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Cystic Fibrosis - New information and therapy for its treatment November 2016

Updated on November 9, 2016
65 roses
65 roses
Using a nebuliser
Using a nebuliser

Cystic Fibrosis is characterised by an inability to digest fats and lungs that produce too much mucus. It is the latter that causes the greatest problem leading to congestion, difficulty breathing and repeated infections caused by bacteria formed in the mucus.

There is as many as 9,000 people with cystic fibrosis in the UK and research suggests around one-in-25 carries the faulty gene which causes the condition.

The infection has been increasing over the past decade and it has been found that a dangerous infection commonly found in people with cystic fibrosis can spread between patients.

Doctors had previously thought that the Mycobacterium abscessus bacteria could only be caught from water and soil but hospitals need to change their thinking.

It is estimated that around 3-10% of cystic fibrosis patients in Europe and the US are infected with this resistant bacteria.

The bacteria is distantly related to that, that causes tuberculosis and is more likely to infect people with cystic fibrosis and could be related to problems with the immune system.

Although the particular infection has been increasing over the past ten years, doctors always believed it could not spread between humans. However, looking at DNA from almost 170 samples of the bacterium, and using that to create a family tree, researchers have found that it can be passed from person to person.

The study into the bacteria is being led by Dr Andres Floto, research director of the Cystic Fibrosis Unit at Papworth Hospital in Cambridge and principal investigator at the Cambridge Institute for Medical Research, who has said that the results had prompted them to completely rethink their infection control.

They that believe it gets aerosolised when people cough and because the bacteria is resistant it remains in the air. In consequence all inpatients at Papworth are now treated in negative pressure rooms to prevent the spread of airborne bacteria and those with the infection are cared for away from the cystic fibrosis unit to avoid the chance of any cross contamination.

Professor Julian Parkhill, head of pathogen genomics at the Wellcome Trust Sanger Institute, said: "By sequencing the complete genomes of bacteria we can accurately describe where they have emerged from and how they pass from person to person. This new information has led to rapid changes in how people with cystic fibrosis are cared for in hospital to protect them from this emerging threat. People with cystic fibrosis who have concerns about this issue should speak to their clinician."

Parents of children with CF are taught physiotherapy to help, several times a day, to drain the child’s lungs and at this stage essential oil treatment can be carried out.

To use essential oils to their best effect we need are oils that are expectorant, mucolytic and anti-infectious and because the treatment is over a long period and required constantly the blend needs to be varied regularly. The blend should consist of one immunostimulant, one resin and one other and varied each week but for no longer than 3 weeks.

The oils should be:

Resins – Benzoin, Frankincense, Myrrh and Elemi

Anti-infectious and immunostimulant – Lavender, Manuka, Ti-tree, Niaouli, Ravensara

And – Globus Eucalyptus, Radiata Eucalyptus, Citriodora Eucalyptus, Inula, Sandalwood, Pine, Bergamot and Cedar.

Use Peach kernel or Apricot kernel carrier oil and add no more than 1% of the essential oil blend for young children and 2% for older. This should be massaged into the chest and back and it will make it much easier for the child to cough up the mucus and reduce the risk of infection. The same essential oil blend used in a steam inhaler, bath or aerosol diffuser is highly efficient for respiratory problems of any sort.

Docosahexaenoic acid (DHA) is one of the omega-3 fatty acids which is normally found in fish oils. The amount is very variable being anything from 70 to 310mg. In patients suffering from Cystic Fibrosis there has been shown to be an imbalance between levels of DHA and AA (arachidonic acid) which is another fatty acid. DHA has been used to reduce aggressive behaviour in patients who are highly stressed.

Boswellia is a fragrant resin supplement used in ayurvedicmedicine as an anti-inflammatory. It is believed to be beneficial to people with cystic fibrosis.

Finally much is said about the treatment of a child with CF but often the parents or carers are overlooked. Always ensure that they have access to essential oils for reducing stress and relaxation.

