EU-IPF Federation Associate Membership

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EU-IPF Federation Official Logo

We are pleased to announce that the Bolton Pulmonary Fibrosis Support Group has been accepted as an Associate Member of The European Idiopathic Pulmonary Fibrosis and Related Disorders Federation (EU-IPFF). Obviously we are delighted to to have been accepted as an Associate Member of this internationally recognised body, thereby increasing our support for Idiopathic Pulmonary Fibrosis (IPF) internationally but our own members also.

Who Are The EU-IPF Federation

The European Idiopathic Pulmonary Fibrosis and Related Disorders Federation (EU-IPFF) is a non-profit organisation that brings together European national patient associations committed to defending their vision of equal access to treatment and care for all IPF patients, regardless of geography, socio-economic status or age. Our common view has encouraged us to officially establish the first European IPF and other related disorders federation in July 2016.

Our commitment and ambition are grounded in one of our key achievements: the development of the European IPF Charter, which was launched in the European Parliament in September 2014. The Charter lays down the rights of IPF patients and concrete policy recommendations that, if adopted, would ensure improvements in patients’ quality of life whilst supporting efforts to find a cure.

We aim to serve as a trusted resource for the IPF community by raising awareness, providing disease education, advancing care, and supporting research for IPF whilst defending the interests of IPF patients at European level. We fight for equal access to treatment, information and ensuring exchange of information between national patient groups.

Each year, during IPF World Week, IPF patient associations across the world join forces to raise awareness of IPF, call for better access to care, and bring hope to those living with the disease. If you would like to know more about the EU-IPFF and the work they do download their leaflet EU-IPFF Breathing Hope or visit their website

Stem Cell Therapy and IPF

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Lung Stem Cells

Pulmonary hypertension, high pressure in the blood vessels of the lungs, is a common and serious complication of chronic lung diseases, including idiopathic pulmonary fibrosis (IPF).

IPF is a type of interstitial lung disease, which causes progressive scarring, leading to the lung tissue thickening and stiffening. In particular, it affects the interstitium, or the area between the alveoli where gas exchange takes place in the lungs, and the blood vessels. The scar tissue reduces lung capacity and restricts gas exchange, resulting in less oxygen being transferred to the blood.

The exact mechanism through which IPF can cause pulmonary hypertension is not well understood, but it is thought that the scarring restricts blood vessels, impeding blood flow and making it more difficult for the heart to pump blood through the lungs. Another theory is that the damage response and resulting scar tissue reactivates pathways involved in lung development and triggers vascular remodelling. This can change the shape of the blood vessels, such as causing them to narrow and restrict blood flow.

IPF is a debilitating and fatal condition, and while current therapies can help to improve survival and quality of life, there is  no known cure. However, research into promising approaches like stem cell therapy is ongoing.

What is stem cell therapy?

The body is made up of many different types of specialised cells that fulfil specific functions.

Once a cell is specialised, it generally cannot change and will only divide to produce similar cells. Stem cells are different in that they have the potential to develop and specialise into multiple different types of cells. Depending on where the stem cell originates, the type and range of cells it can develop into will differ.

By administering stem cells to IPF patients, it may be possible to generate the new lung tissue necessary for effective gas exchange.

Mesenchymal stem cells (MSCs) are now being investigated to treat IPF due to their ability to potentially reduce inflammation in the lungs. Damage caused by inflammation can lead to scarring in the lungs, so reducing lung inflammation may be able to lessen further scarring.

MSCs can be obtained from various tissues, including the adult bone marrow, umbilical cord blood, and the placenta.

Stem cell therapy in clinical trials for IPF

Both ongoing and completed clinical trials have investigated stem cell therapy in IPF patients.

A proof-of-concept, open-label Phase 1 clinical trial (NCT01385644), carried out at The Prince Charles Hospital in Brisbane, Australia, aimed to determine whether MSC therapy was safe and feasible. The study enrolled eight IPF patients, who received either a high or low concentration of MSCs. Trial results, published in the journal Respirology, suggested that the therapy is feasible and both doses were well-tolerated, with only minor and short-term adverse effects. At six months after treatment, the patients showed no worsening in their condition.

A Phase 1 randomized and blinded, placebo-controlled clinical trial, called AETHER (NCT02013700), enrolled 25 IPF patients at the Interdisciplinary Stem Cell Institute at the University of Miami. The trial aimed to assess the safety of MSC therapy, and gain a preliminary idea of its efficacy over a 60-week period. Participants were randomly assigned a single dose of one of three concentrations of MSCs or to a placebo. The trial is ongoing, but no longer recruiting participants.

Results from nine patients, who were treated and monitored for the full 60 weeks, have been published in the scientific journal, Chest. These results did not include a placebo arm. The treatment appeared to be well-tolerated and no serious side effects due to the therapy were recorded. However two patients died for reasons attributed to disease progression.

