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Tragic case of dad with rare genetic disorder

Billy Agnew suffered from a rare genetic disorder which affected his lungs.
Billy Agnew suffered from a rare genetic disorder which affected his lungs.

Father-of-two Billy Agnew laughed and said he would see his family soon after he was taken into Ninewells Hospital but just 26 hours later his partner was taking the decision to turn off his life support machine.

The 46-year-old suffered from a rare genetic disorder which affected his lungs.

As he awaited a double lung transplant, he was regularly admitted to hospital for infections, and saw the antibiotic treatments as routine.

However, his organs unexpectedly began to shut down because of blood poisoning. Partner of 11 years Samantha Strachan is now waiting to discover whether their daughters have the same genetic condition.

She said: “I always expected him to come home. Even he didn’t know the seriousness of his condition. He laughed and said he would be home shortly.

“When he went into A&E they said he was going to be put in high dependency. Two hours later I was told he had developed septicemia and his body had gone into septic shock.

“We were told he had a very slim chance of pulling through and the best thing to do was to turn his life support machine off and let him go peacefully.”

Doctors discovered Billy, father to Sophie (6) and Kayla (5), suffered from a rare inherited condition called Alpha-1 antitrypsin deficiency.

A lack of protein made by the liver affected his lung capacity and limited his activity.

He had previously worked as a wood machinist at McTavish Ramsay.

“The illness had been a hindrance on his life and he couldn’t do the things he enjoyed,” Samantha said.

“He was devastated when he had to stop fishing because of his illness. He was a brilliant, loving and doting dad to the girls. His life was made complete when we had them.

“He said he wanted to take them fishing but he never got the chance. Sophie was over the moon at the thought of going fishing. I have to at least take them once now it’s something I’ve got to do because he loved it so much.”

Samantha said the strength of her two daughters, who go to Rowantree Primary School, has helped her get through the days following the tragedy.

She said: “The girls are really clever they knew their dad wasn’t well. And their teachers at school have been brilliant. They have been walking around with teddies that their dad gave them.

“I explained to them that daddy had died and it meant they would never see him again that was hard. They have broken down a couple of times but they have both been amazing. They have definitely been a big part of pulling me through.

“I will help the girls remember how he was before he was ill. It’s just a shame that he’s going to miss out on them growing up.”

Samantha now faces a wait to see if her daughters have the same genetic disorder as their dad.

Doctors have warned the girls will have to be checked for the Alpha-1 antitrypsin deficiency, which is passed on from generation to generation.

Although A-1AT deficiency is present from birth, the effects often do not become apparent until someone is older than 50.

Samatha added: “We were together for 11 years and got engaged on our first Christmas. Every time either of us went out the door there was always a kiss.”