Transcript: Coordinating care across the child-to-adult transition
Eric Legius, MD; Rosalie Ferner, MD; Mandy Myers
All transcripts are created from interview footage and directly reflect the content of the interview at the time. The content is that of the speaker and is not adjusted by Medthority.
- [Eric] Hello, this is the "Unpacking NF1 care - Expert voices" podcast series. My name is Eric Legius. I'm a emeritus professor at University of Leuven in Belgium, and I want to introduce the two guests of today, Professor Rosalie Ferner, a neurologist from the Guy's and St. Thomas Hospital in London, UK, who has a very long experience in the care for individuals with neurofibromatosis. And then we have Mrs. Myers, a nurse expert, also working at Guy's and St. Thomas Hospital in London, and she is heavily involved in individuals both children and adults with neurofibromatosis type one. So what first got you thinking about transition care in neurofibromatosis type one? Was there a particular moment in time that made it feel important and appropriate?
- [Rosalie] Well, that's an interesting question, because as you've said, I've had quite a long experience of looking after patients with neurofibromatosis one, but I'd have to say back at the end of last century, I was looking after a lady who was in her mid-30s and she always came to the clinic with her mother. And every time I made a question about healthcare or suggested an intervention, it would be this patient's mother that would intervene and answer. And I tried the usual tactics of looking straight into the patient's eyes of turning my chair, but it was to no avail. And this experience really said to me that transition care from pediatric to adulthood is a very important part of looking after NF1 and that really we needed to start this early so that people had agency and independence. So that's really how I started to think about it.
- [Eric] Thank you Professor Ferner for this experience from a long time ago, I suggest. But now, from a pediatric perspective, what are the most significant challenges patients with NF1 face as they approach transition to adult services?
- [Rosalie] Well, I think we'd all agree that the process of adolescence is a challenging experience and even more so for a young person with NF1 as they gradually seek to gain independence from their families. So I think if we think about NF1, we know that it's a condition that predominantly affects the skin, the nervous system, the eye and the bone. And we know that it also has a number of complications, sort of very varied. And if I think about adolescence and complications, the one that really comes to mind, the cognitive issues because they can have a significant impact on the adolescent. If they have literacy problems, then that might affect them doing their exams. We know that the frontal lobe is impacted in NF1, and that might stop them from planning their future efficiently and also for advocating for themselves. So I feel that that's the overriding thing that one has to think about when one thinks about these challenges. If we come onto skin sometime during adolescence, the cutaneous neurofibromas might begin to be visible causing itching and of course, cosmetic issues. The plexiform neurofibromas, well, they're the benign peripheral nerve sheath tumors and I think it's generally agreed that most of them are congenital, but you and I know that you can see some growth during adolescence, maybe contributing to pain, disfigurement functional impairment. So it's quite a heavy burden for them. Of course, in the background is this lifetime risk of around 16% over a lifetime of malignancy of these lesions turning malignant. I mentioned the eye and optic pathway gliomas are usually detected and cause their problems in early childhoods, but I'm sure we've both seen adolescents who have the consequences of these optic pathway gliomas so that they've got visual difficulties and they really struggle with trying to go onto the next stage of their life independently. And then we want to think about bone, which is really central in NF1, because it causes us displays of the long bones and the skeleton and even dental caries. And so, if you've got a growth spurt, it may mean that you get a worsening of the dystrophic scoliosis and you're contemplating surgery at this difficult time. Or if you've had surgery for a long bone dysplasia called pseudarthrosis, you may need to have a revision of a prosthesis or an orthosis. And of course, dental health is great importance as they get away from their parents' care. So yeah, I think those are the main challenges before we come onto surveillance,
- [Eric] Thank you very much. Now, I want to turn to Mrs. Myers. From your experience as a nurse working closely with patients and families, how do these challenges present in practice for adolescents with NF1 during the period of transition?
