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Cystic fibrosis

Category: Illnesses and disabilities

Type

Involuntary

Introduction and description

Eva Markvoort died of cystic fibrosis but kept a blog about her experiences
which becamse so well known that a documentary film -   65_RedRoses was
made about her.  The film follows Eva as she lives her life undaunted by her
disease, waiting for a lung transplant. 
She died on March 27, 2010, aged 26

Cystic fibrosis is a recessive genetic disease which affects the entire body, causing progressive disability and often early death.  The name cystic fibrosis refers to the characteristic scarring (fibrosis) and cyst formation within the pancreas.

 CF is caused by a mutation in the gene for the protein cystic fibrosis transmembrane conductance regulator (CFTR). This gene is required to regulate the components of sweat, digestive juices, and mucus. Although most people without CF have two working copies of the CFTR gene, only one is needed to prevent cystic fibrosis. CF develops when neither gene works normally.

Kyle Monk photography - see references

There is no cure for cystic fibrosis, but there are several treatment and management methods, many of which put a heavy burden upon the families of the sufferer. This emphasis upon managing the disease has resulted in prolonged life expectancy, which may be regarded as both a curse and a blessing, as families are often heavily emotionally affected by seeing the progress of the disease in their child. 

In 1959, the median age of survival of children with cystic fibrosis was six months. In the United States, the life expectancy for infants born in 2008 with CF was 37.4 years, based upon data compiled by the Cystic Fibrosis Foundation.   The median survival age in Canada increased from 24 in 1982 to 47.7 in 2007, based on data compiled by the Canadian Cystic Fibrosis Foundation.  This means that as adults, the dying CF sufferer often is able to relate their spiritual experiences – and spiritual experiences they do have, principally from the problems with lung infections.

The map below shows both the incidence rate and the gene mutations.  In terms of countries, Ireland has both the highest incidence of CF in the world and the highest carrier rate in the world. The state of Quebec, however, tops the list with the highest incidence overall.  Cystic fibrosis is Ireland's most common life-threatening inherited disease. Ireland also has the largest proportion of families with more than one child suffering from CF.

 

Symptoms

A multitude of symptoms, including sinus infections,  diarrhea, and infertility result from the effects of CF on the body.  Two of the symptoms of cystic fibrosis are poor growth and poor weight gain despite a normal food intake. The causes of growth failure are multi-factorial and include poor absorption of nutrients through the gastrointestinal tract and increased metabolic demand due to chronic illness.

 

But the most frequent and noticeable symptoms of CF are an accumulation of thick, sticky mucus, frequent chest infections, coughing and shortness of breath.  Difficulty breathing is the most serious symptom and results from frequent lung infections.

Lung disease results from clogging of the airways due to mucus build-up, decreased mucociliary clearance and resulting inflammation.  Inflammation and infection cause injury and structural changes to the lungs, leading to a variety of symptoms.

In the early stages, incessant coughing, copious phlegm production, and decreased ability to exercise are common. Many of these symptoms occur when bacteria that normally inhabit the thick mucus grow out of control and cause pneumonia.

 

In later stages, changes in the architecture of the lung such as pathology in the major airways (bronchiectasis) further exacerbate difficulties in breathing. Other symptoms include coughing up blood (hemoptysis), high blood pressure in the lung (pulmonary hypertension), heart failure, difficulties getting enough oxygen to the body (hypoxia), and respiratory failure requiring support with breathing masks such as ventilators.

The lungs of individuals with cystic fibrosis are colonized and infected by bacteria from an early age. These bacteria, which often spread among individuals with CF, thrive in the altered mucus, which collects in the small airways of the lungs. This mucus leads to the formation of bacterial microenvironments known as biofilms that are difficult for immune cells and antibiotics to penetrate. Viscous secretions and persistent respiratory infections repeatedly damage the lung by gradually remodeling the airways which makes infection even more difficult to eradicate.

In effect, the poor CF sufferer experiences a gradually diminishing supply of oxygen and a permanent hypoxic state.

 

Cause

First I will quote the official version.

At 21, Laura Rothenberg authored the memoir Breathing for a Living,
about living (and living well), with cystic fibrosis and her experiences
She died on March 20, 2003 aged 22.

