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1 Welcome to Allied Health Telehealth Paediatric lymphoedema A challenge for clinicians and families To receive an attendance certificate please complete your online evaluation at: Paediatric lymphoedema A challenge for clinicians and families Margaret Patterson Senior Physiotherapist Sydney Children s Hospital Randwick Johanna Newsom Senior Physiotherapist Children s Hospital at Westmead 1

2 What is lymphoedema? Swelling of a part of the body caused by accumulation of interstitial fluid secondary to a malformation or malfunction of the lymphatic system Lymphoedema Framework document Low flow, high protein oedema What is lymphoedema? 2

3 Lymphoedema Chronic condition Not curable Alleviated by appropriate management If ignored it can progress and become difficult to manage Aetiology Congenital malformation of the lymphatic system (primary) Damage to lymphatic vessels and /or lymph nodes (secondary) Trauma Cancer and its treatment Infections eg: filiarisis 3

4 The Lymphatic System A one-way drainage system which transports lymph from the tissues to the vascular system Healthy lymph system will transport 2-4L/day Continuous rapid removal of interstitial fluid, plasma proteins, cells and debris The Lymphatic System Plays an integral role in the immune functions of the body First line of defence against disease Organised in groups that drain specific regions 4

5 The Lymphatic System Incidence At birth, about 1 in 6000 will develop primary lymphoedema Overall prevalence estimated as % In developed countries, main cause widely assumed to be treatment for cancer 5

6 Primary vs Secondary Primary: 3-10% of all lymphoedemas Idiopathic, with no identified cause Genetic causes Associated with a syndrome Secondary: very rare in paediatrics Identified cause eg: infection, surgery, tumours, radiation, trauma, obesity, filiarisis Milroy s Disease Visible at birth or soon after 5-10% of all primary lymphoedemas Usually affects lower limbs 2:1 females to males Familial pattern of inheritance 6

7 Milroy s Disease Lymphoedema Praecox Approximately 80% of cases of primary lymphoedema Occurs during 2 nd and 3 rd decade of life 7

8 Lymphoedema Tarda Occurs after age 35 Begins in foot and ankle progressing for months or years About 70% experience swelling in one lower limb Impacts of lymphoedema Lymphoedema may produce significant physical and psychological morbidity Increased limb size can interfere with mobility and affect body image Pain and discomfort are frequent symptoms 8

9 Impacts of lymphoedema Increased susceptibility to acute cellulitis/erysipelas can result in frequent hospitalisations and long-term dependency on antibiotics Cellulitis Stagnant oedema fluid provides an ideal medium for bacterial growth Acute, diffuse, spreading, oedematous, suppurative infection of deeper subcutaneous tissue and fat. 9

10 Lymphoedema with cellulitis Lymphoedema staging Several staging systems have been devised ISL International Society of Lymphology 10

11 ISL 0 A sub clinical state where swelling is not evident despite impaired lymph transport This stage may exist for months or years ISL 1 Early onset of condition Accumulation of tissue fluid Subsides with limb elevation Oedema may be pitting 11

12 ISL 2 Limb elevation alone rarely reduces swelling Pitting is manifest Late stage 2 (also called stage 3) Tissue fibrosis more evident May or may not be pitting Elephantiasis Usually develops in untreated cases of primary and filiarial lymphoedema Gross oedema with loss of limb shape Reduced lymphatic transport capacity Increase of subcutaneous fat and fibrous tissue 12

13 Elephantiasis Poor posture Impaired gait Lymphorrhoea Elephantiasis 13

14 Diagnosis and Recognition Clinical history History and behaviour of swelling Symptoms such as heaviness, tightness or hardness History of skin or nail infections Family history Recognition Objective findings: Distribution of swelling Pitting oedema Positive Stemmer s sign Increased skin folds Changes to skin texture and quality 14

15 Clinical findings Investigations Albumin Markers of immune function Renal function tests Lymphoscintigraphy MRI CT Ultrasound 15

