CCHS: Transitioning to Adulthood

Congratulations! You are an adult now. This means that you’ll have to handle all the medical history, details, and care that your parents originally took care of.
With good medical care and planning, a healthy, long, normal, and productive life with CCHS is possible.

Many issues need to be addressed as a young person with CCHS begins the transition to adulthood. Please consider the items below.

Suggestion: Before a move to independence work with your family and physician to organize all this information.

  • Medical condition and past history
  • Genetic data
  • Past surgeries
  • Significant test results
  • Current baseline information:
    • Sleep study
    • Echocardiogram
    • EEG
    • CXR, respiratory cultures
  • Any other associated conditions:
    • GI, seizures, heart, eye, orthopedics, Endocrine
  • Respiratory symptoms of CCHS:
    • What do I look like when I am sick?
    • Can I tell if I am getting sick?
    • Normal 02 saturation and CO2 baseline.
    • Ventilator settings
    • Tracheostomy size and type
    • Diaphragm pacer and settings
    • BiPAP and other ventilator devices
    • What treatment do I need when I have respiratory changes?

Suggestion: Identify 3-5 family or friends that will be part of your support network as you live independently.

  • Do I hear alarms and respond appropriately?
  • Should I live on my own or have a roommate?
  • Who needs to know my CCHS issues outside my family?
  • Is my future living arrangement safe?
  • What arrangement should I make to ensure that my breathing is supported if my mechanical ventilation fails?
  • Should I invest in a 24/7 emergency monitoring system?
  • Should I purchase a Medical Alert ID?

Suggestion: Plan how you will get around–to work, to events, to home–before transitioning to independence.

  • Do you have a driver’s license?
  • Will you have a car?
  • Will you need special transportation arrangements?

Suggestion: Speak realistically and honestly with your medical team about drugs, alcohol, and safe-sex.

  • Will I still need to rely on my family?
  • Does independence mean I should be reckless with:
    • Tobacco and Alcohol?
    • Recreational drugs?
    • Unprotected sex (Are you aware that unprotected sex might result in pregnancy with a 50% chance of having a CCHS baby?)

Suggestion: Before independence plan out all these issues.

  • Who manages my help health issues and appointments?
  • Who maintains my insurance?
  • Who will keep track of my Medical records?
  • Who will handle my medication refills, pharmacies, vendors, hospitals, emergency rooms?
  • Are their adult practitioners that know about CCHS in my local area?
  • Who will arrange my home nursing service if I need that?
  • What/who should I include in my ICE contact list (In Case of Emergency Numbers)?

Suggestion: Before independence plan out all these issues.

  • College
  • Financial planning
  • Household skills (Can I cook? Keep my environment clean? Can I grocery shop?)


The above information was extracted from presentation by Sheila Kun, RN, BSN, BA, MS, Nurse Care Manager, Division of Pediatric Pulmonology, Children’s Hospital Los Angeles entitled Surviving the Night: Independent Living for CCHS Young Adults.

Here is a link to the video of the presentation.