Comprehensive care for children with differences of sexual development

Psychology

• Infants are dependent on caregivers for everything at this age 
• Infants grow and develop rapidly (feeding, sleeping, motor & language skills)

 Considerations 

• How might your child's medical diagnosis impact development (example – medications impact sleep or feeding)

Endocrinology

• The external appearance of the child can suggest hormonal variations 

 Considerations 

• Additional lab tests may be required over time to monitor hormone function 
• Endocrinology will discuss the possibility of preserving tissue for the future

 Gynecology
 

• The external genitalia are often red and appear somewhat swollen as a result of mother’s hormones that are passed during pregnancy and still active for 1-2 years. 
• Vaginal blood spotting/discharge may occur in the first weeks of life.

Urology
 

• 6-12 wet diapers daily and 1-3 soft bowel movements daily.
• Testicles should descend by 6 months of age in boys.

 Considerations for Gynecology & Urology 

• If any concern for urinary tract infection, a catheterized specimen with microscopic urinalysis and urine culture is the best study 
• Labs, imaging, and examination under anesthesia may be considered 
• Surgery is considered for some children in this age group but is always a personalized decision based on what is best for the child

Genetics

• Has your child had genetic testing in the past? 
• How can we help you better understand your child’s differences and/or diagnosis?

Considerations 

• What is it/what types of tests can be done
• Timing and discussion of results 
• Cost/insurance coverage/value
• Risks for other/future family members

Psychology
 

• Potty-training is typical in this age range and children may pay more attention to genitals and similarities or differences in children  
• Children become more verbal (rapidly learn more words and speak in sentences); Children may repeat or share information about themselves to others  

Considerations  

• With potty-training, your child may start to ask more questions about their own genitals 
• Begin discussing with your child their medical diagnosis and treatment; Identify ways that they can actively participate in treatment (e.g., taking medication)  
• Use developmentally appropriate language to teach your child basic information about their diagnosis (e.g., You take medication to keep your body healthy)  
• What is your child’s understanding of why they attend GUIDE medical appointments?  
• Will your child be cared for by other people that need to be aware of their condition?  
• What does your family know about your child?
  
  

Endocrinology

• During this time, hormone levels in both males and females are low. The external male and female structures are not changing rapidly in appearance. 
• Children may begin to express identity for one gender or another through play or expression. Tomboyish behavior by girls is not unusual at this age. 
• Varying degrees of awareness of external genitalia may be present.
      

Gynecology

• During this time, the external female structures change in appearance, with the skin often thinning and becoming redder in appearance. It is often easier to see directly into the vagina during this time. The vagina, and uterus are inactive and are normally small.  The ovaries may have occasional small cysts but do not produce hormones during this time.  
• Toilet training may be occurring during this time and hygiene habits can lead to irritation or redness of the external genitalia. Gentle cleansing and application of protective ointments may be all that is needed.  
• Occasionally children may become aware of clitoral sensations or pain during this time .
  
  

Urology
 

• Toilet training for daytime urinary continence typically occurs between ages 2-5 years but some children do have continued accidents on occasion 
• 1-2 soft bowel movements daily without straining. Constipation can influence ability to toilet train  

Considerations for Gynecology and Urology 

• If any concern for urinary tract infection, a clean catch or catheterized specimen with microscopic urinalysis and urine culture is the best study 
• Your child may begin to ask questions about their anatomy.  We can help guide your discussions with them and use of appropriate medical terminology 
• Surgery is less likely during this time in a child’s life.  This is always an individualized decision.  Each child has a unique set of circumstances that inform the decision of the family and care team about surgery.
  
  

Genetics

• Has your child had genetic testing in the past? 
• How can we help you better understand your child’s differences and/or diagnosis? 

Considerations 

• What is it/what types of tests can be done
• Timing and discussion of results 
• Cost/insurance coverage/value
• Risks for other/future family members 

Psychology

Early elementary

• There are rapid changes in cognitive development  
• Children are attending school, learning to read, write, etc.
• Children develop closer peer relationships   

 Considerations  

• Children may be having sleepovers or changing clothes in front of peers.  
• Your child may want to share information about their diagnosis with friends.  
• Discuss what information would be appropriate to share and how your child can answer questions (e.g., “Why did you go to the doctor today?”)  

Pre-adolescence  

• Pre-adolescents develop a more mature, realistic worldview, but may struggle with impulsivity and not consider longer term implications of choices/behavior  
• Peer relationships continue to be important for pre-adolescents  
• In pre-adolescence, youth will begin to show signs of puberty (e.g., pubic hair growth, breast development, testicular enlargement)  

 Considerations  

• Consider starting conversations about early or late puberty and about fertility potential with your child.  
• Consider involving your child and seeking their input on making decisions about their treatment.  

