Cystic Fibrosis

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Cystic Fibrosis

Cystic Fibrosis Clinic Little GirlAccredited Cystic Fibrosis Center

The CHI St. Alexius Health Heart & Lung Clinic's Cystic Fibrosis Center is the only independent Cystic Fibrosis Center in North Dakota that is accredited by the Cystic Fibrosis Foundation.

Our Approach to Care

The Cystic Fibrosis Center adopted the care model developed by the Cystic Fibrosis Foundation. Our CF care center delivers effective and efficient healthcare for the chronic disease. Patients receive collaborate care from an interdisciplinary team of specialists.

Our dedicated care team includes:

  • Adult and pediatric physicians
  • Nurse practitioner and registered nurses
  • Dietitian
  • Respiratory therapist
  • Pharmacist, and
  • Social worker
About Cystic Fibrosis

Cystic fibrosis (CF) is an inherited chronic disease that affects the lungs and digestive system. According to the Cystic Fibrosis Foundation, about 30,000 people in the U.S. are living with CF. The disease causes the body to produce thick, sticky mucus which obstructs the airways of the lung leading to chronic infection and progressive lung injury. In addition, obstruction of the ducts of the pancreas impairs digestion and absorption of essential nutrients from the intestine.

Decades ago few children with CF lived into their teens. Now advances in research and treatments allow many CF patients to live into their 40s and beyond. In fact more than 45 percent of patients living with CF are now 18 or older.

Symptoms
People with CF can have a variety of symptoms, including:

  • very salty-tasting skin;
  • persistent coughing, at times with phlegm;
  • frequent lung infections;
  • wheezing or shortness of breath;
  • poor growth/weight gain in spite of a good appetite; and
  • frequent greasy, bulky stools or difficulty in bowel movements.

For more information on Cystic Fibrosis - click here
For more information on Cystic Fibrosis Clinical Care Guidelines - click here

Recent Story

New Cystic Fibrosis Therapy...read more!

About Cystic Fibrosis CenterWe believe in partnering with patients and families in an effort to promote health and longevity. We make every effort to assure that:

  • Our patients and their families are full members of the care team.
  • Children, adolescents and adults with CF will have normal growth and nutrition.
  • People with CF will receive appropriate therapies for maintaining lung function and reducing acute episodes of infection. Pulmonary exacerbations will be detected early and treated aggressively to return them to previous levels of lung function.
  • Our patients, their families and CF healthcare professionals are well informed and active partners in reducing acquisition of respiratory pathogens, particularly Pseudomonas aeruginosa (Pseudomonas) and Burkholderia cepacia (cepacia) complex.
  • People with CF will be screened and managed aggressively for complications of the disease, particularly CF-related diabetes (CFRD).
  • Patients and their families will be well supported by our CF healthcare professionals when facing decisions about transplantation and end-of-life care.

Cystic Fibrosis Foundation Accreditation
The Cystic Fibrosis Foundation is the world’s leader in the search for a cure for cystic fibrosis. CHI St. Alexius Health Heart & Lung Clinic’s Cystic Fibrosis Center has been a Cystic Fibrosis Foundation accredited center for more than 25 years. Our center undergoes thorough review by the CF Foundation’s Center Committee before it receives accreditation and funding. This review for accreditation occurs every year.

Newborn Screening for CF
Since Jan. 1, 2006, the North Dakota newborn screening panel of tests includes a screening for cystic fibrosis. While newborn screening should identify approximately 99 percent of infants with cystic fibrosis, an occasional child will continue to be diagnosed only when clinical manifestations suggestive of cystic fibrosis develop.

For more information on Cystic Fibrosis - click here
For more information on Cystic Fibrosis Clinical Care Guidelines - click here

Children’s Special Health Services, North Dakota Department of Human Services, may be able to assist families to pay for the testing to confirm diagnosis and treatment of cystic fibrosis. Please contact Children’s Special Health Services for more information at 701.328.2436 or 800.755.2714. To make an appointment at CHI St. Alexius Health Heart & Lung Clinic’s Cystic Fibrosis Center, call 701.530.7502 or toll free at 800.932.8848. If emergent care is needed, please immediately go to your nearest emergency and trauma center.

