A brother and sister with cystic fibrosis could die if they share toys due to the risk of infection, their parents have revealed.
Sienna Woods, two, and Noah Woods, one, are unable to kiss each other – but the adorable siblings are difficult to keep apart.
Their mother Shelby Woods, 25, from Burscough, Lancashire, has to rigorously clean everything with disinfectant and can’t allow her children to play in sandpits.
Cystic fibrosis (CF) sufferers are a danger to each other due to cross-contamination of bugs they grow in their own lungs.
The incurable condition affects the internal organs, especially the lungs and digestive system, clogging them with thick, sticky mucus.
Sienna Woods, two, and Noah Woods, one, who both have cystic fibrosis, are unable to share toys or kiss each other due to the dangers of cross-contamination
![Mother Shelby Woods, 25, from Burscough, Lancashire, has to rigorously clean everything](https://i.dailymail.co.uk/1s/2019/03/14/09/10972736-6808039-image-a-2_1552557119035.jpg)
Mother Shelby Woods, 25, from Burscough, Lancashire, has to rigorously clean everything
![Sienna and Noah were each diagnosed with CF shortly after they were born](https://i.dailymail.co.uk/1s/2019/03/14/09/10972734-6808039-image-a-3_1552557124137.jpg)
Sienna and Noah were each diagnosed with CF shortly after they were born
Ms Woods said: ‘It’s so difficult to stop Sienna and Noah being together. It makes me sad that they can’t be so affectionate with each other.
‘We allow them to hug but they’re not allowed to kiss because of the cross-infection risk. There’s a possibility they won’t get be able to fight it and could actually die.
‘If Sienna is playing with a toy we have to make sure Noah doesn’t pick it up and disinfect it before he does.
Sienna and Noah were each diagnosed with CF – a debilitating, life-shortening condition – shortly after they were born.
More than 10,000 people in the UK have it and there’s currently no cure. Half of the people in the UK with it will die before they’re 31.
Receptionist Ms Woods said they were shocked when they found out Sienna had CF three weeks after she was born, having been unaware there were any carriers in her family.
![Ms Woods said if one of the children is playing with a toy, it must be disinfected before the other child picks it up. The children pictured at home](https://i.dailymail.co.uk/1s/2019/03/14/10/10973600-6808039-image-a-4_1552558639410.jpg)
Ms Woods said if one of the children is playing with a toy, it must be disinfected before the other child picks it up. The children pictured at home
![People with cystic fibrosis (CF) are normally kept apart due to the danger of cross-contamination of bugs they grow in their own lungs](https://i.dailymail.co.uk/1s/2019/03/14/10/10973610-6808039-image-a-5_1552558643760.jpg)
People with cystic fibrosis (CF) are normally kept apart due to the danger of cross-contamination of bugs they grow in their own lungs
![Sienna was diagnosed with CF three weeks after she was born](https://i.dailymail.co.uk/1s/2019/03/14/10/10973604-6808039-image-m-18_1552558911257.jpg)
![Noah had a 25 per cent chance of also having the condition, Ms Woods said](https://i.dailymail.co.uk/1s/2019/03/14/10/10973592-6808039-image-a-19_1552558914201.jpg)
CF affects the internal organs, especially the lungs and digestive system, clogging them with thick, sticky mucus
They were told Noah had a 25 per cent chance of having it – before discovering he, too, did have the condition.
CF patients grow different bacteria in their lungs which are usually harmless to people who don’t have CF, though they can be harmful to those who do.
Although coping well on a daily basis, the problems occur when the children get sick – struggling with wet coughs that bring up mucus.
They have to take around eight different medicines a day and make regular visits to the hospital as well as having daily physiotherapy.
Ms Woods said: ‘They have separate branded drinks bottles, separate cutlery and bowls when they eat.
‘I have to watch them constantly. They’re not allowed to play in sandpits or jump in puddles because of the risk of spores.
![The children have to take around eight different medicines a day and make regular visits to the hospital as well as having daily physiotherapy](https://i.dailymail.co.uk/1s/2019/03/14/10/10973582-6808039-image-a-9_1552558670789.jpg)
The children have to take around eight different medicines a day and make regular visits to the hospital as well as having daily physiotherapy
![Ms Woods, a receptionist, with a picture of Sienna when she was in hospital as a baby. She was diagnosed with CF three weeks after birth](https://i.dailymail.co.uk/1s/2019/03/14/10/10973584-6808039-image-a-10_1552558674209.jpg)
Ms Woods, a receptionist, with a picture of Sienna when she was in hospital as a baby. She was diagnosed with CF three weeks after birth
![Ms Woods said she is fighting a daily battle for her children. Pictured, Sienna as a baby](https://i.dailymail.co.uk/1s/2019/03/14/10/10973586-6808039-image-a-11_1552558676069.jpg)
Ms Woods said she is fighting a daily battle for her children. Pictured, Sienna as a baby
‘They’re getting to that age where they want to have a sleepover but we can’t allow them to share a bedroom which is really upsetting.
