What it’s like to live with cystic fibrosis

kaleb-choc-childrens-patient
Kaleb chronicles life with cystic fibrosis

By Kaleb B., 18-year-old CHOC Children’s patient

I was born with cystic fibrosis (CF), an inherited life-threatening disorder that damages the lungs and digestive system. For years, I blamed myself for having CF. It made no sense to blame myself for something I was born with, but I couldn’t stop. I now understand that CF isn’t my fault.

What is cystic fibrosis?

CF is a disease caused by a missing protein in your genes. In a person with CF, the body produces mucus that is thicker and stickier than it should be. Everyone’s body makes mucus, and a person without CF can get rid of that mucus by coughing. But for someone with CF, the thick mucus is harder to expel, so the bacteria get trapped in the body, which leads to infections. This can also damage and scar the lung tissue. If left untreated, it could end up causing organ failure.

What does this mean for the rest of the body? The thicker mucus doesn’t move as easily, and it clogs passages in many of the body’s organs. Mucus buildup leads to complications ranging from mild or inconvenient to detrimental. The digestive system and lungs are the two systems CF hits the hardest. It can cause difficulty processing food, but the worst consequence of mucus buildup is the infections that can stem from it.

CF is a cyclical journey. I’ll be healthy for a while, but then I’ll catch something new, and my body will respond well to antibiotics. Then I’ll come down with something else, or the infection I’m fighting will become resistant to a medication that used to work. When I get really sick, I have to be admitted to the hospital for IV medication to try and get my health back on track.

Great strides have been made to cure the genetic mutation that causes CF, but for now, the infections caused by thickened mucus are dangerous and hard to treat.

There is no cure for CF. As little as two decades ago, most people with CF wouldn’t live to see their 18th birthday. Now, statistics show that nearly 40% of people living with CF in the U.S. are 18 years or older. More than half of people with CF will live into their thirties.

Learning to accept your cystic fibrosis diagnosis

There are limitations to what I can do daily because of my health and medicines that affect me. For example, my energy level isn’t consistent—some days I can make it through the day with ease, and other days I run out of steam fast. It’s frustrating, but I am trying to work with it and accept it.

It’s not my fault that my body works this way; I just have to work with what I have.

Despite having CF, there’s still a lot I can do— like writing articles such as this one, sharing my experience with CF.

Cystic fibrosis is a team sport

Over the last year, I’ve had more health challenges than ever, including unexpected infections and too many antibiotics to count. For a while, I was losing hope and felt overwhelmed, but I kept pushing on— and people around me helped keep me going. Their support was crucial to me being able to persevere, and now I’m on the other side.

Your disease doesn’t define you

What I’ve learned over the past year, through these health issues and my success despite them, is that disease doesn’t define me. It’s what you do despite disease that shows who you truly are. It’s how you fight through those battles that reveals your character.

It’s never easy to deal with a chronic illness. But now, I can separate my disease from my self-worth. My CF has never been tied to how valuable I am. Although it is an enormous part of my life and a strong force behind many of my frustrations and insecurities, CF is not who I am. My identity isn’t tied to my disease. I can’t change that I have it, nor the effects it has on me physically. I can only approach how I adapt to living with it.

Myths of living with cystic fibrosis

CF isn’t all medication and hospitalizations; there are some benefits to CF. For example, you usually get to eat a lot of high calorie and tasty food without worrying about gaining weight, which is nice! Enduring treatments usually isn’t too bad either, since you can play games or read while you do them, in order to multitask and make it more enjoyable. There’s a lot of ways to keep a good mood and stay thriving during the mundane parts of living with CF, so being creative with that can be helpful!

Questions I’ll hear a lot about CF usually relate to how “sick” I am or if I’m contagious. Rest assured I’m not contagious. I’ll catch illness a bit easier than others, but usually I’m not that sick, as long as I keep up with my meds. If the infections get bad enough, I’ll need a tune-up in the hospital with some stronger meds. But luckily, I’ve been taking pretty good care of myself lately!

To other teens with CF, I would say take every day and make the most of it. Live your life and enjoy it while you have breath in your lungs! Don’t let CF stop you from living.

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Kieran’s Balancing Act: Living with Cystic Fibrosis

For young gymnast and CHOC Children’s patient Kieran Cacciatore, life is a balancing act in more ways than one.

Living with cystic fibrosis since age 1, Kieran is an accomplished gymnast who recently competed at a national level and will start middle school this fall.

“I can’t say enough about what it’s been for him and how he’s been able to succeed regardless of his health,” his mother, Robin, says. “What he’s accomplished this year is amazing.”

gymnast with cystic fibrosis

Kieran has become an expert at balancing his athleticism, school work and treatment. Like his gymnastics practice schedule, Kieran’s daily care routine is arduous: He swallows six capsules before every meal and snack to aid digestion. Twice daily, he has sessions with a nebulizer, which allows him to inhale medication that rehydrates his nasal passages, and a special vest that vibrates against his chest to loosen mucus.