Every May, the cystic fibrosis community comes together for CF Awareness Month. Cystic Fibrosis is a genetic disorder that affects the CFTR protein which is responsible for regulating the flow of salt and fluids in and out of the cells in different parts of the body. When both parents are carriers of a CF mutation gene there is a 1 in 4 chance of the child having Cystic Fibrosis.

For more information on Cystic Fibrosis, click.

Here is Mandy's story.

What changes have been offered by your provider or the CF community to help you stay healthy? Mandy
The team at the CF Clinic have been like family. I’ve known them my entire life. As an adult with CF, it’s great to have a CF team you can talk to about your concerns. It can be hard to advocate for yourself at times. When things come up and you have to have a tooth surgery or any other health related procedure, the provider might not be as familiar with CF and it can be challenging to advocate for yourself. I’m learning to speak up and connect the CF team with the other health care providers I’m working with.

Growing up with CF decades ago, we didn’t know CF patients couldn’t be in the same room together. The last couple of decades that has been something we’ve been missing in the CF community. A social worker on the CF team at St. Alexius started a quarterly call on Zoom and I’ve been able to see people I haven’t seen in decades.Now I see everyone at the clinic quarterly and every three to four years for a tune up. As a kid, I would come in monthly for checkups and every three to four years for a tune-up.

How has CF affected your life? 
As a kid, I was always very healthy. I have the mentality that taking care of CF is like brushing my teeth – just something that must be done every day to stay healthy. As an adult, I’ve had to learn how to manage anxiety over medical procedures, and I’ve also learned how important it is to take care of my mental health and talk to a counselor.

Daily life typically includes two treatments a day. Every three to four years I need a tune-up to kill off the bacteria that is stuck in my lungs. When I’m having a tune-up, I have four treatments a day and IV meds every eight hours. Usually when I was a kid I would be in the hospital the whole time. Now I’m in for three days and then they send me home with a PICC line. It’s great to be home but it can also be a little stressful to manage the extra medical care on top of daily life. I don’t let CF stop me from living my life. I’ve been married for 19 years and live an active life. In 2019, I ran my first half marathon. Running and being active outside is a great stress relief for me. My life with CF has helped me meet the challenge of owning a company, writing books, and public speaking to help equip others with the skills they need to overcome their life obstacles.

What are the challenges you have faced? 
I like to live this life with CF in a way that I don’t think about it all the time. However, when I’m having a tune-up, I find that I think about it more because it requires more care due to the extra treatments and medication. That’s when I start to wonder whether going into the hospital is going to be more normal for me in the future as I get older. Hopefully, with hard work and diligence, it won’t be. But there’s always a chance. I really don’t know any different than this life of CF. Other people sometimes look at me like a victim. I surround myself with people who see me as a person and not a walking disease. There’s so much more to me than that.

How do you see your future and has this changed from 10 years ago? 
Ten years ago I went down a path of not doing my medicine. I took care of my health with holistic solutions only and it nearly cost me my life. I spent a month in the hospital in 2012 working hard to get back on track and the miracle is that my health was restored without a lung transplant. I’ve experienced the worst of CF and lived to tell about it. Now I share my story so that others will know how important it is to take care of themselves and stay diligent with their medicine as well as exercise and healthy nutrition.

Today the outlook for CF is so hopeful. I started Trikafta a little over a year ago and it’s a big leap forward. My body is absorbing nutrients differently. It’s like the opposite of struggling with weight. What I’m feeling in my clothes is really different. For a solid month, I said: these are not my legs. I’m working out and muscle mass is growing. I’m so thankful for my CF family. They are people I can count on. They are really there for me.

 

Here is Kyle's story.

