A whopping big can of worms

Yesterday a friend on Twitter re-tweeted a post from the UK Telegraph about ‘the world’s smallest mum’ having had her third child and not ruling out having a fourth.

Curious about just how you get the moniker ‘world’s smallest mum’ I checked it out and have not been able to shake the story out of my head since.

Stacey Herald is 36 and has a rare genetic condition called Osteogenisis Imperfecta, which according to the article stunts her growth and causes underdeveloped lungs and brittle bones.  

From my very cursory research this condition is what we commonly hear referred to as brittle bone disease.

According to the Osteogenisis Imperfecta Foundation people with this condition:

  • In addition to fractures people with OI often have muscle weakness, hearing loss, fatigue, joint laxity, curved bones, scoliosis, blue sclerae, dentinogenesis imperfecta (brittle teeth), and short stature. Restrictive pulmonary disease occurs in more severely affected people.
  • OI is caused by an error called a mutation on a gene that affects the body’s production of the collagen found in bones, and other tissues. It is not caused by too little calcium or poor nutrition.
  • OI is variable with 8 different types described in medical literature.
  • The types range in severity from a lethal form to a milder form with few visible symptoms.
  • The specific medical problems a person will encounter will depend on the degree of severity.
  • A person with mild OI may experience a few fractures while those with the severe forms may have hundreds in a lifetime.
It goes on to say that the characteristics of the disease vary from person to person – which is to be expected in any genetic condition as we all bring our own genetic material to the party. The key health issues commonly seen in people with OI include:
  • Short stature
  • Weak tissues, fragile skin, muscle weakness, and loose joints
  • Bleeding, easy bruising, frequent nosebleeds and in a small number of people heavy bleeding from injuries
  • Hearing loss may begin in childhood and affects approximately 50% of adults
  • Breathing problems, higher incidence of asthma plus risk for other lung problems
  • Curvature of the spine
The website points out that childhood and adolescence are particularly difficult for people with OI but that most go on to lead “productive and successful lives”. 
So, with all that said if you had this condition, would you a) try to have a baby when doctors advised you against it and b) there was the chance your child would inherit the condition? 
My answer to a) would be probably as I know how strong the desire to have your own child can be. 
But my answer to b) is absolutely not. If it was possible to undergo IVF procedures which ensured the embryo did not carry the mutation then I may barrel on down that ethical minefield of genetic selection. But would I have a baby knowing it was pretty darn likely they would inherit what is clearly a painful and debilitating (there are different types of OI with varying levels of severity) condition? Absolutely not. 
 
Hear that kebang? That was the lid popping off and those darn worms heading in every different direction.
Of Ms Herald’s three children, two have inherited OI. Her second child does not have the condition and at age two is already taller than her mother. Ms Herald says in the article: “I can’t physically stop Makya if she’s upset,” said Mrs Herald. “She’s too big for me now, so Wil has to take care of that.”
Here’s hoping Makya only gets upset when her dad is home from work. 
It is so not my place to stand in judgement on this family but man is it wreaking havoc with my brain. 
Who am I to say she should not have had children. I mean, plenty would stand and look at my history of depression and anxiety and point an accusatory finger that I should not have had children for fear of passing on any genetic predisposition to these conditions. 
I mean, where do you draw the line? 
If say, a permit to procreate came into affect which meant we all had too undergo genetic testing in order to be allowed to have a child then I suspect very few of us would ever become parents. 
Oscar’s genetic disorder is a rare, freakish result of chromosome four getting carried away way back when he was just a moosh of cells. No hereditary predisposition. No dodgy chromosome from mum or dad. Just ‘one of those things’. 
Had I undergone genetic testing with that pregnancy and discovered something was wrong would I have terminated? Yes. Why? At the time, at the tender age of 24 it probably would have been because I didn’t think I would be able to handle it. At the wise age of 37 and now far more experienced in the world that is having a disability it would have been because I did not want my child to have to suffer all that comes with having a dodgy chromosome. When I see how hard the world is for Oscar …
Now look – many will argue that the idea of not wanting our kids to suffer is universal and undergoing trials and tribulations, tragedies and all the rest are what make us stronger and better people. Absolutely. 
But would you knowingly inflict it on your child? And rather than those hurts and hardships come from life experience have it stem from part of the very fibre of their being? For me the answer is a resounding no. 
I keep looking at the photo of this family and there are two children there who are going to endure endless painful procedures and myriad health issues for the rest of their lives. Then there is another child who by the miracle that is genetics dodged the bullet. I can barely imagine the ‘issues’ that child may have down the track. And for what? Because this couple wanted a family? 
It just doesn’t feel right. 
Your thoughts?
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  • BabelBabe

    what's wrong with adoption? I think it's pure selfishness to have a child knowing you can pass on such a disease. there, i can be all the judgmental for you.

  • BabelBabe

    i also think it's pure selfishness to have a child you haven't got a prayer of parenting properly. what, the kid only misbehaves when the husband is home?

    see FB post of 66yo old woman giving birth to IVF triplets. yeah, I am The Judge.

