Allied Conditions and rehabilitation

Transcription

1 Allied Conditions and rehabilitation Dr Helen Cohen Consultant Rheumatology & Chronic Pain, Royal National Orthopaedic Hospital Cmglee, Radially-symmetrical five-set Venn diagram devised by Branko Grünbaum, illustration, viewed 12 th April 2015, <

2 Allied conditions Castori M. ISRN Dermatol. 2012;2012: Ehlers-danlos syndrome, hypermobility type: an underdiagnosed hereditary connective tissue disorder with mucocutaneous, articular, and systemic manifestations.

3 Allied conditions Association does NOT equal causation eg. look at colour of front door of 500,000 people could probably find an association with depression, favourite breakfast cereal, pets etc!! Castori M. ISRN Dermatol. 2012;2012: Ehlers-danlos syndrome, hypermobility type: an underdiagnosed hereditary connective tissue disorder with mucocutaneous, articular, and systemic manifestations.

4 Chronic pain conditions overlap Fibromyalgia Chronic fatigue syndrome/me Irritable Bowel Syndrome Chronic Pelvic Pain Interstitial cystitis Temperomandibular joint dysfunction Complex Regional Pain Syndrome (CRPS) Dystonia - overlapping pain mechanisms?

5 Common Associations with EDS / HMS Fibromyalgia Chronic Fatigue Syndrome Autonomic dysfunction / Postural Orthostatic Tachycardia Syndrome (POTS) Depression/anxiety/other psychology Gastrointestinal dysfunction Urogynaecological dysfunction Headache Arnold Chiari type 1 malformation (?) Mast cell dysfunction (?) Illustration, viewed 12tth April 2015, <

6 Fibromyalgia Very common overlap Generalised ache/flu-like Myofascial trigger points Worse with over/under activity Worse with weather Worse with stress Poor sleep Illustration, viewed 12 th April 2015, <

7 Fibromyalgia Do you want another label? Treatment is similar *Emphasis on improving physical fitness & activity Pain killers take edge off; do not make it go Medications have only limited role / balancing side effects against benefits Role of pain sensitisation/amplification Illustration, viewed 12 th April 2015, <

8 Chronic fatigue/me Another very common overlap Exclude other problems eg. thyroid, anaemia Multiple factors contribute to fatigue Some CAN be modified but need lifestyle & behavioural change Importance of correcting disrupted sleep wake cycle Day time naps/bed days Sleep hygeine Pacing; avoid boom-bust Illustration, viewed 12 th April 2015, <

9 Castori M, Morlino S, Celletti C, Celli M, Morrone A, Colombi M, Camerota F, Grammatico P. Management of pain and fatigue in the joint hypermobility syndrome (a.k.a. Ehlers-Danlos syndrome, hypermobility type): principles and proposal for a multidisciplinary approach.am J Med Genet A Aug;158A(8): *

10 Autonomic Dysfunction Also seen in fibromyalgia, CRPS A mild variant probably very common in EDS Low BP, sudden head-up postural shift Worse in physical deconditioning / vicious cycle Different patterns/type including POTs Illustration, viewed 12 th April 2015, <

11 Dizziness Palpitations Faints Venous pooling POTs Often linked to postural changes Panic attacks/anxiety/stress may have similar symptoms and contribute Full assessment includes autonomic testing including tilt table, carbohydrate meal etc Photograph, viewed 12 th April 2015, <

12 ANS dysfunction & rehabilitation Adequate fluid and salt intake Calf pump exercises before getting up Avoid prolonged sitting positions and prolonged recumbency Avoid sudden head-up postural change Avoid nicotine and alcohol intake *Improve physical fitness & activity If despite usual measures fainting is uncontrolled, needs to be assessed & treated Illustration, viewed 12 th April 2015, <

