«cycles of neutropenia for years without problems, while others may have aphthous ulcers with every neutropenic period. For many patients with ...»
Oral Hygiene for Children with Barth syndrome
Shelley Bowen, BSF President
Richard I. Kelley, PhD, MD
Shelia Mann, RDH
Good Oral hygiene is very important for the health of a Barth
individual, but is often difficult to maintain due to complications caused
by neutropenia. Many cases of neutropenia are diagnosed because of
chronic or recurrent dental problems. Neutrophils serve as the first
line of defense to bacterial infections when a break in a cutaneous or
mucosal barrier (inner tissue lining) occurs.
Neutropenia can be cyclical or chronic and can be serious for those who have Barth syndrome. Some individuals may pass through many Oral Hygiene Fact Sheet cycles of neutropenia for years without problems, while others may have aphthous ulcers with every neutropenic period. For many patients with cyclical neutropenia, there is a predictable pattern of oral ulcers, cervical lymphadenopathy (swollen lymph nodes in the neck) and painful gingivitis about every three weeks, coinciding with the low point in the neutropenic cycle. Gingivitis, periodontal disease, and the (1) Inflammation of the gum loss of permanent teeth are common problems across the spectrum of tissue causes recession from neutropenic syndromes. Most patients with an absolute neutrophil the tooth, which ultimately results in (2) bone loss.
count (ANC) persistently less than 500 have problems with gingivitis and increased periodontal disease, despite good efforts at oral hygiene. These problems can be attributed to a loss of the natural bacteria-killing function in the oral tissues caused by the deficiency of neutrophils, which normally migrate to the gingival crypts where bacteria tend to proliferate. As a result, good dental hygiene is extremely important in overall care of a Barth individual to decrease the potential for infection.
The quality of life of the patient with severe chronic neutropenia can be severely compromised by chronic oropharyngeal inflammation, recurrent skin and soft tissue infections, or, for more severely affected individuals, repeated hospitalizations for treatment of fever and deep tissue infections. For essentially all patients with severe neutropenia, recurring episodes of fever and illness with lost days from work and school are chronic problems, despite antibiotic treatment.i
Treatments and Pain Relief for Mouth Ulcers:
Always consult with a physician before administering any of these medications
• To reduce inflammation, your physician may prescribe topical or oral corticosteroids.
Kenalog in Orabase is a topical corticosteroid commonly used.
• A prescription of 2% viscous Xylocaine solution may be prescribed and applied directly to the sore with a cotton swab. After the sores are dry, apply Zilactin medicated gel, which can be purchased in any pharmacy.
• A prescription of Maalox(one part), Benadryl (one part), and Viscaine Xylocaine (one part) may be prescribed. Mix then rinse mouth and spit out solution.
• Colgate Orabase Soothe-N-Seal Gel (a liquid formula with applicator that forms a barrier to promote natural healing)
• Colgate Orabase with Benzocaine (temporary relieves pain, and shields from further irritation)
• Colgate Peroxyl rinse (antiseptic rinse that has bubbling action to clean and alleviate discomfort to promote healing)
• UlcerEase Anesthetic Mouth rinse (or similar product). Follow directions listed on the product.
• Warm salt water rinses (1/2 teaspoon salt in 1/2 cup of water)
• Apply hydrogen peroxide (1 part hydrogen peroxide and one part water with a cotton swab) on the canker sore. Then dab a small amount of Milk of Magnesiaon the ulcer 3-4 times a day. This is not only soothing, but also appears to speed healing.
• Avoid hot, spicy, salty, or citrus foods and carbonated beverages to minimize discomfort.
• Take a good multi-vitamin with minerals and eat a well-balanced diet.
• Mouthwash will reduce particulate matter and bacteria in the mouth. Use non-irritating neutral washes, or Hydrogen Peroxide. The bottle will have instructions for a ½ water and ½ Hydrogen Peroxide mixture.
• Avoid commercial mouthwashes and products that contain alcohol, which are very drying.