  • Three Owls

    hmm… trying to gather my thoughts into a concise sentence… I agree with most of your points, and I don't know how it would be to walk in that woman's shoes… it does seem really selfish and it brings up all sorts of issues I have with the medical establishment… that said, I am super healthy and so are my kids so I have a very biased opinion that allows me to sit on a high horse… meanwhile my taoist sensibilities force me to try and be compassionate and accepting…

    in an effort to stop babbling on I am going to go have a cuppa and contemplate the state of the world…

    bravo on another thought provoking post.

  • Paola

    BabelBabe couldn't have expressed my thoughts better.
    "My name is Paola and I am judgemental."

  • peskypixies

    a NO
    b NO

    stupid and selfish!

    yes I am also judgemental

  • Reemski

    Being a carrier of a serious genetic disorder, the thought of knowingly passing it on is terrifying. But I also understand the drive to have children. How do you reconcile it? I don't know. To each their own. But Kim I'm with you. My partner will be tested to see if he has the "matching gene" and we'll make a judgment call together when and if it's necessary.

  • Frogdancer

    I used to breed dogs for years. It's left me with a very practical viewpoint, as we try so hard NOT to accentuate genetic abnormalities.

    It's just not right. It's pure selfishness.

  • sooz

    oh it is a mire alright. I know someone with progressive, degenerative MS. At 38 and single she decided to conceive a child because of her drive to mother. I find it astonishing and confronting, and difficult to understand. I can't say what I would do in her place, I really can't but, wow it messed with my head for a good long while.

  • Ms Brown Mouse

    My thoughts, pretty much exactly the same as yours, well put.

  • Corrie

    no I couldn't have children knowing they would have such a serious condition. I just couldn't do that, I would much rather adopt children.

    I really feel sorry for those children and the struggles they will face later on.

    Corrie:)

  • kim at allconsuming

    Look, if I really just laid my reflex reaction out there it'd be somewhere in the vicinity of 'whatabunchofcrazygodbotheringselfishcunts'.

    And dudes, while that would get some awesome blog traffic I just know it's all a little more complicated than that. Just.

    I think Reemski nailed it. She knows she's the carrier of a genetic condition so when the time comes her husband will also be checked and they will make an educated informed decision.

    It strikes me that there has been little education and a selective informed decision being made here which probably involves a lot of prayer and that every child is a miracle.

    Personally, if I had to watch my child undergo one painful procedure because of a decision I had made? well, I'd be retiring to the library with a revolver toot sweet.

  • Carol

    Fabulous topic, Kim! And what a shit of a situation to have to deal with. I remember reading an interview quite some years ago with a profoundly deaf couple who had a child who inherited the deafness. Their decision was to deny the child a cochlear implant. To me, that is tantamount to child abuse. To them, there isn't anything wrong with being deaf, they live their lives fully, so why should they put their child through surgery.

    I have two gorgeous sons, Mr 8 and Mr 7. Mr 7 was born with a potentially fatal congenital heart condition – transposition of the great arteries. We are lucky in that his heart has been successfully and fully repaired, although he will need to be checked for life. Plenty of kids aren't that lucky – 1 in 100 children is born with a CHD!

    As little as 10 – 20 years ago, children born with this defect died, or lived shorter lives, or had palliative surgical repairs. And that's in rich western countries. Kids born with this in poor countries or countries without health care still die.

    We had wanted a third child, but I couldn't bring myself to do it. "What if we had a child that COULDN'T BE FIXED?" The decisions that other people make are their own and I won't discuss the rights or wrongs of it, but for me it would have been a life sentence for all of us, including the two children I already have. So no third child for us.

    I remember, though, that two people asked particularly hurtful questions after he was born. My mother-in-law asked, "So, who else in your family has heart problems?", the insinuation being it was ALL MY FAULT. As a matter of fact there have been NO other CHDs in my family. She, however, wouldn't know as she never got to meet her own father (WWII). The other was a complete stranger who asked, "So what do you think you did wrong for it to happen?"

    I'm all for screening and selecting if it means a child, a family, doesn't have to suffer.

    But that's just me.

  • Jodie at Mummy Mayhem

    Aaaah, Carol – I so hear you on the hurtful questions! I've been known to call my MIL a "genetic snob" (not to her face – what am I, stupid?), even though, her family is not without genetic problems also. In fact, which family *doesn't* have *something* that could potentially be passed on?

    Of course, a condition that is life-threatening or could mean a very, very difficult life for your child – and importantly – one that could not be 'fixed'…I just don't think I could make that decision to go ahead and have children. It would be a difficult decision to make and you make a great point, Kim, about the strong desire to have a child possibly playing a huge part in that decision-making process – but it would be selfish in my opinion.

    Fabulous, thought-provoking post, Kim.

  • Anonymous

    I have OI and had a conversation about this very topic last week with someone else who has a genetic disease. My OI is a 'spontaneous mutation', which means my mother wasn't in the position that I will be in if I decide to have kids one day.

    I am glad that the IVF option exists and that would be the most 'ideal' option in a difficult situation. But one of the main points of the conversation I had last week was – if you decide not to have kids (ie roll the dice), isn't it a bit like wishing you weren't born?

