40 that allow for normal psychosocial functioning despite their physical limitations. The difference between physical severity measured by physical function and subjective severity perception measured by general health perception illustrates that patients may perceive the disorder differently from health care professionals. This is important for health care providers to acknowledge when discussing patient reported symptoms in clinical practice. Patient reported QoL should be incorporated into clinical practice to ensure the patient’s perspective is included in clinical decision-making. The mean pain in patients with OI is significantly increased compared with the control group, but between the OI types there are no significant differences. The presence of pain would imply a more-severe disease, but there is no evident association between pain and OI type in our cohort. This is comparable with observations of other studies 15,21, and has also been observed in review studies for pain in children with OI 22. Vitality in patients with OI is only slightly lower than the control group. Some studies reported diminished vitality and social functioning abilities 23 with reduced mental health and emotional functioning compared with the adult control group 17. In our study, only for patients with OI type 1 is vitality significantly lower than in the controls. This is important to know when seeing patients with OI with complaints about reduced vitality: Other possible causes should be excluded first, and reduced vitality should not immediately be assumed to be a feature of patients with OI. In our cohort, there is no significant difference in social functioning between patients with OI type 1 and type 4 after the age of 25 years. There seems to be reduced social functioning in patients with OI type 3 under the age of 25 years. This improves around 25 years of age. A possible explanation could be a transitional phase where patients are becoming independent, must handle problems themselves, and acquire better social functioning skills. The large role of caretakers in daily care, frequent health care appointments, and the effort required to stay safe 24–29 are increasingly transitioned to the adults with OI giving them more control. Also, a decline in fracture rate in adults with OI compared with children with OI can play a role. Influencing the quality of life This study provides a baseline measurement of QoL in adults with OI. It is no surprise that the overall QoL in patients with OI is significantly lower at all age ranges and in all OI types compared with the control group. However, this baseline measurement is important because it signals which components are most affected in which health domain in which OI type at what age. It does not provide an answer for the question regarding factors that influence the different health domains of
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