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Ehlers-Danlos syndrome

Category: Illness or disabilities

Type

Involuntary

Introduction and description

 

Ehlers–Danlos syndrome (EDS) is a  connective tissue disorder, that causes defects in the structure, production, or processing of collagen or proteins that interact with collagen.  The syndrome is named after two physicians, Edvard Ehlers from Denmark and Henri-Alexandre Danlos from France, who described it at the turn of the 20th century.

As the cause is believed to be one [or more] defective genes, there is currently no cure for EDS, although many benign treatments can ease the problems associated with the disease, including the use of food to help with collagen production, pain relievers that are not opiate based and thus not addictive, and various forms of gentle exercise to help strengthen the skeleton, muscles and ligaments.

Collagen is the main structural protein in the extracellular space in the various connective tissues in animals. As the main component of connective tissue, it is the most abundant protein in mammals, making up from 25% to 35% of the whole-body protein content.  Collagen is formed of elongated fibrils.  It is mostly found in fibrous tissues such as tendons and ligaments. It is found in the artery walls, skin, intestines and the uterus.  It is also abundant in corneas, cartilage, bones, blood vessels, intervertebral discs and the dentin in teeth.  Collagen constitutes one to two percent of muscle tissue, and accounts for 6% of the weight of strong, tendinous muscles.

So there is a lot to go wrong if the collagen does not work.

Although the illness has been around since Hippocrates’s time, there has been a rather worrying increase in the number of sufferers over the past few years. 

As many as 1 in 10,000 to 15,000 suffer from this illness.

 

Symptoms

Emily Comber's joints 'pop out' up to five times a day and she has dislocated
her shoulder, wrist and thumb changing her son's nappy. She is pictured
with her children, Ruben, one, and Finlay, three

The collagen in connective tissue helps tissues resist deformation, so any problems in its formation produces more ‘bendy’ structures, rubber joints and stretchy, rubbery skin!  And this is what characterises Ehlers-Danlos sufferers - bendiness and stretchiness.  Bendy limbs, rubbery limbs, stretchy skin.

Because none of these structures – the muscles, ligaments, tendons and so on are as solid or reliable as they should be, there is a risk from falling and other injuries and because nothing is very supportive, more strain is put on the joints and the person gets pain, sometimes a lot of pain.

Sometimes there is extreme joint hypermobility causing severe instability and chronic musculoskeletal pain. Sufferers with this hypermobility can then get full or partial joint dislocations and as a result, pain is severe.

The vascular type of Ehlers-Danlos is considered one of the more serious forms of Ehlers–Danlos syndrome because blood vessels and organs are fragile and prone to tearing (rupture). Many patients with what is called ‘EDS type 4’ have large eyes, a small chin, sunken cheeks, a thin nose and lips, and lobeless ears.  They are small and slim – fragile looking - with thin, pale, translucent skin, almost ethereal, little spirits enrobed.  They bruise very easily.   

Additional symptoms may include an ‘insensitivity to local anaesthetics’, meaning they don’t work.  For those who cannot get a grasp of what this means it means that when you go to the dentist, normal [injectable] pain relief is not there to help you.  And pregnancy will be no picnic.

Lillie-Grace Tomlinson aged 6, was bornwith Ehlers-Danlos syndrome (EDS)
but was only diagnosed last year.

There may also be problems with ‘Platelet aggregation’ – meaning you have thin and not normal or thick blood.  If you hurt yourself in this case, then you may bleed a lot and a scab will be slow in forming.  

One of the perhaps worst aspects of having the disease is that no one has heard of it, except those who have managed to find out that is what they have.  Furthermore, by calling it a syndrome and not ‘collagen formation failure disease’ the medical community subconsciously think – ‘oh lord another syndrome’ – and not ‘Crikey, collagen failure disease!’  Which is what they should be thinking.

There is nothing more frustrating than knowing you are not well, trotting along to the doctors and being prescribed anti-depressants, or paracetamol dressed up as complex pain killers.  What this doctor is saying is – ‘you are a depressed hypochondriac with imagined pain, just like the people with Chronic fatigue syndrome whose illness I also cannot treat simply with pills and therefore dismiss in much the same way’.

Rage does not help.  Doctors do not respond well to rage, they do not respond well to sarcasm either, pleading on hands and knees and then not being able to get up again, at least raises awareness that the problem is real.  

