‘Smiley, happy’ baby dies of rare disease just months after birth

Ava Louise was diagnosed with the rare genetic disorder Alagille Syndrome after being born prematurely and had to undergo a liver transplant before her kidneys failed

A family has paid tribute to their “happiest, smiling, sassiest baby” after she died of a rare disease when she was just nine months old.

Ava Louise was born prematurely at just 34 weeks without her mother Hannah realizing she was pregnant.

Assuming she had problems with her appendix, Hannah was taken to the emergency room by her mother, but went into labor when she went to the bathroom.

Ava was born with yellow skin due to a premature birth and was placed in an incubator before being diagnosed with Alagille Syndrome.

The genetic disorder affects around 1 in 100,000 children from birth and can affect many different parts of the body, including the liver, heart, eyes, face, skeleton, blood vessels and kidneys.

Those born with this condition can have a severe combination of symptoms.

It is a very difficult disease to treat and in Ava’s case it affected her liver.

Doctors informed the family that Ava needed a transplant, but because of her small size—she weighed about the same as a bag of sugar—they had to wait to have the surgery performed.

The whole family volunteered for tests to see who would be suitable for the transplant.

Hannah ended up being a match and after the surgery, doctors found that her liver function had returned.

Unfortunately, Ava then developed problems with her kidneys.

Ava’s aunt Samantha Brittain spoke to LeedsLive about Ava’s journey.

Samantha said: “She was with us over Christmas and then her journey began on December 29 when she was transferred to Leeds Children’s Hospital.

“She had numerous tests, blood transfusions, surgeries and was tube fed.

“Everyone suggested their names for the transplant and Hannah was a match.

“They both went for the transplant in March and in general the transplant worked and after the tests they saw the liver was working but the doctor said the kidneys should start working again.

“But in this case it wasn’t like that, her kidney failed.

The morning after the transplant, the family experienced their first moment of shock.

Ava went into cardiac arrest and it took four minutes before she was successfully resuscitated.

Unfortunately, after a third cardiac arrest, Ava passed away on June 1st of kidney failure and sepsis.

Samantha added, “It was very shocking to all of us, for everything she’s been through she’s been very strong and very happy.”

“She was always smiling no matter what she was going through, we will all miss her very, very much.

“We couldn’t spend a lot of time with her because we were in the hospital but it was appreciated, the nurses were remarkable, all the nurses gave great praise to Ava and I’m not sure this ward won’t be the same without her.

“The hospital was brilliant, they couldn’t have done more than what they did.

“She was the happiest, smiling and cheekiest baby, despite everything she was going through. We went down as a family to see her and we had lots of pictures with her, she used to have a red rattle and she loved it.

“It’s something I will remember forever.”