Why Adherence to Treatment Is Important for Cystic Fibrosis Patients

Thanks to advances in medicine, people with cystic fibrosis (CF) are living longer. However, much of the care that improves health and quality of life is also self-directed and performed at home.

What is treatment non-adherence?

The time-consuming combination of airway clearance techniques, inhaled medications, good nutrition, enzymes, and exercise, all require consistent dedication and organization. This leaves people with CF vulnerable to what is called non-adherence. Non-adherence is the medical term for partially following or not following all treatments as prescribed.

In fact, studies estimate that between 30-70% of people with CF are non-adherent in at least some portion of their treatment plan. Most people with CF take their enzymes consistently (65%-95%) but perform breathing treatments only 40-60% of the time.¹

What are some reasons for treatment non-adherence?

Non-adherence is particularly important in people with CF because good adherence is directly related to longer life and better quality of life.¹ People with CF may be non-adherent to their medical regimen for a number of complex reasons, and sometimes multiple reasons, including:

• Time constraints – They are prioritizing school, work or family obligations ahead of their health. “Not enough time” is the most common reason for skipping airway clearance.
• Denial – They don’t feel sick at the moment so they don’t think there’s a need to make time for airway clearance, for instance. “I don’t think I need it” or “It doesn’t make me feel better” are common forms of denial.
• Misplaced hopefulness or optimism – Similar to denial in that the person believes they’re not as sick as their CF care team says. A person with CF who says they can substitute exercise for airway clearance may be overly optimistic about exercise.
• Lack of education - They don’t fully understand the short-term and long-term ramifications of not following their treatment plan. This is more common in young adults with CF or parents caring for a child with CF.
• Frustration – They don’t feel comfortable talking with their CF care team about ways to reduce treatment burden or are tired of fighting with their insurance company for coverage.
• Accessibility – Their care team may recommend a drug or treatment that isn’t covered by insurance, or their hospital is far away, or they don’t have a car to pick up prescriptions refills.
• Embarrassment – Teens and young adults, in particular, may avoid their treatment regimen in an effort to appear “normal” to their peers.
• Forgetfulness – “I simply forget it” is the most common reason given for not taking enzymes with every meal and every snack.
• Hopelessness – They’re physically and emotionally tired, anxious or depressed, and don’t see the point or can’t rally the energy to do everything that needs to be done to stay healthy.²

Ways to help with treatment adherence

For friends and family

Education and encouragement are among the best ways to support someone who is skipping treatments. Teens and young adults with CF, like all people their age, tend to feel invincible. However, if they haven’t experienced an exacerbation yet, they are especially vulnerable to non-adherence.¹

Taking the time to understand the time commitment of CF treatment can also be helpful. For example, you can schedule your favorite shared activities around their treatment needs or take your medications publicly before a meal to help make their enzymes seem like no big deal.

For people living with cystic fibrosis

Some daily practices can be helpful in establishing a daily routine that incorporates treatment adherence. These practices may include setting an alarm as a reminder, keeping a daily calendar, or adding a reminder to your virtual schedule.

Being honest with friends and family about your health needs is the first step in gathering the support you need to stay as healthy as possible. If new friends don’t understand why you cough, or that you need to take breaks for breathing treatments, it may be hard for them to be supportive.

The transition between pediatric and adult care can make it hard to feel comfortable communicating with your new team. It may help to remember that it took many years for you to build the relationship you had with your old pediatric team, so it may take some time for you to trust your new adult team.

You must be your own best advocate by communicating with your CF care team if a particular drug or equipment isn’t working for your lifestyle and personal preferences. There may be other drugs you can try, or they may be able to suggest modifications and workarounds to help you overcome your barriers to self-care.