Joint Hypermobility in Plain Words.
ALAN G. POCINKI, MD, PLLC
2141 K Street, NW, Suite 600
Washington, DC 20037
Joint Hypermobility and
Joint Hypermobility Syndrome
...
Dedicated to my hypermobile patients, from whom I have learned so much. I’ve
seen hypermobility syndrome, but you’ve lived it.
Many people have flexible or loose joints. They’re the people, maybe
like you, who did gymnastics or ballet when they were young and are
“good” at yoga.
Their joints move farther and more easily than most people’s joints,
so they often can do tricks like bending their thumbs forward until
they touch their forearms. Sometimes these people are called
“double-jointed,” and some may even have dislocated or popped their
joints out of the socket. The medical term for joints that move too far
is hypermobility, and the word for joints that are too loose and move
too easily is laxity.
Experts estimate that up to 10% of the general population may have some
degree of hypermobility, with women affected about three times more often than
men. Most hypermobile people do not develop any problems from their loose
joints, but some suffer chronic pain and other symptoms. Those who do suffer
chronic joint pain and other symptoms related to their hypermobility or to the
looseness of other tissues that often accompanies hypermobility have a condition
called joint hypermobility syndrome (JHS).
Often, people who suffer from hypermobility syndrome are called
hypochondriacs or lazy because they avoid many everyday activities,
because these
activities that cause them pain. Most of them don’t look sick and, as a result,
friends, colleagues, and even doctors can be unsympathetic. Furthermore, they
may spend years unsuccessfully searching for the cause of their chronic pain
and other symptoms because many doctors are unfamiliar with hypermobility
syndrome and its complex set of symptoms. Such long delays and lack of
understanding can lead to frustration (with doctors and with daily life), anger,
anxiety, and depression.
Hypermobility
The Beighton score is used to measure a person’s degree of
hypermobility. One point is assigned for the ability to accomplish each
of the following
movements:
• bending your small finger back further than 90 degrees (1 point each side)
• bending your thumb forward to touch your forearm (1 point each side)
• hyper-extending your elbows and knees, that is bending them beyond a
straight line (1 point each joint, each side)
• putting your palms flat on the floor without bending your knees (1 point)
Hypermobility – and hence the Beighton score – often decreases with age, as
joints become less mobile, both as a natural result of aging and because loose
joints are predisposed to premature osteoarthritis, the wear-and-tear form of
arthritis.
Joint Hypermobility Syndrome
Joint hypermobility syndrome can include a wide and diverse array
of symptoms, but the muscles and joints are most often affected, giving the
syndrome its name. People with JHS often develop chronic joint pain and
stiffness, most often in the larger joints; for example, the joints of the neck,
shoulders, back, hips, and knees. However, smaller joints such as the ankles,
wrists, and elbows often are affected as well. Joint pain often comes from the
muscles and tendons around the joint rather than the joint itself, so that x-rays
may be normal.
People with hypermobility syndrome may have a whole group of other
conditions, in addition to joint problems, because of excessive stretchiness of
other body tissues. For example, mitral valve prolapse and uterine prolapse,
hernias, and gastroesophageal reflux disease (GERD) are more common in
people with hypermobility syndrome.
The Brighton criteria generally are used to decide if a person with
hypermobile joints also suffers from JHS.
Major Criteria
• Beighton score of 4 or more
• Joint pain affecting 4 or more joints and lasting more than 3 months
Minor Criteria
• Beighton score of 1, 2, or 3
• Back pain or pain in one to three joints lasting more than 3 months, or spinal
spondylosis, spondylolysis, or spondylolisthesis
• Dislocation of more than one joint, or of one joint more than once
• 3 or more soft-tissue problems (e.g., tendonitis, bursitis)
• Tall, thin body shape
• Skin hyper-extensibility, stretch marks, thin skin, or abnormal scarring
• Drooping eyelids, nearsightedness
• Varicose veins, hernia, or uterine or rectal prolapsed
• Mitral valve prolapse
Any of the Following Required for Diagnosis
• 2 major criteria
• 1 major and two minor criteria
• 4 minor criteria
• 2 minor criteria and a clearly affected first-degree relative
And there are even more symptoms. Unexplained bruises often appear
“out of nowhere.” Many hypermobile people complain of dry mouth or constant
thirst, often with a craving for salty foods. They are uncomfortable standing for
long periods, so avoid lines and like to sit with their feet up. Many patients with
JHS also have problems with their autonomic nervous system, the part of the
nervous system that regulates circulation, breathing, and digestion. This can
lead to symptoms such as lightheadedness, palpitations, and digestive problems,
and probably plays a role in difficulty sleeping and overall fatigue, which also are
common complaints.
