You Know You Have Eds When

The Ehlers-Danlos syndromes are a group of atmospheric condition that touch on the stretchiness and forcefulness of supporting tissues in the trunk, including pare, joints, blood vessels and internal organs. They vary in their affect from very mild through to very severe.

The Ehlers Danlos syndromes (EDS) are a grouping of weather commonly (although non always) inherited from your parents. People with EDS take a trouble with the formation and structure of connective tissue in the body. Connective tissue is a particularly important component of skin, muscles and ligaments, blood vessels and eye valves.

Whilst the symptoms of EDS may be mild and undiagnosed, for some people with EDS, symptoms can be astringent, life-irresolute, and fifty-fifty life-threatening.

Nearly forms of EDS touch on the skin (which can be unusually stretchy and, sometimes, fragile) and the joints which can be 'double-jointed' (hypermobile). Many types likewise cause general symptoms such as tiredness, generalised pain, and poor slumber. Some affect other parts of the body, including blood vessels, the digestive system, the eyes, the teeth and the heart valves.

The syndromes are genetic conditions. The genes which cause near types of EDS have been clearly identified and can be tested for. However, the most common form of EDS, hypermobility EDS (hEDS), is an exception to this, as the cistron which causes it has not even so been identified.

In some cases, the faulty gene is inherited from an affected parent. However, a person may also exist afflicted if in that location is a new mutation in the factor. In which case EDS may occur in people with no history of the disorder in their family unit.

The more than mutual types of EDS show autosomal dominant inheritance, meaning if you have ane faulty gene, from i of your parents, you will have the status (and your parent will also have the condition). Rarer types of EDS show autosomal recessive inheritance.

Until fairly recently, the proper name 'Ehlers-Danlos syndrome' was used to depict all forms of the condition. However, there are many dissimilar types of EDS and then it was felt that this was not i syndrome, but many. EDS is now therefore chosen 'the Ehlers-Danlos syndromes'.

It is estimated that EDS can occur in betwixt 1 in v,000 and 1 in 250,000 births.

Symptoms range from mild to very severe, and vary with which type of EDS you have. Milder forms of EDS are oft not diagnosed until early machismo, as it can take some time earlier the symptoms and signs become noticeable. The more severe types, however, are diagnosed mainly in childhood. Some types have been seen and then few times that it is hard to generalise nearly what symptoms are normal for them. Below are the main symptoms seen in the dissimilar types of EDS.

Joint changes

Joints are often loose and 'double-jointed' (hypermobile), so that they have a greater than normal range and degree of motion.

Ehlers-Danlos syndrome finger hyperreflexia

Ehlers-Danlos Syndrome

Piotr Dołżonek, CC Past-SA 4.0, via Wikimedia Eatables

By Piotr Dołżonek, CC BY-SA iv.0, via Wikimedia Eatables

Dislocations of joints may easily occur, and people with EDS can often put their ain joints back into place because the tissues are and then stretchy. Articulation pains are besides common.

Skin changes

In all types of EDS except the vascular type, the skin feels soft and is unusually stretchy.

Ehlers-Danlos syndrome hyperelastic skin

Ehlers-Danlos Syndrome

Whitaker JK, Alexander P, Chau DY, Tint NL, CC BY 2.five, via Wikimedia Eatables

By Whitaker JK, Alexander P, Chau DY, Tint NL, CC By 2.5, via Wikimedia Commons

The movie shows how easy it is to pull the skin downwards and away from the torso. When you let become, the pare immediately returns to its normal position.

Many affected people find that their skin splits and bruises easily and that skin injuries heal slowly. People with some types of EDS have scars that tend to become broad and sparse. Blood vessels nearly to the skin surface can be very delicate and can be damaged hands.

Mobile lumps tin can develop under the skin of people with EDS, particularly around the elbows and knees, and tiny hard lumps may also form effectually the heel.

In the vascular blazon of EDS, the skin is non stretchy but it may wait slightly transparent, and then that you tin can see blood vessels underneath the skin.

Muscles and skeleton

The muscles of people with many forms of EDS tend to be floppier and less potent than those of people without EDS. This may be noticed considering of difficulty with walking, such equally falling over often, or because of problems with rest. In more than astringent cases, people find it helpful to utilize a stick or a wheelchair to help them get about.

Poor grip and difficulty writing are sometimes a problem. Special aids may be needed at school, in the kitchen and in other places where grip is important. Sometimes the voice tires easily when shouting, singing or talking loudly. Piece of cake tiring of the muscles is common.