 

CF is caused by a mutation in the gene cystic fibrosis transmembrane conductance regulator (CFTR). The most common mutation, ΔF508, is a deletion (Δ signifying deletion) of three nucleotides that results in a loss of the amino acid phenylalanine (F) at the 508th position on the protein. This mutation accounts for two-thirds (66–70%) of CF cases worldwide and 90% of cases in the United States; however, there are over 1500 other mutations that can produce CF. Although most people have two working copies (alleles) of the CFTR gene, only one is needed to prevent cystic fibrosis. CF develops when neither allele can produce a functional CFTR protein. Thus, CF is considered an autosomal recessive disease…… There is increasing evidence that genetic modifiers besides CFTR modulate the frequency and severity of the disease. One example is mannan-binding lectin, which is involved in innate immunity by facilitating phagocytosis of microorganisms. Polymorphisms in one or both mannan-binding lectin alleles that result in lower circulating levels of the protein are associated with a threefold higher risk of end-stage lung disease, as well as an increased burden of chronic bacterial infections

But what caused the gene to mutate?  And why do people keep on appearing with cystic fibrosis whose mothers and fathers have no such gene mutation?

 

If we take one well known example, Fraser Brown is the son of the ex-British Prime Minister Gordon Brown. He was diagnosed with cystic fibrosis as a result of routine screening tests for newborn babies. The finding on Fraser was a 'surprise' to the Brown family as they 'were not aware that they carry the CF defect'.  They probably don't.

As in all these illnesses we may have to look for more than one cause.

Viruses

One answer may be viruses.  But which virus is not yet known.  We know for example that viruses are perfectly capable of affecting the fetus in the womb and altering genes.

Fetal membranes (FM) derived mesenchymal stromal/stem cells (MSCs) are higher in number, expansion and differentiation abilities compared with those obtained from adult tissues, including bone marrow. ….. We present here evidence that FM-MSCs are fully permissive to infection with Herpes simplex virus 1 and 2 (HSV-1 and HSV-2), Varicella zoster virus (VZV), and Human Cytomegalovirus (HCMV), but not with Epstein-Barr virus (EBV), Human Herpesvirus-6, 7 and 8 (HHV-6, 7, 8) although these viruses are capable of entering FM-MSCs and transient, limited viral gene expression occurs. PMID:  23940750

One virus which appears to be implicated is RSV.  Many babies with lung diseases are born with the virus, which means that the virus may have been passed from mother to baby, for example:.

Morbidity due to respiratory syncytial virus (RSV) disease is still high in infants and children worldwide during the first two to five years of life.  …… Over the last years several rare diseases have been identified demonstrating high morbidity associated with RSV disease, ….. including children with malignancy, congenital and acquired immune deficiency, Down syndrome, neuromuscular impairment, cystic fibrosis, congenital diaphragmatic hernia and other severe respiratory diseases PMID:  25132870

and

Of 36 patients hospitalized for lower tract infection from October 2012 to April 2013, 18 (50%) newborns were infected with RSV. The study included 36 neonates. Patients with RSV-positive infants at admission had greater need for respiratory support, supplemental oxygen and prolonged stay in the NICU.  PMID: 25211292

 

One of the difficulties for a researcher in this area is that it is not always clear whether a child has caught a virus subsequent to being born because of their compromised immune system and frail condition, or whether the virus was with them at birth and simply multiplied over time.

The lungs of these poor children are full of mucus because mucus is the body's mechanism of trapping invaders - viruses and bacteria, as such the body is doing a sterling job to help protect the child, even though the result may mean the child is short of breath. For example, here is another virus which may be implicated

Sixty-four sputum samples collected from 46 adult patients were included in the study: 33 samples were collected during exacerbation of CF, and 31 during the stable phase. After extraction, nucleic acids were tested for the presence of respiratory viruses. …. Viruses were found in 25% of samples. The rhinovirus [HRV] viruses were the most frequently detected followed by coronaviruses. Only the rhinovirus [HRV] detection was significantly associated with the occurrence of CF pulmonary exacerbation …. HRV were the most frequently detected viruses; their detection was significantly associated with the occurrence of an exacerbation. The reality of viral recombination between HRV was demonstrated in CF patients for the first time, raising the role of viruses in lung microbiota. Further studies are now warranted to decipher virus impact in CF.  PMID: 24637203

Rhinoviruses (from the Greek (gen.) "nose") are the most common viral infective agents in humans and are the predominant cause of the common cold.

