Este sábado se celebra la II Marcha Solidaria MTB Quismondo a beneficio de la Federación Española de FQ

El próximo sábado 12 de abril se celebrará en Quismondo, Toledo (Carretera de Extremadura N-V km. 67) la II Marcha Solidaria MTB Quismondo a beneficio de la Federación Española de Fibrosis Quística.
aquí para ver el vídeo de presentación del evento y aquí para ver la rueda de prensa de su presentación, en la que participó Esther Sabando, vicepresidenta de la Federación Española de Fibrosis Quística.

Puedes inscribirte para participar en la marcha antes del día 9 de abril, indicando tu nombre, apellidos y DNI a través del correo y realizando un ingreso de 10 euros en la cuenta 2105 0094 49 1290014555.

La salida tendrá lugar el día 12 de abril a las 10:30 horas (imprescindible llevar casco y DNI) y habrá tres rutas diferentes de 35 km. (fácil), 55 km. (media) y 15 km. (para niños).

También puedes colaborar a través del dorsal 0 haciendo tu donativo en el mismo número de cuenta o en las mesas de inscripciones el día de la carrera.

¡Anímate a participar y colabora con la Fibrosis Quística!

U.K. cyclist hits Peninsula pavement today to honor friends, raise awareness for cystic fibrosis, leukemia

PORT ANGELES — A Scottish-born bicyclist is expected to arrive in Port Angeles today to end the first day of a 1,800-mile ride to honor the lives of two friends who died because of cystic fibrosis and leukemia.

Will King, 32, of London began his ride this morning in Vancouver, B.C., to raise awareness for organ and tissue donation, encourage donor registration and raise funds for the Anthony Nolan donor matching service and the Cystic Fibrosis Trust.

King’s friends Milly Douglas-Pennant and Millie Forbes both died before the age of 30.

Douglas-Pennant died at age 29 of cystic fibrosis, and Forbes died of leukemia at age 23.

Both diseases can be treated through organ or tissue donation, which can improve a patient’s quality of life and extend or save a life, King said.

“Most people with cystic fibrosis need a lung transplant at some time in their lives,” he said.

Forbes, who was 21 when she was diagnosed, was on a donor list for a bone-marrow transplant.

“They never found a match for her,” King said,.

Donations can be made at one of several websites King established for his solo trip.

“It goes straight to the charities,” he said.

To donate to the Cystic Fibrosis Trust, visit

To donate to the Anthony Nolan organization, go to

King said he plans to ride about 50-65 miles per day for five or six weeks to reach San Diego, where he has friends and family waiting to greet him at the end of his journey.

The solo ride will follow U.S. Highway 101 from the time he enters Port Angeles and will stay on the roadway as he travels through Forks and beyond to Los Angeles, where he will switch to coastal roads to San Diego.

“I plan to hug the coast all the way down,” he said.

King carries camping equipment for most nights, but if the weather becomes severe or there are no available campsites, he said he plans on staying in hotels, with friends who live along the route or with generous hosts willing to take him in the for the night.

It’s by far the longest trip he has ever attempted, he said.

He has trained by making twice daily 15-mile rides while commuting to work, practicing with several 65-mile rides and embarking on a 330-mile trip from London to Amsterdam, utilizing a ferry.

Training in London has prepared him for riding in tight spaces with cars, but dodging log trucks on Highway 101 will be a new experience, he said.

King said he will dress in plenty of bright neon colors to increase his visibility for safety.

Starting his trip in the north in early spring means the weather may be changeable, but he is prepared for storms and expects that the weather will get better as he travels south.

He said he plans a second ride this summer, beginning in San Diego and arriving in Colombia by the end of August.

He then will return to Scotland take part in the Lonach Highland Gathering and Games.

Bridget Eklund Works with Cockroaches for Cystic Fibrosis Research

While numerous stu­dents may earn money in college by serving food at a restaurant, selling clothes in retail or making coffee, one NDSU student is injecting cockroaches with bacteria as a way to earn money.

