Transition of Care - Patients' point of view

Ernica Transition of Care videos, chapter 3. Patient's point of view. Together with clinicians, patient organizations are actively working to define standardized disease specific pathways leading patients from pediatric to adult care. Nonetheless, patients with rare congenital anomalies can have similar experiences when it comes to transition. Sharing these experiences can lead to the identification of common challenges and areas of good practice. Let's hear what our patient representatives think about this. Hello, my name's Graham Slater. I'm the lead patient representative for Ernica. Transition is a really hot topic, and it's a very, very important topic for the patients and their families. It's a big issue for two reasons. Firstly, transition is not comprehensively delivered by all hospitals. And secondly, transition begs the question to whom, to where do we transition these patients. The truth is that there is a gap. In the healthcare provision for adult patients, uh, adult patients of congenital malformations which are covered by the diseases covered by Ernica. And what you will hear following this brief introduction are the views of my colleagues in response to some very specific questions about transition and about the need for lifelong adult follow-up care. Thank you. Within your patient community, do you think that ongoing long-term follow-up programs see all patients born with the disease you represent? And unfortunately, I have to say that in my country, ongoing long-term follow-up programs do not see all patients. In addition, there are hardly any ongoing long-term follow-up programs at all. And one huge problem we have in Germany is that the operation and follow-up is not centralized. For example, there are around 200 new HD cases per year, and we have 130 pediatric surgical departments. Not all patients are treated by experts, and this has a lifelong impact for the children. There are also clinicians who tell the parents before the child's corrective surgery. That after the operation they won't have any problems at all and no follow up is necessary, what is not true in the majority of the cases. And even the expert clinicians we have in Germany do not all have a structured ongoing long-term follow-up program. A kind of mirror of our current situation in Germany are the national guidelines for the treatment of Hirschprung disease. They even do not mention the necessity of follow-up. These guidelines are being reviewed right now, also under consideration of the Erniker Hirschbung disease guidelines and in cooperation with us as patient organization. HD patients often face constipation and continence problems, which is a taboo. Therefore, they are sometimes too embarrassed to seek medical help, and HD patients are followed up by pediatric surgeons who are generally not allowed to treat them past the age of 18. They then have to consult clinicians for adult patients who often do not know much about the treatment of this rare malformation. It is one of the main goals of our patient organization to ensure that all patients are operated and followed up in centers of expertise and to establish a structured ongoing follow-up program for children and for adults. The dissemination of the European guidelines with their emphasis on the necessity of long-term follow-up contributes to a better understanding of Hirschprung disease, and this makes us hopeful that follow-up care in Germany will change from being absent or unstructured to becoming a standard part of the treatment of a child with Hirschsprung disease. Do you consider it to be important to build a transitional program for patients with congenital anomalies? Yes, I think it's very important. My son has a short bowel from the age of 5, and he just turned 18, so he just had his transition last year, June, but due to COVID, it was not well prepared. Only after 3 months after he turned 18, he had an appointment with his new doctor, so it was a little bit late. And yeah, he's 18 and he couldn't care less about anything about his illness, so the doctor called him with the blood results and he was OK. And I said, what did she say? He said, Oh, I don't know. So I think it's very important to build a program for the children, but involving the parents. I think it's a good idea and I also know that there is going to be a new program or protocol of transition, so I'm very happy with that. So my daughter will go into hopefully a smooth transition. Uh, now they, they are used to being the center, being, uh, all kinds of support in, in the, the team, uh, in, uh, all kinds of nice activities when they are hospitalized because there's also a point I think is have to be taken into account for this program because, um, yeah, when they're turning 18 and they are hospitalized then they are on a different. Ward with all kinds of adult people and I know that she's not looking forward to that item as well apart from a new doctor, a new team. And uh I would yeah I would like to see maybe if there is a a special ward designed for 18 to 25 year old people, uh, young adults who have then sort of smoother transition into the adult life that they can do some more activities or plans or whatever they, uh, they are, uh, uh, organizing. And hopefully we will find some, yeah, as discussed, the surgeons for the adult surgery are also a little bit different than the pediatric surgeons, so hopefully the transition will be good that we can see some doctors in advance instead of only when you're 18. OK, this is your new doctor. Uh, have a nice time because I can imagine it's very emotional as well to, uh, to have this transition and uh hopefully, uh, for both parties, uh, it will be a good thing to have such a program. In your opinion, what is the main goal of transition of care programs, and what is the main benefit? The main goal to me is to ensure that the patient receives uninterrupted, good quality treatment and follow-up regardless of where he or she is being taken care of. Like if, if the pace of care is in the children's hospital or even in the adult hospital, then at least the practitioners on both sides have to cooperate. Mm. And then even though this involves a deep knowledge transfer, uh uh where her medical history is taught to the new practitioners, it's still um needs to be effective. And then one of the issues is that um the Parties in the adult care don't necessarily Have the emotional relationship with the patient as well as the pediatrics do have as they have probably been treating the children from birth until 18 and so so there is a link that has to be built. I'd say that. Um, there needs to be a lifelong follow-up with a timely, uh, suitable intervals, and those, those intervals are very different when we are talking about children, patients who are children and who are adults. But the main goal and main benefit needs to be quality of life to the patient regardless of the age. Do you think it's also important for you as a parent or carer or as a patient, to be guided through this process in which you or your child transitions from pediatric to adult care? Having been part of SOA, our German patient organization, for almost 10 years, I think the subject of how to go about transition is a really important question. Because parents of teenagers with Hirschprung's disease ask themselves how can we support our child to become independent, to become a confident person