Heart Health

CFReSHC CF-SRH Resource Guide by Patients for Providers and Patients

 

Quick Clinic Checklist

 

SRH Guide Contents:

In this section:

Key

For Providers

 

For Patients

 

For Patients and Providers

 

Introduction

As CF patients age and therapies improve, cardiovascular complications are becoming more prevalent among the population. With the increasing rates, researchers and clinicians are paying more attention to these co-morbidities.[1] The emergence of heart disease as a CF issue now demands a wider approach to CF care,  including the CF-specific management of heart disease and the prevention of age-related CF conditions. 

 

Several factors contribute to the increased risk of heart disease in individuals with cystic fibrosis:

  1. Chronic systemic inflammation:  People with CF have chronic and systemic inflammation, which can contribute to the emergence of heart disease.[1] Plaque buildup and CFRD can also make CF patients more susceptible to Coronary Vascular Disease (CVD).[1] 
  2. High-sodium diet: Because people with CF lose more salt when they sweat than the average person, clinicians have historically recommended that patients eat a high-salt diet.[2] Over time, a diet high in sodium may contribute to a higher risk of cardiovascular disease.[1,3]  
  3. Obesity: With the use of CFTR modulator therapies among CF patients, there has been a notable increase in the prevalence of obesity. Modulator therapy can make patients hungry, resulting in significant weight gain.  The latter is a known risk factor for heart disease.[1,3] 
  4. Mental health disorders: The high prevalence of anxiety, depression, and post-traumatic stress disorder (PTSD) among CF patients may indirectly impact cardiovascular health.[1] 
  5. Cystic Fibrosis Related Diabetes (CFRD): CFRD affects over half of the CF population. As a person with CF ages, the risk of developing CFRD increases.[1] 
  6. Smoking: Though not recommended to CF patients, smoking, vaping, or participating in recreational drugs of any kind can put unnecessary stress on your heart.[3]  
  7. High blood pressure: High blood pressure can damage the arteries, decreasing the amount of oxygen delivered to an individual’s heart. This is a known risk for heart disease.[1]

 

Priority Questions on Parenthood for Providers and Patients to ask during a Clinic Visit:

  1. How many calories are you eating per day? [Patients can work with a dietitian to determine how many calories they consume per day, if they do not know already.]
  2. How much salt (mg) are you eating per day? [Patients can work with the dietitian to determine how much salt they are consuming each day, if they do not know already.]
  3. How much exercise do you get per day?
  4. Do you smoke/vape or participate in recreational drugs? 
  5. What is your CFRD status?
  6. Modulator status? 
  7. Screen patients for anxiety, depression, or other mental health concerns. 
  8. Do you currently work out?
    1. If not, would you like to know how exercise can lower your risk of heart disease? 
    2. Suggest an exercise program, or for those with advanced lung disease, suggest pulmonary rehab.
  1. Am I at risk for heart-related issues due to my CF? 
  2. What can I change today to lower my risk of heart disease? 
  3. What is the best way to start an exercise program? 
  4. How do I measure my salt intake?
  5. How can I track my calorie intake? 
  6. What are some ways to improve my mental health, and how will this help my heart health?

What can a person with CF do to decrease their risk?

 

Individuals with CF can ask their CF Pulmonologist and/or CF Dietician about how they can decrease their risk of heart-related complications. Their care team’s advice will be individualized, but some general suggestions are delineated below. 

Exercise Regimen

Implementing a structured exercise program is crucial for maintaining cardiovascular health. 

  • Patients can work with their care team to develop an appropriate exercise plan.
  • Patients can inquire about the availability of a physical therapist at their CF center. This provider can offer some helpful information about how to design an exercise program.
  • Patients with CF can ask their CF doctor if it is appropriate to start their fitness journey by engaging with a structured, pulmonary rehab program.
  • Patients with CF can use a fitness tracker to make sure they are exercising regularly.

Nutritional Consideration

Patients with CF can talk to their CF pulmonologist and/or registered dietitian about their calorie and salt intake.  Recommendations by the care team will be dependent upon whether a patient is on modulators or not.

CFRD Management

Individuals with CF-related diabetes (CFRD) should:

  • Maintain close communication with their CF pulmonologist or endocrinologist to ensure their CFRD goals are met.
  • Check their blood sugar as often as their care team recommends. 
  • Confirm they are taking your CFRD medications as prescribed. 

 

Mental Health Care

Individuals with CF should prioritize their mental health.  To do so, they can:

  • Schedule regular follow-ups with their CF pulmonologist, CF social worker, or other mental health professional to address any mental health concerns. These can include, but are not limited to, anxiety, depression, or post-traumatic stress disorder (PTSD).
  • Confirm they take all medications as prescribed.

Lung Transplant

Individuals who have undergone a lung transplant could be at increased risk of heart-related complications:

  • Researchers have extensively noted the presence of atrial arrhythmias in people who have undergone lung transplantation.[4] 
  • Researchers and clinicians have described post-operative cardiovascular complications, like pulmonary artery stenosis and/or kinking, anastomosis complications, pulmonary embolism, and other vascular abnormalities.[4]
  • Studies have shown that patients with end-stage CF may have right ventricular abnormalities associated with hypoxemia and hypercapnia.[4]

 

People who have undergone a lung transplant can consult their transplant pulmonologist to learn how to decrease their risk for heart-related complications.

 

Peer-to-Peer Advice: 

  • Make sure to have a form of cardio exercise in your routine (e.g., walking, biking, stationary bike, hiking, tennis, team sports, etc.)
  • Keep track of your blood pressure. If possible, buy a small BP machine to have at home to check periodically.  Address high blood pressure by consulting a cardiologist or internist.
  • There are free apps that can track calories, weight, and fitness (separately or in combination).  Consider using them to monitor preventive measures to protect your heart health.

 

 

 

Works Cited

  1. Despotes A. CF and Cardiovascular Disease: Listen to your Heart! https://cfri.podbean.com/e/alex-d-video/.
  2. Cystic Fibrosis Foundation. Minerals. https://www.cff.org/managing-cf/minerals#:~:text=Not%20getting%20enough%20salt%20can,especially%20during%20hot%2C%20humid%20weather.
  3. Centers for Disease Control. Heart Disease Risk Factors. https://www.cdc.gov/heart-disease/risk-factors/.
  4. Poore TS, Taylor-Cousar JL, Zemanick ET. Cardiovascular complications in cystic fibrosis: A review of the literature. J Cyst Fibros. 2022;21(1):18-25. doi:10.1016/j.jcf.2021.04.016.

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