SHIELD CF: Towards personalized treatment for kids with Cystic Fibrosis
Cystic Fibrosis is more common in Ireland than anywhere else in the world due to the high incidence of carriers of the gene. There is no cure for CF, but progress has been made in the treatment of CF lung disease over recent years, and survival continues to increase. However, it is still a life shortening disease, with a predicted survival age only in the mid-thirties.
The best way to study CF – and improve the life span and quality of life for children with the disease – is with a well-designed, long term, and wide ranging clinical research programme. This is where SHIELD CF comes in. Began at the NCRC in 2010, by Professor Paul Mc Nally and Dr Barry Linnane, the ultimate aim of SHIELD CF is to include all children born with CF in Ireland in the research programme, and to follow them through until adulthood with matched research samples and clinical information painting a complete picture of each child at each time-point. Ireland is a perfect setting for this, given our small geographical area, high incidence of CF and small network of CF centres. To date 3 of the 6 centres on the island have been able to take part.
Keeping young lungs healthy
The backbone of SHIELD CF is its Discovery Bank. The first phase of this research project is tracking children from the time of their diagnosis – now through the new-born heel prick test – to 6 years of age. This is a critical time as we now know that by this age, some children will already have irreparable damage to their lungs without any apparent symptoms. The stored research samples will lead to discoveries of biological ‘red flags’ that will separate out the children with infection or damaging lung inflammation from those with healthier lungs. This will allow targeted, early intervention and will keep young lungs healthy.
The strength of SHIELD CF is the detailed data on each child: a range of blood tests, x-rays, lung function tests, and a bronchoscopy (a lung scope) in very young children are taken annually. These are catalogued and samples are deep frozen in the CF Discovery Bank for current and future use, with matched clinical information on each patient including complications of the disease, infections and treatments received.
National and International Collaboration is already a feature of the CF research programme. Scientists and doctors are currently mining this resource, with a number of studies ongoing. These include studies:
- of the microbes living in the lower reaches of the lungs;
- of how we can use natural occurring molecules to protect the cells lining the lungs;
- identifying the best method to collect and store lung washings.
- Running for 7 years
- Already has 196 samples in the SHIELD CF discovery bank
- Collaborates with 3 centres nationally
- 22 international papers have been published since programme started
- First in Ireland: introduced a new lung function test
- Developed a new scientific test to identify a protein in the CF lung fluid that indicates lung damage