Surviving cystic fibrosis without modulators

Benefit from the experience of an old CF patient

latest extensions: 
The Vest is not efficient and therefore not necessary (01.08.24)
Actually self-evident: No smoking! (01.08.24)

 

If you or your child are not currently receiving or being reimbursed for modulators (Trikafta), I would like to share some thoughts with you: I was born in 1965, at a time when the life expectancy for cystic fibrosis patients in Germany (as in most other countries) was only a few years. These few years of life expectancy have grown with me because knowledge, experience and medical options have continued to improve. In 2020, I have already lived with cystic fibrosis for 55 years, am still breathing with my first lung (i.e. without a transplant), have run three marathons and have a lung function greater than 80% of the expected value for healthy people of my age, even though my mutation dF508 is homozygous, i.e. typical cystic fibrosis. (Foto: svklimkin auf Pixabay)

Of course you will and should try everything to get you or your child on Trikafta. However, while you are "waiting" for modulators, you must (i.e. you should) use all methods possible to maintain your lung function during this time. There is no sensible reason to become inactive while waiting for Tricafta and accept unnecessary deterioration.

Preliminary remark: I am not a doctor and these lines are not medical recommendations, but my experiences as a patient. If you would like to change your therapy or are unsure about individual points, please always ask your cystic fibrosis doctor.

Second preliminary remark: The course of the disease can vary greatly in CF from person to person: The different CFTR variants have an influence, there are also other modifying genes that influence the personal course of the disease, and external influences (family, environment, work) can also only be regulated to a limited extent by oneself. This also means that not everyone can achieve the same positive effects through their own commitment. So if you don't achieve your own successes at first, that's no reason to feel guilty...

So here is some simple advice from a “survivor”:

The lung is not a compact filled organ, but a fantastic machine for highly efficient gas exchange between the air and blood circulation. The inner surface of the human lung is remarkably large at around 70 to 100 square meters and contains many millions of tiny alveoli (pulmonary alveoli). The alveoli must be in contact with the air at all times, otherwise they die. The lungs must therefore be fully ventilated at all times, i.e. air must be able to flow in and out of every region of the lungs. If the alveoli of a lung region are closed off from the air supply, germs multiply, inflammation occurs and this leads to the death of lung tissue and, in the long term, to a loss of lung function, i.e. the FEV1 (the amount of air exhaled in the first second in litres/s) decreases. For ventilation, it is therefore necessary to thoroughly remove the viscous secretions that accumulate in the lungs in cystic fibrosis several times a day.

The most important measures for this are:

Lung cleansing through physiotherapy

The thick secretion from the cystic fibrosis lungs cannot simply be coughed up. Before this becomes possible, it must be loosened and transported into the bronchial tubes. To do this, patients learn "autogenous drainage according to Chevallier". An alternative method for clearing secretions is called "stacked breathing" and is explained here.

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Inhaling hypertonic saline twice daily

Lung cleansing works better if you inhale (approx. 5 ml) hypertonic saline solution two to three times a day beforehand. This is because the salt settles on the surface of the lungs and attracts moisture, just as a slice of cucumber on a plate forms small drops of water when it is salted (known as osmosis). Seawater contains about 4% table salt (also known as sodium chloride, NaCl), and in Australia it was observed that cystic fibrosis patients were much healthier because they had practiced wave surfing in the sea spray for several hours a day (read the story here). You can also try higher  concentrated NaCl solutions, I always inhale with 7%, and there are reports that 10% to a maximum of 20% saline solution was also effective.

In case of need: Produce NaCl solution yourself

If you can't buy a ready-made inhalation solution from the pharmacy, you can make it yourself: Boil one litre of normal tap water in a thoroughly cleaned container for about 10 min (to kill possible germs) and then let it cool to room temperature. For each percentage of saline solution, weigh out 10g of pure table salt (sodium chloride, not iodized and not fluoridated). So for a 5% solution you need 5*10g = 50g salt, or for half a litre of water 25g etc. Add the salt to the cooled water and stir well until the salt has completely dissolved. Pour the solution into a clean, sealable container and store it in a cool, dark place. Only use the solution for a few days to minimize the risk of contamination with bacteria and fungi.