Recent development work, in early 2015, has produced a new cystic fibrosis therapy that could profoundly improve patients' quality of life. So many patients often die before their 40th birthday as mucus clogs and damages their lungs and leaving them prone to infection. A major trial in America using 1,108 patients, reported in the New England Journal of Medicine, suggests that a certain combination of drugs could bypass the genetic errors that cause the disease and may even increase life expectancy. The Cystic Fibrosis Trust said it could improve the lives of many of the 2,500 babies in the UK born with cystic fibrosis.

It seems that sufferers’ inherit faulty DNA from their parents which results in damage to the microscopic machinery that controls salt and water levels in the linings of the lungs. The result is a thick mucus that inexorably damages the lungs. While antibiotics help prevent infection and other drugs can loosen the mucus, nothing deals with the fundamental problem for most patients. The combination of new drugs - lumacaftor (code number VX-809 Vertex Pharmaceuticals) and ivacaftor (trade name Kalydeco, developed as VX-770 also from Vertex) - were designed to repair that microscopic machinery. The trial showed that those patients given the cocktail for 24 weeks had improved lung function and also gained weight in the trial.

Queen's University Belfast said this is very exciting and it really demonstrates that we can correct the basic defects in cystic fibrosis and is likely to become a fundamental treatment. Starting early with children may prevent the disease process developing if we correct the basic defect early in life.

Treatment for the young seems to offer the greatest success, but will this improve survival for older people with cystic fibrosis? The anticipation is that it would but current research cannot confirm this for sure yet. There are however, many types of error in the DNA that can culminate in cystic fibrosis. The proposed treatment combination should work on around half of patients, while one of the drugs on its own corrects a small proportion of errors but new treatments are still required for the remaining patients. The therapy is being examined by regulators around the world.

Additional research

A new therapy that corrects the underlying cause of cystic fibrosis and could transform treatment, has been announced.

Only half of the people with cystic fibrosis manage to live into their defects in their DNA mean they produce thick, sticky mucus that clogs and inexorably damages their lungs.

Data presented at the North American Cystic Fibrosis Conference has indicated that a drugs combination - which enables production of a low viscosity mucus thus slowing the irreversible decline in lung function.

Errors in people's DNA - or genetic code - can lead to defective microscopic machinery in the lungs and instead of producing a normal protective layer of mucus, they get the balance of salt and water wrong and it becomes damaging.

While antibiotics help prevent infections settling in and drugs can loosen the mucus, nothing deals with the fundamental problem for most patients.

Early studies have proved “Very reassuring” and had suggested the drugs lumacaftor and ivacaftor could alter the microscopic machinery so they made the lower viscosity mucus.

Initially those studies followed patients for just a 24-week period, but now doctors have assimilated nearly two years of data on both of them.

The drug combination is clearly not a cure, but the researchers said the untreated patients would expect their lung function to decline by 2.3% a year, but this fell to 1.3% in those given the therapy. In addition, they were also less likely to need hospital treatment.

Dr Michael Konstan, who led the trial from the Case Western Reserve University School of Medicine, said: "These data suggest that the benefits of lumacaftor/ivacaftor are sustained and indicate that the medicine may modify the progression of cystic fibrosis lung disease by treating its underlying cause."

Dr Stuart Elborn, from the Royal Brompton Hospital in London where some of the patients were treated, told the BBC News website: "This is an incremental advance rather than a breakthrough, but it is very reassuring that the effect does not wear off. I'm really excited by the therapy and also the pipeline of other powerful drugs that could get us closer to a cure."

The therapy is not available on the NHS after it was rejected by funding bodies in England and Scotland as it costs more than £100,000 a year for each patient.

There are also many types of error in the DNA that can culminate in cystic fibrosis.

This treatment combination should work on around half of patients, while one of the drugs on its own corrects a small proportion of errors. New treatments are still required for the remaining patients and one in every 2,500 babies in the UK has cystic fibrosis.

© 2012 Peter Geekie


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    • Peter Geekie profile imageAUTHOR

      Peter Geekie 

      6 years ago from Sittingbourne

      Thank you urmilashukla, it is a difficult subject.

      Kind regards Peter

    • urmilashukla23 profile image


      6 years ago from Rancho Cucamonga,CA, USA

      Great information. Thanks for posting it. Voted up!


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