People with EDS are more prone to hernias in the groin and in surgical scars. In some types of EDS, curvature of the spine (scoliosis) can develop.

Tiredness and sleep disturbance

Tiredness, getting tired easily after physical activity (fatiguability) and dizziness are very common in EDS of many types. Sleep disorders are as well common. EDS can seem like to, and is often mistaken for, chronic fatigue syndrome and/or fibromyalgia.

Pain

Widespread pain or hurting in the limbs is a common feature of almost types of EDS. This typically gets worse with exercise. Many patients have frequent headaches.

Mood

Difficulties with mood, particularly feet, panic disorder and depression, are common conditions in the general population only they are more ordinarily experienced past people with EDS.

Heart

An uncomfortable feeling of thudding of the eye (palpitations) and noticeable extra beats are symptoms experienced by many people but they are more mutual in EDS. Many people feel dizziness and a fast pulse rate when they stand chop-chop.

Some people with EDS take problems with their heart valves, specially equally they go older, and may develop a murmur.

In the rare vascular type of EDS, serious complications involving claret vessels can develop, including aneurysms and blood vessel rupture. This risk can be lowered by medication.

Lungs

Asthma-like symptoms can occur in some forms of EDS, particularly the hypermobile type.

Digestive system

Constipation and diverticulosis (in which there are bulges in the wall of the large intestine) are more mutual in people with EDS. Hiatus hernia and irritable bowl syndrome are likewise more common than in the general population, and many people with EDS experience 'heartburn' (dyspepsia). Nausea is commonly noticed, and people with EDS are more normally prone to travel sickness. Prolapse of the bowel (rectal prolapse can occur) is quite mutual.

Bowel rupture is a possible (rare) complexity of some rare types of EDS.

Eyes, ears and teeth

Tinnitus is mutual in several types of EDS. Many people also accept dizziness (vertigo).

Some very rare types of EDS affect the eyes. The areas around the pupils (sclera) may exist blue rather than white and the eyeballs themselves may exist very fragile. One rare subtype also affects the gums and teeth.

Children's health

Children with EDS often show delay in crawling, walking and continuing. They tend to be more than floppy than usual. Some rare types of EDS can affect growth and cause early on joint dislocations.

Women's health

Prolapse of the womb (uterus) is more common in EDS. Prolapse is a common condition in women who have children; however, it is seen in EDS in women who take non had children more commonly than you would otherwise expect.

Bug that tin occur when pregnant

These include:

  • Varicose veins, haemorrhoids and vulval varicosities.
  • Reflux, which tends to become worse: many people with EDS have ongoing reflux and symptoms may worsen during pregnancy.
  • Swelling of legs and hands, which is mutual in pregnancy but tends to be worse in EDS.
  • Carpal tunnel syndrome, which is mutual in pregnancy in EDS.
  • Women with EDS - may be more than than usually prone to morning sickness.
  • Many people with EDS feel regular headaches and these tend to get worse in early pregnancy.
  • Many people with EDS have tinnitus due to instability of the bones in the heart ear. Pregnancy may exacerbate this condition.
  • Women with EDS who need to protect their joints throughout pregnancy.
  • Women with EDS who often have worse aches and pains in pregnancy, including:
    • Pelvic girdle pain (symphysis pubis dysfunction).
    • Back pain.
    • Generalised aches and pains.
  • Palpitations and actress heartbeats, which may increase in pregnancy.
  • Breast changes which occur in almost pregnant women. The stretchy skin of EDS means that extra support is important. People with EDS are more prone to stretchmarks mostly.
  • People with EDS, who are more than decumbent to feet and depression. This can exist made worse by pregnancy.

Problems that can occur during delivery

These include:

  • Early breaking of the waters - preterm rupture of membranes (PROM).
  • Women with EDS, who ofttimes take a poor response to lidocaine (used in epidurals). You should see an anaesthetist before your labour to talk about what options might be effective for you.
  • Delivery of your infant which can be fast in EDS because your tissues 'give' hands and don't resist pushing the baby out. It's important for your midwife to continue it irksome and controlled, for your baby and for you.
  • Abnormal position of the babe, which is more common, as the baby is not held so tightly by your tissues, but this does non generally brand labour more hard in EDS.
  • Rarely, the womb (uterus) can rupture during labour.