For Bridget Eklund, a sophomore double major­ing in biotechnology and microbiology, working as an undergraduate laboratory as­sistant in the Van Es building has been a building block to­ward discovering new ways to study bacteria outside of the traditional classroom and textbooks.

Completing research in the lab with Nathan Fisher, a department of veterinary and microbiological scienc­es assistant professor, has given Eklund a clear picture of what she wants to do with her education. “I came to NDSU because they have such a strong focus on re­search, and I’ve always had an interest working in a lab setting,” Eklund said.

Last semester in the lab, Eklund experimented with an amoeba model that helps with the understanding of how bacteria avoid phago­cytosis, also known as the process of “being eaten,” as Eklund explained. After this project, Eklund attended and presented at a conference in South Dakota in October de­tailing her work with these amoeba models.

Similarly, this semes­ter Eklund’s latest project also deals with phagocyto­sis. However, this project consists of cockroaches, catheters, antibiotics and biofilms. This research with cockroaches is designed to potentially help understand the connection between how the bacteria Stenotrophamo­nas maltophilia affects cys­tic fibrosis patients.

“This bacteria is oppor­tunistic, so it attacks patients that already have a com­promised immune system,” Eklund said. “Cystic fibrosis patients are really suscep­tible to an infection to this bacteria.”

Eklund explained that cystic fibrosis patients could develop various bacterial infections from using cath­eters. In order to study this phenomenon, Eklund and her lab partners are injecting cockroaches with catheters containing bacteria. Then, the cockroaches will be given various antibiotics and Eklund will later study the biofilms that were formed after taking the catheters out of the cockroaches.

Basically, Eklund is re­searching how biofilms compete with antibiotics in catheter-injected cockroach­es.

Even though at first Eklund was uncertain about having to work with cock­roaches, she discovered shortly after that this ex­periment has rarely been done before and the findings could have life-changing benefits for those afflicted with cystic fibrosis.

“At first I was a little leery about working with cockroaches,” Eklund chuckled. “But this project gives us a better understand­ing about what is actually happening in a patient be­cause there is no way to do this experiment in a human.”

As Eklund’s love for science and the natural world has since grown from working in the lab with Dr. Fisher, her initial apprecia­tion for the field is largely contributed to her family’s background.

Growing up, Eklund was always fascinated by watch­ing her father work in a sci­ence lab. “I always watched my dad work in the lab, and I always thought it was su­per cool,” Eklund explained. “It’s intriguing to know how all of the little, tiny things work so perfectly in the nat­ural world.”

Eklund’s lab experience working with the amoeba and cockroach models has since helped her realize that pathology is her passion. Her plan is to go to graduate school to pursue a focus in pathogenic bacteria.

“Working with Dr. Fish­er has been really beneficial for figuring out my pas­sion,” Eklund said. “Getting hands-on experience has been really fun. I guess I was lucky and found out what I liked right away by working in the lab.”

Eklund plans to present her cockroach model data in Valley City later this month.

Xenetic Biosciences Announces Positive Phase 1 Clinical Data for PulmoXen(TM) for Treatment of Cystic Fibrosis

LEXINGTON, Mass., Apr 07, 2014 (GLOBE NEWSWIRE via COMTEX) —

Xenetic Biosciences, Inc., a biopharmaceutical company focused on developing next-generation biologic drugs and novel oncology therapeutics, today announced the positive results from its Phase I clinical trial of PulmoXenTM for the treatment of cystic fibrosis. The single center, open-label trial was conducted in Russia by Xenetic’s partner, OJSC Pharmsynthez. In this First-in-Human study, PulmoXen was administered to 12 healthy volunteers via inhalation daily for seven days, in two doses of 2500IU and 5000IU, and was found to be safe and well tolerated. PulmoXen is a novel, modified form of recombinant human DNase I (rhDNase I) designed to be a next-generation version of Pulmozyme.

“The positive data on this next-generation molecule targeting cystic fibrosis, a debilitating Orphan Disease, will allow Xenetic to pursue an Investigational New Drug (IND) filing with the U.S. Food and Drug Administration (FDA), in order to advance toward FDA-sanctioned Phase 1 clinical development,” said Scott Maguire, CEO of Xenetic Biosciences. “These Phase 1 results demonstrate the success and effectiveness of our business strategy to pursue initial development of our clinical pipeline through human trials with our Russian partners, providing Xenetic a potential pipeline of therapies while mitigating drug development risk. We look forward to working with the FDA to further advance the clinical development of PulmoXen.”

