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A Report on a Survey of Late Emerging Manifestations of Congenital Rubella Syndrome

by Helen Keller National Center on May 1, 1991
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By Nancy O’Donnell

Coordinator, Special Projects

October 1991

Helen Keller National Center 111 Middle Neck Road Sands Point, NY 11050

Author’s note:Since the following report was published in 1991, the response from families, individuals with CRS and professionals has been incredible. There was an obvious need for discussion to continue on this topic. To that end:

  1. The Helen Keller National Center maintains a listserv to facilitate discussions among an international community of parents, consumers and professionals interested in CRS. Individuals from countries such as Australia, New Zealand, Japan, the Nordic countries, Scotland and England participate in this list. To join, send an e-mail to me at HKNCNOD@aol.com.
  2. The National Family Association for Deaf-Blind newsletter, News From Advocates for Deaf-Blind, often includes articles or updates on CRS. To become a member or to receive a sample newsletter, contact NFADB at 111 Middle Neck Road, Sands Point, NY 11050; 1-800-255-0411; or NFADB@aol.com
  3. Canada conducted a survey of their rubella population in the 1990s. That report is available from the Canadian Deafblind and Rubella Association, 350 Brant Avenue, Brantford, ON Canada N3T 3J9; Internet: http://www.cdbaontario.com/about/about-cdba-ontario_e.php

We continue to hear about late onset medical conditions developing in those with CRS. Unfortunately, there has been little medical research conducted to clinically determine a definitive link between congenital rubella syndrome and the various medical conditions reported. Anecdotal reports of medical and emotional issues surely convince us of the need for further clinical investigation. Families and individuals living with CRS deserve answers to their concerns so that they can manage their lives in a healthy and proactive way. We implore the medical research community to renew their interest in, and commitment to, finding answers about CRS.

As always, we appreciate your feedback. We may not have all the answers, but we will continue to seek them. Nancy O'Donnell, Helen Keller National Center


Statement of the Problem

This report will discuss information on individuals with deaf-blindness whose parents/caretakers responded to a survey regarding late emerging manifestations of Congenital Rubella Syndrome (CRS). The survey was conducted in response to concerns expressed by parents and service providers who had observed dramatic changes in behavior and/or medical conditions in their adult children/clients with CRS. There has been very little recent information written about this phenomena, although late onset medical problems have been documented, particularly in the Australian Rubella population boom in the 1940s. An excellent resource for parents and professionals about CRS is the November 1980 edition of "American Annals of the Deaf," Volume 125, No. 8. The entire volume is dedicated to the topic of rubella. Although the information is not current, it provides a comprehensive overview of the population and the challenges they face. Other resource articles are listed at the end of this report, in the bibliography.

Procedure

The survey was conducted in two phases. In Phase 1, which took place from November 1989 to April 1990, an interviewer contacted parents/caretakers by phone, asked questions and filled out a survey form while talking with the parents. In Phase II, June 1990 to 1991, a large scale mailing was conducted across the country. The mailing list was composed of names received through parent organizations, rehabilitation agencies, the Helen Keller network of Affiliated Agencies, HKNC Regional Representatives and through word of mouth. Information was requested on each person's:

  • Residential Placement
  • Employment
  • Hearing and vision
  • Communication methodology and opportunities to communicate
  • Increase or decrease in negative behaviors
  • Changes in lifestyle
  • Medical conditions and age of onset

Most of the questions were in a yes/no format or multiple choice with space for comments.

Our goal was to gather information on individuals 16 years of age and older who were reported to be deaf and blind as a result of CRS. During Phase I of our effort, 39 interviews representing 19 states were completed by telephone. Much of the information received during these interviews was anecdotal in nature.

Since Phase II, we have received and analyzed 88 additional responses representing another 14 states. The percentages reported herein are based on those 88 responses. However, the impressions, quotations and interpretation of the data also reflect the wealth of information gathered from the initial 39 interview

The following report is divided into two main sections. Section I provides a general overview of the respondents. Section II focuses on medical information.