What changes have been offered by your provider or the CF community to help you stay healthy? Kyle H
I grew up three hours away from the CF clinic. Every three to six months it was a trip. My parents and I would leave at 4 am for an 8 am appointment. We were there all day and head home around 6 or 7 pm. That was our routine because that’s where the best people were. Luckily for me my parents were willing to take me there. I had a double lung transplant in September of 2019. The city of Rolla - I grew up there 10 miles from the Canadian border and its six miles from where I teach now - they did a benefit for me the summer of my transplant. The CF Association in North Dakota matched whatever funds they raised. They had BBQ and silent auction and raised $30,000 in a town of 1,500.

How has CF affected your life? 
My treatments were about an hour each. When I was a kid, I had two to three a day. At age 17 or 18, I had to start doing four a day. I had a stubborn teenage phase. In college I didn’t want to do four treatments a day so it was more like one or two. It was a burden. If I wanted to go on trips I had to pack up all of my meds. I lived with three baseball players. I wanted to be able to do what they did.

I was listed on the transplant list on June 12, 2019. My lung function was at 12 percent. I’m a tough old bird. I was still walking. I was doing oxygen and I was on full time oxygen when I went into the hospital. I had labored breathing and I would tire out easily. I’d sit down on the couch and fall asleep really quickly. It was a sloth-like lifestyle. Since the transplant, now I’m just on anti-rejection medications. Life is a lot easier. In January, my dad, my brother and I took a trip to Texas. I kept feeling like I was forgetting something. I felt like I should set up a treatment. It took a long time to get used to the idea. For 32 years it was natural for me.

What are challenges you have faced? 
I wanted to be an elementary teacher and I started in elementary but switched to social studies. My prep hour was right before lunch so I could bring a treatment with me if I needed to. My students would ask “Are you sick today?” It became so normal. I didn’t want people feeling bad for me. A lot of students didn’t know I had cystic fibrosis. I wanted to be the normal guy.

Deb at the CF clinic started talking transplant when I was 25 or 26. I was hesitant. I was probably in denial. I always thought you should go out with the same organs you were born with. I just realized I wasn’t ready to give up. I wanted more of the possibility to see life in a new light. When you’re younger, you don’t realize how many people are in your corner and that you should take all of the support you can get. Know that your doctors and caretakers, the people who look out for you, they might get on your nerves, but they only want the best for you. At the end of the day, they know what’s best for you.

How do you see your future and how is it different than 10 years ago? 
Ten years ago if someone said “Hey do you want to go for a walk?” I would say “No, I’m good.” Now I’m walking every day, depending on the weather, I’ll go three or five miles with my faithful companion, my dog Rocco.

The future is bright for me now. Even two years ago, I didn’t see a future. Life has gotten a lot easier and it’s hard to believe that. It really took some time to get accustomed to it. For sure it’s different now. When I was at the University of Minnesota waiting for the transplant, one of the girls I grew up with (who also had CF) was in the same hospital. I read her obituary. It was quite a shock. It’s almost like guilt. I’m thankful for the opportunity I have and I want to make the most of it. The future is definitely different now.

You have to keep up the fight. Some days are harder than others, you have to stay positive. Don’t lose sight of where you want to be in life. Life is different with CF, but that doesn’t mean you can’t get to where you want to go. You have to have goals to achieve what you want.

 

Here is Katie's story.

What changes have been offered by your provider or the CF community to help you stay healthy?Katie W
The CF team is wonderful. They are like family to me. I have been with the same doctor for over 20 years and some of the CF team has known me since I have been a baby. They inform me about new medications, sometimes before they are made available yet, such as Trikafta (the CF “miracle” drug). We discuss the pros and cons together of how these new medications will fit into my daily life and benefit my health. Every clinic appointment (which occurs every three months or less), we fully discuss how I am physically and mentally and how I can continue to improve.

They also tell me about different programs that are available for financial assistance to help pay for my CF medications, equipment, and supplements. We also discuss insurance and if needed, someone from the CF team will get in contact with insurance companies to take the measures needed to help get any medications approved and running as smooth as possible.

