A true story based on the life of Roy L Dennis a kid who lived with Craniodiaphyseal Dysplasia
• History of Movie, character, and disease
• Functional Assessments Pgs. 1, 2,3, and 4
• Health Assessment Analysis
• Additional Observations
• Nursing Considerations
• A very rare genetic disorder that features skull deformities (bone enlargements). Also known as CDD and Lionitis.
• Autosomal recessive bone disorder that causes calcium deposits to build up in the bones of the skull.
• Calcium deposits decrease the size of the cranium foramina and decreases size of cervical spinal canal. This eventually results in squeezing of the brain and spinal nerves.
• Severe Intracranial Pressure arises from the lack of space for the nerves resulting in headaches, and many other signs and symptoms that eventually lead to death during the childhood years.
History of “Mask” and character
• Movie made in 1985.
• Roy “Ricky” L Dennis the boy the movie portrays, lived with a disorder that created severe deformity of his face, and ultimately took his life. He was born to a mother who later had issues with drugs, and older brother who dies from aides. He had a family that was struggling, and had no additional room for any problems. His mothers friends where biker’s, and they became his support system.
• Craniodiaphyseal Dysplasia is the name of the genetic autosomal disorder that Roy was born with. It is also known as CDD and Lionitis. This disorder is a very rare genetic disease that features skull deformities (bone enlargements). This bone disease is characterized when calcium is not carried away by the blood, and the calcium then builds up in the bones of the skull. These calcium deposits decrease the size of the cranium foramina and decreases size of cervical spinal canal. Infants born with this rare disease live to be a pre-teen or teenager. • Roy the boy in the movie mask, started school at age 6 years, against M.D.’s advice. He faced alienation, criticism, humiliation, and many other types of discriminatory experiences.
• His family was informed he would more than likely die before age seven due from pressure in his cranium. • He died at age 16 from complications of this disease.
Functional Assessments pg. 1
Health Perception/Health Management: --Imaging tests to confirm diagnosis include: Radiography imaging of the clients cranium, x-rays, monitoring thickness of facial bones, and with a major focus on assessing thickness of his or her mandible. -Neurological radiography and, tests, need performed on an ongoing basis. In addition, MRI’s should be performed routinely to monitor for any changes relating to compression of nerves, and increase in calcium deposits. - Intracranial pressure signs and symptoms such as: with infants drowsiness, separated sutures of the skull, bulging fontanel, lethargy, and vomiting. With older children they may have behavior changes, decreased alertness, a headache, seizures, vomiting, weakness, tingling, abnormal eye movements, and double vision. -Facial paralysis needs to be recognized which can indicate compression of the trigeminal as well as the fifth and seventh cranial nerves.
-Delayed tooth eruption should be assessed regularly, auditory checks for hearing loss, •Eyes- Ophthalmic assessments to monitor vision changes or loss. Character was legally blind from compression of optic nerve from disorder/ condition. Despite this, client had glasses which helped him to see. Client was able to read after thinking he couldn't.
•Nutrition/Metabolic: Regular diet. Monitor for any swallowing difficulties as needed. As related to nerve compression of vagus nerve.
Assessments pg. 2 continued…
• Pattern of Elimination: Normal, should have regular urinary bladder and bowel movements daily. In the movie there was no indication of an issue in this area.
• Pattern of Activity/Exercise: In the movie...
References: • Crump, S. K., Schaffer, M. A., & Schulte, E. (2010). Critical care nurses ' perceptions of obstacles,
supports and knowledge needed in providing quality end-of-life care. Dimensions of Critical Care
Nursing, 29(6), 297-306
• Jarvis, C. (2008). Physical examination and health assessment (4th ed.). Philadelphia: W. B.
• Orphanet.Craniodiaphyseal dysplasia. Website:http://www.orpha.net/consor/cgi-bin/OC_Exp.php?
• Wikipedia, the free encyclopedia. Mask (Film).Website:
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