Case Report 1 – “I want to drive” Abstract Patient AA is a 17 year-old high school student with a diagnosis of a non-specific mild learning disability recently diagnosed Stargardt’s Maculopathy. Patient objective is to gain driving privileges and parental concerns surrounding academic success. Step-wise management is required for goal attainment. I. Case History from record review a. Ocular history based on record review from physicians in NJ i. 2006 presented to ophthalmologist because of failed school screening 1. Entering: VA RE 20/60 and LE 20/50 2. Refraction and BCVA RE 20/25+ LE 20/25+ 3. Diagnosis: hyperopia 4. Rx: wet refraction RE +1.25 +0.50x 010 ; LE +300 sph ii. March 2008 – lost glasses 1. Entering: VA RE 20/40+ and LE 20/40+ 2. Refraction and BCVA RE 20/20- LE 20/25 3. Diagnosis: amblyopia RE per notes iii. March 2009 – “sees better without glasses” 1. Entering VA (cc): VA RE 20/60 and LE 20/400 2. Entering VA (sc): VA RE 20/25 and LE 20/30 3. Refraction RE +0.50sph 20/20- LE +1.50sph 20/25 4. Diagnosis: amblyopia improved 5. Rx: Separate distance and reading glasses b. Neuropsychological Evaluation i. January 2010 to evaluate difficulties with HW completion, focus and organization – ii. Diagnosis of “Learning Disorder” 1. Verbal memory – high average 2. Visual memory – low average 3. Difficulty with reasoning and problem solving when there are time constraints 4. Easily overwhelmed by too much visual information being presented at once 5. Dx: deficits in functional reading comprehension & executive function skills. 6. Recommendations: a. Borrow/photocopy notes so he can attend better to information provided in class II. b. Decreased homework volume c. Books on tape d. Alternative testing modalities c. Ocular history (cont.) based on record review from physicians in NJ i. February 2010 1. BCVA RE 20/30+ LE 20/252. Diagnosis: accommodative spasm 3. Rx: CL’s “full plus” ii. February 2012 – CC: Problem with distance vision w/glasses” 1. BCVA RE 20/50 LE 20/50 2. Diagnosis: macular RPE changes, ? Stargardt’s, latent hyperope, mild ciliary spasm 3. Refer to retina specialist iii. February 2012: Retinal eval – dx Stargardt’s Maculopathy 1. Refer to Wills Eye Hospital for dx confirmation a. Diagnosis confirmed b. Recommended ERG c. Parents requested low vision evaluation Case History from low vision evaluation - May 2012 a. Chief complaint: “I want you to sign off on my driver’s license” b. Demographics - 17 year-old completing 11th grade in New Jersey c. Visual function history i. Noticed initial problems with vision ~ 2 years ago ii. Reading 1. Denies problems reading and viewing the computer; parents state he gets close to 27 inch monitor 2. Accessibility options on computer rarely used with exception of enlarged font on 15 inch laptop at school 3. Has reading glasses which help “some” 4. Uses Kindle w/ transference of PDF’s and enlarging font iii. Visual Information/seeing 1. Wears separate distance glasses when watching TV and sometime walking which help some; never wears when playing sports 2. Sits in the middle to back of classroom and has some difficulty seeing the board iv. Mobility and visual motor skills –denies problems d. Current academic history i. B/C student ii. Switched from “academically intense” private to public school ~1 year ago iii. Current accommodations: extra test-taking time e. Social history i. Accomplished soccer player on travel team III. IV. V. ii. Lives with both supportive parents; older sister in college iii. Parents anxious about current and future impact of ocular diagnosis iv. Patient has good friends at school Pertinent findings a. May 2012- Initial low vision rehabilitation evaluation i. Habitual and best-corrected VA RE: 20/60+ and LE 20/40ii. Contrast sensitivity 1.50 log units (mild reduction) iii. Near – habitual work distance 10 inches (25cm) 1. Reading errors and slowing begin at 20/63 2. Resolution VA 20/35 and then uses relative dist. mag 3. Scotoma to right of fixation evident iv. Goldmann visual field show periphery full and intact b. August 2012- follow up low vision rehabilitation evaluation i. Habitual and best-corrected VA RE: 20/40- and LE 20/40ii. Contrast sensitivity 1.40 log units (mild reduction) iii. Telescope evaluation: 1. No definite eye preference with some rivalry 2. 