As a baby, Kieran seemed to always be sick, his mother recalled, and frequent doctor visits, tests and medications didn’t help. When Robin discovered one morning that Kieran’s facial muscles had weakened on one side, like he’d suffered a stroke, she immediately rushed him to the hospital.

There, after a series of tests, Kieran was diagnosed with cystic fibrosis. The genetic condition leads to frequent lung infections and causes breathing difficulty and digestive system problems.

“We never had health problems, and here we were talking to gastroenterologists, pulmonologists and social workers,” Robin said. “I wanted to get educated and make this disease management a part of our daily life. It wasn’t easy, but we had a very good support system.”

Robin was determined not to let illness isolate Kieran or the family. So, they attended athletic events, enrolled Kieran in school, and began gymnastics training when he was 4.

Already a good climber, Kieran was a natural, the family discovered. Weekly practices soon turned twice weekly, and a after a year, he joined a team.

Now 12, Kieran’s longest practice is a weekly five-hour session – and his hard work has paid off. He recently competed against more than 200 athletes at USA Gymnastics’ 2017 Men’s Junior Olympic National Championships, and progressed to the final rounds.

Kieran’s interest in gymnastics is likely only to grow, his mom says.

“I do think he’s going to stick with it,” Robin says. “It’s as much a part of his life as the treatments are.”

Learn more about our Cystic Fibrosis Center

Related posts:

  • What it’s like to live with cystic fibrosis
    By Kaleb B., 18-year-old CHOC Children’s patient I was born with cystic fibrosis (CF), an inherited life-threatening disorder that damages the lungs and digestive system. For years, I blamed myself for ...
  • Meet Dr. Amy Harrison
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Meet Dr. Amy Harrison

CHOC Children’s wants its patients and families to get to know its specialists. Today, meet Dr. Amy Harrison, a pediatric pulmonologist. Dr. Harrison attended Indiana University School of Medicine, and completed both her pediatrics residency and fellowship in pediatric pulmonology at the University of Minnesota Children’s Hospital. She currently serves as co-director of CHOC’s Cystic Fibrosis Center, and has been on staff at CHOC for five years.

Dr. Amy Harrison
Dr. Amy Harrison

Q: What are your special clinical interests?

A:  Cystic fibrosis (CF), muscle weakness (muscular dystrophy and spinal muscular atrophy), asthma and general pulmonary health, chronic disease.

Q: Are you working on any current research?

A: Our CF program was awarded the CF Fundamentals Learning and Leadership Collaborative in June 2015, which has allowed us to improve our CF care processes and clinical outcomes for people with cystic fibrosis. We are currently studying ways to improve our patients’ knowledge of, and adherence to, prescribed pulmonary therapies.

Q: Are there any new programs or developments within your specialty?

A:  Our CF program was awarded a mental health grant through the Cystic Fibrosis Foundation to develop and implement a depression and anxiety screening program for patients with CF and their caregivers. Funds will enable the team to expand its social worker’s availability and have a designated psychologist to help patients and caregivers. They will provide screenings, evidence-based guidelines and follow-up care for depression and anxiety, as well as develop a community referral network of mental health providers. CHOC’s CF model, if successful, could be replicated in other specialty clinics.

In addition, we are excited to offer many cutting-edge therapies for CF patients including Orkambi and Kalydeco, medications that help the defective protein in CF function more normally, thus treating the underlying cause of CF.

Q: What are your most common diagnoses?

A: I see patients with a range of conditions, including asthma, chronic cough, recurrent pneumonia, cystic fibrosis, muscular dystrophy and spinal muscular atrophy.

Q: What would you most like community/referring providers to know about you/your division at CHOC?

A: The pulmonary division at CHOC offers comprehensive care for all patients with lung disease and sleep disorders from extensive lung function testing to sleep studies and diagnostic procedures such as bronchoscopy. We also have comprehensive services for patients with sleep apnea, sleep breathing disorders including apnea monitors and BIPAP data analysis for clinical management. In addition, we also offer services in Huntington Beach, Mission Viejo, Corona and Pomona Valley.

Q: What inspires you most about the care being delivered here at CHOC?  

A: I enjoy caring for children with chronic conditions and helping them to find ways of living normal fulfilling lives. I take great pride in my patient’s accomplishments and find their stories inspiring. I most enjoy creating a true partnership with my patients and their families to find ways to improve their care.

 Q: Why did you decide to become a pediatric pulmonologist?

A: I was always interested in science from a very young age and pursued a medical education due to my own fascination with learning more about how our bodies work. During my training, I developed asthma myself and found my strong relationships with my own medical professionals enormously helpful in empowering me to deal with a chronic disease.

Q: If you weren’t a physician, what would you be and why?  

