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
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
Stages of the disease
Usual life expectancy
Symptoms and prognosis
> 30 breaths/min
Quickly developing and severe problems with lungs (possible
cor pulmonale), chronic indigestion, etc.
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.
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, ...
Initial stage of the disease
Up to 40-60 years
Mild GI symptoms; slight deterioration in lung function tests
< 12 breaths/min
over 40 s 24/7
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.