Newborns with myelomeningocele: their health-related quality of life and daily functioning 10 years later

Jochem K. H. SpoorDepartments of Neurosurgery,

Search for other papers by Jochem K. H. Spoor in
jns
Google Scholar
PubMed
Close
 MD, LLM
,
Oscar H. J. Eelkman RoodaDepartments of Neurosurgery,

Search for other papers by Oscar H. J. Eelkman Rooda in
jns
Google Scholar
PubMed
Close
 MD, PhD
,
Charlotte KikDepartments of Neurosurgery,

Search for other papers by Charlotte Kik in
jns
Google Scholar
PubMed
Close
 MD
,
Jetty van MeeterenRijndam Rehabilitation, Rotterdam, The Netherlands
Rehabilitation Medicine, and

Search for other papers by Jetty van Meeteren in
jns
Google Scholar
PubMed
Close
 MD, PhD
,
Tessa WestendorpRijndam Rehabilitation, Rotterdam, The Netherlands

Search for other papers by Tessa Westendorp in
jns
Google Scholar
PubMed
Close
 PhD
,
Philip L. J. DeKoninckObstetrics and Gynaecology, Erasmus Medical Center, University Medical Center, Rotterdam; and

Search for other papers by Philip L. J. DeKoninck in
jns
Google Scholar
PubMed
Close
 MD, PhD
,
Alex J. EgginkObstetrics and Gynaecology, Erasmus Medical Center, University Medical Center, Rotterdam; and

Search for other papers by Alex J. Eggink in
jns
Google Scholar
PubMed
Close
 MD, PhD
,
Marie-Lise C. van VeelenRehabilitation Medicine, and

Search for other papers by Marie-Lise C. van Veelen in
jns
Google Scholar
PubMed
Close
 MD, PhD
,
Clemens DirvenDepartments of Neurosurgery,

Search for other papers by Clemens Dirven in
jns
Google Scholar
PubMed
Close
 MD, PhD
, and
T. H. Rob de JongDepartments of Neurosurgery,

Search for other papers by T. H. Rob de Jong in
jns
Google Scholar
PubMed
Close
 MD
Restricted access

Purchase Now

USD  $45.00

JNS + Pediatrics - 1 year subscription bundle (Individuals Only)

USD  $525.00

JNS + Pediatrics + Spine - 1 year subscription bundle (Individuals Only)

USD  $624.00
USD  $45.00
USD  $525.00
USD  $624.00
Print or Print + Online Sign in

OBJECTIVE

Ten years ago, the authors reported on the outcome of their study investigating the degree of discomfort and pain in newborns with myelomeningocele (MMC), using the parameters of unbearable and hopeless suffering. In the current study, they investigated the quality of life, daily functioning, pain and fatigue, ability to communicate, and number of surgeries in the same cohort of patients. They subdivided their study population into severe (Lorber) and less severe (non-Lorber) cases and compared these cases with a healthy population (non-MMC group) and with each other.

METHODS

The parents of 22 of 28 patients gave informed consent for this study. The KIDSCREEN-27 and PEDI-CAT (Pediatric Evaluation of Disability Inventory) were used to assess quality of life and daily functioning. Pain and fatigue were self-reported on a 10-point numeric rating scale. Communication and ambulation levels were determined using the Communication Function Classification System (CFCS) and the Hoffer ambulation scale. Using reference data from the KIDSCREEN-27 and PEDI-CAT, the authors created a healthy population comparison group.

RESULTS

There was no significant difference in health-related quality-of-life (HRQOL) scores between Lorber and non-Lorber patients, except that school environment domain scores were lower in the Lorber group. When comparing the HRQOL of MMC patients with that of the non-MMC group, the physical well-being and parent relations and autonomy domains scored significantly lower. The daily functioning of MMC patients was lower on all domains of the PEDI-CAT compared with the non-MMC group. Lorber MMC patients scored lower on all domains of the PEDI-CAT when compared with non-Lorber patients. All patients were capable of communicating effectively; most patients (n = 18) were considered CFCS level I, and 4 patients were considered CFCS level II.

CONCLUSIONS

This study shows that MMC is a severe, lifelong condition that affects patients’ lives in many domains. All the patients in this study are capable of effective communication, irrespective of severity of MMC. Overall, the data show that in newborn MMC patients, future unbearable suffering with respect to pain, mobility, cognition, and communication is hard to predict and may not always occur.

ABBREVIATIONS

CFCS = Communication Function Classification System; HRQOL = health-related quality of life; MMC = myelomeningocele; NRS = numeric rating scale; PEDI-CAT = Pediatric Evaluation of Disability Inventory.
  • Collapse
  • Expand

Figure from Candela-Cantó et al. (pp 61–70).

