Real-life reads

Tubes keep our girl alive; Strug­gling fam­ily’s windfall; My mum fits through a ten­nis racket; Boy gone wild

Woman’s Day (NZ) - - This week -

Like many young girls, Ava-Grace Makasini is ob­sessed with Queen Elsa from the movie

Frozen and loves noth­ing more than danc­ing about in her flow­ing blue dress.

The gor­geous tod­dler – who lives in Auck­land’s Manukau City with her par­ents Helen and Kali Makasini, and big sis­ter Ariella – seems like any other three-year-old, brim­ming with en­ergy and chat­ter. But in Ava-Grace’s case, ap­pear­ances are de­ceiv­ing.

“Ava-Grace looks like a nor­mal lit­tle girl on the out­side,” ex­plains mum Helen. “But on the in­side, all her plumb­ing is wrong.”

Un­der­neath the satin skirts of her Elsa cos­tume is a small pouch that con­tains the four tubes Ava-Grace needs to sus­tain her life – they feed her nu­tri­ents, take away waste and pro­vide her with med­i­ca­tion.

The bright young girl has a rare and life-threat­en­ing con­di­tion known as mega­cys­tis mi­cro­colon in­testi­nal hy­poperi­stal­sis syn­drome (MMIHS). It’s a con­gen­i­tal de­fect that causes some in­ter­nal or­gans to form im­prop­erly – in Ava-Grace’s case, her blad­der, bowel and in­testines.

While its preva­lence is un­known, MMIHS has been doc­u­mented in med­i­cal his­tory around the world only 230 times and more than 70% of di­ag­nosed ba­bies are fe­male.

Since Ava-Grace was born, she has en­dured 20 ma­jor surg­eries, mostly to con­nect her stom­ach to her bow­els so she can process food and not rely on a feed­ing tube. Yet de­spite spend­ing more than half of her short life in hospi­tal, she con­tin­ues to defy the odds. More than 70% of chil­dren with MMIHS do not live long enough to cel­e­brate their first birth­day.

“Ava-Grace is strong al­right,” laughs dad Kali. “She’s king of the cas­tle around here.”

The first inkling Helen and Kali, both 27, had that some­thing was wrong was at their 20-week preg­nancy scan. A ra­dio­g­ra­pher no­ticed their baby’s blad­der wasn’t emp­ty­ing prop­erly.

“I re­mem­ber get­ting ‘that look’,” re­calls Helen. “They told us there was some­thing wrong and all I could ask was, ‘Is she go­ing to die?’”

The pair were re­ferred to a spe­cial­ist at Auck­land Hospi­tal and given a list of pos­si­bil­i­ties­pos­si­bil­i­tie – the worst of which was MMIHS. “We were told that if she had MMIHS, she’d prob­a­bly be still­born,” re­mem­bers Helen, watch­ing Ava-Grace run­ning out­side with seven-year-old Ariella and their cousins.

“We were of­fered the chance to ter­mi­nate, but we said no. By then, I could al­ready feel her mov­ing in­side of me.”

Crane driver Kali adds, “We knew life would be hard, but we weren’t go­ing to play God.”

Lit­tle bat­tler

From day one, Ava-Grace has proven the doc­tors wrong. De­spite her par­ents be­ing told she would likely not sur­vive birth, she came out bel­low­ing. “Ava-Grace is feisty and has a strong will – it is just the way she’s built,” says Helen.

Op­er­ated on just one day af­ter she was born, her par­ents were told once more to ex­pect the worst. Again, the re­silient girl ral­lied – and has con­tin­ued to fight ever since.

But while Ava-Grace’s health is sta­ble for now, the young girl’s fu­ture looks bleak. It is rare for kids with MMIHS to reach adult­hood and many die of or­gan fail­ure, in­fec­tions or mal­nour­ish­ment.

“We don’t think about death and we don’t talk about death,” in­sists Helen. “The doc­tors have men­tioned it, but my an­swer is al­ways the same: ‘No, I’m not go­ing to be­lieve that. Ava-Grace will pull through.’”

Helen and Kali have been told their girl’s best chance is a bowel trans­plant. With sup­port from the Im­mune De­fi­cien­cies Foun­da­tion of New Zealand/ Kids Foun­da­tion, the fam­ily is fundrais­ing on Givealit­tle to get her to The Royal Chil­dren’s Hospi­tal in Mel­bourne for an as­sess­ment.

While the idea of an or­gan

trans­plant fright­ens Helen and Kali, they say they’ll do what­ever it takes to see Ava-Grace grow up. Though their daugh­ter is be­lieved to be one of only two chil­dren in Aotearoa with MMIHS, there have been good re­sults over­seas fol­low­ing trans­plants.

Last year, a Texas tod­dler with the dis­or­der made med­i­cal his­tory af­ter suc­cess­fully re­ceiv­ing eight do­nated or­gans, in­clud­ing a liver, stom­ach, large and small bow­els, pan­creas, two kid­neys and a blad­der.

For the Auck­land fam­ily, it’s been a tough few years, but they’ve got through with the sup­port of their church and ex­tended fam­ily. “We have held on to our faith a lot since Ava-Grace was born,” as­serts Helen. “It brings us peace to know that what­ever hap­pens, she will be OK.”

And af­ter spend­ing so much time in hospi­tal, Helen is de­ter­mined to train as a nurse. “See­ing how hard life can be for so many kids, it amazes me how strong they are,” she says. “My dream now is to be a nurse. I have such a heart for all the kids out there who are strug­gling.”

As for their pre­cious AvaGrace, they’re want­ing to give her the best chance at life. “It has been a long jour­ney so far for us as a fam­ily,” re­veals Helen. “But Ava-Grace has de­fied the odds so far and we have to be­lieve she’s strong enough to keep go­ing.”

Dad Kali, mum Helen and big sis Ariella are keep­ing the faith for Ava-Grace. Ava-Grace’s blad­der, bowel and in­testines didn’t form prop­erly and she’s al­ready en­dured 20 ma­jor surg­eries. “On the in­side, all her plumb­ing is wrong,” says her mum.

Doc­tors said this wee Frozen fan wouldn’t sur­vive, but she won’t “Let it Go” and keeps on bat­tling!

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