The Complexities of Chronic Illness: Chronic Fatigue Syndrome

1
The Complexities of Chronic Illness Chronic
Fatigue Syndrome
CAPHC 2004 Annual Meeting 9 November 2004

• Peter C. Rowe, MD

2
Chronic Fatigue c. 1992

• Our culture witnesses a kind of collective
  hypervigilance about the body and a readiness
  of a large number of people to cling tenaciously
  to a given diagnosis CFS, refusing to abandon
  their belief despite medical reassurance to the
  contrary.
• Shorter E. In From Paralysis to Fatigue A
  History of Psychosomatic
• Illness in the Modern Era. 1992.

3

• Complexities of chronic illness
• CFS clinical features
• Insights into
• orthostatic intolerance
• joint hypermobility
• abnormalities on PT examination
• 4. Lessons

4

• the clinician who cares for a child with a
  chronic disorder is surrounded by uncertainty.
• I. Barry Pless, MD
• Ped Clin N Amer 1984

5
Complexities of Chronic Illness

• Illness often affects physical development and
  appearance
• Periodic and unpredictable crises
• High absenteeism and scholastic challenges
• Discordance between expectations of peers and
  limitations of illness
• Development of an identity distinct from the
  illness
• Behavioural/adjustment disturbances common
• Uncertainties for parents and physicians about
  optimal treatment approach

6
Accentuation of uncertainty with CFS

• No single cause
• No definitive diagnostic test of fatigue
• No single treatment cures CFS
• Responses to symptomatic Rx variable

7

• one of the worst things for patients is
  apprehension about going to a hospital or ER
  because of concerns that the doctor is going to
  be unsympathetic and know less about the disease
  than they do. GS.
• Lancet 19993531504

8

• Complexities of chronic illness
• CFS clinical features
• Insights into
• orthostatic intolerance
• joint hypermobility
• abnormalities on PT examination
• 4. Lessons

9
16 Year Old With Fatigue

• Healthy and active until 9 mo. before visit
• Insidious onset of fatigue
• Sleeps 12-14 hrs per night, awakens unrefreshed
  has to lie down the day after an active day
• Difficulty concentrating
• Muscles sore, headaches, dizzy
• Has difficulty getting going in the AM, has to
  lie down after showering
• Unable to attend school

10
Fatigue in CFS

• Self-reported persistent or relapsing fatigue
  lasting 6 or more consecutive months, which
• Is of new or definite onset (not lifelong)
• Is not the result of ongoing exertion
• Is not substantially alleviated by rest
• Results in substantial reduction in previous
  levels of occupational, educational, social, or
  personal activities
• Fukuda et al. Ann Int Med 1994121953-9.

11
Symptom Criteria For CFS4 of 8 needed for
diagnosis

• unrefreshing sleep
• postexertional malaise lasting gt 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

12
(No Transcript)
13
16 Year Old With Fatigue

• On exam Acrocyanosis. BP 117/81
• Standing test HR 80 bpm to 121 bpm in 10 min
• Tilt test Symptoms fatigue, warmth,
• LH, nausea, diaphoresis
• Presyncope at 17 minutes
• BP 78/48 HR 70
• Diagnosis POTS and NMH
• Treatment Increased salt and fluid intake
  Fludrocortisone, potassium

14
Early Follow-up

• Improvement in all symptoms within 2 wks
• Began working 2 jobs, feeding livestock at family
  farm, able to spend time with friends
• Full school attendance
• Fatigue only after 45 minutes of swimming
• Repeat standing test
• HR increase 76 to 86 after 10 minutes

15
(No Transcript)
16
Late Follow-up

• Mild fatigue when allergies active
• Return of impressive fatigue with attempts to
  wean Florinef, despite good level of exercise and
  physical conditioning
• Off meds wellness 50-70/100
• On meds wellness 85-90/100

17
CFS Clinical Features

• Affects previously active individuals of all SES
  groups females gt males (1.81)
• Can follow infectious illness but no single
  pathogen identified in the majority of cases
• No unique immunology allergy common
• No pathognomonic exam or lab findings
• Systematic reviews confirm that symptomatic
  improvement, but hardly cure, can result from CBT
  and graded exercise

18
Chronic Conditions in Adolescence

• Prevalence/1000
• Asthma 58.1
• Congenital heart disease 7.0
• Diabetes 1.8
• CFS 1.0
• Cystic fibrosis 0.2

19
CFS and Psychiatry

• Internalizing scores higher ( partly by
  definition)
• Severity of depression usually mild, anhedonia
  uncommon
• Treating depression and anxiety can improve
  function, but usually does not cure CFS

20
Questionnaire scores for anxiety (Spielberger
State-Trait Anxiety Inventory )
Smith MS et al, Pediatrics 2003111e376-e381
21
School days missed in past 6 months
Smith MS et al, Pediatrics 2003111e376-e381
22

• Complexities of chronic illness
• CFS clinical features
• Insights into
• orthostatic intolerance
• joint hypermobility
• abnormalities on PT examination
• 4. Lessons

23
Relationship of orthostatic intolerance to
chronic fatigue
Common
Chronic Fatigue
Uncommon
Low
High
Tolerance of orthostatic stress
24
Common Symptoms Of Orthostatic Intolerance

