Hypermobility Spectrum Disorders

The term “hypermobility spectrum disorder” refers to a condition in which a person experiences symptoms due to hypermobile joints but does not exhibit all of the characteristics of Ehlers-Danlos syndrome. These issues mainly affect youngsters and early adolescents who have extra-flexible (hypermobile) joints and experience discomfort during exercising that lasts even after they stop exercising.

The disease can have a significant influence on one’s ability to perform well in school and sports. However, while it is not typically connected with long-term damage, it can be related to injuries and can sometimes last into adulthood in rare cases. The goal of treatment is to increase muscular strength and joint resilience without causing damage to the patient.

Are there any differences between joint hypermobility and Ehlers-Danlos syndrome?

We now refer to hypermobility spectrum disorders (HSDs) rather than joint hypermobility syndrome because everyone has a unique experience with the condition. Some people are simply incredibly adaptable – they experience no discomfort and experience no other challenges in their lives. In contrast, Ehlers-Danlos syndrome, hypermobility type, is a condition that affects the joints (EDS-HT). Additionally, joint hypermobility with persistent pain is caused by this, in addition to the many other more generalized symptoms that it usually produces.

The HSDs are located in the middle of these two extremes. Many people suffer from serious difficulties such as pain, joint dislocations, or repeated joint injuries. The symptoms of the two illnesses are quite similar, and their symptoms are incredibly similar. On the other hand, EDS is often at the more severe end of the spectrum and manifests itself in a wider variety of symptoms.

  • Joint hypermobility without pain occurs when youngsters have elastic or flexible joints but do not experience exercise-induced discomfort. This is a distinct advantage for particular youngsters, and it is frequently correlated with athletic prowess in general.
  • HSDs are a diagnosis that is made when the primary or sole symptoms are exercise-related discomfort and joint hypermobility.
  • EDS is typically considered the correct diagnosis when a family history of comparable symptoms or problems such as hernias and dislocations arise.

Is joint hypermobility a distinct condition from growing-pain syndrome?

Growing pains symptoms share some similarities with those of HSDs. However, growing pains are more sporadic, are not often associated with activity, and generally afflict children under the age of 12 years. However, growing pains are more prevalent in physically active youngsters and in children who have hypermobile joints.

An ache or throbbing in the front of the thighs or the back of the legs is the most common sensation associated with growing pains. They usually affect both legs and occur at night, although not always. They are more common in women.

Mild cases of HSD may appear to be nothing more than growing pains; however, if your child’s pain is clearly tied to activity and is preventing them from participating, it is more probable that HSD is the underlying diagnosis.

Is it possible to get a diagnosis of hypermobility spectrum disorders?

Joint hypermobility is characterized by joints that are more flexible than they should be (this is referred to as being ‘double-jointed’), which is associated with joint and muscular discomfort that is frequently worse by activity. This condition is most commonly observed in children and young people; however, it can occasionally continue into maturity. Some children with ‘double-jointedness’ have painful muscles, whereas others do not. It is currently unknown why some children have painful muscles while others do not.

What is the root cause of joint hypermobility?

Some believe that persons with hypermobile joints have stretchier elastic tissues in their bodies, notably in their muscles and ligaments than people who do not have hypermobility joints. Persons differ significantly in their stretchiness,’ and hypermobility is most likely just the stretchiest subset of a large but typical group of people with this trait.

Nobody knows why certain children and adults with hypermobile joints experience unpleasant symptoms, while the majority do not, although these children and adults may have the most elastic tissues of all. A variety of other characteristics, such as more relaxed muscles at rest than the norm, may also have a role. In addition, there may be inherent variances in the way bone joints are structured, resulting in some people’s joints having wider ranges of motion than those of others.

In what ways do people who suffer from hypermobility spectrum disorders show signs and symptoms of their illness?

The most common symptoms of HSDs are joint hypermobility, which is accompanied by muscle and joint discomfort after activity and fatigue. From children who can ‘exercise through it’ reasonably easily to children who find that it has a significant influence on their capacity to be active and who suffer considerable discomfort, there is a great deal of variety in the intensity and impact.

