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EDNF Learning Conference July 22-23, 2011. Chronic Fatigue in EDS. Peter C. Rowe, MD Sunshine Natural Wellbeing Foundation Professor of Chronic Fatigue and Related Disorders Department of Pediatrics Johns Hopkins University School of Medicine. Chronic Fatigue in EDS.

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chronic fatigue in eds

EDNF Learning Conference

July 22-23, 2011

Chronic Fatigue in EDS

Peter C. Rowe, MD

Sunshine Natural Wellbeing Foundation Professor of Chronic Fatigue and Related Disorders

Department of Pediatrics Johns Hopkins University School of Medicine

chronic fatigue in eds1
Chronic Fatigue in EDS
  • Chronic fatigue and CFS definitions
  • Lessons from CFS
  • CF and CFS in EDS
  • Insights of treating chronic fatigue
    • Treating orthostatic intolerance
    • Non IgE-mediated food protein allergies
    • The paradox of movement restrictions in EDS
    • Ovarian vein varices/pelvic congestion
fatigue
Fatigue

An overwhelming sustained sense of exhaustion and decreased capacity for physical and mental work.

Piper BF. 1989

fatigue definitions
Fatigue Definitions
  • Prolonged fatigue: fatigue lasting 1 – 6 mo.
  • Chronic fatigue: fatigue lasting > 6 mo.
  • Chronic fatigue syndrome: new onset fatigue, lasting > 6 mo., unrelieved by rest and 4/8 somatic symptoms

From MJA 2002; 176:S17-S55

symptom criteria for cfs 4 of 8 needed for diagnosis fukuda et al ann int med 1994 121 953 9
Symptom Criteria For CFS4 of 8 needed for diagnosisFukuda et al. Ann Int Med 1994;121:953-9
  • unrefreshing sleep
  • postexertional malaise lasting > 24 hours
  • self reported impairment in short-term memory or concentration
  • sore throat
  • tender cervical or axillary glands
  • muscle pain
  • multijoint pain without swelling
  • headaches of a new type, pattern, severity
cfs clinical evaluation fukuda et al ann int med 1994 121 953 9
CFS Clinical EvaluationFukuda et al. Ann Int Med 1994;121:953-9
  • History, physical, mental status exam
  • Screening labs:
    • CBC, ESR/CRP, Chemistries, TSH
    • Urinalysis
    • Iron studies, vitamin B12, celiac screening, and, in endemic areas, labs for Lyme and tick-borne infections
  • Other labs as clinically indicated
chronic fatigue in eds2
Chronic Fatigue in EDS
  • Chronic fatigue and CFS definitions
  • Lessons from CFS
  • CF and CFS in EDS
  • Insights of treating chronic fatigue
    • Treating orthostatic intolerance
    • Non IgE-mediated food protein allergies
    • The paradox of movement restrictions in EDS
    • Ovarian vein varices/pelvic congestion
cfs epidemiology
CFS Epidemiology

General Affects previously active individuals

Heterogeneous precipitating & perpetuating factors

Shift in perception of CFS:

No longer considered a single disease

More likely a convergence of co- morbid pathophysiologic influences

cfs epidemiology1
CFS Epidemiology

Prevalence 4/1,000 adults; 1/1,000 adolescents

Age Uncommon under 10 years

Peak prevalence 40-49 years

Gender 2-4 F : 1 M

SES Affects all groups

GeneticsTwice as common in MZ as DZ twins

Associated with EDS

Associated with joint hypermobility

research findings
Research Findings
  • Acute illness appears to precipitate symptoms in up to 2/3, but evidence of active infection not detected in chronic state

(enteroviral infection, Lyme may be exceptions)

  • Severity of acute infection, not psychological factors, is key determinant of who develops CFS after acute illness
  • XMRV not an etiologic agent
  • Immune abnormalities inconsistent & mild
  • Post-exercise increases in cytokines and genes involved with adrenergic function and pain
slide11

Light AR et al.

J Pain 2009;10:1099

research findings1
Research Findings
  • Orthostatic stress and exercise consistently provoke CFS symptoms
  • All pediatric and most adult studies confirm higher prevalence of orthostatic intolerance
  • Open treatment of OI leads to improvement in function
  • CBT and graded exercise provide modest improvement in function but not cure
  • Low rates of spontaneous improvement for those with > 3 yrs of symptoms
orthostatic intolerance
Orthostatic Intolerance

The term “orthostatic intolerance” refers to a group of clinical conditions in which symptoms worsen with quiet upright posture and are ameliorated (although not necessarily abolished) by recumbency.

