The Condition’s Effects on the Child / Young Person
Effects on the individual
Cystic Fibrosis (CF) affects a person’s life in many ways and at times considerably challenges his or her health and lifestyle. People with CF may find it difficult to study or work full-time while coping with hospitalisations, fatigue and a demanding treatment regimen. Some adults choose to relinquish full-time employment to better manage their health and this may impact seriously on personal income. Despite these pressures, research has shown that people with CF are no more likely than those without the condition to suffer from depression or mental illness.
Time away from school or work is inevitable for people with CF. Many will have 2 week ‘tune-ups’ to have intravenous antibiotics and extra chest therapy. Some children and adolescents with CF do not experience periods of hospitalisation until later in life while others and adults may require frequent admissions. Being in hospital and not being with family and friends is very distressing for the person with CF. While in hospital, children strive to keep abreast of their schoolwork and parents may request that teachers provide work for the child. Aside from admissions, all people with CF require hospital based check-ups that may take a full day.
Fatigue and Endurance
A person with CF may tire more easily than their peers for numerous reasons including:
- Waking early for morning treatments – this may in itself be very tiring for someone with CF, especially if they have low lung function
- Having a chest infection – which may require longer sessions of airway clearance and more coughing. Coughing absorbs energy and can be very frustrating, especially if the person is trying to maintain quiet in a classroom or workplace.
- Low lung function – a person with CF who has low lung function will tire more easily than if they had high lung function. This is due to: burning up energy with increased breathing; having lower oxygen saturation levels; coughing more; spending more time doing airway clearance techniques; and having a lower appetite due to tiredness. Someone with low lung function may find it harder to exercise but should be encouraged to do so because of numerous benefits.
- Malnutrition – people with CF require a high energy diet of between 120-150% of the recommended daily allowance (RDA). They are encouraged to consume foods high in calories, protein, fat, salt and sometimes sugar. Meeting this requirement can be a struggle for some and many people with CF look underweight. Some may require a feeding tube to meet their dietary needs.
People with CF are encouraged to take responsibility for their health regimen from a young age. Children are encouraged to be independent with all their medications and to recognise the signs and symptoms of their condition. Some of the medication self-administered in early life may include:
- Inhaling medication such as Ventolin
- Taking enzyme capsules with food and salt tablets to prevent dehydration
- Monitoring blood sugar levels and injecting insulin for CF related diabetes (CFRD)
The length and complexity of treatment regimens varies greatly. People with lengthy and or complicated regimens may often have difficulty finding sufficient time each day for treatment.
Many people with CF are concerned about symptoms that affect their image, particularly during adolescence. Young people with reduced height and weight or delayed puberty can have a hard time. They often look younger than their peers. Worse, they may be treated as though they are younger than they actually are. Another concern is that some are tempted to skip taking enzymes to lose weight. Adolescents and adults with CF sometimes find it difficult to do the things their friends enjoy, such as joining late night parties and attending events held in smoky venues. Smoke filled rooms are particularly hazardous for people with CF. All people with CF face the difficult decision of whether and when to tell others about their condition.
Effects on those close to the child / young person
Caring for a child or adolescent with Cystic Fibrosis (CF) can be very demanding for the parents and caregivers. They facilitate their child’s health regimen and provide the education and the motivation needed for the person with CF to take responsibility for their own treatment. In addition, parents take the child to regular hospital appointments at specialist CF centres based in Melbourne. At times people with CF will be admitted to hospital for intensive treatment and the parents will support their child’s care.
Caring for a child with CF is also expensive. Families must meet the high cost associated with ensuring that their child is kept as well as possible. Ongoing expenses include medication, enzymes, vitamins and other supplements, high calorie foods, physiotherapy equipment and nebuliser pumps. Some couples find that one parent needs to relinquish their job in order to care for the needs of their child. This places an additional financial burden on the family.
