Marten J. Poley - Erasmus Universiteit Rotterdam

General Discussion: A Guided Tour Providing Four Different Views of the Results 127related quality of life (HRQoL) of survivors of ARM and CDH from childhood toadulthood (Chapter 4). A discussion of the findings of this study is preceded hereby the following point, which is that it appeared essential to include various typesof outcome measures to gain a full understanding of the long-term outcomes ofneonatal surgical diseases—a point equally relevant to the researcher (see furtherSection 8.5). Each type can capture patient outcomes that the others cannotreveal. A first measure of health includes biological and physiological factors,which focus on the function of cells, organs, and organ systems. Then, anindividual may or may not have the perception of an abnormal physical,emotional, or cognitive state (which can be termed 'symptoms'). On anotherlevel, there may occur problems in body function or structure, such as loss ofvision or loss of a leg. Then, one may want to study physical, social, or emotionalfunctional status, that is, the ability of the individual to perform particular definedtasks. Finally, to estimate the significance to an individual of impairments orlimitations in functioning, HRQoL measures should be used—containing physical,mental, and social domains. To convey the full effect of the disease from theperspective of the patient, or the parent on his or her behalf, measuring HRQoLshould be an important component of long-term follow-up. The research by VanDeurloo from the Netherlands on esophageal atresia is a noteworthy example ofanother recent study in neonatal surgery that differentiated between a broadvariety of outcome measures. 1 The outcomes studied included esophagealfunction, gastrointestinal symptoms such as dysphagia or gastroesophagealreflux, limitations in daily life functioning, and generic HRQoL.Our examination in patients with surgically corrected ARM using different outcomemeasures produced a mixed picture. The patients appeared to retain substantialresidual symptomatology. Their HRQoL was somewhat lower than that of thegeneral population, especially for the youngest patients. Nonetheless, theirHRQoL improved considerably with age, beyond the level that we expected apriori. This finding was essentially confirmed in a recent Dutch nationwide studyin adult patients with ARM that used the same generic HRQoL questionnaire(SF-36) and arrived at largely the same HRQoL scores. 2 A couple of other recentHRQoL studies in patients with ARM did not include a comparison with a referencegroup, but studied the effect of new treatment modalities on HRQoL. The resultssuggest that individualized biofeedback training 3 and artificial bowel sphincterimplantation or the gracilis neosphincter procedure 4 can improve patients' HRQoL.These are relevant findings, and more such studies should be carried out with theaim of optimizing the management of this patient group. After all, as this thesisshowed, there is room for seeking to improve the HRQoL of especially theyoungest patients with ARM.From a comparison with the general population we found that the patients withCDH experienced disease-specific symptoms such as respiratory difficulties andstomach aches. However, the HRQoL of adolescent and adult CDH survivors couldhardly be distinguished from that of the general population. It is interesting to