You are on placement in the children’s emergency department when 8-year-old Noah is brought in by ambulance from school. Noah has asthma and this is his third admission to the ED in four weeks. On Noah’s last admission, his mum admitted that she hadn’t collected his prescription for the ‘brown’ inhaler as it ‘doesn’t do anything’. Noah also uses a ‘blue’ inhaler, but forgot to take it to school today. Noah’s teacher says that he often forgets his inhaler and when you check his previous ED admission card, you notice that he had not taken his inhaler to school then either.

• What are the differences between the ‘brown’ inhaler and the ‘blue’ inhaler?

Answer: The ‘brown’ inhaler is a preventative inhaler and is used to reduce the reactivity of Noah’s lungs to his asthma triggers. This in turn reduces the severity and frequency of an asthma attack. The ‘blue’ inhaler is a reliever inhaler and is used to relieve the symptoms of an asthma attack by relaxing the smooth muscle and making it easier for Noah to breathe.

• Should Noah be taking a preventative inhaler as well as a reliever?

Answer: Current guidelines produced by the British Thoracic Society and Scottish Intercollegiate Guidelines Network state that a preventative inhaler should be considered when the reliever inhaler is used more than three times a week. Noah’s repeated admissions to the emergency department suggest that his asthma requires preventative treatment to maintain control.

• Is Noah’s current asthma management a potential safeguarding concern?

Answer: Yes, because asthma can be life threatening. In not giving Noah his preventative inhaler, Noah’s mother is not following his asthma action plan and potentially placing him at risk of an asthma attack. Noah’s asthma is not being effectively managed. Although his mother may think his ‘brown’ inhaler ‘doesn’t do anything’, it is reducing the reactivity of his airways and making the chance of an asthma attack less likely. Failure to ensure Noah has his ‘blue’ inhaler with him when he goes to school means that Noah is not able to treat his symptoms should he be exposed to an asthma trigger and develop symptoms of an asthma attack. His risk of having a severe asthma attack, which could be potentially life threatening is therefore increased. Of greater concern is the fact that this is Noah’s third admission in four weeks, which may indicate that advice from the previous admissions has already been ignored.

• What health promotion could be put into place to improve control of Noah’s asthma?

Answer: Noah and his mother need to be provided with further education to ensure they fully understand the implications of not following his asthma action plan, the difference between his ‘brown’ and ‘blue’ inhaler and why they are both important. This would also be an opportunity to explore triggers, such as smoking, pets and house dust mites within the home.

The antibiotic Azithromycin is usually taken for three days and is licensed for the treatment of infections such as bronchitis, tonsillitis, ear and skin infections.

Regardless of infection status, where patients are deteriorating on conventional therapy, current guidelines recommend the consideration of a six-month trial of azithromycin, but azithromycin is not licensed to treat cystic fibrosis (Cystic Fibrosis Trust, 2009). To obtain a licence, the manufacturer must be able to demonstrate evidence for the effectiveness and safety of the medicine when used to treat a certain condition or a certain group of patients. Currently, it isn’t clear how azithromycin works to treat CF, but it is thought that it might reduce inflammation in the lungs and prevent infection by Pseudomonas aeruginosa. When azithromycin is used to treat CF, the prescriber is prescribing it ‘off label’. This means that azithromycin has been prescribed differently to the terms set out in its licence as it’s being used for a different condition and is prescribed over a longer period of time. 

The National Institute for Health and Care Excellence (NICE) reviewed the evidence for azithromycin as a treatment for CF and found that because each study researched azithromycin differently, the results could not be compared with confidence (NICE, 2014). The long-term safety of azithromycin could not be established either, although no serious adverse events had been reported.

• Why is it important to have an evidence base for treatment decisions?

Answer: An evidence base enables us to make decisions about treatment and care with confidence. In the past, healthcare professionals may have carried out care or treatment in the belief that it was in the patient’s best interests but may have been doing more harm than good.

• What are the reasons why a child/young person and their family might agree to a trial of azithromycin?