2.0x FDTS: 20/25iv. Near evaluation – unchanged ability from 5/2012 1. With 8 D of magnification with illumination, working distance improves; minimal change in VA 2. Electronic magnification – reading speed improved c. November 2012- follow up low vision rehabilitation evaluation i. Vision unchanged; parents concerned about obtaining necessary accommodations (e.g. Reader) for SAT 1. Using portable electronic magnifier and “doing well” ii. Habitual and best-corrected VA RE: 20/50+ and LE 20/40iii. 4.0x bioptic TS LE 20/20- ; excellent response iv. MNRead performed – 1. Critical print size 20/80 2. Maximum reading rate at CPS 125-150wpm Differential Diagnosis a. Learning disability Vs. problems with learning secondary to visual disability Treatment and Management a. May 2012- Initial low vision rehabilitation evaluation i. Patient meets NJ driving requirements (20/50 in better eye) ii. Extensive discussion with patient and family regarding vision, maturity, potential future loss of vision and driving iii. Completed necessary forms to initiate driving permit b. August 2012- Patient obtained driving permit and driven 2x i. Father want son to initiate using tools/adaptive equipment and skills to assist with reading performance VI. ii. In addition to driving practice with parents, they are initiating driving with driving school for extra support/practice iii. Rx: portable electronic magnifier iv. Evaluate text to speech approaches and telescope at next follow up c. November 2012- follow up low vision rehabilitation evaluation i. Discussion at length on issue of SAT “Reader accommodation” 1. Inconsistent with past accommodations yet clear need for auditory support ii. Evaluation and training on electronic magnification iii. Rx: 4x focusable bioptic TS for use in classroom and ultimately driving Conclusion and Clinical Pearls High school students seeking outpatient low vision services require a careful communicative approach to gain rehabilitation “buy-in” to encourage adaptation for success to maximize visual and social function. Often patients at this point are willing to consider engaging in adaptive approaches, including visual assistive equipment, that they commonly have rejected during middle school years given the peer social environment. The major concern of high school students with visual impairment is whether or not they are able to drive as their peers are gaining their independence. For those patients who meet driving standards and have chronic progressive conditions, it is important to discuss the emotional impact of gaining licensure and potentially discontinuing if/when vision deteriorates. Additionally, consideration of recommending licensure should include patient and parental insight, maturity, and a clear understanding of driving limitations if imposed. It is common that patients with vision impairment also present with a diagnosis’ that include of some type of learning disability. It is essential to differentially diagnose how much the academic challenges are related to vision impairment vs. learning disability to ensure accommodations are designed appropriately. Case Report 2 – "I just want to keep teaching" Abstract Patient AD is a 59 year-old chemist with myopic degeneration. He requires significant low vision adaptations to achieve the goal of returning to his full abilities as both a researcher and chemistry professor. I. Case History • Patient demographics: 59 year-old Caucasian male, PhD in Chemistry • Chief complaint: decreased vision over the past few years; wants updated CL Rx; also, work suggested an evaluation as students had commented that patient AD was having difficulty seeing when teaching—patient feels he is functioning well in all aspects of his job and but is open to other accommodations • Ocular History: 1. Myopic degeneration with macular involvement OU 2. Strabismus OU (bilateral ET) 3. Retinal detachment OS with minimal residual vision • Medical history: no reported medical problems • Medications: no current medications • Other salient information: Low Vision Functional History 1. Difficulty reading printed materials 2. Removes CL to read on the computer and adds built-in accessibility for enlargement; has to sit very close to the computer screen 3. Difficulty recognizing faces in the classroom/ lecture hall 4. Patient is no longer driving and now feels less confident with his mobility over the past few years 5. Social History: Patient is married and is a chemistry professor and