How To Be a Good Patient

Doctors, nurses and respiratory therapists agree that when it comes to being a good patient, individuals who have cystic fibrosis tend to lead the pack. Although they develop some of their “patient personality” largely out of necessity, there’s no question about their ability to face the challenge of CF with every ounce of dedication and diligence they have to give.

Good patients typically embody the following qualities:

1. Actively involved
2. Self-awareness
3. Inquisitiveness
4. Willingness to learn
5. Compliant

-Active Involvement-

Being actively involved in your own health care is just one of many ways to take back some control in what is largely uncontrollable. You may not be able to avoid catching a bacteria or virus from the world at large, but you can be proactive in remedying it. A patient who is actively involved in their care understands the importance of making appointments in advance even when they don’t feel particularly sick. They know that managing CF requires ongoing monitoring of their status, particularly when it comes to charting the results of pulmonary function tests (PFTs) and sputum cultures. An actively involved patient understands the value of “an ounce of prevention.”

Good patients who are actively involved in what their doctors are doing, often bring along a supportive friend or family member to their clinic appointments. A second set of ears to hear what the doctors have to say is a good way to make sure you don’t miss anything important or new. Active involvement may also involve bringing a notebook to each appointment to record the doctor’s instructions and recommendations. Making lists of questions for the doctor ahead of time, as well as a list of any medications that need refills are things that good patients do to stay on top of things.

-Self-Awareness-

Similar to being actively involved in your health care program, you must be self-aware. By learning what constitutes your particular “base line” or “status quo” you will be more adept at recognizing when things are starting to decline. For example, if your cough is usually dry and tight but starts to become wetter and more productive, you get on the phone to schedule an appointment at the CF Clinic.

Another aspect of self-awareness is that you have a healthy attitude about your limitations because of CF. You are able to live life to the fullest, but do so without exhausting yourself or putting yourself in harm’s way. A good patient who is self-aware knows how to set appropriate boundaries to keep friends and even family from pressuring you into giving too much of yourself.

Patients who are self-aware are honest with their doctors about the non-physical aspects of life with CF. They understand that the emotional burden that comes with having a chronic condition like CF is sometimes too much for one person to bear. A good patient asks for help when they feel overwhelmed.

-Inquisitiveness-

Good patients ask questions and expect answers. They ask medical professionals about any new treatments or medications they’ve heard about or read about online. An inquisitive nature not only helps a CF patient understand his or her own body, but enables them to gain clearer understanding of what the doctors are doing and why.

An inquisitive patient does not assume that s/he has all the answers. Patients who are inquisitive don’t assume that everything they read on the Internet about CF is true or applicable to their individual situations. They bring their questions to the professionals and ask for clarification. They respect the doctor’s expertise and training, and ask questions about things they don’t understand. A good patient is not afraid to ask a doctor to explain things more than once. After all, cystic fibrosis has so many facets, it’s unreasonable to expect to learn it all in a short clinic appointment.

-Willingness to Learn-

When a doctor recommends a new course of treatment or a new therapy, a good patient is willing to learn to adapt. Instead of refusing to try something unfamiliar, they give it a fair chance before making a final decision. For example, if your doctor recommends that it’s time to get a port-a-cath instead of a PICC line, a good patient will be willing to learn why this is so. Similarly, if a patient is diagnosed with cystic fibrosis related diabetes (CFRD), the willingness to learn how to manage the new condition should override any desire to complain or become depressed at the news.

Although there is certainly a time and a place for complaining and “venting” about the struggle of life with CF, a patient with a healthy outlook and willingness to learn will be able to successfully balance it all with a bit of help. Patients who are willing to learn are not afraid to step outside their comfort zones and learn how to let other people help them with things.

-Compliant-

Rarely are cystic fibrosis patients 100% compliant with all their treatments and medications, though there are some who set the bar quite high! Compliant patients stand a much greater chance of finding a sense of peace and acceptance of their disease. Patients who are compliant can rest assured to know that they are doing everything within their power to make their lives as full and beautiful as possible, even with CF.

Doctors appreciate their patients who are compliant, because it makes their jobs a bit easier. For example, if a patient is compliant to take their enzymes as directed suddenly begins to have a change in their bowel movements, the doctor will know that either the diet has changed or the enzymes are no longer effective at that particular strength. Less time is wasted in trying to find an appropriate solution.