Kyle Monk photograohy - see references

Coronaviruses primarily infect the upper respiratory and gastrointestinal tract. Six different currently known strains of coronaviruses infect humans. The most recently publicized human coronavirus, SARS-CoV which causes SARS, has a unique pathogenesis because it causes both upper and lower respiratory tract infections and can also cause gastroenteritis.

The Corona virus is a stealth virus.  Replication of Coronavirus begins with entry to the cell which takes place in the cytoplasm in a membrane-protected microenvironment. Upon entry to the cell the virus particle is uncoated and the RNA genome is deposited into the cytoplasm.  Coronaviruses have a protein known as a replicase encoded in its genome which allows the RNA viral genome to be transcribed into new RNA copies using the host cell's machinery.   In other words, every time a new cell is create by its host, the virus goes with it.  The virus behaves more like a parasite than a virus, although it does a great deal of damage.  And it can be acquired by babies in the womb from their mothers.  So we have a likely candidate here.

I found no more information on this, but I will keep on looking as the fact that viruses can alter genes and that the RSV and coronavirus viruses in particular are found in children with respiratory diseases and cystic fibrosis is a link worth exploring.

Vaccines

Kyle Monk photography - see references

Cystic fibrosis may be caused by a virus.  When we vaccinate someone we give them a dose of a virus in the hope they will build up immunity.  Where the vaccine used in the injection is a live virus, there are instances where it, although apparently vanquished by the immune system, lays low and continues to attack, albeit at a much reduced rate.  One example is the herpes virus, read on:

Herpes simplex viruses types 1 and 2 (HSV-1 and HSV-2) are human neurotropic viruses that establish latent infection in dorsal root ganglia (DRG) for the entire life of the host. From the DRG they can reactivate to cause human morbidity and mortality. Although they vary, in part, in the clinical disorders they cause, and in their molecular structure, they share several features that govern the biology of their infection of the human nervous system. The biology of their ability to establish latency, maintain it for the entire life of the host, reactivate, and cause primary and recurrent disease is being studied in animal models and in humans.  PMID: 24142852

This is not the only virus to do this, thus any vaccine containing a live virus carries great risk with it.  Many of these can cross from the mother to the baby...

The zoster vaccine (trade name Zostavax) is a live vaccine developed by Merck & Co. …... The zoster vaccine is, essentially, a larger-than-normal dose of the chickenpox vaccine (Varivax, Varilrix), as both shingles and chickenpox are caused by the same virus, the varicella zoster virus (VZV).

All these viruses can cause pancreatic disease and pancreatic disease is a hallmark of cystic fibrosis.

The RSV virus which may be implicated in some cystic fibrosis cases is currently not the subject of a vaccine - but it won't be long before it is...

Novavax, Inc. is a clinical-stage biopharmaceutical company, which specializes in creating recombinant protein nanoparticle vaccines.  The company announced preliminary immunogenicity data for its RSV vaccine candidate in its Phase 2 clinical trial in women of childbearing age

There are no coronavirus vaccines for humans as yet, but there are for animals and work on a human vaccine are ongoing.

We must therefore take great care. 

In some ways development of vaccines is putting the cart way before the horse and it is a runaway cart liable to do untold damage.  We do not know the entirety of diseases or illnesses caused by viruses or bacteria, nor do we know precisely whether each one is eradicated by the immune system or simply lies low.  To give anyone a live virus when this information is not known, carries enormous risk - and one of the risks may be that a child develops cystic fibrosis and the genes in a mother or father mutate so that this condition becomes forever a curse hanging over a family.

Given the other viruses implicated, this may already have happened.

Fluoride

One additional culprit which appears to be gradually raising its head is fluoride.  There appear to be high incidences of cystic fibrosis in areas where water is artificially fluoridated, and there is a known link between fluoride and the gene causing cystic fibrosis...

