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Sixteen-year-old (DOB: 10/31/83) B.P is a Caucasian
male with Velo-Cardio-Facial Syndrome, also referred to as DeGeorge
Sequence, Catch 22 or Shprintzen Syndrome.
While at the Cincinnati Center for Developmental Disorders, on
April 2, 1991, he participated in a third chromosome study because the
November 1983 and 1990 studies demonstrated no fragile X aberrant on
variant. Using a
"N25" probe designed for testing chromosome #22 evidence was
present for a deletion, consonant with a diagnosis of VCF syndrome.
The 25th gene section of the chromosome 22 is thought to be the
genetic marker for VCF syndrome (22-q11 deletion).B.P. demonstrates
multiple congenital anomalies. B.P.'s
cardiology evaluation, by history demonstrated a heart murmur, mild
pulmonic stenosis, branch stenosis of the right pulmonary artery and
aberrant right subclavian artery. By
history his ENT exam revealed recalcitrant recurrent otitis media, cleft
palate and bifid uvula, which were repaired in November of 1987.
For his defect in his velum he underwent surgery in February of
1995. He is at risk for
respiratory tract infections and constipation.
He has had at least 30 hospitalizations for related various
emergencies and planned procedures.B.P was treated for his attention
deficit disorder and psychosis at The Affinity Center, Inc., while at the
Affinity Center, it was reported on September 28, 1999 that his mental
status had deteriorated due to the underlying genetic disorder VCF
syndrome. Apparently he was
referred to Cincinnati Children's Hospital. The Department of
Radiology at the Cincinnati Children's Hospital performed an MRI without
contrast dated 09/30/99, revealing mild cerebral atrophy.
On 10/19/98, during a stay at Cumberland Hospital, he was diagnosed
with VCF syndrome, scoliosis, organic personality syndrome and mixed
developmental disabilities. B.P is appropriate for a 60-day
evaluation and treatment course at FINR.
A comprehensive evaluation by the neurologist and neuropsychiatrist
will be conducted to develop a treatment plan and to follow progress.
The neuropsychologist will assess, through neuropsychological
testing his brain behavior dysfunction associated with his cerebral
atrophy, evidenced in his MRI in September of 1999.
B.P. will have a
comprehensive evaluation from the department of speech and language
pathology, physical therapy and occupational therapy.
These disciplines will develop a comprehensive treatment plan to
prevent further deterioration and actualize his abilities.
The nursing department will coordinate pertinent records from past
treating medical facilities and manage his physical involvement related to
his VCF syndrome.
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Health. B. P. is
capable of managing all bowel and bladder functions. He has no
difficulties with chewing, swallowing, or deeding activities.
He has a mild to moderate hearing loss in his left ear. He has medical problems consisting of episodic constipation,
heart murmur and mild pulmonic stenosis, and recurrent upper
respiratory infections and ear infections. He is currently on
Haldol,Cogentin, and Lithium Carobonate.
The nursing department will coordinate all outside medical
appointments and instruct B.P and family in following medication
regiments.
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Personal and
Self-Care. B.P. is
independent in all self-cares. It is recommended that staff proctor
him to complete his grooming and hygiene adequately, reinforcing good
performance.
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Physical
Functioning and Mobility. B.P
is independent in ambulating but will need physical therapy to focus
on posture, strength and endurance considering his recent operation
for scoliosis. He
demonstrates mild difficulties in all transfers and which will also be
a focus of physical restoration activities.
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Cognitive Skills.
A neuropsychological battery is indicated to assess higher
cortical dysfunction associated with cerebral atrophy. Due to
the recent MRI and evidence of cerebral atrophy, his cognitive ability
for making safe and gainful decisions regarding financial, social and
personal matters is moderately to severely impaired.
His memory for retrieving and storing information and for
processing visual information, more so than for auditory information
is impaired. He is alert
and attentive but he can not sustain concentration for extended
periods. He is oriented
to person, place and time.
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Community
Integration. B.P.does
not spontaneously initiate recreation activities. He needs to focus on
intense socialization skills. Money
management is poor. He
depends on others to prepare meals but he can make smacks on his own.
He does not initiate household chores but can perform.
Simple tasks such as dusting, sweeping, and making his bed.
He is able to identify household hazards but con not plan for a
crisis nor has an ability to access emergency services. Direct care staff will prompt him to maintain a clean and
organization bedroom and he will be rewarded for his achievements.
He will also. be reinforced for engaging in group activities in
order to improve socialization skills.
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Communication.
B.P has adequate comprehension for verbal directions and
conversations and he can express his needs. He does have a mild
articulation and voice disorder at times making speech unintelligible.
Speech and Language Pathology will develop a program to improve speech
skills in conversation. Additionally he will be enrolled in FINR'S
School Program where an educational program will be tailored to his
level of achievement, learning and neurological development.
B.P. will be reinforced for task completion and sustained
concentration to task and for his accomplishments.
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Behavioral
Adjustment. The
Neuropsychiatrist will evaluate B.P. and his needs for psychotropic
intervention considering that he suffers from auditory hallucinations.
He is reclusive and never seeks friendship from other children.
While at FINR, he will be placed in a group setting with other
children. He will be
reinforced for his social participation.
Despite B.P's low frustration tolerance, he rarely is
physically aggressive however will complain and whine.
Due to his poor self-esteem he will also be enrolled in group
or individual therapy.
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Psychosocial.
B.P will be evaluated by the psychiatrist for both thought and
affective disorders and treated as needed. He will be seen in group
and individual therapy, if needed.
He demonstrates lack of insight into his problems; therefore
treatment staff will reward him for his improvement in that area as
well as his ability to continually relate to his peers.
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Discharge
Recommendations. B.P will return home to live with his family post
treatment. His family
will be invited to attend aftercare education sessions at FINR in
order to learn coping strategies as well as behavioral techniques to
improve generalization of skills to the home environment.
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