Exposures and the Human Response--Partnerships for Sustaining Daily Care

From my experience, the NACFC has always been saturated with heavy science…which is fantastic, and has helped create what many may call the most incredible story of progress in medicine today.  I attended my very first Conference in 2011 to see the science for myself, and hell would have to freeze over to keep me away at this point.  In my 6 years attending this event, I’ve watched the CF research environment EXPLODE. There are 50+ clinical trials that will take place in the coming year, and more companies are entering the ring of CF drug development than ever before.  The NACFC is attended primarily by the research community and our clinical care teams, which left me feeling a bit out place occasionally attending as a CF Mom…so I faked it, and acted like I belonged. After all, my educational background is in Chemistry, so I wasn’t a fish completely out of water.  This year though, I felt a shift. The theme of “patient centered care,” and “patient driven research” came up again and again. I was completely thrilled to see 2 adult patients with CF, and the Mother of twins with CF participate live in a Plenary session and have the opportunity to address a room of 4500+ professionals who dedicate their careers to CF care and research. In every way, shape, and form—the patient voice has arrived front and center.  Acknowledgement of community participation in research and care is deeply appreciated. It has been my opinion that we, as the CF community, have the most to lose or gain—and that our voices should not only be a valued part of the discussion, but are VITAL to moving forward if we want research and care to reflect the needs and desires of the patients and families living with this disease.

This year at the NACFC, I had the opportunity to discuss a collaborative project that I’ve been a part of for the last 3 years —Partnerships for Sustaining Daily Care (PSDC). Actually, we originally began as a part of a Strategic Planning Initiative by the CFF, which brought together several committees to address the direction of the Foundation’s resources and programs moving forward. Amazing things, such as the CF Adult and Family Advisors (AFA) have resulted from these thoughtful efforts. I participated in the strategic planning process as a member of the committee addressing adherence. Basically, the CFF could see from Registry and patient reported data, that consistently completing the care regimen was an issue for many. There is also plenty of research that shows better health outcomes associated with successfully completing treatment regimens, and the Foundation saw this as an area where improvements in health and well-being might be gained WITHOUT a new drug—but by helping patients and families utilize what we already have at our fingertips.

One thing is for sure—incorporating CF care into daily life can be extremely difficult. There are numerous challenges that arise: coverage or access issues to even OBTAIN the medication, time (there are only so many hours in the day!), or mental health issues like depression or anxiety that can make completing the care regimen exponentially more difficult—just to name a few.

FACT: Completing the CF care regimen day after day is difficult.

FACT: Regular successful completion of the CF care plan results in BETTER HEALTH OUTCOMES.

FACT: Engaging and empowering patients to give input about their personal goals, and work closely with their care teams to "co-create" the care plan, results in a regimen that is more likely to be sustained. Once we walk out those clinic doors--it is all up to us to balance CF care with our actual lives. I completely understand that there are certain standards of care that our clinic teams must ethically follow, but there is also a tremendous amount of flexibility and creativity that can be employed to also allow patients the opportunity to participate in life in the ways that are most meaningful to them. After all, what is the point of it all, if we miss out on the moments that make life WORTH living?

For me, discussing some of these challenges with Brady’s care team has also been difficult. Let me be clear, I absolutely LOVE Brady’s clinical care team. They have cared for him since birth, and I consider them family! Brady’s care team is comprised of people that I admire MOST in the entire world. Even with the great relationship I share with them, being open and honest about challenges with the care regimen can be difficult because: 

1) For me (type A personality), admitting to my care team that I was having issues with the care plan felt like admitting that I'm not perfect...and that was tough.

2) Not everyone shares a wonderful relationship with their care team, and discussions around "adherence" to the regimen can take on a judgmental tone. "Why weren't you able to complete every dose of Pulmozyme last week?" can feel more like "What's the matter with you?" The tone and intention behind the communication is extremely important because... 

3) We are sensitive about these issues. Even when we miss doses or treatments, we may still be bending over backward to incorporate CF treatments into our lives.  We may have to sacrifice time with our friends, and sometimes have to say no to invitations because it just doesn't work with our CF care that day. There can be some bitterness about missing out on LIFE to care for CF. Also, because of the progressive nature of the disease, we sometimes don't succeed at reaching our goals--even when we do every single goddamn thing right. I know that as a parent, I often took it very personally if Brady didn't meet his weight or lung function goals. It made me feel like a FAILURE.

4) Clinic visits are so long and jam packed already, that bringing up a tough topic like a struggle with depression or anxiety might be the LAST thing you want to do. I know that toward the end of a long clinic visit...I would probably say or do anything just to escape, and get the hell out of there! I may not feel like bringing up my anxiety or depression at that point. These discussions can't take place as an afterthought, because that means they may not happen at all. 

5) Mental health issues, though they are getting talked about more and more, still carry a certain taboo in our society. 

As someone who has personally struggled with anxiety and depression related to CF, I think it is time to move beyond these taboos, and bring mental health out into the open, as a part of our regular clinical care. Last year, mental health was addressed in a big way, as the topic of a Plenary Session entitled: There is no Health without Mental Health. I encourage everyone to follow the link and read more about that amazing session.

FACT: People living with a chronic disease and those that care for them are more likely to suffer from anxiety or depression 

If you've lived with a chronic disease, or cared for someone who has, these statistics may not come as a surprise. Anxiety and/or depression are absolutely normal human responses to stress and trauma, which are something we are regularly exposed to dealing with CF. For me, it felt like there were sort of 3 main categories: 1) chronic stress, 2) acute stress, and 3) traumatic stress

Chronic Stress: The daily burden of care can absolutely be exhausting and stressful. Also, when a shortened life expectancy is attached to the disease, worry about long term survival and quality of life are things that can keep us awake at night. I remember when Brady was an infant, it felt like these constant worries were assaulting my brain like a machine gun. It just wouldn't stop. 