Problems that can occur afterward delivery

These include:

  • Bleeding later on delivery.
  • If y'all take fragile skin, it is more prone to tearing and may have longer to heal.
  • If you need stitches, dissolvable stitches may dissolve too early, so silk sutures are recommended.

EDS is usually suspected because y'all take the typical symptoms of ane of the types. The doc may enquire about close family members and any problems they may have had with their pare or joints. Once the condition is suspected, specialist testing, including genetic testing, may exist offered to make up one's mind which type of EDS you lot have.

The exception to this is double-jointed (hypermobility) EDS (hEDS), the most common type, for which at that place is so far no genetic test. The diagnosis of hEDS is made from discovering you have a very specific set of symptoms. This is described nether the description of hEDS beneath.

At that place is no cure for EDS. Treatment is aimed at trying to protect the pare and the joints from farther damage, and to managing other symptoms which arise. For well-nigh people with EDS, generally speaking:

  • Physiotherapy can exist very helpful to build strength and assist remainder, too as to provide support for joints by strengthening muscles.
  • The 'talking therapy' known every bit cognitive behavioural therapy (CBT) is often recommended for anyone living with a chronic status that affects their life.
  • For patients with fragile skin, injuries should ideally be stitched by a plastic surgeon who understands EDS, and stitches should exist left in twice as long equally usual.
  • Anti-inflammatory tablets and analgesics may be required to control hurting.
  • Ascorbic acid is sometimes recommended to reduce bruising.

More detail almost managing EDS is given under the description of each type, below.

The outlook (prognosis) is variable and depends on which type of EDS a person has. Most forms of EDS do not touch on a person'due south lifespan, and people with milder symptoms are able to live their their lives without any restrictions. Nevertheless, the demand to protect the peel and joints in those with more marked symptoms can limit a person's chosen activities - peculiarly those hoping to take function in activities with a run a risk of physical injury, such as rugby - and can cause significant inability.

Many people with EDS accept more severe symptoms and may accept significant disabilities and therefore a astringent restriction of their lives.

If someone has the rare type of EDS that affects the blood vessels (the vascular type) and so there is a risk of bursting (rupture) of big blood vessels or internal organs, and therefore premature expiry.

The classification of EDS into different types has changed several times over the years. In 2017 there was a major change to the current classification. This divides EDS into thirteen types. They are classified by what they are like to experience and to examine (their presentation) and likewise by the gene changes that cause them.

Under the new classification a few people previously diagnosed with hEDS may find that their atmospheric condition no longer 'fit' the new rules. People who already have a diagnosis should proceed their diagnosis, fifty-fifty if the 'fit' to the new definition is not perfect.

This is the most mutual form of EDS. It is inherited but so far no factor has been identified equally the cause. Information technology tin exist mild and is probably often undiagnosed.However many people with hEDS have meaning or even severe hurting and disability.

The main symptoms of hEDS are stretchy skin (which is not delicate simply which may heal slowly) and unusually flexible joints.

Many people with hEDS accept tiredness, hurting and mood changes. The symptoms may include include:

  • Sleep disturbance and tiredness.
  • Fast heart rate.
  • Dizziness and fainting on standing upward speedily.
  • Unexplained tummy pains, constipation and irritable bowel syndrome.
  • Trend to nausea.
  • Anxiety and low and panic attacks.
  • Bug with passing urine.
  • Widespread pain in the muscles and/or limbs.
  • Headaches.
  • Prolapse affecting the bladder, womb (uterus) or back passage.

How is hEDS diagnosed?

hEDS can exist quite difficult to diagnose, as at that place is no specific test for it. As a result information technology oft goes undiagnosed.

When symptoms are relatively balmy, hEDS may be like to benign joint hypermobility syndrome (BJHS). Some doctors think that the 2 weather condition may exist different degrees of the same thing.

Criterion 1: generalised articulation hypermobility (GJH)
This is detected by doctors using a serial of stretching and joint-bending tests which lead to the 'Beighton score'. GJH is diagnosed if you lot have a score or 5 or more (if you're aged under 18 years), 5 or more (if you're aged 18-l years) and four or more than (if you're aged over 50 years). Some doctors also utilise the '5-indicate questionnaire' which asks you lot:

  • Can you, or could you e'er, put your hands flat on the flooring without bending your knees?
  • Tin y'all, or could you ever, curve your thumb to impact your forearm?
  • Every bit a child, could you contort your body into strange shapes, or exercise the splits?
  • Equally a child or teenager, did your shoulder or kneecap dislocate more than one time?
  • Do y'all consider yourself 'double-jointed'?