Compromised fitness

Sleep, energy, focus, and overall activity are all affected by a lack of fitness. As muscle tone deteriorates, posture deteriorates, and headaches and back discomfort become increasingly prevalent. As the core muscles become less supportive, the ability to maintain balance is compromised. As a result, athletes’ abilities in sports such as hockey and football would deteriorate.

Weight gain, bad mood, a sense of alienation and distinction, and a loss of self-esteem are all possible consequences of a lack of fitness. This may become a vicious cycle when irritation develops in while attempting to increase physical activity, making the situation worse.

Tiredness

Children who are less fit get wearier, and their muscles tire more readily due to physical exercise as they grow older. The result is that they become less and less active, which creates a vicious cycle. Muscles that have not been utilized become restless and fidgety, resulting in an uncomfortable sensation of leg fidgetiness that can be added to the feelings of soreness and fatigue. Handwriting may become unpleasant, and writing may become more challenging to shape correctly and more challenging to read.

Pain

Pain is present mainly in the knees, feet, and ankles, and it occurs most frequently after physical exercise and during nighttime. The pain appears to be more severe in younger children. Following the increased activity, muscles and joints are often stiff and uncomfortable for a few days, and youngsters may become discouraged from exercising. Back discomfort and headaches are also frequent, primarily due to poor posture, which worsens as muscles grow frailer.

Pain is typically described as a scorching or throbbing sensation that causes muscles to become restless and fidgety.

Pain can cause sleep and emotional disturbances, and it can linger for up to 48 hours. Taking pain relievers, mainly if they induce side effects such as nausea or loss of appetite, might exacerbate the situation. Children who are afraid of increasing discomfort may altogether forego physical activity.

Other signs and symptoms

Children with HSDs are more prone to bruising than other children, even though this is completely harmless and the cause is unclear. They also have a tendency to develop ‘clicky’ or snappy joints, which they may develop the habit of clicking on and off frequently (this can sometimes lead to dislocation).

Tummy (abdominal) discomfort and constipation are frequent. They are most likely caused by a lack of abdominal muscle tone, which may be obtained by regular exercise and which aids in the proper function of the intestine.

It is rare for children with HSDs to develop hernias or other outward symptoms of muscular weakness; however, this (as well as dislocation) is more common in children with EDS than in children with HSDs.

Fainting, or even simply feeling faint, is an unusual sign of this condition. This is assumed to be related to relatively slow blood pressure reflexes,’ which means that when the blood pressure has to rise fast, such as when standing up quickly, the blood pressure tends to increase quite slowly as well. A contributing factor may be that weaker leg muscles do not pump the circulation nearly as well compared to stronger leg muscles.

What is the prevalence of joint hypermobility?

Joint hypermobility and hereditary susceptibility disorders (HSDs) are extremely frequent in children of school age. Everyone may recall students at school who could perform ‘the splits’ with ease and others who were unable to do so despite their best efforts. Young adolescent girls are more likely than young teenage boys to have hyper-flexible joints, with up to 4 in 10 being female and 1 in 10 being male. The majority of these youngsters, on the other hand, do not experience any discomfort.

Approximately one in every ten children with hypermobile joints will suffer discomfort during physical activity, which is referred to as a hypermobility spectrum condition. Most people will recover with help and graded exercise, but a minority will find it more difficult to recover. Some children experience pain, although the majority do not, for reasons that are now unknown.

HSDs are more prevalent in Asian-American children than in African-American children, with Asian-American children having the highest prevalence. The causes behind this are most likely genetic in nature. In addition to being hypermobile, many persons who have Down’s syndrome are also clumsy.

What is the procedure for diagnosing hypermobility spectrum disorder?

HSDs are diagnosed based on the existence of a specific collection of symptoms, referred to as ‘criteria.’ These are a mix of the Beighton hypermobility score and the symptoms experienced by the kid or young person.

There are nine points in the Beighton hypermobility score, and it is calculated using tiny fingers, thumbs, elbows, knees, and the trunk. Hypermobility is determined by performing a regular sequence of motions with the thumb and wrist, the fifth finger, the elbows, the lower back, and the knees.

If  I suspect my child has a hypermobility spectrum disorder, where can I go for help?

