Transition to Independence

Growing up is hard—for everyone. It’s a long process marked by challenging situations, powerful emotions, and changes in the brain and body.

If you are living with cystinosis, growing up can be even more difficult because there are so many health issues to manage. But there is hope! Growing up is all about learning how to take care of yourself. For caregivers, it’s about teaching the child to take responsibility for his or her life and stepping back as he or she grows into an independent adult.

The following tips may help people living with cystinosis and their caregivers handle the transition to independence.

Transitioning tips for individuals with cystinosis

  • Learn about your disease. Learn what cystinosis is and what it does to your body. Learn about cystine and why it’s so important to keep it under control—not just today, but throughout your lifetime. You should understand the disease well and be able to explain it to others in simple terms.1
  • Learn about your medications and dosing schedules. This is one of most important things you can do as a young person with cystinosis. As a child or young teenager, you may have gotten used to your parent or caregiver telling you when you needed to take your medications. But as you get older, you’ll need to learn to take your medications on your own, consistently and on time. There are tricks to help you do this successfully, such as setting alarms or creating a medication routine.1
  • Learn about medication side effects. Some of the medications you may take as part of your cystinosis treatment plan may have side effects. You should learn what to do to make side effects less uncomfortable or stressful. For example, body lotions and sprays, gum, and mouthwash may help with the bad breath and body odor that cysteamine might cause.1
  • Learn to advocate for yourself and your needs. When you’re young, a parent or caregiver should accompany you to your medical appointments. As you get older, your parent or caregiver can step back and let you take a larger role in managing your own health. When you know about your disease, your medications, and your treatment routine, you’ll be better able to explain what you need—especially to people who don’t know much about cystinosis. If you ever find yourself in a situation where you need to ask for help, don’t hesitate to do so. It’s OK to stand up for yourself and your needs!1
  • Take responsibility for your own care. As a person with cystinosis, you will probably find that taking care of yourself involves frequent interactions with doctors and other healthcare providers. Get to know your healthcare team, including their contact information, and be familiar with your emergency care plan. Learn how to make—and keep—appointments, including those for white blood cell cystine testing. You may encounter doctors and other healthcare professionals who don’t know as much as you know about cystinosis. For example, a nutritionist may have a lot of experience with people who have had a kidney transplant, but maybe you’re the first person she’s meeting who has cystinosis. In these cases, you might educate them about cystinosis while they help you with their specialty.1
  • Build and maintain social support. Friendship is an important part of everyone’s life. If you have cystinosis, a strong network of friends and family can help you relieve stress and stay connected, especially during those times when you’re focused on your health. Learn about cystinosis patient advocacy programs—like those run by the Cystinosis Foundation (CF), the Cystinosis Research Foundation (CRF), and the Cystinosis Research Network (CRN)—and make regular use of their support services. Visit the Resources & Support section to learn more about patient advocacy groups.2

 

Transitioning tips for parents and caregivers

  • Educate and assist, but leave room for personal responsibility. It’s normal to want to do as much as we can for our children and those under our care—especially when those children have an illness like cystinosis. But eventually, doing too much for children becomes detrimental to their growth as individuals. Therefore, as children with cystinosis get older, it’s important to help them learn to take care of themselves. Teach them everything you can about managing the disease—and then let them start learning for themselves. It’s never too early to start transitioning.2
  • Help children and teens take control of their treatment routine. In order to successfully control cystine over their lifetime—which is the only way to limit the damage caused by cystinosis—children and teens with cystinosis must learn how and when to take their medications.1,3-7 “Scare tactics” should be avoided; instead you might explain to children and teens that not taking their medicine today can make life much more unpleasant in the future. Help them build good habits like setting alarms or developing individual routines that can be repeated every day.2
  • Teach children and teens how to call their doctors’ offices. It’s important for everyone to learn how to make and keep their own appointments, but this is especially true when a teen’s health is at stake. Once your teen is old enough, help them learn how to call the pharmacy, hospital, or doctors’ offices to schedule visits—and teach them how important it is to follow through with their appointments.2
  • Seek a balanced approach. Yes, children, teens, and young adults with cystinosis will always have special needs that require close attention. But they will also encounter a wide range of very normal experiences—from trials and successes at school, to first love, to learning what it is they’d like out of life. Encourage them in their passions and balance these “normal” parts of life with the unique healthcare requirements of the disease.2

For a much closer look at the transition from childhood to adulthood, including more advice for people with cystinosis and their caregivers, download the Transitions brochure, created by the Cystinosis Research Network.

References

  1. Cystinosis Transitioning Guide, Cystinosis Research Network website. Available at https://cystinosis.org/research/article-library/transition. Accessed August 20, 2015.
  2. Cystinosis Parent Handbook, Cystinosis Research Network website. Available at https://cystinosis.org/images/research/article-library/overview/Cystinosis-Parent-Handbook-Final.pdf. Accessed August 20, 2015.
  3. Brodin-Sartorius A, Tète M-J, Niaudet P, et al. Cysteamine therapy delays the progression of nephropathic cystinosis in late adolescents and adults. Kidney Int. 2012;81:179-189.
  4. Gahl WA, Thoene JG, Schneider JA. Cystinosis. N Engl J Med. 2002;347(2):111-121.
  5. Gahl WA, Balog JZ, Kleta R. Nephropathic cystinosis in adults: Natural history and effects of oral cysteamine therapy. Ann Intern Med. 2007;147:242-250.
  6. Levtchenko EN, van Dael CM, de Graaf-Hess AC, et al. Strict cysteamine dose regimen is required to prevent nocturnal cystine accumulation in cystinosis. Pediatr Nephrol. 2006;21:110-113.
  7. Nesterova G, Gahl WA. Cystinosis: The evolution of a treatable disease. Pediatr Nephrol. 2013;28:51-59.