Sporting activity is essential

The best way to ventilate the lungs is sporting activity or exercise in general. Evolution has optimized the human body over several million years for the life we led until about 10,000 years ago: Gathering berries and hunting animals, covering between 10 and 20 km a day in the fresh air, some of it running. Then we became sedentary and reduced our daily exercise to a few 100 meters per day. Sport is already healthy for normal people and protects against most modern diseases, but it's vital for cystic fibrosis patients. Chilren's Hospital in Cincinnati shows all the benefits of exercise for people with cystic fibrosis (CF).

Children exercise playfully and voluntarily if we give them the opportunity. Older patients should learn to jog, starting very slowly with 15 minutes a day and gradually increasing. A medical examination should clarify in advance how much exertion is possible, e.g. in order not to overload the heart in the case of severe lung damage. Several cystic fibrosis patients have become marathon runners with as a consequence almost healthy lungs, and many more could achieve this through training. I started jogging around the year 2000 and have now run four marathons (42km) and once 70km in one day. Running has saved my life, and getting the lungs to sweat from the inside is much more important than any medication.

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Get networked

You should actively network with other patients and affected families. Nothing works better for coping with a complicated metabolic disorder than the exchange of experiences, tips and knowledge between those affected. Get involved in self-help work, join a cystic fibrosis association if there is one in your country, or found one. I have been involved in cystic fibrosis self-help since 1988 and have not only been able to help other people, but have also benefited a lot personally. I always got the most important suggestions for improving the therapy from other patients. In many countries, cystic fibrosis doctors warn against people with cystic fibrosis meeting physically. From my experience, however, it is possible to follow certain rules to reduce the risk of germ transmission so that it is small compared to the great benefit that such encounters make possible. In collaboration with doctors, the German Cystic Fibrosis Association has developed a concept that I believe makes it possible for patients to meet: Guidelines for hygienic behavior at events of the German CF Association (Foto: Gerd Altmann auf Pixabay)

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Use nose and mouth as intended by nature

Teach your child to use their body parts in the way nature intended: We should breathe through the nose, this not only cleans the air, but also warms and moisturizes it before it flows into the lungs. Mouth breathing is only intended for emergencies, for example if the nose is blocked due to an infection. It is just as important to chew each bite of food before swallowing (e.g. 20 times) until the food has been reduced to a pulp, as the stomach has no teeth and finds it very difficult to deal with larger chunks.

Why daily therapy is necessary

Be consistent and "forceful" with regular lung cleansing and exercise. The human lung has large reserves; people can still do sport with half a lung. The disadvantage of this is that we don't notice when the lung function drops from 100 to 90, from 90 to 80 or from 80 to 70%. Only measuring lung function at least four times a year can indicate this. Prof. Warren J. Warwick (+2016), an American pediatrician and professor of pediatric pulmonology at the University of Minnesota, led the cystic fibrosis outpatient clinic at the University of Minnesota from 1962 to 1999 and achieved exceptionally good health and survival scores. He explaines the necessety of daily therapy on the basis of a mathematical calculation with (fictive) propabilities: “It is important to do treatment every day because risks are day-by-day events and prevention is a day-by-day process: The risk of a person without CF of developing a serious lung infection today is about 0.01%. The chance of staying well, of not catching serious lung infection today requiring antibiotics treatment, is therefore about 99.99%. The risk of a person with CF, who does not do therapy every day, of developing a serious lung infection today requiring antibiotics treatment, is about 0.5%. The chance of staying well and not catching serious lung infection today is therefore about 99.5%. The risk of a person with CF, who does therapy every day, of developing a serious lung infection today requiring antibiotics treatment, is about 0.03%. The chance of staying well and not catching serious lung infection today is therefore about 99.96%. How these risks multiply up over a year becomes clear from this table:

CF care	Today’s chance of being well	Today’s risk of being sick	Chance of being well this year	Chance of being sick at least once this year
No CF	99.99%	0.01%	96%	4%
CF, no therapy	99.5%	0.5%	16%	84%
CF plus therapy	99.97%	0.03%	90%	10%

Doing therapy daily as prescribed can reduce the risk of a pulmonary infection 8.4 times as compared with not doing it daily. A pay off, staying well, occurs about every six weeks.” (note: The chance of being well over a year is calculated as the chance of being well today with the power of 365 days: 0,9997^365 = 0,9).