A recent study has shown that the primary care-giver of a young child with CF has a one in three likelihood of suffering from depression, compared to the national average of one in five. Carers often seek the support of others who are dealing with the same or similar issues. They may also seek opportunities for respite from the daily demands of their child’s health regimen.
Research has shown that siblings of a child with CF often feel the effect of growing up with a brother or sister with a progressive condition. Some display anxiety over the future of their sibling or feel overshadowed by their brother or sister. They can become angry and may resent the attention given to their sick sibling by their parents. Yet, some siblings develop considerable independence while spending time in the care of friends or relatives when their brother or sister is in hospital. Other siblings display a greater understanding of the needs of people who are sick or disabled.
Siblings of a child with CF need to have particular attention paid to their needs and require help when they experience difficulty dealing with the condition.
“In their shoes” – stories from children / young people with the condition
Eliza, aged 16
I was born June of 1994, and I have this crappy disease. It has indeed made my life challenging. I dont go to school, I’m on homebound, due to all the sicknesses that goes around, so goodbye to socialization. I dont have many friends because people either see me as “nasty” or they just slowly leave my life. I’m in the hospital a lot more than some CF’ers. Some say its real hard just dealing with like 3 times, in twelve years. Thats nothing compared to mine, which is… 2-3 times every year. and if I get sick, its more times. I have very very poor weight gain. I just turned 16, in June & I jump from about 80-95 pounds. I have never reached 100 yet. Thats my goal, but each time I try, I get sick and I lose a good 15 pounds in just a week or two. I eat a lot, and I’ve tried just about everything with nutrition drinks. Nothing just seems to work. I get picked on more than I should about my weight, but I can’t help it. I get called “anorexic” a lot too. I am border-line diabetic. I am also, on the normal CF medications which are a total DRAG! They always interfere with things I want to do, or am in the middle of doing. I have also, almost died due to this disease. I also suffer from depression & anxiety problems. I try to have a normal life, but it is very hard to do. When I grow up, my dream is to become a nurse, and work with pediatrics and people who have CF. I hate it when people say “I know what you are going through” or “I know what you’re talking about”, when they really have no idea. This way, I can talk to kids, and tell them that “I as well have CF,and I actually do know what you go through” Well, thats my story as of now.
Eliza, aged 13
I was born in December of 1994 and was immediately diagnosed with a chronic illness called Cystic Fibrosis, or CF. As a child I never really understood what CF was, I just thought it was an annoying condition that made me cough a lot. At the age of three, my mother died, which was hard for me to cope with. Dealing with CF and mum’s death was the hardest thing I’ve ever done and my dad didn’t really know what to do. CF has effected my life more than I could ever imagine it would. I’ve been to hospital three times in twelve years, and one of those times I was very close to actually dying. Every day I have an average of twenty-six tablets and five times a day I do breathing excercises to help clear my lungs. At school, no one really understands why I cough all the time, and many people think that CF isn’t a very major or bad disease. It bothers me that there is no cure as yet, but the fact that so many people have CF helps me know that I have support. At home, doing my medications and excercises cuts into my free time a lot, and I often have to stop fun activities just to do them. Now I am learning more about my disease. I never realised it was life-threatening until earlier this year, and I never actually thought that my life was going to be so severely changed by the effects of the disease that I have learnt to hate.
Dean, aged 18
“Having CF didn’t really affect me when I was little. I was only in hospital once a year. I don’t think it starts to matter until you really understand it. For me that was when I turned twelve. I started going to high school and suddenly I was around all these different people. At primary school there were less people and everyone was kind of close to each other and I didn’t think about it much. It changed when I went to high school.
CF began to affect me seriously when I was sixteen. It was my last year at school. It was a bad year, my worst. I was getting really bad colds and lots of chest infections mainly. The antibiotics would relieve it for a while. I’d be three weeks in hospital, one week at home and then back into hospital. It was hard because I kept missing school, lots of day off, and I got behind in my school work.
