Cystic Fibrosis Prognosis Depends on Body-Oxygen Levels
Cystic fibrosis abnormalities and symptoms cannot exist or appear in conditions of normal body oxygenation. This was proven in several medical studies related to 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
|Normal breathing||6 L/min||-||Medical textbooks|
|Healthy subjects||6-7 L/min||>400||Results of 14 studies|
|Cystic fibrosis||15 L/min||15||Fauroux et al, 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|
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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 have numerous negative effects on all systems and organs, including 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 CO2||Body-oxygen level||Stages of the disease||Usual life
|Symptoms and prognosis|
|> 30 breaths/min||<3.5%||1-5 seconds||End-stage disease||Some months||Quickly developing and severe problems with lungs (possible cor pulmonale), chronic indigestion, etc.|
|26-30 breaths/min||3.5-4.0%||6-10 s||Clinical stage (hospitalization or
palliative care is required)
|Less than 1-2 years||Worsened health state with progressive respiratory deterioration: bronchitis and bronchiolitis transform into bronchiectasis; possible complications include hemoptysis and pneumothorax.|
|20-26 breaths/min||4.0-4.5%||11-20 s||Moderate individual symptoms||Up to 30-40 years||A typical patient with classical symptoms of mild cystic fibrosis including chronic infections, poor exercise tolerance, inflammation, GI symptoms, ...|
|12-20 breaths/min||4.5-5.5%||20-40 s||Initial stage of the disease||Up to 40-60 years||Mild GI symptoms; slight deterioration in lung function tests|
|< 12 breaths/min||>5.5%||over 40 s 24/7||Normal health||Normal||No symptoms, normal life expectancy|
Damage to lungs, sinuses, pancreas, liver, intestines, and sex organs in cystic fibrosis is proportional to 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 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, development of this medical condition leads to increased ventilation-perfusion mismatch and problems with effective gas exchange. Another practical aspect related to cystic fibrosis is that for 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.
References: O2 and cystic fibrosis.
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