Des Cox


Des Cox

Consultant in Paediatric Respiratory Medicine

Des qualified in medicine from University College Dublin (UCD) in 2001. He completed his paediatric training in Ireland and received his CSCST in 2011. He completed a fellowship in paediatric respiratory medicine at Princess Margaret Hospital, Perth, Australia and was appointed to his current position as consultant in paediatric respiratory medicine at Our Lady’s Children’s Hospital, Crumlin (OLCHC) in March 2012. Des is currently head of the respiratory department at OLCHC. In addition to his current role, he received an adjunct appointment as an associate clinical professor with the UCD School of Medicine in 2016. Des is also the chair of the RCPI policy group on Tobacco and is a member of the national clinical advisor group for Cystic Fibrosis.

Des has a number of current research interests including the role of viruses in wheezing disorders and Cystic Fibrosis (CF) in young children. In particular, his research to date has focused on examining the impact of human rhinovirus (HRV) on children with preschool wheezing. He was awarded an MD thesis from UCD in 2014 on “the role of different rhinovirus species in acute respiratory illnesses in children”. He is currently working with Dr Cillian De Gascun in the National Virus Reference Laboratory (NVRL) on developing a HRV species typing assay to examine the prevalence and role of different HRV species in young children with CF.

He is a co-investigator on the SHIELD CF programme (Study of Host Immunity and Early Lung Disease in Cystic Fibrosis) which is a comprehensive, longitudinal study examining the factors which influence early lung disease in cystic fibrosis.

 

Des Cox

Consultant in Paediatric Respiratory Medicine

Dr. Jenny Lennon
Position: National CF research Co-ordinator
lennonje@tcd.ie

Role:
Jenny has developed an active CF clinical trials site in OLCHC and now co-ordinates all CF clinical research and clinical trials in OLCHC and throughout Ireland via the CF: INK clinical research network.

Dr Rebecca Reilly
Position: Clinical Research Coordinator, Children’s Clinical Research Unit
rebecca.reilly@ncrc.ie

Role:
Rebecca is co-ordinator for clinical research in OLCHC and throughout the SHIELD CF network. She manages the SHIELD CF biobank and databank. She also works on a number of interventional and non interventional clinical trials.

Aine Fleming
Position: Clinical Research Coordinator
aine.fleming@ncrc.ie

Role:
Aine is the primary coordinator for a non-interventional clinical trial ongoing at multiple CF centres in Ireland. She is also works on a number of CF research projects.

Rebecca McLoughlin
Position: Research Respiratory Physiologist
rebecca.mcloughlin@ncrc.ie

Role:
Rebecca is coordinating a research study looking at a new lung function measurement device which the CF centre in Crumlin have recently acquired, called the Exhalyzer D a Multiple Breath Washout (MBW) test. Rebecca aims to validate the test in a population of healthy Irish children and in children with cystic fibrosis.

The role of rhinovirus infections in young children with Cystic Fibrosis

Viruses are the major cause of respiratory illnesses in children under the age of five years and yet little is known about the impact of viruses on children with CF. Viruses have been well studied in other chronic lung conditions such as asthma and recent evidence suggests that Human Rhinovirus (HRV) may have a significant impact on the development of asthma and recurrent wheezing in young children. The main aims of the study are to examine the prevalence of different HRV species in young children with CF and to examine the impact of different HRV species on clinical outcomes in young children with CF.

The Study of Host Immunity and Early Lung Disease in CF (SHIELD CF)

SHIELD CF is an ambitious long term translational research programme based in NCRC and running in the CF centres in OLCHC, Tallaght and Limerick. SHIELD CF is based around our annual assessment service, which includes a range of blood tests, x-rays, lung function tests and a bronchoscopy in children who cannot produce sputum specimens. Children are followed up until adulthood. All samples that are taken are catalogued and stored in a specialised biobank for current and future use. In parallel to our clinical sample collection, we are collecting a wide array of clinical information about our patients including complications of the disease and access to medical care. The design of the program allows for a significant degree of collaboration with national and international colleagues in an effort to gain a greater understanding of lung disease in children with CF.

The airway microbiome in children with CF (AIM CF)

In recent years we have come to understand that a vast array of bacteria lives in our airways – even in healthy people. It has become clear that the interaction between the healthy and harmful bacteria in the lungs is complex. We are conducting a long term and very wide-ranging study in children with CF looking at how these bacteria vary over time and whether they are related to lung inflammation, changes in antibiotic use and lung function at school age.