The Cystic Fibrosis Association of North Dakota has helped me by assisting with a percentage of medication expenses, mileage to and from your appointments, and hotel costs. They have great fundraising events every year and I am very thankful we have the CFA of North Dakota.

How has CF affected your life?
I was diagnosed with Cystic Fibrosis within 3 months of being born. My parents did a great job of keeping me on a schedule and routine so I could stay healthy to be a kid like everyone else. My treatments are 45 to 60 minutes, two to three times a day, and sometimes four if I feel even the slightest cold starting. I also take around 30 pills a day and insulin for my CF related diabetes. My treatment time is my quiet time and I don’t know any different. Yes, sometimes it is difficult to take that time out of my day, but I know I have to do it so I can stay healthy. I am also a wife and mother. I have a 10, 8 and six-month old – two boys and a girl and have been married over 12 years. My family knows I need to keep myself healthy so I can give them 100% and be here for them. Mom with CF is normal for my children, they don’t look at me any different.

Exercise is a huge part of my life. I don’t exercise to lose weight (I still need to gain weight), but to keep my lungs healthy. Running is like a treatment for me, sometimes feels better than a treatment (but I never replace it for a treatment). I also keep myself busy and my children keep me young. I couldn’t sit around doing nothing for days, I would just decline physically and mentally.

also work 30 hours a week for the Burleigh County State’s Attorney’s Office. I have been there almost 13 years and they also know, for me to give 100% at work, I have to take the time for my health. I worked a year at home because of COVID and being pregnant during it, so my work is very flexible and considerate with understanding my health needs. I have always been more watchful of germs and I am a huge hand washer and keeping my distance. This is nothing new to the CF community.

What are challenges you have faced?
have to discipline myself to give 100% to my daily health schedule. Growing up, my parents let me be a kid, but they made it a priority to fit all my pill popping, treatments, high calorie meals, and rest in my day. I have always made sure to fit my treatments in between school, sports, and friends, all the way to now being a mother, wife, and worker.

Another challenge with having CF is how I can become easily exhausted. I never considered myself sick, but between the coughing, headaches, body inflammation, it takes a lot out on a person. I definitely have to set the time for myself to have a rest day and relax. I also have to make sure I am eating healthy, drinking enough fluids, and getting sleep. This all plays a part in staying as healthy as possible with CF. Approximately six years ago is when I started to have more of a decline in my lung functions and I could tell it was taking a toll on my body. I lost weight and energy (coughing is exhausting). Come to find out within a year, I had a new bacterial infection and ended up on IV antibiotics for over four months. I am a fighter, so with that and prayer, I gave it my all and got through it. Some days CF won, some days I won!

CF can also affect you emotionally and mentally. Turn to your support group, CF team, and each other. I have such a great support team of my friends, family, and work, along with other CFers going through the exact same challenges. I consider myself a positive person, always looking at the best of things, but everybody has their moments and days. I turn to my family and prayer a lot during those hard times.

How do you see your future and has this changed from 10 years ago?
I never considered an age as my life expectancy. I always told my grandma I would live to 100. Now I want to be here for as long as I can for my children and husband. I am 36 years old and I can see me enjoying the many milestones my children will have. I take a day at a time and want to be living my life to the fullest with my family and friends, but I know our life doesn’t end on Earth and we will be together in heaven.

A huge change that occurred in the CF community is when Trikafta became available in 2019. I have the gene mutation, which for me is one F508del mutation, that makes Trikafta available to me. Since I have started taking Trikafta in December 2019, I have been able to laugh without coughing, dance for more than 3 minutes, run faster and longer, have so much more energy with my children, gained weight, not had a “tune up” in over 1 ½ years (prior I was going in twice a year), increased my lung function, and the best part, became a mother again.

Every day is a new day. I’m just happy with how good I feel now. I just take a deep breath and give my life 100%. I always consider everybody has their cross to carry – mine just happens to be Cystic Fibrosis.