Cystic Fibrosis Prognosis Depends on Body-Oxygen Levels

- Updated on August 3, 2019

Cystic Fibrosis Prognosis Depends on Body-Oxygen Levels 1By Dr. Artour Rakhimov, Alternative Health Educator and Author

Doctor provides cystic fibrosis prognosis Cystic fibrosis abnormalities and symptoms cannot exist or appear in conditions of normal body oxygenation. This was proven in several medical studies related to the function of the CFTR (cystic fibrosis transmembrane conductance regulator) gene (Bebk et al, 2001; Guimbellot et al, 2008; Yeger et al, 2001; Zheng et al, 2009).

Oxygen tensions in cells directly influence ionic pumps that transmit ions of chloride and sodium, water and other substances across the epithelium. All cells require oxygen for normal function. It is especially true for those cells and protein complexes that are involved in constriction (mitochondria) or active transport of substances (transport of ions in epithelium).

There are also independent studies unrelated to cystic fibrosis prognosis that showed that efficiency of ionic pumps in lungs depends on oxygen levels in a dose-dependent manner (Clerici & Matthay, 2000; Karle et al, 2004; Mairbaurl et al, 1997; Mairbaurl et al, 2002).

What are the causes of low body oxygenation in people with cystic fibrosis?

Minute ventilation in cystic fibrosis patients at rest

Number of
Normal breathing6 L/min Medical textbooks
Healthy subjects6-7 L/min>400Results of 14 studies
Cystic fibrosis15 L/min15Fauroux et al, 2006
Cystic fibrosis*13 (�2) L/min10 Bell et al, 1996
Cystic fibrosis10 L/min11Browning et al, 1990
Cystic fibrosis11-14 L/min6 Tepper et al, 1983
Cystic fibrosis*10 L/min10Ward et al, 1999
CF and diabetes*10 L/min7Ward et al, 1999
Cystic fibrosis16 L/min7Dodd et al, 2006
Cystic fibrosis18 L/min9McKone et al, 2005
Click here for all Cystic Fibrosis References

Medical studies that measured respiratory parameters found that CF patients suffer from chronic hyperventilation (breathing more than the medical norm). Overbreathing leads to alveolar hypocapnia (low CO2 levels in the lungs) which destroys the tissue of the lungs (see Cystic Fibrosis in Lungs). Minute ventilation in CF patients in these 7 medical studies ranged from 10 to 18 Liters per minute at rest (adjusted to 70-kg normal weight), while the medical norm is only 6 L/min.

Hence, low body-oxygen levels are caused by hyperventilation which worsens lung function and increases patient’s complaints about dyspnea (breathlessness) and inability to exercise. Therefore, breathing parameters predict stages, prognosis, and life expectancy in cystic fibrosis.

Medical research has conclusively proven that chronic hyperventilation has numerous negative effects on all systems and organs, including the destruction of lungs tissue, more problems with digestion and blood sugar control and many others. Fortunately, a large group of over 180 Russian MDs have developed and applied a breathing therapy for cystic fibrosis and revealed the following relationships between respiratory parameters and life expectancy or prognosis in CF patients.

Cystic fibrosis prognosis (chart)

Stages, life expectancy, and prognosis for cystic fibrosis patients and their respiratory parameters
Respiratory frequency Alveolar CO2Body-oxygen level Stages of the diseaseUsual life
Symptoms and prognosis
> 30 breaths/min<3.5%1-5 secondsEnd-stage diseaseSome monthsQuickly developing and severe problems with lungs (possible cor pulmonale), chronic indigestion, etc.
26-30 breaths/min3.5-4.0%6-10 sClinical stage (hospitalization or
palliative care is required)
Less than 1-2 yearsWorsened health state with progressive respiratory deterioration: bronchitis and bronchiolitis transform into bronchiectasis; possible complications include hemoptysis and pneumothorax.
20-26 breaths/min4.0-4.5%11-20 sModerate individual symptomsUp to 30-40 yearsA typical patient with classical symptoms of mild cystic fibrosis including chronic infections, poor exercise tolerance, inflammation, GI symptoms, …
12-20 breaths/min4.5-5.5%20-40 sInitial stage of the diseaseUp to 40-60 yearsMild GI symptoms; slight deterioration in lung function tests
< 12 breaths/min>5.5%over 40 s 24/7Normal healthNormalNo symptoms, normal life expectancy

Damage to lungs, sinuses, pancreas, liver, intestines, and sex organs in cystic fibrosis is proportional to the degree of pulmonary abnormalities, while a complete clinical remission in patients with cystic fibrosis takes place in cases of normalization of outer respiration. Patients with cystic fibrosis have an excellent prognosis and a normal life expectancy if they can normalize their respiratory pattern.

Bear in mind that cystic fibrosis prognosis depends on processes in the lungs and alveoli. As a result, the development of this medical condition leads to increased ventilation-perfusion mismatch and problems with the effective gas exchange. Another practical aspect related to cystic fibrosis is that for the overwhelming majority of CF patients, the lowest body oxygenation and heaviest breathing (or worst respiratory parameters) take place during early morning hours (or the last portion of the night sleep) due to the Sleep Heavy Breathing Effect (or nocturnal hypoxemia). Obviously, the morning CP (body-oxygen test – see below) is the main easy-to-measure parameter that influences prognosis, stages, life expectancy and quality of life in CF patients.

Clinical References: body oxygen and cystic fibrosis.

Bebk Z, Tousson A, Schwiebert LM, Venglarik CJ, Improved oxygenation promotes CFTR maturation and trafficking in MDCK monolayers, Am J Physiol Cell Physiol. 2001 Jan; 280(1): C135-45.

Clerici C, Matthay MA, Hypoxia regulates gene expression of alveolar epithelial transport proteins, J Appl Physiol. 2000 May;88(5):1890-6

Guimbellot JS, Fortenberry JA, Siegal GP, Moore B, Wen H, Venglarik C, Chen YF, Oparil S, Sorscher EJ, Hong JS, Role of oxygen availability in CFTR expression and function, Am J Respir Cell Mol Biol. 2008 Nov; 39(5): 514-21.

Karle C, Gehrig T, Wodopia R, Haschele S, Kreye VA, Katus HA, Bartsch P, Mairbaurl H, Hypoxia-induced inhibition of whole cell membrane currents and ion transport of A549 cells, Am J Physiol Lung Cell Mol Physiol. 2004 Jun; 286(6): L1154-60

Mairbaurl H, Wodopia R, Eckes S, Schulz S, and Bartsch P, Impairment of cation transport in A549 cells and rat alveolar epithelial cells by hypoxia, Am J Physiol Lung Cell Mol Physiol 273, 1997: L797–L806,

Mairbaurl H, Mayer K, Kim KJ, Borok Z, Bartsch P, and Crandall ED, Hypoxia decreases active Na transport across primary rat alveolar epithelial cell monolayers, Am J Physiol Lung Cell Mol Physiol 282: 2002, L659–L665,

Yeger H, Pan J, Fu XW, Bear C, Cutz E, Expression of CFTR and Cl(-) conductances in cells of pulmonary neuroepithelial bodies, Am J Physiol Lung Cell Mol Physiol. 2001 Sep;281(3):L713-21.

Zheng W, Kuhlicke J, Jackel K, Eltzschig HK, Singh A, Sjablom M, Riederer B, Weinhold C, Seidler U, Colgan SP, Karhausen J, Hypoxia inducible factor-1 (HIF-1)-mediated repression of cystic fibrosis transmembrane conductance regulator (CFTR) in the intestinal epithelium, FASEB J. 2009 Jan; 23(1): 204-13.

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