A FOUR-year-old schoolgirl diagnosed with such a rare medical problem she could die at any moment has become a medical phenomenon.
Little Harlee-Jae Procter has endured countless hospital visits and procedures as a result of a condition which means that part of a chromosome, which could contain vital genetic material, has been lost or deleted.
And as a result the brave youngster from Bacup, has issues including biliary atresia, which causes inflammation within the bile ducts, polycystic kidney disease, where fluid filled cysts develop in the kidneys, and nephrocalcinosis, which causes a build up of calcium in the kidneys that can cause devastating kidney failure.
Harlee-Jae’s mother Sasha Gill said her case, known as microdeletion 17 q12 syndrome, is ‘so rare’ doctors have written a paper about her.
The 25-year-old, of Anderson Close, Bacup,HarkH said: “We have been warned the worst can happen at any moment without warning.
“One of the main problems is it causes internal itching.
“She takes four different medications for it but it does not stop it from happening.
“Her skin is constantly full of open sores and it stops her from sleeping. She is up all night.
“I think that is probably the worst thing about it for her.”
Sasha added that Harlee-Jae has to take 15 different types of medication every day, 10 of which she takes three times a day.
She said: “She constantly gets an infection called cholangitis - an infection of the bile duct that can cause yellowing of the skin, fever, abdominal pain, low blood pressure and confusion - which makes her really sick.
“She has had that about four times and it is really hard to treat.
“She is also not meant to be near any children who are not immunised as she is really prone to things that other children might not be.
“Because of the microdeletion she is also very small for her age so people talk about her as though she is a baby and it stops her from doing age appropriate things.”
The mum-of-two said Harlee-Jae’s condition also had a knock-on effect for the rest of the family.
She said: “My older daughter Codiee-Lee often feels left out because of all the trips to hospital and things like that.
“We do not go on any days out because Harlee-Jae has really bad anxiety.
“This has been going on since birth but she deals with it really well and doesn’t think there is anything wrong with her.”
In a bid to create some precious memories for theThorn Primary School pupil the family have created a ‘bucket list’ of things she wants to do.
Sasha said: “People laugh at me because I am not sad about it.
“I just don’t want to sit here crying and let her condition define her life.
“I’ll do my crying when she is gone.”
One of the things Harlee-Jae wanted to do was to spend a day being a police officer.
And after her mum told officers at Rossendale police station of her wish, they organised for her and Codiee-Lee, six, to go on a police car ride, a tour of the police station, and take part in fingerprinting and handcuffing.
Sasha said: “After we were told that Harlee-Jae could have renal failure at any time, we decided to create a bit of a list of things that she wants to do.
“It’s really scary thinking that something could happen to her at any minute. We just want to make as many memories as we can.
“Rossendale police were fantastic, she had an amazing day and hasn’t stopped talking about it since.
“We had been hoping to see the police horses but unfortunately we couldn’t, so the police are arranging another day where she can go to the Mounted Horse Branch.”
Insp Paul Lee said: “When we heard Harlee-Jae’s story we were all extremely touched and wanted to help her make her dream come true.
“We are so pleased that she enjoyed the day with us.”
Harlee-Jae was diagnosed with biliary atresia at four-weeks-old after suffering severe jaundice.
She was taken to Leeds General Infirmary where she underwent an operation called Kasai Procedure to allow bile to drain from the liver to the gut.
The operation has improved her liver function though doctors have said that her condition could deteriorate, which may mean she would need a liver transplant.
Since then she has been treated at Birmingham Children’s Hospital, Manchester Children’s Hospital and Oldham Royal Hospital.
Sasha said: “In August we were taken to PortAventura in Spain by the Make a Wish Foundation to meet Elmo from Sesame Street. She had a fantastic time and even got to go in the cockpit of the plane on the flight over.
“She would love to go Ice Skating with Elsa from Frozen and she would also love to meet Danny Ings and Bertie Bee.”
Sasha is now appealing for people to join the organ donor register.
“Not many people realise that you can be a living organ donor, she said. “As the liver regenerates it is possible to have 10 per cent removed for transplantation.”
Comments: Our rules
We want our comments to be a lively and valuable part of our community - a place where readers can debate and engage with the most important local issues. The ability to comment on our stories is a privilege, not a right, however, and that privilege may be withdrawn if it is abused or misused.
Please report any comments that break our rules.
Read the rules hereLast Updated:
Report this comment Cancel