A Cross to Bear – the tragedy of Fragile X syndrome

Review: Vivien Horler

The Trouble with my Aunt, by Hedi Lampert (Porcupine Press)

Aunty Vi is Granny’s cross to bear, a metaphor that 10-year-old Leah doesn’t entirely understand, but she gets the gist.

Being at a Jewish day school she doesn’t pick up the Christian reference, and imagines the cross as a large X of the sort Leah gets in class if she’s made a spelling mistake. If Aunty Vi is Granny’s cross to bear, then Granny must have made a mistake.

And so it turns out. Aunty Vi is strange – what in 1971 we might have called retarded or even simple. This suited Leah as a very little girl, as Aunty Vi was on her wavelength, but as Leah  grows older she sees that her aunt is not like other people.

One day Amy, Leah’s mother, confides that her mother, Sadie, became pregnant again when Amy was just nine months old and Sadie herself was only 20. She tried to abort the baby, and irreparably damaged her. Hence Sadie’s cross.

More than 20 years later Leah is 32, assistant MD of an events company and doing very well by everyone’s accounts except Sadie’s, who thinks it’s time Leah found a nice Jewish boy and settled down. Leah flies from Joburg to Durban to start organising a boat show, and meets Steve, who designs yacht interiors.

Steve is not Jewish, but he is impulsive, charming and extremely attractive. Leah arranges a second trip to Durban, arriving on a Friday so that she has the entire weekend free. As she had hoped, she and Steve end up in bed, but afterwards he tells her he can’t see her over the weekend  as he’s “pretty tied up”.

Upset, Leah flies home without a further word from Steve. A few weeks later her breasts feel tender, and a test confirms she is pregnant. In a tizz she rings Steve, only to have him propose to her. He’s about to turn 40, he tells her, and would love to be a dad.

All of which plunges Leah into further turmoil. And then there’s Aunty Vi to think of. Is her condition really the result of an abortion gone wrong, or could it be caused by something that Leah’s baby could inherit?

Various tests including an amniocentesis reveals all is well with the baby. Then, when Leah is around 20 weeks pregnant, her cousin Julie gets in touch. Julie’s youngest son, Nathan, has always been odd, and the family has spent a fortune trying to figure out exactly what is wrong with him. It emerges Nathan has Fragile X syndrome – now known to be one of the most common inherited genetic causes of mental retardation. Julie says she believes Vi has Fragile X. Julie has discovered she is a carrier, and Leah might be one too.

Anyone who has been pregnant knows the fears that go with it. Will the baby stick, will it be all right – and you don’t even want to think of possible inherited problems. This may not be a book to read if you are pregnant or thinking of having a baby, and yet there is a great deal of detail about the joys and trials of the experience of pregnancy and childbirth.

Hedy Lampert’s writing is attractive and she produces some delightful touches of dialogue.  When Leah discovers she’s pregant she tells her best friend she doesn’t want Steve to think she’s trapped him. “Come on,” replies the friend. “He’s a 50% investor. You didn’t get this way through wind pollination.”

In an author’s note Lampert says Vi was based on her own aunt, born in 1933 and who never got much further than Standard One at school, although her mother persisted in trying to teach her to read and write.

In the 1980s the family became aware of Fragile X syndrome, and Lampert’s grandmother, mother, aunt and Lampert herself were all tested. The grandmother was a carrier, the aunt had the mutation, while Lampert and her mother were negative. It has since emerged that girls, who have two X chromosomes, tend not to be as badly affected as boys, with just one X chromosome.

There are a couple of gripes – in 1971 no South African school pupils were in Grade 5 – that system was to be introduced only after 1994. Seeing the heroine was 10, one assumes Lampert meant Standard Three. Then there’s a reference to a registry office wedding, and a justice of the peace, neither of which we have in South Africa.

But this is an engaging and readable novel. And one must be grateful, when one thinks of all the things that could go wrong with the conception and birth of a baby,  that most are born just fine.


2 thoughts on “A Cross to Bear – the tragedy of Fragile X syndrome

    1. Hedi Lampert

      Thanks for this review Vivien.
      I thought long and hard about using the standard three terminology, but decided I wanted to make it accessible to readers globally, hence the use of the Grade system.


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