Inflammatory and Microbiological Implications of Pooling of Bronchoalveolar Lavage samples in Preschool Children with Cystic Fibrosis (the POOL study)

Bronchoalveolar lavage fluid is collected when salt water is instilled temporarily in the lungs during a bronchoscopy procedure. It collects the proteins and bacteria from the lower airways that otherwise could not be collected. This is potentially a very useful test for clinical trials of new medications in children with CF, but there are several different approaches internationally to the way the test is performed. This multicentre study seeks to determine if different ways of handling and processing the fluid impact on its utility as a marker of lung pathology.

CF Urinary biomarker study (CUBS)

Lots of substances that are produced in the body are excreted in the urine. This has been shown to be true of markers of lung damage in people with lung diseases. This is of significant interest to doctors looking after children with CF as the collection of urine is a painless and harmless procedure, urine is always accessible, and it is an easy fluid to work with. This study is looking to collect information about what substances are in the urine, how they naturally vary over time and whether we can see any patterns in these substances relating to lung infection and inflammation in children with CF. We are working with a company called Mologic in the UK who have experience in this area and are currently working on a similar study to this in adults with CF.

Protease activity: Lung Measurement in Children with Cystic Fibrosis (PALM)

This is a longitudinal study looking at a type of enzyme called a protease. Different types of proteases such as neutrophil elastase, are risk factors for lung damage in children with CF. Presently not a lot is known about the role of other proteases in the preschool CF lung. The study is designed to establish the degree and pattern of protease and anti-protease activity (PAPA) in the lungs of preschool children with CF, and to determine if specific proteases or anti-proteases are associated with either pulmonary infection or clinical disease parameters. The study also proposes to determine whether markers of PAPA can be detected in urine.

Children's Follow up Orkambi Real Word MBW Study (C-FORMS)

Cystic fibrosis is a common inherited life threatening disease which is particularly common in Ireland. People with CF have a mutation in a gene that produces a protein called cystic fibrosis transmembrane conductance protein or CFTR. CFTR regulates the movement of salt in and out of cells. When CFTR doesn’t work well the result is thick, sticky mucus in the respiratory, digestive and reproductive systems, and inability to fight lung infections. Medications have now been designed to specifically target the CFTR protein. These are called CTR modulators. One such modulator is Orkambi, which has recently been approved to treat children over 6 years in Ireland. In this study we are using a new type of specialised lung function technique called ‘multiple breath washout’ (MBW) to look at the lung function of children aged 6-11 years with CF. Children will undergo MBW and a new form of low dose CT scanning called ‘spirometrically controlled CT’ before they commence on Orkmabi. In the first study of its kind in the world, we will be working with colleagues in Rotterdam, London and Cincinnati to assess the effects of Orkambi on the lung structure and lung function of these Irish children for two years following commencement of Orkambi in a real world setting.

Predicting REsponses in Disease outcomes in CF using iPSCs for new CFTR Therapies (PREDiCT)

The introduction of promising new medications to treat the basic defect in people with CF holds great promise to improve the survival and health of our patients with CF. New CF treatment such as Orkambi are currently only available for CF patients with the most common mutations. Furthermore only individuals with common or well-recognised mutations are being recruited to clinical trials of these new investigational medications. There are likely to be a significant group of children and adults with rarer mutations who may be left behind, with no prospect of having new drugs licensed for use in these people. What is required here is a patient specific ‘test’ to determine whether an individual may benefit from treatment with a given drug. The purpose of this research is to create a repository or “bank” of a type of stem cell called “induced pluripotent stem cells” or iPSCs. Stem cells are special kinds of cells that can renew themselves. Under certain experimental conditions, iPSCs can be directed to grow into any type of tissue or organ. In the case of this study, we are working with colleagues in RCSI and Boston University to develop a special type of lung cell which can readily be used to determine whether the child’s own lung cells may respond to new CF drugs. Ultimately, we would like to develop a test that can be offered to all patients with cystic fibrosis, particularly those with rare mutations not currently able to avail of approved drugs, to determine whether or not they will respond to a given drug.

