; and my age than for medical reasons alone); but again with the support of the Fetal Management Unit (FMU) we have our plan mapped out.

I need to smile again and look forward with a positive focus. For me this is what I need to do, for Laura and our family.

Take care
Nicole
xoxo

[Amity]

Dear Nicole, thinking of you on the due date of your beautiful daughter Laura Grace. What a lovely name for an angel.

I am so glad that you have some answers about Laura's issues and it's great news that her birth and death have provided you with hope for the future.

Wishing you all the best.

[MistyFying]

Nicole thinking of you and remembering your precious daughter Laura Grace.

[Rommy'sMummy]

Nicole, have you been told that it is a palliative and not repair pathway? I would hate to think you had this thought from me and not your cardiologist. There is such a great difference in the outcome and quality of life; as you can see Mistyfying's son and mine have quite different stories.

If you have been told of the longer term needs/issues that can make a significant difference to your decision. Also, the best information they can give you is based on todays technology. So my sons life expectancy is too short (for me to be comfortable with) but is based on todays ability not on what medical sicience will be able to offer us in the next decade or two. We have stored our sons stem cells from birth to be used for him when the science has the abilty to grow another heart for him.

I am not pro-life and would not dream of trying to sway you either way. I just am trying to offer you a longer term view - such a hard thing to get a hold of when you are confronted with the imediacy of your babies need for surgery soon after birth and time in hospt etc.

The last few years have been incredibly hard for us and at the same time as we have experienced great and terrible sorrow and despair we have also experience overwhelming joy and thankfulness for things that may appear trivial to others.

Rachel

[Rommy'sMummy]

DOH! Posted at the same time as you Nicole.

Our DS has a Fontan!

[Berry1]

Nicole, I hope you are doing ok hun. Thinking of you

[Nicolabee]

Laura Grace was born at 2.14 on Monday 25th May 2009 - to early for this world.

She was perfect, tiny but perfect - but with a heart that could not sustain her life.

Thank you everyone for your support and the sharing of your stories. You are all very brave - much braver than I could ever be.

[Rommy'sMummy]

Nicole, I am so sorry for you loss. My heart goes out to you and your family. You are brave. Just know that I am thinking of you and sending you love.

Laura Grace is a beautiful name.

Rachel

[Tegam]

Laura Grace was born at 2.14 on Monday 25th May 2009 - to early for this world.

She was perfect, tiny but perfect - but with a heart that could not sustain her life.

Thank you everyone for your support and the sharing of your stories. You are all very brave - much braver than I could ever be.

Beautiful name for im sure a beautiful Girl! So sorry her little heart was not for this world...

YOU ARE BRAVE! I can not comprehend how you are feeling and what you have been through. I hope your DH and extended family are ok and that your other children give you strength at this time.

Wishing i could give you a real life hug or at least cook you dinner......