What is hypermobility?
A rare and heritable connective tissue disorder affecting approximately 1/5000 people. Hypermobility can be acquired or secondary through training where the joints are stretched beyond its limit. When the connective tissue, which makes up the joint structures are easily stretched beyond its normal range, then it would be considered a hypermobile joint. Many children who are hypermobile do not experience any difficulties but for few, it may cause problems.
Risk factors
Some factors that play a role in hypermobility are:
- Genetics
- Gender: females are more than males
- Age: children and sometimes young adults in their 30s and 40s
- Ethnic Background: African or Asian descent in comparison to Caucasians
- Training/Exercise: yoga or gymnasts or ballet dancers
- Co-morbidities: osteogensis imperfect (fragile bones that can fracture easily), Marfan syndrome (rare disorder that also affects the connective tissue) and Ehlers-Danlos Syndrome (rare inherited collagen disorder).
Types of Hypermobility Syndromes
Joint hypermobility is most common form of Ehlers-Danlos syndrome and present with a wide range of joint hypermobility with or without other symptoms. There are several terms used interchangeably and these are as follows:
- Hypermobility EDS
- Benign Joint Hypermobility Syndrome (BJHS)
- Hypermobility Syndrome (HMS)
- Joint Hypermobility Syndrome (JHS)
What causes hypermobility?
JHS is likely inherited where specific genes responsible for predisposing the child to developing hypermobile joints encode a protein called collagen. Collagen is essential to keep the tissues together like glue. If the collagen is weak, it would cause the tissues to become delicate making the ligaments and tendons loose and stretchy. Connective tissue, which consists of proteins like elastin (gives stretchiness) and collagen (provides strength), make up the ligaments that hold joints together. The connective tissue should hold the joints together quite firmly and limit joint movement however, in a hypermobile joint, the ligaments allow for more stretch to occur than usual during movements. In addition, muscles also become more malleable causing less tone and stiffness. This results in the muscles working harder to generate and produce the movement and stability.
So to break it down, you can say the 2 factors involved in hypermobile joints are:
- Weak or stretched ligaments: imbalance of collagen to elastin in the ligaments result in weakness plus increased elasticity
- Muscle tone (stiffness): more relaxed a muscle, more movement and lax the joints
What to look for?
Signs of movement limitation may be observed due to increased laxity of joints and poor muscle strength and endurance. Some signs noted early are hypermobile knees where the knee moves backward to a point where the knee locks into position and allows for the quadriceps muscles (muscles in the front of the thigh) to work less to maintain joint stability. The knee at this point is protected by staying in a locked position, however, when the knee is slightly bent then the quadriceps are not functioning to its ability due to lack of strength causing instability. Other joints of the body that are likely to be affected are spine, shoulders, elbows, ankles, hips and ankles.
Signs and Symptoms:
- Joint and muscle pain*
- Easily bruised*
- Fatigue/tiredness*
- Muscle weakness*
- Exercise intolerance*
- Poor balance and coordination
- Joint dislocation/subluxations
- Clicking joint
- Stomach/digestive problems
- Headaches
- Nausea
- Heart/eye problems
- Allergic reactions
- Insomnia
* These signs and symptoms are commonly reported by children but there can be other symptoms or co-existing conditions
Recognizing hypermobility
Few characteristics are:
- Standing posture: slightly popping out the stomach and knees hyperextended
- Sitting posture: slumped or sitting propped up on one knee
- Difficulty standing up straight or leaning onto something
- May walk on the toes
- May have difficulty walking with a normal heel strike due to tight muscles
Diagnosis
Generally, your GP makes the diagnosis of generalized joint hypermobility or JHS through two ways involving a series of questions.
The Beighton score:
It is a quick test to measure/assess joint range of movements and uses a 9-point system where the higher you score, the more lax your joints are.
The Beighton criteria:
This helps to determine whether your child has JHS. This criterion examines how many joints are hypermobile and how many of those joints have had pain. If you have 4 or more hypermobile joints and have had pain in those joints for 3 months or more then it is likely your child has JHS.
How does this affect your child in School?
Pain – main problem
Your child may experience muscle or joint pain throughout the day and it can vary in the area affected and the intensity. It is good to be aware that your child may find it difficult or uncomfortable to sit/stand in certain positions or may get complaints from school that your child is distracted and fidgety. Teachers may notice the child’s handwriting is poor or that they have problems with writing fluently. This may be due to difficulty with fine motor manipulation; a consequence to having a painful hand/wrist joint resulting in writing much slower and easily tired with repetitive writing tasks. These difficulties may be linked with general coordination problems and poor grip strength.
Fatigue
This is commonly experienced and is due to a period of high energy followed by energy loss immediately. Be aware the energy levels fluctuate from day to day and require the child to learn to pace activities and would find it challenging to do homework or after-school activities as a result. The section on treatment provides you with some information on ways to reduce fatigue but it is always a good idea to consult with a qualified health professional.
Absence
Your child may miss school due to illness or medical appointments and result in the absence of participating in school activities or even falling behind class and feeling excluded. Medical appointments/illness may be due to having dysautonomia, which is the autonomic nervous system not functioning properly. One of the most common forms of dysautonomia is Postural Tachycardia Syndrome (PoTS) where the heart rate varies leading to light-headedness or fainting.
Management
Muscles are an important component as its main function is to move and/or stabilize a joint. Muscles help control the range of movement and force production and this is very important to note in a hypermobile child since their muscles tend to be weak and are unable to function properly. The main treatment here would be to improve muscle strength.
PHYSIOTHERAPY
Main purpose of physiotherapy is to treat musculoskeletal features of JHS. The aims of physiotherapy are to:
— Improve spinal posture through core stability
— Improve joint proprioception
— Enhance joint stability through exercise
— Increase muscle strength
— Gait re-education (walk)/correction of abnormalities
— Avoid end-of-range postures during rest
— Advice on self-management to restore self-esteem and self-efficacy
— Prevent deconditioning by improving fitness and stamina
Some Rules for Exercise:
Avoid
1. High-impact exercises
2. Stretching that involves grabbing, pushing or pulling on a joint
3. Heavy lifting, pushing and pulling
4. Hyper-extending the joints
Do
1. Light resistance exercise for strengthening and stabilization
2. Be consistent with exercise
WHAT CAN I DO TO HELP MY CHILD?
Treatment success is dependent on collaboration between family and physiotherapy as regular therapy is important to ensure the child is maintaining the exercise and managing the condition. For long-term management, providing a home exercise program would be beneficial and to ensure joints are well protected through muscle strengthening to prevent secondary complications.
Support and enable your child to grow up strong and healthy through regular physical activity, which benefits not only children with JHS but children in general. It is important for your child to take part in activities that allow for strengthening muscles, development of balance, body awareness and confidence with movement. Some activities that may be enjoyable yet at the same time be helpful in developing physical skills are swimming, bike riding, scooter, ball games, dance, rock climbing and martial arts.
This page is intended to serve parents and caregivers to help understand what joint hypermobility syndrome is, recognize common signs and symptoms and forms of management/treatment.
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