Role of Exosomes in CF Airway Pathogenesis (RECAP)

RECAP is a new joint project between our CF clinical team and our CF Basic Science core in NCRC led by Dr Judith Coppinger. The team has discovered that there are small capsules within cells called exosomes. These exosomes are released by lung cells from people with CF and can help regulate inflammation. Exosomes form part of a cell-to-cell communication system used by most cells to send messages across the human body. Understanding their role promises to improve our understanding of many diseases and how we treat them. These small exosomes are carriers of proteins and other signals that can impact neighbouring inflammatory cells in the fight against infection and may offer the potential to be markers of lung disease in CF. From our research we have discovered that CF cells produce more of these exosomes than healthy cells. Using state of the art scientific technologies, we examined what protein molecules are contained in these exosomes and found many proteins are involved in immune cell function and inflammation. We are now using samples collected through SHIELD CF in children of different ages, some of whom are on new CF modifier drugs to understand this phenomenon in greater detail and how exosomes may cause disease in CF and respond to treatment strategies. We hope to determine in the future whether certain exosomes will help us to non-invasively determine how patients are doing in terms of lung inflammation and damage.

Baseline LCI OF Children With OR without CF (BLOC WORC)

Understanding early lung disease in CF is an international focus to try and tailor treatment to slow or prevent decline in lung function. In Ireland, we currently have limited capacity to formally assess early lung disease in this group. In recent years, there has been a move towards closer monitoring and active treatment in younger patients with CF, in an effort to improve long term survival. Lung Clearance Index (LCI) is a lung function measurement performed using the multiple breath washout (MBW) method. The MBW technique is an effort independent, low-risk, non-invasive procedure performed on spontaneously breathing children. The LCI is a value that is used to indicate the performance of the lungs, particularly the smaller airways. LCI is much more sensitive than other established tests for CF lung disease and can be performed in younger children. It is therefore a very helpful test for doctors looking after children with CF. We have recently acquired the equipment for MBW testing (N2) and wish to validate the test in a population of healthy Irish children and in children with CF, both when well and unwell and to compare our results with established values using this equipment. We are working with our colleage Dr Barry Linnane in Limerick, who has been using this device for a number of years.



The list of publications below is automatically derived from MEDLINE/PubMed. As a result, there may be incorrect or missing publications.

