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European Journal of Pediatrics

, Volume 178, Issue 7, pp 1095–1103 | Cite as

Care for children with severe chronic skin diseases

  • Hannelore De Maeseneer
  • Dirk Van Gysel
  • Sofie De Schepper
  • Carsten R. Lincke
  • Barbara J. Sibbles
  • Josette J. W. M. Versteegh
  • Welling Oei
  • Robert F. Pangalila
  • Suzanne G. M. A. PasmansEmail author
Original Article

Abstract

In this study, the care for children with a severe chronic skin disease in our national expert center of pediatric dermatology was evaluated. Patients and their parents were questioned by using existing questionnaires: 50 pediatric patients completed the modified “my positive health” questionnaire of Huber and 51 parents completed Pelentsov parental needs scale. Nineteen involved professionals answered a questionnaire with open boxes. Parents of children with a variety of chronic skin diseases and young adult patients were interviewed to find out what an optimal approach would look like according to them. Children with a severe chronic and/or congenital skin disorder score high on the “my positive health” questionnaire, indicating they are able to adapt and self-manage. Their highest median score was measured for the dimension “quality of life.” Their parents expect improvement of “working with health care professionals,” more specifically they want them to adopt a more holistic approach throughout the patient’s life. Structured interviews showed they expect that a multidisciplinary team of care providers determine together with the patient and its family—in advance—which care is needed, at what time and by whom. The interviewed professionals indicated adoption of a holistic multidisciplinary approach as the single largest improvement to achieve better care.

Conclusion: Although these children with a severe chronic and/or congenital skin disease were able to adapt and self-manage, they need a more personalized integrative multidisciplinary and systematic transmural approach covering all aspects of life during their lifetime.

What is Known:

Severe skin disorders affect the child and its family in several ways. In our expert center, we try to optimize the care for these children through a multidisciplinary approach.

What is New:

To our knowledge, no English publication describes the requirements for good care for pediatric patients with severe chronic skin disorders and how to optimize this care. We evaluated the health status of children with severe chronic skin disorders and the strengths and weaknesses of past and current care by questioning these children, their parents, adult patients, and involved professionals.

Keywords

Pediatric dermatology Severe congenital skin disorders Multidisciplinary approach Evaluation of care Quality of life 

Abbreviations

CDLE

Chronic discoid lupus erythematosus

CLOVES

Congenital, lipomatous, overgrowth, vascular malformations, epidermal nevi, and spinal/skeletal anomalies and/or scoliosis

CMN

Congenital melanocytic naevus

DIM

Dimension

ED

Ectodermal dysplasia

e.g.

For example

Erasmus MC

Erasmus University Medical Center

etc.

Etcetera

F

Female

IFAP

Ichthyosis follicularis, alopecia, and photophobia

IPEX

Immune dysregulation, polyendrocrinopathy, enteropathy, X-linked

LUMBAR

Lower body hemangioma, urogenital anomalies, myelopathy, bone deformities, anorectal malformations, renal anomalies

M

Male

N/A

Not applicable

Pt

Patient

SWS

Sturge-Weber syndrome

Notes

Acknowledgements

Special thanks go to all the children and parents who participated in this study. We thank Machteld Huber and Lemuel Pelentsov for their permission to use their questionnaires. Further, we thank Anneke Jonker, Jolien van der Geugten, Hanneke Vandijk and Karin Veldman for their input in the preparing interviews. This project is a contribution to the aims of the European Reference Network Skin (ERN-SKIN).

Authors’ contributions

Hannelore De Maeseneer designed the study, performed data collection and analysis, and drafted the manuscript.

Dirk Van Gysel and Sofie De Schepper critically reviewed the manuscript.

Carsten R. Lincke, Barbara J. Sibbles, Josette J.W.M. Versteegh, Welling Oei, and Robert F. Pangalila were involved in the design of the study and in data interpretation and they critically reviewed the manuscript.

Suzanne G.M.A. Pasmans conceived the study, coordinated all study phases, and has supervised and extensively revised the manuscript.

All authors read and approved the final manuscript including the authorship list.

Compliance with ethical standards

Conflict of interest

The first author (Hannelore De Maeseneer) has received an unrestricted grant of Laboratoires Expanscience. The other authors declare that they have no conflict of interest.

Ethical approval

This article does not contain any studies with human participants or animals performed by any of the authors.

Informed consent

Informed consent was obtained from all individual participants included in the study.

Supplementary material

431_2019_3366_MOESM1_ESM.pdf (258 kb)
ESM 1 (PDF 257 kb)

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  • Hannelore De Maeseneer
    • 1
  • Dirk Van Gysel
    • 1
  • Sofie De Schepper
    • 2
  • Carsten R. Lincke
    • 3
  • Barbara J. Sibbles
    • 3
  • Josette J. W. M. Versteegh
    • 4
  • Welling Oei
    • 5
  • Robert F. Pangalila
    • 6
  • Suzanne G. M. A. Pasmans
    • 7
    Email author
  1. 1.Department of PediatricsO.L.Vrouw HospitalAalstBelgium
  2. 2.Department of DermatologyGhent University HospitalGhentBelgium
  3. 3.Department of PediatricsErasmus MC-Sophia Children’s HospitalRotterdamThe Netherlands
  4. 4.Department of Child and Adolescent Psychiatry/PsychologyErasmus MC-Sophia Children’s HospitalRotterdamThe Netherlands
  5. 5.Department of DermatologyErasmus MC-Sophia Children’s Hospital Rotterdam-Center of Pediatric DermatologyRotterdamThe Netherlands
  6. 6.Department of Rehabilitation MedicineErasmus MC University Medical CenterRotterdamThe Netherlands
  7. 7.Department of DermatologyErasmus MC-Sophia Children’s Hospital Rotterdam - Center of Pediatric Dermatology, Health Care Provider of the European Reference Network SKINRotterdamThe Netherlands

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