- [Mandy] Well, I think it's like any other chronic condition or lifetime condition. Young people just want to be like their peers. So we find that, you know, they don't necessarily want to come to hospital for their appointments. We know in NF1, they experience more pain, stress, anxiety, and this can lower their self-esteem. And as Rosalie mentioned, they can have visible difference. The cutaneous neurofibromas may become more visible and this can lead to loneliness. And to remember, it's not just a time to transition in health, but you may transition in education, be going from school to college or to university, and relationships may be transitioning. So it's a time of big change not only for the young person but also for their parent or carer. They've built up the trust in coming to one hospital and they may need to change locations. For us in the national center at Guy's, we are very lucky that our adult service is based in the same location as our pediatric service. So that transition is slightly smoother, but it would be a value for people to become familiar with where they need to go for their adult service. As you would view a nursery or as you would transition from a primary school to a secondary school setting, you would have a transition day and it should be just the same for health. So one of the things that we've attempted to do as part of our transition processes offer a teenage day where we bring young people together in our MediCinema at the hospital, so it's a familiar location. And for a lot of our young people, they haven't met anybody else with NF1, and this gives them opportunity so they don't feel alone. We provide some simple education, we introduce them to members of our multidisciplinary team. For example, our physiotherapist will get them up moving, talking about general health. We offer pizza and a movie so that they can begin to talk to each other in a social setting. And you know, what we've seen from these days is they will turn to the person next to them, be comparing their cafe au laits or talk about the complications and their experience of bullying. So hopefully they don't feel alone. One of the feedbacks that we've had from this session is they would like some drama. So we've got a local charity to offer a short drama session to help them feel engaged. From these sessions, it's fostered not only friendships with the young people but also with their parents. So parents struggle with transition as I said, because they've built up that trust. And I think also for us as health professionals, we may have known these young people for a long period of time and it's about giving them knowledge and power so that they feel that they can move on. And one of the things that we offer is to sit in as a pediatric service with their first adult appointment so that that transition can be done more smoothly and those new trusting relationships can be built.
- [Eric] Thank you very much for explaining the difficult period of transition for individuals with NF1. But Professor Ferner, what components define a strong, well-coordinated transition plan for people with NF1 starting from the pediatric services?
- [Rosalie] Well, I think the first thing to say is that we usually start about the age of 14 years, but it does of course depend on the maturity of the individual young person. WHO says that an adolescent is anyone between the ages of 10 to 19 years. So it's quite a large scope. I think the main things to bear in mind are good planning as evidence by the anecdote I told you right at the beginning and good communication. The young person should really be at the center of the process. But again, referring back to the anecdote, we really need to give support to the parent or the carer as their role gradually changes over time and the young person gets more agency. And then our aim really is to address medical needs, psychological needs, educational and social needs. So it isn't all just about the physical side. Mandy's mentioned about how the trust and familiarity build up over years of pediatric care. And so, as clinicians we really need to identify the new team and liaise with them at an early stage using our nurse specialist to meet with them if the lead clinician is not able to. I mean, we are fortunate that we've gradually built up our service here so that pediatric and adult patients are seen in the same department, removing some of the strangeness for the young people. But we learned over time because initially, we set it up as both the adult neurologist and the pediatric neurologist seeing the patient together, and then we'd have ad-hoc consultations with the physiotherapist or the psychologist or the nurse. But over time, that proved not to be optimum care and certainly not cost effective. And that is why we moved over to the nurse model of nurturing the young people through this difficult time. So the keys are planning and communication at the very basis.
- [Eric] Thank you very much. Now, for Mrs. Myers, how do especially nurses support adolescent with NF1 in developing the confidence skills and autonomy needed to manage their condition in adulthood?
- [Mandy] I think it's very much that education is empowering and it was a disservice to be having a 10-minute corridor chat with a young person and their family to be able to transition them. And as nurses, we play an integral role to support that independence, provide the young person with autonomy and confidence. So it is very much a process. And as a result of this, we've set up a nurse-led transition clinic. One of the first things that we like to establish in that is what does the young person know about their NF1? We find that some families have provided very limited information on their diagnosis. So that is our starting point to build on the information that is a genetic condition. Some of the signs and symptoms of NF1 and how they can monitor their own health and lead to self-management in the future. For example, having annual blood pressure, vision checks, and to be checking their skin. It's also about looking at things holistically and we look at health promotion including contraception to enable them to have more choices, which can lead to genetic counseling if they choose to have their own children. But also general advice about smoking, drinking, helping with sort of maintaining a healthy blood pressure. We also look at their aims for the future and if we can support with letters for education, make sure they're receiving appropriate benefits and we have the support of a multidisciplinary team, including a social worker to help with this. We try and set goals within our clinic appointment, and this often looks like a a five-minute time where the young person will sit in the room alone with us, with the parent or carer outside just to give them some practice in becoming more independent in their adult consultations. As Rosalie mentioned, some of our young people will have intellectual disabilities or learning disabilities and need more support. And for this, we have a healthcare passport. So this is a document that we can help fill in and they can then take to any healthcare setting and provide that for a new healthcare provider. If for example, they were admitted to an adult ward without their parent or carer, we encourage using electronic systems so they can monitor when their next appointment will be, get copies of their clinic letters. And we're lucky because we are in the same environment as our adult service. We try to introduce them to the adult nurse specialist, physiotherapist, social worker, psychologist, so they can begin to build up those trusting relationships. But no one model fits all. It's about providing education through written information, pictorial information, and reiterating this when they get to their first adult appointment.