Some experts believe that JHS is the same thing as the hypermobile
type of Ehlers-Danlos syndrome (EDS), a condition also caused by extreme
elasticity of body tissues. People with the hypermobile type of EDS have loose
joints and soft, velvety skin, as well as other symptoms almost identical to those
described here. For most people, the distinction between joint hypermobility,
joint hypermobility syndrome, and the hypermobile type of Ehlers-Danlos is not
an important one clinically, as treatments are similar. Exceptions include more
severely affected patients, such as those who require braces or surgery to
stabilize their joints, or those who have unusual symptoms such as weakness or
loss of feeling in arms or legs, and those with certain eye problems or a family
history of aneurysms, all of whom if possible should see a specialist with
knowledge of EDS, in part to rule out other more serious types of EDS. Those
considering having children may wish to see a clinical geneticist to see if they
carry any of the known genes associated with Ehlers-Danlos, although many
such genes have not yet been identified. People with lax joints fall along a
broad spectrum, from those with joint hypermobility but only mild or no
related symptoms, to those moderately affected by JHS, to those more
severely affected, many of whom truly have the hypermobile type of EDS.
Joint Pain and Muscle Strains
Joint hypermobility syndrome, as the name implies, primarily affects the
musculoskeletal system. Loose joints cause increased strain on nearby soft
tissues (muscles, ligaments, tendons) that stabilize them. These soft tissues
themselves often are overly lax, and because of their laxity and the increased
strain on them, they are prone to tearing and spasm, leading to pain and stiffness
around joints. The pain may or may not be clearly related to any specific activity.
For some, any repetitive movement, such as walking, lifting, or carrying can be
painful. Standing or sitting for any period of time can cause stiffness and pain, as
can something as simple as cleaning a kitchen counter or bending down to pick
up laundry.
Because of their role in stabilizing the trunk and the head, the neck and
lower back are almost always affected. Chronic neck strain affects nearly
every patient with JHS for two main reasons. First, the ligaments that are
supposed to support the head are too loose and therefore cannot do their job
well. The muscles of the neck are forced to do more of the work of supporting
the head than they are meant to do, so they become strained. Second, most
JHS patients have shoulders that are too loose, that is the “ball” of the upper arm
is not held tightly in the “socket” of the shoulder. Because of the weakness of the
shoulders, almost any activity that uses the arm, including reaching, pushing,
pulling, and carrying, pulls not only on the shoulder but also on the neck. For
these two reasons, neck muscles are constantly being strained, and what little
healing may occur overnight is promptly undone the next day. Remarkably, this
process occurs so gradually that many people with JHS do not even notice it,
and when asked they may say, “My neck is fine,” when in fact their necks are a
mass of knotted soft tissue, soft tissue that does not feel soft at all!
Lower back pain also is very common in people with JHS, again for a
number of reasons. As in the neck, the ligaments that should support and
stabilize the spine and pelvis usually are too loose, putting extra strain on
muscles to try to support the upper half of the body. Like the relationship of
the shoulders to the neck, loose hips also put extra strain on the lower back to
try to stabilize the pelvis. Among these muscles is the piriformis muscle, a small
muscle at the base of the pelvis (in the buttock). When called upon to play a
bigger part in supporting the pelvis than it is meant to, it can easily be strained.
Once strained, it may tighten up and pinch the sciatic nerve, which runs directly
beneath it. The resulting pain, called sciatica, can be felt in the buttock and often
radiates down the back of the leg. This condition, sometimes called piriformis
syndrome, is often mistaken for a pinched nerve from a ruptured disc in the
spine.