The natural enzymatic function of DNase I is to digest DNA. When delivered to the lungs, it can thereby reduce the viscosity of infected lung secretions, which contain significant amounts of extracellular DNA, as well as reduce bacterial biofilm formation in the lungs of cystic fibrosis patients. These effects of DNase I can facilitate clearance of sputum, and improve lung function in cystic fibrosis patients.

PulmoXen is a polysialic acid – conjugated form of rhDNase I that is being developed using Xenetic’s patented PolyXen® delivery technology, with the intent to reduce the required dosage frequency by enhancing the stability and pharmacodynamic profile of the enzyme in sputum. A comparative evaluation of PulmoXen versus Pulmozyme, using ex vivo models of cystic fibrosis sputum, confirmed superiority of PulmoXen with respect to stability and enzymatic activity when digesting DNA in sputum from cystic fibrosis patients.

About Xenetic Biosciences

Xenetic Biosciences is a biopharmaceutical company developing next-generation biologic drugs and novel oncology therapeutics. Xenetic’s proprietary drug technology platforms include PolyXen® for creating next generation biologic drugs by extending the efficacy, safety and half-life of biologic drugs and OncoHist® for the development of novel oncology drugs focused on orphan indications. Xenetic’s lead product candidates include ErepoXen®, an improved, polysialylated form of erythropoietin (EPO) for the treatment of anemia in pre-dialysis patients with chronic kidney disease and OncoHist®, a recombinant human histone H1.3 molecule which Xenetic is developing for the treatment of refractory Acute Myeloid Leukemia (AML). Xenetic is developing a novel series of polysialylated blood coagulation factors through its license agreement with Baxter International Inc. Xenetic is also developing a broad pipeline of clinical candidates for next generation biologics and novel oncology therapeutics in a number of orphan disease indications. For more information, please visit the company’s website at .

Forward-Looking Statements

Certain statements in this press release are forward-looking within the meaning of the Private Securities Litigation Reform Act of 1995. These statements may be identified by the use of forward-looking words such as “anticipate,” “believe,” “forecast,” “estimate” and “intend,” among others. These forward-looking statements are based on Xenetic’s current expectations and actual results could differ materially. There are a number of factors that could cause actual events to differ materially from those indicated by such forward-looking statements. Factors that could cause actual results to differ materially include, but are not limited to, uncertainties associated with completing preclinical and clinical trials for our technologies; the early stage of product development; the significant costs to develop our products as all of our products are currently in development, preclinical studies or clinical trials; obtaining additional financing to support our operations and the development of our products; obtaining regulatory approval for our technologies; anticipated timing of regulatory filings and the potential success in gaining regulatory approval and complying with governmental regulations applicable to our business. Xenetic does not undertake an obligation to update or revise any forward-looking statement. The information set forth herein speaks only as of the date hereof.

Ed Sheeran serenades teenage fan over phone moments before her death from cystic fibrosis

Singer Ed Sheeran helped make a dying teenager’s wish come true on Sunday.

Thanks to a Twitter campaign dubbed #SongForTri that asked the 23-year-old British guitarist to sing a song for Dublin teenager Triona Priestley, Sheeran gave the 15-year-old a phone call the same night as her death.

According to the Irish Independent, Sheeran called Priestley and sang her favorite tune, “Little Bird.”

“Triona slipped into a sleep as Ed was singing to her and passed away shortly afterwards,” Priestley’s brother Colm told the paper. “So Ed Sheeran sang her to sleep.”

Priestley had battled with cystic fibrosis since her childhood. The campaign to contact Ed Sheeran was launched last week, when Priestley revealed that hearing him sing to her was her last wish.

“We are all so proud of Triona and how she fought cystic fibrosis,” Colm Priestley said. “Everyone loved Triona so much and she knew that.”

