Hypermobility syndrome.
Its impact on the young patient.
Pam Baxter MSc
CPD evening 14th January 2013.
Edinburgh Branch SOCAP.
Welcome!
• Format of presentation:
• Ok Podiatrists have a good understanding of
this phenomenon. But lets revisit!
• Tonight I want to focus on the Child’s
experience.
• Why? Because they are often overlooked and
therefore help comes late!
•
Style
•
•
•
•
CPD event.
Reflective practice
Anecdotal
Case history
• Later we take a look at symptoms, briefly
management and where to locate more
information.
Content.
•
•
•
•
•
Information is multisource.
MSc and focus groups
Patients
Journals, text books.
Web sites and media.
Hypermobility syndrome.
• For many children joint
hypermobility does not
cause problems.
• However this condition
can result in a very
miserable child.
Significantly affecting
their daily life.
• If a young patient
complains about sore
legs, please don't assume
they're just being lazy.
The following is a description of a patient
who is struggling to get a diagnosis.
• This is a classic case!!!!!
• A nine-year-old girl, complained that the sole
of her left foot was sore, and a large lump had
formed in the medial arch.
• Visiting the GP- these symptoms were put
down to the ill-fitting shoes (she lived in Ugg
boots all the time)!
The GP’s advice….
• GP said the footwear was unsupportive, and
the boots had appeared to have irritated the
arch of her foot, that is why it was inflamed.
•
• The advice was: Get rid of the boots and it will
all settle.
• The Uggs were duly binned, and parents and
patient waited for the swelling to go down.
However…..no change!
• Instead, the lump got bigger until it became so
painful the girl couldn't walk.
• A trip to casualty resulted in crutches, but no
answers.
• The girl was examined by several doctors and
the foot was X-rayed.
• Nobody could work out what this lump was.
• And the parents were urged to get a referral
to an orthopaedic surgeon.
Several weeks later….
• The girl was still in
constant pain.
• No diagnosis.
• No resolution of
symptoms.
• Mum “Her passion was
dancing, so to see her
on crutches was
heartbreaking”.
What happened at orthopaedics?
• Mum talking about the
consultant“Well, he wiggled her
feet around, bent her
knees and manipulated
her arms before taking
hold of one of her
hands”.
And…
• Mum continues “Watch
this,” he said, and promptly
bent her thumb so far back
that it touched her wrist.
• “ I was horrified”……..
•
“I then realised my
daughter hadn't so much as
flinched. Nor did she when
he bent her whole hand
back until it was almost
flat”.
A diagnosis then?
• “She's double-jointed” the consultant said.
• “Your daughter has hypermobility syndrome,”
he explained.
• “In other words, she's super bendy”.
Hypermobility syndrome.
• Hypermobility syndrome (HMS)?
• Many HCPs describe the patient as doublejointed.
• Why are they double jointed? The collagen in
the ligaments in their joints is far more flexible
than it should be.
• Hypermobility goes beyond the normal flexibility
enjoyed by the young.
• The joints have double or more the range of
movement than they should.
What advice was given?
• Mum queries..
• “Well, being hypermobile had weakened my
daughters ankles, making her turn in on her
feet as she walked. And a recent growth spurt
made this worse” ?
What caused the pain then?
• Mum “Well, eventually a scan showed she had
a stress fracture in a long bone in her foot.”
• Mum “I was told lots of people are doublejointed and to varying degrees; but
hypermobility syndrome can create all sorts of
problems”.
MSc research?
• I will come back to mum and daughter later.
• Qualitative research…..focus groups. Looking
at different health care professionals (HCP)
attitude to patients with overly flexible joints.
• Generally poor! Podiatrists one the best!
Specialists in the field are growing in
number.
• Prof Rodney Grahame is still one of those
consistent voices talking about HMS.
• A consultant rheumatologist at University
College Hospital, London and the
Hypermobility Clinic at Great Ormond Street
• Although, he still believes the medical
community still hasn't got to grips with
hypermobility.
Mis-information
• For example…
• Prof Grahame: “Many doctors reassure
patients that HMS disappears before
adulthood.” That’s not the case.
• It may decrease in intensity, but adults with
HMS still suffer with pain and are at increased
risk of dislocations, stress fractures, frozen
shoulder and carpal tunnel syndrome.
