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FACT SHEET: Cystic Fibrosis (also known as “CF” and “mucoviscoidosis”)

Date of Publication: April 12, 2016
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Is the initiation of non-invasive dental hygiene procedures* contra-indicated?

  • No.

Is medical consult advised?  

  • No, assuming CF is well controlled and there are no concerns with respiratory status.

Is the initiation of invasive dental hygiene procedures contra-indicated?**

  • No. 

Is medical consult advised? 

  • See above. Additionally, if the patient/client is on high-dose non-steroidal anti-inflammatory drug (NSAID) therapy, medical input should be sought before treating oral pain with additional NSAIDs (such as ibuprofen). 

Is medical clearance required? 

  • No, not typically. However, if respiratory function is suspected to be severely compromised and/or unstable, medical clearance should be obtained. Also, persons who have undergone lung, heart, and/or liver transplants will be on immunosuppressive medications to reduce organ rejection, and such patients/clients should be medically cleared. Similarly, prolonged use and/or high doses of systemic (oral) corticosteroids may predispose to infection, and medical clearance (and potential dose adjustment) should be sought in these circumstances.

Is antibiotic prophylaxis required?  

  • No, not typically. However, antibiotic prophylaxis should be considered for patients/clients who are taking medications that induce immunosuppression (e.g., immunosuppressants for lung, heart, and liver transplants, as well as prolonged use and/or high doses of systemic steroids).

Is postponing treatment advised?

  • No, if CF is well controlled. Yes, if the patient/client is acutely ill or has respiratory compromise (or other co-morbidities) incompatible with the delivery of dental hygiene care; routine dental hygiene treatment should be postponed until resolution of the acute illness or better CF respiratory control is achieved.

Oral management implications

  • The dental hygienist can play an important role in promoting health in patients/clients with CF. Disease control, nutritional status, and status of the oral care regimen should be routinely assessed. 
  • Patients/clients with CF typically have medical evaluations every several months. Therefore, the patient/client’s medical history should be regularly updated, including questions regarding lung function, hospitalization, potential contraindications to dental hygiene care, and possible need for medical consult prior to continuing care delivery.
  • Patients/clients with CF typically follow a high-fat, high-carbohydrate diet, which can promote tooth decay. Prophylaxis with topical fluoride treatment and sealants should be considered, along with referral to registered dietitian to promote dietary choices that support oral health.
  • Use of sodium bicarbonate rinses or xylitol after consuming sugary drinks or snacks, and after using an inhaler/nebulizer, should be encouraged.
  • Appointment scheduling may need to be flexible, because patients/clients with CF may spend several hours per day undergoing breathing/respiratory therapy and keeping other health-related appointments.
  • Chair position should be semi-supine or upright to help improve the patient/client’s breathing and respiratory comfort; the upright sitting position aids in the clearance of secretions from the bronchi and trachea via coughing. Short appointments are indicated if there is significant compromise of lung function.
  • Persons with a known respiratory risk or a chronic pulmonary disorder, such as CF, can potentially aspirate septic material or pathogenic microorganisms from biofilm associated with periodontal inflammatory disease. Therefore, they should not be treated with ultrasonic scalers.
  • While many persons with CF have a chronic cough and may appear to be ill, they are likely not contagious to the dental hygienist or other patients/clients, unless they have signs/symptoms of acute respiratory tract infection such as fever and coryza1.
  • While CF is not an immunosuppressive condition (unless there is associated drug-mediated immunosuppression associated with transplantation), persons with CF, given their compromised respiratory function, should have their exposure minimized to others who have contagious respiratory illnesses such as influenza or the common cold.
  • A plan to assess alveolar bone health should be implemented due to the increased risk of osteoporosis.
  • Patients/clients with CF may incur bullae or bubblelike cavities filled with air in the lungs. Nitrous oxide may exacerbate this situation given the expansive nature of the gas. Therefore, medical consultation is advisable if N2O sedation is contemplated.
  • Patients/clients with CF may be on carefully calibrated high-dose non-steroidal anti-inflammatory drug (NSAID) therapy (such as ibuprofen) to reduce lung inflammation. Therefore, the oral healthcare provider should be cautious about treating oral pain with additional NSAIDs; medical input should be sought beforehand.

