Palliative care in pediatric hematological malignancies Dr Matthias SCHELL, IHOP, ESPPéRA, Lyon

1. Palliative care in pediatric hematological malignancies Dr Matthias SCHELL, IHOP, ESPPéRA, Lyon Équipe de soins de support, soins palliatifs pédiatriques, de ressource et d’accompagnement

2. Survival for acute lymphoblastic leukemia (ALL) trials EORTC Children Leukemia Group % 100 5-year survival rate ALL-3 (1999 –2008 ) 90 90% ALL-2 (1989 – 1998) 82% 80 80% ALL-1 (1983 – 1988) 70 60 50 40 30 20 10 0 (years) 0 5 10 15 20 O N Number of patients at risk : ALL-3 66 1199 26 0 0 ALL-2 406 2216 1263 203 0 ALL-1 165 735 556 230 23

3. Survival in pediatric hematological malignancies B-cell lymphomas: LMB 96

4. As not all patients may be cured • Even if it is a rare event, PCC is a reality in our daily work • Death may occur in all patients we are treating • PC is difficult for all involved persons • The child himself • Parents • One of the most serious traumas an adult may experience • siblings, grandparents, proxies • Caregivers : nurses, doctors…. • Difficult to admit that treatment failed • Very difficult to admit that other treatment options will not cure the child

5. Clinical situations of PC in hematological malignancies Relapse and resistant diseases • Questions to ask: • Offer the treatment proposal a realistic chance of remission ? • Or • Is it a palliative chemotherapy ? • With the goal to treat illness without chances to cure • Or • Is it an alibi to avoid difficult discussions ? • Further questions to be asked: • Is the new treatment plan reasonable for all persons involved? • Or • it is “just” too difficult to stop thinking that doing more is doing better?

6. Curative treatment period Palliative care Chances and hope to cure Période palliative with remission Terminal phase AGONIE Specific cancer treatment Supportive care Diagnostic ofterminal phase Death Diagnostic of resistant disease or non treatable side effects

7. Choosing the right palliative chemotherapy The TwiSt theory determine Qol

8. Best specific treatment options in PC • We should not aim duration of life • We should not aim unrealistic remission • But • We should aim Qol and TwiSt Therefore • New and aggressive treatment options that will not cure are not the best treatment options • Less toxic treatment may exist and often offers • Survival with lower rate of side effects • More outpatients days for the kid and his family • And all kid would prefer staying at home • Lower costs • Better Qol

9. To take care and to cure • When you can’t cure any more • Treatment failure is not your fault and you are not a bad doctor Further on you should be convinced that • Palliative care is not “to do nothing” • Palliative care is not an abandonment • Palliative care is to recognize that resistant disease or life-threatening side effects are not the only parameters which should be taken into account. • You will never stop taking care of a child that might not be cured PC is to take care if can’t cure

10. What is PC? • Palliative care for children is the active total care of the child's body, mind and spirit, and also involves giving support to the family. • PC is a challenging care with an ongoing need of competence, implication and engagement of all care givers Questions to ask • What wants the child • What wants the parents • What do you want?

11. Clinical situations of PC in hematological malignancies Life-threatening side effects of treatment • i.e. acute or severe chronic GVH, infections, • Questions to ask: • What do we aim, what is the target of the treatment • Is it still useful • Is it better to stop or to continue the treatment? • Should we replace it or add another? • What is the aim and the potential consequence of investigations • Does they change any decisions • Or are they realized to get more results, without changing our attitudes ?

12. PC challenges in resistant diseases • To avoid unreasonable specific treatments (chemotherapy…) that increases suffering but not Qol • To avoid unreasonable treatment options • Like transfer into an ICU PC challenges in life-threatening adverse effects • To avoid escalating treatment options without solutions • To determine when treatment options will be unreasonable • To be able to decide stopping • treatments that become useless and sometimes even harmful The challenge is to take care of the child and his family and not to focus only on treatment of malignant disease

13. End of life symptoms 100% present Induces suffering 80% Souffrance uncontrolled 60% 40% 20% 0% Dyspnea Anorexia Diarrhea Nausea fatigue pain Constipation J. Wolfe, N Engl J Med 2000 ; 342: 326-33

15. BACKGROUND: The aim of the study was to make a comprehensive inventory of the physical, psychological, and social symptoms of children with cancer and their parents during the palliative phase and the extent to which health professionals address those symptoms. PROCEDURE: Forty parents of children who died 1-3 years prior to data collection (structured questionnaire) were invited to participate in the study. RESULTS: The response rate was 32 out of 40 parents (80%). Most frequently mentioned physical symptoms were pain, poor appetite, and fatigue. The children's most mentioned psychological symptoms were sadness, difficulty in talking to their parents about their feelings regarding illness and death and fear of being alone.The symptoms of fear of death of the child and fear of physical symptoms were most frequently mentioned parents' psychological symptoms. Health professionals addressed 82% of the children's physical symptoms, 43% of the children's psychological symptoms, and 56% of the parents' psychological symptoms. Parents indicated that after professional attention the proportion of children's physical symptoms that were completely or partially resolved was 18 and 26%, respectively. For children's psychological symptoms the figures were 9 and 25%, respectively, and for parents' psychological symptoms 2 and 23%, respectively. CONCLUSIONS: The burden of symptoms of the child with cancer during the palliative phase and their parents is high. Health professionals focus mainly on the physical symptoms of the child. Relief of symptoms could not be achieved for a large proportion of symptoms. Further prospective research is necessary to investigate the kind, frequency and intensity of symptoms in order to tailor optimal palliative care to the needs of both child and parents

16. How to improve PPC • Recognize that palliative care is reality in hematological malignancies • Recognize that palliative care is an active and challenging care focused on the child's suffering and needs more than focused on disease • Remember the TwiSt theory when choosing treatment options • Initiate a national working group to discuss difficult cases and to develop training programs for care givers?