researcher; responsible for lecturing and lab work Conversation with HR per patient request: HR has significant concerns about patient’s ability to see at work; student evaluations comment on patient’s inability to accurately grade papers and to monitor lab work; HR would like to work with patient to establish best accommodations to keep patient working to his fullest ability; HR was advised that workplace evaluation would be completed by OT and OD in low vision service and that patient is undergoing active treatment and therefore full evaluation may not be completed for several weeks to months. II. Pertinent findings: Visit 1 • Clinical VA (ccL) OD: 20/800 @1M on the ETDRS chart; large scotomas to right and left of fixation VA (sc) OS: HM Contrast Sensitivity (CST): 0.50 log units (severe to profound loss) NVA (ccL): 4.0M MRx (over CL) OD: -5.00 -2.00 x 090 with VA 20/640+1 @1M (ETDRS CHART) • Physical Bilateral Esotropia with EOMs restricted in all directions OU 3+ NS OD; 4+ NS OS Large staphyloma and retinal scarring OD; no fundus view OS 2’ to dense cataract Plan: 1. Refer for retina consultation given vision changes and 5 years since last retina visit (BCVA 20/63 OD at that time; still HM OS) 2. Refer for new CL fit given high over-refraction 3. RTC 6 weeks for full low vision assessment once wearing new CL 4. Retina surgeon referred for cataract consultation • Laboratory studies A Scan: Axial Length 38.00 mm OD and 37.61 mm OS B Scan: difficult to interpret due to fixation; bilateral shallow membranes noted in both eyes Strabismus surgery performed in the left eye on recommendations of retina and cornea evaluation. Residual large angle ET noted after maximal resection and recession. Visit 2 CC: improved vision post CL fit; interested in determining best accommodations to maximize ability to see and read at work Additional hx: patient has concerns with how HR has been handling his case; has been given a reduced course load for the semester, does not want paperwork completed for HR at this time; pt plans to communicate independently with HR VA (ccL) OD: 20/400- @2M on ETDRS; OS HM vs. LP Contrast Sensitivity: 0.30 log units (profound loss) Initial LV Device Assessment: 1. CCTV allowed for good reading ability including hand-writing 2. Accurate distance spotting with 4 x 12 TS Plan: 1. Refer for comprehensive occupational therapy LV Assessment to better determine the best accommodations in the workplace 2. Patient to trial ZoomText software on computer at home 3. Portable and Stationary CCTV will play a valuable role; portable CCTV was more functional than TS during OT assessment for spotting instruments 4. Consideration of 24D AL for reading with contact lens in 5. OT to perform on the job evaluation once patient has navigated HR paperwork IV. Diagnosis and discussion This is a case of a working adult with a long-standing vision condition who is experiencing significant changes as the vision has changed. The patient presented with paperwork from his HR department requesting an assessment although the patient felt he was generally performing to his past standards at work. The initial evaluation established the need for co-management with a variety of departments including a retina consultation due to changing vision and longduration since the last retina visit. It also included a contact lens evaluation for a refit of the GP lens to maximize distance vision. The patient was also evaluated by the cornea service and has planned cataract surgery in the worse seeing eye with consideration of surgery in the better seeing eye in the future. The patient was seen for low vision follow-up with a much better contact lens fit and much better visual function. In the interim, the patient had a lengthy discussion with his HR department who determined that a reduced work load was the best option while the low vision evaluation and further surgical management was completed. The patient’s functional priorities included distance goals (i.e. seeing students, reading items at a distance in the lab), computer goals, and near vision goals (grading papers, reading student’s work, and general work-related and pleasure reading). V. Treatment, management Treatment included consideration of the best types of technology to maximize the patient’s vision. Classroom spot reading and overall continuous reading goals will be best met with a desktop CCTV on