While studying the regulation of the cystic fibrosis transmembrane conductance regulator (CFTR), we found that addition of F- to the cytosolic surface of excised, inside-out membrane patches reversibly increased Cl- current in a dose-dependent manner. Stimulation required prior phosphorylation and the presence of ATP. F- increased current even in the presence of deferoxamine, which chelates Al3+, suggesting that stimulation was not due to AlF4-. F- also stimulated current in a CFTR variant that lacked a large part of the R domain, suggesting that the effect was not mediated via this domain. Studies of single channels showed that F- increased the open-state probability by slowing channel closure from bursts of activity; the mean closed time between bursts and single-channel conductance was not altered. These results suggested that F- influenced regulation by the cytosolic domains, most likely the nucleotide-binding domains (NBDs). Consistent with this, we found that mutation of a conserved Walker lysine in NBD2 changed the relative stimulatory effect of F- compared with wild-type CFTR, whereas mutation of the Walker lysine in NBD1 had no effect. Based on these and previous data, we speculate that F- interacts with CFTR, possibly via NBD2, and slows the rate of channel closure. PMID: 9530164

 

According to 'Health Canada', 45.1% of Canadians drink fluoridated public water. Canada is one of the most fluoridated countries in the world. In comparison, only 5.7% of the world’s population has their public water supply fluoridated.  Approximately 72% of Americans drink fluoridated public water. The United States of America is one of the most fluoridated countries in the world.

 

In the Republic of Ireland the majority of drinking water is fluoridated; 71% of the population in 2002 resided in fluoridated communities. The fluoridation agent used is hydrofluorosilicic acid (HFSA; H2SiF6). In a 2002 public survey, 45% of respondents expressed some concern about fluoridation.

Around 10% of the population of the United Kingdom receives fluoridated water, about half a million people receive water that is naturally fluoridated with calcium fluoride, and about 6 million total receive fluoridated water. The Water Act 2003 required water suppliers to comply with requests from local health authorities to fluoridate their water.  The following UK water utility companies fluoridate their supply:

  • Anglian Water Services Ltd
  • Northumbrian Water Ltd
  • South Staffordshire Water plc
  • Severn Trent plc
  • United Utilities Water plc
 

It is worth noting that fluoride is found naturally in foods such as the potato and in black tea and it is possible to overdose on fluoride from these foods alone.

It is also added to some mineral waters - occasionally without the obvious packaging messages shown left. 

It is also added to some table salt. Fluoridated salt is used in France, for example, again this is often not made explicit on the packaging.

As such any addition of fluoride to water supplies severely exacerbates the problem.

Where water has not been artificially fluoridated, there is still a link with water supplies as some water is naturally very high in fluoride.  One area in which this is a severe problem is Quebec - a state with the highest incidence of cystic fibrosis in the world.

Consumption of unusually high concentrations of F(-) in groundwaters of the Maria area in the Gaspé peninsula of Quebec have resulted in symptoms of skeletal fluorosis in two members of the population. ..... It is estimated that, until these cases came to light, approximately 15-20% of the rural population (total approximately 1,600) in the area were consuming groundwaters with F(-) levels between 5 and 28 mg L(-1) for at least 6 years. The high concentrations of F(-) in well waters of the Maria area occur only in wells completed in Carboniferous sandstone-siltstone-conglomerate sediments that underlie a thick blanket of alluvial-colluvial-glacial overburden. These fluoriferous groundwaters exhibit high Na and HCO3 (-) contents and low Ca and Mg concentrations compared to those associated with the overburden sediments. The high F levels greatly increase the risk for fluorotic diseases such as skeletal fluorosis and skeletal radiculomyopathy. Wells completed in overburden, although having suboptimal F(-) levels are safer for the health of individuals in this region. Effective regulations for well drilling need to be formulated for regions underlain by Carboniferous formations in the Maritime provinces of Canada.PMID: 24194033

There is also a link between cystic fibrosis and the taking or 'administration' of fluoride tablets to children and adults

 

Paediatric Dentristry:  Oral hygiene habits and oral health  in cystic fibrosis J.K.M. APS, G.O.G. VAN MAELE, L.C. MARTENS
The present study was designed to investigate oral hygiene habits and fluoride use in cystic fibrosis (CF) homozygotes, heterozygotes and healthy controls as a function of caries experience (DMF-T) and oral cleanliness (dental plaque, dental calculus and gingival bleeding).  
Methods   Oral hygiene habits in CF homozygotes (n=42), heterozygotes (n=48) and healthy controls (n=62) were used in a multivariate analysis with caries experience (DMF-T) and oral cleanliness (dental plaque, calculus and gingival bleeding) as response variables.
Results  CF homozygotes had a significantly lower caries experience (p ≤ 0.001) and less gingival bleeding sites (p ≤ 0.02). Oral hygiene habits were not significantly different between the three groups, except for intake of fluoride supplements. Significantly more CF homozygotes had received fluoride supplements. Oral  hygiene habits did affect caries experience or oral hygiene differently in each study group.
Conclusion  No matter what oral hygiene habits were, CF homozygotes had an overall better oral health status. Apparently they seemed to possess intrinsic salivary compensatory mechanisms, as the significant higher use of fluoride supplements appeared not to be responsible for the better oral health.