13 Depression, anxiety & other psychology HMS is associated with a variety of psychological conditions Anxiety and depression Also attention deficit (and hyperactivity) disorder (ADHD), autism spectrum disorders, obsessivecompulsive disorder (OCD), personality disorder We have also noticed: eating disorders, self harm, body image disorders, trans gender, PTSD Chronic pain is associated with depression & anxiety Adverse childhood experiences associated with later development of chronic pain syndromes Sinibaldi L, Ursini G, Castori M. Psychopathological manifestations of joint hypermobility and joint hypermobility syndrome/ Ehlers-Danlos syndrome, hypermobility type: The link between connective tissue and psychological distress revised. Am J Med Genet C Semin Med Genet Mar;169(1):

14 Depression, anxiety & other psychology Relevance to rehab: Motivation, insight, cognition Ability to cope in groups Ability to cope with other people Coping with new routine, different environment Taking in information Poorly controlled psychological problems will massively impact on ability to undertake rehab This is NOT weakness; identify it & deal with it! Illustration, viewed 12 th April 2015, <

15 Broad spectrum Top to bottom! GI dysfunction Swallowing, reflux, constipation, prolapse, rectal evacuatory dysfunction, delayed emptying etc Association with anorexia/bulimia/body image BMI from low to very high Diet from normal to markedly restricted Photograph, viewed 12 th April 2015, <

16 GI dysfunction & rehab Need to be consuming enough calories to support exercise Uncontrolled anorexia, untreated severe malabsorbtion, low BMI will prevent effective rehab *Gut motility improves with physical activity Poor diet & eating habits may need correcting Problem of morbid obesity & EDS Illustration, viewed 12 th April 2015, <

17 Urogynaecological problems Broad spectrum Bladder instability, voiding difficulties Prolapse rectal, vaginal Menstrual problems especially pain Hormonal fluctuations do affect pain and joint stability eg. pregnancy Rehab considerations If severe problems & pain are preventing exercise, may need advice & treatment If severe pain related to menstrual cycle, may need appropriate advice Prolapse and exercise consideration Pelvic floor advice Illustration, viewed 12 th April 2015, <

18 Headache Many potential causes and contributing factors Migraine Cervical spine disorders Temporomandibular joint disorders Medication overuse headache ANS dysfunction/coat-hanger headaches Carotid arterial dissections Intracranial hypotension, Arnold Chiari malformations-type 1 Illustration, viewed 12 th April 2015, <

19 Castori M, Morlino S, Ghibellini G, Celletti C, Camerota F, Grammatico P. Connective tissue, Ehlers-Danlos syndrome(s), and head and cervical pain. Am J Med Genet C Semin Med Genet Mar;169(1): Martin VT, Neilson D. Joint hypermobility and headache: the glue that binds the two together--part 2. Headache Sep;54(8):

20 Headache & Rehab * Castori M, Morlino S, Ghibellini G, Celletti C, Camerota F, Grammatico P. Connective tissue, Ehlers-Danlos syndrome(s), and head and cervical pain. Am J Med Genet C Semin Med Genet Mar;169(1):84-96.

21 Arnold Chiari Evidence for association is poor 4 papers (2 by same author) Can be no symptoms/never cause a problem Even if present, may NOT be the major factor causing symptoms Even if present, surgery may NOT be appropriate ; evidence very poor; could be worse off Evidence for need of positional MRI also very poor In summary: a rare cause of headache in EDS Maintain an index of suspicion ; look for & treat other common causes * Chiari malformation & EDS ; Alan Hakim et al. Chiari 1 malformation ; Ions & Milhorathttp:// chiari-1-malformation

22 Headache: conclusion Lots of causes If recurrent, severe and preventing exercise and activities of daily living, may need further investigation and advice Illustration, viewed 12 th April 2015, <

23 Mast cell dysfucntion Poor evidence Many common overlapping symptoms Should not be diagnosed in absence of positive blood results Treated with various medications Low histamine diet no evidence Rehab considerations Avoidance of triggers Illustration, viewed 12 th April 2015, <

24 Allied conditions, EDS & rehab: summary There are several recognised conditions associated with EDS Other associations remain unclear eg. mast cell dysfunction, Chiari A mild degree of some of these conditions is common and may not need any other treatment Beware of collecting labels Insurance For many of these conditions, exercise & improving physical conditioning is the main advice Illustration, viewed 12 th April 2015, <

25 Illustration, viewed 12 th April 2015, <