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Palliative care for children with a yet undiagnosed syndrome

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Abstract

The number of children without a diagnosis in pediatric palliative home care and the process of decision-making in these children are widely unknown. The study was conducted as single-center retrospective cohort study. Between January 2013 and September 2016, 198 children and young adults were cared for; 27 (13.6%) of these were without a clear diagnosis at the start of pediatric palliative home care. A definite diagnosis was ultimately achieved in three children. Median age was 7 years (0–25), duration of care 569 days (2–2638), and number of home visits 7.5 (2–46). Most patients are still alive (19; 70.4%). Median number of drugs administered was eight (range 2–19); antiepileptics were given most frequently. Despite the lack of a clear diagnosis (and thus prognosis), 13 (48.1%) parents faced with their critically ill and clinically deteriorating children decided in favor of a DNAR order. Comparing this with 15 brain-injured children, signs, symptoms, and supportive needs were similar in both groups.

Conclusion: Children without a clear diagnosis are relatively common in pediatric palliative care and have—like all other patients—the right to receive optimized and symptom-adapted palliative care. Parents are less likely to choose treatment limitation for children who lack a definitive diagnosis.

What is Known:

• A clear diagnosis is usually considered important for best-practice pediatric palliative care (PPC) including advanced care planning (ACP).

• Timely initiation of pediatric palliative care (PPC) is highly recommended in children with life-limiting conditions.

What is New:

• SWAN (syndrome without a name) children show similar signs and symptoms (mostly neurological) and have similar supportive needs as brain-injured children.

• Defining treatment limitations in advance care planning is more difficult for parents of SWAN compared to brain-injured children.

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Abbreviations

ACP:

Advanced care planning

ACT:

Association for Children with Life-Limiting and Life-Threatening Conditions and their families

PaPaS Scale:

Pediatric Palliative Screening Scale

PPC:

Pediatric palliative care

PPCT:

Pediatric palliative care team

SWAN:

Syndrome without a name

References

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Acknowledgements

The authors thank the families for confiding in the PPC team, all members of the palliative care team Duesseldorf for their deep commitment, and the “Elterninitiative Kinderkrebsklinik Duesseldorf e.V.” for long-standing financial support.

Funding

This work was supported by the Elterninitiative Kinderkrebsklinik e.V. Duesseldorf.

Author information

Author notes

  1. Gisela Janßen and Michaela Kuhlen—these senior authors contributed equally.

Authors and Affiliations

  1. Medical Faculty, Department of Paediatric Oncology, Haematology and Clinical Immunology, Centre for Child and Adolescent Health, University of Duesseldorf, Moorenstr. 5, 40225, Duesseldorf, Germany

    Jessica I. Hoell, Jens Warfsmann, Gabriele Gagnon, Laura Trocan, Stefan Balzer, Prasad T. Oommen, Arndt Borkhardt, Gisela Janßen & Michaela Kuhlen

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  1. Jessica I. Hoell
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  2. Jens Warfsmann
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  3. Gabriele Gagnon
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  4. Laura Trocan
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  5. Stefan Balzer
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  6. Prasad T. Oommen
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  7. Arndt Borkhardt
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  8. Gisela Janßen
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  9. Michaela Kuhlen
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Contributions

JIH designed the study and drafted the manuscript. JW performed data analysis. GG, LT, SB, and PTO cared for the patients and acquired data. AB critically reviewed and revised the manuscript. GJ conceptualized the study, filled in the PaPaS Scale, and supervised data collection. MK conceptualized and designed the study, supervised data analysis, and drafted the manuscript. All authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work.

Corresponding author

Correspondence to Michaela Kuhlen.

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Conflict of interest

The authors declare that they have no conflict of interest.

Informed consent

Informed consent was obtained from the parents and legal guardians respectively.

Additional information

Communicated by Mario Bianchetti

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Hoell, J.I., Warfsmann, J., Gagnon, G. et al. Palliative care for children with a yet undiagnosed syndrome. Eur J Pediatr 176, 1319–1327 (2017). https://doi.org/10.1007/s00431-017-2991-z

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  • DOI: https://doi.org/10.1007/s00431-017-2991-z

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