CFTR Effects in Cystic Fibrosis Are Reversible

Decades of clinical observations suggest that correct application of breathing techniques can reverse CF

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Abstract

Several medical studies recently discovered that CFTR mutation gene is controlled by levels of oxygen in cells. As a result, it is smart to investigate breathing parameters in people with cystic fibrosis and see how they can get more oxygen in body cells.

1. What is wrong with respiratory parameters in cystic fibrosis?

While most mainstream medical doctors continue to measure EVC (expiratory vital capacity), RV (residual volume), TLC (total lung capacity), FEV1 (forced expiratory volume in 1 second), FEV1 (forced expiratory volume in 1 second), FEF (forced expiratory flow 25-50-75%), DLCO (diffusion lung capacity for carbon monoxide) and some other secondary characteristics, it is obvious that cellular respiration and the state of the immune system are defined mostly by the level of tissue hypoxia. In its turn, oxygen transport is linked with outer respiration and alveolar CO2 since carbon dioxide is the main player in normal oxygen transport.

Why could oxygen levels in tissues are important in CF (cystic fibrosis)? Several recent medical studies suggested that CFTR gene mutation (the key cause for CF symptoms) is controlled by cell hypoxia. (For additional information about CFTR gene mutation and these recent medical discoveries, visit CFTR Mutation Is Triggered by Cell Hypoxia.) Hence, oxygenation of the body cells is the key factor for normal health in cystic fibrosis.

Therefore, the smart approach to pulmonary parameters in CF lies in the analysis of CO2 and cellular O2 changes. This analysis should be based on 3 factors: route of breathing (mouth vs. nose), respiratory mechanics (chest vs. diaphragm), and minute ventilation (to predict alveolar CO2). Let is focus on minute ventilation in CF.

Minute ventilation in cystic fibrosis patients at rest

Condition

Minute
ventilation

Number of
people

References

Normal breathing

6 L/min

Medical textbooks

Healthy subjects

6-7 L/min

>400

14 medical studies

Cystic fibrosis

15 L/min

15

Fauroux et a, 2006

Cystic fibrosis*

13 (±2) L/min

10

Bell et al, 1996

Cystic fibrosis

10 L/min

11

Browning et al, 1990

Cystic fibrosis

11-14 L/min

6

Tepper et al, 1983

Cystic fibrosis*

10 L/min

10

Ward et al, 1999

CF and diabetes*

10 L/min

7

Ward et al, 1999

Cystic fibrosis

16 L/min

7

Dodd et al, 2006

Cystic fibrosis

18 L/min

9

McKone et al, 2005

 

* – Some of these studies were conducted on subjects with abnormal average weights. As a result, minute ventilation for 2 studies (Bell et al, 1996) and (Ward et al, 1999) was adjusted to normal weight (70 kg). More detail about this medical summary can be found here: Cystic Fibrosis Cause: Chronic Hyperventilation

Conclusions. Available medical research suggests that a typical person with mild/moderate CF breathes at rest from about 10 to 18 liter or air per minute instead of 6 L/min (the medical norm). Note that numerous studies have found that modern healthy people have light and easy breathing at rest (with about 6-7 L/min for minute ventilation).

2. Hyperventilation causes tissue hypoxia and other systemic abnormalities that favor chronic inflammation, immune dysfunction, oxidative damage, destruction of lung tissue and other negative effects

When lungs are preserved, chronic hyperventilation leads to arterial hypocapnia (lack of CO2 in the arterial blood). This leads to hypocapnic vasoconstriction (a spasm of smooth muscles of arteries and arterioles) that causes reduced perfusion of vital organs (the brain, heart, liver, kidneys, colon, spleen, etc.)

Hypocapnia also leads to the suppressed Bohr effect (increased affinity between hemoglobin and oxygen molecules) that hampers release of oxygen in tissues (Carter & Grønlund, 1983; diBella et al, 1996; Dzhagarov & Kruk, 1996; Grant, 1982; Jensen, 2004; Kister et al, 1998; Lapennas, 1983; Tyuma, 1984).

These two effects are independent from each other, but both of then reduce oxygen transport to cells. As a result, it is a proven fact that hyperventilation reduces cell oxygen level in vital organs of the human body and body tissues in general (Laffey & Kavanagh, 2002; Nunn, 1987).