Thankfully a number of support groups do exist to help those with this problem, but it is still a challenge to get it diagnosed, and it does need to be diagnosed, because your whole life has to be adjusted around the diagnosis.

Causes

Little Lewis Green, who attends John Wheeldon School, has Ehlers-Danlos syndrome

The disorder is, according to all the literature on this subject bar those papers from a few enlightened beings, 'caused by mutations in the FBN1 gene, encoding the microfibrillar protein fibrillin-1'. 

And there doctors have tended to leave it.  Oh dear, another gene mutation, so many, so many new ones.  Researchers and doctors ought to wonder why, don't you think?

Mutations in genes have a cause. 

The cause can be nanoparticles, but in the case of Ehlers-Danlos there is a link – a tentative link - with the Simian virus. There is also a link with recent vaccines, we thus have an hypothesis one and a hypothesis two.

Everything that follows is thus an hypothesis based on research so far.  Treat it as an hypothesis, please.

HYPOTHESIS ONE - SV40

SV40 is an abbreviation for Simian vacuolating virus 40 or Simian virus 40, a polyomavirus that is found in both monkeys and humans. It was named for the effect it produced on infected green monkey cells, which developed an unusual number of vacuoles. “Like other polyomaviruses, SV40 is a DNA virus that has the potential to cause genetic damage, but most often persists as a latent infection.” So, it has latency – it is a latent virus!!

Virus latency is the ability of a pathogenic virus to lie dormant (latent) within a cell. A latent viral infection is thus a type of persistent viral infection. Latency is the phase in certain viruses' life cycles in which, after initial infection, proliferation of virus particles ceases. However, the viral genome is not fully eradicated. The result of this is that the virus can reactivate and begin producing large amounts of viral progeny without the host being infected by new outside virus, denoted as the lytic part of the viral life cycle, and stays within the host indefinitely.

Interest in virus latency has increased with the realisation that some very major viruses have it. 

The Herpes Virus family, Herpesviridae, can all establish latent infection. Herpes virus include Chicken-pox virus [VCZ] and Herpes simplex viruses [HSV-1, HSV-2], the Epstein-Barr Virus [EBV], the Human papilloma virus [HPV],  Cytomegalovirus [CMV], the Measles virus, the Mumps virus and the Rubella virus.

If we use an example, the varicella zoster virus [VCZ], can reappear years later as shingles.  After an initial acute infection (chickenpox) the virus lies dormant until reactivated as herpes zoster.  Persistent infection.

The Simian 40 virus can do the same, lie low and then become reactivated at times of low immunity. 

SV40 and collagen

There are quite a number of research papers that investigate the effect the Simian virus has on collagen, this is one of the easier ones to understand [we hope!].

Primary culture of human uterine endocervical epithelial cells was performed. When cells were cultured on a plastic dish, these cells developed into a flat form, then lost their proliferative ability in a short time during continuous subculture. On the other hand, when cells were cultured on collagen gel and subcultured by explanation of this cell containing gel, the cells took on a roughly ovoid rather than a flat shape and active proliferation was attained which maintained confluency for as long as 8 weeks. Furthermore by adopting the Simian Virus 40 in the culture system for these cells, it was possible to extend the lifespan to approximately twice that of the control cell group.  PMID:  1647431

 

So the Simian virus thrives on collagen and is able to live in cells, doing what is called preventing apoptosis – normal cell death – thus affecting the proliferation of cells.

It may be worth adding that there may be other viruses that can also do this, but a search of PubMed has so far not come up with anything else that is as firm or with as many papers.

So the next question, where did the virus come from?

SV40 and vaccines

The existence of SV40 was only discovered in the 1960s.  It was first identified by Ben Sweet and Maurice Hilleman in 1960 when they found that polio vaccines in the USA were contaminated with SV-40.  On further investigation, it was found that between 1955 and 1963 around 90% of children and 60% of adults in USA were given SV40-contaminated polio vaccines. 

And this is not all. 

Although it is unclear, it appears many other polio vaccines were developed along similar lines to the American one and children from other nations given the same American vaccine.  And thus many other populations of the world are possibly affected.  An analysis presented at the Vaccine Cell Substrate Conference in 2004 suggested that vaccines used in the former Soviet bloc countries, as well as China, Japan, and Africa, could have been contaminated up to 1980, meaning that hundreds of millions more could have been exposed to the virus unknowingly.