Modified from: Low PA, Sandroni P, Joyner M, Shen WK. Postural tachycardia syndrome (POTS). J Cardiovasc Electrophysiol 2009;20:352-8.

slide15

Rowell LB

Human Cardiovascular Control, 1993

symptoms of orthostatic intolerance
Lightheadedness Dyspnea

Syncope Chest Discomfort

Diminished concentration Palpitations

Headache Tremulousness

Blurred vision Anxiety

Fatigue Nausea

Exercise intolerance Nocturia

Symptoms Of Orthostatic Intolerance
slide17

↑pooling,

↓vasoconstriction

↓ intra-vascular volume

Orthostatic stress

↑ sympatho-adrenal

response

NE/Epi

NE/Epi

NMH

POTS

response to upright tilt cfs
Response To Upright Tilt: CFS

Abnormal Normal

Stage of tilt

1 2 3

CFS 16 3 3 1

CONTROL 0 1 3 10

OR for abnormal tilt in those with CFS: 55 (95% CI, 5.4 - 557)

Bou-Holaigah, Rowe, Kan, Calkins. JAMA 1995;274:961-7.

cfs and psychiatry
CFS And Psychiatry
  • Many CFS patients have anxiety or depression, but prevalence estimates vary widely depending on the case definition used
  • Severity usually mild, anhedonia uncommon
  • Post-exertional malaise more common in CFS
  • Treating depression and anxiety can improve these symptoms, but usually does not cure CFS
slide21

12 wks

24 wks

52 wks

White PD et al. PACE trial. Lancet 2011

chronic fatigue in eds3
Chronic Fatigue in EDS
  • Chronic fatigue and CFS definitions
  • Lessons from CFS
  • CF and CFS in EDS
  • Insights of treating chronic fatigue
    • Treating orthostatic intolerance
    • Non IgE-mediated food protein allergies
    • The paradox of movement restrictions in EDS
    • Ovarian vein varices/pelvic congestion
slide23

Classical type EDS:

“Fatigue is a frequent complaint.”

Beighton P, De Paepe A, Steinmann B, Tsipouras P, Wenstrup R. Ehlers Danlos Syndromes: Revised nosology, Villefranche, 1997

slide24

Orthostatic

Intolerance

CFS

EDS/

Joint hypermobility

orthostatic intolerance and chronic fatigue syndrome associated with eds
Orthostatic Intolerance And Chronic Fatigue Syndrome Associated With EDS

Among approximately 100 adolescents seen in the CFS clinic at JHH over a 1 year period, we identified 12 subjects with EDS

6 classical-type, 6 hypermobile-type EDS

11 female; median age 15 yrs (9-21)

NMH in 9/12, POTS in 10/12

Rowe PC, Barron DF, Calkins H, Maumanee IH, Tong PY, Geraghty MT. J Pediatr 1999;135:494-9

joint hypermobility in children with cfs
Joint Hypermobility In Children With CFS

Study question: do children with CFS have a higher prevalence of joint hypermobility?

Beighton scores obtained in 58 new & 58 established CFS patients, and in 58 controls

Median Beighton scores higher in CFS (4 vs. 1)

Beighton score > 4 higher in CFS (60% vs. 24%)

Barron DF, Cohen BA, Geraghty MT, Violand R, Rowe PC. J Pediatr 2002;141:421-5

beighton joint hypermobility scores in 58 adolescents with cfs and 58 healthy controls
Beighton Joint Hypermobility Scores in 58Adolescents With CFS And 58 Healthy Controls

#

Barron, Geraghty, Cohen, Violand, Rowe. J Pediatr 2002;141:421-5

Beighton scores

how might joint hypermobility be associated with oi and cfs
How might joint hypermobility be associated with OI and CFS?

Working hypothesis:

Connective tissue laxity in blood vessels allows increased vascular compliance, promotes excessive pooling during upright posture, leading to diminished blood return to the heart, and thus to OI symptoms

Rowe PC, et al. J Pediatr 1999;135:494-9

slide29
Fatigue is a frequent and clinically relevant problem in EDS (Voermans NC, et al. Semin Arth Rheum 2010; 40:267-74)
  • 273 patients with EDS
  • 77% severe fatigue
  • 57% reported fatigue as 1 of their 3 most important symptoms
  • Severe fatigue was more common in hypermobile than classical EDS (84% vs. 69%; P=.032)
  • Fatigue had a greater impact on daily function than did pain
slide30
Fatigue is a frequent and clinically relevant problem in EDS (Voermans NC, et al. Semin Arth Rheum 2010; 40:267-74)