  • 1

    De Jong TH. Deliberate termination of life of newborns with spina bifida, a critical reappraisal. Childs Nerv Syst. 2008;24(1):1356.

  • 2

    Lorber J. Spina bifida cystica. Results of treatment of 270 consecutive cases with criteria for selection for the future. Arch Dis Child. 1972;47(256):854873.

  • 3

    Verhagen E, Sauer PJJ. The Groningen protocol—euthanasia in severely ill newborns. N Engl J Med. 2005;352(10):959962.

  • 4

    Adzick NS, Thom EA, Spong CY, et al. A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med. 2011;364(11):9931004.

  • 5

    Ottenhoff MJ, Dammers R, Kompanje EJ, Tibboel D, de Jong TH. Discomfort and pain in newborns with myelomeningocele: a prospective evaluation. Pediatrics. 2012;129(3):e741e747.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 6

    Ravens-Sieberer U, Auquier P, Erhart M, et al. The KIDSCREEN-27 quality of life measure for children and adolescents: psychometric results from a cross-cultural survey in 13 European countries. Qual Life Res. 2007;16(8):13471356.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 7

    Custers JW, Wassenberg-Severijnen JE, Van der Net J, Vermeer A, Hart HT, Helders PJ. Dutch adaptation and content validity of the ‘Pediatric Evaluation of Disability Inventory (PEDI)’. Disabil Rehabil. 2002;24(5):250258.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 8

    Hoffer MM, Feiwell E, Perry R, Perry J, Bonnett C. Functional ambulation in patients with myelomeningocele. J Bone Joint Surg Am. 1973;55(1):137148.

  • 9

    Vander Zwart KE, Geytenbeek JJ, de Kleijn M, et al. Reliability of the Dutch-language version of the Communication Function Classification System and its association with language comprehension and method of communication. Dev Med Child Neurol. 2016;58(2):180188.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 10

    Bolton JE. Accuracy of recall of usual pain intensity in back pain patients. Pain. 1999;83(3):533539.

  • 11

    Gladman D, Nash P, Goto H, et al. Fatigue numeric rating scale validity, discrimination and responder definition in patients with psoriatic arthritis. RMD Open. 2020;6(1):e000928.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 12

    Berman AH, Liu B, Ullman S, Jadbäck I, Engström K. Children’s quality of life based on the KIDSCREEN-27: child self-report, parent ratings and child-parent agreement in a Swedish random population sample. PLoS One. 2016;11(3):e0150545e0150545.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 13

    Marquez YB, Johnson AR, Pata AU, Mantese B. Closure of a large lumbosacral myelomeningocele defect with a human pericardial graft: a case report. Childs Nerv Syst. 2022;38(4):851854.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 14

    Aronin PA, George TM. Commentary on ‘quality of life and myelomeningocele: an ethical and evidence-based analysis of the Groningen Protocol’ by Sean Barry, Pediatr Neurosurg 2010;46:409-414. Pediatr Neurosurg. 2010;46(6):415416.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 15

    Chervenak FA, McCullough LB, Arabin B. Why the Groningen Protocol should be rejected. Hastings Cent Rep. 2006;36(5):3033.

  • 16

    Gesundheit B, Steinberg A, Blazer S, Jotkowitz A. The Groningen Protocol—the Jewish perspective. Neonatology. 2009;96(1):610.

  • 17

    Jotkowitz A, Glick S, Gesundheit B. A case against justified non-voluntary active euthanasia (the Groningen Protocol). Am J Bioeth. 2008;8(11):2326.

  • 18

    Kodish E. Paediatric ethics: a repudiation of the Groningen protocol. Lancet. 2008;371(9616):892893.

  • 19

    Kon AA. Neonatal euthanasia is unsupportable: the Groningen protocol should be abandoned. Theor Med Bioeth. 2007;28(5):453463.

  • 20

    Verhagen AA, Sol JJ, Brouwer OF, Sauer PJ. Deliberate termination of life in newborns in The Netherlands; review of all 22 reported cases between 1997 and 2004. Article in Dutch. Ned Tijdschr Geneeskd. 2005;149(4):183188.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 21

    Ramstad K, Jahnsen R, Skjeldal OH, Diseth TH. Characteristics of recurrent musculoskeletal pain in children with cerebral palsy aged 8 to 18 years. Dev Med Child Neurol. 2011;53(11):10131018.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 22

    Sällfors C, Hallberg LR, Fasth A. Well-being in children with juvenile chronic arthritis. Clin Exp Rheumatol. 2004;22(1):125130.

Metrics

All Time Past Year Past 30 Days
Abstract Views 1567 1567 134
Full Text Views 250 250 40
PDF Downloads 194 194 52
EPUB Downloads 0 0 0