high contrast and high magnification. The patient plans on using this device to read hand-writing, read journal articles, and manage his mail and bills without having to remove the contact lens on a regular basis. The best option for spot reading measurements on beakers and other instruments in the lab was to work with the Ruby Portable CCTV. The patient had difficulty using a telescope to localize these types of numbers when he was evaluated in the clinic and with the occupational therapist. The patient’s computer goals will be met with ZoomText software installed at home and in the classroom. Additionally, the patient planned on installing a “buzzer” system in his classroom so that students can alert him to when they have a question allowing him to focus on teaching without needing to use a telescope to scan the classroom and determine if any students are raising their hands. Patient AD is very motivated to improve his overall visual function, but presented with concerns regarding the directives from HR. Review of the HR information and student evaluations suggest that patient AD Is having more difficulty at work than he describes during low vision assessment. VI. Conclusion and Clinical Pearls Low Vision Assessments are multidisciplinary and often require co-management with other specialists. Communication with the patient is essential in developing a rehabilitation plan and establishing an appropriate timeline and priority for the various referrals. Additionally, it is often necessary to coordinate completion of paperwork for HR departments and begin to help the patient understand the best accommodations to allow each person to continue doing his job to the best of his ability. In this particular case, the patient had clear functional deficits and self-reported many areas of difficulty, but it had the added complication of the patient not feeling comfortable with the HR strategy for managing his difficulties. The patient appreciated the assistive technology and understood the role that it would have for him in the classroom but he had concerns with the best way to approach these needs with HR. The patient also had multiple goals and there is no device that can meet all of these goals. The patient was counseled on the need for the specific devices for the specific tasks and he felt comfortable integrating a variety of technologies into his day-to-day life. The patient’s vision has been decreasing steadily over a five-year period, but he did not return for retina or contact lens follow-up in that time. He was prompted to come in for low vision assessment by his HR department which led him on the path to the additional management strategies including strabismus surgery, cataract surgery and refitting of contact lenses. Low Vision Assessment referrals rely on co-managing doctors, but also on educated consumers to seek care if they develop functional difficulties, even with long-standing vision impairment. Each case must be managed individually based on the patient’s vision, goals and motivation/willingness to integrate technology. Case Report 3 – “I want to work again” Abstract Patient JM is a 41 year-old aerospace engineer with bilateral traumatic optic neuropathy secondary to a self-inflicted gunshot wound (GSW). He requires multiple modalities of visually assistive equipment and low vision adaptations to return to work. 1. Case history from low vision evaluation August 2011 (s/p 5 months) a. Patient demographics: 41 year-old Caucasian male b. Chief complaint: “I want to see better.” Loss of vision since GSW March, 2011; referred by ophthalmologist at the University of Maryland for comprehensive low vision evaluation. c. Ocular History: i. Traumatic optic neuropathy OU ii. Enophthalmos OD d. Medical History: Depression, which initiated prior to the incident on March 2011 1. Self-inflicted gunshot wound was a suicide attempt 2. Currently seeing a psychiatrist and counselor 3. Now under control and denies current suicidal intentions e. Medications i. Fluoxetine (Prozac) 60mg QAM (Anti-depressant) ii. Oxybutynin ER 5mg QAM (reduces muscle spasms of the bladder and urinary tract) iii. Geodon 40mg BID (Anti-psychotic) f. Other salient information: Low Vision Functional History i. Reading 1. Difficulty reading small or normal sized print 2. Able to read headlines and large print, but vision seems foggy and washed out 3. Difficulty