Toxins

In areas like Romania, where the incidence of CF is also very high, there is some evidence that the cause relates to not only fluoride, but a host of other toxins including heavy metals, pesticides and insecticides,  run off getting into the groundwater and from there into drinking water supplies, for example:

The objective of this study was to examine the potential environmental risk of tailings resulted after precious and base metal ores processing, stored in seven impoundments located in the Aries river basin, Romania.
RESULTS:  Tailings were found to be highly contaminated with several hazardous/priority hazardous metals (As, Cu, Cd, Pb), and pose potential contamination risk for soil, sediments, surface and groundwater. Two out of the seven studied impoundments does not satisfy the criteria required for inert wastes, shows acid rock drainage potential and thus can contaminate the surface and groundwater. Three impoundments were found to be highly contaminated with As, Pb and Cd, two with As and other two with Cu.
CONCLUSIONS:  Tailings stored in the studied impoundments were found to be contaminated with some hazardous/ priority hazardous metals, fluoride and sulphate and thus presents different contamination risk for the environment. A long term monitoring program of these tailings impoundments and the expansion of the ecologization measures in the area is required. PMID: 23311708

As such this may be a contributory factor in other countries such as Bulgaria, where both contamination from toxins and heavy industry is high.

The North Circular road in London carries more than 60,000 vehicles a day,
and has the worst traffic fumes of any road in London, according
to official statistics

There is a very clear link between air pollution and nanoparticles and the worsening of the disease for existing sufferers

....higher uptake of NP by alveolar epithelial cells and prolonged, acute inflammatory responses to NP exposure indicate an increased susceptibility of lungs to adverse effects of inhaled NP in Cftr mutant mice and provides potential mechanisms for the increased susceptibility of CF patients to air pollution. PMID:24758489

Rather terrifying to see supposedly body friendly names on this list...

Nanoparticles can occur as a result of pollution, but they are also being deliberately released into the environment via cosmetics, sunscreens, pharmaceuticals and a host of other mechanisms by scientists in the belief that they do no harm, except that yet other researchers have realised they do a lot of harm......

....DNA damage occurs chemically or physically by nanomaterials. Chemical and physical damage are associated with point mutation by free radicals and double strand brake, respectively. The failure of DNA repair and accumulation of mutations might occur when inflammation is prolonged, and finally normal cells could become malignant. These free radicals can not only damage cells but also induce signaling molecules containing immunoreaction. Nanoparticles and asbestos also induce the production of free radicals. .... Taken together,... a variety of diseases [may be] induced by nanomaterials. PMID: 25097864

Genetic engineering of humans on a vast scale.

No doubt as one branch of the scientific community release nanoparticles, other branches of the scientific community will be employed in finding out which genes they destroy.

In effect, these particles could also not only be responsible for the worsening of outcomes for existing cystic fibrosis sufferers, but be contributing to the growth in the number of victims.

are you altering your genes, or those of your baby?

Five classes of nanomaterials have attracted controversy recently including nanoparticles in cosmetics; anti-bacterial silver nanoparticles used in cleaning products and even clothing; nanoparticles of zinc oxide in sunscreens; cerium oxide nanoparticles in fuels; and titanium dioxide nanoparticles in some sunscreens.
The Friends of the Earth environmental lobby targeted cosmetic giant L'Oreal at the Melbourne Fashion Festival earlier this year for being the top nanotechnology patent holder in the United States.
And in New Zealand, the Sustainability Council today warned that New Zealand women are being exposed to cosmetics containing types of nanomaterials that have been stripped from shop shelves in Europe and Australia.

 

Nanoparticles alter genes.

Pharmaceuticals

The eHealthme site now has a growing list of pharmaceuticals implicated in cystic fibrosis. 