People with JHS do have an increased tendency to have disc problems,
sometimes at an early age, because the intervertebral discs that help cushion
and support the spine may be less rigid than normal. Softer discs are more
prone to leak or rupture, allowing disc material to ooze out of the disc and pinch
nearby nerves, causing pain. Disc problems in the neck cause pain down the
arms, and discs in the lower back cause pain to be referred to the legs. Less
often, tissues within the disc itself can break down, causing pain within the disc,
which can be very difficult to treat.
Some people with hypermobility also develop neuropathic pain, which
may be felt as burning, stinging, tingling, shooting, numbing, etc. Sometimes
such pain is caused by disc problems, but often it is quite localized or does not
follow the usual patterns of pinched nerves. Conventional nerve testing usually
is normal, so these symptoms may be attributed to psychological rather than
physical causes. This type of pain also can be particularly difficult to treat.
As mentioned earlier, osteoarthritis occurs more rapidly in loose joints.
Therefore, arthritis in the neck and lower back is another frequent cause of neck
and back pain and stiffness in JHS patients.
Hypermobility also commonly causes pain in the hips, shoulders, knees,
and elbows. The shoulder in particular depends a great deal on its ligaments for
support, and when the ligaments are loose, there is extra strain on the soft
tissues of the shoulder. When these tear, tendonitis often develops. Similarly,
hyper-extending the elbows can tear the tendons on the sides of the elbow. Pain
in this area often is referred to as “tennis elbow” and “golfer’s elbow.” In addition,
many people with JHS suffer frequent ankle sprains, which like shoulder and
elbow injuries, may take a very long time to heal because they tend to get injured
again and again while they are trying to heal.
Unstable hips often cause pain which, like neck pain, may go unnoticed
for a long time, since the hip joint does not move around as much as the knee,
shoulder, and ankle joints. Examination of the hip in people with JHS often
causes them to cry out, “Ouch! I didn’t even know it hurt there.” Also, many
people mistakenly describe pain from the back part of the hip joint as “back pain.”
The most common source of knee pain in hypermobile people is the
cartilage between the kneecap and the knee. Because the soft tissues that are
supposed to hold the kneecap in place are loose, the kneecap itself is often
loose. After years of sliding around too much, the cartilage underneath the
kneecap starts to wear down (a condition referred to as chondromalacia),
causing pain – and sometimes a crunching or grinding noise – while kneeling,
squatting, or climbing stairs. Osteoarthritis of the knee joint itself is not as
common, but it can occur, especially in those who have done years of highimpact exercise or who are overweight.
Other joints that can be affected include the joints where the ribs meet the
breastbone and where the ribs meet the vertebrae of the spine. Many people
with JHS feel chest pain and tightness, and may even seek emergency care to
rule out heart disease, when the source of their symptoms is the joints of the rib
cage, a condition called costochondritis, or inflammation of the rib cartilage.
In addition, the jaw, or temporomandibular joint (TMJ), is often affected by
hypermobility. Just like other joints, looseness of this joint leads to strain on the
muscles around it and wear of the cartilage in the joint. A variety of treatments
are used for TMJ pain, but strengthening and stabilizing the joint offers the best
hope for many for long-term relief.
Finally, there is an association between hypermobility and increased risk
of osteoporosis, although it is not clear whether this is simply from inactivity
because of pain or, more likely, that there is a specific defect in bone
metabolism. The identification of receptors for adrenaline-like hormones in the
bone suggests the possibility that loss of bone may even be related to autonomic
nervous system dysfunction (see below).
Treatment of Musculoskeletal Symptoms
The treatment of musculoskeletal symptoms primarily consists of
medication for pain relief, and exercise and physical therapy to relieve muscle
spasm – in the short run – and strengthen the tissues around loose joints to
stabilize them – in the long run. These are general treatment principles, which
are more easily applied and more effective for some joints than others.
A brief word about pain: pain is not a good thing. “Toughing it out,”
“putting up with it,” “learning to live with it,” etc. are not productive approaches to
dealing with chronic pain. “Medication can’t be good for me,” is similarly illogical.
Pain is not good for you. Pain strains your system, wears you down, disturbs
your sleep, and makes you irritable and even depressed. Medication that
relieves pain often does a lot more; it often improves sleep, concentration,
energy, and mood. Be hopeful that with appropriate treatment you will gradually
need less and less pain medication, but take it when you need it.
