Cystinosis Parent Handbook Final - Cystinosis Research Network

Taking (and Not Taking)MedicationsFor Teens• Think about how much privacy is important to you atmedication time. (See next column.)• Find a pill case, small sack, or cool case for carryingdaily medications and eye drops that can be tucked in abackpack or pocket during social activities.• Speak honestly with your parents or your healthcareteam if you are having a hard time taking medications –whether because of side effects, a busy schedule,or sheer frustration. There may be options that couldmake things easier.For Parents• Many teens will be taking medications both to controlcystinosis and to protect a kidney transplant. The realityof getting a kidney transplant in adolescence can beoverwhelming.• When a teen’s chronic illness reaches an unstable statedue to “non-adherence with treatment recommendations”(such as not taking medications as needed), aim fordiscussion of what’s happening rather than arguing orpunishment. Decrease your and the healthcare team’sfrustration and replace it with support.• Be honest about the results of not adhering to medicationschedules, but try to avoid scare tactics. Long-termoutcomes may not make sense to teens. Focus on hownon-adherence or poor self-management can affectdaily activities such as going to school, playing sports,and learning to drive.• Talk about what the non-adherence is really about –taking meds can be a burden, it may interfere with socialactivities, the side effects may be too troublesome, etc.• Not taking medications can also be a way of “acting out,”which other teens might express with different troublesomeactivities (skipping school, drugs, alcohol). It can be a wayof expressing rebellion…or a way of trying to feel incontrol. Work together as a family and with healthcareprofessionals to find a treatment plan that works for theyoung adult individual.Maintaining Privacy WhileTaking MedicationsSome teens prefer absolute privacy while taking medications(in the nurse’s bathroom at school) while others arecomfortable being very public. Plan with school staff wheremedications will be stored, and know school systemregulations about students carrying medications.At home, try to keep medication time low-key. Keep it publicif you can – in the kitchen at breakfast or dinner. A parent’sknowledge that a dose is taken may help avoid conflict later.Coping with the Smell andSide Effects of Cystagon ®Cystagon ® is THE lifeline for individuals with cystinosis. Aschildren approach adolescence and young adulthood, it canbe tempting to skip Cystagon® due to social concerns, selfconsciousfeelings about the drug’s odor, and complicationssuch as gastrointestinal distress.Without Cystagon ® , cystinosis will take its natural course,and more complications of the disease can happen at anaccelerated rate. It may take cystine a while to build up in thesystem, so young teens and adults may not be immediatelyaware of the damage to their health. The issue of odor andways to decrease the adverse side effects of Cystagon ®should be discussed and managed, so that a young personis not tempted to skip or stop taking this vital medication.Methods used to deal with the smell of Cystagon ® include:• Vitamin B2 (Riboflavin), either isolated or as part of aVitamin B-Complex• Chlorophyll• Breath sprays and body spraysPlanning ahead and talking openly with trusted family, friends,or healthcare providers, while discussing worries and possiblesolutions, will ensure that adolescents with cystinosis donot feel the need to either withdraw socially or stop takingCystagon ® .43Cystinosis Parent Handbook