Cystic Fibrosis (CF)MATRIARCH_CFNCT06797206
A Study of Females With CF Throughout Pregnancy and Post-partum, and Follow up of Their Offspring
An observational study of Cystic Fibrosis (CF) and Pregnancy, run by Royal Brompton & Harefield NHS Foundation Trust at 1 site in United Kingdom.
Trial summary
In this study, investigators aim to study in detail the physical (including nutritional and pulmonary) and mental health of females with cystic fibrosis (CF) planning a pregnancy, during pregnancy, and in the early parenthood period. Read more about this study
- Who is it for?
- Any age
- Conditions the study is for
- Cystic Fibrosis (CF), Pregnancy, Infant, Newborn
- How many people
- 60 participants
- Where
- 1 siteUnited Kingdom
- Study dates
- 12 Jun 2025to May 2028
- Phase
- Observational
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages Any age
- All sexes
- No healthy volunteers
- Child, Adult, Older adult
Study population: 1. Females aged 16 years or older with CF under the care of the Royal Brompton Hospital adult CF Unit who are planning a pregnancy or pregnant. 2. Biological offspring of people with CF (both mothers and fathers) cared for by the Royal Brompton Hospital Adult CF Service, from birth to aged six years.
Who may be able to join
- 'Mama' sub-study:
- Written informed consent obtained from participant.
- Under the care of Royal Brompton Hospital CF Reproductive and Maternal Health Service
- Age 16 years or above at time of recruitment
- Confirmed diagnosis of CF
- Planning a pregnancy or pregnant at time of recruitment.
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- Ability to adhere to the required visits and investigations.
- 'Mini' sub-study:
- Written informed consent obtained from participant's legal guardian.
- Infants who have one biological parent with a confirmed diagnosis of CF under care of Royal Brompton Hospital Adult CF Service
- Less than 12 months of age at first visit.
- Ability to adhere to the required visits and investigations.
- 'Midi' sub-study:
- Written informed consent obtained from participant's legal guardian.
- Children who have one biological parent with a confirmed diagnosis of CF who is under the care of Royal Brompton Hospital Adult CF Service.
- Age three to six years at time of visit
- Ability to adhere to the required visits and investigations.
Who cannot join
- 'Mama' sub-study:
- Any significant health condition which would cause inability to comply with protocol based on investigator discretion.
- History of lung transplantation
- 'Mini' sub-study:
- Legal guardians' inability to provide consent to participate in the study.
- Mother of infant has significant comorbidities unrelated to CF which could affect infant outcomes based on investigator discretion.
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- 'Midi' sub-study:
- Legal guardians' inability to provide consent to participate in the study.
- A significant health condition which is known to affect lung function or imaging based on investigator discretion
Trial locations and contacts
1 site in 1 country. Contact the study team or a site near you to ask about taking part.
Idan Bokobza contact
United Kingdom1 site, 1 recruiting
- Recruiting
Royal Brompton Hospital
London
Amy Downes, +44 (0) 207 351 8997, a.downes@imperial.ac.uk
Idan Bokobza, +44 (0) 207 351 8997, i.bokobza@imperial.ac.uk
About this study
Official title: An Observational Study to Evaluate the Impact of Pregnancy and Parenthood in Females With Cystic Fibrosis and Their Offspring in the CFTR Modulator Era.
In this study, investigators aim to study in detail the physical (including nutritional and pulmonary) and mental health of females with cystic fibrosis (CF) planning a pregnancy, during pregnancy, and in the early parenthood period.
Additionally the health of offspring in infancy and early life will be studied to understand if further screening investigations or clinical care models should be part of clinical guidelines
Read the detailed description
Improvements in treatments for people with CF have meant that more are becoming pregnant. CFTR modulators (CFTRm) are one of these treatments. They work by tackling the underlying cause of CF. These changes have created a need and an opportunity for research into the health and experiences of people with CF and their children in the CFTRm era.
The study is called 'MATRIARCH_CF' and includes 3 related sub-studies:
'Mama' is enrolling participants aged 16 years or older with CF under the care of the Royal Brompton Hospital (RBH) adult CF Unit who are planning a pregnancy or pregnant. The aim is to describe the impact of pregnancy and the first 12-24 months of parenthood in females with CF on their physical and psychological health. Investigations in eight visits include blood tests, lung function, imaging, and interviews.