Because so little is known about the virus before its discovery no one knows how many people had the virus before the vaccine infected so many people.  There are some indications that there were already people with the virus before the vaccine, the vaccine simply added millions more to the numbers.

Olivia Court (pictured) could barely walk without her knees
or hips dislocating, but the toddler can now run and play
thanks to a 'second skin' suit
 

SV40 and horizontal infection

Simian virus 40 (SV40) is a monkey virus that was administered to human populations by contaminated vaccines which were produced in SV40 naturally infected monkey cells. Recent molecular biology and epidemiological studies suggest that SV40 may also be contagiously transmitted in humans by horizontal infection.  PMID:  17620119

A horizontal infection is one where transmission does not necessarily rely on people, for example, via the bite of an infected organism, or via contact with infected faeces. 

So cleaning out a monkey's cage, for example, might be just the thing the virus was waiting for. 

And a little nip from the fleas the little monkey he has just killed was harbouring and another researcher bites the dust.

We are not very interested in conspiracy theories, but there is one concerning the Simian 40 virus and HIV that is worth knowing about at least.  The Simian 40 virus is ‘not dissimilar’ [their words not ours] to a family of viruses called the Simian immunodeficiency viruses (SIVs).  These, however, are retroviruses.  They occur in primates such as chimpanzees, macaques, sooty mangabeys and a host of other poor little creatures used in laboratory work.  It is the virus strains from these very primates that ‘are believed to have crossed the species barrier into humans, resulting in HIV-2 and HIV-1, respectively’.  In effect, the conspiracy theory goes, HIV did not come from Africa, it came from laboratories, scientists in other words, experimenting on animals.  The same could be true for Simian 40 virus.  End of conspiracy theory!

Viruses cause numerous diseases.  Why should some people be infected and not others?

Administrative routes

Jodie Vasquez, of Carlisle, Cumbria, is an 18 year old with Ehlers-Danlos Hypermobility Type III. 

Jodie has had to undergo more than a dozen operations and has not eaten since 2009, instead

taking nutrients via an intravenous drip.  Every day she deals with intense pain and needs a

wheelchair to get about as her joints dislocate easily and she suffers from chronic fatigue.

Given orally, any vaccine is slow in taking effect.  The immune system has time to muster its forces and the stomach acid has enough time to weaken or even kill off the majority of virus, leaving a scant few for the immune system to fight and build up an immunological record of.  In this slow route system, the virus may be wiped out.  It has no chance to become latent, because it isn’t alive any longer, it is an ex-virus.

But injecting the virus, or worse, by sticking it up someone’s nose, the immune system not only has no time to muster its defences, but you have by-passed two key parts of the immune system’s defences – the stomach and the gut.  In the latter case, you have sent it straight into the brain because this route avoids the blood brain barrier.  There is every likelihood that the virus has plenty of time to become latent and spread and spread and spread, slowly and inexorably every time our immune system reaches a low point.

The Simian 40 virus is in fact little different to all the other latent viruses in that it can cause a host of other diseases.  In 1997, Herbert Ratner gave some vials of the vaccine to researcher Michele Carbone. Ratner was the Health Commissioner at the time the vaccine was introduced and had kept these vials of vaccine in a refrigerator for over forty years. Upon testing this vaccine, Carbone discovered that it contained not only the SV40 strain already known to have been in the vaccine, but also an SV40 strain that caused some malignant tumours and lymphomas.  So this vaccine may be responsible for a good deal more than just Ehlers-Danlos.

It may help to know that both the Sabin vaccine (oral, live polio virus) and the Salk vaccine (injectable, killed polio virus) were infected.

dogs too ................. after vaccination ..................

SV40 footprints in humans have been found associated at high prevalence with specific tumour types such as brain and bone tumours, mesotheliomas and lymphomas and with kidney diseases   PMID:  17620119

HYPOTHESIS TWO - Gelatine

There is one more very worrying aspect to this disease, dogs are now getting it.  And the one thing that has changed in most dogs' lives is that they now have a lot of  vaccinations. [Time to read the section on Vaccines I think].

But dogs have not had the polio vaccine, so what is happening here? This may be another cause, not an alternative cause. 

