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Navigating teenage years with a congenital disorder

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When Jenica Leah’s classmates asked her what she’d been up to over the summer holidays, they received an unexpected response.

In her weeks away from school, and at the age of just 13, she’d experienced a stroke – a known complication of sickle cell disease. But, as always, she told her friends she was fine and quickly changed the subject, keen to conceal the true impact the congenital disorder was having on her health.

“I always used to play my condition down,” explains Ms Leah, remembering those tricky playground conversations of 14 years ago. “I’d say I had this condition, but it never affects me.

“I think when you’re young, you just want to be like everybody else. So I just used to lie. But I think for me 13 or 14 was the age when it hit home that actually I do have sickle cell, and actually it does affect me.”

”It hit home that actually I do have sickle cell, and actually it does affect me”

Ms Leah’s experience underscores the additional difficulties of adolescence for those with chronic conditions, particularly potentially serious ones such as haemoglobinopathies. In these disorders, the oxygen-carrying haemoglobin molecule is faulty in some way. In sickle cell disease, red blood cells are deformed and break down more quickly than healthy cells. They can also become stuck in blood vessels. The result can be pain, anaemia and an increased risk of infection.

Of the 15,000 people in England living with sickle cell, and the 1,500 with thalassaemia, a significant proportion are under 19. That means there are many adolescents out there who will have to navigate their difficult teenage years while coming to terms with their condition.

“It’s a tricky time for many reasons,” says Luhanga Musumadi, advanced nurse practitioner in haemoglobinopathies at Guy’s and St Thomas’ Foundation Trust. “There are a lot of changes going on in the individual, psychologically and physiologically. It’s also a time of leaving primary school, and going to secondary school.

”Having a chronic condition adds additional challenges, because [patients] have to come to hospital every so often, they’re missing school, and they may have difficulties with their own identities”

Luhanga Musumadi

“Having a chronic condition adds additional challenges, because [patients] have to come to hospital every so often, they’re missing school, and they may have difficulties with their own identities – some haemoglobinopathies cause delayed growth, for example, and so they may look smaller than their peers.”

Since these conditions are congenital, an adolescent with a haemoglobinopathy will have experienced regular hospital visits during childhood. But as their teens wear on, their care will transfer from paediatric to adult services. This period – in which young people become expected to take responsibility for their own condition – is a huge change among multiple other huge changes.

Ms Leah’s first direct experience with adult services came at 16, and in the form of a trip to A&E via ambulance, and then admission to a respiratory rather than haematology ward. She found it a difficult time. “I was used to my mum being able to stay overnight [at the children’s hospital, but on the adult ward] I was told it’s not the visiting times, and so your mum can’t come on the ward.

“And I wasn’t confident about speaking up. When I was in hospital at the children’s, I didn’t have to think about anything. I didn’t have to say: ‘I’m in pain, can I have some painkillers?’ I wasn’t used to that.”

According to Isabel Adams, haemoglobinopathy liaison sister at Birmingham Children’s Hospital Foundation Trust, the challenges of the move from paediatric to adult services cannot be underestimated. “The children’s hospital is what these patients have known their entire life,” she explains. “As they are usually diagnosed in the first few weeks of life, we will have predominantly worked with their parents.

”By adapting the way we work, we enable the young person to relate to their own physical and emotional wellbeing”

Isabel Adams

“If we don’t then adapt the way we teach and work with the young person, he or she may find it difficult to transition to adult care. By adapting the way we work, we enable the young person to relate to their own physical and emotional wellbeing, and allow them to develop their own skills to manage their condition.”

The value of getting this right is sufficient to have been recognised by the National Institute for Health and Care Excellence (NICE). Earlier this year, the body issued guidance on how best to support teenagers during the move from paediatric to adult services. The document argues young people are more likely to have positive outcomes if they are adequately supported to move to adult care.

Mr Musumadi agrees. “In the absence of this kind of support, young people can get disillusioned and disengage with services – especially at the point of transfer.”

He continues: “Expecting a young person who’s not been prepared to take the responsibility of negotiating a very complex healthcare system on their own, without any support, is just completely unrealistic.

“Some haemoglobinopathy patients have multiple complications. Some can have heart complications, so they’re being seen by cardiology; or they could have damage to their hip joints because of the effects of sickle, so they’re being seen by orthopaedics; and others have renal problems so are being seen by nephrology.

“Everything needs to be managed really carefully. Because if patients disengage from services, then of course they will probably turn up later on with huge complications, which could have been prevented.”

”I was just hoping I wouldn’t get sick, and wouldn’t have to deal with it”

“I’ve known people, friends, who just don’t go to appointments,” reports Ms Leah, who now keeps a blog and has written a children’s book on sickle cell in the hope of helping young people who are in the position she was once in. And while she says she was offered support to introduce her to adult services, she admits: “At that time, I didn’t want to go anyway. I was just hoping I wouldn’t get sick, and wouldn’t have to deal with it.”

At Guy’s and St Thomas’, Mr Musumadi serves as the lead nurse for adolescent transition in haemoglobinopathies. As patients go through their teenage years, he and his colleagues ensure they receive age appropriate information to help them better understand their condition and medicines.

“Transition is a process,” he says. “And that process includes them understanding their condition, how it impacts on their life, what sort of things they need to do to be able to optimise their long term health outcomes.”

But it’s also very much focused on the whole person. Staff use a structured assessment tool which covers everything from a young person’s confidence in asking questions, to how well supported they are by friends, to how they are managing at school.

Similar tools are in place at Birmingham Children’s Hospital, and a point is made of enabling young people to visit the adult services before transfer takes place. Notably, there is a specific section for transition in the patients’ notes. Ms Adams says this is a crucial part of making transition everyone’s role, and enabling all members of the multidisciplinary team to contribute. “So for example, when a young adult goes to radiology, if there’s some information that the department can impart about the adult setting, then that all contributes to preparing them for transition.”

Ms Leah is keen for all nurses and healthcare professionals, in whichever setting, to be aware of how hard the teenage years can be. “Sometimes people think you’re not trying, or you’re not trying to help yourself. I think it’s just having a little bit more understanding; just to try a bit more understanding of anything that age group is going through.”

”If we keep them well, they go to school, they become productive members of the community. If we don’t support them, they develop other complications and they become ill”

Luhanga Musumadi

It is a point with which Mr Musumadi agrees. “Sometimes as healthcare professionals we are not always mindful of the fact that a young person who has missed a clinic appointment may be attending an important lesson they couldn’t afford to miss, or trying to finish off homework, or that they have just come out of full time education and now have to pay for public transport and just don’t have the money to come to clinic. And this is generic, it’s for all adolescent patients. It’s being aware of all of those things.”

He feels that efforts at understanding are particularly worth making in the case of young people with haemoglobinopathies. “If we keep them well, they go to school, they become productive members of the community. If we don’t support them, they develop other complications and they become ill.

“And a lot of these kids are really super-talented: they’re future leaders, they’re politicians, they’re prime ministers; they’re musicians, and singers; they’re lawyers. The condition doesn’t define the individual, they’re unique individuals and they have real potential. If we don’t support them through the transition, we could easily – as a community, as a country – be depriving ourselves of the tremendous potential of young people.”

 

This article has been developed in partnership with Novartis Oncology UK, which fully funded the piece as well as suggesting the topic and the two nurse interviewees. Novartis Oncology UK reviewed the content to ensure it is scientifically accurate and compliant with appropriate regulations. Final editorial control remains with Nursing Times. 

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