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Lung transplantation in children

Lung transplantation in children. Paul Aurora Respiratory and Cardiothoracic Transplant Units Great Ormond Street Hospital for Children Portex Respiratory Unit, Institute of Child Health London, UK. AGE DISTRIBUTION OF PEDIATRIC LUNG RECIPIENTS By Year of Transplant. ISHLT. 2010.

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Lung transplantation in children

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  1. Lung transplantation in children Paul Aurora Respiratory and Cardiothoracic Transplant Units Great Ormond Street Hospital for Children Portex Respiratory Unit, Institute of Child Health London, UK

  2. AGE DISTRIBUTION OF PEDIATRIC LUNG RECIPIENTSBy Year of Transplant ISHLT 2010 Aurora, JHLT, 2010

  3. NUMBER OF PEDIATRIC LUNG TRANSPLANTSBY CENTER VOLUME ISHLT 2010 Aurora, JHLT, 2010

  4. DIAGNOSIS IN PEDIATRIC LUNG RECIPIENTSBY YEAR OF TRANSPLANTAge: 12-17 Years ISHLT 2010 Aurora, JHLT, 2010

  5. This is a small field • International pediatric lung transplant collaboration formed in 2004 • Number of multicentre descriptive reports published, most important being CMV care1, fungal infections2,3, and outcomes of mild rejection4 • Recent/ongoing studies re effect of transplantation on QoL, and impact of post-transplatation viral infections 1 Danziger-Isakov, Transplantation 2009, 2 Danziger-Isakov, JHLT 2008, 3 Liu, JHLT 2009, 4 Benden, Ped Transpl, 2010

  6. LUNG TRANSPLANTATIONKaplan-Meier Survival by Age Group (Transplants: January 1990 - June 2008) ISHLT 2010 Aurora, JHLT, 2010

  7. PEDIATRIC LUNG TRANSPLANTATIONKaplan-Meier Survival by Age Group (Transplants: January 1990 - June 2008) ISHLT 2010 Aurora, JHLT, 2010

  8. PEDIATRIC LUNG TRANSPLANTATIONConditional Kaplan-Meier Survival by Age Group(Transplants: January 1990 - June 2008) ISHLT 2010 Aurora, JHLT, 2010

  9. PEDIATRIC LUNG TRANSPLANTATION Kaplan-Meier Survival by Era (Transplants: January 1988 - June 2008) ISHLT 2010 Aurora, JHLT, 2010

  10. ISHLT 2010 PEDIATRIC LUNG TRANSPLANT RECIPIENTS (1/1991-6/2008) Risk Factors For 1 Year Mortality/Graft Failure N=773 Aurora, JHLT, 2010

  11. ISHLT 2010 PEDIATRIC LUNG TRANSPLANT RECIPIENTS (1/1991-6/2008) Risk Factors For 1 Year Mortality/Graft Failure N=773 Aurora, JHLT, 2010

  12. ISHLT 2010 PEDIATRIC LUNG TRANSPLANT RECIPIENTS (1/1991-6/2008) Risk Factors For 1 Year Mortality/Graft Failure Recipient Age Aurora, JHLT, 2010

  13. ISHLT 2010 PEDIATRIC LUNG TRANSPLANT RECIPIENTS (1/1991-6/2008) Risk Factors For 1 Year Mortality/Graft Failure Center Volume Pediatric Transplants Aurora, JHLT, 2010

  14. Indications for listing • Children are only listed for transplant if: • Predicted life expectancy without transplant is two years or less • No contraindications • Poor quality of life • Full informed consent

  15. Post transplant monitoringPaediatric aspects

  16. Pulmonary complications • Complications with graft are the main impediment to good long-term outcome, i.e. • Rejection • Infection • Bronchiolitis Obliterans Syndrome (BOS) • BOS has multifactorial origin, but repeated rejection and infection are probable causes • Early detection and treatment are essential

  17. Graft monitoring, 1: Symptoms • Rejection and lower respiratory infection can present with mild, non-specific symptoms • Dyspnoea • Reduced exercise tolerance • Cough • Low grade fever • Malaise • The child and family must be educated as to the importance of these symptoms

  18. Graft monitoring, 2: Lung function • Most children over 4 years age can perform spirometry, following training • Outcome measures (and quality control criteria) may need to be modified, e.g. report FEV0.75 rather than FEV1 • Monitor in clinic, but also at home • Drop of greater than 10% should cause concern

  19. WHICH ANIMATION…? …FLYING TOASTER? ...CANDLES? …BALLOON? Onlyfor initial training or PEF Too complex for the very young Ideal for preschoolers

  20. Graft monitoring, 2: Lung function • Children aged 6 years and older can perform eNO manoeuvres using similar techniques to adults • In younger children modified techniques are probably necessary