'Mini' is enrolling biological offspring of people with CF (mothers and fathers) cared for by the RBH Adult CF Service, from birth to age two. The aim is to collect information that will allow for assessment of health outcomes in offspring of parents with CF in the short term. There will be up to four visits over two years with investigations including blood tests, sweat tests, and brain ultrasound.
'Midi' explores the same question as 'Mini' but in the longer term for those aged three-to-six. There will be up to two visits, and they include lung function testing and a lung MRI.
This study is described as 'observational' as investigators will not provide or change any treatment. Participant's health will be monitored with a range of investigations, many of which are optional. Knowledge gained from this study will be used to create guidelines to help families with CF and their medical teams make decisions around pregnancy and their offspring.
- Keywords
- Pregnancy/obstetric health in women with cystic fibrosis, Pregnancy, CFTR modulators
- Medical subject headings
- Cystic Fibrosis
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
| Study group | Role | What they receive |
|---|---|---|
| Mama | Females with Cystic Fibrosis who are pregnant or planning a pregnancy | |
| Mini | Children aged 0-24 months with one biological parent who has a diagnosis of cystic fibrosis | |
| Midi | Children aged 3-6 years with one biological parent who has a diagnosis of cystic fibrosis |
What is being measured
The primary outcome is the main question the study is designed to answer.
Change in FEV1
Change in FEV1 percent predicted (ppFEV1) from pre-conception/baseline to end of pregnancy, and at 12- and 24-months post-partum.
Measured: 9 months
Incidence of CF-related pulmonary complications during pregnancy.
Measured: 9 months
Incidence of premature delivery
Defined as birth <37 weeks gestation.
Measured: 3 years
Frequency of liver dysfunction in infants
Defined as ALT/AST/GGT/bilirubin above upper limit of normal using laboratory normal ranges. This is prospectively obtained in the 'Mini' sub-study, and retrospectively reviewed from medical records in the 'Midi' sub-study.
Measured: 2 years
Incidence of congential abnormalities
Based on medical history and examination
Measured: 3 years
Frequency of CF diagnoses in infants despite negative newborn screen testing
The number of participants with normal IRT subsequently diagnosed with CF (based on a combination of CF genetic tests, sweat chloride, and faecal elastase where appropriate).
Measured: 3 years
Frequency of abnormal lung clearance index results in young children
A lung clearance index (LCI) measurement above the normal levels as defined by investigating laboratory values (Midi only)
Measured: 3 years
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted28 Jan 2025
- Study start12 Jun 2025
- Primary completionApr 2028 (estimated)
- Study completionMay 2028 (estimated)
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Publications (1)Papers the sponsor has linked to this study.
- Other trials for Cystic Fibrosis (CF)Studies of the same condition, open and under way.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Observational
- Observational model
- Cohort
- Time perspective
- Prospective
- Enrolment
- 60 participants (estimated)
Sponsor and investigators
- Lead sponsor
- Royal Brompton & Harefield NHS Foundation Trust
- Sponsor type
- University, hospital or other
- Collaborators
- Cystic Fibrosis Trust, King's College London, Imperial College London
- Responsible party
- Sponsor
- Investigators
- Imogen Felton, Principal investigator, Royal Brompton Hospital and NHLI Imperial College LondonJane Davies, Principal investigator, Royal Brompton Hospital and NHLI Imperial College London
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT06797206
- Sponsor's protocol ID
- 352398
- Acronym
- MATRIARCH_CF
- Status last verified
- Sep 2025
- First submitted
- 16 Jan 2025
- Last updated
- 1 Oct 2025
- Results posted
- No
- Data monitoring committee
- No
- FDA-regulated drug
- No
- FDA-regulated device
- No
Publications
Downes A, Bokobza I, Weitnauer L, Scott R, Dobra R, Short C, Semple T, Beaumont O, Williamson C, Davies JC, Felton I. Maternal, Infant, Reproductive and Child Health in Cystic Fibrosis (MATRIARCH_CF): a prospective, observational study to evaluate pregnancy and parenthood in females with cystic fibrosis and health of the offspring in the CFTR-modulator era. BMJ Open Respir Res. 2026 Jun 30;13(1):e004270. doi: 10.1136/bmjresp-2026-004270.
PubMed 42379640