A Day in the Life

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.

  • 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.

  • 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.

  • 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.

  • 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.

  • 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.

  • 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.

  • 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.

  • 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.

  • 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.