3. Hyperventilation worsen ventilation-perfusion ratio

Modern research suggests that alveolar hypocapnia (low CO2 in the lungs) causes various negative effects on the respiratory system, including:

1. Bronchoconstriction, which is a normal physiological reaction to alveolar hyperventilation present in all people.

2. Chronic inflammation, according to recent biomedical research, is either associated with or even caused by tissue hypoxia (reduced oxygen level in cells). Medical biologists have finally been able to pinpoint the mechanism. Among the key driving forces of chronic inflammation, according to recent research studies, are pro-inflammatory transcription factors, such as nuclear factor kappa B (NF-kappaB) and activator protein (AP)-1, and hypoxia-inducible factor 1. The link between tissue hypoxia and chronic inflammation is so strong, that there are dozens of recent research publications that use the term “hypoxic inflammation”.

3. Immunosuppression can be a result of chronic hypoxia. Here is a part of the recent abstract from one of these studies, “… Here, we attract attention to the possibility of iatrogenic exacerbation of immune-mediated tissue damage as a result of the unintended weakening of the tissue-protecting, hypoxia-adenosinergic pathway. These immunosuppressive, anti-inflammatory pathways play a critical and nonredundant role in the protection of normal tissues from collateral damage during an inflammatory response. We believe that it is the tissue hypoxia associated with inflammatory damage that leads to local inhibition of overactive immune cells by activating A2AR and A2BR and stabilizing HIF-1alpha. We show in an animal model of acute lung injury that oxygenation (i.e., inspiring supplemental oxygen) reverses tissue hypoxia and exacerbates ongoing inflammatory lung tissue damage…” (Hatfield et al, 2009).

D. Lung injury, according to Canadian biomedical researchers, is proportional to the degree of hypocapnia (Laffey et al, 2000; Laffey et al, 2003). Another medical study suggested, according to its title that Airway hypocapnia increases microvascular leakage in the guinea pig trachea (Reynolds et al, 1992) worsening airways injury. While evaluating effects of alveolar hypocapnia on ventilation-perfusion heterogeneity, it was found that “Hypocapnia worsens arterial blood oxygenation and increases VA/Q heterogeneity in canine pulmonary edema” (Domino et al, 1993), where VA/Q is the ventilation-perfusion ratio.

Hence, hyperventilation creates conditions for destruction of lungs in CF patients: Cystic Fibrosis in Lungs.

4. Hyperventilation contributes to impairment of thoraco-abdominal mechanics in CF

Predominantly upper chest breathing is a normal result of worsening cell oxygenation, chronic inflammation of airways, and reduced ventilation-perfusion ratio (all caused by alveolar hyperventilation). This effect is normal for obstructive lung diseases. Some studies even found that contribution of chest breathing correlates with degree of CF (Szeinberg et al, 1985; Pinet et al, 2003). These observations suggest that development and maintenance of diaphragmatic breathing 24/7 should be a part of any rehabilitative therapy for any group of people with CF.

5. Symptoms and stages of cystic fibrosis correlate with respiratory abnormalities

It is common for many health problems that worsening of hyperventilation leads to advance of various diseases. The same is true for cystic fibrosis. Medical doctors from tedical doctors from the Department of Medicine, University of Texas Health Science Center in Houston in their publication “Importance of respiratory rate as an indicator of respiratory dysfunction in patients with cystic fibrosis” observed that “Respiratory frequency was increased in the patients with cystic fibrosis compared with a group of healthy control subjects, as was minute ventilation and mean inspiratory flow. Respiratory frequency was a sensitive predictor of respiratory dysfunction” (Browning et al, 1990). More information about how breathing parameters predict stages and life expectancy in cystic fibrosis: Cystic Fibrosis Life Expectancy.

6. Breathing normalization is the therapy that can reverse CF

While the past attempts has focused mostly on training inspiratory muscles and application of pursed lip breathing for cystic fibrosis patients, clinical experience of Russian MDs indicates that application of the Buteyko breathing technique can reverse cystic fibrosis provided that the person normalizes his or her breathing pattern 24/7. Here is a YouTube video (Cough Medicine, Syrups, and Best Cough Suppressants) that explains that people with chronic cough, instead of taking ineffective medication and syrups, should learn how to cough with the mouth (and nose) closed and use simple breathing exercises to stop coughing.

Even better results are expected for application of breathing devices that increase CO2 in the lungs and provide resistance to flow of air. Among the most popular devices are DIY breathing device, Frolov breathing device and Samozdrav. Further details regarding breathing retraining in CF: Treatment for Cystic Fibrosis.

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