on the computer and has not yet tried accessibility options ii. Visual information/seeing 1. Everything appears “grayed out” 2. Watches a large, 52” television, sitting 14 feet away, and reports the pictures “appear light and yellow” iii. Driving 1. Discontinued since the event (March, 2011) 2. Not ready to consider returning to driving 3. Work is approximately 37 miles from home and could be driven by his wife or a coworker iv. Mobility 1. Two falls since the event a. The first was due to dehydration b. The second was vision-related when he misjudged the placement of objects in a dark bedroom 2. He is not currently traveling alone and is always accompanied by either his wife or friends. 3. Difficulty seeing street signs and pedestrian signals. 4. Difficulty navigating poorly lit curbs or stairs 5. Glare – wears transition lenses and feels they darken sufficiently in the bright sunlight v. Visual Motor Skills 1. Performs many tasks by using memory and sense of touch 2. Able to assist with household tasks such as cooking, can use the microwave, but rarely the stove top 3. Assists with childcare vi. Social History 1. Lives with wife and 21-month-old son in a house 2. Receiving long-term disability 3. Previous occupation was an aerospace engineer, involving computer and field work on airplanes. The patient has been promised a job with his former employer in Delaware and the patient would like to return to work as soon as possible. 2. Pertinent findings, Visit 1, August 2011 a. Clinical i. VA (cc) 1. Habitual spectacle Rx a. OD -8.50-1.75x035 VA NLP b. OS -9.75-1.75x135 VA 20/400 (ETDRS) 2. Scotoma to the left of fixation evident OS ii. Manifest Rx (same as habitual) 1. OD no improvement with refraction VA NLP 2. OS -9.75-1.75 x 135 NI in VA iii. Contrast sensitivity: 0.75 log units (severe reduction) iv. Near VA: 1. cDVRx: 5.0M @ 30 cm (approximately equivalent to 20/300) 2. sRx: 1.3M @ 12 cm (approximately equivalent to 20/200) a. Reading slowed at 2.0M v. Goldmann visual field in the left eye showed mild generalized constriction with a large central scotoma, which involved fixation. b. Physical i. Constant right exotropia with EOM restriction in all directions OD, full OS. ii. Nonreactive pupil OD, 3+ reactivity OS, 4+ RAPD OD. iii. Intraocular pressure by Goldmann applanation tonometry 7mmHg OD, 11mmHg OS at 9:55 a.m. iv. Undilated fundus examination 1. Cup-to-disc ratio of 0.30 OU with optic nerve head pallor OD>OS 2. Peripapillary RPE changes and atrophy RE c. Assessment and Plan i. Referral to the Division of Rehabilitation Services (DORS) to assist in the provision of low vision rehabilitation services for vocational and ADL tasks. ii. Orientation and mobility (O&M) training to enhance safe, independent travel iii. 22” desktop CCTV for reading and writing tasks. The device will help to minimize visual fatigue to perform extended reading activities. iv. Obtain a portable CCTV which provided the patient portability and ability to use in a variety of settings for work applications, especially when the patient is performing field work. v. 360 CCTV with adjustable camera to allow viewing detail of airplane parts at a distance vi. Computer adaptations system to provide print scanning, enlargement and speech output of text documents. vii. +20D/5x illuminated LED hand magnifier for reading of text material. viii. +28D/7x illuminated LED hand magnifier for verification tasks such as numbers or symbols on a graphic drawing. ix. Monocular telescope in the 2.5x – 4x power range. Further investigation is required to determine if appropriate for patient with special emphasis on recognition of his child in the home setting and with the support of O&M training. x. Technology evaluation to further address computer adaptations and modifications. Software provided the patient the needed magnification for reading the toolbar on the computer as well as speech output. xi. Large print overlays for the computer keyboard, or large print keyboard xii. Full-time wear of polycarbonate lenses for monocular precautions xiii. Information on legal blindness was given to patient including property tax exemption. xiv. RTC 3 months for follow-up 3. Visit 2, November, 2011 a. CC: Case with DORS is open; interested in determining additional work accommodations, as he is planning to return to work January 2012. b. Additional history: i. Visually assistive