HSDs are becoming more widely recognized among medical experts, yet the disorder is still readily overlooked. Children who have it appear healthy, and inspection of them is routine.

The majority of general practitioners will not have access to children’s physiotherapy through the NHS. Appointments in secondary care can be challenging to come by, and they are sometimes only available to children who match specified requirements.

This may account for part of the reason why HSDs are frequently underappreciated. Several different health care experts commonly visit patients before a diagnosis is made.

If your child is hypermobile and appears to be experiencing symptoms, read information such as this leaflet and make an appointment with your doctor, explaining what you believe is going on and that you would like a referral to physiotherapy or occupational therapy or to a pediatrician who has a particular interest in joint disorders. Consider speaking with your child’s school as well, as many physical education instructors will be well-versed in this illness and may be able to provide information on the most beneficial local services.

You can also contact the Hypermobility Syndromes Association, which is situated in the US and UK.

What criteria are used to evaluate hypermobility spectrum disorders?

Children who are suspected of having an HSD are often visited by a specialist who will assess the kid and speak with them about their activities, which may include hobbies, physical activity, and sleeping patterns. They will also discuss the impact their symptoms are having on their performance at school and home. A specialist referral or blood tests may be required to ensure that your kid does not have another ailment such as early rheumatoid arthritis, and these may be arranged.

The clinician will use the diagnostic criteria to assess HSD diagnosis and rule out the possibility of an EDS diagnosis. He or she will test your child’s muscular strength by tugging and pressing on various muscles, notably those in the legs and arms, to determine his or her overall strength. Your youngster may be asked to walk or run.

There are no additional tests performed; the diagnosis is made solely based on these findings. A blood test or X-rays may be required if your doctor still wants to rule out other disorders such as arthritis, in which case more testing may be required.

What are the consequences of having a hypermobility spectrum disorder (HSD)?

HSDs are a concern for both children and adults. This is not just because of its symptoms but also because of the potential consequences that may arise.

Having flexible joints (hypermobility) is often considered an advantage in sports and dancing and may be observed in many affected individuals. Because of the flexibility of their joints and the stretchiness of their muscles, these individuals are more mobile and agile than the average person.

Increased flexibility and the capacity to stretch, on the other hand, can not only cause discomfort, loss of fitness, fatigue, and low self-esteem, but it can also result in damage. It can also have a more widespread impact on students’ academic achievement.

Injuries

  • High-strength drugs (HSDs) not only cause pain when there is no injury, but they also make people more susceptible to harm. Tendon injuries and dislocations are more likely to develop around joints that are not as well supported as they should be.
  • This implies that, while encouraging children with exercise-related discomfort to exercise while they are in pain is frequently a part of the answer, doing so runs the danger of their harming themselves since they are being taught that they should not stop when it hurts, which is the polar opposite of what we would typically do. It is, therefore, necessary to provide careful assistance and management to strike a balance between growing muscular strength and risking damage.
  • Recurrent dislocations can cause persistent discomfort in the joints and increase the likelihood of developing wear-and-tear type arthritis (osteoarthritis) later in life.
  • Spondylolisthesis is a condition in which the backbones move slightly out of alignment, resulting in back discomfort. It can occur very rarely in people who have hyper-flexible backs.

The academic performance of the students

  • The leg muscles are the most typically affected by the muscular signs of HSDs. Arms, back, and shoulders, on the other hand, can be impacted as well. Handwriting can become an issue since it causes pain in hand and wrist when done for an extended period of time. Some students may benefit from the assistance of a scribe during exams.
  • Children may also experience overall fidgetiness and fatigue, and their ability to concentrate in class may deteriorate quickly, resulting in poor academic achievement.
  • It may also impact a child’s capacity to participate in team activities as well as the skills that children gain through sports, such as leadership, teamwork, and perseverance. The results of a child’s education may be negatively affected as a result, and crucial learning opportunities may be missed. Schools must be aware of this and create other ways for students with severe HSD, who cannot manage team sports well, to participate in and lead other team activities.

What other diagnoses are comparable to hypermobility spectrum disorders?

