Developments in the treatment of Cystic Fibrosis

A recent article titled ‘Cystic fibrosis drug given green light in England’ explains that a new drug which aims to improve lung function in patients with CF has been released to the public. Before you read the article...

What do you know about CF?

• For more information about CF – go to the Cystic Fibrosis Trust website
• The term cystic fibrosis comes from:
  cystic: describing a cyst, from the Greek word kystis which means bladder or pouch. In the case of CF, the cysts form in the pancreas.
  fibrosis: fibrous growth in an organ (in the case of CF, in the pancreas), from the Latin word fibra which means a fibre or filament.
• CF was recognised as a specific disease by Dorothy Hansine Andersen, an American pathologist and paediatrician in 1938, although descriptions of the condition are evident from the late 16^th century.

Language Focus 1: Adjectives with life
life-extending: helping people live longer
life-shortening: condition which causes people to have a shorter life than the average person
life-threatening: condition which may cause a person to die
life-affirming: something which makes you feel glad to be alive
lifespan: the period of time that a person exists
lifetime; the period of time something or someone exists (I’ve seen many changes in my lifetime)

Language Focus 2: describing death
fatal: something which is able to cause death (I a fatal illness) or a serious consequence (a fatal flaw in the design of the building)
mortal: something that causes death, e.g. a mortal blow to the head
lethal: something which is specifically designed to cause death, e.g. a lethal injection to execute someone
terminal: likely to cause death, often used to describe the end stages of an illness, e.g. terminal cancer

deathly: something which resembles death, e.g. a deathly pallor (very pale skin)

Now, read the article and answer the questions.

1. Why do people with CF require frequent hospitalisation?
2. Why might the new drug be ‘good for taxpayers and patients’?
3. What is a ‘green light’? What other expression do you know with the same meaning? 

Cystic fibrosis drug given green light in England

A life-extending drug for cystic fibrosis will be available on the NHS in England, health bosses say.

NHS England reached a deal with the manufacturers of Orkambi, Vertex Pharmaceuticals, after months of negotiations. Patients should be able to get the drug within 30 days.

The drug improves lung function and can be given to children as young as two.

The firm wanted to charge £100,000 per patient per year, but a compromise has been reached in a confidential deal.

It is understood to be significantly less than the sum originally asked for.

It comes after the Scottish Government reached an agreement with the manufacturers last month.

Wales and Northern Ireland can also access the drug under the same terms negotiated by NHS England.

'Good for patients and taxpayers'

Two other drugs made by Vertex - Symkevi and Kalydeco - will be made available as part of the deal.

These also treat cystic fibrosis symptoms, but cannot be used until a patient is 12.

The treatments do not work for all patients with cystic fibrosis - only those with certain mutations.

It is estimated about half of the 10,000 patients in the UK will benefit from these drugs.

Cystic fibrosis is a life-shortening genetic condition that causes fatal lung damage.

Only around half of those with the condition live to celebrate their 40th birthday.

NHS England chief executive Simon Stevens said the deal was "good for patients and fair to British taxpayers".

Health Secretary Matt Hancock described it as "wonderful news" that would improve  [sic]

Answers:

1. Why do people with CF require frequent hospitalisation? Children with CF have frequent chest infections. Read more here: https://www.blf.org.uk/support-for-you/cystic-fibrosis/treatment
2. Why might the new drug be ‘good for taxpayers and patients’? frequent hospitalisation is costly to health providers and to families who often have to take time off work to care for a sick child.
3. What is a ‘green light’? What other expression do you know with the same meaning? Another expression is the go-ahead (‘give something the go-ahead’). Read more about the development of the new drug here: https://www.statnews.com/2019/10/23/we-conquered-a-disease-how-vertex-delivered-a-transformative-medicine-for-cystic-fibrosis/