Other physical effects

Heat stroke and salt depletion

Sweat is much saltier than normal in cystic fibrosis because the ability to move salt and water across membranes is impaired. This means in hot conditions a child cannot conserve salt and water and they may quickly become salt depleted and dehydrated.

Fertility

Most males with cystic fibrosis are infertile because the vas deferens, a tube that connects the sperm production area in the testicle to the penis is blocked. They can still conceive using assisted reproduction techniques to overcome this. Most females with cystic fibrosis have near normal fertility although they may have difficulty conceiving because of thick cervical mucus or the disruptive effects of chronic illness especially malnutrition, if present, on ovulation.

Summary diagram effects of cystic fibrosis on the body

Image reproduced from Wikimedia Commons (Creative Commons Attribution-ShareAlike 3.0 Unported License)

Psychological effects

Cystic fibrosis is a genetic disorder that damages many of the body’s organs and shortens life expectancy. Currently there is no cure. Consequently children and their families may need help to adjust psychologically and emotionally. Recent studies indicate that cystic fibrosis patients generally lead active age-appropriate social lives and that good information and support can reduce negative effects on families.

Treatment regimens can be time consuming, tiring and unpleasant or boring. Some young people may become depressed, and this can affect their ability to learn how to manage their condition for themselves.

Rebellion in the form of non-adherence to treatment for short periods in adolescence is common. Examples of rebellion include not taking vitamins, treatments or pancreatic enzymes or not doing chest physiotherapy. This is a common phase in growing up with cystic fibrosis for many children. Parents are advised to support and encourage but not to intervene or hover over their children, who are learning to take responsibility for their own medical management and learning the consequences of their actions. More rarely some children with cystic fibrosis display typical rebellions in relation to their cystic fibrosis treatment and have more severe behavioural problems, for example self harming and using drugs or alcohol. Intervention will be required in these cases. Good adherence is associated with better outcomes. Poor adherence is associated with adolescence and depression.

Young people carrying out much of their routine treatment for themselves will require support from their parents in a variety of ways including prompting, being there, stepping in to look after them during periods of illness, troubleshooting by liaising with primary and secondary care services, e.g. incorrect prescriptions or arranging admission to hospital.