From Lab to Life

Hello, and welcome to our program titled, Advancing Cystic Fibrosis, Addressing Disparities, Challenging Perceptions, and Innovating Patient-Centered Care. I'm Dr. Susanna McColley, Professor of Pediatrics and Pulmonary and Sleep Medicine at Northwestern University Feinberg School of Medicine, and Director of Interdisciplinary Research Partnerships at Stanley Manning Children's Research Institute.

and in Robert H. Lurie Children's Hospital of Chicago in Chicago, Illinois. I'm joined today by my esteemed colleagues, Denis Hadjiliadis Paul F. Heron Jr. Professor of Medicine, pulmonary, allergy, and critical care at the Perlman School of Medicine, University of Pennsylvania, and Director of the Adult Cystic Fibrosis Program and member of the Lung Transplantation and Palliative Care Programs,

at the Hospital of the University of Pennsylvania in Philadelphia, Pennsylvania. Our disclosures are displayed on the screen.

This educational activity is supported by an independent educational grant from VirTex. We would like to thank them for their support of this initiative. The learning objectives for this program are displayed on the screen. Let's now discuss caregiver support in cystic fibrosis. As we all know, despite all the advances that we have had in cystic fibrosis, this is still a burdensome disease. There's a lot of burden to parents.

of young children living with cystic fibrosis, but there's also a lot of burden even in adults with cystic fibrosis, and this might be related to their partners, their relationships, or even their parents. And sometimes when they're parents, there is also burden in a different way when they have strain for taking care of their kids. The challenges that the caregivers face are a lot. Caregiver burden kind of defined as the level of multifaceted strain perceived by the caregivers from caring for a family member

or a loved one over time. The chances for caregivers can include many different aspects of life. Those include emotional strain, include financial strain, and also demands on time. You have to go to clinic, you have to have unplanned hospital stays or other things that might be required. And you also have to adhere to a very complicated regimen. And even though things have improved, it is estimated that many times parents have to spend up to 75 minutes helping children with their regimen every day.

This leads to a lot of problems in the caregiver support and there's consequences for both the caregiver and the person that is living with CF. So the financial issues are significant, you have less resources than you need, you might have multiple responsibilities that conflict with each other, taking care of other children, your work. That means that you might have fewer social activities and that creates caregiver burden that leads to inability to provide the care that you need. You also have decreased quality of life.

And as a result, you end up having a lot of physical and psychological health deterioration for the caregiver. The consequences can be bad for self-perception of the caregiver. It can create problems for the person with cystic fibrosis and also create a lot of strain in many different aspects and leads to worse outcomes. There's different things that we can do to try and help caregivers of persons living with CF. First of all, we have to understand better how the impact of caregiving

leads to problems in the care of the persons with CF. Screening caregivers for unmet needs is important because those needs might change. At different times, there might be financial strain. At other times, there might be a time strain or there might be other competing priorities. In situations where there's anxiety or depression, you can provide support and resources from that perspective. And if there's access to palliative care when appropriate for persons with CF, this might help indirectly.

the person with CF, but also the caregiver. So those are important things to think about. There's a lot of different resources. Obviously, the most important one is the Cystic Fibrosis Foundation, including the local chapters. One aspect of the Cystic Fibrosis Foundation, Compass, helps a lot in providing resources, especially when it has to do with insurance, financial challenges, and other things that are related to life with CF. And by helping, the person with CF also helps you with caregiver by taking away some of the burden.

I want to also highlight the NOAA-CF, which is an organization that helps the African-American community living with CF. Another foundation is the Take a Breather Foundation, which provides breathers for families of persons living with CF, similar to what Make a Wish does. And some other foundations are CFRI, the Boomer Esciation Foundation. They provide a lot of educational research support, but they also have resources for persons living with CF.

and there's CF News Today, Bono Foundation, and many others. And I would encourage anybody that's looking for resources to also look at their state and regional foundations, which might be useful, and they might have specific areas where they provide help. Thanks for telling us about online resources. Now let's talk about patient-centered care. Thank you so much, Dr. Macaulay. Anybody that lives with a chronic disease that's different needs and they come from a different background,