Endocrine

• Growth and development may begin and changes in the genitalia, breast and pubic hair may begin.  Some conditions may lead to early, delayed or even absent development  
• Gender identity is usually firmly established and can be stated by your child.  
• Questions relating to the ability to have children and be a parent may be forming during this time .
• Your child may not produce some of the hormones typically produced during puberty and may need to be started on hormone supplements.
• Your child may produce some of the hormones typically produced during puberty too early and may need to be started on hormone blockers. 

Considerations  

• Your children may have questions about any differences between themselves and other children, like their siblings.  We can help you talk to your children and know how to disclose medical information to them in a way that is appropriate for their age.  

Gynecology

• Growth and changes in the genitalia, breast and pubic hair may begin. Some conditions may lead to early, delayed or even absent development  
• Often expectation relating to the ability to have children and be a parent are forming during this time  

Urology

• The majority of children are toilet trained for bowel movements and daytime urinary continence.  
• Nighttime accidents can continue to occur in some children and if child is otherwise dry rarely indicate a serious problem  
• If any concern for urinary tract infections, a clean catch or catheterized specimen with microscopic urinalysis and urine culture is the best study 

Considerations for Gynecology and Urology 

• Age appropriate discussions about anatomic differences between boys and girls, reproduction and anticipated menstruation is normal and should be encouraged as children get close to puberty. We can help you talk to your children and know how to disclose medical information to them in a way that is appropriate for their age.  
• While major surgery is less likely during this time in a child’s life, it often is the time to reevaluate the genital structures for healing and function. This is always an individualized decision. Each child has a unique set of circumstances that inform the decision of the family and care team about surgery.  

Genetics

• Has your child had genetic testing in the past? 
• How can we help you better understand your child’s differences and/or diagnosis? 

 Considerations 

• What is it/what types of tests can be done
• Timing and discussion of results
• Cost/insurance coverage/value
• Risks for other/future family members 

Psychology

• Interest in romance, sexuality, and relationships are typical in adolescence.   
• Adolescents show more concern for the future and adulthood. 
• Adolescents continue to grow in their independence as they prepare for adulthood.  

Considerations  

• Your child may be experiencing many emotions, and parents should monitor for mood changes.  
• Children’s Mercy does universal screening for suicidality starting at age 12. Our team can speak with you about how to make your home as safe as possible from a child’s impulsivity as it relates to suicidal ideations. 
• Your child may need to speak with someone about safer sex, STI prevention, or birth control. 
• Your child should be participating in making decisions about all aspects of their treatment.  

Endocrine

• For girls, puberty typically begins with breast and pubic hair development between the ages of 8 and 13.5 years.
• For boys, puberty begins with enlargement of the testes, and then pubic hair development between the ages of 9 and 14.3 years.
• Adolescents may be worried about their hormone levels and genitalia appearance and may not want to discuss this with family.  

Considerations  

• As individuals move through adolescence and gain independence it will be important for them to have a full and clear understanding of their medical issues so that as adults, they can take care of their own bodies.  We can help you know how and when to continue this process of disclosure.
• Body autonomy becomes important during this time. Adolescents may be able to independently assess their readiness for medical treatments and surgical procedures.  

Gynecology

• Average age of first menstruation is 12.5 years of age  
• Careful recording of timing, duration of flow and other symptoms is an important to assess reproductive health 
• Sexual exploration and interest are normal. Discussions about safe sex practices and sexual education are important.  We can help you know how best to have these discussions with your child, taking into account their unique circumstances. 
• Girls are commonly worried about their genitalia appearance, the ability to have romantic relations, and the ability to have children. Often, the need for privacy may prevent them from discussing this with family and friends.  

Considerations  

• Body autonomy becomes important during this time. Adolescents may be able to independently assess their readiness for surgical and medical procedures and treatments.
• Surgery is sometimes necessary or desired during this time in a child’s life for a variety of reasons (appearance of the genitalia, ability for normal menstruation, sexual development and function, reduction of cancer risk, etc.). It is important at this age the child be involved in these discussions and have an understanding about what is going on with their body and why medical treatments, including surgery, are happening.

Urology

• It is normal for boys to experience nighttime erections and ejaculation with the onset of puberty.

Considerations for Gynecology & Urology 

• Discussions about safe sex practices and sexual education are important.  We can help you know how best to have these discussions with your child, taking into account their unique circumstances. 
• Surgery is sometimes necessary or desired during this time in a child’s life for a variety of reasons (appearance of the genitalia, ability for normal menstruation, sexual development and function, reduction of cancer risk, etc.). It is important at this age the child be involved in these discussions and have an understanding about what is going on with their body and why medical treatments, including surgery, are happening.

Genetics

• Has your child had genetic testing in the past? 
• How can we help you better understand your child’s differences and/or diagnosis? 

Considerations 

• What is it/what types of tests can be done
• Timing and discussion of results 
• Cost/insurance coverage/value
• Risks for other/future family members