Acute Stress: There are so many acute stressors in CF, that I don't even know where to start: pulmonary exacerbations, holding a screaming child down for a procedure/blood draw/throat swab, clinic visits or hospitalizations, explaining CF to someone new, missing something important to you because of CF, the fear of exposure that can come with flu season...the list could go on and on.

Traumatic Stress: Unfortunately, living with CF can also mean exposure to traumatic stress: a massive lung bleed or lung collapse, undergoing transplant, or losing someone you care about to CF can have a major impact on mental health.  

Exposures and the "Normal" Human Response

We honestly wouldn't be human if these stressors and traumas didn't impact us...and there needn't be any judgement attached to that fact. Let's consider a couple of scenarios.

1) Your friend was seated next to someone coughing and sneezing their head off with a viral infection on a long international flight. Each cough or sneeze exposes your friend to germs, and a few days after she returns home, she becomes ill. Do you think she is weak because her body succombed to the same virus? No. Of course not. That seems ludacris.This is a normal human response. 

2) A child exposed to high levels of carcinogens in their environment develops cancer as a result. Do you blame the child for not being able to handle the pollution?... Not unless you're an asshole! This is absurd. The child's cells began dividing out of control because that is a normal human response to high levels of carcinogen exposure. 

3) A person living with a cystic fibrosis is exposed to the constant stress of the daily management of the disease, and then suffers from a massive hemoptysis resulting in a long hospitalization and time away from family, friends, and the other important things in their life. They become depressed. Do you see that person as incapable because they are struggling? A resounding NO. This is a normal human response.

4) A Mother worries constantly about the long term health and quality of life for her child because of a cystic fibrosis diagnosis. She finds support by connecting with others in the CF community, and becomes very close to other Moms in the same boat. The support helps her manage daily stress, but she suffers a major surge of anxiety when she learns that someone in her circle of support just lost their child unexpectedly due to a complication of CF. She just can't shake the fear that something similar might happen to her own child. Do you judge this Mom as weak, or incapable? Would you recommend that she just "buck up" and shake it off? I hope it is easy to see the pattern developing here. Anxiety, in this situation, is a completely normal human response. 

When I first began working on the PSDC project 3 years ago, I think I sort of assumed that feelings of anxiety and depression came as a package deal with the CF diagnosis. As a part of the strategic planning process that led to this program, our group poured through literature on how depression and anxiety in chronic illness, and particularly CF. Even though we intuitively understand that mental health is linked to our overall well-being, it made a huge impact on me personally to see data on how mental health was directly linked to lung funtion, and other measures of physical health that we attach great importance to. Similarly, I learned that the mental well-being of the care provider was linked to the physical health of the one being cared for. 

I learned that MY MENTAL HEALTH was linked to BRADY'S LUNG FUNCTION. 

That fact was like a slap in the face to me. I think it is pretty common for caregivers to neglect their own needs, and just give and give to their child or the person they are caring for.  Along the same lines, I can imagine that if you are one living with the chronic illness, there are so many physical manifestations of the disease to deal with, that the invisible mental health issues tend to move further and further down the list of priorities. This is why mental health screening and treatment in CF is important. This is one of the important reasons why we need "Partnerships for Sustaining Daily Care." It honestly wouldn't have even occured to me to bring up my own feelings of anxiety and depression in one of my son's clinic appointments, because that didn't seem like the appropriate place and I didn't think that there was anything that could be done about it anyway. In hindsight, it is easy to see that Brady's ability to successfully complete his treatment regimen as an infant and small child was entirely dependent upon my husband and I. If we suffer...he suffers. That was a big AHA moment for me, moving mental health into the "zone of legimate concerns" portion of my brain. 

As the PSDC project takes shape, our hope is to create a cultural upgrade to our clinical care that incorporates mental health into part of the landscape of a routine visit, and create an environment where we feel that we can be safe and honest discussing ANY challenges that stand in the way of completing our daily care. This isn't going to happen overnight, as this is new territory for both patients AND providers. The PSDC group decided that the best way to see these changes take place is to try and understand they we are all human, with similar goals and desires. We participated in several "empathy experiments" to help the care team understand more fully how hard it is to live with CF day in and day out. I also gained a huge amount of respect for how difficult it is for the care teams to do their jobs as well. They also face challenges and frustrations that interfere with the delivery of the best care. We learned to look at one another as people, rather than simply patient and provider. This is about bringing humanity and empathy into our care, and that goes both ways. If anything I've said in this blog resonates with you, the PSDC group would like to hear from you!  As we begin creating the ripple for this cultural shift, we want to learn from you. What does partnership with your care team mean to you? Can you think of any instances where you felt like you worked really collaboratively and creatively with your care team to acheive something great? Tell us about it. Can you recall a time when you felt like communication with the care team completely missed the mark? We want to know about that too. Please send your comments to PSDCteam@cff.org.

I also invite you to join the Adult CF and Family Advisors. Sign up and share your input! 

I had the opportunity to chat about the PSDC in an interactive Facebook Live session at the NACFC this year and you can check it out here! You'll also learn about other cool initiatives the CFF is developing to help connect our community. You can find that interview here: 

https://www.facebook.com/cysticfibrosisfoundation/videos/10154731243262112/

If you are having issues with access to medications as a challenge to sustaining your daily care regimen, contact CFF Compass Program for help. compass@cff.org