Upon news of her death, Sheeran tweeted a little tribute to the teenager.

“Rest in peace Triona, so heartbreaking x,” Sheeran posted.

Do you want to Challenge CF?

The idea? Get a small straw and a peg, say “im …name…. and I challenge cf, place the peg on your nose and the straw in your mouth and try breathing like this for 1minute – This is a campaign to raise awareness of the condition Cystic Fibrosis and the daily struggles faced. Hoping to raise money to fund new trails and treatments and work towards a cure! Cystic Fibrosis is a degenerative disease and will only get worse with time, the average life expectancy is in the mid 30’s. Please get involved and Challenge CF!
Want to donate text “CCFD50” and amount £3/£5/£10 70070   or
This is the info to put on the bottom of your videos for people to see x See More
Learn more by visiting Challange CF on Facebook

Cystic Fibrosis, COPD Bacterial Respiratory Infection Treatment Being Developed By Arch Biopartners, University of Cincinnati

Arch Biopartners, a biotechnology company that develops new products and technologies for pharmaceutical and industrial companies, has entered into a one year option agreement to license the commercial rights of a University of Cincinnati-developed technology for treating bacterial respiratory infections associated with diseases such as cystic fibrosis and COPD. The novel treatment utilizes acidified nitrite, a non-antibiotic method developed by Dr. Daniel Hasset, a UC Professor in the Department of Molecular Genetics, Biochemistry and Microbiology.

During the one year option, the company will be working with Dr. Hassett and his team to assess the potential and logistics of conducting a Phase II clinical trial. The goal is to test how effective the UC technology can be when bacteria is resistant to antibiotics.

Gram-negative bacterium Pseudomonas aeruginosa (PA) represents a major concern in this area. Highly resistant to antibiotics and phagocyte-mediated killing, it is the primary cause of pulmonary exacerbations that leads cystic fibrosis and COPD patients to frequent hospitalizations. These two diseases, characterized by airway bacterial infections, are both deadly and chronic, and affect approximately 40,000 and 14.2 million individuals, respectively, in the United States.

When patients come in contact with the mucoid form of PA, their overall lung function declines, which is why it is so urgent to develop novel treatments in this field. In the first phase II human trial, Arch and Dr. Hassett’s team are will test the technology on cystic fibrosis patients presenting with mucoid PA. If the technology proves to be successful, testing will be expanded to include COPD patients.


Phase I Clinical Trial Results

The Phase I results revealed that the new drug is safe at or below the maximal tolerated dose. The previous trial involved over 80 healthy volunteers with single dose and multiple ascending dose studies for up to one week.

Although some adverse events were observed, such as dizziness, methemoglobinemia (methemoglobin was less than 5% of hemoglobin), and a drop in systemic blood pressure, the drug had no adverse effect on human epithelial cells at concentrations up to 300 millimolar, 20 times higher than the dose needed to kill mucoid PA.  The 14 patients with pulmonary arterial hypertension (PAH) had no significant adverse events.

