Adult Cystic Fibrosis Clinic - health support services

Information on patient services offered by the Adult Cystic Fibrosis Clinic at St. Paul's Hospital.

St. Paul's Hospital building

Overview

Consistent inpatient and outpatient care by a specialized CF team, as well as good self-care, have been important in improving the length and quality of life for people living with CF.  

The sections below provide information on patient services offered by the Adult Cystic Fibrosis Clinic in Vancouver, as well as other supportive, patient-related services.

Nursing

Dietary Services

Maintaining a healthy weight and optimizing your nutrition has been shown to be closely related to the best outcomes in cystic fibrosis (CF). 

This can be a challenge as people with CF commonly have to deal with pancreatic insufficiency, diabetes, bowel obstructions, and chronic infections. The nutritional needs of a person with CF can be up to twice the amount of daily calories than someone who does not have CF. The CF Canada website provides some general nutrition information.

Below are some of the nutrition-related concerns in someone with CF.  

Physiotherapy

The CF physiotherapist provides consultation and support to patients regarding airway clearance techniques and equipment, exercise and CF-related musculoskeletal issues.

The physiotherapist is available at outpatient clinic appointments and via telephone or email in addition to seeing patients who are admitted to hospital for treatment of exacerbations. Individual consultations outside of regular clinic hours are also available for those who would like more assistance with learning new airway clearance techniques, developing an effective exercise program or improving adherence to their treatments for better overall health.

Equipment FAQ

Exercise and CF

“If exercise could be purchased in a pill, it would be the single most widely prescribed and beneficial medicine in the nation.” – Robert H. Butler

People with CF have a lower risk of heart disease and hypertension, but physical activity is still an important part of the treatment plan. Research has shown that exercise capacity is the best predictor of long-term survival in cystic fibrosis. Exercise can help with airway clearance and glycemic control, and is very important in the development and maintenance of healthy bones. There  are seldom any contraindications to participation in exercise for people with CF. 

Starting an exercise program

There are three components to any well-balanced exercise program:
 

How much physical activity is enough?

The Canadian physical activity guidelines call for 150 minutes of moderate-intensity aerobic activity per week, plus two sessions of strength training. Balance and flexibility exercises should also be incorporated. If these guidelines are challenging to meet, the  Activity Pyramid is a helpful tool for learning simple ways to get more daily activity:

Please ask your CF Clinic physiotherapist if you have more questions about exercise or would like help in developing a program that suits your needs.

Exercise FAQ

Pharmacy

Pharmacists at St. Paul's Hospital are responsible for ensuring safe and appropriate use of medications, and to tailor therapy to meet the needs of each individual patient to optimize their health outcomes and quality of life.

Medication

How to mix inhaled medications

Tobramycin and colistin are commonly used and administered to help control the growth of Pseudomonas in the lungs of people with cystic fibrosis. Colistin and some forms of tobramycin require you to prepare each dose.

Vaccinations

The CF Clinic strongly recommends that patients remain up-to-date on all vaccinations to protect against respiratory illness, which includes influenza vaccine, COVID-19, and Streptococcus pneumoniae (the most common bacteria responsible for community-acquired pneumonia. Please speak to the CF Clinic team to learn more about these vaccinations and which ones you would be eligible for. 

For all other vaccinations, please discuss your eligibility with your primary care provider.

Social work

CF is a multi-system condition which can have a significant impact both physically and psychologically on people living with CF, as well as their families.

This is particularly true as people live longer, and better, with CF. There are many life transitions, milestones, and challenges to manage throughout life. These include employment, higher education, having a family, travelling, and the need for various supports through times of illness.

The CF social worker can assist people with a variety of these issues through psychosocial supports and advocacy, and works closely with other members of the CF team to promote quality of life for people living with CF. The CF social worker sees patients during clinic visits and can also be contacted directly.

Drug and Medical Coverage

Living with CF means keeping up a health care routine which usually includes medications, therapies, clinic visits, specialist visits, and at times hospitalization. This is why it is so important to make sure you have drug and medical coverage in place. All residents of BC need to be registered with both the Medical Services Plan (MSP) and Fair Pharmacare to ensure they are properly covered for their medical needs.

BC Cystic Fibrosis (CF) Subsidy Program (formally known as the CF Grant)

People living with CF in BC have access to a CF Subsidy administered by CF Canada.  This grant assists with the Fair Pharmacare deductible for medications, as well as with some equipment,  travel, and accommodation costs related to clinic and hospitalization. The Subsidy is administered through the CF Canada office in Toronto.

You can find the information and application package here.

Disability Benefits and Employment Insurance Sickness Benefits

As a person living with CF you may need to be off work for periods of time for treatment or hospitalization.

For some people living with CF there may come a time when it is necessary to stop working completely in order to focus on health, rest, and CF treatments. It is important to know that there are a variety of benefits available that provide either short term or long term support if you are unable to work because of your health.

Remember, a diagnosis of CF does not automatically mean you qualify for any of these benefits. You will qualify based on symptoms and/or treatments that are severe enough to interfere with your daily activities either periodically or all the time.

Note:  Many benefit and vocational support programs use the term "disability" to indicate supports and resources for people living with chronic conditions such as CF. Not everyone living with CF relates to this term or is comfortable with it. However, these programs and services can be very helpful to people living with CF and it is important to be aware of them and use them when you need to. 

Vocational and Educational Resources

Travelling with CF

Many people living with CF travel successfully. Some even go to school or work overseas. The key is to make sure you are prepared so you can practice good CF care when you are away from home and ensure you have proper medical coverage in case you need to see a doctor or be hospitalized in another country.

The CF clinic would be happy to provide you with a travel letter indicating the medications and equipment you will be traveling with. Just call the main clinic office.

CF Canada has a great information brochure on traveling successfully with CF.

Gas rebate programs

Supportive Resources

There are many supportive community resources that CF patients can access. These are constantly changing and so it is important to ask your CF social worker if you have a need so they can try to connect you to a current resource. Here are just a few examples, but there are many more.

Mental Health

People living with CF have to manage their condition along with all the other everyday challenges of life, which can impact mental health. The Adult CF Clinic at St. Paul's Hospital views mental health as an integral and important  part of overall health and CF care, equal to physical health. As the World Health Organization (WHO) states, “There is no health without mental health.” The clinic aims to always provide safe space and time to discuss mental health issues and offer resources and supports when needed. Annual anxiety and depression screenings are offered to all CF patients and there is a social worker,  psychologist, and psychiatrists attached to the clinic. CF Care BC has put together a Speak to the social worker or any other member of the CF team if you are struggling with your mental health or feel you would benefit from a referral to CF psychiatry or psychology.