A 'yes' answer to 2 or more than questions suggests you probably have hypermobile joints.

Criterion 2: two or more of the following (A-C) MUST be present

  • A: yous accept at least Five of the following:
    • Unusually soft or velvety pare.
    • Mild pare hyperextensibility.
    • Unexplained stretchmarks.
    • Piezogenic papules (piffling hard lumps under the skin) of the heel.
    • Recurrent or multiple abdominal hernia(s).
    • Thinned scarring in at least two places.
    • Any sort of prolapse without another reason, such as childbirth.
    • Tooth crowding and loftier, narrow palate.
    • Long, slender fingers (arachnodactyly).
    • Unusually long arms.
    • Floppy mitral (center) valve.
    • Stretched aortic (centre) valve.
  • B: yous have a close family unit fellow member diagnosed with EDS.
  • C: you lot take at least 1 of the following:
    • Pain in ii or more limbs, daily for at least three months.
    • Widespread pain for ≥iii months.
    • Recurrent joint dislocations or instability.

Benchmark 3: all the following MUST be the instance

  • Skin must Not be unusually fragile (this suggests other types of EDS).
  • No other medical explanation for symptoms.

Some of the common symptoms of hEDS, such as tiredness and tummy pain, are not included in the list of symptoms needed for diagnosis. This is because they are normally found in many other conditions too, including other sorts of EDS, so they don't help identify hEDS specifically.

How is hEDS managed?

  • Regular gentle do and keeping your weight within the recommended range for your pinnacle will help go along y'all fit and stabilise your joints with practiced, working muscles. Pilates can be beneficial in helping maintain core stability and maintaining good posture.
  • Some activities are better avoided, in guild to protect the skin and joints from injury. Contact team sports, such as rugby and football game, increase the chance of injury to joints, and it is improve to cull less high-take a chance activities such as tennis or pond.
  • Some people do good from seeing specialists in hurting direction, rheumatology, physiotherapy or occupational therapy.
  • Painkillers may be helpful for generalised pains.
  • Some patients find talking therapies such as CBT helpful.

Is hEDS the same as articulation hypermobility syndrome?

Some doctors believe that hEDS is a more astringent form of hypermobility syndrome and there is certainly an overlap in symptoms. However, hypermobility syndrome mainly affects joints. EDS is more than likely to be the diagnosis where in that location are associated, more serious atmospheric condition, such as center valve problems, prolapse or repeated dislocations.

Classical EDS (cEDS) affects 1 in xx,000-50,000 people. It is autosomal dominant genetic condition, which means that if you lot inherit one affected gene (1 from either parent will do) and so you volition take the condition.

cEDS is like to hEDS but in add-on patients have very delicate skin, which is prone to splitting, particularly over the forehead, elbows, knees and mentum. Those affected likewise have a trend to grade specially sparse scars. People also go rolled-up peel around joints which tin can be easily injured, footling mobile nodules nether the skin and easy bruising. Floppy muscles tin can exist a feature, and children with cEDS may exist dull to stand and walk.

cEDS is suspected if yous have skin hyperextensibility and thin scars, PLUS joint hypermobility. The other possible effects of cEDS include tiredness, poor sleep, tummy pains and prolapse. Sometimes there are issues with the heart valves, which can become floppy, causing a heart murmur.

Management of cEDS

The main way in which management of cEDS differs from hEDS is in the need to protect the fragile peel. The important things are:

  • Protecting the peel to minimise injuries, with clothing and, when needed, protective pads and helmets.
  • If you lot take an injury that needs stitching, this should be done by a plastic surgeon. Stitches need to be left in place for longer than usual and wounds need extra support, with Steri-strips® or bandaging.
  • Children with cEDS need to find a remainder betwixt restrictions and risks. Contact sports such every bit rugby, ice hockey, boxing and martial arts, and loftier-bear upon activities like trampolining, are loftier-risk for injury and may demand to be avoided. Lower-touch on physical activities such every bit badminton, table lawn tennis, bowling and swimming are amend alternatives.
  • Regular gentle exercise is appropriate. Tiredness and joint pain tin exist role of cEDS and regular gentle practise helps to reduce these effects.
  • Adults with cEDS may find Pilates helpful to build strength and help protect joints.
  • Physiotherapy is helpful when joint hypermobility is making joints skid out of place.
  • Occupational therapy tin can suggest aids to daily living and propose on pacing activities to avoid the extreme fatigue that tin can follow a period of over-activity.
  • There are some increased risks in pregnancy which are discussed in the split section 'How do the Ehlers-Danlos syndromes affect pregnancy?', above.

