HPLS - Left heart syndrome

[Nicolabee]

We went for an amnio at 16 weeks due to my age of 38. Our biggest concern was the risks associated with the amnio - so we thought.

nstead we walked out of the clinic with the probable diagnosis that our baby has Hypoplastic left heart syndrome or double outlet right ventricle or both! They can't usually diagnose at this early stage, so would suggest the severity of the condition.

We were advised to have an additional test done as a part of the amnio to rule out any genetic problems which sounded horrific, and the recommendation was termination.

We were told the best case was 3 open heart surgeries before our child is 2 and they probably wouldn't live past 6!

Thank goodness we have two healthy children who have kept our feet on the ground and made us realise how lucky we are, while we wait the two weeks till our 18 week ultrasound to determine a confirmed diagnosis.

We live in regional area with limited support services - and 3.5 - 4 hours from Melbourne.

My frustration was we were handed this "diagnosis" with no further information or access to support services for this 2 weeks. The major focus seemed to be on termination.

Has anyone else been in a similar situation? If I was to find this out later in pregnancy there would be no decisions and we would be going down the operation path to save our child. Instead we have been handed this at 16 weeks - and told we will have a decision to make.

As my husband says, if only someone would tell us what we have to do...

Instead we have another week to wait for the next ultrasound.

[MistyFying]

Nicolabee my heart goes out to you I was in the same situation about 6 years ago when my son was diagnosed with Left Heart Syndrome inutero. We were told to terminate, too, but we didn't. He did have two surgeries when he was born (for other congenital heart defects) and today is a healthy and happy boy.

I know how upsetting and awful it is to hear that diagnosis hope the next scan brings good news for you.

[Rommy'sMummy]

Nicolabee - Yes we have this too - actually our son has Hypoplastic Right Heart plus five other heart defects.

We found out at 20 weeks and were offered termination up to 24 weeks. He has had several heart operations - in Royal Childrens Melb. We are from Adelaide.

Our son is now 4.5 years old and still has issues with his heart - there is no "cure". If you want to chat here you can ask anything (I mean anything) and I will be honest with you. Or you are welcome to PM me if you would prefer.

My heart goes out to you. Is this your first baby? Our son was our first - I am now expecting our second and have just passed the 20 week u/s with a clean bill of health for this ones heart!! Yay!!

Thinking of you and your DH.

Rachel

[Nicolabee]

Hi Rachel

Thankyou for your willingness to share - I really do appreciate it - and understand that it is not easy.

Do you mind me asking if they diagnosed all the problems with your sons heart in utero or did you find out more when he was born?

In response to your question, we have two children, a son 6 (Grade 1) and a 3 year old daughter - they are currently fighting over whether it is a boy or a girl - they have no idea what is going on.

So, we have them to consider in whatever decision we make; and like you we do not live near Melbourne (rather a 4 hour drive)

I guess my big question is - did you at any time consider termination? What was the driving force behind your decision to continue your pregnancy? (a question I am sure that is hard to answer now when you look at your little boy and the joy he has no doubt brought you)

One other question - what is PM? I am new to the forum!

Nicole

[Rommy'sMummy]

Hi Nicole,

All but one of our son's heart defects were picked up in-utero. The only one that wasnt was not possible to see via a scan. We had the initial 20 week scan with a normal technician and then were refered to our major Children's hospital for a scan with a Pediatric Cardiologist within four days. That is what you need, not another technician but a Paed Cardiologist. Do you have private helath insurance?? If you do it means that you can have the same cardiologist and surgeon each time but if not you will still get the best person for the job - just not the same one each time. We went private and I know that helped me cope as we were able to build up very strong relationships with our care givers.

By the way RCH in Melb is the best hospital in Australasia for this surgery. People fly in from not only other states but overseas to go to RCH!

Once we had the (three hour!!) scan with our Paed Cardiologist (Dr Gavin Wheaton) he then spent the next hour talking us through what he had found, what it meant for the baby in terms of surgery and life expectancy and that yes we did need to think about termination. So my DH and I took a couple of days off work and just talked. I really wanted to at least talk about termination just to be sure we were both on the same page and being honest with each other - not trying to hide anything from the other to save an upset. So we considered it and our decision was based on the information gathered from Gavin (and there were phone calls to him as well). We decided to go ahead because while our son's life would start with surgery and need a couple in the first few years it was felt that from that point on he would have a physically limited but otherwise good quality of life. Albeit a shorter one than we had expected. So really it was about how much of an impact this would have on his life - would it mean constant pain or significant limitations or very short life etc.