The worst part about being in hospital is that it’s a totally different lifestyle. In hospital everything’s a routine whereas at home you’re pretty much free to do what you want. I found missing my family the hardest. When you’re away from them, it’s like they’re not there, you know? You get separated from normal life, from what’s going on in their lives. It’s really good when they visit. At the start of the year they were really worried about me, but later on it almost became a routine, ‘Oh, he’s back in hospital again.’ Everyone got sick of it. …”
Leesa, aged 15
“People can tell there’s something wrong with me because I cough a lot. I used to say I had asthma, but now I say it’s complicated; asthma and something else. My closest friends know I’ve got CF, but others don’t. Most just assume it’s asthma. I read that Cameron, another CF patient who died, always kept it a secret, so I’m not the only one who does. I’d like everyone to know I’ve got CF but it’s so hard to sit down and explain.”
Jason, aged 17
“Doing everything I have to for my treatment takes a lot of time each day. Too long. I get angry when I’m told to do things, especially when I don’t want to. I shut down and don’t talk to anyone. I come right after a while; time’s the only thing that brings me out again. I don’t get angry about being sick. What’s the point? It’s my way of life. The hardest thing about having CF is not being able to do things, like contact sports. I used to play a lot of sport. I loved footy before I was twelve, but I had to stop because I got a portacath put in. … I go to the gym every day and I enjoy it although I get sick of it too. I usually feel better after I’ve been. The gym instructors are okay.”
All three story extracts (above) are reproduced with permission from: Cameron, Heather (ed.), “Different but the same. Young people talk about living with serious illness.” Lothian, Port Melbourne, 1998
Maddison, aged 11
I have CF as well as both CF related Diabetes and CF related liver disease and I have to say that CF itself is not too bad. Before I was put on the transplant list after my liver failed in June 2009, I only went into hospital for a tune-up every 18-24 months. The least time I ever had between hospital visits was a year or so. My mum and I have always handled CF quite easily – it was only when I was very young that I would be too skinny – and that was only ever after returning from hospital. I’ve almost always eaten well and had a 110% lung function test, after I had learnt not to suck on the mouthpiece as a baby or toddler.
Sometimes though, I would overhear other patients complaining about CF; “I’m over this illness!” or “There are too many tablets!” or “This lung function machine is cursed! I am 90%, not 82%!”
Sometimes I would see teenagers or adolescents refusing to have their creon because they were “too full” to have two measly capsules. I would be five and have my tablets, then sit watching others refuse. The one thing I used to run away from would be a canula / drip / IV. But I got over this fear when I was- it just made it worse to be kicking and trashing.
CF really isn’t that bad and twenty-five tablets a day are nothing. I have twenty-five at breakfast, not to mention lunch, afternoon, dinner and overnight ones as well. I think too many people complain when they should just go and live life to the fullest while they can – there’s only so much you can do in 35 years.
Janelle, aged 17
This excerpt is from the transcript of ‘Background Briefing’ broadcast on ABC Radio National on Sunday 13 January 2008. In his presentation Dr Atul Gawande of the United States is discussing why some doctors produce better outcomes than other doctors. In this example, he discusses how a specialist in Cystic Fibrosis works with an adolescent to overcome her reluctance to maintain her treatment regime. For further details of Dr Gawande’s work see: www.gawande.com
“‘I could give you lots of examples of this, but I’m going to tell you about one that I found the most interesting Bell Curve, and that’s the Bell Curve for cystic fibrosis. The reason I find cystic fibrosis interesting is because it’s medicine the way we want all of medicine to work. Children with cystic fibrosis are born with a genetic disease that we have no cure for. It’s a disease, by the way, that gums up their lungs, interferes with their ability to digest food, and it is the disease they will die from some day. So we take care of those children in 117 focused CF centres and they’re high volume centres of excellence. All of the people who participate in those centres are super-specialists in the disease. They follow detailed guidelines, more detailed guidelines than we have for most of the rest of medicine, and they all participate in clinical trials. This is what we would love for everything that we do in medicine, isn’t it? And yet even they have a Bell Curve, and it’s a wide curve. The average survival for an American with cystic fibrosis in a typical centre, is 33 years. But at the best centre it’s 47 years, a 14 year difference.