Compliant patients keep their appointments. They understand the need for preventive care in treating cystic fibrosis, and they take every opportunity to monitor their health.

Managing life with cystic fibrosis can be tough, but it doesn’t need to be overwhelming and depressing. If you work on being a good patient, chances are you’ll cope with the disease much better.

Adoption and Cystic Fibrosis

Today's Q&A is courtesy of Laura Christianson, author of The Adoption Decision and The Adoption Network.

Q. If I have a chronic medical condition, will it exclude me from being eligible to adopt a child?

A. Not necessarily. While adoption professionals do evaluate the health of the prospective adoptive parent(s), their main concern is whether a person's health issues will interfere with or prevent that person from being an effective parent.

If you have a chronic medical challenge and want to adopt, it's important to share openly and honestly with your adoption social worker, and explain the ways in which you are being proactive about managing your health issues. Adoption social workers aren't looking for excuses to turn prospective parents down; they are looking ways to say "yes."

I know several adoptive parents who have chronic medical conditions—they often adopt a child who has the same condition. Adoption social workers are delighted when this happens, because children with medical issues are considered "hard-to-place." When a parent adopts a child who has the same medical challenge, the child often receives the best treatment possible because the parent knows exactly what the child needs.

Laura is a freelance journalist specializing in adoption-related issues. She is the author of The Adoption Decision: 15 Things You Want to Know Before Adopting and The Adoption Network: Your Guide to Starting a Support System.

Her Exploring Adoption blog received Forbes magazine's 'Best of the Web' rating. Laura has written numerous general-interest features and essays for national and regional publications. She speaks regularly at writers' conferences and other events.

Living With CF

Because I was diagnosed at birth, I'm unable to give an account of what it was like to hear the diagnosis for the first time. I've heard my parents' version of what that was like. I was born on the fringe edge of all kinds of new developments for CF treatment, but there still wasn't much out there in terms of reading material or guidelines for my folks to go by. My mom says they basically handed her a leaflet about CF and told her that I might not live to see my 10th birthday.

That was nearly three decades ago.

I never considered myself "sick" and still don't. I have recollections of what it was like to discover at an early age that I was different. When I was in kindergarten I thought it was the other kids who were strange for not needing to take enzymes with their food. I remember my dad teaching me at breakfast one morning how to swallow multiple pills at once with the help of a nice big glass of acidophilus milk. I remember getting in heaps of trouble for refusing to take my meds and instead stashing them around my bedroom. I remember wanting to play the xylophone in the school band and being told by my parents that I had to pick a big wind instrument that would provide good lung therapy (I chose the French horn - lots of sustained breathing!)

The most notable part of understanding that I had CF came when I was in 5th grade. This was the first time my family had ever heard of CF specialty team clinics. We got an appointment and were just astonished at the volume of information that was presented to us. I remember being more thrilled than scared. There were booklets about nutrition which for me was a real turning point. I typically have more difficulty with malabsorption than with lung infections. Being told that I could have as much fat as I could tolerate and as many calories as I could tolerate was the most exciting thing I had ever been told in my life. Up until that point I think I could count on one hand the number of times my family had eaten at a fast food restaurant.

We celebrated that night on the way home from the doctor's office by stopping at Denny's for a hot fudge sundae-my first ever.

I consider myself fortunate to have not been hospitalized until I was 13. I have to admit, this was largely due to my rebellion against taking my meds. The teenage years with CF were rough, but I'm sure that's true of anyone's teenage experience. Throughout high school I was hospitalized an average of once a year. When I got to college I had learned enough about how my body was affected by CF during times of stress, so I learned to voluntarily schedule my "tune-ups" around my breaks from school.

As I have gotten older, there have been additional diagnoses made, and those have been handled with as much grace as possible. Osteopenia. Allergies. Impaired glucose tolerance. Cepacia. Sinusitis. Each time something new is discovered, we (my family, husband and I) initially suck in a breath of air to brace ourselves for what these things mean. We have learned to plan for the worst but hope for the best. Throughout everything we have faced, we have learned that we don't need to be afraid of CF.

I'm not dying from CF-I'm living with it. And not just living. I'm thriving.

