Pain
in the Joint Hypermobility Syndrome (JHS)
(Written in language for patients)
Most physicians do not know that the Joint Hypermobility Syndrome (formerly called Ehlers-Danlos Syndrome (EDS) type III) can cause severe recurrent pain and occasionally incapacity. Rheumatologists need to know that JHS is the principal cause of pain in their clinics. After seen more than a 1,000 JHS patients I do not have any doubt that this is true.
The joints in JHS are lax, which makes them
unstable and vulnerable to episodic traumatic inflammation, even with minimal
trauma. The excessive motion of the joints produce abnormal stretching of the
articular capsule, which results in pain. It is for this reason that patients
with JHS have pain. Since these patients have Autosomal Dominant Inheritance
(50% of the siblings will have it), the pain can appear in infancy,
adolescence or adulthood. It can be triggered by trauma, a change in life
style, when starting a new sport or exercise, other disease or even because of
emotional problems. These do not cause it , but they can trigger or aggravate it. Due to the increased
laxitud of the articular capsule, it is possible that the person may
occasionally have the sensation that the joint is going to give way, or get out
of place. If the laxitud is extreme, the patient can suffer a subluxation of
the joint , like it happens at times with the shoulder, elbow or knees. There
are people that can subluxe their joint voluntarily, specially in the Classic
Ehlers-Danlos (CEDS). This type of EDS,
formerly called EDS type I-II is characterized by extreme joint mobility and
recurrent subluxations with minor injury or voluntarily. Especially the Classic Ehlers-Danlos
Syndrome (CSED) formerly called SED type I – II.
It is possible for a JHS patient to have only
one, or a few lax joints, that can be painful. In such cases usually neither
the patient nor the physician know that he has joint hypermobility.
Exercise or sports that require sudden,
gyratory movements of the joints, like ankles in tennis or knees in football or
basketball, can produce sprains and subluxations. Swimming that in general is a
good exercise for JHS, can at times produce shoulder dislocation, especially
when practicing butterfly style swimming. In volleyball and football, finger
sprains and tendinitis or pain in the wrists are common.
Adults doing sports competition or children
whose parents force them to compete, in spite of having problems with the
joints, can end with more pain and complications, such as, tendinitis, sprains
or subluxations.
Since hypermobility is a hereditary alteration
of the collagen fibers, not only the joints are affected, but all tissues are
fragile due to the alteration of the collagen, that is a protein that
constitute the matrix of all tissues. I tell my patients that collagen is like
“ the iron in constructions”, that if weak, makes tissues fragile. It is easy
then to understand that recurrent lesions occur, like tendinitis (“tennis
elbow, Aquiles tendonitis, “trigger fingers”, etc), bursitis, muscular sprains,
rupture of tendons and ligaments that are painful. Also these patients can have
hernias, varicose veins at an early age, they can also have ruptured organs
such as the lungs (spontaneous pneumothorax) or of the gravid uterus during
labor.
The pain can be acute or chronic if it lasts for more than 3 months. Acute pain
usually appears after a blow, due to hyperextension of a joint or as a
consequence of a sudden movement while practicing a sport or exercise and
usually lasts for a short period. Due to the alteration of the tissues, the
great majority of these patients have chronic pain., that can be of varying
degrees. Severe pain can alter quality
of life and at times can be incapacitating. These fragile tissues take longer
to heal. In the cases in which there is cartilage rupture, since it has no
circulation, the healing is not good and the new cartilage is not normal, but
fibrotic and this ultimately leads to osteoarthritis. This condition is painful
and since it has a degree of inflammation, we prefer to call it osteoarthritis
rather than arthrosis. It is a kind of rheumatism due to wear and tear of the
joints that we all get with age, but these patients get it at an early age.
Patients should not confuse Osteoarthritis with Rheumatoid Arthritis that is
more inflammatory, can destroy joints producing deformities and can be
disabling.
Osteoarthritis appears in JHS patients, due to
abnormal motion of the joint (like wheel misalignment) and because the cartilage
is not of good quality, due to the collagen alteration. I tell my patients
“that they have tires that are not Michelin”. Besides measures to
control pain, it is necessary to reduce activities that aggravate the injury
and also is necessary to avoid being overweight. It is also necessary to
fortify the key muscle of the joint, like the quadriceps for the knee, by doing
daily exercises.
Chronic pain alters quality of life, by
reducing sleep, sexual functions, social and working relations, physical
activities and recreation.