If your kid is experiencing joint and muscle problems, it is essential to rule out the following conditions:

  • Ankylosing spondylitis
  • Juvenile idiopathic arthritis
  • Rheumatoid arthritis
  • Marfan’s syndrome
  • Fibromyalgia

Is it possible to recover from hypermobility spectrum disorder?

The majority of instances will react to gradual increases in physical activity and assistance, and for the vast majority of children, there will be no long-term physical problems.

For a tiny minority of children, symptoms are more severe, necessitating more comprehensive intervention. Some people will experience HSD throughout their lives, even into maturity. Some of these youngsters will exhibit additional symptoms and may indeed be suffering from the hypermobility kind of EDS in the first place.

What is the treatment for hypermobility spectrum disorders?

The initial goal of therapy is to increase the strength of the muscles that must support the hypermobile joints during movement. Most children’s posture, strength, and fitness will improve as a result of this. Because exercise hurts and increased activity hurts, even more, it might be difficult for young patients to endure this. Since children with the illness are at greater risk of hurting their joints and muscles, this may be challenging since it requires exercising while experiencing some discomfort and knowing when to stop exercising.

Education and moral support are required for youngsters to believe in the therapy and endure to achieve a booming increase in fitness. If an accident occurs, it will be a significant setback to the child’s confidence in exercise as a means of controlling their condition moving forward. This necessitates the implementation of a progressive supervised exercise regimen. Physiotherapy can be of assistance in this situation. A visit to a podiatrist to determine whether or not you require shoe inserts to adjust your foot posture might also be beneficial. The objective is to fully participate in all activities while also recovering strength and reducing or eliminating symptoms.

Other activities you and your kid should engage in at home and when your child is physically active are also recommended.

If a diagnosis of HSD is made, many NHS practitioners, including physiotherapists, podiatrists, and counselors, can assist. The amount of therapy offered through the NHS will vary depending on the severity of the ailment and the availability of resources in your region. However, your child’s school’s physical education department, as well as particular sports clubs for some youngsters, may be a precious source of support and assistance.

·        Physiotherapy

1.     Physiotherapy employs specific exercises to target the muscles that are in the most need of assistance. It also assists in encouraging your youngster to incorporate regular physical activity into their daily routine.

2.     Children with HSDs will first experience a great deal of discomfort and exhaustion the day following physiotherapy. It might also be difficult for them to precisely repeat the exercises when they are alone at home. It is also critical for someone to ensure that they are not over-exercising and endangering their health and wellbeing.

3.     Parents may play a crucial part in monitoring and checking the exercises, and your child’s physical education teacher may be able to assist.

·        Occupational therapy

This sort of treatment is intended to assist patients with their regular routines. In some instances, an occupational therapist (OT) will look at the kind of chair your child uses, the mattress they sleep on, or a pen that makes writing more comfortable. An occupational therapy evaluation in the school setting might be beneficial.

·        Podiatry

A podiatrist is a medical person who is trained in the diagnosis and treatment of foot diseases. A podiatrist may be able to provide corrective insoles to help rectify the posture of the feet if necessary.

·        Taking care of one’s fatigue

Tiredness is prevalent among young individuals who have a learning disability. They must realize that being weary does not necessarily imply that they require extra sleep. In reality, weariness can sometimes indicate that the body needs more physical activity. The most effective therapy for daytime fatigue is to increase one’s level of activity over time gradually.

However, young people must understand that exercising can result in discomfort and exhaustion; they must learn to listen to their bodies and pace themselves accordingly. They also need to be realistic; even though they will experience relapses from time to time, they mustn’t cease all activities for extended periods of time.

·        Medications for the treatment of pain

Pain has been shown to impair focus, memory, mood, and sleep. To reassure your kid that the pain is not hazardous, it is necessary to determine whether or not there is an injury. It is also essential to decide on how to assist your child in managing the pain.

It is critical to check for injuries before proceeding, especially if your child’s suffering appears to be becoming worse. An acute injury is frequently noticeable due to increased heat and edema, as well as a fast exacerbation of discomfort. The pain associated with HSDs is often symmetrical (i.e., it occurs on both sides of the body) and ‘the same as normal.’ Injury is indicated by much severe pain, the pain only in one location, the discomfort accompanied by heat, swelling, or bruising, or muscles and joints that your kid cannot bear to move.

