Last updated at 5:36 PM on 8th March 2011
Doctors say it is 'unheard of' - and now worry younger sister may have it too
When their baby daughter was diagnosed with a rare cancer-causing blood condition, Leona and Hugh Smith-Kerr were told their only hope was to find a bone marrow donor.
Doctors suggested a sibling for their daughter Merlyn would be the best prospect of curing her of Diamond Blackfan Anaemia, a blood condition that affects just a handful of people in Britain.
So when Mrs Smith-Kerr discovered she was pregnant and that the baby was a perfect match, the pair were overjoyed.
But tragically, in a case that doctors have described as ‘unheard of’, the family have now learned that their baby Freyja may also be a sufferer.
Leona and Hugh Smith-Kerr with their daughters Freyja and Merlyn. The family has moved to Leicester to be nearer a specialist hospital
Merlyn, now three, was diagnosed with DBA when she was just one years old. It is a condition that effects just 600 people worldwide.
causes the body not to produce enough red blood cells while at the same
time making sufferers susceptible to up to five types of cancer.
Merlyn was diagnosed with leukaemia - a cancer related to DBA - not long after her first birthday meaning she has to survive on a daily cocktail of medication.
Last month specialists at London's St Mary's Hospital told the couple from Herefordshire that Freyja could have DBA too.
far two tests have shown her rate of ADA - a measure of blood
production - being on average over 100, meaning she is positive for DBA.
She will have the results from a third and final test in August.
In this together: Doctors diagnosed Merlyn (left) with an extremely rare cancer-causing anaemia. Now they suspect Freyja (right) may have the condition as well
If confirmed, the diagnosis would mean a bone marrow transplant from Frejya, two, would be useless for Merlyn.
Doctors said either Hugh or Leona, 38, would be a carrier of the disease, but neither has tested positive leaving specialists baffled.
Mrs Smith-Kerr who married her husband last October, said: 'When we were told Freyja might have DBA too we couldn't believe it,
the doctor told us it was the first time he had heard of it without
either parent being a carrier.
'DBA sufferers are more susceptible to five types of cancer and they can get one or even all five at any time of life.'
She added that it was hard to make sure Merlyn led a normal life while battling two illnesses.
Merlyn has chemotherapy twice a day, morphine, paracetemol, antibiotics, vitamins and food substitutes through a feeding tube but she still managed to have a smile on her face. She is being treated at Leicester Royal Infirmary.
'Merlyn is such a brave little girl and she doesn't let all the medication and things get her down, she just wants to play with her sister,' Mrs Smith-Kerr said.
'When Merlyn was diagnosed with leukaemia it was the most treatable form, but she has still been in out of hospital regularly. In the 12 weeks before Christmas she had seven hospital stays due to infections from her lowered immune system.
'This destroys normal family life and puts our individual realtionships under a massive amount of stress.
'I think she is starting to realise there is something wrong, but most of the time she just gets on with it. It's what she thinks is normal.
'There was a moment when I was bathing Freyja and Merlyn asked 'Why hasn't she got a wiggly', meaning the Hickman line which the access point on her body for the drugs.
'But she just accepted it and said it must be only something older girls get.'
Sisters: A collect of Merlyn, who is receiving chemotherapy twice a week, with Freyja
She went on: 'Obviously we are worried about what they will tell us in August, we are hoping Freyja will prove not to have DBA.
'The thought of going through this journey again, knowing what's to come is terrifying. We don't want to see Frejya suffer the way Merlyn has.'
'But if she does we may have to find two donors.
'The dream is to have both Merlyn and Freyja happy and healthy so they can live normal lives.'
Pub landlord Hugh, 41, said the dream was to have both his little girls leading normal happy lives.
He said: 'Both Merlyn and Freyja are happy bubbly girls, they are so close and it's really great to see them together.
'The only thing that keeps us going is the girls' smiling faces when they wake in the morning.
'It has been hard since Merlyn was diagnosed and we have had to move to Leicester from Herefordshire so we could be close to a hospital that could treat her.'
'Leona is so strong as a mother and her parents have been a tower of strength throughout everything.
'She found out she was pregnant with Freyja before Merlyn's leukaemia was diagnosed. But when doctors suggested the baby might prove to be a match as a bone marrow donor - it gave us hope and confidence.'
He added that they didn't want to try and have a 'saviour sibling' as it didn't 'feel right'.
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The comments below have been moderated in advance.
...but neither parent tested positive as a carrier of this disease. If it really is a 1 in 10 million occurrence I suggest that one or both parents is/are indeed a carrier and the test itself is just not sophisticated enough. That being said, unlike breeding pedigree animals when tests like these are undertaken before offspring are created, humans have children first and only submit to tests if/when a problem is found. Who we choose as a partner and as the mother/father of our offspring is basically a lottery. Mostly things fall in our favour as there is enough diversity in the world population to seriously weaken the chance of hereditary disease. Unfortunately for some, this does not shield all from harm. I hope this family find the bone marrow donors they need.
- 7,200th Virgin, Sitting on a Cloud, 08/3/2011 15:28
I would quite happily be tested for compatability to see if I were a match - if the DM could provide contact details
- Liz, Surrey, UK, 08/3/2011 15:23
gibraltar is a small but a very generious place and most people would go on a bone marrow list - if there was one.
- me thinking, Europe, 08/3/2011 14:40
I had my first baby when I was 37 , then went on to have twins 14 months later, (39 years old), they were beatiful babies, extremely healthy, they a grown into lovely adults. No problems at all.
- D.Brown, Glastonbury, Somerset, 08/3/2011 13:26
Lula it is nothing to do with their age at all.please think before you comment on something you know nothing about at all!!!!.. i was 23 and had a daughter with DBA she is now 14, to find out you have a child with a rare illness is a smack in the face beleive me I know and to know the only cure is a risky bone marrow transplant where if it goes wong your child could die I could'nt bring myself to put our daughter through that risk.Neither myself or my ex husband have any other relative with DBA this was a huge thing to come to terms with and we still have monthly transfusions and various other problems due to this dam illness.Their lives are even harder due to the cancer problem, I hope and pray that they have some good luck cause they need it. If the goverment pumped money into rare disease research that would help but no they would rather give it to countries abroad, parentsof children DBA have to raise money for research themselves and thats hard work!!
- lgiles, mids, 08/3/2011 12:50
Meanwhile the human race wastes it's resources fighting stupid wars and sending crazy scientists in to orbit, It's time we got our priorities sorted and found cures for these awful diseases.
- Genbac, Shropshire, 08/3/2011 11:48
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