Terapia con células madre y Fibrosis Quística


Células madre adultas son encontradas en diferentes lugares del cuerpo incluyendo cerebro, hígado,  medula ósea, vasos sanguíneos etc… Los científicos creen que están “dormidas” en los tejidos hasta que ocurre la lesión o daño, entonces ellas se diferencian en las células que necesitan reemplazar.
¿El pulmón tiene células madres propias?
Si, parece que  tiene células madre que se encargarían de reparar cuando ocurre alguna lesión o enfermedad, pero todavía no se ha podido identificar cuales serian las células “multipotenciales” que podrían cumplir esa función.
Existen en el epitelio que cubre la vía respiratoria unas células que se llaman basales y parece que esas podrían ser las células madres locales del pulmón. Pero el otro problema parecería ser que esas células madres locales no alcanzan a  reparar el daño si este es muy importante-
¿Que otras células madre pueden utilizarse para reparar el daño pulmonar en enfermedades como fibrosis pulmonar y Fibrosis quística?
La medula ósea que está en los huesos tiene células madre hematopoyéticas y mesenquimales que se sabe pueden adoptar la forma y función de células de otros tejidos. Krause y col en el 2001 demostraron injertos en varios órganos y tejidos a través de la infusión de una única célula madre.
Un ratón hembra fue irradiado letalmente para destruir su medula ósea. Luego se le hizo una infusión de una única célula madre hematopoyética de un ratón macho. Esta única célula transfundida fue capaz de repoblar la medula ósea con todas sus líneas celulares y también se injerto en varios órganos. Hasta el 20 % del  tejido pulmonar  de la rata hembra contenía Cromosoma Y (masculino). Cell 2001; 105:369-77
por el pulmón dañado para ayudar en su reparación. Aunque este injerto pulmonar no ha sido confirmado en todos los estudios, parece que si ocurre pero en muy pequeño porcentaje. La reparación pulmonar parece ser un fenómeno tanto local como por células madre circulantes.
Terapia génica Las células madres pueden ser genéticamente modificadas, luego trasplantadas  e injertarse en pulmón para curar enfermedades como la Fibrosis Quística.
En un estudio se usaron  células madres y se mezclaron en cultivo con células de epitelio respiratorio de pacientes FQ. Las células madres fueron capaces de diferenciarse en células epiteliales y parcialmente corregir el defecto del CFTR.
Células madre embrionarias Estas son cultivadas de blastocitos en desarrollo (embriones) y son pluri o totipotenciales Tienen la habilidad de desarrollar todas las células del cuerpo, incluidas las cel. epiteliales de la Vía aérea. Problemas: riesgos de transformación maligna y dilemas éticos.
Posibilidades terapéuticas
Las células madres tienen muchas aplicaciones a nivel pulmonar. Enfermedades Agudas:   SDRA Enfermedades Crónicas: Enfisema, fibrosis pulmonar, fibrosis quística.
Controversias y dudas ¿Qué células se deben injertar? ¿El injerto realmente ocurre? ¿Se pueden desarrollar tumores con estas células madres injertadas? ¿No serán estas células rechazadas? ¿Pueden las células injertadas producir más daño que reparación?
Estas y otras mas son las dudas sobre el uso de las células madres para reparar el daño pulmonar o curar enfermedades como la Fibrosis Quística
Sabemos que la reparación celular no es una realidad por ahora pero quizás en los próximos años tengamos importantes avances con estas líneas de investigación. Bibliografia: Stem cells for lung disease-, Loebinger M ,Janes S.- Chest 2007,132:279-285

New help for children with cystic fibrosis

Children with cystic fibrosis and their families are to be offered new support to assist with daily physiotherapy sessions.

Young people with cystic fibrosis have to adhere to a daily regimen of for their condition which some children and parents find lengthy and difficult.

However, academics at the University of Stirling are embarking on a £200,000 project to develop a treatment support kit – for DVD, phone apps and web – that will make adherence to treatment more appealing.

The project – which is being funded by the Chief Scientist Office (CSO) of the Scottish Government and the Cystic Fibrosis Trust – will draw on the collaborative expertise of academics and clinicians from across the UK.

Project leader Dr Emma France, who works in the CSO-funded Nursing Midwifery and Allied Health Professions Research Unit based in Stirling’s School of Nursing, Midwifery and Health and Glasgow Caledonian University’s Institute for Applied Health Research, said: “Our earlier research funded by the CSO showed that when daily chest physiotherapy was perceived as difficult by children and their families, physiotherapy sessions were often skipped.

“In developing this new audio-visual package, filled with a range of engaging activities, our work will lead to a cost-effective and accessible intervention tool.  It will change the way children and their carers experience their condition and treatment, particularly in the crucial early years when commitment to that treatment is so vital.

“Some of the approaches will be just for the adults, with a focus on boosting parents’ morale and determination.”

Cystic fibrosis – a life-shortening, genetic condition which causes breathing difficulties, chest infections, digestive problems and malnutrition – affects approximately one in every 2500 babies born in the UK.  Damage to the lungs is exacerbated by poor adherence to treatment – especially in relation to chest physiotherapy.