The only study currently recruiting patients is a Phase 1/2 clinical trial (NCT02745184) taking place at two sites in China. Researchers intend to isolate lung stem cells from the patient’s own bronchi (the large tubes of the lungs) and expand them in the laboratory. About 20 enrolled patients will then receive a single injection of their cultured lung stem cells directly into the area affected by IPF.

To assess the safety of the treatment, patients will be monitored for side effects for up to one year. Efficacy will be measured by  changes in several lung function and exercise ability tests. The primary test, at 48 weeks post-treatment, will be the change in forced vital capacity (FVC), or the volume of air that can be quickly exhaled after a deep breath. The trial is expected to finish in December 2018.

Genome England Project

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100,000 Genomes Project

About Genomics England

Genomics England, with the consent of participants and the support of the public, is creating a lasting legacy for patients, the NHS and the UK economy, through the sequencing of 100,000 genomes.

Genomics England was set up to deliver the 100,000 Genomes Project.

This flagship project will sequence 100,000 whole genomes from NHS patients with rare diseases, and their families, as well as patients with common cancers.

Our aims

  • To bring benefit to patients
  • To create an ethical and transparent programme based on consent
  • To enable new scientific discovery and medical insights
  • To kickstart the development of a UK genomics industry

Our history

Genomics England was announced by Jeremy Hunt, Secretary of State for Health and Social Care, as part of the NHS 65th birthday celebrations on 5 July 2013.

Jeremy Hunt

Secretary of State for Health,
Rt Hon Jeremy Hunt MP

He said: “The NHS has a long track record as a leader in medical science advances and it must continue to push the boundaries by unlocking the power of DNA data.

“The UK will become the first ever country to introduce this technology in its mainstream health system – leading the global race for better tests, better drugs and above all better, more personalised care to save lives.

“Genomics England will provide the investment and leadership needed to dramatically increase the use of this technology and drive down costs.”

Genomics England will be efficient, flexible, and able to move quickly as the market changes. It will contract with others for most of its needs including sample collection, analysis and data storage but crucially, it will maintain clear responsibility for data protection.

Genomics England will manage contracts for specialist UK-based companies, universities and hospitals to supply services on sequencing, data linkage and analysis. It will also strictly manage secure storage of personal data in accordance with existing NHS rules designed to securely protect patient information.

This project represents a great opportunity to translate our world class genomic science into world leadership in genomic medicine.

Genomics England is funded by the Department of Health & Social Care in the medium term, and any surplus will be invested back into improving health. It is chaired by Sir John Chisholm, former chair of the Medical Research Council.

Sir John said:

“This project represents a great opportunity to translate our world class genomic science into world leadership in genomic medicine. Genomics England will create a dataset of anonymised whole genome sequences matched with clinical data at a scale unique in the world.

“Participating patients will have the opportunity to benefit from clinical insights derived from the sequencing of their genome while at the same time contributing to knowledge which will be valuable to the whole patient community. It is from that knowledge that world leading therapeutic products and processes will become available to all patients.”

It is estimated that one in seventeen people are born with or develop a rare disease during their lifetime. At least 80% of rare diseases have an identified genetic component, with 50% of new cases of rare diseases being identified in children. However, it can take considerable time and expense between a patient first presenting at a doctors and receiving an accurate diagnosis. The time taken to sequence a whole human genome has been dramatically reduced and will become more affordable for routine use as the price continues to fall.

Professor Dame Sally Davies, Chief Medical Officer

Professor Dame Sally Davies,
Chief Medical Officer

Chief Medical Officer Professor Dame Sally Davies said:

“By putting firm foundations in place through Genomics England, this technology will let us make ground-breaking discoveries about how diseases work, who could be susceptible to them, how we can treat them, and what treatments might work.

“Earlier diagnoses will help to reduce uncertainty and stress for patients and families involved.”

Professor Mark Caulfield is the chief scientist for the company. Mark is a NIHR Senior Investigator at the Queen Mary University of London and the Barts National Institute for Health Research Biomedical Research Unit.



About This Site

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Bolton Pulmonary Fibrosis Support Group was formed in February 2018 at the request of the Royal Bolton Hospital Interstitial Lung Disease Team and Action for Pulmonary Fibrosis the National Charity for people diagnosed with Idiopathic Pulmonary Fibrosis. Since 2013 over 60 support groups like ours have sprung up around the UK.

The aim of the group is to provide; support and information for people diagnosed with pulmonary fibrosis, along with their family, friends and carers in an environment where people can learn about their condition, .A place where they can share their thoughts, fears and feelings with other with the same condition..  A place of mutual understanding, care, support and learning,. to facilitate a better quality of life.


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