Families in Nepal - Deafblind International

esearch briefingdevices for mobility functions. In the non- rubellagroup this deterioration takes place early in life, andin the rubella group only takes place from age 30 andonwards. This may indicate the presence of a latemanifestation of their condition.In both groups there is a relatively high incidence(15,5%) of muscular deterioration and poor balancein adulthood. This reinforces the requirement thatcongenitally deafblind persons need treatment fromphysiotherapists and occupational therapists duringadulthood.Vision and hearingThe majority of the participants, in both groups, havea visual impairment that was diagnosed during thefirst year of life.Around two thirds of the population withcongenital deafblindness have a progressive visionloss (like glaucoma, retinal detachment, decreasingvisual field, and removal of an eye). We can alsoattribute the generally occurring age related changesin vision to much the same level in both groups. TheDanish survey indicates that: persons with CRS are at high risk of developinglate manifestations e.g. glaucoma;the combination of glaucoma and cataract isspecific to the diagnosis of congenital rubellasyndrome;retinal detachment is also significantly morefrequent in the rubella group. A possible triggerfactor might be cataract;Microphtalmus is present in every secondindividual in the rubella group and in every fourthin the non-rubella group;there may be a relationship betweenmicrophtalmus and later development ofglaucoma.In both groups individuals have severe hearinglosses or deafness (the latter is more frequent).“Moderate” hearing losses are only identified in thenon-rubella group. Both groups show that the hearingimpairments are identified later than the visualimpairments.Individuals in the non-rubella group are generallydiagnosed earlier than individuals in the rubellagroup. This finding may surprise, as a congenitalcataract, possibly in combination with low birthweight and heart failure should cause suspicion ofa concurrent hearing loss caused by a rubella virusinfection.Hearing aidsAccording to our information 50 individuals had ahearing aid at the time they were diagnosed. Todayonly 16 individuals still wear their hearing aids.Progressive hearing losses are identified in bothgroups. However, the prevalence of progressivehearing losses may be higher than shown in the datacollected, as, unlike progressions in vision loss, theprogression in hearing loss will not be immediatelydetected in observations of changes in behaviour. Forthis very reason it seems alarming that appropriateexaminations are not being undertaken at regularintervals.With regard to hearing and vision losses the surveyrecords that the awareness of the risk of progressivesensory impairments is lacking in the environmentswhere congenitally deafblind adults live. The surveyalso indicates that there is more awareness of thepossibilities of decrease in vision. This is becauseevery second individual is regularly taken for eyeexaminations, and only one in five gets their hearingchecked.Hormonal conditionsProblems with metabolism are a known latemanifestation of Down syndrome, and have beenmentioned in foreign studies as a late manifestationof congenital rubella syndrome as well. None ofthe participants in the Danish survey reported anydiagnoses with regard to metabolic problems, so itmay seem not to be a matter of late manifestation.Nevertheless, we must state from the datacollected that 10 out of the 20 individuals in therubella group are overweight and in the same 10people between 2 and 6 symptoms of low metabolism(e.g. tiredness, sadness, increase in emotionalinstability, passivity, less stamina and weight gain)are reported.Foreign studies demonstrate that CRS entails amajor risk of developing diabetes. In our surveywe identified a few cases of diabetes (3), in therubella group only. Whether this finding is astatistical coincidence or it actually expresses a latemanifestation cannot be judged from the presentstudy.Growth retardation has been mentioned as aspecific problem in persons born with CRS. We haveidentified cases of growth retardation in both groups,but the prevalence is clearly higher in the rubellagroup.Neuropsychological conditionsEpilepsy, according to our information, occurs withalmost identical frequency in both groups, but the ageof diagnosis is different.In the non-rubella group the epilepsy is diagnosedat an early age and must be considered a part of theclinical picture. The reports of individuals havingepilepsy in the rubella group confirm the assumptionthat epilepsy is a late manifestation – likely to appearin the teenage years.In our survey we also asked for information onchanges in neuropsychological phenomena likeattention, concentration and memory. Here we findJanuary – June 2004 5