Dentinogenesis Imperfecta is a rare inherited disease of the teeth, that effects 1 in out of 8,000 people throughout the world. Not a lot is known about Dentinogenesis Imperfecta because of the rarity of the disease.
Dentin is a boney soft tissue that is the protective middle layer of a tooth, which is yellow in color with people that have normal teeth. People with Dentinogenesis Imperfecta or DI, the dentin is abnormal causing the dentin to be softer then normal and discolored. Abnormal dentin causes the teeth to be soft, also making the enamel of a tooth chip off, which makes the teeth break, chip far faster and easier. The teeth also wear down from chewing far faster then normal teeth. The teeth are also discolored ranging from a translucent color to a yellow brown. DI effects the formation and structure of the permanent teeth along with the baby teeth. People with DI have very small to no root canals and very small pulp chambers. This causes the teeth to have small or no nerves, making people with DI unaware of chips, breaks and tooth infections because of little or no feeling from the absent of the nerves. Tooth infections or dental abscess are very common among DI patients due to the absent and or short root canals. DI also makes the teeth easier to pull by dentists and or knocked out of the gums due to impact. Some DI patients have reported hearing loss but doctors haven't found the link to DI and the hearing loss.
Treatment of DI can be long and costly depending on severity of the disease. Dentist recommend treatment options as soon as the baby teeth are present in a child to help preserve the teeth longer and protect from chips and breaks. In children most common treatment option are sliver crowns that are fitted over the baby teeth until their adult permanent set comes in. When the adult set some comes in there are a few more options that open up to people who suffer from DI. In some mild cases of DI where the teeth and enamel are strong enough, braces are used to straighten the teeth and a bonding material used to help mask the discoloration. Most DI patient's teeth are not strong enough for braces and the teeth crumble or break apart from the pressure. Most commonly used treatment options are caps or also called crowns. Crowns are made out of with either metal or ceramic. Crowns are used to cover the entire tooth to help protect and persevere the teeth. In come cases the tooth has already broken down to the gum line and corrective surgery is needed to build the tooth up for crowns. Root canals are not recommend as a treatment option for people with DI because the teeth lack roots and the deformed dentin make it impossible for root canals. Bonding or veneers are also sometimes used to cover the front teeth in the smile line but are not used or recommend on the back molar teeth, the act of chewing puts to much pressure on bonding material or veneers and they crack under it. Dentures are also an option for people who have server Di; dental implants are also options for patients that have to have full mouth extractions of the teeth. Dental implants are very pricey and the recover time is far greater and most dental implants are not covered under dental insurance. Dental implants also have a great chance of failure, 50% of patients have been reported to have their dental implants fail within the first 3 years of having them due to the DI disease.
What is Dentinogenesis imperfecta? Dentinogenesis imperfecta is a rare abnormal tooth condition that causes the teeth to be weakened and easily broken then normal teeth. DI affects 1 out of 8000 people throughout the world. Dentists have discovered three types of DI. Type 1 DI is linked to with osteogenesis imperfecta (OI). OI is a condition of fragile bones, Bones that are weaker then normal and tend to break more easily. Not everyone diagnosed with OI are also diagnosed with DI, only about 50% of OI patients have DI. Type 2 DI is not associated with any other disease. Type 2 DI is inherited in an autosomal dominant pattern, meaning that the affected person with DI has at least one if not both parents with the condition. Doctors have located the gene responsible for DI. The gene is called Dentin Sialophosphoprotein or DSPP for short. The DSPP gene is responsible for making a protein that is essential for normal tooth development. People with Di have been found to have more than 20 different mutations in the DSPP gene. This gene is also the cause of a rare type 3 DI. Type 3 DI is far more rare then type 1 and 2 and even less is known about type 3. What is known about type 3 is that it is less severe then the other two. Type 3 Di is also an inherited type passed on by at least one parent that has it. Type 3 is also known as the Brandywine symptom because it is most commonly found in southern Maryland in the Brandywine area.
I picked Dintinogenesis imperfecta as a topic for my research paper because I suffer from type 2 DI. DI runs in my family on my dad's side. My dad has DI along with his two haft brothers, a haft sister and his farther. I did a little research of my own and traced DI to a great -great- great -great -great grandma that was from Great Britain that had DI. I have also talked to people in a Di support group and found out that most people had at least one ancestor from Great Britain that had DI, it is not clear if it orientated from there or not but it seems to. Everyone in my family has type 2 and with each generation the teeth are in better shape then the one before. Like my dad and I, my teeth were in a lot better shape then his. My dad's teeth were in such bad shape that at 17 he had to have all his pulled and dentures placed in. I am 29 years old and just had to have all my teeth pulled over the summer due to the fact all my teeth had abscessed. Since root canals are not an option, I had dentures done for a temporary time. My dentist thinks that dental implants would work best in my mouth and will not fail because I have enough bone to support the implants and I have no other underline condition. I do wish I had this done a few years sooner because since I have had this done, I feel better and my skin as cleared up. I have suffered from very bad acne and have been on every acne medication on the market and nothing seemed to work. My dentist thinks there might be a link between DI and my ache problem since my face cleared up but since it's a rare disease he can't be to sure. DI has been very expensive and costly throughout the years, I probably had close to haft a million dollars of dental work in my mouth, thank god for very good dental insurance. Past treatments I have had done, is bonding the front six top and bottom teeth you could see when I smile, so my teeth looked normal. The bonding material used was a special mixture my dentist had came up with because normal bonding material would chip right off. Even with the special mixture the bonding material would have to be replaced every two years to make sure it stayed in place and didn't crack or break off. Since my teeth were soft and they chipped so easily I have to be super careful about the foods I ate, so not to chip any teeth but that always didn't work and if I did chip a tooth the same special bonding material was used to fixed the teeth along with any cavities I had. Normal silver fillings for cavities didn't stay in they would fall out, the same thing happened with a few teeth that they crowned, the crowns wouldn't stay and would break off taking pieces of the tooth with it. I do have to say the only neat thing about having DI was that I never had a toothache and never had to have Novocain to have a cavity filled, because none of my teeth had nerves I didn't feel a thing. Even when I had to have a few teeth pulled when I was younger I had a very small dose of Novocain but that was just to numb the gums.
Dentinogenesis Imperfecta is a rare disease of the mouth that affects the teeth.