Don't worry - be happy!

Don’t worry too much about your disease. Prof. Warren J. Warwick claimed that cystic fibrosis was not a disease and wrote: “If CF is not a disease what is CF? Cystic Fibrosis is the inheritance of a mutation in two CFTR genes, one each from both father and mother. These two inherited genes produce an increased risk of the person to acquire a large number of diseases. These diseases are common in the general population and include: bronchitis, pneumonia, sinusitis, nasal polyps, pancreatic insufficiency, diabetes, male infertility, and less frequently cirrhosis, symptomatic gallstones, and even kidney stones. While this seems like a horrid number of unwanted problems there is a good side to this information. A person who has genetic cystic fibrosis and the CF Doctor know which specific illnesses he is at risk for and so will be able to take preventive precautions. Everybody has unknown genetic risk factors for other diseases but do not know what the risks or diseases are. So they have no idea of what actions might be taken to prevent their unknown potential problems.”

Coughing is important and good

Never instruct your child to suppress a cough. Coughing is a sign that secretions have built up and the lungs need to be cleared. If this warning signal is suppressed, it can easily lead to the lungs not being cleaned enough and to a loss of lung function in the long term. Warwick wrote on this issue: “Coughing is important and good. Even infants with no detectable lung disease need to be encouraged to cough frequently. Every cough compresses the chest, narrows the airway diameters and forces the expiratory air to flow at higher frequencies. This accelerates the flow of mucus towards the mouth from which the sputum can be spit out or swallowed. Every patient who has the CF genes needs to develop a style of life in which each can cough many times a day in a way that their peers do not notice or ignore the frequent coughing. All CF Patients need to learn to never suppress a cough. Cough suppression is a dangerous accomplishment. Not to cough in order to be socially acceptable is not acceptable. The goal is to learn how to cough frequently so no one notices the coughing.”

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Do you have questions about cystic fibrosis?

Your questions are often questions that others have already had! That's why there is the Cystic Fibrosis Expert Advice ecorn-cf. I have created a guide to help you find answers to your questions: How to use the European Expert Advice on Cystic Fibrosis.

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The Vest is not efficient and therefore not necessary

Perhaps you have the impression that your child with cystic fibrosis needs the “Vest”, a physiotherapy equipment also known as a High Frequency Chest Wall Oscillation device (HFCWO). It creates electric air pulses and pushes these into an inflatable jacket (vest) which vibrates the chest of the person wearing it. In Germany, almost nobody uses it, because compared to physiotherapy air clearance methods and sportive activity, the Vest has low effect and is inefficient considering the time spent within the Vest. And even worse, to cite the CF-Trust (UK): Two recent well-constructed studies showed that people using the High Frequency Vest Oscillation, (the Vest) as their chosen airway clearance technique, may get more infections and get them more quickly than people using other types of airway clearance techniques. (Foto: respirtech.com)

"These two studies were done in children. There are at least two studies in adults that show that less or just the same amount of secretions were cleared in people using the Vest compared to people using other techniques. There are at least two studies demonstrating an improvement in lung function when using other airway clearance techniques when compared to the vest. One study identified that a significant drop in oxygen saturations can occur in people with moderate to severe disease when using the Vest when compared to other techniques, recommending that oxygen monitoring should be carried out in this group". You may wish to read the details in the Cochrane-Report on "Airway clearance techniques for cystic fibrosis". My opinion: For most people with CF, the Vest is not necessary.

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actually self-evident: No smoking!

Cigarette smoke is especially harmful to people with cystic fibrosis. If you smoke as parents, I strongly encourage you to quit. Your CF doctor can provide you with resources to help you quit. Until you are able to quit, there are ways to reduce your child’s exposure to smoke: Don’t smoke in the home or car, even if windows are open. Always sit in the non-smoking sections of restaurants or visit restaurants that are smoke-free. Do not smoke when your child is near you, even if you are outside. If choosing a child care provider, choose one who does not smoke

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Author: Stephan Kruip, CF, born 1965 in Germany, married, father of three sons, physicist and patent examiner