Interdisciplinary Palliative Care for children with congenital heart defects and other severe heart conditions

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1 Interdisciplinary Palliative Care for children with congenital heart defects and other severe heart conditions Ayda Duroux, Markus Loeff, Klaus Kinast, Gian Domenico Borasio, Monika Führer Coordination Center for Pediatric Palliative Medicine University Hospital Munich

2 Deaths in childhood and adolescence caused by life limiting diseases in Bavaria 2005 (n = 602) 3% 9% 3% 11% infections 27% 3% 9% 4% 3% neoplasia inborn metab. errors nervous system heart and circulation pulmonary system perinatal period genetic disorders not classifiable other 28% Causes of deaths in patients < 20 years (Bavaria 2005, n = 602) Quelle: Statistisches Landesamt

3 Patients of the Coordination Center for Pediatric Palliative Care (CPPC) in Munich March 04 - May 08 number of children 149 Gender Migration background 20% Age (Median) 47 % male 5.0 ys. (0 43 ys.) <1 y. n=31 >18 ys. n=12 cardiologic diseases 23 (15%) prenatal palliative care 7 died 88

4 Cardiologic diseases: Patient characteristics March 04 - May 08 n=23 Age (Median) 4.0 yrs. (0 18 yrs.) Duration of care 5.6 mths. (0.7 12) prenatal palliative care 1 died 15 (7 at home 47%)

5 Diagnoses - Hypoplastic left heart failure n = 7 - Pulmonary atresia n = 4 - Dilatative cardiomyopathy n = 3 - Complex dysmorphic syndromes n = 5 - Marfan syndrome n = 1 - Heart failure post heart transplant n = 1 - Endocarditis n = 1 - Interrupted aortic arch n = 1

6 Type of Care provided by the CPPC n = 1 prenatal PC n = 1 Consultation on pain control n = 10 Consultation on therapeutic goals n = 6 Coordination of PC at home n = 5 Consultation on therapeutic goals and coordination of PC at home

7 Case 1: Hypoplastic Left Heart Syndrom (HLHS) male, diagnosed with HLHS at birth decompensated heart failure (NYHA 4) at 1.7 years - waiting for heart transplant (HU) since 2 months - team and parents recognize severe suffering thirst dyspnea insomnia edema pain Parents decide against HTX Palliative Care: morphine for pain and dyspnoea relief Life expectation: few weeks

8 Anxiety and Pain Pain and low cardiac output increased heart rate decreased coronary perfusion

9 Case 1: HLHS Specialized Pediatric Palliative Home Care intensive medical and nursing care continuation of complex pharmaceutical treatment general condition improved significantly through - emotional well-being - better sleep and rest - less pain and dyspnea (morphine) cardiac stress psychosocial problems (siblings, divorce, financial situation)

10 Kindergarden at age 3 - developed social skills - less dependent still alive at age 4 deterioration after last intracardiac catheter after 1 week in children s hospice back home

11 Main symptoms in cardiologic patients Dyspnea Edema, ascites and pleural effusions Signs of acute and chronic multi-organ failure Cyanosis (painless) Asphyxia (painful) Sequelae from chronic hospitalisation

12 Case 2: Hypoplastic Left Heart Syndrom (HLHS) male, prenatal diagnosis of HLHS Intensive counselling of all specialists involved (obstetrics, neonatology, cardiac surgery, CPPC) muslim parents decide against termination of pregnancy and against post-natal correction or heart transplantation spontaneous birth, good clinical condition regular visits of the CPPC-team

13 Case 2: Hypoplastic Left Heart Syndrom (HLHS) Main fields of parent counselling - healthy looking child - suffering of the child - attachment to the child - place for the child - faith When is our child going to die? How is our child going to die?

14 Place of death (1 16 years) % of patients who died at home % p< % 0 Bavaria (2005) CPPC ( )

15 Place of death (< 1 year) p<0.001 % of patients who died at home % 41% 0 Bavaria (2005) CPPC ( )

16 Summary Up to 15% of patients in pediatric palliative care suffer from inborn or acquired cardiologic diseases. Main areas of support are - counselling in the decision making process - providing specialized palliative home care Intensive interdisciplinary cooperation between the CPPC team and the cardiologic team is necessary.

17 Conclusions The CPPC team can address disease-specific medical and nursing aspects of care and assist in advance care planning. Effective palliative home care can help to substantially increase the rate of children with complex cardiologic conditions who die at home.

18 !Thank you! All Children and their families Chair in Palliative Medicine: Prof. Dr. G.D. Borasio CPPC team: Dr. Ayda Duroux Dr. Monika Grasser Dr. Marion Eckert Klaus Kinast René Vollenbroich Tanja Hackl Director of the Children s Hospital: Prof. Dr. Dr. D. Reinhardt

19 Wie wird unser Kind sterben?

20 Todesfälle 2005 in Bayern Altersverteilung 61% 11% 7% 10% < 1 Jahr 1 bis 5 Jahre 5 bis 10 Jahre 10 bis 15 Jahre 15 bis 20 Jahre 11% Gesamt: 602 Fälle Quelle: Statistisches Landesamt

21 Patients of the Coordination Center for Pediatric Palliative Care - Diagnoses (n=149) 1% 5% 1% 27% 10% 2% 2% 18% 23% 11% infections neoplasia inborn metab. Errors neurvous system heart and circulation pulomary system perinatal period genetic disorders not classifiable other

22 Diagnoses Diagnosis Hypoplastic left heart syndrome Pulmonary atresia Dilatative cardiomyopathy Complex dysmorphic syndrome Marfan syndrome Heart Transplant Endocarditis interrupted aortic arch n Additional information 4 patients with failing Fontan 1 patient died after cessation of bridging before HTX Severe cardiac insufficiency Organ failure 8 years after TX, bridging zu Re-TX Mutli-organ failure died after RSV infection