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When Jo-Ann Caltabiano took her daughter Bianca to the doctors with a fever she had no idea it was going to be the biggest thing her girl would ever face.
Eighteen-year-old Bianca Caltabiano was in the city with her boyfriend when her mother received a call that changed their lives.
After getting blood tests earlier that day, the doctor called and said “Get Bianca to a hospital straight away, I think there is something very wrong with her liver”.
Bianca had a host of life threatening complications — an enlarged liver and spleen, severe anaemia and over 40 degree temperatures.
No one knew what it was or what to do about it.
After nearly a month of testing and no answers, Bianca was eventually diagnosed with secondary Hemophagocytic Lymphohistiocytosis. Otherwise known as HLH.
Bianca passed away six weeks after diagnosis on October 16, 2013 at the Royal Prince Alfred (RPA) Hospital, Sydney.
HLH is a rare blood disorder where the body’s reaction to infection or a virus such as glandular fever is to go into overdrive resulting in organ inflammation and damage.
According to emedicine, incidences of HLH have increased over time from 1.2 cases per million people, to one in 50,000.
HLH is a deadly disease that can affect people of all ages and ethnic groups.
Stephen Larson, a haematologist from RPA, explains that this disease can occur in anybody at anytime.
“There are no obvious predisposing factors where we can predict that someone is more at risk than another,” he told ninemsn.
“It can be anybody. Anybody can get HLH and it’s not something that you can prevent.”
Due to the fact that HLH is a rare disease, many health care providers are unfamiliar with its symptoms and it goes undiagnosed.
“There is a lot about HLH that we don’t understand,” Dr Larson said.
“There would be many doctors and GPs that know very little about it and because of the way it presents itself, it is very non-specific.
“You could have fevers and a low blood count, but there are a lot of things that can cause that, so unless it is thought of, it’s not going to be diagnosed early.”
If left undiagnosed the fatality rate of HLH is 100 per cent within two months.
If diagnosed early, patients are treated with drugs that help suppress the immune system.
If it persists then chemotherapy and/or a bone marrow transplant will take place.
“If it is thought of earlier and treatment started earlier I’m sure that survival would improve,” said Dr Larson.
“If it is only thought of when they are really unwell, then the survival is not going to be high.”
Mrs Caltabiano experienced this first hand, as she felt she was left in the dark for the majority of the time Bianca was sick.
“They tested for everything else, they did so many tests. Bianca was seen by a gastroenterologist, serologist, rheumatologist, immunologist and then a haematologist and no one thought it was a blood disorder,” she said.
“They just kept coming up with nothing.”
So after the first nine days in hospital they sent her home.
“The haematology team considered that a primary haematological disorder was unlikely,” her discharge papers read.
“They raised the possibility of a haemophagocytic syndrome but this was ultimately felt to be an unlikely diagnosis.”
A week after her first discharge when Bianca went back for blood tests and still had fevers it was then noted she was also severely anaemic.
“When her haemoglobin was really low and she needed blood transfusions, that’s when they seriously thought, maybe it could be HLH.”
Even after diagnosis, on Bianca’s second admission Mrs Caltabiano said they still had no idea what HLH entailed.
“At the time we were told, I was devastated,” Mrs Caltabiano said.
“We weren’t given much information, we weren’t really told much. We were told not to look at information online because it will just give you the worst case scenario.”
Bianca was sent home after two weeks when blood transfusions and immunosuppressive treatments appeared to be settling her HLH.
Her fevers stopped and blood counts improved.
For the next three weeks Bianca was seen as an outpatient and her immunosuppressant treatments continued and were increased as her fevers had returned and her blood counts were also getting worse.
This was until she was admitted for the third time.
Due to the lack of diagnosis and receiving little information, the family believed the doctors had it under control and just didn’t realise just how rapidly fatal this disease could be.
“She was amazing and stayed positive throughout. I remember she said ‘I’ll be okay, we’ll get through this, I can do it’,” Mrs Caltabiano said.
“I kept saying that it wasn’t fair, why you? And she would respond with, ‘why not me mum, I’m no more special than anyone else?”
Dr Larson said there is a lot to learn and education is the key for the future.
“There are centres especially in the States that are doing research in this area, but there is still so much that we don’t understand about it,” he said.
“I think awareness and education amongst doctors is what is most important, and this is starting to happen.”
Although Australia has a long way to go, Mrs Caltabiano urges people to start thinking about HLH and raise awareness.
“Doctors need to start questioning it and we need a lot more awareness. It’s one of those things where you think, how many times have you had a fever and thought nothing if it? We need to push for diagnosis and treatment earlier,” she said.
“I mean it’s already starting to happen. Since Bianca passed away I’ve heard of two more cases of HLH in Sydney and they were diagnosed straight away, and they have survived.”
Mrs Caltabiano, her son and husband along with close to 100 of Bianca’s family and friends have set up Team Bianca – Steve Waugh Foundation and are currently fundraising for the Steve Waugh Foundation to help raise funds and raise awareness of rare disorders and diseases.
She will be participating in the City2Surf with Team Bianca on August 10 in honour of her daughter.
Author: Danielle Robertson. Approving editor: Nick Pearson.
© ninemsn 2014