equipment (VAE) received: 1. Desktop CCTV 2. Portable CCTV 3. Computer software accessibility (magnification and speech-output) 4. Large print keyboard 5. Orientation and mobility training initiated ii. LED Hand-magnifiers not provided, DORS counselor was unsure why two powers were recommended c. Clinical i. VA (cc) 1. Habitual spectacle Rx a. OD -8.50-1.75x035 VA NLP b. OS -9.75-1.75x135 VA 20/200 (ETDRS) 2. Central scotoma evident OS ii. Contrast sensitivity: 1.05 log units (moderate-severe reduction) d. Plan i. Send updated report to DORS ii. +20D LED hand-magnifier for quick spot-reading printed materials with greater field of view iii. +28D LED hand-magnifier for spot-reading small print, especially detailed information, which requires accuracy iv. Large print speaking scientific calculator v. 360 CCTV for viewing distant detailed information at work vi. RTC 3 months for follow-up, to re-visit visual accommodations after resuming work. 4. Visit 3, February, 2012 a. CC: Most equipment is working great! But, still having some trouble, such as on the computer at work. b. Additional history: i. Returned to work x 2weeks ii. Visually assistive equipment (VAE) received: 1. Desktop CCTV x 2 (at work and home) 2. Portable CCTV 3. Computer software accessibility (magnification and speech-output) a. Works well at home b. Not compatible on desktop computer at work c. Work plans to supply laptop computer at work, for better compatibility of computer software accessibility 4. Large print keyboard (at work and home) 5. +20D LED hand-magnifier for quick spot-reading printed materials with greater field of view 6. +28D LED hand-magnifier for spot-reading small print, especially detailed information, which requires accuracy 7. Talking scientific calculator 8. Orientation and mobility training continued a. Considering guide dog c. Clinical i. VA (cc) 1. Habitual spectacle Rx a. OD -8.50-1.75x035 VA NLP b. OS -9.75-1.75x135 VA 20/200 (ETDRS) 2. Central scotoma evident OS ii. Contrast sensitivity: 0.95 log units (severe reduction) d. Plan i. Refer to technology specialist for additional computer software accessibility programs 1. Better compatibility with work computer 2. Power-point presentations. ii. Encourage follow-up with health professionals as scheduled iii. RTC to Low Vision service annually or sooner PRN 5. Discussion This is a case of a working adult with a tragic and acute loss of vision, experiencing many functional deficits due to the changes in vision. The low vision evaluation highlights the multiple modalities of low vision aids in the rehabilitation of a motivated individual with many visual demands. Multiple types of magnification were recommended for reading tasks: video magnification, illuminated hand-magnifiers and also text-to-speech technology. Video magnification is the best option to enhance the patient’s ability to perform extended reading tasks with the use of his vision. The variable magnification and contrast enhancement will assist with reducing visual fatigue. A portable CCTV is necessary for times when the patient is not in his office, and requires high levels of magnification and contrast enhancement, such as when performing field work. Text-tospeech will further reduce visual fatigue by allowing information to be obtained auditorily. Illuminated hand-held magnifiers are appropriate for quick spot-reading tasks. Two powers were recommended: the lower power allows a greater field-of-view for reading small amounts of printed information, and a higher power was necessary for verification of detailed printed material. Computer software accessibility with options for magnification and speech output is also important for the patient to perform work-requirements accurately and efficiently. Concern over mobility and navigating environments independently, with a history of falls, indicated a referral for orientation and mobility training to enhance independence and safety when traveling. It is also important to inquire about the emotional status of the patient, and to refer to appropriate health professionals or social workers if indicated. Vocational rehabilitation can be challenging, because the visual demands are unique to the job setting and the tasks required of the individual. This patient has high visual demands of detailed information, where accuracy is crucial.
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