What Causes Cystic Fibrosis? Low O2 in Body and Ion Pumps
What is the cause of cystic fibrosis
(CF)? The cause of cystic fibrosis on the cellular level is tissue hypoxia
or low oxygen levels in body cells. This makes complete sense since ionic pumps
require oxygen, and it has been known for decades that chloride and other pumps
are ineffective in conditions of tissue hypoxia even in normal people
(Clerici & Matthay, 2000; Karle et al, 2004; Mairbaurl et
al, 1997; Mairbaurl et al, 2002).
However, there are even more specific findings. It
was recently discovered that CFTR mutation gene (cystic
fibrosis transmembrane conductance regulator) is
controlled by levels of oxygen in body cells in a dose dependent manner (Bebk
et al, 2001;
Guimbellot et al, 2008; Yeger et al, 2001; Zheng et al, 2009).
These revolutionary discoveries force us to reconsider the real causes of cystic
fibrosis. It is not just a genetic disease. Advance of cystic fibrosis requires
low oxygen levels. Normal oxygen levels do not allow development of the thick mucus and respiratory infections,
which are the main symptoms of cystic fibrosis. What is the cause of low
body oxygenation in people with cystic fibrosis?
All available medical evidence has shown the presence of chronic hyperventilation in CF
patients. Medical research has also confirmed the injurious effects of alveolar
hypocapnia on lung tissue. Furthermore, medical 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).
of cystic fibrosis occurs when the body oxygen level drops below 20 seconds
(e.g. even if it only drops this low for some hours as during the early morning
hours when the patients is at rest sleeping). See the links below for
description of the Control Pause (CP) as a means of assessing body oxygen
Note that most modern people have less than 20 s in the morning and, hence,
having genetic predisposition to cystic fibrosis means gradual development of
this fatal health condition for modern man. Poor results for the body-oxygen
test (less than 20 s) cause the abnormality related to regulation of the
chloride transport across epithelial cells on mucosal surfaces and this causes
decreased secretion of chloride ions and increased reabsorption of sodium ions
and water molecules across epithelial cells. This leads to reduced thickness of
epithelial lining fluid and lowered hydration of mucus. Hence, mucus become more
as for people with bronchitis and sinusitis, less than 20 s
CP means the suppressed immune system. That causes frequent infections in
sinuses and lungs and chronic inflammation. As a result, during physical
exercise people with cystic fibrosis often develop coughing, wheezing, chest
tightness and/or breathlessness. Such clinical picture is typical for other
chronic disorders (asthma, bronchitis, etc.) in conditions of cell hypoxia
and elevated lactic acid on blood. Anaerobic energy production mechanism in
cells is also caused by less than 20 s CP. This is the key factor that
triggers expression of the CFTR (cystic fibrosis transmembrane conductance regulator)
gene. Thus, while a genetic mutation determines susceptibility to cystic
fibrosis, chronic hyperventilation initiates expression of and exacerbates the
pathological processes with mucosal abnormalities, due to
pathological changes in CFTR, take place in
pancreas, sweat glands, GI tract, and other exocrine tissues. Instead of
helping the immune system to fight pathogens, the increased viscosity of
mucosal secretions slows down immune responses and promotes infections
causing more severe symptoms of CF.
Too viscous mucus can also block small tubes that transport digestive
enzymes from the pancreas to the small intestine. Enzyme deficiency causes inability to
digest foods and development of pathological bacterial flora in the
gut causing foul-smelling, greasy stools; constipation and distended abdomen;
reduced weight and poor growth in children; and even blockage in the intestines,
especially in the newborn. Digestive complications include
pancreatic insufficiency, meconium ileus, peptic ulcer, rectal prolapse,
and gastroesophageal reflux. Among typical hepatic involvements are fatty
liver and liver cirrhosis.
Cystic fibrosis is reversible
Russian medical doctors have found that symptoms of cystic fibrosis are
reversible, if the person slows down his or her breathing pattern back to the
medical norm and achieves more than 40 s for the body-oxygen test.
So far, hundreds of Russians with cystic fibrosis reversed their
health problems and achieved normal health. For more information about health
zones related to the body oxygen level and typical symptoms in each zone, visit
cystic fibrosis links below.
YouTube video: Trailer of the Amazon Kindle Book "Cystic Fibrosis:
Defeated with Higher Body Oxygenation"
Clinical References: What Causes 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.
Browning IB, D'Alonzo GE, Tobin MJ, Importance of respiratory rate as an indicator of respiratory dysfunction in patients with cystic fibrosis, Chest. 1990 Jun;97(6):1317-21
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.
Cystic fibrosis (From Wikipedia.org)
Cystic fibrosis (From MayoClinic.org)
What is Cystic Fibrosis? What Causes It? (From WebMD.com)
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