• Prescription mouth rinses like Peridex or PerioMed may be recommended by your dentist, if needed.
• Avoid toothpastes that contain whitening or tarter control agents. These often have more fluoride or abrasives, which tend to burn or make the gums tingle and can trigger a mouth ulcer to begin. A low-abrasive toothpaste without whitening properties or even a child’s toothpaste sometimes works best.
• Rembrandt promotes a product that claims that regular use limits the appearance of aphthous ulcers. However, there have been no studies other than those produced by the Rembrandt company to support this claim.
• Additional recommendations for toothpaste:
Dental Care for the Heart Patient There are specific aspects of dental care that could, in some patients, affect the heart. Dental procedures with patients who have a heart condition should be taken very seriously. Patients with Barth syndrome should advise their dentists of their condition.
Most dentists will ask a patient to complete a checklist prior to any dental care. Patients with heart murmurs, mitral regurgitation, or artificial heart valves are at increased risk of developing endocarditis, a serious infection of the heart valves, and must take precautions prior to any procedure that could introduce bacteria into the bloodstream. The patient with Barth syndrome should consult with his physician for recommendations on the use of prophylactic antibiotics with dental procedures, including routine dental repairs and cleanings. Many dental procedures require going below the gum line and thereby provide an opportunity for bacteria to enter the blood stream. Standard dental x-rays alone do not require antibiotic coverage.
The guidelines of the American Heart Association for endocarditis prophylaxis (prevention of an infection of the heart and valves) should be followed for people at increased risk for this infection, which includes all individuals with neutropenia. A wallet card can be downloaded from http://www.americanheart.org/downloadable/heart/1023826501754walletcard.pdf.
Heart Medication Precautions & Dental Treatment Because patients with heart disease often are on multiple heart medications, the potential risk from drug interactions during dental treatment must be considered. For example, if adrenaline-containing local anesthetics are administered to patients taking heart medications such as beta-blockers, a drug interaction could induce a steep rise in blood pressure, which could trigger cardiovascular complications. During these steep rises in blood pressure, disturbances of cardiac rhythm could also be precipitated, and the use of adrenaline or a related vasoconstrictor should be considered very carefully.
Oral reactions to prescribed drugs, including altered taste, impaired salivary function and gingival hyperplasia (gingival overgrowth) may be seen in cardiac patients. Altered taste, typically described as ‘metallic’ may be noted. If an offending drug can be identified, it may be possible (in consultation with your physician) to discontinue it or give an alternative.
While abnormal growth of the periodontal tissue is mainly associated with plaque-related inflammation, some heart drugs have been implicated in causing gingival overgrowth, which may be brought to the dentist’s attention because of pain, bleeding, or undesirable appearance.ii Why is preventive dental care so important for Barth syndrome Individuals?
Individuals with Barth syndrome fall into a "high risk" category with regard to infections due to neutropenia. Good dental hygiene is essential in the overall Barth care program to reduce the potential for infection in the neutropenic patient. Because of the risk of endocarditis (infection of the inner surface of the heart), people with Barth syndrome should keep their teeth as healthy as possible to minimize the number of restorative dental procedures needed. Because periodontal disease (gum disease) provides a breeding ground for harmful bacteria, keeping gums healthy by practicing good oral hygiene is essential, and routine dental visits are a must! More frequent cleanings (2-4 times per year) at your dental office may be required to help maintain optimal oral hygiene.
What is Periodontal Disease?
Periodontal disease affects the periodontium (the supporting structures of the teeth). The cause of this disease is multifactorial, but the presence of bacteria in plaque certainly plays a major role.
The supporting periodontal structures begin to breakdown. This can mean that part of the bone that supports the teeth or the ligaments that hold the teeth securely in place are destroyed. This disease is generally not reversible and may require treatment from a dental professional specializing in periodontal disease. Periodontal disease can develop as a result of poor daily plaque control (e.g. inadequate brushing and flossing). However, not everyone with poor brushing and flossing techniques will develop this condition. It is wise to visit your dental hygienist or dentist regularly to detect early stages of the disease and to prevent further damage.
