Watch the moment Portsmouth six-year-old gets first dose of 'magic pills' Orkambi for cystic fibrosis

It was announced in October that a deal had been struck between manufacturer Vertex and the NHS to license Orkambi in England after years of campaigning and yesterday the Weir family, who used to live in Paulsgrove but now live in Portchester, got a knock at their door from the postman with a special delivery. Today Ivy started her treatment.

Gemma, 36, said: ‘Opening the door to a delivery of Orkambi was absolutely amazing. From the day Ivy was born we were told about this wonder drug that doesn’t just deal with the symptoms of cystic fibrosis but actually makes changes in the genes.

‘It has been a long fight for this drug. It has been gruelling and it has been hard going on all of us. We have had to give it our all to get where we are and take on the powers that be to fight for what it right, for Orkambi and fight for our kids lives.’

Ivy must take four pills a day - two in the morning and two in the evening – and will be monitored over the next few days for side effects. A box will be delivered every three weeks to ensure the youngster has enough medicine.

She told The News: ‘These are my magic pills. It is very exciting and I am super happy.’

Gemma added: ‘These pills are hope for the future.

‘Before these drugs arrived we knew what the future held but now we have hope and we are very fortunate to have them. We also need to carry on fighting for Trikafta which is a similar drug but is for people with two different types of gene copy.’

The family were preparing to to Argentina for treatment of an alternative drug similar to Orkambi when health secretary Matt Hancock confirmed the news of a deal.

Gemma and Ivy delivered a petition to Downing Street and also appeared in a music video as part of their efforts in the Orkambi campaign.

Vertex had been locked in dispute with the NHS over the pricing the drug – said to cost £104,000 per patient per year, which the NHS said was too expensive but both organisations managed to find an agreement.

Cystic fibrosis is a life-shortening genetic disorder which means chloride ions cannot flow freely into or out of the cells as they should, leading to an imbalance of salt and water – which can create a mucus in the lungs.

The mucus in the lungs and digestive system causes breathing problems and nutrient absorption issues.

Orkambi – which is up made from lumacaftor and ivacaftor – helps more chloride ions to pass into and out of the cells, keeping a balance of salt and water in certain organs.