April 1, 2017

We have just completed the first quarter of the 2017 year and I want to send out an update on the Clinical Registry Investigating Bardet-Biedl Syndrome (CRIBBS) and address a health issue that is often overlooked.

We have been following the health of 308 individuals with BBS. Individuals are located all around the world including individuals from 42 states in the US, many provinces in Canada, Central America and South America, Europe, Russia, Asia, Egypt, the Gulf States and the Faroe Islands.

The purpose of CRIBBS is to improve the health and quality of life of individuals with BBS. One health topic that is often overlooked is the relationship between dental health and overall health. There is growing recognition that poor dental health can impact other organ systems such as the cardiovascular system and the kidneys. Oftentimes when I see individuals at the Treatment Center for BBS I note dental concerns. Using the CRIBBS data the following information was found:

• Dentofacial abnormalities (meaning dental or oral cavity abnormalities) were present in more than 50% of individuals.

• Mouth breathing was present in 59% of individuals. This can lead to drying of the mouth and increased risk of cavities.

• Dental crowding and space problems with the teeth in 46%

• High arched palate in 35%

• Problems with tooth eruption (delayed or unerupted teeth) in 9%

• Periodontal problems/Gum infections in 7%

• Individuals undergoing orthodontic treatment in 28%

• Pain with eating food 5%

Increasingly, evidence shows obesity can increase the risk of dental disease including gum infections (periodontitis) while dental disease can increase the risk of inflammation that increases risk for heart disease, kidney disease and diabetes.

It is very important to be aware that some medications can aggravate dental problems. Individuals with organ transplants usually are treated with cyclosporine or tacrolimus. The medications are very important but can cause thickening and damage to the gums. The same is true with phenytoin (Dilantin ®) and calcium channel blockers (blood pressure lowering medications such as amlodipine). In addition, medications may lead to ‘dry mouth’ due to a decreased saliva levels and put people at risk for gum disease and cavities.

So what can you do? First, it is important to encourage dental care in early childhood. Find a dentist that you like and your child likes. See the dentist at least twice yearly. Work with your child on brushing and flossing techniques. For the teenagers and adults with BBS be sure and take time to care for your teeth by brushing and flossing. Use of

oral rinses to reduce levels of oral bacteria or that stimulate salivary flow for people with dry mouth may also help with maintaining oral health. See your dentist and work with the dentist to make sure you are receiving the very best care.

Here are some great suggestions from David McGlohon DDS, a dentist and father of a child with BBS.

• Nutrition is key: Keep exposure to sugar and carbs to a minimum. Sodas should be eliminated and watch for sugar in other drinks like juices and chocolate milk. Chocolate milk actually is higher in sugar content than most sodas!

• Dry mouth: Many medications cause dry mouth. These patients are very prone to tooth decay because they lack the natural cleansing mechanism of salivary flow. Biotene is a mouth rinse which is helpful with moisturizing. Dry mouth patients need to be very meticulous with their oral hygiene. At times a prescription strength fluoride toothpaste can be prescribed by your dentist. A sleep study can also be helpful for patients who snore and sleep with their mouth open.

• Brushing and flossing: Getting a child to brush and floss can be a struggle for many parents. I recommend that the parent perform this until the child is at least seven years of age. The easiest position to use is with the parent sitting on the side of the bed with the child resting his head on the parent`s thigh. This allows better vision and control of a "moving target." Also, use only a "smear" of toothpaste on the brush. Many kids swallow the toothpaste and too much consumption can lead to fluorosis of the tooth which can develop white spots in the enamel. Using an electric toothbrush like an Oral B or Sonicare is much more efficient. For example, an example an electric toothbrush can give 60,000 strokes per minute as opposed to the 60 per minute a patient can do by hand.

• Fluoride varnish is another effective preventive treatment. This is done in the dental office as soon as the child has teeth and can be done through adulthood as needed.

A paper on the importance of dental health has been submitted for publication using information from CRIBBS. It will be made available to you to share with your dentists once it is published.

Thank you for supporting CRIBBS. Thank you for donating your time for the annual interviews. Thank you for signing and returning the medical release forms. And many thanks to those that donate even a few dollars to keep CRIBBS alive and thriving. If you are interested in learning more about CRIBBS please visit our webpage at www.bbs-registry.org.

Sincerely,

Dr Bob Haws

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