Finding a doctor – whether a family practitioner, internist, orthopedist,
rheumatologist, physiatrist, osteopathic physician, or any other physician –
knowledgeable about hypermobility is critical. A physical therapist or personal
trainer who is familiar with hypermobility is another valuable resource, especially
in helping to develop an appropriate home exercise program. Many people with
joint discomfort start exercising, only to find that their symptoms get worse. The
basic rules of exercise I recommend are:
• AVOID high-impact exercises, such as sports that involve running, jumping,
or physical contact. Swimming or water exercises, walking, Pilates, and Tai
Chi are good choices. Some forms of yoga are OK, but others are not – see
stretching, below.
• AVOID most forms of stretching that involve grabbing a joint and pulling or
pushing on it to “loosen it up.” Many people are reluctant to give up
stretching, because it “feels so good.” But in this case, pulling on tight
muscles does not relax them, and the relief is only temporary. Stretching
further strains and loosens joints. This is why many hypermobile people
make their conditions worse by doing yoga. However, stretching hamstrings
is OK and an important exception.
• AVOID heavy lifting, pulling, and pushing. Be particularly careful around the
house and in the yard, where odd-shaped weights and unusual angles often
lead to injury.
• AVOID hyper-extending your joints. That is, don’t straighten your arms out
fully so that your elbows lock, and don’t straighten your knees to the point that
they lock. This applies to carrying groceries or a gallon of water just as much
as it does to doing exercises with weights.
• DO light resistance exercises, which are the cornerstone of strengthening and
stabilization. Any degree of straining only causes injury. For weights, a good
rule of thumb is that if you can’t do 8 repetitions (reps) without straining, then
the weight you have chosen is too heavy for that exercise. You should not
consider increasing weight or resistance level until you can do two sets of
15 reps without straining. In general, more reps with a lighter weight are
preferable to fewer reps with a heavier weight. Most people find no need for
weights heavier than 10 pounds; hand weights of 2 or 3 pounds for some
exercises and 5 pounds for others are usually sufficient.
• DO be persistent and consistent. You don’t need to spend an hour in the
gym. Even on days when you are “too tired” or “don’t have time” to exercise,
5 minutes of light weights for shoulder strengthening and 5 minutes of
isometrics for core strengthening every day will yield big benefits in the long
run. No “two week cure” here!
Fibromyalgia
Fibromyalgia is a common diagnosis in people with JHS. Once muscles
around loose joints become strained and painful with daily use they may become
more and more painful, until chronic unrelenting pain begins to disrupt sleep and
cause fatigue and depression. Each of these symptoms reinforces the others.
For example, depression can disrupt sleep, cause fatigue, and increase
sensitivity to pain, setting up a vicious cycle of pain, fatigue, poor sleep, and
depression, which is the crux of fibromyalgia. Hypermobility also may predispose
people to develop chronic fatigue syndrome, which has much in common with
fibromyalgia.
Extra-articular Manifestations of Hypermobility
Problems affecting parts of the body other than the joints are referred
to as the extra-articular manifestations of hypermobility. Lax joints are very often
associated with increased tissue elasticity elsewhere in the body, especially
in the blood vessels and digestive tract. In recent years, hypermobility also has
been associated with a variety of autonomic nervous system problems.
JHS and the Autonomic Nervous System
The autonomic nervous system regulates all body processes that occur
automatically, such as heart rate, blood pressure, breathing, and digestion. To
compensate for stretchy blood vessels and increased venous pooling (too much
blood collecting in over-stretched veins) most people with hypermobility appear
to make extra adrenaline, which may account for the high-energy,
always-on-thego lifestyles of many hypermobile people. Unfortunately, if
you get too tired, your
body responds by making more adrenaline, so you keep going, not realizing how
tired you really are. It appears that as you get more and more run down, your
body gets more sensitive to adrenaline, so the small amount you have left can
produce the same response a larger amount used to, so you still don’t feel tired
even when you are. Even when you do feel tired, you may continue to “push
through” the fatigue, collapsing when the adrenaline wears off. Years of not
feeling, ignoring, or pushing through fatigue may be one factor in the
development of illnesses like chronic fatigue syndrome.