1. GENERAL OVERVIEW OF RESPONDENTS

Fifty-eight percent of the respondents were male, forty-two percent were female. The largest percentage of respondents (32 %) fell into the 25-26 year old category, reflecting individuals who were born as a result of the rubella epidemic of the 1960s.

A. Residential Profile

  • 41% were reported to live with parents/family
  • Almost 20% were in some type of group home
  • I I% live in MH/MR facilities (institutions, state hospitals)
  • 9% were in foster care
  • 9% were in an intermediate care facility

B. Employment Profile

  • Approximately 32% work in a sheltered workshop or work activity center
  • 27% are students
  • 20% are in a training facility, including employment training
  • 2% are competitively employed
  • 3% are engaged in Supported Employment

C. Hearing & Vision

  • Almost 50% reported little or no usable vision, while 30% have significant problems
  • Approximately 90% were considered to have no functional hearing
  • 45% fell into the category of profoundly deaf with no vision and another 28% were profoundly deaf with significant vision problems

D. Communication

The following percentages indicate a yes/no response to a particular method of communication. It does notrate the capability of the individual in each category. Respondents were allowed to check as many methods as applied

Receptive Method

Out of a list of ten possible methods for receiving communication, sign language was the most frequently indicated (83%).

  • Gestures and prompting were checked by 60% of the respondents.
  • One fourth of the cases were reported to understand fingerspelling.
  • 5 individuals could read braille (6%).
  • 3 individuals were described as having no receptive method of communication (3%).

Expressive Methods

  • Sign language was the most common method of expressive communication (70%).
  • 55% are reported to use gestures.
  • 11% use speech, either alone or in combination with another method.
  • 6 cases reported no method of expressive communication (7%).

Opportunities to Communicate

  • 16% of the respondents answered "no" when asked if there was someone in the deafblind person's situation who could communicate effectively with that person.
  • 23% answered "no" when asked the same question in response to their work placement/day program.

E. Behaviors

Respondents were asked to indicate which out of 9 behaviors were currently demonstrated by the deaf-blind person and whether the frequency of these behaviors had increased, decreased or remained the same over the past year.

  • Self-stimulation and vocalization were the most commonly occurring "behavior problems," often reported every day. Approximately 60 cases (68%) reported that this behavior decreased over the past year.
  • Disruptive activities were the next most frequently noted behaviors. Ten individuals (11%) were reported to do this every day. Forty percent (40 %) are disruptive only occasionally. Approximately one-third of the respondents are not disruptive at all.
  • Tantrums/outbursts were noted at least once a week in 35% of the cases. This behavior occurred occasionally in one-third of the individuals. Almost 20% did not demonstrate this behavior at all. The frequency of this behavior was noted to have decreased or remained the same in 60 cases (68%) over the past year.
  • Self-injurious behavior was reported to occur at least once a week in 35% of the cases. 40% did not demonstrate this behavior. About 40 cases (45%) indicated a decrease in this behavior since the past year.
  • The most serious demonstration of behavior problems (Damages Property and Assaults Others) were reported to occur in 11 cases (12.5%) at least once/week. Thirty-four (34%) were assaultive occasionally; 28% damaged property occasionally. Approximately 50% did not demonstrate either behavior.

In general, there was a definite trend in all behaviors decreasing or remaining the same over the past year.

F. Lifestyle Changes

Individuals with CRS are frequently described as having difficulty accepting changes in their routine/environment. Information was requested on the number and types of changes that occurred during the past year. The results were as follows:

  • 27% - change in health
  • 26% - change in living arrangement
  • 43% - change in family/roommate/staff (residential)
  • 33% - change in work
  • 36% - change in staff/fellow employees
  • 18% - change in transportation

Obviously, this is a group that experienced significant changes in their environment. Many were involved in several changes over the past year. What effect did these changes have on their behaviors, their communication opportunities and their general quality of life? How can we plan for more consistency, particularly in staffing patterns?