Oral manifestations

  • Enamel defects are common, including demarcated opacities, diffuse opacities, and enamel hypoplasia.2 
  • Poor nutrition may affect tooth growth and structure. As well, high calorie foods consumed by CF patients/clients often contain a lot of sugar, which, while beneficial for the treatment of CF, may promote dental caries.3 
  • There are conflicting studies regarding plaque levels in children with CF, with more recent studies showing no difference from healthy children (while older studies reported decreased plaque). Recent studies also suggest that children with CF have less gingival inflammation than controls. 
  • Persons with CF may be at reduced risk of periodontal disease (possibly related to certain antibiotic — e.g., azithromycin — penetration of the periodontal tissues and retention in the periodontal pocket). 
  • Patients/clients with CF tend to experience fewer gingival bleeding sites (possibly related to long-term antibiotic regimens) than persons without CF. 
  • Oral candidiasis occurs at elevated rates, due to long-term antibiotic regimens and the use of inhaled steroids, as well as from the effects of CF-related diabetes. 
  • CF affects all exocrine glands, including the salivary glands. However, the parotid glands are virtually pure serous (rather than mucus-secreting) glands, and thus their saliva is only minimally affected. Submandibular, sublingual, and minor salivary glands, by contrast, show greater changes in gland architecture and saliva composition. As well, the submandibular glands are usually enlarged and easily palpable.
  • Mouth breathing and anterior open bite are often seen in patients/clients with CF, given the association with chronic nasal and sinus obstruction.
  • Some medications used to manage CF — such as bronchodilators and corticosteroids — can contribute to oral disease. β2 (beta2) agonist inhalers (i.e., bronchodilators such as salbutamol) reduce salivary flow, resulting in xerostomia. In addition, β2 agonist inhalers lower plaque pH, cause unpleasant taste sensation, and are associated with increased prevalence of dental caries and gingivitis in patients/clients who frequently use them. Oral candidiasis, gingivitis, and/or periodontitis occur in some patients/clients who use inhaled corticosteroids for long periods of time or at high dose. The use of steroid inhalers can also result in throat irritation, voice impairment, cough, dry mouth, and, rarely, tongue enlargement. Patients/clients using inhaled medications are also at increased risk of dental erosion and periodontal disease.
  • Pancreatic enzyme replacement treatment can lead to irritation of the oral mucosa if the capsule contents are (inappropriately) held in the mouth.