A: I would probably set up my own Etsy shop and sell things I create! I often make homemade gifts for friends and family members for birthdays and holidays. I also love to travel and exposing my children to new cultures.

Q: What are your hobbies/interests outside of work?

A: I love spending time with my family and my three children, ages 2, 6 and 10. In addition, I enjoy reading, visiting museums and traveling.

Q: What have you learned from your patients?

A: I am continuously amazed by how strong and resilient my patients can be. I learn so much from them, and I have now had three patients tell me they were inspired to pursue a career in medicine because of our relationship and their disease.

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Depression and Anxiety in Cystic Fibrosis Patients and Parents

A recent study of over 6,000 cystic fibrosis patients, ages 12 years through adulthood, and over 4,000 parents, found that depression and anxiety were two to three times more elevated in patients with cystic fibrosis (CF) and in parents of children with CF compared to the general population.[1] Although depression and anxiety are common in CF, the stigma associated with mental health issues often make people less likely to talk about them.  When left untreated, depression and anxiety can affect a patient’s ability to effectively manage his treatment plan.  CF patients with untreated depression, anxiety or both tend to have worse lung function and lower body mass index, in addition to experiencing more hospitalizations.

To address this issue, the Cystic Fibrosis Foundation (CF Foundation) announced newly developed guidelines for screening and treating depression and anxiety.  CHOC Children’s  —  one of only a few affiliated care centers of the CF Foundation in Southern California and the only CF treatment center in Orange County — is one of the first care centers to adopt the recommended guidelines.

“The psychology component of our cystic fibrosis program really makes CHOC stand out,” said Dr. Amy Harrison, pulmonologist and CF specialist at CHOC. “Our multidisciplinary approach allows our care team to manage all aspects of CF from diagnosis to physical and mental health.”

Dr. Amy Harrison

The program is a recent recipient of the CF Foundation’s grant, Implementation of the Depression and Anxiety Guidelines: Award for a Mental Health Coordinator. The grant provides CHOC the opportunity to have a psychologist at every CF clinic to help patients and families, and extends the CF social worker’s availability. They will provide annual screenings, evidence-based interventions and follow-up care for depression and anxiety, as well as develop a community referral network of mental health providers.

Guideline Recommendations

The guidelines propose that screening and treating depression and anxiety should become a routine part of CF care. The voluntary screening consists of completing a couple of short questionnaires.

Recommendations include:

  • All patients 12 years and older should receive annual screening for depression and anxiety.
  • Parents and caregivers of patients 17 years and younger should be offered annual screening for depression and anxiety.
  • A stepped process for prevention, screening, assessment and intervention. This can include talk therapy, medication or a combination of the two.

Staying Well

Patients and caregivers who recognize the beginning signs of depression or anxiety should speak to their CF care team.  In partnership with the patient and/or family, CHOC’s team will recommend the appropriate interventions.

“CHOC wants to help patients and parents break their goals into manageable pieces and focus their energy where they would get the most ‘bang for their buck.’  Patients and caregivers are experts on their own lives, so this is truly a partnership to help families continue the strategies that work for them and make changes to those that don’t,” explains Adrianne Alpern, Ph.D., a postdoctoral fellow in CHOC’s pediatric psychology department and a member of the CF team.

The CF Foundation offers the following general guidelines to help mitigate the impact of depression and anxiety:

  • Talk with someone, preferably in person.
  • Spend time with people who lift your spirits.
  • Avoid alcohol and drugs.
  • Continue with your CF treatment plan.
  • Practice good sleep habits.
  • Spent 30 minutes outside each day.
  • Make time for things you enjoy.
  • Be physically active.
  • Practice relaxation techniques.
  • Avoid caffeine and cigarettes.

Referral to the CHOC Cystic Fibrosis Center

When a child is diagnosed or is suspected to have CF, a referral to an accredited CF care center can ensure that the child gets diagnosed properly, lives a long and healthy life, and receives treatment based on the most advanced research available. With access to a full range of CHOC pediatric subspecialists, the CHOC Cystic Fibrosis Center offers a number of life-enhancing technologies and treatments.

CHOC’s multidisciplinary CF clinic includes a board-certified pulmonologist and gastroenterologist, respiratory and physical therapist, nutritionist, nurse specialists, social worker and a psychologist. An endocrinologist and other specialists are available to attend the appointment, if needed. Genetic testing and counseling can also be provided.

For appointments, please call the Patient Access Center at 888-770-2462 (888-770-CHOC).

[1] Quittner AL, Goldbeck L, Abbott J, Duff A, Lambrecht P, Solé A, Tiboshc MM, Brucefors AB, Yüksel H, Catastini P, Blackwell L, Barker D. Prevalence of depression and anxiety in patients with cystic fibrosis and parent caregivers: results of The International Depression Epidemiological Study across nine countries. Thorax. 2014;69:1090-1097. doi:10.1136/thoraxjnl-2014-205983

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