    I think unless you've lived the life, it's really difficult to have an informed opinion on this. And, even when you have lived the life, it's still an insanely difficult idea to consider…

  • kim at allconsuming

    Oh Carol – I remember that story about the deaf couple and I think it encapsulates some of the issues around this perfectly.

    For all we know Stacey Herald may see her situation as the making of her, that is has made her strong not weak, that each and every broken bone and respiratory and other issue she has lived due to her condition has been a blessing of some sort.

    But I don't know, there's a niggling cynic in me that she's had children as a 'we'll show them' snub at medical professionals. And well, there's just nothing redeeming in that.

    I think the thing that undoes me is trying to find or determine where that line is. For us it was having thorough genetic testing before each subsequent child and having very clear agreement between me and Chef that if there was anything, anything out of the ordinary we would terminate (and suffer the consequences emotionally).

    But what if there was a history of alcoholism in your family? Or mental illness? Or breast cancer? Or you knew you carried the gene which causes a disease that comes into affect in your late 40s?

    I know the answer to that is different for everyone and maybe this is a bit more in-your-face that most cases. But what quality of life are these kids going to have?

  • kim at allconsuming

    oh Anonymous – thank you SO MUCH for commenting.

    And no, I certainly would never ever say that making the decision not to have kids because they may inherit a condition you live with each and every day is like saying you never wished you'd been born. Absolutely not.

    One of my friends was sexually abused as a child and made the decision not to have children for a number of key reasons. One of those is the inescapable statistic that if you have been abused it is highly likely your children will be (I know I'm expressing that badly, my apologies) even though in her situation the abuser is long long gone. The other is her own emotional fragility when it comes to protecting those she loves and how raw any slight is no matter how un-intended it may be. She has made the decision FOR HER in complete and frank honesty with her partner that she could not bring a child into this world.

    Now I think she and her partner would make awesome parents but it is so no my decision to make is it.

    As for you – the decision comes down to that of you and your partner and yes, it is an insanely difficult decision to make.

  • Anonymous

    Kim, no problem re: me posting (it's weird being anonymous, but its a very personal topic and I'd rather comment anonymously than not at all).

    It's such a hard decision for so many people for so many reasons. I know someone who has made the same decision as your friend and although they don't admit it, I am sure for the same reasons. Definitely no one's choice but theirs…

    You know one thing the kids in this story have on their side? A mother who knows what they face.

    Ok, so she can't wrangle her two year old, but ALL parents have limitations (physical, emotional, etc). In this case, they're physically obvious, and therefore easy for people to spot and criticise, but that doesn't mean that Stacey can't be a brilliant mother on all other accounts.

    Treatment for OI has improved immensely in the last few years and I know that had I had the treatments that are around now when I was a child, things would be much different in regards to my physical state now. There's a lot that can be done to make these kids' lives good!

  • Janet

    Well, as someone who did make the decision to have a late termination due to a genetic condition that could have caused our son to have a mild disability or quite a severe one, I don't think it can ever be an easy decision. It seemed like the only decision we could make at the time and honestly, in the same situation we would probably make the same decision again. For the same reasons – not only might the child face pain and suffering, but who would look after him/her when we were gone? Particularly as older parents – that was one that got us. But then again, after the grief had subsided a bit, to make that decision still seemed a bit wrong. As if we were messing with something bigger than us – a reaction I never expected to have.

    But even so, I wouldn't knowingly go out bring children into the world with a lifetime of pain ahead of them. It seems cruel and selfish.

  • @c_oreilly

    I'm so glad I found your blog, and this post certainly resonates. Before I even had kids I was a little worried about one of the problems in my family – but there was no evidence that it could be inherited. Then my second child was very premature and diagnosed with cerebral palsy. So perhaps I was selfish to go on and have high-risk third pregnancy? Who knows. I went full term, but just 12 months ago my son was diagnosed with Asperger's. Both these conditions came out of nowhere. But I do have a friend who chose not to have children due to her inherited condition. The urge to have children is so strong, it's hard to condemn all parents, especially if there is a good chance that there children may escape the inherited disorder.

  • Christina

    I too am judgmental about this issue – I don't think I could have a baby if I knew it would most likely inherit this condition. But then maybe I would. I don't know. But I think these parents have so much courage, and must have such faith that they can help their children overcome the difficulties of living with this condition, that I can't help but respect them for that.
    Thank you for your comment, you left it ages ago but I only just looked my blog (such a dedicated blogger am I!)

  • Elizabeth

    I am very judgemental. I would never even contemplate having children in this situation. But then I wouldn’t have had kids if I couldn’t provide for them in certain ways (not only financially but a list of totally arbitrary control freak ways that matter to me). I feel very responsible for my children so I don’t get the "its in God’s hands" (and yes I do think lots of things are in his hands) when it comes to raising kids.
    Great post thanks for sharing.

  • reality raver

    I think having children is not a right it is a responsibility. I cannot work out why she did not have genetic testing? Was she actually capable of looking after those kids because of her disability?

    I like your honesty.