And this is despite how wonderfully trained everybody is, how careful they’re controlling the quality, how much they’re trying to keep everybody on a regimen of careful guidelines. So I wanted to find out exactly why there would be this kind of variability. So what I did is, I described going to a centre, which was at the middle point actually, just below the middle, for the care of children with cystic fibrosis, and it’s a centre in Cincinnati, the University of Cincinnati Children’s Hospital. And I went there looking for trouble, problems. They were kind enough to let me in. And I’ll tell you what I found was a hospital with a great reputation. This is a place that routinely ranks in the Top 10 of American children’s hospitals. The polio vaccine, the Sabin polio vaccine was discovered there, that’s the oral polio vaccine we depend on for the global eradication of polio. The head of the Cystic Fibrosis Centre is someone who had actually developed several of the guidelines that we depend on nationally for how to treat cystic fibrosis. He had written the chapter in Nelson’s Paediatrics, which is the textbook of paediatric care for many physicians. So why, then, were they not at the top?
Well one answer could be that, well, they have a great reputation, but what’s the care really like? And so I went with the patients in clinic for a full day. And what I found was care just the way you’d want it, with attentive people, conscientious, who were very aware of the current knowledge in research in cystic fibrosis, who were devoted to their patients, and the patients were devoted to them, they felt well cared for. And yet there they were, in the middle. So then I decided what I needed to do was see a place at the top of the curve, because surely what they had at that place is a secret drug, because how else to explain why they would be having 14 year longer survival there.
Well the top place turns out to be the University of Minnesota Program in Minneapolis, run by a guy named Warren Warwick. And I went, and I found that they didn’t have a secret drug, they were following the same guidelines that everybody else was following, they were using the same treatments and they were getting different results. One explanation could be that the kids had a different genetic form of CF. But it turns out that they are not any more likely or any less likely to have the most severe form of CF, there’s one gene called the Delta-F508 mutation. Their kids are just as sick there as they are elsewhere, and yet they survive longer.
So I had a hard time trying to put my finger on what might be the difference. Until I spent a day in clinic there, and I wanted to read to you a little bit about what it was like to see a patient in clinic there, because it seemed that everything they did was the same in one sense, following the same treatment guidelines, and yet completely different.
In the clinic one afternoon, I joined Warren Warwick as he saw a 17-year-old high school senior named Janelle, who had been diagnosed with CF at the age of 6, and had been under his care ever since. She’d come for her routine 3-months check-up, she wore dyed black hair to her shoulder blades, black eyeliner, 4 ear-rings in each ear, 2 more in an eyebrow, and a stud in her tongue. Warwick was 76 years old, tall, stooped and frumpy-looking, with a well-worn tweed jacket, liver spots dotting his skin, whispey grey hair, by all appearances a doddering, mid-century academic.
He stood in front of Janelle for a moment, hands on his hips, looking her over, and then he said, ‘So Janelle, what have you been doing to make us the best CF program in the country?’ She muttered, ‘It’s not easy, you know.’ They bantered. She was doing fine. School was going well. Warwick pulled out her latest lung function measurements. There had been a slight dip. Three months earlier, Janelle had been at 109% of normal. Her lung function indicated she was actually doing better than the average child without CF. Now she was at around 90%. That was still pretty good. And I knew from looking at the numbers in Cincinnati, that some ups and downs are to be expected. But this was not the way Warwick saw the results.
He knitted his eyebrows. ‘Why did they go down?’ he asked.
‘Any cough lately?’
Was she sure she’d been taking her treatments regularly?
‘Yes, of course’.
‘Did she ever miss treatments?’
‘Sure. Everyone does once in a while.’