We need to know as healthcare providers how to provide the best care that fits their values, their wishes, and provides information that's culturally appropriate. We're going to go back to the same things that we spoke about before. So looking for unmet social needs is one of the more important things that we can do as providers for cystic fibrosis. In different parts of the stock, we've talked about food insecurity, for example. That's a very important issue to look for. Anxiety and depression.

anybody living with a chronic disease is prone to have that. So if we screen, we're more able to find it. Tobacco smoke exposure, and this can happen in parents, it can happen in partners, and providing information on smoking cessation and education on how this leads to worse outcomes might help the person and their family indirectly. We also know that this is associated with lower socioeconomic and educational status, which many times leads to worse, you know, disease.

There are some best practices to improve patient care for cystic fibrosis, and those include making referrals to community organizations that help alleviate the needs of persons with CF. We can do our best to try and have equitable access to treatment. This is especially true when we think about lung transplantation and highly effective modulator therapies, because those can be very unique and important for people either living with advanced cystic fibrosis or to prevent progression of disease and ensure quality of life.

And also another aspect that's very significant is making sure there's equitable enrollment in clinical trials so that we learn more about people that are underrepresented otherwise and we treat them appropriately. Thank you for that important information. Now that we've discussed patient centered care, let's explore how to transition patients from pediatric care like what I do to adult CF care like you do.

Thank you for the segue, Dr. McColley. As we all know, this has been the new phase of CF compared to the time that many of us started practicing. And thanks to the excellent job that all the pediatricians have done, cystic fibrosis is now a disease that affects a lot more adults than children. The last data suggests that they are close to 60%, but it's continuing to increase compared to the total population. So one of the more significant times when

there can be complications, lung function lost, loss to follow up and problems is during the late adolescence and the early adult golden transition should not happen. So it is extremely important to be able to get patients safely from the pediatric to the adult care. And there are many different ways to do that, but it's best to have a well-defined process. So the first part is the pediatrician meets with a parent and the family.

Usually at the latest that will happen in early adolescence, but as we said, sometimes it can happen a little earlier to start talking about the transition. When the time gets closer, then the patient and the family meet with the adult team to discuss the process and make sure that there's less anxiety about this transition. And then usually the transition happens somewhere between the age of 16 and 21 based on the locker circumstances, the ability to see people less than 18, what other things that happen. It usually should happen with a protocol between

the pediatric center and the adult center. But at any point there's a lot of disengagement or a problem with the transition process. Sometimes you go back and you try to reestablish contact and start from an earlier stage until you hopefully achieve a successful transition. A program that was developed a few years ago is called the CF RISE and that facilitates transition for patients from pediatric to adult sphere care and includes mostly a focus on responsibility and independence for the person that's being transitioned.

It has educational material, has knowledge assessments, it has a responsibility checklist, and educational resources for everybody to be able to do that transition as smoothly as possible. Cystic fibrosis and multi-system disease, that would require educating the patients about the possible areas where cystic fibrosis might be affecting them. All of us know that airways are the more common, more morbid area for persons that live with cystic fibrosis.

And there's a lot of infectious bronchiectasis exacerbations, but nobody should forget issues that have to do with areas like CF related, diabetes, osteoporosis, infertility in males, muscle weakness, stress incontinence, a lot of GI issues related to pancreatic insufficiency, obstructions like DIOS, pancreatitis for some patients, liver disease. So all those things are areas where the person needs to know what might be the complications.

you know, educating them early and appropriately also based on, know, what their conditions are. Denis, thank you very much for that discussion. Transition can really be a key point in management of cystic fibrosis. And I would say many of us in pediatrics actually talk about adult CF care at the time of diagnosis because it gives

families the information that they should expect their baby to live into adulthood. Speaking of another important issue with families, let's now talk about structural competency and cultural humility and how training can support CF care in those areas. As we all know, we are seeing increased diversity in our population with persons with CF.