Cox DW, Khoo SK, Zhang G, Lindsay K, Keil AD, Knight G, Gern JE, Laing IA, Bizzintino J, Le Souëf PN, 2018 Aug, Rhinovirus is the most common virus and rhinovirus-C is the most common species in paediatric intensive care respiratory admissions. Eur Respir J, DOI: 10.1183/13993003.00207-2018
Carew C, Cox DW, 2017 Sep 5, Laps or lengths? The effects of different exercise programs on asthma control in children. J Asthma, DOI: 10.1080/02770903.2017.1373806
Khincha PP, Bertuch AA, Agarwal S, Townsley DM, Young NS, Keel S, Shimamura A, Boulad F, Simoneau T, Justino H, Kuo C, Artandi S, McCaslin C, Cox DW, Chaffee S, Collins BF, Giri N, Alter BP, Raghu G, Savage SA, 2017 Jan, Pulmonary arteriovenous malformations: an uncharacterised phenotype of dyskeratosis congenita and related telomere biology disorders. Eur Respir J, DOI: 10.1183/13993003.01640-2016
Cox DW, Mullane D, Zhang GC, Turner SW, Hayden CM, Goldblatt J, Landau LI, Le Souëf PN, 2015 Dec, Longitudinal assessment of airway responsiveness from 1 month to 18 years in the PIAF birth cohort. Eur Respir J, DOI: 10.1183/13993003.00397-2015
Halmø Hürdum S, Zhang G, Khoo SK, Bizzintino J, Franks KM, Lindsay K, Keil AD, Cox DW, Goldblatt J, Bochkov YA, Gern J, Ulrik CS, Souëf PN, Laing IA, 2015 Aug, Recurrent rhinovirus detections in children following a rhinovirus-induced wheezing exacerbation: A retrospective study. Int J Pediatr Child Health, DOI: 10.12974/2311-8687.2015.03.01.2
Walsh A, Phelan F, Phelan M, Ryan M, Healy F, Slattery DM, Elnazir B, Greally P, Linnane B, Ní Chróinín M, Mullane D, Herzig M, Cox DW, Javadpour S, McNally P, 2015 Mar, Diagnosis and treatment of sleep related breathing disorders in children: 2007 to 2011. Ir Med J, DOI: 10.12974/2311-8687.2015.03.01.2
Turner S, Fielding S, Mullane D, Cox DW, Goldblatt J, Landau L, le Souef P, 2014 Nov, A longitudinal study of lung function from 1 month to 18 years of age. Thorax, DOI: 10.1136/thoraxjnl-2013-204931
Cox DW, Le Souëf PN, 2014 Sep, Rhinovirus and the developing lung. Paediatr Respir Rev, DOI: 10.1016/j.prrv.2014.03.002
Cox DW, Bizzintino J, Ferrari G, Khoo SK, Zhang G, Whelan S, Lee WM, Bochkov YA, Geelhoed GC, Goldblatt J, Gern JE, Laing IA, Le Souëf PN, 2013 Dec 1, Human rhinovirus species C infection in young children with acute wheeze is associated with increased acute respiratory hospital admissions. Am J Respir Crit Care Med, DOI: 10.1164/rccm.201303-0498OC
Mullane D, Turner SW, Cox DW, Goldblatt J, Landau LI, le Souëf PN, 2013 Apr, Reduced infant lung function, active smoking, and wheeze in 18-year-old individuals. JAMA Pediatr, DOI: 10.1001/jamapediatrics.2013.633
Cox DW, Verheggen MM, Stick SM, Hall GL, 2012, Characterization of maximal respiratory pressures in healthy children. Respiration, DOI: 10.1159/000342298
Cox DW, Kelly C, Rush R, O'Sullivan N, Canny G, Linnane B, 2011 Nov-Dec, The impact of MRSA infection in the airways of children with cystic fibrosis; a case-control study. Ir Med J, DOI: 10.1159/000342298
Bizzintino J, Lee WM, Laing IA, Vang F, Pappas T, Zhang G, Martin AC, Khoo SK, Cox DW, Geelhoed GC, McMinn PC, Goldblatt J, Gern JE, Le Souëf PN, 2011 May, Association between human rhinovirus C and severity of acute asthma in children. Eur Respir J, DOI: 10.1183/09031936.00092410
Stiskal JA, Dunn MS, Shennan AT, O'Brien KK, Kelly EN, Koppel RI, Cox DW, Ito S, Chappel SL, Rabinovitch M, 1998 Jan, alpha1-Proteinase inhibitor therapy for the prevention of chronic lung disease of prematurity: a randomized, controlled trial. Pediatrics, DOI: 10.1183/09031936.00092410
Hyland RH, Gordon DA, Broder I, Davies GM, Russell ML, Hutcheon MA, Reid GD, Cox DW, Corey PN, Mintz S, 1983 Jun, A systematic controlled study of pulmonary abnormalities in rheumatoid arthritis. J Rheumatol, DOI: 10.1183/09031936.00092410

Name:Professor Paul McNally
DepartmentConsultant in Paediatric Respiratory Medicine
Institution:Our Lady’s Children’s Hospital Crumlin
Country:Ireland
Name:Dr Barry Linnane
Position:Consultant in Paediatric Respiratory Medicine
Email:Barry.Linnane@hse.ie
Role:Dr Linnane is paediatric CF centre director in University Hospital Limerick and co-founder of SHIELD CF. He is an active clinical researcher and local principle investigator on several international clinical trials.
Name:Dr Judith Coppinger
Position:Senior Lecturer, RCSI
Email:Judith.coppinger@ncrc.ie
Role:Judith is an accomplished scientist who has worked in leading institutions in Ireland and North America, and published extensively in the areas of CF and cancer. Judith heads up the basic science research core in NCRC.
Name:Dr Peter Greally
Position:Consultant in Paediatric Respiratory Medicine
Email:peter.greally@amnch.ie
Role:Dr Greally is CF centre director in Tallaght Hospital. He is an active clinical researcher and local principle investigator on several international clinical trials.
Name:Dr David Rea
Position:Consultant Paediatric Radiologist
Email:david.rea@olchc.ie
Role:Dr Rea is a consultant paediatric radiologist in OLCHC. He is lead radiologist for the SHIELD CF network. Dr Rea leads the imaging analysis aspects of SHIELD CF. OLCHC is part of the SciFi network – an international imaging network for children with CF.
Name:Dr Michael McDermott
Position:Consultant Paediatric Pathologist
Email:Michael.mcdermott@olchc.ie
Role:Dr McDermott is a consultant paediatric pathologist in OLCHC. He is lead pathologist for the SHIELD CF network, and leads the optimization of techniques for cell imaging and analysis for SHIELD CF.
Name:Dr. Cillian De Gascun
DepartmentVirology
Institution:National Virus Reference Laboratory, UCD
Country:Ireland