The physician needs to decide if the pain is
due to overuse of the joint, to trauma or due to early osteoarthritis, since
the treatment is different. Interestingly enough, the pain in JHS is more than
that, since at times it may look like it lacks anatomical distribution, can be diffuse,
can be associated to emotional problems (suffering), can be aggravated by
chronic fatigue, anxiety, frustration and resentment, due to familial and
doctor incomprehension and the poor results from different treatments. Some
have termed this condition ”the invisible disease”, since the person looks
healthy and the laboratory test are normal. Frequently we receive patients that
have seen many different specialists and had done all kind of laboratory tests,
multiple x-rays, scans, magnetic resonance, ultrasound, bone scintigraphy,
etc., which have given negative results. Because of this, they are frequently
labeled as lazy, antisocial, uncooperative, and depressed, with chronic fatigue
or Fibromialgia. The truth is that many JHS patients have Dysautonomia, that
because of low blood pressure causes fatigue, dizziness or even syncope. The
diagnosis of Fibromyalgia has always been doubtful as a true diagnosis in the
mind of many rheumatologists and usually is related mainly to stress. I believe
that it shares with JHS many signs and symptoms and for many authors they
usually go together, but to me, I feel that in many cases they are the same
disease. I wrote an article referring to “JHS as the Fibromyalgia of the XXI
century”, that can be seen in my Web Page (www.reumatologia-dr-bravo.cl) .
Another painful problem that is seen in young
people with JHS is cervical or lumbar disc disease, including herniated nucleus
pulposus. In JHS the intervertebral disc disease is caused by the weakness of
its collagen.
It is useful for the patient to recognize and
accept that she/he has chronic pain, to try to help herself and to see the
doctor or Kinesiologists. It is necessary for them to see someone with expertise
in the subject and to learn which activities do not harm the affected joints
and to better select which exercise or sport to practice. It is also important
in the selection of which professional career to pursue. Many patients find by
themselves a way to do things with less damage to the joints and with energy
saving. For some people it is beneficial to prioritize the tasks that need to
be done, in this way, patients with chronic fatigue (Dysautonomia), that is
frequent in JHS (64% of JHS women younger than 30 years old, as seen in our
recent study of 1,000 patients), prefer to do most of their activities in AM
and to rest in PM, when they have less energy.
It is necessary to discontinue the activity or
sport, before the pain caused by it gets to be extreme, since at that time the
treatment is less efficacious. Extreme tiredness or exhaustion leads to falls
and fractures. Due to the collagen alteration the bones in JHS show low bone
mineral density (BMD). We are seeing every day more Osteoporosis in young
JHS patients, including adolescents of both sexes. We found Osteoporosis in
19% of males and 19% of females, in our recent study. It appears necessary to
do Densitometries to JHS patients at any age. I would recommend doing it after
puberty, since at that time there is a great increase in BMD. Patients need to
learn how to evaluate pain intensity in a scale from 0 to 10, in which 0 is
absence of pain and 10 is maximal pain. This helps to stop the activity that is
causing the pain when this is high, but before it gets to be extreme. It also
helps to communicate the degree of pain to the doctor. It is also useful to
learn how to call different types of pain. Pain can be acute or chronic, like a
pinprick, dull, pins and needles, burning, electric, that moves, permanent or
comes and goes, it can be generalized (“everything hurts”), irradiates to..,
etc. Lately has been known that JHS patients have poor response to local
anesthetics, such as when having a dental procedure or when having sutures
for a laceration. Another factor that participates in JHS pain is a propioception
alteration. This means that the patient does not know exactly where parts
of his body are at the time of examination. With the eyes closed he would not
be able to determine if the big toe is up or down, when moved by the examining
doctor. This propioception alteration makes these patients more liable to
traumatic lesions and falls.
How to protect hypermobile joints:
·
Avoid vicious
positions of the joints, like standing with the knees backward, in case of genu
recurvatum.
·
Avoid static
positions, it is better to move and to change positions on and off. Activity is
better than inactivity. If the hands or feet feel are swollen, it is convenient
to move them, since improving circulation will decrease the discomfort. This
happens at times when walking with the hands hanging, without motion and also
during hot weather days.
·
Do not
hyperextend too much the joints, because this produces pain at the moment and
Osteoarthritis in the long run.
·
It is necessary
to protect fingers and wrists from sprains and to use a splint if necessary
(better with metallic reinforcement and velcro strap).
·
Do not rotate the
joints brusquely, as it happens in basketball or tennis. Also avoid sudden
turns of the neck or back, at times it might be better to move the spine as a
block.
·
Plan the daily
work and other activities to avoid fatigue. Remember that small periods of rest
are beneficial.
·
Use the bigger
joints and the most potent muscles to do the task. For example, it is better to
lift a weight with the forearms and not with the hands and to carry it near
your body than far away.
·
The pain is
indicating that there is a need to give rest to the joint, to avoid any harm to
it.
·
Use a wheelchair
or an electric cart, if needed, this will alleviate joint pain, increase
mobility, decrease fatigue and improve quality of life. Do not let appearances
interfere with your decision to use a cane, walker, wheel chair or an electric
cart if you need it.
In an acute process the presence of pain and
joint swelling indicates inflammation and rest and anti-inflammatories are
needed. Hot or cold packs and splints will help. A plastic bag with
frozen peas or with ice or a hot pack, will reduce swelling and pain. It is
necessary to be careful and prevent skin burns by placing a towel between the
skin and the hot pack.
The use of Ultrathermia and Ultrasound
is recommendable. Massage is beneficial to reduce muscle spam or the muscle
contraction that might be present. Partial or total body hot baths (Hydrotherapy)
are of help, as long as this is not too hot or too long, since this can produce
hypotension, fatigue and occasionally a syncopal episode, especially in JHS
patients that have Dysautonomia.
