Research indicates that although cystic fibrosis (CF) has a high treatment and psychological burden, most people are very resilient, having a good quality of life and are able to develop benefits from their CF related experiences. This article discusses some of the developmental struggles and some useful strategies to navigate through them.
Newborn
The complexity of CF and its treatment often means that it becomes a family diagnosis; it effects the whole family and how the family manage this diagnosis will impact on treatment and health outcomes.
Many parents often experience sadness and guilt due to the hereditary component of CF. If unresolved, parental distress can have negative impacts on attachment. Elevated levels of anxiety are frequently accompanied by hypervigilance and parents may misinterpret normal newborn behaviours as CF symptoms.
Confirmation of diagnosis can be a relief as CF is then a “manageable disease”, however many parents have fluctuating moments of psychological distress often termed “chronic sorrow or chronic grief.” This psychological distress can be ongoing and unresolved, easily triggered by medical interventions, hospitalisation or even the expectations of treatment. Feelings of anxiety at challenging moments are normal, and most of the time, manageable, however when they persist, can have significant impacts on both the child and parent’s mental health.
Strategies:
Developmentally appropriate routines are essential around general daily activities that incorporate CF-specific treatment tasks. This will promote a positive relationship between the parent and child and increase the likelihood of better adherence later on. CFWA can provide home care worker support to assist with good routines.
Other useful links:
- CFWA- Newborns
- http://raisingchildren.net.au/behaviour/newborns_behaviour.html
- http://www.ngala.com.au/
- https://www.carerswa.asn.au/
Early Childhood
Language and cognitive development help the child to process and communicate their personal experience of CF. Children start to make assessments about what they can control in their environment, however for the child with CF there can be a number of stressful situations that they can’t control e.g. medical treatments and interventions. Extreme behavioural reactions could occur at this age in an attempt to avoid or escape particular procedures. Such situations could result in trauma or aversions for future medical procedures if not handled well.
These children are learning about behavioural/emotional self-regulation whilst taking on CF related tasks. Parents try to develop strategies to enhance compliance, whilst children continue to test “limits”. Conflict is not uncommon with parents trying various strategies; sometimes either a more authoritative approach in an attempt to get their child to do essential health related treatments or a more permissive/over protective approach, only doing minimal treatments or choosing the battles.
Mealtime behavioural issues are noted as being more problematic than in non CF families, with reportedly less than a quarter of children reaching recommended dietary requirements. Nutritional intake has direct correlations to lung function and overall health and can be an ongoing battle throughout the life span. Children with CF may demonstrate behaviours that interfere with eating such as delaying meals by talking, leaving the table, crying and whinging. To counteract this behaviour, parents may use increased coercion, commands, and physical prompts, sometimes feeling that they have little control over their child’s eating.
Strategies:
Behaviour modification can alter future maladaptive issues around food. Behavioural techniques include the praising of desired eating, ignoring noneating behaviours, setting time limits for meals, and use of goal setting and reinforcement of goal setting. Speak with PCH treatment team, dietician and or psychologist
Useful supports:
- CFWA- Children
- CFFood Nutrition and Cystic Fibrosis: A Guide to Feeding Infants or Nutrition and Cystic Fibrosis: A Guide to Feeding Children.
School Age
Peer relationships become increasingly important at this stage. For some children, going to school can bring about a second diagnostic period where they realise at a deeper level the impact of having CF and being different to their peers. There can be a high cost in appearing to be “normal” through minimising the more visible aspects of CF. Keeping CF a secret can have a negative impact on the ability to maintain a supportive peer network. Children at this age can develop anxiety and or depressive symptoms, particularly if parental distress is not managed.
Strategies:
Age appropriate and accurate information about CF is important to assist the child to understand their illness, the relationship between CF and their daily treatments and medical procedures. Coping strategies about CF related experiences are also very important e.g. distraction and pain management if undergoing a medical procedure.
- CFWA- Children
- Procedural Anxiety factsheet
- Rozee (Kids magazines)
- https://www.dcp.wa.gov.au/CrisisAndEmergency/Pages/Familyandparentinghelplines.aspx
Adolescence
Adolescence is typically a challenging phase with proportional time spent between peers and family; peers often becoming more influential. For some teens, CF symptoms such as cough and fatigue can worsen, especially for females. This effectively means more reliance on family and at times hospital rather than with friends where they would more often rather be.
On the up side there are some positive associations with having CF such as increased social skills, assertiveness and a higher degree of negotiation skills due to regular discussions with adults about health treatment and the need to manage treatment.
Several barriers to adherence have been identified such as a lack of personal knowledge about CF and their treatment plan, disagreement with treatment plans, complexity of treatment, psychological issues (child/parent or both), “forgetting”, time management, skills to manage routines and low level of connection to disease symptoms. Research indicates that adherence to treatment plans could be as low as 50%, particularly if the teen or their parent is struggling with anxiety or depression.
Strategies:
Teens need to start owning their treatment and setting individual goal plans with their treatment team, home care worker and or parent. Research has shown that teens who believe that their treatments are necessary are more motivated to do them. Self-management generally increases at this age, however supervision by an adult still correlates to better adherence. Motivational interviewing or health coaching can be a useful strategy to help set some health goals and break down barriers to treatment. CFWA can assist with this.
Typical adolescent autonomy is developed by being able to take a few risks and learn the consequences. This has bigger implications for a teen with CF and parents often need to stay in a supervisory role much longer than teens without CF, but need to shift their role into a more collaborative one where their teen can start managing one small aspect at a time. This may be tied to natural consequences of more freedom in other areas. Useful resources for teens:
- CFWA- Young People
- http://headspace.org.au/headspace-centres/ (including regional)
- http://youthfocus.com.au/
- https://www.youthbeyondblue.com/
Second Diagnosis
CF related diabetes (CFRD) and CF related bone disease (CFRBD) are not uncommon secondary diagnoses on top of having regular CF symptoms. Getting a second diagnosis such as CFRD can be considered a medical milestone associated with worsening symptoms of CF. This can occur during late adolescence and affects many adults. A new diagnosis can trigger a lot of anxiety, particularly if CF symptoms have worsened. Some adults find that they have to take a closer look at their management strategies and perhaps cut back on some activities, such as work. If people are already managing CF well, it is generally easier to manage the requirements of a second diagnosis.
Follow these links to get more information on how to manage CFRD and bone health:
- CFWA CFRD factsheet
- CFWA Bone Health factsheet
- https://www.cysticfibrosis.org.uk/life-with-cystic-fibrosis/publications/factsheets
CF-Related Pain
CF related pain is not uncommon for both children and adults, particularly when unwell. Pain can significantly impact quality of life and willingness to do airway clearance techniques, affecting health outcomes. Anticipatory fear affects approximately 80% of both children and adults when facing particular procedures such as insertion of intravenous therapy. The following links may help with strategies for managing pain and anxiety:
Weight and Nutrition
With each developmental stage comes a readjustment in both perspective and treatment. Dietary requirements are one significant factor that can constantly need adjustment, particularly at times of illness where higher energy requirements are necessary. Studies indicate that approximately 12-18% of people with CF have issues around food and weight; including the omitting of enzymes, insulin, overusing laxatives, not complying with supplements, over exercise and food restricting. Low weight has serious implications for overall health; including lung function, the ability to fight infection and bone health. Seek advice from your dietician and set goals for your ideal weight
Useful resources and links:
- http://95.138.169.194/search.php?sid=8eb4382926b9a478280f4d67526a55b6 (CF Trust Forum)
- CFFood – Booklets
Article from RED Magazine, Edition 3, 2016.