The eHealthme site collects the Adverse Drug reports submitted by doctors to the FDA and SEDA in the USA.  It then summarises them for ease of use.  We originally provided a direct link to Cystic fibrosis listed on this site and the pharmaceuticals that can cause it, but the eHealthme website developers frequently reorganise the site and thus break the links.  Thus in order to find out which pharmaceuticals are implicated in Cystic Fibrosis

  • Follow the LINK to the eHealthme website
  • Using the ‘All conditions’ index find the appropriate entry
  • Now scroll down until you get to the section marked ‘Drugs that could cause

The list shows you all the drugs implicated in CAUSING Cystic fibrosis as well as the number of people who have made a complaint to their doctor and had their case reported by him.  Note that it is up to the doctor whether he reports or not.

As of October 2016, about 100 pharmaceuticals were in this list.  The chart below shows the trend of cystic fibrosis resulting from pharmaceuticals, again from eHealthme.

High on the list of drugs implicated are anti-biotics, which might suggest that the antibiotics destoyed the natural gut flora of a child or adult, making it susceptible to a virus or bacteria which then caused the disease - the gene mutation.

There also appears to be a tenous link with anti-depressants and anti-psychotics - possibly being taken by the mother..........

Amitriptyline is a tricyclic antidepressant, used … to treat … major depression and neuropathic pain. In the 1970s the ability of tricyclic antidepressants to inhibit acid sphingomyelinase (ASM) was discovered. The enzyme ASM catalyzes the hydrolysis of sphingomyelin to ceramide. ASM and ceramide were shown to play a crucial role in a wide range of diseases, including cancer, cystic fibrosis, diabetes, Alzheimer's disease, and major depression, as well as viral (e.g., measles virus) and bacterial (e.g., Staphylococcus aureus, Pseudomonas aeruginosa) infections. PMID:  25228885

Kyle Monk photography - see references

Infertility treatments

Cystic fibrosis is caused by a gene mutation.  Most infertility treatments attempt to artificially produce fertilisation in sperm which would have otherwise been rejected as being  genetically 'poor', or in eggs that because of the woman's age or other factors is also not viable.  In effect, one is by-passing the natural and normal bodily control process of rejecting sperm and eggs which together produce fetuses which are not 'viable' because of genetic abnormalities. 

If the baby gets to full term, these poor little children are thankfully helped to live a non normal, but full life whilst they do live, but without this help they would die only too soon - nature in a sense was 'right'.

.... ICSI [Intracytoplasmic sperm injection, a medical technique used in assisted reproduction]  bypasses the physiological selection of spermatozoa that occurs at the level of the testis and epididymis, and in the female reproductive tract as well as at the sperm-oocyte interface. As genetic abnormalities are present in a significant percentage of infertile males with impaired spermatogenesis, ...... Screening for cystic fibrosis gene mutations should be performed in azoospermia caused by congenital absence of the vas deferens and seminal vesicles. Appropriate genetic counseling should be made available to all ICSI couples whenever a gene or chromosomal anomaly has been identified.  PMID: 9851748

Given the terrible emotional burden that often results from having to see a greatly loved child die before you as a result of this disease, there would seem to be a very urgent need for more help in this area and far more information about the risks

Psychological symptoms were reported by 6088 patients with CF and 4102 parents.

  • Depression - Elevated symptoms of depression were found in 10% of adolescents, 19% of adults, 37% of mothers and 31% of fathers.
  • Anxiety - Elevations in anxiety were found in 22% of adolescents, 32% of adults, 48% of mothers and 36% of fathers.

Overall, elevations were 2-3 times those of community samples.  PMID:25246663

 

 Bacterial infection and Fungal infection

Bacterial infection is one of the curses of cystic fibrosis.  Up to now, research has assumed, usually with good reason, that bacterial infection is an effect of cystic fibrosis and not a cause - a symptom not a pathogen.  But there is now some research that appears to be based on the idea that there may be bacteria that affect the baby in the womb at a crucial stage and cause the genetic mutation. 

Note that this research is based on a hypothesis, and I could find no firm evidence that this is the case, but if it is so, it would not be the only bacteria to cause illnesses of this sort.