Related signs and symptoms

  • Cystic fibrosis is an autosomal recessive (i.e., inherited) disorder that primarily affects Caucasians of Northern and Western European ancestry. Currently incurable, it is the most common lethal genetic disease affecting Canadian children and young adults. 
  • 1,431 Ontarians live with CF, of a total of 4,338 nationally, and about 100 children are newly diagnosed annually in Canada (about 1 in every 3,600 births), as are about 20 adults. While disease prevalence is holding steady, the median age is shifting upward given the advent of genetic testing, prenatal testing, earlier detection via newborn screening, and better treatments. 65% of all persons with CF in Canada are adults, and the median age of survival of Canadians with CF now exceeds 57 years.   
  • The disease affects all exocrine (secretory) glands, and it is characterized by the production of thick, sticky mucus that causes blockages in the lungs, pancreatic ducts, and other organs. This situation leads to an environment that facilitates the overgrowth of bacteria in affected tissues, particularly in the lungs and sinuses. 
  • Cystic fibrosis is a multi-system disorder that produces a variety of signs and symptoms including:
    • failure to thrive in infancy and childhood;
    • persistent cough with productive thick mucus;
    • wheezing and shortness of breath;
    • frequent respiratory tract infections, including pneumonia, bronchitis, and bronchiectasis4;
    • bowel disorders, such as intestinal obstruction or frequent, foul-smelling, oily, loose stools;
    • weight loss or failure to gain weight despite possible increased appetite;
    • salty tasting sweat; and
    • infertility (males) and decreased fertility (females).
  • Gastroesophageal reflux disease (GERD) occurs at an elevated rate in children, adolescents, and adults.
  • Episodic intestinal blockage is frequent in newborns and young children.
  • Complications and sequelae of CF in adolescence and adulthood include: CF-related diabetes (CFRD)5; liver disease (sometimes requiring liver transplantation); distal intestinal obstruction syndrome (i.e., build-up of stool in the intestinal tract secondary to mucus plugging); osteoporosis (associated with poor vitamin D absorption, poor nutritional status, physical inactivity, and corticosteroid therapy); nasal polyps (resulting in bilateral nasal blockage, runny nose, decreased sense of smell, facial pain, congestion, and, less frequently, nose bleeds); gallstones; pancreatitis; rectal prolapse (caused by frequent coughing and defecation problems); pneumothorax; and clubbing of the fingers (which is indicative of continual poor oxygenation of the blood). Depression and anxiety are also common. 
  • The five main approaches to CF treatment are nutritional repletion (which includes pancreatic enzyme replacement therapy, fat soluble vitamin supplementation, and high-fat, high-carbohydrate diet); clearance of airway obstruction (via chest physical therapy, exercise, and bronchodilator medications); addressing airway infection (via ad hoc antibiotic treatment); suppressing infection (via long-term antibiotic prophylaxis); and CFTR6 modulator therapy.7  
  • For some persons with advanced CF, organ transplantation may be an option. Double-lung, heart-lung, and liver transplants are considerations for CF patients/clients with progressive disease.
  • All newborns in Ontario are screened for cystic fibrosis via their newborn blood-screening test. If a physician suspects a patient/client has CF, a “sweat chloride test” (to detect elevated salt content) may be administered, genetic testing may be undertaken, and a test for the presence of enzymes in the intestine can be performed. Without early detection and treatment, irreversible damage to the lungs and digestive system is likely to occur.
  • There are 12 specialized, hospital-based CF clinics (paediatric, adult, and combined) in Ontario, and an additional 29 nation-wide. 
  • Respiratory failure is the most common cause of death.

References and sources of more detailed information


Date: November 3, 2015
Revised: May 29, 2020; July 10, 2024


FOOTNOTES

1 Coryza (or rhinitis) is irritation and inflammation of the mucous membranes of the nose. Common signs/symptoms are nasal congestion, runny nose, sneezing, and post-nasal drip.
2 Metabolic insult in enamel development related to the cystic fibrosis transmembrane conductance regulator (CFTR) may lead to the enamel defects in the postnatal period. Also, historically, there was a higher prevalence of enamel hypoplasia and tooth discolouration related to tetracycline therapy for pulmonary exacerbations. However, newer alternative antibiotic regimens have reduced the risk.
3 Paradoxically, some studies have concluded that CF children had lower caries prevalence than control children, and few studies report increased caries risk in CF patients.
4 Bronchiectasis is characterized by enlargement and “pocketing” of the bronchi/bronchial tubes, in which mucus collects and bacteria breed, thereby leading to recurrent lung infections and cumulative damage to the airways.
5 33% of adults have CF-related diabetes.
6 CFTR = cystic fibrosis transmembrane conductance regulator
7 Cystic fibrosis is a disease whose management is in transition. CFTR modulator therapy targets the underlying defect in the CFTR protein caused by mutations of the CFTR gene. As of 2021, nearly 25% of persons living with CF in Canada were treated with highly effective triple-combination modulator therapy (elexacaftor/tezacaftor/ivacaftor). Building on the therapeutic success of other CFTR drugs, triple-combination treatment is an option for an increasing number of patients/clients with specific gene mutations, with benefit potentially accruing to an estimated 90% of patients/clients with CF.


* Includes oral hygiene instruction, fitting a mouth guard, taking an impression, etc.
** Ontario Regulation 501/07 made under the Dental Hygiene Act, 1991. Invasive dental hygiene procedures are scaling teeth and root planing, including curetting surrounding tissue.