despite his or her rare condition they live with every day. Where do we find the right kind of support for our child outside our home? And where can our child get medical support when the pediatric surgeon cannot treat him or her any longer as an adult? I would say that many teenagers with Hirschpring's disease, if they have remaining symptoms, manage these really well, but they do not necessarily want to talk about their condition very much at their age, and there are a lot of private subjects like changes in their body, sexuality, etc. which they do not want to discuss with their parents. Also, it's important to bear in mind with Hirschsprung's disease that it generally affects a very private, intimate part of life which in society is a taboo. People in general don't talk about how they go to the toilet, but the symptoms of Hirschsprung's disease include things like constipation and incontinence, which in everyday life is just impossible to deal with in an open way. Taking a brief look at adults living with Hirschsprung's disease shows the effects of not having a structured transition process. According to the experiences reported within our German patient organization, often adults do not have adequate follow-up care, or they have residual symptoms or new problems. They often assume that they are supposed to be healthy, and even if they seek medical advice, they don't know where to find doctors for adults who have sufficient experience with their childhood condition. And as a result, some adults have a reduced quality of life. Rather than improving their health situations, they end up restricting their social lives, like not going out very much because of fear of embarrassing situations or even restricting sexuality and staying single just because they're ashamed of their body and don't know how to go about this with potential partners. And not knowing where to turn to also means that their rare condition is a little bit like their embarrassing secret, which is an enormous psychological burden. So going back to the original question, is it important to be guided through the process of transition? I would say looking at teenagers with Hirschprung's disease who might have to manage remaining symptoms on an everyday basis but really just want to live a normal independent teenager's life, plus looking at the general situation with follow up for Hirschprung's disease in Germany. Where the necessity of long term follow up and support does not have the significance it should have and therefore Hirschsprung's disease patients may have reduced quality of life as adults, I would say yes, absolutely, it would be so helpful to have a structure, to have a routine to follow and to be guided through the process rather than transition just being the family's responsibility alone. And it would be so helpful to have contacts to know which medical experts to address in order to find adequate treatment at every stage of life, and it would be so helpful if follow-up care and transition were just normal steps on the patient's pathway, just like diagnosis and surgery. Transition assumes that adult care and follow up is required. Do you think there's a need for lifelong follow-up for the disease you represent? Um, we know little about the impact on the adult population, and because we see more babies surviving, we naturally see more ongoing issues, and we can learn from the adult population, which hopefully helps to improve research, care, lifespan, and ultimately quality of life. Um, in particular, I think we need to learn more about pregnancy and childbirth, and these are normal everyday life events, and we don't know how this really truly impacts the adult population, um, and the community in general. And this will not only help patients to live normal lives, but it will help people such as researchers and doctors and allied healthcare professionals to monitor things like familial clusters. Um, so hopefully, if we can follow these patients from birth to end of life, we will learn much, much more about the disease, and that ultimately will help us to refine the patient pathway in the future. What would be the most appropriate form of lifelong follow up for the disease you represent? If I could bake my own hospital or my own transition in a perfect world, patients with a rare congenital malformation of the esophagus called esophageal resa have a lifelong, regular checkup to which they are actively invited by an adult expert in their treating hospital. If any observation or developments have been previously seen, the patient already announced this for preparing the more individual diagnostics at the same day. In a broad anamnesis, all possible issues are checked, and if there is a need for further investigations, this must be explained and discussed with the patient. And in the end of the day, the patient has all necessary information and knows when his next checkup will take place. In your opinion, what are the main challenges in building long-term follow-up programs within the adult care system? Recently we published a work which we analyzed around 96 hospitals. Half of these were children's hospitals and half of these for adults, and we asked the patients what was what happened when they started moving to the adult age. The result was quite disappointing. 73% of the hospitals did not have a specific program for transition to adulthood. So the first thing I would change is that we should really have a specific program of transition to adulthood. This has some specific requirements, and it's bridging the gaps between what we call medical training for pediatric age and medical training for the adult age. Currently there's no one. That is specifically dedicated or specialized in this specific field. So the other point is the interdisciplinary approach. What we see is that if a gap between an interdisciplinary approach in H-prune disease is one of the main problems for, for young children, like for the, you know, when under 14 years old, this problem increases exponentially in adults. What it means is that at the beginning, we are dealing with surgeons, but in the end, the surgeon is, uh, is not the expertise to handle the vast majority of the problem that, that, that the, that the grown-up child, that the adult encounters. That looking at these problems, we see these are bowel management. Gastroenterological dietary problems, sexual problems, psychological problems, and this means that we really need to build a new figure or new figures of medical doctors that can handle this transition. And since one person cannot do this all. We really think that the key factor is that we need a structured training program, programming in medical school that will train coordinators of the transition programs. And we can't do this without the help of Ernica. We cannot do this without the. Health of the European Union because the issue is that since all the national states, all the national health care system are have one focus is to reduce the costs this is the main driver spending less, you know, this thing is not going to get you spend less and uh so what I think we foresee for the future as Hiprung patients my association is the Ammori Association in Italy. Is to create these figures at least in a small number of specialized centers for hisprung disease, maybe 4 or 5 in the country, but this is really needed to really improve the quality of life of the adult and of the families. Thank you.