16 Assessment of swelling Circumferential limb measurements Other options: Water displacement method Perometry Bioimpedance Assessment of skin condition Dryness Dermatitis Fragility Pigmentation Redness/pallor Cyanosis Warmth/coolness Stemmer s sign Fungal infection Hyperkeratosis Lymphorrhoea Cellulitis 16

17 Assessment of skin condition Other assessments Pain Psychosocial Mobility and functional Nutritional 17

18 Management Early and accurate diagnosis is essential Life-long condition Emphasis on management rather than cure Multidisciplinary input ideal Lymphoedema therapist to coordinate care Basic principles Reduction of swelling and improvement of shape Skin care and treatment of skin problems Prevention of infection Pain management Psychosocial intervention Participation with peers Education 18

19 Treatment decisions Holistic approach based on principles Specific treatment tailored to individual Treatment & Management Gold standard is decongestive lymphatic therapy Complex, time-consuming, intensive, expensive and challenging Education 19

20 Treatment & Management - cautions Drug therapy Diuretics Antibiotics Surgery Decongestive Lymphatic Therapy Phase 1 initial treatment Skin care Massage Compression (bandaging) Exercise 20

21 Decongestive Lymphatic Therapy Phase 2 maintenance Compression (garments) Skin care Exercise Massage as needed Skin and limb care Daily inspections for cuts/bites/scrapes Regular moisturising Anti-microbial and anti-fungal washes Immediate ABs if any signs of infection Nail care 21

22 Skin and limb care Skin and limb care Dos and Don ts No BP or injections on affected limb No sunburn or overheating No heavy weights or strains Sensible footwear and clothing 22

23 Massage Sequential massage following the principles of lymphatic drainage Aims to reduce effects of oedema Re-route flow of stagnant lymphatic fluid into centrally located healthy lymphatic vessels Multi-layered inelastic lymphatic bandaging Firm but flexible to counteract the elastic insufficiency of the skin Increases tissue pressure and assists the musculo-lymphatic pump Soften fibrosis and restore limb shape 23

24 Bandaging Bandaging 24

25 Bandaging Bandaging 25

26 Bandaging Bandaging 26

27 Compression garments Maintain reduction In children may be the only intervention available Custom made Graduated compression Compression garments 27

28 Compression garments Exercise Mostly discussed in terms of healthy lifestyle and weight management Bandages or garments need to be worn during exercise. 28

29 Challenges for clinicians gaining education Literature and professional development focuses mainly on adults and secondary No specific guidelines for paediatric patients Accessing courses: time and money Maintaining skills in rare patient population Challenges for clinicians - services Dedicated services for children with lymphoedema are rare Local services are often unavailable or private Lack of knowledge among other professionals 29

30 Challenges for clinicians - paediatrics Effects of compression When to start bandaging/garments Manual lymphatic drainage - modified Challenges for clinicians tricky areas 30

31 Challenges for clinicians tricky areas Challenges for families Obtaining a correct diagnosis Accessing clear and correct information Accessing treatment and on-going management Lack of local services 31

32 Challenges for families Advocating for child s needs Ensuring adequate supply of garments, shoes and bandages Lack of funding Parents as therapists Challenges for families Impact on adolescents e.g. body image Managing physical and psychosocial needs Allowing children normality 32

33 Questions? Margaret Patterson: Ph: (02) Johanna Newsom Ph: (02) References Lymphoedema Framework. Best practice for the Management of Lymphoedema. International consensus. London: MEP Ltd, 2006 International Lymphoedema Framework. Care of Children with Lymphoedema. Focus Document, 2010 Textbook of lymphology for Physicians and Lymphedema Therapists. 5 th Edition. Authors M.Foldi, E Foldi, S Kubik 33

34 References Connell, Brice, Mansour and Mortimer: The Presentation of Childhood Lymphoedema. Journal of Lymphoedema 2009 (4); Moffat and Murray: The experience of children and families with lymphoedema a journey within a journey. Internation Wound Journal 2010 (7);14-26 Preston, Seers and Mortimer: Physical therapies for reducing and controlling lymphoedema of the limbs. Cochrane Database of Systematic Reviews 2004 (4), republished