This is not surprising because we see that in the United States population. So right now there's basically about two thirds of the population or more that is white and non-Latino. But it's expected that by 2050, that percentage of population will be close to 50%. As a result, we see more people of African-Americans, especially Latinos, but even from other places that traditionally we didn't even think that cystic fibrosis was very common.

So developing structural competency among the health care team is needed to be able to mitigate disparities and create better outcomes in underserved populations. Structural barriers to health care are very embedded. Institutional racism, classism, sexism, homophobia, ableism, and intersections of these aspects of identity can lead to significantly higher prevalence of chronic health outcomes.

And the structural competency is a concept that's developed to overcome those barriers and define the trainability to discern how a host of issues defined clinically as symptoms, attitudes, or diseases also represent the downstream implications of a number of upstream decisions about such matters, health care and food delivery systems, zoning laws, urban and rural infrastructure, medicalization, or even about the very definition of illness and health. So this definition tries

to go into the root of all those problems where we have significant, you know, issues that are very difficult to untangle. And many times as healthcare providers, we see the tail end of this, but we need to know about that to be able to do our best to help treat our patients. Cultural and community cultivates person-centered care. So cultural and community is a component of the structural competency and is an approach to caring for persons and their families. So that means that you have to have self-reflection and assessment.

You have to appreciate patients' expertise on their social and cultural background. You have to be open to establishing a power balance relationship with the patients. That means listening to their side of things and how they view their life and what's important to them. And also means that you have to learn from patients all the time. You have to be able to do that and reassess your values, beliefs, and bias and be able to adjust based on this. Components of cultural and community training work together to help patient care. And there's the five R's.

of cultural humility that drive interactions between the clinician and the caregiver. So one is reflection. That's the approach of trying to look at every encounter with humility and understand. There is the respect, treat every person with respect and strive to preserve dignity and respect. Regard, hold every person in their highest regard to try and avoid any unconscious bias to interfere with any interactions. Relevance, expect cultural humility to be relevant and apply the practice to every encounter. And resiliency.

embody the practice of cultural community to enhance personal resilience and global compassion. There's many steps to take into this. So you have to engage the patients, familiarize with patients, their families and their treatment. You can endorse collaboration in interviews and assessments, and you can integrate culturally relevant information and themes. You can gather culturally relevant collateral information, select culturally appropriate screening and assessment tools.

and then determine the readiness and the motivation for change, provide culturally responsive management, and incorporate all those cultural factors into the treatment plan on how you approach every patient. Let's discuss a patient case that brings some of these concepts to real life. Imran is a 10-year-old devout Muslim boy with cystic fibrosis, has been losing weight for the past six months. He has stored stature.

as malnutrition and declining lung function. Imran admits that he stopped taking his enzyme replacement therapy without his parents' knowledge six months ago when he learned that it was porcine derived. How will you approach this situation with Imran and his parents? The interesting thing with this is that a child has made a decision based on religion without really engaging parents. And of course, any of us who are parents know that a 10-year-old, although they need a lot of supervision, does a lot of

things independently and particularly when he's not at home. So I think this is one of those situations that starts with asking questions. And the best question is also why you made that decision. In pediatrics, it's also important to be prepared to calm family tension because a family that finds out that their kid

has not been following something that they thought they were, can be very upset by that. And that's normal. In this case, I'll finally comment that it is important for healthcare providers to know some of the tenets of Islam and that like many Abrahamic religions, there is a focus on health.

that can supersede other, for example, dietary restrictions. And so one of the things that's happened a lot in my own practice is discussions with teenagers about why they should not observe fasting during Ramadan because they have increased nutritional needs. I'm gonna add here that we can make the religious community of Imran's

and allied of ours. So utilizing the imam in this case, and how you know this is appropriate might provide even more validity to us saying that this is something that's okay, because it's medically you know, oriented. That's an excellent point. And indeed, I have done that in the past as well. I think that would be you know, the

most important approach is engage, you know, emran, engage the parents, maybe have them separately also to see, know, where they're coming from and see what their concerns are as we do that. We've reached the end of this episode. I want to thank Vertex for their support of this program. Be sure to claim your CME credit by filling out the evaluation and post-test.

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