All did not go to plan (sorry if this is distressing but I would have wanted someone to be honest with me) and our boy was small at birth (unrelated pregnancy complications - 3lb 10oz / 1.6kg birth wt) and his first trip to RCH resulted in not a great outcome with additional complications. That is the ONLY time I have second-guessed our decision about continuing with the preg. I was told that DS wasnt expected to live due to complications and at that point I thought that I had brought our son into the world only to go through surgery and complication and then to die. BUT he didnt.

He has had more surgery than any of us could have predicted and as I said in my previous post has not received a "cure" or in medical terms a "repair".

There are great resources available to you in Vic. Please go to the HeartKids Vic website and find out your local contact or ring the RCH and ask for Leanne Foster who is the head Social Worker attached to the cardiac ward - she often deals with families of children with HL/RHS. Also ask her for the book "There is no such thing as a silly question" it will help you a lot. You can tell her Roman's mum (from Adel) sent you!

PM means Private Message and is an icon next to my details at the top right of the panel with any message from me and I think you can also click on my name to do it??! Not good at that myself yet. Its like sending me an email instead of chatting in the public forums.

Rachel

[MistyFying]

Nicole a PM is a private message, but this function is not available until you've reached a minimum post count.

[Tegam]

will be thinking of you over the next two weeks.... So sorry there is no one to tell you what to do either. Hope your other two kids can give you some extra strength in the coming days.......

[Nicolabee]

Thank you everyone for your good wishes and explanations on how things work on the site.

It will be a long week, but until we know what are particular "issues" are there is little that we can do.



Rachel, the sharing of your experience is truly appreciated - and thank you for being so open and honest, we do need to know the good the bad and the ugly, so that we go into this with our eyes open. (Not that I expect you could ever be prepared)

I had no idea the scan would take 3 hours! I think I will phone the social worker you referred to and see what information they can offer us prior to our appointment.

MistyFying, you give me a glimmer of hope that perhaps we may walk away on Friday with a different result to our original scan? (However small) Do you have the all clear following your initial operations or do you face further down the track?

We do so treasure our two beautiful children, and count our blessings that they are happy and healthy.

[Nicolabee]

I thought I would return and update, given our appointment with the cardiologist yesterday - while we do not have HPLHS or DORV the prognosis is apparently much the same - it is either double inlet left ventricle or Hypolplastic tricuspoid valve and Hypoplastic right ventricle.

In all - this is a 1 in 20,000 event, while HPLHS is 1 in 5000. We initially thought that perhaps this is a better result - but the cardiologist tells us that this is not the case there is no differentiation between the two (HPLHS and this). Thought the risks with the first operation seem significantly reduced.

We spent a pretty solid day yesterday with Dr's obstetricians, Social Workers and the like finding out about our options in a fair bit of detail.

Basically we have 4 weeks maximum to decide if we are to proceed with the pregnancy or terminate. There is no easy answer.

My mind is going around in circles - next step is a Psycologist (I think that is it) on Tuesday who will help us work through the pros and cons of each option.

Nicole

[Berry1]

Hi Nicole,

I am so terribly sorry that you have found yourself in this situation, I sympathise with you. It sounds like you are taking all of the right steps to get as much information as possible. I have been in a very similar position.

At my 12-wk scan there was a very high nuchal fold, after doing a CVS we found out that there was a chromosome problem called mosacism. Instead of there being a 'trisomy' so a whole extra chromosome, there were extra 'fragments' of DNA in 70% of the cells, which geneticist's have told me is extremely rare (I also had an amnio to confirm this). Anyway, all of this resulted in a serious heart condition very similar to what your bub has. There were some other markers that were starting to appear like fluid around the brain and and obstructed bowel. She was also behind in development. No one could tell us if other problems would arise, as they couldn't tell what chromosome the fragments belonged to, but it wasn't looking good.

We decided to terminate at 19-wks based on the seriousness of the heart condition, the chromosome abnormality & the other problems, like the fluid on the brain.