How I Let my Employer Know I Have CF

I was very discrete and private about CF for a long time. I didn't start blogging until 2 years ago which was after I had already gotten a job. You'll notice that my last name does not appear on my blog, nor does my location. That affords me just enough privacy.

Don't think I haven't weighed it heavily about how and when to disclose CF to my employer. When I was being considered for the position (I started as a temp) I was up front with my supervisor and explained that I have CF. I also explained that although there are times when it slows me down, there is no part of my job that I am UNABLE to do because of it. The company I work for is HUGE (44,000 employees worldwide) and I'm a very small fish. I'm an environmental scientist working as a consultant, which is very flexible. I chose that job because I knew it would be the ideal situation for me with CF. This have proven to be a very good fit both for me and my employer.

My supervisor knows that when I'm in the office, he can count on me to give my utmost effort. My work ethic is outstanding (according to my last performance review) and I have a lot to contribute. My work speaks for itself, and I have a great deal of job security.

It's no secret to the people in my office that I have CF. There are others in our group who have other health issues (MS for example) and they have been there longer than I have. Basically, the company is great because it offers so much flexibility. The benefits are phenomenal too. I am even able to get life insurance (WOW!) up to a certain $ amount without a medical exam.

It's far more likely that I would resign from my job before I'd be terminated. In fact, I'm thinking I may only be there for 4 more years. If I were terminated, my husband and I have a safety net in place. (I won't bore you with the details) but suffice to say, we've got all the angles covered. I am also a professional freelance writer. That's ramping up to be a more full-time gig in the next year or so.

My College Experience

I used my experience with CF to write a great essay that was part of my application. I did not live in the dorms in college, however, my parents and I did look into getting a single dorm room if I did live on campus. We would not have even considered that an option, except for the fact that the housing director at the school I went to has a daughter with CF, so he was willing to work something out for us.

At first I didn't tell people at college I had CF. Then there was a quarter where I needed to retroactively withdraw from my classes because I got sick and missed my final exams. At that point I went to the dean of student affairs with a letter from my doctor explaining CF and what reasonable accommodations I needed in order to get the most out of my college experience. That letter went in my file, and we also brought a copy of it to the student special services office. They arranged for me to have a note-taker in class, or in some cases, for my professor to provide his lecture slides to me so in case I missed class. I also had a tutor come to the hospital when I was having a tune-up.

One of the most important things that student special services was able to do for me was to arrange with the environmental health and safety office to get me a specially fit respirator mask to wear in organic chemistry lab. I was a science major, and o-chem was something I had to take (I LOVED it by the way.) However, nearly all the chemicals we worked with were aromatic hydrocarbons, which are a respiratory concern. Even with the fume hoods, I had to be careful, especially if someone dropped their test tube or spilled something like acetone.

I don't think I used CF to gain an advantage, but I did make sure to ask for assistance on the things that I needed to stay competitive in my class. When you're a science major at a research university, things can be pretty cut-throat. I was a very serious student, but without the note-taking services and tutoring when I missed class, I would have had a more frustrating time than I already did.

One of the best things I did, knowing that I'd have to work hard to keep up when CF slowed me down, was that I started taking college courses as a high school student. By the time I was actually in college, I had already knocked out 10 classes toward my degree. That way my academic standing never faltered. I took full loads (12 unites) every quarter my first year because I needed to in order to stay on my parents' insurance. The first quarter of my sophomore year I got a part-time job at a environmental engineering and research lab. I worked anywhere from 12-20 hours a week for the next few years. My senior year I was recommended for another research position at UC Irvine, and like an idiot, I thought I could handle that, my lab job and classes at UC Riverside, my health, being a newlywed, and commuting 200 miles a day.

I got sick, but I continued to push myself because I wanted so badly to graduate. There were times when my parents and I didn't think I'd make it. Eventually I pared it all down, and by graduation I was in much better shape. I love seeing my diploma on the wall, but I wouldn't recommend ANYONE push themselves as hard as I had. I can't even stand to look at my graduation pictures because of how visibly sick I was.

That's my college experience. I'm fortunate to have survived. I couldn't have done it without the support of my husband and parents. I'm especially thankful that my parents lived so close to the campus. I ended up spending the night there many times when I was too tired to drive home to my husband.