This is as well called tenascin-10 deficient Ehlers-Danlos syndrome (clEDS).

This very rare condition is similar to cEDS, except that affected people exercise not make broad scars. It is autosomal recessive, which means that in club to inherit it yous demand to inherit one faulty gene from EACH of your parents. If a parent has only i faulty cistron each, they will generally NOT have the condition. If they only have one faulty cistron each, they volition more often than not Non have clEDS.

The other features of clEDS which are less common in other types of EDS are long thin fingers, 'flat' anxiety (pes planus), swollen legs, nervus pinch syndromes (such as carpal tunnel syndrome), diverticular illness and wasting of the muscles in the easily and feet.

Management of clEDS

clEDS is managed like cEDS, to a higher place. It is specifically recommended to avoid smoking.

Vascular EDS (vEDS) is the about serious form of EDS. Information technology affects about 1 in 50,000-200,000 people.

vEDS has autosomal ascendant inheritance so just i copy of the lacking factor from either parent will cause the condition. A person may also be afflicted if there is a new mutation in the gene, and and so vEDS may occur in people with no history of the disorder in their family.

The main risk people with vEDS face up is leaking or bursting (rupture) of medium/big arteries. There is besides a small adventure of rupture of the large bowel (colon), and of the womb (uterus) during labour, and of spontaneous pneumothorax (caused by popping of an air space in the lungs). These are life-threatening effects, which commonly occur in immature machismo, making this an extremely difficult diagnosis to be given.

Other features of vEDS include early on varicose veins, premature skin ageing on the easily and feet, and fine, thinning hair, pointing and thinning of the cornea (the transparent front of the centre), and a characteristic facial appearance with small or absent earlobes.

Management of vEDS

Treatment with a medicine called celiprolol greatly reduces the take a chance of arterial rupture. It may as well be possible to repair blood vessels at risk of rupture earlier they do. This is a very specialised area.

vEDS patients should wearable a medical alert bracelet, necklace or like. The EDS National Diagnostic Service has produced a medic alert canvas with the information that may be needed in case of an emergency. Information technology is best to avoid invasive tests or invasive treatments unless strictly necessary, because of the risk of damage to skin and blood vessels.

Cardiac-valvular EDS (cvEDS) causes bug with the heart valves, in addition to other, clEDS-like symptoms. It is a rare condition and in order to inherit it you lot need to inherit i faulty cistron from EACH of your parents. (If they only have 1 faulty cistron each so they will mostly NOT have cvEDS.)

Direction is as for cEDS but with regular centre scans and monitoring, as the heart valves may need repair or replacement.

Arthrochalasia EDS (aEDS) is a very rare, severe type of EDS in which the joints are very, very much looser than they should be. It has autosomal dominant inheritance so just 1 copy of the defective cistron from either parent volition cause the condition. It is commonly diagnosed before long after birth, as babies are built-in with two dislocated hips. They also tend to have rather weak, floppy muscles, and peculiarly stretchy loose peel. Only around 30 cases take ever been described.

In addition to the astringent articulation bug, afflicted people likewise have fragile skin and, sometimes, a tendency to curvature of the spine.

Management of aEDS

The about important problem for children with aEDS is the early hip dislocation, since this severely affects mobility. Most of the usual surgical treatments for this condition do non work, so it is helpful if the diagnosis of aEDs is made before the hips are treated, in order that a method that will succeed is called start.

Repeated dislocation of other joints tin slow children from progress in continuing and walking. The symptoms are worst in infancy and tend to improve with age, so that some children manage to use orthoses (such as braces and calipers) to assist in walking afterward on.

Dermatosparaxis EDS (dEDS) is an extremely rare type of EDS which has been described just around 10 times. Affected people have extremely fragile, sagging skin. It is usually diagnosed before the historic period of two years. Fragility, bruising and sagging of the skin are severe merely, surprisingly, the skin heals well. Like the other rare types, in order to inherit it yous need to inherit one faulty factor from EACH of your parents. If a parent has merely ane faulty factor, they will generally NOT have the condition.