Many of the autonomic nervous system problems associated with
hypermobility are characterized by an “over-response” to physical and emotional
stresses, which often leads to fluctuations in heart rate and blood pressure, as
well as digestive and respiratory symptoms. Sickness, pain, emotional stress,
and even fatigue itself can raise adrenaline levels, and acute stresses can trigger
adrenaline surges, leaving you jittery, anxious, and even more exhausted.
Worse, such surges can trigger an excessive counter-response, causing nausea,
sweating, lightheadedness, diarrhea, and of course even more fatigue. Even
sensory stimuli, such as bright lights or loud noises, can trigger an exaggerated
or over-response, causing sensitivity to light and sound.
Perhaps the most common symptom of autonomic nervous system
dysfunction in hypermobile people is orthostatic intolerance, or
lightheadedness on standing. Tilt-table testing often reveals abnormalities such
as neurally mediated hypo-tension (NMH) or postural orthostatic
tachycardia syndrome (POTS), fancy names for different ways in which the
body fails to maintain a stable heart rate and blood pressure when a person
stands up. Increasing salt and fluid intake, and avoiding caffeine and alcohol,
which deplete the body of fluid, may reduce such symptoms. It also helps to
keep your feet elevated, wear support hose, avoid prolonged standing, and of
course the obvious—if you get dizzy when you stand up quickly, don’t stand up
quickly!
Hypermobility and the Heart and Circulation
Because too much blood is pooling instead of circulating, people with
JHS typically have cold hands and feet and low or low-normal blood pressure,
in addition to lightheadedness on standing. Drops in blood pressure can trigger
palpitations and racing and pounding of the heart. There is also an increased
risk of migraine headaches, varicose veins, and hemorrhoids.
Although experts disagree on whether or not there is an increased risk
of mitral valve prolapse in hypermobile people, many people with mitral valve
prolapse are loose-jointed. Fortunately, for most people, mitral valve prolapse
does not pose any serious risks. The term mitral valve prolapse syndrome
has been used to describe the various autonomic and other symptoms that many
people with mitral valve prolapse have, but in many cases, these symptoms are
probably related to their underlying hypermobility.
Atherosclerosis (clogged arteries) is uncommon in people with
hypermobility, in part probably because their blood pressures tend to be low and
because most tend to have low cholesterol. However, unusual heart problems
can occur. One of the most serious cardiovascular concerns in hypermobile
people is an increased tendency for blood vessels to tear or even rupture,
although this is primarily a concern for people with the more serious vascular
type of Ehlers-Danlos syndrome.
Hypermobility and Headache
People with lax joints are predisposed to many different kinds of
headaches. Migraine headaches are very common, in part because many
migraines are triggered by fluctuations in hormone levels or blood pressure,
which can be increased by autonomic problems. Headaches from chronic neck
strain also are very common and can often turn into migraines. In addition,
severe autonomic problems can cause a dehydration or “hangover”-like
headache, possibly related to inadequate blood flow. Uncommonly, looseness
of the muscles that control the eyes can cause difficulty focusing and eye strain
headaches. TMJ problems can also cause headache.
Hypermobility and Digestion
Hypermobile people often have digestive problems, both in the upper and
lower parts of the gastrointestinal tract. The esophagus and especially the
tissues around it may be too stretchy, allowing stomach contents to come back
up or “reflux” into the esophagus. Because the stomach contains acid, and the
esophagus is not meant to hold acid, acid reflux causes heartburn in many
people with JHS. Furthermore, frequent reflux can cause serious burning and
scarring of the esophagus, even increasing the risk of cancer of the esophagus.
A stomach that is overly stretchy can cause food to stay there too long, a
condition called delayed gastric emptying, which can cause patients to feel full
quickly—and sometimes continue to feel full for many hours--and can also
increase the risk of reflux. Autonomic problems can also affect digestion, e.g.
causing digestion to occur too slowly or too quickly depending on the situation.
Intestines that stretch too easily increase the risk of constipation and
bloating, and make gas much more painful. Many hypermobile people are
diagnosed with irritable bowel syndrome, a diagnosis that often carries a
negative connotation – that the patient isn’t dealing with stress well or is really
depressed and won’t admit it – when in fact these symptoms, and their cause,
are often physical and not psychological.