There were times where I really lost it thinking I had done the wrong thing and what if the doctors got it wrong etc. The prelimanary results showed that the heart condition was what they expected, but worse. This gave me a little peace. It has been 7-mths since I lost her and it still really hurts but I feel my DH & I made the best decision for her and us, as hard as that is to say.

I don't wish to upset you as I know how distraught you would already be. I just wanted to be honest about my experience. It's encouraging to read some of the other ladies stories, and that there bubs are pulling through. Also, It doesn't sound like your bubs has a chromosome problem, so there is a big difference there. Big hugs to you as you and your DH make this difficult decision . If you have any questions at all please don't hesitate. All the best to you.

[Rommy'sMummy]

Nicole, I was wondering where you were up to with getting the next scan plus all the consults.

Good idea to see the Physc - my DH and I saw one together when we needed to make a decision around palliative or repair surgical options for our DS. We really benefited from someone helping us to get clear on the options and how we would come to a decision. My DH and I process info and decisions so differently that it was really helpful to talk about HOW the decision was to be made and not just WHAT decision was to be made.

If there is ANYTHING you need from me, more info, more quest, just anything; please let me know.

Its such a hard and confusing and conflicting time - I am thinking of you.

Rachel

[Tegam]

Thank you for letting us know how you are doing. Such a pity that the results couldnt have been better on friday.

Just keep in mind that we are all here for you! Whatever you need!

[Tegam]

There were times where I really lost it thinking I had done the wrong thing and what if the doctors got it wrong etc.

for you too hun, so sorry you have had to live through the last few months!

[Nicolabee]

Thank you Berry1 - I totally understand your comment "what if they got it wrong" - that is extremely frightening to me also (they assure me in our case this is not the case) - however I have read how things have been different at birth to that diagnosed during the early ultrasounds. Take comfort from the results, it sounds like they reaffirmed your decision.

I am totally torn at the moment - we have a tentative booking for a termination commencing Friday (the tablets) and induce labour on the Monday - we are under to obligation to proceed, but risk having to wait a week to 10 days after making a decision otherwise. (When I make up my mind, I need to do it "now")

We have gone through all the reasons why a termination makes sense for us - our two children at home while away with operations; what they would miss out on while caring for a child with additional needs (possibly special needs), our distance from Melbourne and specialty services (4 hours), our age, the risk to our unborn child at every turn - complications, infections - from the time it takes its first breath and that this is a palliative option - they will never be "fixed". I question whether I have the strength to watch my child go through so much - I look at my 6 year old and 3 year old and know how I would feel if something was to happen to them or if I were to lose them now.

BUT, then I look at our preliminary diagnosis - because the issue is now with the right ventricle, the risks associated with the first operation are 5% versus 25% with the left ventricle (installation of a stint - no heart bypass or reconstruction of aorta) - and that is if the operation is required. We then join HLHS babies with all the same risks and same operations and probable need for heart transplant. We have been told that there are usually no other associated problems with this abnormality, and have been cleared for genetic issues. Our baby is growing at the normal rate for 19 weeks gestation. Am I being selfish not giving our child a chance at life? Is it my right to take that away?

Had we gone into Friday, determined that we would do anything for this child, we would have been excited with the revised diagnosis.

I think I have a conflict of head and heart - but I can't yet make a decision with any real confidence (it changes daily).

DH is very rational and works through things logically - he is struggling with the choice, but sees termination as our best long term option - while I say that, dealing with a termination is another thing all together.

I am starting to question whether or not I will ever reach a point where I am at peace with my decision.

Thank you Rachel - I am hoping that speaking with the Psch will help us reach some conclusion with confidence.

Thank you everyone for your support.

[Tegam]

I am starting to question whether or not I will ever reach a point where I am at peace with my decision.

you are probably right.... to have a child with special needs (huge),to see what your other children have to miss out on because of this (huge), to add that extra stress to your marriage (huge), to have to live through an induction and a birth of a child that will 'look' perfect but know you made the decision to end the pregnancy (huge)...

All i know is either option will be hard on you and your family. There will be people that will not understand your decision no matter what you choose....

I know that there will be bad days, but i also know that you DO have the strength to get through this.... However much support you need, we are here for you!

[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