·        Self-management with assistance

The goal of therapy, whether provided by a physiotherapist, a doctor, a physical education teacher, or a sports coach, is to assist youngsters in treating their own disease. The majority of children with minor symptoms will recover with the help of their parents and teachers, and they will not require any more professional assistance.

However, in extreme circumstances or in situations when exercise and support are not effective, it is critical to return to your doctor and request more assistance.

The goal of assisted self-management is to help young people understand the need for regular exercise and work through discomfort over some time. It also assists them in distinguishing between the amount of discomfort that may be worked through and the level of discomfort that may result in harm if not addressed.

The essential thing to remember is that muscular soreness experienced after exercise is not always a symptom of damage and does not always imply that exercise is hazardous. Simultaneously, acknowledging that this does not rule out the possibility of injury as a result of over-exercising. The key is to build as many muscle fibers as possible while exercising with caution.

·        Injury prevention and management

Following an accident, it is necessary to treat the joints and muscles with greater caution – but this does not imply that there should be no movement at all. It is possible to exercise in a method that causes minimal discomfort while keeping muscles and joints in good shape and functioning correctly. Children must learn to overcome their fear of movement by beginning with tiny movements as soon as possible.

•        Controlling one’s weight

1.     Your child must maintain a healthy weight at all times. When you are underweight, your muscles will become weaker, and when you are overweight, your joints will be under more pressure. Both extremes will tend to exacerbate symptoms.

2.     A nutritious and well-balanced diet and regular exercise are the most effective ways to maintain fitness and weight management, even if the activity must be mild and careful at the beginning.

·        Choose sport carefully

1.     Some sports are more demanding on the joints than others and hence may need to be avoided for longer than others.

2.     Children with HSDs may be exceptionally talented in these sports. It is critical to encourage them to participate in activities they like while also recognizing a source of discomfort. As long as your kid is not in danger of getting hurt, they should be allowed to continue participating in the sport they have chosen as long as they are able to tolerate the amount of pain and discomfort that it creates.

•        Support for those suffering from joint hypermobility syndrome

The Hypermobility Syndromes Association (see details in Further Reading, below) is situated in the United Kingdom and provides assistance and information to families, patients, and educational institutions.

•        Surgery

In most cases, HSD surgery is not indicated; however, it may be necessary to repair a tendon that has been ripped due to an injury.

•        Complementary and alternative medicine

When it comes to muscular pain in HSDs, there is no evidence to support the use of alternative therapy.

•        Specially designed equipment

Wheelchairs and crutches are extremely inconvenient in HSDs, and they will only serve to exacerbate the condition. Anything that causes your youngster to feel or behave as though he or she is a physically constrained being is likely to be counterproductive.

What is the long-term prognosis for people who suffer from hypermobility spectrum disorders?

Most children’s symptoms improve when their muscle size and strength rise due to growing and maturation. Joint looseness is reduced as a result of this muscle hypertrophy.

Only a tiny percentage of children who are afflicted continue to have issues throughout adulthood. Children with EDS, hypermobility type, and other severe disabilities are more likely to be identified in this group (EDS-HT). These are often the youngsters whose joint laxity makes it simple to sustain an injury, making exercise extremely difficult for them. It is also difficult for them to discriminate between the pain they are advised to exercise and the discomfort that indicates they should stop.

The presence of an HSD, unless at the most severe end of the spectrum, is rarely associated with long-term issues such as arthritis. Joints that have been regularly dislocated, on the other hand, might develop issues. Children and adolescents who continue to experience HSD symptoms when they reach adulthood are slightly more likely to develop osteoarthritis in the joint(s) in question.

What can schools do to assist in this situation?

Schools should encourage students with disabilities to participate in sports while also considering the child’s lower stamina. Backache, fidgeting, and fatigue can all be alleviated with a supportive setting.

Children who struggle with their handwriting may benefit from using a keyboard, the employment of scribes, or the provision of more time during exams and examinations. Extra time may be required to transition between sessions, and the school should make every effort to avoid asking pupils to carry their whole library about with them all day.

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