• Lightheadedness
• Fatigue
• Exercise intolerance
• (especially low impact exercises)
• Diminished concentration
• Nausea
• Headache

25
(No Transcript)
26
Excessive pooling
? intra-vascular volume
? catecholamines ? HR
POTS
Standing
Sympathetic withdrawal
NMH
27
Neurally Mediated Hypotension

• The most common cause of recurrent syncope
• More common in women, the young, those with low
  normal or low BP
• Common following infection
• Family members often affected
• Routine physical and lab tests normal
• Hypotension not detected unless orthostatic
  stress is prolonged
• Fatigue common for hours after syncope

28
(No Transcript)
29
(No Transcript)
30
(No Transcript)
31
(No Transcript)
32
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
  1995274961-7.

33
Therapy For Orthostatic Intolerance

• ? blood volume
• Sodium, fludrocortisone, clonidine, OCPs
• ? catecholamine release or effect
• ?-blockers, disopyramide, SSRIs, ACE inhibitors
  
• Vasoconstriction
• Midodrine, dexedrine, methylphenidate

34
Bou-Holaigah I, Rowe PC, Kan JS, Calkins H. JAMA
199527496-7.
35
How Might Orthostatic Intolerance Be Associated
With CFS?

• Fainting due to NMH is associated with up to 72
  hours of fatigue
• Near-fainting and lightheadedness on a repeated
  basis throughout the day likely cause fatigue
  through the same (as yet unknown) mechanism
• The more lightheaded and intolerant of
  orthostatic stress, the more the tendency to lie
  downleading to reduced blood volume and worse
  OI.

36

• Complexities of chronic illness
• CFS clinical features
• Insights into
• orthostatic intolerance
• joint hypermobility
• abnormalities on PT examination
• 4. Lessons

37
Ehlers-Danlos Syndrome

• Heterogeneous disorder of connective tissue
• Prevalence unknown, perhaps 1 per 5000
• Characterized by varying degrees of
• Skin hyperextensibility
• Joint hypermobility
• Cutaneous scarring
• Early varicose veins, easy bruising
• Easy fatigability and widespread pain common, of
  unclear etiology

38
(No Transcript)
39
(No Transcript)
40
(No Transcript)
41
(No Transcript)
42
(No Transcript)
43
(No Transcript)
44
EDS In CFS Patients With Orthostatic Intolerance

• 12 with EDS among 100 clinic attendees
• Only 1 previously recognized with EDS
• Fatigue present for median of 37 mo before EDS
  recognized (range 12-62)
• Could milder forms of connective tissue laxity be
  a risk factor for developing CFS?

Rowe PC, et al. J Pediatr 1999135494-9
45
Beighton Joint Hypermobility Scoresin 58
Adolescents With CFS And 58 Healthy Controls

Barron, Geraghty, Cohen, Violand, Rowe. J Pediatr
2002141421-5
Beighton scores
46
How Might Hypermobility Be Associated With CFS?

• Connective tissue laxity in blood vessels
  promotes excessive pooling during upright
  posture, leading to OI symptoms
• Hypermobility leads to decreased activity
• Associated with another factor (eg, autonomic
  dysfunction, panic)

47

• Complexities of chronic illness
• CFS clinical features
• Insights into
• orthostatic intolerance
• joint hypermobility
• abnormalities on PT examination
• 4. Lessons

48
Physical Therapy Pilot Observations

• Increased prevalence of postural abnormalities
  and movement restrictions in patients with CFS
  and other chronic multi-symptom illnesses
• Symptoms can be reproduced by selectively placing
  tension on the neural tissues in the limbs

49
Abnormal postures
50
Restricted Ankle Dorsiflexion

• Healthy CFS

51
Restricted Straight Leg Raise

• Healthy CFS

52
Concept of Adverse Neural Tension

• Neuroanatomic studies have emphasized that the
  nervous system must adapt mechanically as we move
• Vertebral canal length increases 5-9 cm from full
  backbend to full forward bend
• Median nerve must adapt to a 20 length
  difference between arm flexion and extension
• Inability to adapt mechanically has
  electrophysiologic and neurochemical consequences

Ref Butler D. Mobilisation of the nervous
system. 1999
53
Changes with SLR over 12 minutes
Severity
Degrees of SLR
54
How Might Movement Restrictions Be Associated
With CFS?

• Pathophysiology of symptoms with neural
  elongation strain awaits clarification
• Meanwhile, improvement in symptoms, ROM,
  orthostatic tolerance, and exercise tolerance
  appears to follow manual therapy designed to
  reduce adverse neural tension and improve
  movement restrictions

55

• Complexities of chronic illness
• CFS clinical features
• Insights into
• orthostatic intolerance
• joint hypermobility
• abnormalities on PT examination
• 4. Lessons

56
Lessons from CFS

• Out of dissonance comes discovery
• William Carlos Williams

57
Lessons from CFS

• CFS research and clinical insights apply to
  symptoms of pain, lightheadedness, fatigue across
  diagnostic categories
• Support, encouragement, and withholding judgment
  go a long way
• Care for chronic disorders like CFS is
  fragmented, but this is yet another opportunity
  to help children