Cystic Fibrosis Clinic
We provide treatment to children and youth living with Cystic Fibrosis.
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.
Our philosophy is family-centered care. We strive to provide the best care possible.
Care We Provide
- The Cystic Fibrosis 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, Ear Nose Throat, Gastrointestinal, 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.
What to Bring
- Your child’s Health Card. The Ministry of Health and Long Term Care requires us to validate your health card at every clinic visit.
- Any relevant x-rays or letters from your child’s doctor or pediatrician.
- Any notes or questions that you may have.
- List of current medications and supplements your child is taking.
- Your PEP device for regular re-assessment of technique and settings with the clinic physiotherapist.
When you arrive, please check-in at the reception desk. The Business Clerk will register you in the electronic system so that your team will know that you have arrived.
Note: If your child 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.
Our team includes physicians trained to provide care to patients with Cystic Fibrosis, nurse, physiotherapist, dietitian, child life specialist, and social worker.
- 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 (905-521-2100 ext. 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.
About Cystic Fibrosis
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.
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
- 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
Any referrals may be faxed 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.