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Cystic Fibrosis

Contact Us:

2G Clinic, McMaster Children's Hospital

Phone: 905-521-2100, ext. 78515. 
Fax: 905-521-5056

Conditions We Treat:

Cystic Fibrosis (CF) is one of the most commonly inherited disorders in childhood, affecting approximately 1 in every 3,200 children born in Canada.  CF occurs in all racial groups with the highest incidence in Caucasians.  CF is a multi-organ disease affecting mainly the lungs and digestive system.

Patients with CF are born with a genetic defect that causes a malfunction of the cystic fibrosis transmembrane conductance regulator protein (CFTR), a chloride channel in the cell membrane that plays an important role in maintaining water and salt balance across many body tissues like sweat glands, lungs, liver, pancreas and reproductive organs.  Due to this malfunction, there is a build-up of thick mucous which makes it difficult to clear bacteria and leads to cycles of infection and inflammation which over time, damages the lungs.  As a result, people with cystic fibrosis must follow a daily routine of physiotherapy to keep their lungs clear of mucous and infection.  In the digestive tract, CF makes it difficult to digest and absorb adequate nutrients from food eaten.  Most people with CF must swallow digestive enzymes (on average 20 pills a day) with every meal and snack to maximize absorption of their food and to gain weight.

Genetics

CF is inherited in an Autosomal Recessive pattern, meaning in order to be affected with CF, an individual must have 2 copies of a CFTR mutation (one from each parent).  Approximately 1 in 25 Canadians carries a defective version of the gene responsible for CF.  Carriers do not have and can never get CF.  However, when two carriers have a child, there is a

  • 25% chance the child will have CF

  • 50% chance of the child being a gene carrier

  • 25% chance they will not have the faulty CF gene

Facts

  • more than 1,900 mutations have been identified

  • nearly 90% of people with CF in Canada carry at least one copy of the most common CF-causing mutation – DF508

  • treatments focus on nutrition, physiotherapy, exercise, and medication

  • early diagnosis and treatment for CF improves weight, height, and lung function which optimizes health and increases longevity

  • 86% of people with CF must take pancreatic enzymes to digest food and absorb nutrients

  • in the 1960s, most children with CF did not live long enough to attend kindergarten, but today, half of all Canadians with CF are expected to live into their 40s and beyond

  • approximately 4,000 people with CF attend one of the 42 specialized cystic fibrosis clinics across Canada, McMaster being one of those specialized clinics

  • ongoing research focuses on finding a cure and together we can make this happen

What Services Do We Provide?

Currently visits are scheduled every 3 months with an increased frequency of appointments for infants and toddlers.  The earlier 1300h appointment times are booked for infants and small toddlers.  For all families, extra appointments are added when there are health issues or concerns. 

The McMaster CF clinic  works in conjunction with the RSV clinic to offer all patients less than 2 years of age RSV immunization.  This government funded immunization program entails monthly visits from November to April.

Our routine clinic practice has most patients that are over 6 years of age undergoing spirometry (breathing test) prior to each clinic visit.  The pulmonary function laboratory for breathing testing is on the third floor of the hospital--3U.  Sputum or throat cultures are done at clinic visits and these are specially processed by our lab.  Patients are also scheduled for yearly chest x-rays and blood-work. 

Patients aged 10 years and older also do yearly oral glucose tolerance testing (OGTT) blood-work.  The clinic’s Child Life Specialist can assist children and adolescents coping with blood tests as needed.

The Children’s Exercise and Nutrition Centre (CENC) provides exercise testing on a regular basis.  Clinic appointments for exercise are coordinated with CF clinic appointments. 

Appointments with other services and clinics are coordinated when possible.  It is often dependent on which days these clinics operate.  This may include appointments with Diabetes, ENT, G.I. and Adolescent Medicine.

Newborn Screening Centre:

McMaster Children’s hospital is one of 5 referral centers in the province for newborn screening.  CF newborn screen positive results are followed up in the CF NBS clinic on Thursdays.

Clinic Location:

The hospital is located at 1200 Main St. W., Hamilton, ON, L8N 3Z5.

2G Clinic, McMaster Children's Hospital

2nd Floor, Red Section

Hospital Map

What Happens When We Arrive?

When you arrive to the clinic please check in with the Business clerk at the reception desk. The business clerk will let the team know that you have arrived.

Staff will do their best to make sure you are seen at your appointment time.  If you feel you have been waiting a long time please speak with a business clerk at the reception desk.

Referral Process:

Any referrals can be faxed directly to the clinic as noted on the referral form.