Tears of the abdominal wall muscles are another very common problem
among people with hypermobility. The muscles themselves do not tear, rather
the fibers connecting different muscles do, creating a gap between two muscles.
Small segments of intestine can occasionally push up through these gaps,
causing pain. As pressure backs up behind this “stuck” segment, pain gets
worse and is felt in a larger area, but eventually resolves when the intestine
that has pushed up into the layer of muscle falls back into place. The timing of
the pain is quite random, depending only on the movement of bowel contents
and the contractions of the intestines. The lack of any correlation to meals or
bowel movements is one clue to the cause of the pain.
Unfortunately, these abdominal wall tears usually will not be found on a
cursory physical exam, nor on x-rays, CT scans, or sonograms, and most
physicians think they are rare and so do not look for them. Depending on where
the tears are located, patients may be incorrectly diagnosed with reflux, ulcer,
gallstones, ovarian cysts, diverticulitis, and most often, irritable bowel syndrome.
Once patients understand the source of the pain, most can tolerate it, or find
ways, such as changing position, to relieve it. Surgical repair is rarely necessary,
except when true hernias occur, i.e. when intestine pushes through the
abdominal wall muscles and stays there. The most important reasons to make
this diagnosis are to prevent unnecessary testing and treatments for other
incorrect diagnoses, and to reassure patients that they don’t have something
terribly wrong that hasn’t shown up in the tests they’ve already had.
Hypermobility and Anxiety
The body’s tendency to overreact to stresses by making too much
adrenaline can lead others to think that hypermobile people are “too sensitive,”
“irritable,” or “anxious.” Patients themselves may notice this, saying, “I’ve always
overreacted to little things. I can’t help it.” It is very important to recognize two
things about this phenomenon. First, it is a physical reaction, so that counselling
usually will not be effective in treating this type of anxiety. Similarly, adrenaline
highs and lows may be mistaken for the mood fluctuations of bipolar disorder, but
mood-stabilizing medications usually are not indicated. When medication is
required, beta blockers, which block adrenaline, may be as effective with fewer
side effects than SSRI’s like Prozac and Lexapro or benzodiazepines like Xanax
and Valium. Second, while a feeling of anxiety can be produced by emotional
stress, it is just as likely that such symptoms have a physical cause, most often
fatigue, pain, or dehydration, and less commonly by a drop in blood sugar or
blood pressure. Not surprisingly, researchers have found that anxiety and panic
disorder are more common in hypermobile people.
Hypermobility and Sleep
Similarly, when hypermobile people try to fall asleep, the stimulating effect
of their extra adrenaline may keep them awake. If they are able to fall asleep,
they may continue to make too much adrenaline overnight, giving them a
shallow, dream-filled sleep, so that they wake feeling unrefreshed. Pain further
stimulates adrenaline, making restful sleep even more difficult. When studied in
the sleep lab, they often have a relative and sometimes complete lack of deep
sleep, and/or an increased number of sleep-disrupting “arousals.” Poor sleep
can cause irritability and fatigue, which in turn can trigger more adrenaline (to try
to overcome the fatigue), which in turn can make sleep worse. This vicious cycle
can eventually cause serious disability.
Like fatigue and pain, many patients are not aware of just how bad their
sleep is. Although some people are aware of waking often or of having frequent
very vivid dreams, many will insist that they “sleep fine,” even while admitting that
after sleeping 8 hours they don’t feel rested when they get up. One obvious
reason for this lack of awareness is, of course, that they’re asleep, so they have
no way of knowing that they’re not getting enough deep sleep or having way too
many arousals. Sleep studies done in a sleep lab are very helpful in
demonstrating the nature and severity of sleep problems, and in ruling out other
sleep problems like sleep apnea and periodic limb movements of sleep, which
can coexist with hypermobility-related sleep problems. Recently home sleep
monitors that can measure sleep stages (shallow, deep, REM) and arousals
have become available. While not as good for initial diagnosis, since they don’t
detect apnea and limb movements, they are often helpful for monitoring and
adjusting treatments over time.