Mild exercises, supervised by a Kinesiologists with expertise
in joint hypermobility, will help to increase the tone of muscles and tendons.
Even though it appears strange, you can do complete range of motion exercises
of the lax joints, as long as they do not give pain. In general we recommend
Pilates, Yoga, Tai Chi, swimming or bicycling.
Electric stimulation (TENS units) produces the same effect
as Acupuncture and both can decrease pain in these patients. Bio-feed
back techniques are used for chronic pain.
Medications
Anti-inflammatories. We call them Non Steroidal Anti-inflammatory
Drugs (NSAIDs), meaning that they do not contain steroids, like cortisone.
They are used when there is pain and inflammation, like in tendinitis,
bursitis, arthritis, etc. One commonly used, in low doses and for a few days,
is Ibuprofen (Motrin), which at this doses acts mainly as analgesic. Higher
doses, such as 400 mgs 3 times a day are anti-inflammatory and should be taken
with food to prevent gastric irritation. High doses need to be prescribed by
physicians, because they can give gastric, hepatic and renal side effects.
NSAIDs are dangerous because of the possibility of developing a peptic ulcer,
with or without bleeding. Remember that a black, tarry stool is an indication
of GI bleed, since digested blood is black. If this happen, the patient needs
to stop the medication right away and see the doctor as soon as possible. These
recommendations are particularly important if there is a history of peptic
ulcers, anticoagulant therapy and also previous to a surgical procedure. The
capillary fragility that exists in JHS increases the risk of bleeding. There
are many anti-inflammatory medications like Diclophenac, Naproxen, etc The new
anti-inflammatories called Anti Cox 2, have less tendency to produce GI
problems and less bleeding tendency. In this category you can find: Meloxicam
(Hyflex, Isox, Mobex, Tenaron, etc.) and Celecoxib (Celebra).
Anti-inflammatories need to be prescribed by physicians, since it is necessary
to be sure (with laboratory tests) that there are no gastric, hepatic or renal
problems. The anti-inflammatory creams like Diclophenac cream, have a
moderate local effect. There are also analgesic creams, like Capsaicin,
that is eye irritant, since is made from Chili.
Muscle relaxants. These
medications (Cyclobenzaprine) are beneficial because muscle pain is due to
muscle contraction. They also help to sleep, but since at times this can
interfere with daily activities, is that we give only half a tablet (5 mg) at
night. Local heat and massage also produce muscle relaxation.
Support Groups.
It is
useful for these patients to alternate with people with similar problems,
comment their symptoms, anxiety and frustrations. This helps them to understand
better their illness, be more positive and learn how to prevent problems. Many
of them tell us that they live afraid, with anxiety and pain, with lack of
family and social support and of not being taken seriously by physicians.
Treatment and prevention of Osteoarthritis.
Osteoarthritis
(wear and tear of the joints) appears early in these patients, due to
the hypermobility of the joints and poor quality of the cartilage. For this
reason, it is necessary to prevent the wear and tear of the joints maintaining
an adequate weight, improving the tone of the key muscle of the joint (i.e.
quadriceps for the knee), avoiding excessive hyperextension of the joints,
avoiding contact sports and taking medications long term to prevent articular
damage, like Glucosamine and Condroitin Sulphate.
Treatment of Dysautonomia (chronic fatigue, dizziness and syncope).
The treatment for Dysautonomia
consists in general measures and medications and is very efficacious. For
details see my web Page www.reumatologia-dr-bravo.cl
and also see “Treatment
of JHS”.
Homeopathy and Alternative Medicine.
We cannot overlook the fact that some patients
have had good results with Homeopathy or some form of Alternative Medicine, including
Apiculture (bee stings). On the other hand it is important to know that natural
medicines can also be dangerous, like snake venom or bee sting, that can be
fatal in cases of allergy, etc. Also is necessary to consider the fact that
sometimes precious time is lost, while trying different alternative medications
and the real, well know treatment is delayed.
It is known that 50% of JHS cases are due to
Autonomic Dominant Inheritance (50% of the siblings will inherit it), but the other
50% are secondary to genetic mutations. For reasons that I explain in my Web
Site, it is my impression that these mutations are due to
lack of Folic Acid (FA), during the periconceptional period. It has
been proven that the lack of this vitamin (B-9), during this critical period
can alter the genes and produce Neural Tube Defects (Espina Bifida) and other
congenital malformations. Further more there are studies that show that
Homocysteine elevation, due to lack of FA, is associated to osteoporosis, which
we frequently see in JHS, even in young patients. I prescribe FA, 0.4 or 1 mg a
day permanently, to these patients thinking that it can help in the prevention
and also as a treatment of the condition. It has no important
contraindications, except if given to patients with Pernicious Anemia, and is
beneficial to prevent arterial and venous damage, improves memory, does not
make patients gain weight and is not expensive.
Jaime F. Bravo, MD
Rheumatology-Osteoporosis
November 9, 2005
Revised: May 1, 2007
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