Affected children tend to take a characteristic facial advent. They often have other features seen in classical EDS, such as hypermobile joints, hernias, tiredness, 'thinning' of the bones, floppy muscles, constipation and tummy pain. They tin can also experience abnormalities of the teeth, gums and vision, requiring spectacles.

Management of dEDS

Direction is as for cEDS. A specialist dentist will demand to be involved if at that place are tooth and gum problems, and an ophthalmologist if there are centre problems.

This is a rare blazon of EDS which has been described only around 60 times. Similar the other rare types, in order to inherit kyphoscoliotic EDS (kEDS), you demand to inherit 1 faulty factor from EACH of your parents. If a parent has merely one faulty gene each, they volition generally Non have the condition. It causes worsening (progressive) curvature of the spine (kyphoscoliosis).

Babies with kEDS are very floppy and they stand and walk late. Eyes are besides afflicted and may have a thin surface (sclera) which appears slightly blue and which can (very rarely) rupture. Some afflicted children have hearing problems. Other signs of this blazon of EDS include thin bones and long thin fingers

Joints are very hypermobile and at that place may be frequent dislocations. Pare may be stretchy, soft and delicate, bruise easily and class widened, atrophic scars.

Management of kEDS

This is as for classical EDS only with particular focus on trying to keep the back growing direct and involving a specialist optician to wait later the eyes.

In this rare blazon of EDS the front coverings of the eye are thinner and less strong than usual and the white of the eye (sclera) may appear blue. Like the other rare types, in order to inherit information technology you need to inherit i faulty cistron from EACH of your parents. If a parent has only ane faulty cistron, they will generally NOT take the condition.

People with brittle cornea syndrome are mostly very brusk-sighted, and the globe of the middle is at run a risk of rupturing. They as well have the typical skin and joint features of classical and hypermobility types of EDS.

Management of brittle cornea syndrome

This is equally for cEDS, with boosted care needed for the eyes.

Spondylodysplastic EDS (spEDS) is a astringent EDS. Like the other rare types, in gild to inherit it y'all need to accept inherited one faulty factor from EACH of your parents. If a parent has only one faulty gene each, they volition generally NOT accept the status. Information technology is marked particularly by curt stature and weak floppy muscles. Bowing of limbs is common.

Unlike other types, at that place may be learning difficulties in children. Some of the features of EDS that affect eyes, bones, joints, claret vessels or the lungs may be nowadays and there is a characteristic facial appearance.

Management of spEDS

Management involves the approaches used in cEDS towards pare and joints, together with specialist physiotherapy as your child grows upward - to help maximise their bone growth and force. It is not possible to generalise nigh the degree of learning didactics difficulty children experience, every bit the condition is and so rare.

Musculocontractural EDS (mcEDS) is a rare type of EDS in which muscles in the legs and arms are severely tightened and shortened - these are called muscle contractures. The pare is severely affected too, being very stretchy, fragile and easily hobbling, with the atrophic scars seen in clEDS. It can also impact basic, eyes, the digestive organization and the kidneys. Like the other rare types, in lodge to inherit information technology you need to inherit ane faulty cistron from EACH of your parents. If a parent has just 1 faulty factor, they volition by and large NOT have the status.

Direction of mcEDS

Management of this status follows the patterns detailed above for cEDS, with special attention to any of the center, bone, kidney or digestive symptoms.

Myopathic EDS (mEDS) is a milder subtype of EDS which causes very floppy muscles which may seem very poorly developed at nascence, but which improve with age. Information technology is possible to inherit mEDS if just one of your parents is afflicted, although some 'versions' of mEDS seem to crave you to inherit a faulty gene from both parents.

Management of mEDS

Management is as for the other types, with a focus on physiotherapy and muscle strengthening.

Periodontal EDS (pEDS) causes dental problems, specially inflamed or absent gums and poor molar evolution, on height of typical features of cEDS. It is rare but can be inherited from one parent just, as simply ane faulty gene is needed to develop the condition.

Management of pEDS

Management of pEDS is as for management of hEDS and cEDS merely with additional, specialist dental care.

EDS is a very varied condition, which primarily affects skin and joints but which can also affect many other organs. Thirteen subtypes have at present been classified.

There is no specific cure for EDS but if symptoms are interfering with your life, physiotherapy, gentle do, talking therapies and, sometimes, pain relief are the mainstays of treatment.

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Source: https://patient.info/bones-joints-muscles/ehlers-danlos-syndrome-leaflet

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