- [Eric] Thank you. That was a whole set of skills. Thank you very much. Now, Professor Ferner, what helps ensure continuity of care across specialties as patients with NF1 transition from pediatric to adult services? - [Rosalie] Well this really is a thorny issue in cash-strapped health services across Europe. So once again, planning, communication and a degree of flexibility is very important. So really, at the basis, what you need is the lead clinician to have a very detailed summary of the patient's care and to ensure that any imaging and any important investigations are forwarded to the new team. Ideally, this should not just be by letter but by personal approach. And as I've said to many students, my main skill is to have a very large phone book. And I think talking to the clinician who's taking over is really important, in person, by phone or with modern technology, by Zoom or MS Teams. So we have the lead clinician, but we also have a very large multidisciplinary team that work across both pediatrics and adult services in our NF group. So that would have neurologists, geneticists, psychiatrists, pain specialists, and surgeons, as well as an ophthalmologist. So all of those individuals will either see both children and adults or they need to then hand on in a cogent manner. And again, this is where people like Mandy, the specialist nurse, come into their own, because not only does the lead clinician hand on, but the nurse can play a very useful coordinating role with all those other specialists that are involved. I think the multidisciplinary team meeting is a very useful way of passing on helpful information because these days, as you and I know people can join from different regions and different countries giving their expertise and helping ensure continuity of care. In England, there is an electronic system that's being rolled out across the country. And the aim, although it's still a way to go, is that every hospital should be able to be connected, getting fast information, clinic letters, scans, investigations and protocols. And there is a patient arm, they can log in on their phones and see when their appointments are, or some people are able to contact, for instance, their specialist nurse. Of course, Mandy mentioned this before, not one size fits all. And you need to be flexible and having other ways like simple phone or letter to contact people who are not comfortable with the new technology. So I think the message is to use a broad variety of skills to ensure continuity.
- [Eric] Thank you very much. This is a quite complex issue. And now, as a final question to both of you looking ahead. What service models or approaches could most improve the transition experience and the long-term outcomes for people with NF1?
- [Mandy] I think for me, it's very much, it should be a process. So it should start early rather than you get to 16 or 18 and you end your pediatric care and the next appointment is suddenly in a different environment with an adult clinician. So it's something that needs to be talked about openly as young people move through their NF1 pathway. It relies on good communication and building up those relationships with clinicians, nurses, allied health professionals in other hospitals or even within your own service. And the use of MS Teams can aid this that you may be able to join a transition appointment initially to relay some of that information as we do, for example, with schools and with colleges. And I think it's providing that ongoing knowledge and to be aware that not everything will be taken in on the first contact, but the need to continue to reiterate that, and it's very much about working together and being able to support them at what can be a very, very stressful and anxiety provoking time, so they have the trust in their ongoing service.
- [Rosalie] So thinking about this with Mandy, we feel that our model of seeing children and adults in the same service is a very helpful model because it allows you to understand the natural history of the disease from birth to old age, and it removes some of the traumas of trying to find a new team. But I think there are ways of getting people involved in your service without having this exact model. It took us 10 years to build our transition service. So I think it's not something that one builds overnight. Regional, national, international meetings are a fertile ground for meeting people that can help you and participate. And I think one should not underestimate the role that the patient organizations can play in signposting and advocating for young people and providing links to specialist clinicians. It is a difficult problem. It is difficult to set up these transition healthcare systems, but I think that we feel optimistic that this is the case where new technology really can help us. And I think just to say that it really behoves us as clinicians to lay the foundations of good health during adolescence so we can secure the future of these young people and really aim to give them the optimum quality of life.
- [Eric] Thank you very much and I think we now have the basis, so that every hospital and every team can develop a transitioning system in their own setting. And we are at the end of this podcast now, and we have discussed many aspects of the transition of care in adolescence with NF1. And I want to thank Professor Ferner and Mrs. Myers for their very, very helpful insights and all the people for listening to this podcast and helping to improve the care of individuals with NF1. Thank you very much.
- Thank you.
- Thank you.
Development of this education content has been supported by a grant/funding from SpringWorks. The content has been independently developed by EPG Health with no input or influence from SpringWorks.
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