‘Well how often is once in a while?’
And then slowly, Warwick got a different story out of her. In the past few months it turned out she’d barely been taking her treatments at all. He pressed on.
‘Why aren’t you taking your treatments?’
He appeared neither surprised nor angry, he seemed genuinely curious, as if he’d never run across this interesting situation before.
‘I don’t know’, she said.
He kept pushing. ‘What keeps you from doing your treatments?’
‘I don’t know’.
‘Up here’. And now he was pointing at his own head. ‘What’s going on?’
‘I. Don’t. Know’, she said.
He paused for a moment. And then he turned to me, deciding to take a new tack. He said, ‘The thing about patients with cystic fibrosis is that they’re good scientists. They always experiment. We have to help them interpret what they experience as they experiment. So they stop doing their treatments, and what happens? They don’t get sick. Therefore they conclude ‘Dr Warwick is nuts’. But, let’s look at the numbers.
He’s still saying all this to me, he was completely ignoring Janelle. He went to a little blackboard he had on the wall. It appeared to be well-used. He said, ‘A person’s daily risk of getting a bad lung illness with CF is 0.5%’, and he wrote the number down.
Janelle rolled her eyes. She began tapping her foot. ‘The daily risk of getting a bad lung illness with CF plus treatment’, he said, ‘is 0.05%.’ and he wrote that number down. ‘So when you experiment, you’re looking at the difference between a 99.5% chance of staying well, and a 99.95% chance of staying well. Seems hardly any difference, right? On any given day you basically have a 100% chance of being well. But, it’s a big difference.’
He went to the board and worked on the calculations. He said, ‘Sum it up over a year, and it is the difference between an 83% chance of making it through the year, without getting sick and ending up in the hospital, and only a 16% chance.’
Now he turned to Janelle. He asked her, ‘How do you stay well all your life? How do you become a geriatric patient?’ Her foot finally stopped tapping. ‘I can’t promise you anything, I can only tell you the odds.’
And in that short speech I realised was the core of Warwick’s world view. He believed that excellence came from seeing on a daily basis the difference between being 99.5% successful, and being 99.95% successful. Many things human beings do are like that. Catching flyballs, manufacturing microchips, delivering overnight packages. Medicine’s only distinction is that lives are lost in those slim margins.
And so, he went to work on finding that margin for Janelle. Eventually he figured out that she had a new boyfriend. She had a new job, too, and was working nights after school. The boyfriend had his own apartment, and she was either there or at a friend’s house most of the time. So she rarely made it home to take her treatments. At school, new rules required her to go to the nurse for each dose of medicine during the day, so she skipped going. ‘It’s such a pain’, she said.
He learned that there were some medicines she took and some she didn’t. One she took because it was actually the only thing that she felt made a difference. She took her vitamins, too. ‘Why your vitamins?’ ‘Because they’re cool’. The rest, she ignored.
Warwick proposed a deal. Janelle would go home for a breathing treatment every day after school and get her best friend to hold her to it. She’d also keep her medications in her bag, or her pocket at school, and take them on her own. ‘The nurse won’t let me’, she said. ‘Don’t tell her’, he said. And thereby deftly turned taking care of herself, into an act of rebellion.
So far, Janelle was OK with this. But there was one other thing he said. ‘She’d have to come to the hospital for a few days of therapy to recover the lost ground.’ She stared at him. ‘Today?’ ‘Yes, today’. ‘How about tomorrow?’ ‘We failed, Janelle’, he said, ‘it’s important to acknowledge when we’ve failed.’ And with that, she began to cry.
There are a few attributes to the people who are in the institutions that are at the top of the curve, for what they do, in something like medicine. One, and you saw this in Warwick, is that they have the capacity for diligence, attention to detail. And you saw it in the way that he dove down into Janelle’s life enough to grasp not only that she was not taking her medicines, but then also why. The way the boyfriend played into her life, her job, everything else.”
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