Vaccines contain an adjuvant, the virus and the excipient.  The adjuvant boosts the immune system to fight anything that has been innoculated into the person and it would seem that sometimes that something is gelatine – just like collagen.  So the body fights its own collagen.

J Allergy Clin Immunol. 1999 Sep;104(3 Pt 1):695-9.  IgE reactivity to alpha1 and alpha2 chains of bovine type 1 collagen in children with bovine gelatin allergy.  Sakaguchi M1, Hori H, Hattori S, Irie S, Imai A, Yanagida M, Miyazawa H, Toda M, Inouye S.
BACKGROUND
:   Anaphylactic reactions to measles, mumps, and rubella vaccines, including gelatin as a stabilizer, have been reported. It had been found that most of these reactions to live vaccines are caused by the bovine gelatin included in these vaccines. Gelatin mainly includes denatured type I collagen, which consists of alpha1 and alpha2 chains.
OBJECTIVE:  The current study was designed to investigate the IgE reactivity to alpha1 and alpha2 chains of bovine type I collagen in gelatin-sensitive children.
METHODS:  Serum samples were taken from 10 children who had anaphylaxis to the vaccines and high levels of specific IgE to bovine gelatin. Bovine type I collagen was isolated from bovine skin and then separated to alpha1 and alpha2 chains by column chromatography. IgE reactivity to denatured type I collagen and its alpha1 and alpha2 chains was analyzed by immunoblotting, ELISA, and histamine release from the mast cells passive sensitized with IgE antibodies in pooled serum of the children.
RESULTS:  All children had specific IgE to bovine type I collagen. Furthermore, IgE antibodies in their sera reacted with the alpha;2 chain but not with the alpha1 chain. Similarly, the mast cells sensitized with pooled sera in the children showed alpha2 chain-specific histamine release but not alpha1 chain-specific histamine release.
CONCLUSION:  In gelatin allergy denatured bovine type I collagen is a major allergen and IgE-binding sites exist in the alpha2 chain of type I collagen.
PMID: 10482848

Thus for anyone in their 60s, the cause may be the SV40 virus in vaccines.  In children now, it is the gelatine in vaccines.

There are incidentally other vaccines that contain gelatine besides the MMR vaccine.  If you go to the Wikipedia entry for vaccine ingredients you should be able to see which ones.

Treatment and helping yourself

Depending in the cause there may be different strategies needed as regards food products.  If the cause is bovine gelatin, then first and foremost this needs to be cut out of a child's [or other person's] diet, so that it does not exacerbate the problem.

The next step, whether the cause is virus or gelatine in vaccines is to always ensure that your body has the right raw materials to make collagen.  In effect, whether the virus is attacking the collagen, or the immune system is attacking it, you need to make sure you are constantly eating enough of the right foods to replace the collagen being lost.  

Eating for health

 

The body makes collagen from a number of basic ‘raw materials’ which are in what we eat

So the first part of the solution is to eat for your health

Collagen has an unusual amino acid composition and sequence:

  • Glycine is found at almost every third residue.
  • Proline makes up about 17% of collagen.
Collagen contains two uncommon derivative amino acids not directly inserted during translation. These amino acids are found at specific locations relative to glycine and are modified post-translationally by different enzymes, both of which require vitamin C as a cofactor.
  • Hydroxyproline is derived from proline
  • Hydroxylysine is derived from lysine - depending on the type of collagen, varying numbers of hydroxylysines are glycosylated (mostly having disaccharides attached).

Here is the full table showing all the amino acids needed to make the collagen in skin, it comes from Wikipedia.  As you can see Alanine is also a very key amino acid.

Amino acid

Abundance in mammal skin
(residues/1000)

Glycine

329

Proline

126

Alanine

109

Hydroxyproline

95

Glutamic acid

74

Arginine

49

Aspartic acid

47

Serine

36

Lysine

29

Leucine

24

Valine

22

Threonine

19

Phenylalanine

13

Isoleucine

11

Hydroxylysine

6

Methionine

6

Histidine

5

Tyrosine

3

Cysteine

1

Tryptophan

0

So in summary what do we need to do?  We need to

  • Eat Glycine, proline, lysine and alanine rich foods, and
  • Eat foods rich in Vitamin C

To help we have provided a number of observations derived from Dr Duke’s phytochemical database that show which plants at least contain these principle amino acids and vitamin C.  The links to the chemicals themselves also show other foods besides plants, these lists are derived from the USDA nutrients database, but care must be used here if the other foods contain gelatine.  Only use a food which is certain to contain no gelatine.