Pseudomonas aeruginosa is associated with increased mortality in cystic fibrosis (CF) patients, and expresses type III secretion system proteins (TTSP), which is a common mechanism used by gram-negative pathogens for delivery of anti-host factors. .....All chronic patients had a strong positive serology to TTSP, although relatively weak reactions to TTSP were observed for some individuals in the negative control group. ..TTSP that were early produced in P. aeruginosa infected CF patients, induced a detectable antibody response in those patients and were easily detected by Western-blot reaction.  PMID: 22779684

 

Pseudomonas aeruginosa is a common bacterium that can cause disease in animals, including humans. It is found in soil, water, skin flora, and most man-made environments throughout the world. It thrives not only in normal atmospheres, but also in hypoxic atmospheres, and has, thus, colonized many natural and artificial environments. Its versatility enables the organism to infect damaged tissues or those with reduced immunity. The symptoms of such infections are generalized inflammation and sepsis. If such colonizations occur in critical body organs, such as the lungs, the urinary tract, and kidneys, the results can be fatal. Because it thrives on moist surfaces, this bacterium is also found on and in medical equipment, including catheters, causing cross-infections in hospitals and clinics. It is implicated in hot-tub rash.

The case for fungal infection as a cause is very very weak, but fungal infection undoubtedly contributes to the progression of the disease

The respiratory tract of cystic fibrosis patients is colonised by bacteria and fungi. Although colonisation by slow growing fungi such as Pseudallescheria, Scedosporium and Exophiala species has been studied previously, the colonisation rate differs from study to study.... Monitoring of respiratory tract colonisation in cystic fibrosis patients includes the use of several semi-selective culture media to detect bacteria such as Pseudomonas aeruginosa and Burkholderia cepacia as well as Candida albicans. …... In total, 589 respiratory specimens from cystic fibrosis patients revealed …

  • For 439 samples from 81 patients, … Exophiala dermatitidis and phaeohyphomycetes.
  • For 150 samples from 42 patients, Pseudallescheria and Scedosporium species,  PMID:  20492536

 

Bob Flanagan was a Los Angeles writer, poet, performance artist,

and comic, who suffered from and later died of cystic fibrosis.

He managed to live quite a long [and full] life being born on

December 26, 1952 and dying January 4, 1996, aged 44. 

A 1997 documentary film was made about him - Sick: The

Life and Death of Bob Flanagan .  The film premiered at the

1997 Sundance Film Festival, where it was awarded a

Special Jury Prize.

 Parasites

There are literally hundreds of parasites and depending on the site and circumstances of infection, they can get everywhere from the intestine to the brain, from the lungs to the pancreas. 

And they do, in high numbers. 

Their presence is known from cysts, which form around the parasites if your immune system is working well.  But the presence of cysts is a sure indicator of damage.  And one of the things cystic fibrosis patients suffer from is cysts in the pancreas, often leading to diabetes.

The only parasite implicated so far is that found in the domestic cat, but more may emerge in due course. 

Toxoplasmosis is a parasitic disease caused by the protozoan Toxoplasma gondii. The parasite infects most genera of warm-blooded animals, including humans, but the primary host is the felid (cat) family - see Being with cats

And they can be passed from other to baby, so even if the parasite is not the principle cause of the disease, it is certainly a contributory factor to its other symptoms.

Echogenic bowel is diagnosed in 0.2% to 1.4% of second trimester ultrasonographic examinations. This finding occurs as a normal variant in the second trimester but also has been associated with several pathologic conditions that include cystic fibrosis, chromosomal abnormalities and in utero infection with cytomegalovirus and toxoplasmosis. …PMID:  18254450

 

Treatment

 Treatment for cystic fibrosis sufferers is palliative and effective, but to make real progress far more work needs to be done on finding the causeso that it can be eliminated or solutions found for its removal from those suffering from the disease.

 

 

 

References and further reading

  • Some of the photos on this page are from  Kylemonk photography - Kyle is working with the CF Foundation to create 'dramatic, powerful, and intimate portraits of people with Cystic Fibrosis to help bring awareness to this genetic disease'.
  • Mycoses. 2011 Nov;54(6):514-22. doi: 10.1111/j.1439-0507.2010.01897.x. Epub 2010 May 19.  Detection of hyphomycetes in the upper respiratory tract of patients with cystic fibrosis.  Horré R1, Marklein G, Siekmeier R, Reiffert SM.

Related observations