Stranger Things season 4 finale is finally available to stream on Netflix this Friday. Of course, Gaten Matarazzo is also there as Dustin Henderson. In character and in real life he has Dressocranial Dysplasia. FITBOOK explains what is behind the rare hereditary disease.
Devoted Stranger Things fans know that Gaten Matarazzo has Clothocranial Dysplasia. The disease is also part of the storyline around series character Dustin Henderson from season one. Put simply, the genetic disease is characterized by the fact that the development of bones and teeth is impaired. FITBOOK explains what symptoms are associated with cranial dysplasia, what we know about the cause and what treatment options are available.
Gaten Matarazzo draws attention to dress-ocranial dysplasia
Gaten Matarazzo rose to fame at just 13 years old on the cast of the Netflix hit «Stranger Things» and realized he could use it to raise awareness of the hereditary condition, Dressocranial Dysplasia. In a 2016 interview with People, the young actor said: «There’s a one in a million chance you’ll get it. Most of the time you get it from a parent, but it just happened to me. I have a very mild case so it doesn’t affect me that much, but it can be a very difficult condition.»
Depending on the severity of the disease, different operations may be necessary. Gaten Matarazzo also had some interventions – in his case on the mouth. The fact that the 19-year-old is open about his illness and that the makers of «Stranger Things» even integrate it into the series is not a matter of course. The feedback from fans is correspondingly positive. «A lot of people write to me, ‘I feel better that you can show your condition on the show and be so open about it.’ The fact that I can draw attention to the topic gives me a good feeling,” says Gaten Matarazzo.
What are the Symptoms of Clothocranial Dysplasia?
Even the name of clodecranial dysplasia or cleidocranial dysplasia (CCD) indicates the predominantly affected body regions. So «cleido» stands for the bones of the collarbone, while «cranial» means skull.
In affected persons, the hereditary disease typically becomes noticeable in infancy. At birth, they have abnormally large, wide open fontanelles that may remain open for life. The fontanelle is a gap between the bones of the newborn’s skullcap that grows together over the course of the first two years of life in healthy babies.
Other symptoms of the disease can include narrow and sloping shoulders and generally slower growth as a child. In addition, the teeth are often affected. Sick children may have extra teeth or, on the contrary, the teeth will not erupt. It can also happen that the second, permanent teeth come through in addition to the milk teeth.
Also interesting: Ireland Baldwin has cardiophobia – what is behind it?
the Symptoms of clochecranial dysplasia at a glance:
skull and face
- Fontanel closes late or not at all
- broad, flat or prominent forehead, sometimes with a vertical groove down the middle
- low, broad bridge of the nose
- small face and small jaw
- arched hard palate or cleft palate
- small or missing paranasal sinuses, which can lead to inflammation (sinusitis) with difficult breathing and pain
- ear discomfort
teeth
- Baby teeth won’t come through
- supernumerary and/or teeth that are too close together
- Adult teeth that are encased in bone and will not come through on their own
- misaligned teeth
- thin enamel and therefore susceptibility to tooth decay
Other features
- small bodysize
- incomplete or absent collarbones, sloping shoulders
- short fingers
- narrow chest
- supernumerary or absent ribs
- deformations of the pelvic bones
- Scoliosis (curvature of the spine)
- malposition of the knee joint
- Osteopenia (decreased bone density) – a precursor to osteoporosis
Also interesting: what exactly is osteoporosis and how is it treated?
What is known about the cause of the hereditary disease?
The disease is caused by mutations in the RUNX2 gene (6p21), which is involved in the differentiation of osteoclasts (cells responsible for the breakdown and resorption of bone substance) and in bone formation.
The research was able to identify different mutations that are probably responsible for the different manifestations and symptoms of the disease. Due to the variety of signs and physical characteristics that manifest themselves with cerebral cranial dysplasia, it can generally be diagnosed quickly and with certainty.
What treatment options are there?
There is no cure; instead, treatment focuses on the specific, individual symptoms and deformities of the people affected. In the case of mild forms of the disease, no therapy is necessary at all because the patients do not feel any pain or impairments in everyday life.
Depending on the severity of the deformities and symptoms, surgery may be necessary or advisable to alleviate the suffering. This can be interventions in the mouth area – like Gaten Matarazzo had for the development of his dress-ocranial dysplasia – or hip operations. If the general bone density is affected, it is important to monitor its development and to undertake preventive osteoporosis therapy.
People with this rare hereditary disease can lead as normal a life as possible by taking advantage of therapy measures that are appropriate to their symptoms.