Denise Hofman, Post Graduate Research Student, School of Psychology, Faculty of Medicine and Health, University of Leeds.
Let me first briefly introduce myself. I’m a PKU patient from the Netherlands, currently completing my PhD at the University of Leeds, in the UK. In my research, I’m interested in adherence of adult PKU patients to the dietary management of PKU, looking at factors influencing adherence as well as the effects of varying levels of adherence on different aspects of patients’ health.
The following blog taps into a mix of peer-reviewed studies, my own experiences and experiences of other patients and their families who I have been fortunate enough to meet and work with during my studies.
"The burden of living with a chronic metabolic disorder may well contribute to mental health issues in PKU"
The burden of living with a chronic metabolic disorder may well contribute to mental health issues in PKU, which can include stress and anxiety about the dietary management and the impact of lapses in adherence to the diet, as well as social withdrawal and, in more severe cases, depression. However, how much PKU affects the mental health of patients and their caregivers is very dependent on mind-set and, probably most importantly, support from others. Personally, I think the medical team can play an important supportive role here, my own paediatrician and especially dietitian certainly did!
Early years
Caregivers of children with PKU often experience stress and anxiety in relation to the diet of their little PKUers1: the diet requires constant planning and involves a lot of food prep2 and as a result caregivers tend to find it’s not easy (but not impossible either) to go on holiday or spontaneously do things with friends and family 3.
This sometimes leads to social withdrawal. Furthermore, it can be worrying when a child refuses to take their protein substitutes or eat something high in protein. Caregivers worry about the impact of missing substitutes and/or high phenylalanine levels and, at the same time, don’t want their kids to feel left out.
Growing up with PKU
Which brings me to PKU patients. Every child, adolescent and (young) adult wants to fit in with their peers. Having PKU obviously doesn’t help and patients often feel embarrassed about eating differently and taking their protein substitutes in front of others. In turn, this could result in low self-esteem and patients tend to either avoid social situations4 or, in order to fit in, stop regularly taking their protein substitutes and eat foods that are high in protein in social situations5, like their friends and colleagues do.
Adherence to dietary treatment often decreases during adolescence2,5 and the resulting increase in phenylalanine levels, and consequent decrease in dopamine and serotonin levels, can negatively affect mood and result in depression and anxiety6.
Pregnancy in PKU
As patients grow older, they might start thinking about having children of their own. To avoid maternal PKU syndrome, women are advised to follow a strict(er than usual) pre-conception diet to lower phenylalanine levels before even attempting to conceive.
Upon conception, phenylalanine levels are closely monitored throughout pregnancy. Because of the possible negative impact of (too) high phenylalanine levels on the health of their unborn child, pregnancy in PKU often goes hand in hand with elevated levels of anxiety7. Furthermore, young mums with PKU have reported to struggle to stick to their own diet whilst taking care of a new-born 3.
Focussing on negative things in life is human, but doesn’t do anyone any favours
The best advice my dietitian gave my parents when I was diagnosed was: “don’t focus on the things you can’t do, but instead, focus on the things you can do”.
It’s not impossible to go on holiday or have an active social life. Sure, it may take a bit of extra planning, but due to early diagnosis and dietary management of PKU, and with the right attitude and support, there is no reason at all why those with PKU can’t do and be anything they want!
"Don’t focus on the things you can’t do, but instead, focus on the things you can do"
Don’t focus on the things you can’t do, but instead, focus on the things you can do. The best advice my dietitian gave my parents when I was diagnosed was: “don’t focus on the things you can’t do, but instead, focus on the things you can do”.
It’s not impossible to go on holiday or have an active social life. Sure, it may take a bit of extra planning, but due to early diagnosis and dietary management of PKU, and with the right attitude and support, there is no reason at all why those with PKU can’t do and be anything they want!
References