On the basis of their results, the authors speculate about a potential treatment:

“A cognitive behavioral intervention focusing on pain, sleep disturbances, the reaction of others to the symptoms, and self-efficacy concerning fatigue could help reduce fatigue and fatigue-related disabilities.”

chronic fatigue in eds4
Chronic Fatigue in EDS
  • Chronic fatigue and CFS definitions
  • Lessons from CFS
  • CF and CFS in EDS
  • Insights of treating chronic fatigue
    • Treating orthostatic intolerance
    • Non IgE-mediated food protein allergies
    • The paradox of movement restrictions in EDS
    • Ovarian vein varices/pelvic congestion
slide32

Infection

Movement restrictions

Inhalant allergies/asthma

Migraines

Food allergies

Chiari type I or c-spine stenosis

Orthostatic intolerance

Anxiety

EDS/JHS

Depression

Pelvic vein incompetence

Chronic fatigue syndrome

treating chronic fatigue
Treating chronic fatigue
  • Careful history and physical exam, supplemented by questionnaires, to develop working hypotheses about the dominant influences on fatigue
  • Begin working on graded increases in activity, physical therapy if needed
  • Begin treating the dominant influences on symptoms
  • Reassess and repeat steps 1-3
16 year old with fatigue visit 1
16 year old with fatigue: visit 1

Gastroesophageal reflux and colic in 1st year of life

Onset of fatigue and daily lightheadedness at age 13

Develops syncope X 3; Migraines

GI: early satiety, reflux, abdo pain, aphthous ulcers

O/E: Beighton score 7/9, blue sclerae, easy eyelid eversion, pes planus, papyraceous scar of L knee.

Limitations on physical therapy ROM despite joint hypermobility

Beck Depression Inventory: dysthymia

visit 1 hypothesis formulation
Visit 1 hypothesis formulation

Imp: EDS

OI (already on Florinef)

Milk protein intolerance

Migraines

Movement restrictions

Dsythymia

Plan: Milk-free diet instituted

Low dose cyproheptadine

slide36

Infection

Movement restrictions

Inhalant allergies/asthma

Migraines

Food allergies

Chiari type I or c-spine stenosis

Orthostatic intolerance

Anxiety

EDS/JHS

Depression

Pelvic vein incompetence

Chronic fatigue syndrome

16 year old with fatigue
16 year old with fatigue

Visit 2: GI symptoms resolved unless he gets inadvertent milk re-exposure; mood more of the problem

Plan: Low dose Lexapro for mood

Visit 3: Mood improved, but still has some orthostatic exacerbation of migraines; still tight on PT exam

Plan: Add midodrine for OI; begin PT

16 year old with fatigue1
16 year old with fatigue

Visit 4: Migraine resolved; better ROM

Trial off Lexapro: mood & HA worse, but able to drop to 2.5 mg daily

“The more I do, the more I can do”

Plan: Continue PT

Visit 5: Good year, on HS soccer and tennis teams No syncope; migraines only if he does not maintain good hydration

Plan: No changes.

non ige mediated food allergy 3 cardinal features
Non-IgE mediated food allergy :3 cardinal features
  • Recurrent vomiting or GER
  • Recurrent epigastric or abdominal pain
  • Food refusal, picky eating, early satiety