Perspectives on Educating Others

Don't Assume

(Author Unknown)

Don't assume that because I look well that I feel well. Looks can be
very deceiving. Many days I look great but feel terrible.

Don't say, "I know how you feel." No one knows how anyone else feels.
We all have varying thresholds of pain, and pain cannot be measured.



Don't tell me about your Aunt with so and so disease or ailment and how
she managed in spite of it. I am doing the best I can.

'Don't tell me, "It could be worse." I don't need to be reminded.

Don't decide what I am capable of doing. Allow me to make those
decisions. There may be times I 'm wrong, but I'll know soon enough.

Don't be upset that you cannot ease my problems. It won't do any good
for both of us to be miserable.

Don't ask me how I feel unless you really want to know. You may hear a
lot more than you are prepared to listen to.

Don't assume because I did a certain activity yesterday that I can do
it today.

Don't tell me about the latest fad cure. If there is a legtimate
treatment, my doctor will let me know.

Do realize that I am angry and frustrated with the disease, not with
you.

Do let me know that you are available to help me when I ask.

Do offer me lots of encouragement.

Do understand why I cancel plans at the last minute. I never know from
one day to the next how I will feel.

Do continue to invite me to activities. Just because I am not able to
bike along with the gang does not mean that I can't meet you for the picnic
at the end of the trail. Please let me decide. Thank you.

Quality Improvement in CF Care

The Cystic Fibrosis Foundation (CFF) continues to make a difference in the lives of patients with CF and their families. In order to facilitate excellence in health care and increase the level of involvement between patients, their families and the cystic fibrosis team clinic, the CFF has provided care center data via their website.

CFF Care Center Data

" Reporting data from our nationwide network of centers is part of a comprehensive quality improvement effort by the Foundation. Our goal is to help people with CF live longer and better lives. In fact, the Foundation is one of the first health organizations in the country to provide health outcomes data to the public, demonstrating an intense commitment to raise quality of care to an even higher level." --Robert J. Beall, PhD, President CFF

This effort has been more than 6 years in the making. The CFF hopes that this data proves beneficial to the CF community. By publicly reporting Care Center Data, everyone involved with CF can be encouraged and inspired to raise the bar to new heights. As the president of the CFF puts it "Quality CF care is more than numbers."

As a person with CF I have a unique perspective on quality being about more than numbers. Some of the numbers that I think about are my weight, body mass index, lung function, the amounts of medications I take and the number of days I have to put life on hold when exacerbations occur. Even as those numbers change, my overall quality of life is excellent, and that is, at least in part, due to the excellent care I receive at the CF Adult Clinic.

Currently, there is no cure for cystic fibrosis. The precious lives of children and young adults are being lost every day to this devastating disease. If you would like to help support the Cystic Fibrosis Foundation in its ongoing research to find a cure for CF, please consider making an online donation here. Approximately $.90 of every dollar is used effectively to fund research and provide many tomorrows for those who battle CF.

If you would like to read a brief description of cystic fibrosis, please follow this link.
If you are interested in learning what a typical day is like for an adult with CF, you can read my article here.

Parents: Communicating the Reality of CF to your Child

My parents filled me in on the details about CF gradually, rather than dropping a big "by the way, it's terminal" bomb on my head. Here are some thoughts I can share that may ease the burden of telling your child about the harsh realities of the disease. I've broken them down by age group.

Kindergarten - Third grade:

They explained that my body didn't work the same way as other people's. We didn't use the word "fatal" or "disease." Mom said that if anyone ever asked why I had to take enzymes or stuff, I should just say "they help my body work better." We talked about tummy aches and why it was important for me to take my pills so that I wouldn't have so many tummy aches.

Fourth grade - Sixth Grade:

This was the age when I started reading more. Discussions about CF were, in some ways, similar to being given "the sex talk." I didn't know enough to ask a lot of questions, and Mom wasn't going to go into detail that I couldn't handle about CF. Mostly I was told that if I wanted to be able to participate in all the things I loved (school, sleepovers at friends, etc.) then I had to be sure to obey the rules about taking my medications.

It was during this time that I also learned to play the french horn. Mom and Dad encouraged me to play a large wind instrument. They said that someday cystic fibrosis would make it very hard for me to breathe well, but the more exercise I gave my lungs, the easier things would be. (And they are!)

