Discussion

Parents of girls with early puberty are reported to be worried, embarrassed and concerned about their daughters’ inability to cope with early sexual development and possible peer rejection.7 It is reported that girls who mature earlier than their peers find pubertal adjustment more challenging. Those girls appear to go through various psychological difficulties and can experience detrimental sequelae.13 Stice et al reported that girls who had CPP were at 1.9 times greater risk for depression.14 In our cohort, two-thirds of the mothers of girls without a disability expressed concern that their daughters might not be able to cope with puberty changes psychologically and might face peer rejection. This concern, however, was infrequent in mothers of girls with a disability. Concerns about how their daughters would cope with the physical changes of puberty were more prevalent in girls with a disability. These concerns included painful breast development and menstrual pain which they might not be able to fully express. In girls who wear a harness in a wheelchair, breast development in puberty can cause discomfort due to chest tenderness with the harness impinging on the developing breast tissue.15 If the girl is unable to express the pain, this might not be recognised by families and may just manifest as irritability.

There is a high level of parental anxiety regarding the impact of menses, particularly when disability is severe.3 The anxiety may relate to the impact on the child’s medical condition such as on the frequency of convulsions or it may relate to the consequences of puberty. The latter mainly includes hygiene issues for girls with a disability, menstrual irregularities, premenstrual symptoms, cyclical pain (which might not be properly expressed), unwanted sexual contact or pregnancy.16 17 These issues collectively were more often raised by mothers of girls with a disability in our cohort.

Less severely disabled children may experience a deterioration in their behaviour and demonstrate socially inappropriate sexual behaviour such as public masturbation at an early age, raising family concern about the risk of sexual contact.15 Abnormal sexual behaviour was an issue of concern in a far greater proportion of mothers of girls with a disability in our study.

Increase in linear growth is a major component of puberty. It is well known that untreated children with CPP have an initial growth spurt then shorter adult height. All mothers of girls in group A raised growth as the main issue when medical advice for puberty delay was sought. Interestingly, none of the mothers with girls with a disability felt that short adult height was an issue. While final adult height might not be a concern for families with disabled children, rapid growth associated with puberty does have an impact on the family and the child with a disability. It raises issues of needing to change medical equipment, such as the provision of an adequately sized wheelchair, progression of scoliosis bracing or altering the size for chest braces or ankle-foot orthoses.15

Parent and caregivers may occasionally approach healthcare professionals requesting permanent surgical measures to abolish puberty, which raises ethical dilemmas if the patient is not able to participate fully in consent.6 All mothers in group A wanted to delay rather than stop puberty. However, 13 (48%) of group B mothers chose stopping of puberty. Surgical intervention (hysterectomy) was a measure that was considered by 7 mothers out of 13. The choice of delaying or stopping puberty did not seem to depend on the daughters’ degree of disability. However, further subanalysis was not feasible due to the relatively small number of participants. Concerns about potential treatment side effects, inconvenience or expense may contribute to why a family may consider more permanent surgical methods to avoid menstruation. Two mothers in group B chose surgical intervention with hysterectomy fearing long-term side effects of medical treatment. Use of GnRHa to treat central PP is proven to be safe and effective.8 However, these medications are relatively expensive when compared with other treatments that can be used to delay or abolish menstruation, and GnRHa are very rarely covered by medical insurance policies. In addition, it is an injectable form of medication which is often a traumatic experience and can cause sterile abscesses. In our cohort, one girl developed an abscess at the site injection which subsided and did not recur on further injections. Treatment with GnRHa requires attention to dosage and interval of injection to avoid intermittent bleeding. The initial injection had a stimulatory effect and two girls had vaginal bleeding after commencing treatment. Another girl had intermittent bleeding during the first 9 months of treatment that subsequently subsided. Overall, GnRHa was found to be an acceptable form of treatment in our group particularly with the low frequency of injection by using the depot form. However, it can be a challenge to persuade the mothers to stop the medicine after the treatment period is completed to avoid long-term side effects, especially the effect of GnRHa on bone mineral density. Although final height data suggest that the reduction in bone mineral density on treatment is reversed by the time final height is attained and peak bone density is unaffected,17 it is recommended to avoid the long-term use of puberty antagonists.11

In our region where mothers may be assisted by male family members and male helpers when undertaking care such as bathing, transferring and lifting their daughter with disability, there are significant issues and difficulties in utilising male help as their daughter matures. Involving a male helper in these daily routines involves physical exposure of the growing girl which might not be acceptable to the mother or the male helper. This stems from both cultural and religious reasons and might be a reason for seeking help to delay or abolish puberty (personal observation). In our cohort, over a quarter of mothers interviewed expressed this issue as a major concern.

Our study shows that early pubertal changes in girls with a disability are associated with major concerns. Mothers of girls with a disability seek medical help to delay puberty due to various reasons, particularly, fear of the difficulty in maintaining hygiene during menstruation. In comparison, short adult height is the main concern expressed by mothers of girls with CPP without a disability. Culture and religion have an impact on the decision-making of delaying puberty and some families would consider surgical measures to stop puberty in girls with a disability.

Limitations of our study include the use of a quantitative questionnaire methodology in a relatively small sample size, and it would be valuable to further explore the differences in a larger cohort. Another limitation is that we explored maternal attitudes through a retrospective study of girls already on treatment and the answers given may be subject to recall bias. It may be more pertinent to explore these questions prospectively. The maternal attitudes highlighted in this study reflect the social, religious and cultural norms of this geographical region and further studies in other populations would be needed to be able to generalise the results more widely.