Urgent Referrals:

The clinics are not for emergencies. If your child needs to be seen on an urgent basis, please contact your family doctor or go to your local emergency department. 

Clinic Hours:

(closed on holidays)

Monday 1:00 p.m. - 4:00 p.m.

Tuesday 9:00 a.m. -12:00 p.m.

Thursday 1:00 p.m. - 4:00 p.m. (Newborn Screening Only with sweat testing)

Changing Your Appointment:

Please contact the 2G clinic #78515 if you need to change or cancel your child's appointment.

Important:

If the patient is ill with any antibiotic resistant organism, MRSA, a communicable disease (such as Chicken Pox), or a respiratory infection, please notify reception staff prior to your scheduled appointment.

What Should You Bring to the Clinic?

  • List of medications and supplements you are taking

  • Your PEP device for regular re-assessment of technique and settings with the clinic physiotherapist

  • Health Card for your child - the Ministry of Health requires us to validate your health card at every clinic visit

  • Any relevant x-rays or letters from your doctor or pediatrician

  • Any notes or questions that you may have

Pharmacy Orders:

  • Please call in your request for orders of routine medications (those used on a regular basis such as enzymes, vitamins, Ventolin, etc.) at least one week in advance of your clinic visit to pharmacy #75019

  • This will provide time for these orders to be ready the day of your clinic visit.

  • If you wait for the day of your clinic visit to order your medications then your full order may not be available at that time

Interdisciplinary Team:

Physician:

A specialist trained to provide care for patients with Cystic Fibrosis.

  • develop comprehensive multi-system CF care plan

  • directs Best Practices in CF care in the clinic

  • maintains current CF standard of care guidelines

  • works with your  primary care doctor (family doctor / pediatrician) ongoing basis

  • Works with patients and families and all CF team members

  • collaborate with other specialty physicians when needed

  • directs quality improvement initiatives in the CF clinic

  • educates Residents and Health professionals on CF care

Nurse:

A nurse that specializes in CF care.

  • CF program coordinator working with all members of the CF care team to ensure that the CF program runs smoothly

  • primary contact to address your concerns, answer questions or direct you to the right CF care team member when discussing a specific part of your treatment plan.

Physiotherapist:

  • Work with the family and child to develop a customized treatment plan for airway clearance, movement, therapeutic exercise and the application of technological equipment along the continuum of childhood

  • Provide ongoing education and support to prevent and / or maintain respiratory, neuromuscular, musculoskeletal and cardiovascular systems that may affect your child

  • Assist with treatment adherence challenges to help maintain health and wellbeing for the entire family

  • Work with the family and child to understand critical infection control procedures

 Dietitian:

  • Optimal nutrition has been shown to be vital in maintaining good health in CF

  • Advise and educate patients and care givers about the principles of nutritional management in CF

  • Monitoring growth and ensuring each child is eating well enough to grow properly through each stage of life

  • Prevention of vitamin and mineral deficiencies

  • Help manage pancreatic insufficiency issues and adjust enzyme dosing when required to help with the digestion and absorption of  food

  • Communicating and working with the other members of the CF interdisciplinary team to help keep each child healthy and to manage issues on an individual basis

  • Counselling families regarding feeding difficulties

  • Helping patients manage CFRD from a nutrition standpoint

 Child Life Specialist:

  • Helps you, your child and family adjust to life with Cystic Fibrosis

  • Helps your child understand Cystic Fibrosis and cope with treatment through medical play

  • Provides ways for your child to safely express his or her feelings through play

  • Helps your child overcome challenges they may face as they develop and grow

  • Understands how children develop and how family members interact etc

  • Helps children cope with tests and procedures

Social Worker:

  • Assist the patient and family adjust to life with Cystic Fibrosis

  • Facilitate family and team communication

  • Assist with patient education (i.e. provide education to patient/family on impact of illness on family and friend relationships)

  • Explore and advocate for resources and services

  • Provide crisis intervention and assist in mediating conflict

  • Assist patients and families with developing healthy coping skills and strategies

Our Philosophy:

Our philosophy is family- centered care. We strive to provide the best care possible

Resources:

Information and Support:

Cystic Fibrosis Canada

Kids Health Website

Ontario Disability Support Program

Disability Tax Credit Forms

Introduction for Parents

Taking Charge of Your Health

Hamilton Health Sciences • Hamilton, Ontario • 905.521.2100

Disclaimer: Hamilton Health Sciences (HHS) offers Google Translate to better facilitate access for our community. However, HHS makes no claims regarding the accuracy of translations. Any and all health information should be verified by a health care professional.