Answer: Where other medications are not being effective a family may feel that they have nothing to lose in trialling a drug for which there is currently unclear evidence as to its effectiveness. As more evidence is gathered, the effectiveness of azithromycin in treating CF may become clearer.

Using your knowledge of cystic fibrosis and its treatment regime, make a list of the ways in which living with the condition impacts on the child/young person and their family.

Answer: Daily treatment regimes such as physiotherapy and nebulisers are time consuming and may impact on family time. Families may find it difficult to be organised in the morning to get to school on time, especially if there is more than one child in the family with CF, or if there are other children who also require attention and assistance to get ready for school. Likewise, at the end of the day, time needs to be factored in for treatment alongside an evening routine.

Managing multiple medications can also be time consuming and stressful, remembering to order repeat prescriptions and which medication to give and when.

If the child is also receiving enteral feeding, this also requires management, and if the feed runs overnight, then care needs to be given to tubing and the feed pump if it should trigger an alarm, resulting in interrupted sleep. Storage space for equipment and feeds is also required.

Parents find themselves delivering nursing care as well as parenting their child. This increases the pressure on parents and although many would prefer not to be in hospital, the responsibility given to and expectations placed on parents of children with chronic health conditions cannot be ignored.

Frequent hospitalisations impact on the family, with one parent staying in hospital while the other remains at home to care for the other children. Visiting the child in hospital is important, but also impacts on the whole family.

Families often experience financial difficulties. Regular hospitalisation and hospital visits are expensive once car-parking, cost of accommodation (not all hospitals are able to provide free accommodation) and food for the parent who is staying on site is factored in. Children with CF are frequently unable to attend school due to chest infections and hospitalisation – this may make it difficult for a parent to remain in employment, further impacting on the family’s finances. In addition, through missing school, the child/young person may find it difficult to maintain friendship groups and could feel isolated.

Transition to self-care and independent living for the child (usually starts at around the age of 10), in preparation for adulthood can be a stressful time as the child starts to take responsibility for maintaining their own health and treatment regime and parents allow their child to have more control of decision making. This is an important stage however.

In the background is the constant uncertainty of living with a reduced life expectancy. This can lead to the young person taking unnecessary risks with their body/life, or with parents becoming overly protective, which may impact on quality of life for the child.

You are on placement with a cystic fibrosis nurse specialist. You and your mentor are going to visit Mia, who is 14 years old and has recently been discharged home on IV antibiotics for a chest infection. During the visit, Mia’s mother asks to talk to you and your mentor in private. She discloses that Mia has been refusing to comply with her daily physiotherapy routine and she is worried about Mia’s long-term health.

• Why is a daily physiotherapy routine important for young people with cystic fibrosis like Mia?

Answer: Daily physiotherapy helps to maintain Mia’s lung function by breaking down her lung secretions and clearing her airways. This also helps to reduce the risk of infection.

• Why might Mia refuse to comply with treatment?

Answer: Treatment is time consuming and Mia may be experiencing peer pressure to spend more time with her friends. Spending increasing amounts of time with friends instead of family is a normal part of adolescence. Mia may feel that by spending time on treatment instead of socialising, she is missing out. In addition, Mia may feel embarrassed and if she has friends over to her house, she may not want to engage in physiotherapy whilst they are present.

• What could be the impact on Mia’s long-term health?

Answer: Failure to maintain lung function and health could result in recurrent chest infections, which in turn may lead to lung scarring and a reduction in lung function. As lung scarring increases, lungs become less compliant and efficient and this in turn could shorten Mia’s life expectancy.

• What could you do to encourage Mia to comply with treatment?

Answer: It’s important that Mia is given the opportunity to take responsibility for her own health so she can live independently. You could offer Mia the opportunity to speak to a counsellor to explore the reasons behind Mia’s non-compliance, especially if she is worried about being different to her friends or is experiencing bullying. Bullying can occur if the young person is underweight, small for their age or has delayed puberty. You could also explain to Mia the importance of complying and outline the implications of not engaging in her treatment plan. This needs to be done sensitively so as not to frighten Mia or cause her to worry about any harm she may have caused to herself.