Non-pharmacologic treatment of sleep problems begins with the basic
rules of good sleep hygiene, such as trying to sleep the same hours each night,
and using your bed only for sleep, not for work or watching television. Don’t eat,
exercise, or do anything stimulating too close to bedtime, and certainly don’t
have caffeine or alcohol in the evening. Be wary of medications, like
over-thecounter decongestants, that can interfere with sleep.
When these measures fail, then medication is indicated, since virtually
every system in the body is strained when you don’t get a good night’s sleep.
Not sleeping well not only makes you tired and irritable and can affect your
mood, it also affects mental functions like memory and concentration, and has
recently been shown to be a major contributor to weight gain in some people.
Besides treatment for sleep apnea and limb movements when these are present,
medications specifically for hypermobility-related sleep problems are often
helpful.
As mentioned earlier, one possible explanation for the frequent arousals
and lack of deep sleep is that patients are making too much adrenaline at night,
just as they often are during the day. Some patients unfortunately seem to make
too little during the day, waking tired and dragging through the day, only to get a
“second wind” of energy (or a “first wind” for many!) at 9:00 or 10:00 at night, just
as they are trying to wind down and get ready for bed. Heart rate monitors
showing increased fluctuations in heart rate and occasional sudden increases in
heart rate corresponding to arousals and awakenings lend support to this theory,
as does the observation that medication to block or offset extra adrenaline helps
many patients get a better night sleep. Adrenaline-blocking medications include
various types of beta blockers, while medications like Valium and Ativan work
partly by raising the levels of calming chemicals in the brain to offset the extra
stimulating ones. Also, since chronic pain is so common in this patient group,
appropriate pain medication at bedtime is often essential to achieving a restful
night’s sleep.
Hypermobility and the Genitourinary System
In women with hypermobility, the ligaments supporting the uterus can
be weak, leading to an increased risk of uterine prolapse, a condition where the
uterus “slips” and presses on the bladder. Weakness and excessive stretchiness
of other tissues in the pelvis increase the risk of cystocele and rectocele,
conditions in which the bladder and rectum, respectively, push on the walls of the
vagina. Excessive stretchiness of the vagina itself and weakness of the muscles
that support the pelvis can make intercourse painful for some women with
hypermobility. Vulvodynia or vaginismus, a painful spasm of the vagina, also
may occur more commonly in the setting of hypermobility. Weakness and laxity
of the muscles of the pelvic floor also contribute to the onset of incontinence at
an early age in some women with hypermobility.
Interstitial cystitis, which causes frequent and often painful urination,
and often more diffuse pelvic pain as well, appears to occur with increased
frequency in patients with hypermobility. A bladder that stretches too much may
not empty completely, or may signal the brain that the bladder is full when it isn’t.
As with digestion, autonomic fluctuations can cause difficulty or frequency of
urination. In addition, hypermobility may be associated with an increased risk of
endometriosis (tissue that normally lines the uterus grows somewhere else, such
as on the ovaries, behind the uterus, or on the bowels or bladder).
Pregnancy and delivery usually are not exceptionally difficult for
hypermobile women. In fact, because circulating blood volume expands
during pregnancy, many women find that their circulatory symptoms, such
as lightheadedness and cold hands and feet, and for some even fatigue,
are much
better during pregnancy. On the other hand, some symptoms, such as
heartburn, varicose veins, and hemorrhoids, can be worse during pregnancy.
There may be an increased incidence of premature rupture of membranes and
rapid (i.e. less than 4 hours) labor and delivery, and women with severe
hypermobility are at risk for unusual complications.
Summary
Joint hypermobility is a very common condition, and many people with
loose joints will have no related medical problems at all. Most people have just a
few, such as achy joints and cold hands and feet, and they should not be overly
concerned that they will go on to develop other complications. On the other
hand, I have described some of the more common conditions associated with
hypermobility, because those who do have many related problems may be
relieved to understand that there is a unifying explanation for so many of the
unusual things they have noticed about their bodies. Beyond the psychological
relief of understanding their symptoms, with proper treatment most people with
JHS can get significant relief from their physical symptoms, too.
My patients have long asked for something to share with their families,
friends, and even physicians, to help educate others about their condition. I hope
this paper helps fill this need.
Copyright ©2010 Alan G. Pocinki, MD. All rights reserved