Sugar Snack Facts:
• Frequent snacking on foods containing sugar increases the risk of getting cavities.
• Each time you eat sugar, plaque in the mouth combines with sugar to produce acids.
• These acid attacks on the teeth over time can destroy the tooth structure.
• Avoid developing a "sweet tooth" by limiting foods high in sugar.
Tips for Good Snacking Habits:
• Cut down on high-sugar snacks and offer snacks that are low in sugar such as vegetables, cheese, or pretzels. These do not promote tooth decay.
• Reduce the number of times a day that you eat sugar-containing food and snacks. With frequent snacks, the acidity of the mouth stays high, exposing the tooth to acid for extended periods of time. Frequent snacking will increase the risk of getting cavities more than eating the same amount at one sitting.
Oral hygiene begins during infancy Often parents think that brushing should not begin until a tooth is present in a child’s mouth;
however, oral hygiene begins long before that. Digestion begins in the mouth when enzymes produced by the salivary glands during feeding begin to break down foods, including mother’s milk. The natural bacteria in the mouth are not harmful to the child, but the by-products of the bacterial breakdown of food can be. Cleaning can be accomplished simply by wrapping a thin clean washcloth or gauze around the finger and wiping excess food and milk from your child’s mouth, especially between the gums and cheeks. This provides an effective way to promote good oral hygiene in your child and also serves as a way to prepare your child early on for future brushing.
When teeth erupt Because individuals with Barth syndrome are prone to infection, the eruption of teeth can be more problematic than in other children, causing fevers or even severe irritation of the gums. A cold compress or a piece of ice wrapped in a 2 x 2 gauze square can be applied directly to the eruption site. Through the numbing effect of the cold, this method can provide temporary relief minutes on, 20 minutes off. Tylenol or Advil can be given for the temporary relief of pain in dosages according to weight or age. Aspirin placed directly on the affected area will cause a chemical burn to the tissue and should be avoided. Prolonged pain, exaggerated irritability (over one week) is unusual and may be caused by inflammation around an impacted or a partially impacted tooth. Prolonged pain is fairly common with eruption of the first permanent molars, the third molars, and the wisdom teeth. A dentist should be consulted for severe pain or fever associated with tooth erruption.
When a tooth comes through the gum line, it is known as tooth eruption. This is a normal process of the body. A child’s first set of teeth are called the primary or deciduous teeth. As a child loses the primary teeth, the second set of teeth, known as the permanent or secondary teeth, come through the gum. Teeth are named for their location in the mouth and the function they serve.
Incisors cut the food, and canines tear the food. Premolars crush the food, and permanent molars grind the food.
Children with Barth syndrome may have delayed tooth eruption. However, extreme delays should be reported to your son’s pediatrician or dentist. On average, all 20 primary teeth have erupted by the age of 30 months (2 1/2 years).
A Water-pic is not an acceptable alternative to brushing and flossing. This device massages the gingival tissue (gums), however it does not remove the plaque, known to cause caries and periodontal disease (gum disease).
Bleeding can be caused by brushing and flossing. A soft bristled, age-appropriate toothbrush along with proper technique of brushing and flossing should minimize bleeding. However, sometimes pre-existing irritation of the tissue may cause bleeding even with the gentlest brushing. This irritation rarely occurs when proper oral hygiene techniques are used. Moreover, irritation also is commonly caused by inappropriate oral hygiene. Bleeding often occurs when the gum tissue has not been stimulated properly. Consult with your dentist for instructions for proper oral hygiene to avoid bleeding. Gum tissue should be stippled like an orange peel and pink. Red, inflamed gingival tissue is indicative of gum disease and should be evaluated immediately.
Oral Hygiene Fact Sheet 6 The Barth Syndrome Foundation www.barthsyndrome.org 21 March 2007/ CAB, RIK DISCLAIMER: This fact sheet is designed for educational purposes only and is not intended to serve as medical advice. The information provided here should not be used for diagnosing or treating a health problem or disease. It is not a substitute for professional care.