Cystic Fibrosis Patient Survey Identifies Problems, Need for Better Clinical Management

Cystic Fibrosis Patient Survey Identifies Problems, Need for Better Clinical Management

A survey that explored the life of patients suffering from cystic fibrosis (CF) pinpointed a number of surprising facts about how patients manage their disease. The survey’s results, while providing some interesting details, are also likely to have implications for research, and identified key points for improvement in clinical practices.

The survey was conducted by GeneFo, an online platform for patient communities intending to connect people suffering various chronic diseases. Conducted by Dr. Yael Wilnai, geneticist and co-founder of GeneFo, the survey summarized the views of 2,400 CF patients. The results were presented as an infographic on the GeneFo CF web pages.

While patients with cystic fibrosis must consume calorie-rich food to stay on top of their disease, results showed that most patients chose meat as their preferred calorie-booster. This might be good for their protein intake, but far better fat sources exist. Full-fat yogurt, nuts, avocado, and salmon are examples of calorie-rich foods that also offer other health benefits. Clinicians might consider discussing diet with their patients since it seems that many patients lack an understanding of dietary recommendations.

One of the survey questions that has profound clinical implications was, “How are you feeling today?” The two responses that scored highest were “tired,” reported by 34 percent of patients, and “depressed,” accounting for another 39 percent. The data indicated that cystic fibrosis is taking its toll on the emotional well-being of patients.

Compared to some other chronic diseases, depression and fatigue are not considered direct symptoms of cystic fibrosis, so interventions to teach patients how to cope emotionally with the disease might improve their quality of life.

Even if patients often feel troubled by their disease, most seem to have found a way to relax. As many as 73 percent of the patients stated that music was their preferred relaxation method. The GeneFo experts added their recommendations, reminding patients that yoga, a technique proven to reduce stress, can actually also help CF patients with their lung and gastrointestinal symptoms. Likewise, walking might be a way both to relax and exercise – and it can be done while enjoying music. The GeneFo team also stressed that getting together online with other patients might also make people more relaxed.

Apart from these major points, the survey reported some surprising lack of associations. For example, despite that fact that the severity of the disease is a result of various mutations, the mutations did not affect the rate of hospitalizations. Also, the number of times a patient had been hospitalized did not alter their risk of becoming depressed.

The survey also explored how people felt about their medications, and found opinions to be highly polarized. Half the patients surveyed stated that voriconazole (Vfend), used to manage fungal infections, was completely ineffective, while half thought it was quite effective. Future studies investigating the characteristics of the two groups might give insights into disease differences, and provide clues for treatment optimization and new drug development.

Also, about half the patients reported having both classic cystic fibrosis symptoms and CF-related diabetes, while the other half reported having only diabetes symptoms.

A much more serious issue emerged when as many as half the patients surveyed said they were struggling to be compliant with treatment. Considering this finding, the GeneFo team urges cystic fibrosis clinic directors to put more educational and psychological resources to work in assisting patients to optimize their disease genefo


    • Adi says:

      Dear Kathy,

      Thank you so much for your kind feedback.
      I am involved with the wonderful CF community that shared the data for this survey, and I can tell you it is a delight to know that the data is used for good, for improving guidelines and optimizing care.

      I would of course be very glad to have you join the community (on, to connect with others with the same mutation set, learn about clinical trials in your area, and of course have your questions answered by our medical team!

      Have a wonderful day, and thank you again CF News Today 🙂

  1. D.Inns says:

    Being compliant with treatment? – well you can throw all the treatment you want at people without thinking of the consequences of the suffering that comes with and little reward. That is depressing.

    In countries where they cannot afford expensive treatment, the people rely on the Doctors advice with no safety net of the treatment. You are told NOT to go to the hospital for worry of catching infections that become a death sentence. They work hard on quarantine, exercise and physiotherapy.

    Keeping cross infection very low by not having everyone converging with all their antibiotic resistant infections in the same place. Common sense that in the UK barely exists because if you get an infection they have a “great” management ready to put you on the conveyor belt of a slow avoidable death!

    In fact in the UK clinic appointments are pushed onto CF people like they are too irresponsible to look after themselves and can’t be trusted to live in isolation of other sufferers to spare them from being infected with a nasty infection.

    In the UK it seems managing infections is more important than preventing them in the first place because managing is affordable. Well if Pseudomonas didn’t take 10-20 years to kill and just a few obvious weeks, I am sure it would be seen as unacceptable collateral damage.

    Ebola is harder to catch for a healthy person than PA for a CF person and look at the quarantine on ebola, but it is treated as perfectly acceptable risk to put CF people in near contact with others. If ebola was treated the same way as PA in CF, hundreds of millions of people would loose their life to ebola. So why is there this hypocrisy? Because PA takes decades not weeks to kill you and you can have a management team and all the drugs (But just a life of suffering of “Compliant” treatment, with little to no quality) and it can be blamed on CF progression, not the fact you got a slow killing infection from the very place you are meant to trust.
    But this is just my honest opinion.

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