Family left devastated as twins diagnosed with rare blood condition
SISTER WAS SET FOR TRANSPLANT FROM SIBLING BEFORE HEARTBREAKING NEWS
TAKING a tumble is just part of life when you’re a two-year-old.
But for Pixie-Rose Sellars – who is inseparable from her twin sister Presley-Pearl – even the slightest bump can result in nasty bruising, days of pain and even internal bleeding.
It was the blue marks on Pixie’s body when she was eight months old that first alerted her parents Johanna and Kevin that something wasn’t right.
After months of testing, Pixie was finally diagnosed with an extremely rare blood disorder affecting the bone marrow. It is a disease suffered by just a handful of children worldwide.
When her condition deteriorated, and Pixie’s twin Presley was the only bone marrow match in the family, their parents made the difficult decision for a twin-to-twin transplant.
But further testing revealed more devastating news – Presley also had the condition.
The twins were born in January 2016, but in September that year, Johanna noticed Pixie was bruising more than her twin. A visit to the doctor offered reassurance – he said newly-mobile toddlers were prone to knocks and bangs.
Johanna put the worry to the back of her mind – until Christmas Eve 2016.
“I was getting Pixie ready for bed when I noticed head-to-toe bruising – even my finger prints on her back left a mark.”
Taking Pixie to A&E for blood tests, Johanna, 30, a support worker, and Kevin, 35, a PE teacher in Prestwich, were told Pixie had a low count and she was referred to North Manchester General with suspected meningitis.
But when further tests brought medics no closer to an answer, they referred her to Manchester Children’s Hospital where they diagnosed an autoimmune condition and prescribed steroid treatment. But she didn’t improve.
Johanna, said: “In April 2017 they finally did a bone marrow biopsy to see why she wasn’t producing her own red blood cells. The result was grade three fibrosis of the bone marrow.”
Pixie had been diagnosed with Myelofibrosis – a rare bone marrow disorder that disrupts the body’s normal production of blood cells and is mostly seen in elderly patients.
The condition means her ‘soft’ bone marrow has been replaced by hard fibrous tissue. It affects the production of red blood cells, which hinders clotting. That’s why Pixie bruises so easily.
Johanna added: “We have to be very aware of any knocks or bumps and be careful of internal bleeding.
“At 18 months she fell and cut her head and had to be admitted to hospital for two days to stop the bleeding.
“As a mum it’s horrible seeing her pain and not being able to do anything about it. She often has to spend an evening in the bath because that’s the only place she’s comfortable.”
In October last year, doctors decided Pixie wasn’t going to get better – and the family decided a transplant would be the best course of action.
But of Pixie’s four siblings, only her twin Presley was a tissue match – and the chance of success was higher than with a stranger donor. During pre-transplant checks, medics tested Presley for myelofibrosis as a precaution.
Johanna added: “She had no symptoms - no pain, no bleeding, no night sweats, and her blood counts were normal. But she tested positive.
“We were devastated. If anything it hit us harder because it was so out of the blue. We were a few weeks from the transplant date and we’d fallen at the last hurdle. At some point Presley’s condition will also progress, she will have to be monitored and she’ll need a transplant too.”
In the meantime, a stranger donor has been found for Pixie by the Anthony Nolan blood cancer charity and today she will be admitted to the Manchester Children’s Hospital for chemotherapy before the bone marrow transplant in two weeks’ time.
After that, it’s about waiting and, supported by blood and platelet transfusions, Pixie faces a battle for her health.
It’s hoped she will then produce normal bone marrow and her symptoms will improve.