Junior High:

This age was awful. I think that's when I was beginning to know that CF was a VERY serious thing. I had read A Time to Die (Lurlene McDaniel) and "Toothpick (K. Ethridge) which were books about teen girls with CF. I had a lot of questions for my mom and dad about CF after reading those. They always answered me honestly. But they also made sure that I understood that I had a responsibility to live in the here and now and not worry about all the "What ifs." They said "there will be time for that, and when that day comes, we'll handle it together."

High School:

Mom and Dad told me once again that I was responsible for making good decisions. They told me that yes, CF is scary, unfair, and all those other things that make it so awful, but that no matter how bad it got, we were a family and we'd get through it.

--

I don't believe in sugar-coating things, but the stark realities don't need to be dropped on a little kid either. Talk about the meds and the treatments and do your best to describe how they work and why they're important.

Bring it up in family time conversation, rather than a "sit down, we have to talk" discussion. Remind her that you love her and wish that she didn't have to do all these things, but since she does, you'll handle it together.

As far as how to say "it's fatal," I wish I had the right words for you on that. Tell your child that CF is a VERY serious disease and people (I stress the word people so that you remain future focused) can and do die from it. Remind your child that there is a lot to live for an a lot of things to experience and enjoy before that happens, and that's what you intend to help him or her accomplish.

This is something you probably want to emphasize: That she can't participate in fun things if she doesn't follow the rules. That's a good life lesson, CF or not. By phrasing it this way, it makes more sense to a child (whose reasoning skills are limited) than to say "take your meds or you're going to die."

Perpectives On Dying with Dignity

This month's installment of "Perspectives On..." is about dying with dignity. The following reflections are courtesy of Allie Stentsland. Her recorded thoughts on her last days with her husband are truly poignant. The words of the hospice nurse as she says "It's obvious that Ry is well cared for...you guys are doing a good job," are a testimony of the dignity of those last days. He fought valiantly with CF from the battlefield of his couch.

Ry's fight ended on May 5, 2005.

The small things

Being in a holding pattern is never easy. I knew Ry was on a downhill slide, but sometimes when I looked at him even now, I could not see it. I looked into his eyes, and there was no fear, no pain, nothing but a little of bit of tiredness, and a lot of love. It made it easy to forget that Ry really was dying. It was nothing like what all the movies, books, and other people had led me to believe. It wasn't a dramatic, horrible giving up. It was this gradual winding down, like a sun setting, where you didn't notice from minute to minute, and even though it's going to go dark, there's a quiet beauty in it.

The hospice nurse had come by to check on Ry, make sure we had enough drugs, and make sure I was doing okay. Ry had listened to his own lungs, interested, with a fascinated smile on his face. "That's so crazy. It sounds like a plastic bag." He laughed and thanked the nurse, going back to leaning back on the couch and watching a documentary on the Amazon, Ahava under the protection of his arm. I showed her to the door, and she turned to face me.

"I know, he's not doing well. I know he's not long for the world." She smiled at me "That's not what I was going to say. I was going to say it's obvious that Ry is well cared for." She put her hand on my shoulder. "You guys are doing a good job."

I looked at the floor and thanked her, ashamed for having cut her off. I shut the door and walked back into the living room. Ahava had fallen asleep, and Ry was stroking her hair. He looked up at me and smiled weakly, and I walked to the couch to sit by him.

"You worry too much. Always have, Al." Before I have a chance to say anything. "How do you know I'm worried?" I say, a bit snottily. I know he knows me all to well, and he's entirely correct..I am worried about him.

"Don't be dumb with me, Allie. I'm dying, not retarded." He looked at me, with a disapproving glance, but quickly broke into a smile. "Allygator, listen. Things are what they are. Worrying isn't going to make it any different. In my mind, I've already been through the worst part. So who cares?" he paused for a minute, eyes closed, catching his breath. "Turn up the tunes, a few more drugs, be with your family...it's all okay. It's okay Al, I promise."

I looked away from hm for a second, and listened to that sounds that wove thought my house. The oxygen whispering into Ry's lungs, the soft snore of my little girl protectively nestled next to her father, the creak of the wood expanding in the April sun.

"How do I do that?" I said, almost to myself.

He took my hand, and kissed it. "Just enjoy the little things. that's all."

That was all. And that was everything.