  21. Graft monitoring, 2: Lung function • Data from Estenne and colleagues suggest that gas mixing studies allow earlier detection of graft dysfunction than spirometry • Although the single breath washout test is not possible in young children, a modified version of multiple-breath washout can be performed instead

  22. Graft monitoring, 2: Lung function • Lung function testing in infants is usually performed during (sedated) sleep • Variety of techniques available, which mirror tests performed in adults

  23. Forced expiratory maneuvers adapted for use in infants

  24. Graft monitoring, 3: Transbronchial biopsy • As for adults, is essential for distinguishing between rejection and infection • Difficult to perform successfully with small bronchoscopes, but newer generation scopes may be better • Use of X-ray screening is essential, and general anaesthesia via laryngeal mask airway is helpful

  25. Transbronchial biopsy Ext diam. 4.9mm 3.6mm 2.8mm Int diam. 2.0mm 1.2mm 1.2mm Age 4 yrs + 1 yr + from birth

  26. Transbronchial biopsy Ext diam. 4.9mm 3.6mm 2.8mm Int diam. 2.0mm 1.2mm 1.2mm Age 4 yrs + 1 yr + from birth Ext diam. 4.9mm 4.0mm 2.8mm Int diam. 2.0mm 2.0mm 1.2mm Age 4 yrs + 1 yr + from birth

  27. Graft monitoring, 3: Transbronchial biopsy • Biopsies should be performed in sick children, where diagnosis is uncertain • Use of routine biopsy in well children is controversial • Most paediatric centres perform biopsies regularly in first year

  28. Graft monitoring, 4: Radiology • Plain radiographs performed regularly post-transplant, but don’t distinguish between rejection and infection • Either process may be present with normal radiograph

  29. Graft monitoring, 4: Radiology • New generation of multislice scanners allow rapid acquisition of HRCT data • Speed of scanner means that sedation is not necessary even in very young children • Protocols MUST be adjusted to minimise radiation exposure • What is the role?

  30. Post transplant OB, inspiratory film

  31. Post transplant OB, expiratory film

  32. Post-transplant infections • Primary viral infections more common in paediatric recipients as many will not have previous exposure • Measles and varicella can be fatal post transplant • Essential to immunise pre-transplant, and to advise family regarding precautions

  33. Post-transplant infections • Post transplant lymphoproliferative disease is much more common in children • Presents clinically with lymphadenopathy, anaemia, low grade pyrexia, malaise… • Possible to monitor quantitative EBV count by PCR, but is this of any help?

  34. Post-transplant infections • Also be aware of • CMV infection • respiratory viruses • PCP • fungi • Low grade bacterial infection • As for adults

  35. Non allogeneic causes of BOS • Recent interest in role of gastrooesophageal reflux, bacterial infections • Approach should be same as for adult subjects

  36. Side-effects of immunosuppression • Maintenance therapy usually triple: • ciclosporin or tacrolimus • azathioprine or MMF • corticosteroids • As well as immunosuppression itself, all agents have specific side effects • Common to adult patients, but still v important

  37. Side-effects of immunosuppression • Ciclosporin and tacrolimus are nephrotoxic, this is partly dose related and reversible, but there is also irreversible progressive component • Marrow suppression with aza/MMF • Diabetes mellitus, particularly in children with CF on tacrolimus

  38. Other CF complications • Bone density is abnormal in many children, particularly those with CF • Also need to be aware of non-respiratory complications of CF

  39. Growth failure • Many children referred for transplant already have growth failure • This is worsened by corticosteroids • Reduce dose as much as possible • Growth hormone is controversial

  40. Do the lungs grow? • Yes, provided the child remains healthy • Forced expiratory flows increase as expected, suggesting that airways grow • Do alveoli multiply, or do they distend?

  41. Psychosocial issues • Many children are socially and physically immature prior to transplant • Behavioural difficulties post transplant are common • Greatest concern is non-adherence to therapy, particularly amongst adolescents

  42. How is this delivered?

  43. Surgical staff Pharmacist Medical staff Nursing staff Psychologist Other medical and surgical teams Local paediatrician Child Community team Non invasive Monitoring i.e. cardiac and respiratory physiology Invasive Monitoring i.e. catheter studies and bronchoscopy Local pharmacist Anaesthesia

  44. Pharmacist Medical staff Nursing staff Psychologist Child Local paediatrician GP Community nurses School Wider family and friends

  45. What should the local paediatrician do?

  46. Shared care • Lung transplantation in children is uncommon • Large centres produce better outcomes • Only a small number of transplant centres are needed, and many patients will live a long way from the transplant centre • Shared care with the local paediatrician or paediatric pulmonologist is essential

  47. Shared care • The local team will see the child regularly in clinic • On each occasion • Spirometry • Full Blood Count • Renal function / blood biochemistry • Immunosuppressant blood levels must be performed

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