Avoiding stress

To help raise awareness of the condition around Rare Disease Day, Annabelle J has recently
taken to the field with her local football club Bury FC as their mascot.

Easier said than done, but very key.  Cortisol stimulates degradation of (skin) collagen into amino acids.  Thus we age and lose collagen via cortisol.  And in Ehlers-Danlos we need all the collagen we can muster.

Cortisol is a steroid hormone, in the glucocorticoid class of hormones, and is produced in humans by the adrenal cortex within the adrenal gland.  It is released in response to stress and low blood-glucose concentration.

So there are two things to be done here – find things that are calming and restful – music, funny movies, poetry, painting, communing with nature, being with loved ones, find things you feel safe about and avoid all threats.  Adrenaline and cortisol are part of the fight and flight system and the adrenaline is the ‘flight’ part, so anything that might have the same effect as seeing a tyrannosaurus rex at ten paces needs to be avoided.  No scary movies, no fear, no fights or arguments.

Remember music calms rage – Soloman knew.

Next is to eat little and often, but only the foods on the list, NOT sugary bars or similar that are going to produce highs and lows of sugar levels, which stress the system.  An apple here, an orange there [vitamin C], some nuts [amino acid] and so on.  Three good meals a day, but all moderate in size and rich in the amino acids and vitamins you need.

Resting and sleeping

Although Cortisol has a negative effect in some cases, it does help in metabolism and our metabolism works faster under threat and fear [because it needs more energy quickly].  But the last thing you want is stress and fear, so a slowed down metabolism is going to be an inevitability.  So, if you are deeply relaxed, you will need more time to digest your food, so you need to rest after every meal, just for a short time, enough time to let the system work. 

And sleep, sleep sleep, hours and hours and hours, to help the immune system and help it heal.

Do not compromise the immune system in any way

support your limbs, support your immune system

Cortisol has the effect of suppressing the immune system, so by avoiding stress one also helps the immune system. 

But since the cause could be a virus, you want the immune system to fight for all its worth. 

In this respect avoid at all costs

  • Immunosuppressants – which do exactly what they say, suppress the immune system
  • Anti-histamines – histamine is part of the immune system, an esential part.  Anti-histames can be secreted away in all sorts of innocuous looking products such as cough medicines.  Take great care with over the counter medication. 
  • Antibiotics – if you have a very bad bacterial infection – pneumonia for example – then you may have to take antibiotics, but otherwise avoid them too, like the plague.  

Some [not all] antibiotics can be viewed as immunosuppressants because although they may get rid of a bacterial infection, they can have the effect of destroying the gut flora and fauna, and this flora and fauna is part of the immune system – a very key part in fact.

Find medications for pain relief other than opiates

In the first place opiates and opioids are addictive.  The body soon adjusts to the level of pain killer you are taking and readjusts the number of receptors.  Thus if you lower the dose you will be in pain of an excruciating nature, because you have a reduced number of receptors able to accept the drug.  You will then be in unbearable pain.  If you want to see what opioids can do to someone read the section on codeine and that on morphine.  

The Simian 40 virus is not the same as the SIV family of viruses, and thus may behave entirely differently, but it is worth knowing that the opiates lead to a suppressed immune system in SIV infected monkeys.  It may be relevant, it may not be, but it is worth bearing this in mind and perhaps using a different sort of pain killer.  After all you have nothing to lose and everything to gain:

While substantive changes in plasma proteins were seen during SIV infection, the addition of opiates led to suppression of these changes as well as increased variance of the proteome. These changes demonstrate that opiates induce broad but variant immune suppression in SIV-infected monkeys.  PMID:  21821369

The section on Inflammation and ways of handling it may be of help, and observations will be added as time goes on below.

Exercising and keeping fit

 

Whatever exercise you choose it should be gentle, the objective is to keep you fit and thus encourage your immune system to function, and also to strengthen those muscles and tendons etc that are in reasonable working order.  T'ai chi for example, might be just the ticket, ballroom dancing, to old fashioned music, a very slow walk through the park, it all helps.

  No bungee jumping I'm afraid.

 

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