Other: aphthous ulcers, unexplained

fevers, diarrhea or constipation, headache,

myalgias, fatigue, asthma

Kelly KJ et al. Gastroenterology 1995;109:1503-12

non ige mediated food allergy
Non-IgE mediated food allergy
  • Reaction to suspected food usually delayed 2-6 hrs
  • IgE level, prick skin tests, RAST tests often neg.
  • Eosinophilic esophagitis only the tip of the iceberg
  • Treated with strict avoidance of offending food proteins (milk > soy > egg > wheat); amino acid formulas occasionally needed in infants
  • Diagnosis supported by clinical response to diet, recurrence of symptoms 2-6 hours after inadvertent dietary challenge, confirmed by DBPCOFC
slide41
Improvements in esophageal eosinophils after amino acid formula dietKelly KJ et al. Gastroenterology 1995;109:1503-12
chronic fatigue in eds5
Chronic Fatigue in EDS
  • Chronic fatigue and CFS definitions
  • Lessons from CFS
  • CF and CFS in EDS
  • Insights of treating chronic fatigue
    • Treating orthostatic intolerance
    • Non IgE-mediated food protein allergies
    • The paradox of movement restrictions in EDS
    • Ovarian vein varices/pelvic congestion
paradox of movement restrictions in eds
Paradox of movement restrictions in EDS
  • Increased prevalence of postural abnormalities and movement restrictions among those with CFS
  • CFS symptoms can be reproduced by selectively placing tension on the neural tissues
  • Focal movement restrictions are common even in those with generalized joint hypermobility/EDS
  • Improvement in ROM, orthostatic tolerance, and exercise tolerance can follow manual therapy
how might movement restrictions be associated with cfs
How Might Movement Restrictions Be Associated With CFS?
  • Pathophysiology of symptoms with neural elongation strain awaits clarification, but we hypothesize that it contributes to central sensitivity
  • Informally, improvement in symptoms, ROM, orthostatic tolerance, and exercise tolerance appears to follow manual therapy designed to reduce adverse neural tension and improve movement restrictions
manual therapy principles
Manual Therapy Principles
  • Use of the hands to restore full, symptom-free mobility within the neuromuscular and articular systems
  • Goal of treatment is the same as that of exercise-based PT, but manual practitioners treat movement restrictions first before advancing the patient to strenuous activity
manual techniques
Manual Techniques
  • Slow non-thrust manipulations
    • Sustained stretching
    • Passive oscillatory movements (neural mobs)
    • Muscle energy techniques
  • Gentle indirect techniques
    • Myofascial release
    • Strain and counter-strain
    • Cranio-sacral therapy
chronic fatigue in eds6
Chronic Fatigue in EDS
  • Chronic fatigue and CFS definitions
  • Lessons from CFS
  • CF and CFS in EDS
  • Insights of treating chronic fatigue
    • Treating orthostatic intolerance
    • Non IgE-mediated food protein allergies
    • The paradox of movement restrictions in EDS
    • Ovarian vein varices/pelvic congestion
slide51
16 yr old with EDS, CFS, OI, and 2 yr history of disabling lower back and pelvic pain
  • Pain worse as the day goes on
  • Pelvic pain present with urination, when back pain present, with menses
  • Unable to tolerate sitting in school
  • Lower abdominal distention as the day goes on
  • X-rays, scans, MRI of lumbar spine negative
  • Unresponsive to OCPs, NSAIDs, TENS unit, neurontin, TCA, lumbar support garments, PT, inpatient evaluation
left ovarian vein venogram
Left ovarian vein venogram

Catheter in distal L ovarian vein plexus; arrows denote reflux of contrast into internal iliac veins

pelvic congestion syndrome venbrux ac lambert dl curr opin ob gyn 1999 11 395
Pelvic Congestion SyndromeVenbrux AC, Lambert DL. Curr Opin Ob Gyn 1999; 11:395
  • Pelvic heaviness or pain with long periods of standing
  • Worse at end of the day, during menses
  • Associated symptoms: fatigue, dyspareunia, bladder urgency
  • Strong association with varicose ovarian veins
  • 89% have > 80% relief after embolization of ovarian vein varicosities
cfs and ovarian varices jhh experience
CFS and ovarian varices: JHH experience
  • 24 consecutive females with chronic pelvic pain unresponsive to NSAIDs, OCPs, & no other cause identified on Hx, PE, imaging
  • median age 19, range 16-54
  • 16 were < 21 yrs; all but 4 nulliparous
  • Median duration of pelvic pain 4 yrs (1-15)
  • All had orthostatic intolerance
  • 14/24 with EDS

Kaushik S, et al. JHH 2003

slide56
16 yr old with EDS, CFS, OI, and 2 yr history of disabling lower back and pelvic pain

Outcome

  • Improved symptoms following ovarian and internal iliac embolization
  • Able to attend school daily
  • Able to wean midodrine for OI
  • No further syncope
  • Wellness score > 90/100
slide57

Opportunities for Research

  • What are the risk factors for fatigue in JHS/EDS?
  • What is the prevalence of OI in EDS patients?
  • What is the prevalence of CFS or fibromyalgia symptoms in JHS/EDS?
  • 4. Do therapies directed at OI & related co-morbidities in JHS and EDS improve QOL?
slide59
Treatment of orthostatic intolerance

Webinar from September 2010 available on the CFIDS Association of America web site:

www.cfids.org

slide60

Relationship of orthostatic intolerance to chronic fatigue

Common

Chronic Fatigue

Uncommon

Low

High

Tolerance of orthostatic stress

slide61

Can we move fatigue levels from A to B by treating orthostatic intolerance?