Current Trials & Studies

Study NameStudy TitleStudy TypeSponsorSponsor Type
SHIELD CFThe Study of Host Immunity and Early Lung Disease in Children with CFNon-Interventional StudyNCRCAcademic - Investigator Led
VX14-661-110A Phase 3, Open-label, Rollover Study to Evaluate the Safety and Efficacy of Long Term Treatment With VX-661 in Combination With Ivacaftor in Subjects Aged 12 Years and Older With Cystic Fibrosis, Homozygous or Heterozygous for the F508del-CFTR MutationClinical TrialVertexIndustry Sponsored
VOICE: VX14-CFR-107Observational Registry of Cystic Fibrosis Patients in EuropeNon-interventional studyVertexIndustry Sponsored
CUBS (CF Urinary biomarker study)CF Urinary biomarker studyNon-interventional studyNCRCAcademic - Investigator Led
PALM StudyPALM study (Protease activity: Lung measurement) – a longitudinal study in children with CF to establish spectrum and influence of protease antiprotease activity in CF Starting in JulyNon-Interventional StudyNCRCAcademic - Investigator Led
VX15-770-124A Phase 3, 2-Part, Open-label Study to Evaluate the Safety, Pharmacokinetics, and Pharmacodynamics of Ivacaftor in Subjects With Cystic Fibrosis Who Are Less Than 24 Months of Age at Treatment Initiation and Have a CFTR Gating MutationClinical TrialVertexIndustry Sponsored
VX15-770-126A Phase 3, 2-Arm, Open-label Study to Evaluate the Safety and Pharmacodynamics of Long-term Ivacaftor Treatment in Subjects With Cystic Fibrosis Who Are Less Than 24 Months of Age at Treatment Initiation and Have a CFTR Gating Mutation.Clinical TrialVertexIndustry Sponsored

Previous Trials & Studies

Study NameStudy TitleStudy TypeSponsorSponsor Type
VX15-371-101A Phase 2a, Randomized, Double-blind, Placebo-controlled, Incomplete Block, Crossover Study to Evaluate the Safety and Efficacy of VX-371 in Subjects Aged 12 Years or Older With Cystic Fibrosis, Homozygous for the F508del-CFTR Mutation, and Being Treated With OrkambiClinical TrialVertexIndustry Sponsored
VX14-661-106A Randomized, Double-Blind, Placebo-Controlled, Parallel-Group Study to Evaluate the Efficacy and Safety of VX-661 in Combination With IvacaftorClinical TrialVertexIndustry Sponsored
VX14-661-109A Phase 3 Study to Evaluate the Efficacy and Safety of Ivacaftor and VX-661 in Combination With Ivacaftor in Subjects Aged 12 Years and Older With Cystic Fibrosis, Heterozygous for the F508del-CFTR MutationClinical TrialVertexIndustry Sponsored
VX12-809-103A Phase 3, Randomised, Double Blind, Placebo Controlled, Parallel Group Study to Evaluate the Efficacy and Safety of Lumacaftor in Combination with Ivacaftor in Subjects Aged 12 years and older with Cystic Fibrosis, Homozygous for the F508del CFTR Mutation (TRAFFIC)Clinical TrialVertexIndustry Sponsored
VX12-809-105A Phase 3, Rollover Study to Evaluate the Safety and Efficacy of Long-term Treatment With Lumacaftor in Combination With Ivacaftor in Subjects Aged 12 Years and Older With Cystic Fibrosis, Homozygous or Heterozygous for the F508del-CFTR MutationClinical TrialVertexIndustry Sponsored
PsAer-IgYProspective randomised, placebo controlled, double-blind, multicentre study (Phase III) to evaluate clinical efficacy and safety of avian polyclonal anti-Pseudomonas antibodies (IgY) in prevention of recurrence of Pseudomonas aeruginosa infection in cystic fibrosis patientsClinical TrialMukoviszidosAcademic - Collaborative Group
The POOL studyInflammatory and Microbiological Implications of Pooling of Bronchoalveolar Lavage samples in Preschool Children with Cystic FibrosisNon-Interventional StudyNCRCAcademic - Investigator Led
Rhinovirus pilot studyThe Role of Human Rhinovirus Infections in Young Children with Cystic Fibrosis - A Pilot StudyNon-Interventional StudyNCRCAcademic - Investigator Led