‘I also try and keep them really clean. I bathe them in the morning separately and then again in the evening to make sure any bugs are washed off.’
Ms Woods said she is too scared even to book a summer holiday as she doesn’t like to plan too far ahead.
She said: ‘With CF you fight daily battles so having to fight this one as well it’s exhausting.
‘Their life expectancy is between 35 to 40 years but we don’t know from one day to the next what might happen.
‘They could be fine and then the next day their lung capacity could drop and that could be it.
‘We try not to plan too far ahead – only a week or two. I wouldn’t think about booking a summer holiday in case something happened.’
Ms Woods said both children are a match for the drug Orkambi often labelled a ‘wonder drug’ that stop the clock on many CF patients’ lung deterioration – the main cause of death for those with cystic fibrosis (CF).
Despite being approved by the European Medicines Agency three years ago, the life-changing drug is still not available in the UK.
![Ms Woods said she doesn't book summer holidays for her children as she doesn't plan more than two weeks in advance, taking each day as it comes](https://i.dailymail.co.uk/1s/2019/03/14/10/10972742-6808039-image-a-12_1552558682787.jpg)
Ms Woods said she doesn’t book summer holidays for her children as she doesn’t plan more than two weeks in advance, taking each day as it comes
![Ms Woods, pictured putting a chest wall oscillation machine vest on Noah to help with his condition, is calling for the 'miracle cure' drug Orkambi to be made available in the NHS](https://i.dailymail.co.uk/1s/2019/03/14/10/10973598-0-image-a-14_1552558693019.jpg)
Ms Woods, pictured putting a chest wall oscillation machine vest on Noah to help with his condition, is calling for the ‘miracle cure’ drug Orkambi to be made available in the NHS
![If Orkambi were available on the NHS Sienna could have had it six months ago and Noah in June this year. The drug could stop the deterioration of CF patients' lungs](https://i.dailymail.co.uk/1s/2019/03/14/10/10973602-6808039-image-a-15_1552558701726.jpg)
If Orkambi were available on the NHS Sienna could have had it six months ago and Noah in June this year. The drug could stop the deterioration of CF patients’ lungs
![Ms Woods said it upsets her that her children are sociable but are restricted](https://i.dailymail.co.uk/1s/2019/03/14/10/10973588-6808039-image-a-16_1552558710300.jpg)
Ms Woods said it upsets her that her children are sociable but are restricted
The NHS is in deadlock with US pharmaceutical company Vertex who is demanding £104,000 per year per patient for the drug.
If it were available on the NHS, Sienna could have had it six months ago and Noah in June this year.
Ms Woods said: ‘My kids are babies so we have more time, but there are CF teens who dying waiting for this drug.
‘We’re really hoping they’re able to come to an agreement and make a deal.
‘It would make a massive difference as far as their lung function is concerned.
‘It could put a halt on damage to their lungs and help keep them healthy as well as keep the condition at bay in the hope that they may one day find a cure.’
Following a meeting with the Health Select Committee, NHS England, NICE and Vertex are due to meet next week in the hope an agreement can be made.
Ms Woods said: ‘Your whole life changes when you have a baby never mind a baby with CF. It can be difficult to come to terms with.
‘They both cope with it really well, they’re both really bubbly children even if they’re unwell and are in hospital.
‘They love to play and talk to people. It definitely upsets me more than them.’
![Ms Woods and her ex-partner Martin Woods, 34, are one of many families urging the NHS and drug company Vertex to come to an agreement about the price of Orkambi](https://i.dailymail.co.uk/1s/2019/03/14/10/10973596-6808039-image-a-17_1552558731024.jpg)
Ms Woods and her ex-partner Martin Woods, 34, are one of many families urging the NHS and drug company Vertex to come to an agreement about the price of Orkambi
A spokesperson for NHS England said: ‘We understand how difficult it must be for families affected by cystic fibrosis and that is why it is vital that Vertex re-engages with the NICE process. Understandably families are calling for this company to make Orkambi affordable and accessible.’
Meindert Boysen, director of NICE’s Centre for Health Technology Evaluation, said: ‘NICE remains committed to working with Vertex and NHS England to find a solution that will allow patients in the NHS in England access to Orkambi, and other cystic fibrosis drugs, at a fair price that reflects their value to patients.’
Rebecca Hunt, corporate affairs international at Vertex Pharmaceuticals, said: ‘We are committed to working with all parties to make our potentially life-changing medicines available to people living with cystic fibrosis in England as quickly as possible.
‘To this end, Vertex, NHS England and NICE have agreed to meet again next week to continue discussions.