Common

A

Chronic Fatigue

B

Uncommon

Low

High

Tolerance of orthostatic stress

step 1 non pharmacologic measures
Step 1: Non-pharmacologic measures

Where possible, avoid factors that precipitate symptoms

precipitating factors for oi
Precipitating Factors For OI
  • Increased pooling/decreased volume

Prolonged sitting or standing

Warm environment

Sodium depletion

Prolonged bed rest

Varicose veins

High carbohydrate meals

Diuretics, vasodilators, alpha-blockers

Alcohol

precipitating factors for oi1
Precipitating Factors For OI
  • Increased catecholamines

Stress

Exercise

Pain

Hypoglycemia

Albuterol

Epinephrine

step 1 non pharmacologic measures1
Step 1: Non-pharmacologic measures

Compression garments

  • Support hose

(waist high > thigh high > knee high)

  • Body shaper garments
  • Abdominal binders
step 1 non pharmacologic measures2
Step 1: Non-pharmacologic measures

Use postural counter-measures

  • standing with legs crossed
  • squatting
  • knee-chest sitting
  • leaning forward sitting
  • elevate knees when sitting (foot rest)
  • clench fists when standing up

[Use the muscles as a pump]

step 1 non pharmacologic measure s
Step 1: Non-pharmacologic measures

Fluids: Minimally 2 L per day

Drink at least every 2 hours

Need access to fluids at school

Avoid sleeping > 12 hrs/day

Salt: Increase according to taste

Supplement with salt tablets

step 1 non pharmacologic measures3
Step 1: Non-pharmacologic measures

Exercise

Avoid excessive bed rest/sleeping

For most impaired, start exercise slowly, increase gradually

Recumbent exercise may help at outset

Manual forms of PT may be a bridge to better tolerance of exercise

“Inactivity is the enemy”

[Similar to principles of CBT regarding graded increases in activity]

treatment of orthostatic intolerance
Treatment Of Orthostatic Intolerance
  • Step 1: non pharmacologic measures
  • Step 2: treating contributory conditions
  • Step 3: medications
    • Monotherapy
    • Rational polytherapy
slide72

Infection

Movement restrictions

Inhalant allergies/asthma

Migraines

Food allergies

Chiari type I or c-spine stenosis

Orthostatic intolerance

Anxiety

EDS/JHS

Depression

Menstrual pain; ovarian varices

Chronic fatigue syndrome

treatment of orthostatic intolerance1
Treatment Of Orthostatic Intolerance
  • Step 1: non pharmacologic measures
  • Step 2: treating contributory conditions
  • Step 3: medications
    • Monotherapy
    • Rational polytherapy
therapy for orthostatic intolerance
Therapy For Orthostatic Intolerance
  •  blood volume

Sodium (PO & occasionally IV),

fludrocortisone, clonidine, OCPs

  •  catecholamine release or effect

-blockers, disopyramide, SSRIs, ACE inh.

  • Vasoconstriction

Midodrine, dexedrine, methylphenidate, SSRIs, SNRIs, aescin (horse chestnut seed extract)

  • Misc

pyridostigmine bromide

slide75

↑pooling,

↓ vasoconstriction

↓ intra-vascular volume

Vasoconstrictors

Volume expanders

↑ sympatho-adrenal

response

Reduce catecholamine release/effect

Orthostatic stress

↓ NE/Epi

↑ NE/Epi

NMH

POTS

how to select initial therapy
How to select initial therapy?

Algorithm vs. individualized approaches

slide77

Algorithm approach for POTS from Mayo Clinic investigators

Johnson JN, et al. Pediatr Neurology 2010; 42:77-85

individualized approach
Individualized approach
  • SBP < 110: fludrocortisone, midodrine
  • Increased HR at baseline or when upright:-blocker
  • Based on other clinical clues

Increased salt appetite: fludrocortisone

HA: -blocker

Dysmenorrhea/worse fatigue with menses: OCP, Depo

Anxiety/low mood: SSRI, SNRI

Myalgias prominent: SNRI

FH of ADHD: stimulant

Hypermobility: stimulant, midodrine

Modified from Bloomfield, Am J Cardiol 1999;84:33Q-39Q

management of orthostatic intolerance
Management of orthostatic intolerance
  • requires careful attention by the patient and the practitioner to the factors that provoke symptoms
  • requires a willingness to try several medications before a good fit is achieved
  • requires a realization that meds often can treat symptoms but do not necessarily cure OI
  • management of OI is one part of a comprehensive program of care for patients with other disorders (GI dysautonomia, CFS)