The HMS patient.
• This person is prone to
all manner of soft-tissue
injuries.
• Such as severe bruising
around the joints.
• Which is why children,
and adults should avoid
showing off their bendy
joints!
Children with HMS
• Unfortunately kids who complain are often
dismissed, ignored, or just fail to get noticed.
•
• In the UK approx. 15 -30 per cent of children
have the condition, which can affect just one
or two joints or every joint in their body.
• Clinical scoring systems - 4 joints or more, pain
for 3 months plus.
Symptoms (child)
• Stamina can be affected.
• Enthusiasm can be diminished about joining in ... “I don’t
do p.e.” “Things are just sore all over, tired……….” “My feet
wobble” “ I can’t keep up”
• Muscle weakness
so prone to injury.
• Muscle pain (muscle spasm)
• Movement pain. (Writing …..sport)
• Joint pain very common.
• Many children suffer from dislocations.
• Soft-tissue injuries. Stress fractures.
Growing pains then?
• The symptoms are often put down to growing
pains or simply having a moany child.
• Prof Grahame – “A child's muscles aren't
strong enough to cope with the fact that their
connective tissues are so flexible. They have to
work incredibly hard just to put one foot in
front of the other and stay upright”.
And so….
• He says that by the evening the child is often
exhausted.
• It's like when someone first goes to the gym they may lie in bed aching after that activity.
•
• A child with HMS will feel like that most
nights.
Back to our…. Mum
• “As a toddler my daughter would cry for her
pushchair after walking the shortest of distances.
When she got older she would sob during family
walks, saying her legs hurt. My husband and I put
it down to laziness and told her she was being a
wimp.”
• “She complained of sore arms and legs at
bedtime, she said it was stopping her from
getting to sleep, I thought she was playing up and
told her to stop making a fuss.”
Clumsy?
• Mum “My daughter is also very clumsy - she's
forever knocking things over”.
• Professor Grahame states that children with HMS
are clumsy because their sense of where things
are around them is impaired.
• Co-ordination is poor and they appear gangly.
This is because they don't have enough control
over their limbs and often fall as a result.
I asked earlier what should a podiatrist
be aware of?
• What’s normal for a child?
• I am often asked, how do you know if your
young patient is hypermobile? And not just
very flexible because of their young age?
• Get a detailed patient history! Get the clues.
• Explore the day to day experiences. What
appears normal for your young patient……
pGALS
•
•
•
•
•
Paediatric
Gait
Arms
Legs
Spine
• Tells you what is normal
Clinical Scoring?
• Beightons vs
Brighton Criteria
vs Bulbena
Criteria????????
• For ease stick with
Beightons.
What's normal rom?
Some facts to remember.
• The average age of when children start to complain is
about 6 years.
• But the age at diagnosis is most likely to be 9 years—(a
2- to 3-year delay in diagnosis).
• Major presenting complaints:
• History of joint dislocation.
• Arthralgia (74%). ( More than one joint).
• Abnormal gait.
• Apparent joint deformity (10%).
• Back pain (6%).
• Information from Medscape Dr Adib (Manchester Uni).
Also.
• Rheumatologists consider that HMS is usually
hereditary and can appear as a symptom of
other serious conditions, such as EhlersDanlos syndrome, Osteogenesis Imperfecta,
and Marfan syndrome.
• Here an early diagnosis and treatment are
advised.
• So ask parents when taking that history!
Management 1 Prevention of injury!
• HMS is normally managed through exercise
and physiotherapy, to treat physical problems
involving the body’s systems such as the
musculoskeletal, cardio vascular and
respiratory.
• Core stability-pilates.
Management 2
• Medication
• PRICE
• Surgery ?
• Many HCP involved: OTs
• Podiatrists - attempt to normalise gait, making
it more efficient, less energy being expended.
Orthoses usually very successful.
Patient education.
• ARC no longer exists.
• Arthritis Research UK has taken its place.
• HMSA “Hypermobility Syndrome Association”.
• Has a section dedicated to the younger
patient /teenager.
CPD on HMS
• Short course for health practitioners.
• Where:
University of Hertfordshire
• What:
“Hypermobility Symposium”
• When:
March 2013
Thanks for listening.
The end.
Thanks!