The support tool will encourage children with cystic fibrosis and their parents to view chest physiotherapy as a positive experience by providing them with a bank of novel and enjoyable strategies – such as activities, games and songs.
Many of these approaches – which are being collated in consultation with parents, children, physiotherapists, psychologists, clinicians and the Cystic Fibrosis Trust – allow members of the wider family to get involved.

Ideas already proposed include clearing the lungs by blowing through a straw to move a Ping-Pong ball around a table; creating an atmosphere of joviality during treatment by singing songs together; and keeping spirits high by telling stories or watching television programmes together while treatment is in progress.

The researchers hope the tool will be adopted by multiple organisations and disseminated widely – contributing to an increase in physiotherapy treatment adherence in young children with cystic fibrosis.  They anticipate this would reduce the health burden on children and the costs for families and the NHS, and lead to an improvement in families’ quality of life.

Minister for Public Health, Michael Matheson said: “The Scottish Government wants to see more patients benefit from the range of care that research like this makes available.

“That is why our Chief Scientist Office has provided £188,000 funding for this research into a debilitating condition, which was identified as a national priority for children and ‘s services in Scotland.  I hope this research will inform further improvement in the care and treatment of those affected by cystic fibrosis.”

Dr Janet Allen, Director of Research at the Cystic Fibrosis Trust said: “It is important parents know how vital physiotherapy is to prevent lung damage and fundamental they encourage treatment adherence in their at as early an age as possible.

“The support kit created from this research project will provide inspiration and encouragement.  We hope it will change families’ perceptions of and chest physiotherapy treatment by reassuring them that support is available and showing them how the treatment can be turned into an enjoyable experience.”

Bacteria in cystic fibrosis lung infections become selfish

Bacteria that infect the lungs of cystic fibrosis sufferers lose their ability to work together, becoming more selfish and less cooperative the longer the infection, say scientists.

X-ray of lungs

Researchers hope that by better understanding how the potentially-fatal infection changes over time it will make it easier to treat.

The team wanted to confirm whether Pseudomona aeruginosa bacteria become more inactive during chronic lung infections in cystic fibrosis patients.

‘This infection is fairly special because it’s very long-lived. You can normally cure a bacterial infection in a week but for people with cystic fibrosis, a lung infection can persist for years,’ explains Dr Ashleigh Griffin of the University of Oxford, lead researcher on the study published in Plos One.

‘During chronic infections, bacteria will change their behaviour towards each other. It’s interesting, because we can watch the change over time in patients.’

Griffin and her colleagues looked at four different so-called cooperative traits to see how the bacteria evolved during infections.

These included production of signal molecules bacteria use to communicate with other – called quorum sensing molecules, the concentration of protein-digesting molecules called proteases, the production of bright green pyoverdine, which is used to bind iron, and how good the bacteria were at forming biofilms. Biofilms are formed by groups of microorganisms in which cells stick to each other on a surface, in this case, a Cystic Fibrosis patients’ lungs.

They found that the longer the bacteria had been infecting the lung, the fewer signals they sent out to other cells, the less biofilm they formed and the less pyoverdin they produced.

Pyoverdin is a bright green secretion which the bacteria use to bind iron, which is essential for their respiration.

‘When cystic fibrosis sufferers first contract Pseudomona aeruginosa, it’s what you might call healthy. It’s making lots of this bright green pyoverdin, but over time it becomes very pale and quiet, as it’s not making this molecule and it doesn’t send out many signals. It seems odd because not communicating is not usually in the bacteria’s best interest,’ Griffin says.

Griffin explains that people have been very sceptical of the idea that the bacteria are getting themselves into a hole, where they can’t make these molecules and the team have encountered resistance to this idea.

The team are now interested to understand whether the bacteria are adapting to the lung environment or forced to behave in a way that may harm them by competition with neighbouring bacterial cells of their own species. They think there may be alternative explanations for what they’ve seen.

‘If bacteria don’t need iron because the lung is an iron-rich environment, then they won’t need to make this pyoverdin, or something else may be happening that means they don’t need to signal to one another as much,’ Griffin explains.

By understanding how these bacteria evolve, and why they choose certain behaviours over others, will make it easier for treatments to eventually be found.