Recommendations for the treatment of the Joint Hypermobility Syndrome (JHS)

(Written in a language for patients)

 

It is necessary that the patients with JHS know that this is a common condition (15% worldwide and 40% in Chile and probably in other Latin countries) and is usually not diagnosed. In spite that it can cause acute and chronic pain that can be disabling, it does not have the possible serious complications of the other types of Hereditary Diseases of the Connective Tissues (HDCT) such as the vascular type of Ehlers-Danlos Syndrome (VEDS), formerly called EDS type IV; the Marfan Syndrome (MFS); or the Osteogenesis Imperfecta (OI). Women are more hypermobile than men and children more than adults, since laxity decreases with age.

 

The problem lies in that the joints are too lax, which makes them unstable and painful, due to the extreme mobility. In general, there is more problem with hyperextension than hyper-flexion of the joints. Due to this, these patients are prone to recurrent traumatic lesions such as sprains and subluxations, which are painful and at times require immobilization.

 

It is necessary to avoid extension of the joints, especially lax joints, for example when putting weight on the hand it is better to put the weight on a full fist rather than on the palm of the hand. When standing it is necessary to avoid the knees from going backwards (genu recurvatum), for this, the patient needs to stand with mild flexion of the knees and to use a strong 3 cm shoe heel.  We recommend children not to show others how lax they are (not to do “party tricks”), since every time that they extend too much the joints they are damaging them, which in the long run will result in osteoarthritis (joint wear and tear). If possible, it is better not “to crack the joints” on purpose.

 

In order to prevent ankle sprains it is necessary not to wear high heels. Falls should be prevented, since fractures can happen, because these patients frequently have osteoporosis (22%) and more importantly it is to realize that in JHS patients younger than 30 years old, the frequency is 19% in both sexes, as we have found in our recent study of 1,000 JHS patients.

 

We recommend these patients to avoid been overweight to prevent osteoarthritis of the knees and hips.

 

It is necessary to do exercises carefully to strengthen the tendons, ligaments and muscles. Most JHS patients awake with muscular rigidity and for them stretching and local heat are useful. They should not stretch too much, since they usually get leg cramps. Stretching before sports or exercises is even more necessary in these patients, than for people without hypermobility.

 

Violent sports, such as: karate, box, rugby, skating, etc. should be avoided. Also contact sports such as volleyball and basketball are harmful to patients with lax fingers, because they develop frequent sprains and tendinitis. In general swimming, cycling, Yoga, Tai Chi, and Pilates are recommended. Each person needs to determine which sport gives them problems as to avoid them, and some may not be able to practice soccer, tennis or other sports.  This also depends which joint is affected, if the hands or wrists, the person will not be able to be a goalkeeper or to play volleyball. If someone has knee or ankle problems he/she may be unable to play some sports. If a patient wants to play a sport anyways, he should protect the weak joints, using elastic bandages, splints, etc., and to consider quitting the sport if problems are recurrent.

 

Due to mark agility, some adolescents begin Ballet classes or Gymnastics, but a warning is necessary, since some may not be able to continue and may have to quit because of articular problems. They need to start slowly and evaluate the situation and if they start having musculo-skeletal problems, they need to see a physician or retire from that activity.

 

Elastic bandages and splints with metallic reinforcement are useful. In offices or industry, it is important to evaluate working conditions, to prevent problems such as neck or back pain, carpal tunnel syndrome, sprains, tendinitis, bursitis, subluxations, etc.

 

In cases of capillary fragility and ecchymoses, Duo CVP and Vitamin C are beneficial, besides avoiding contusions. The use of Glucosamine alone or with Condroitin Sulphate, in optimal doses and taken for a prolonged period of time, are useful to prevent progression of Osteoarthritis, that can appear early in these patients. It is necessary to look for Osteoporosis that can appear even in young men and to treat it as early as possible.

 

Chronic Fatigue, which at times is associated with dizziness or even syncope, causes poor quality of life and is frequently misdiagnosed as Fibromyalgia or Depression, with which it is usually confused. Every day we see patients that have seen many physicians, had many tests, and have been having a very poor quality of life for years and that the proper diagnosis has not been made. Dysautonomia is very frequent in JHS, as we have seen in our recent study, where we found that in patients younger than 30 years old, it affects 64% of the women and 40% of the men. The good news, though, is that it can be treated effectively.

 

A good measure is to take short periods of rest and not to stay for hours in the same job, without changing body positions. It is necessary to advise music students about which instrument to prefer. School children should not carry heavy back packs, because this is a frequent cause of back pain. It is advisable to have school accident insurance, since JHS children are prone to recurrent musculo-skeletal injuries, such as sprains, subluxations, tendinitis, etc.

 

Treatment of the acute phase.  The immobilization of the affected joint is of paramount importance. The use of splints or casts is at time indicated. Short periods of immobilizations are better than very prolonged ones. Hot or cold packs are beneficial. At the house a pack of frozen peas, covered by a towel, can be used effectively. Hot compresses or hot baths reduce pain and muscle contraction. Physical therapy is recommended, including massage, ultrasound, ultratermia, etc. It is necessary to avoid the repetitive activity that caused the acute problem. Medications such as Tylenol or anti-inflammatory, for short periods, are useful. Muscle relaxants reduce pain, but if the pain is intense, then Tylenol with Codeine or Tramadol is suggested. Topic analgesics or anti-inflammatory creams can be used. If the problem persists, it will be necessary to see a Rheumatologist or an Orthopedist, to study the cause of the problem. Occasionally an infiltration with steroids and lydocaine is needed to treat a tendinitis or a bursitis. This procedure is very efficacious and painless, since a local anesthetic is applied.

 

I recommend to see in my Web Site the following articles:

 

The Joint Hypermobility Syndrome (JHS).

Dysautonomia  (Chronic Fatigue, dizziness and syncope).

When to suspect  JHS.

Importance of JHS diagnosis  (in Spanish).

Pain in JHS  (in Spanish).

Osteoporosis in JHS  (in Spanish).

 

www.reumatologia-dr-bravo.cl

 

An easy way to find information, that I have written in my Web Site, is to use the “Search”, that is found at the beginning of the Web Site.

 

Jaime F. Bravo, MD

Rheumatology-Osteoporosis

Revised:  March 30, 2007