2. MEDICAL CONDITIONS

I would like to give special thanks to Barbara Fedun whose knowledge, patience and expertise were critical in preparing this section.

This section is divided into seven categories according to problems noted in the following areas:

A) Auditory Disorders B) Ocular Damage C) Cardiac Problems D) Endocrine System (Diabetes and Thyroid) F) Degenerative Conditions G) Esophageal/Gastrointestinal Symptoms H) Miscellaneous

Each category will be introduced by information cited in previous articles or studies. This will be followed by our own findings, including comments, questions or impressions from parents who participated in the survey.

A. Auditory Disorders

“Long Term follow-up of these children indicates that almost all eventually show signs of some neurologic damage, the most common being sensorineural hearing loss, which approached 93% at seven to nine years of age.” (2)

“The most common handicap associated with rubella is hearing loss which affects about 73% of the cases. The losses are generally bilateral and sensorineural.” (1)

“…The hearing defect may become progressively worse after the first year of life. There have also been cases in which children with CRS, for whom audiograms and speech patterns are normal suddenly develop mild-to-profound sensorineural hearing loss. The latest age at which this occurrence has been well documented is ten years.” (4)

Ninety-two percent of our respondents in Phase II indicated that their child had little or no hearing at the time of the survey. One case of late onset hearing loss was reported at age 10. Another mother mentioned that her daughter seems to not hear as well 'as the years go by." In Phase I, however, several parents reported a decrease in their child's hearing. One case involved a female, age 20, who also developed glaucoma at age 16 and hirsutism (increase in facial hair, body hair) accompanied by an increase in weight and breast size. Another case involved a male, age 25, who also was experiencing many seizures, had developed glaucoma at age 20, had a detached retina repaired at age 24 and had demonstrated "a lot" of head banging. A third case involved a young man, age 24, whose mother has noticed a decrease in hearing over the past five years. This individual also has glaucoma.

An interesting case surfaced, however, during the telephone survey I will leave it to you to come to your own conclusions:

One mother described a son with some usable vision and no apparent hearing. At age 12, he lost his remaining vision. Soon after this, she noticed that his hearing seemed to "kick in." He began responding to environmental cues and directions. At the time of this survey, he was 23 years old and his primary means of receiving communication is aurally. How does the mother explain this unusual development? She related this to information she had learned from a seminar she attended on autism years ago. It was explained that many autistic children experience "sensory overload" there is too much information coming in through their eyes and ears which they can't process so the children just shut these systems down. Teachers were reported to have more success if they focused just on visual input or auditory, not both. Perhaps in her son's case, she theorized, he had experienced the same type of overload. When he later lost his vision, the overload was reduced and perhapshe was then able to "tune in" to his hearing. Interesting food for thought.

B. Ocular Damage

Vernon, Grieve and Shaver reported “about 33% of rubella youths have visual defects…Glaucoma is present in 4% of rubella infants…20-50% of rubella infants…have cataracts.” (1)

Glaucoma with late onset was reported by Boger in 13 patients with CRS. The diagnosis was first made when the patients were between 2 and 22 years of age. In 11 cases, the eyes were microphalmic, 2 had eyes of normal size. All had cataractous lenses either removed surgically or absorbed spontaneously. (4)

In one study, almost 10% of patients with prior ocular damage developed additional forms of eye damage as a delayed manifestation. Cataracts occur in 25% of those infected between 25-93 days gestation. (4)

Cooper stated that 50% of the time, cataracts are unilateral and associated with microphthalmia. In 1969, he reported that while he had seen no patients with both cataracts and glaucoma, they do not appear to be mutually exclusive in the same eye. (This is not to be confused with the glaucoma that is secondary to a poor outcome of cataract surgery.) (10)

Cataracts

Sixty-six percent (66%) of our respondents reported having cataracts, with 57% indicating that these were congenital. Two cases of cataracts were identified at age 7, one case at age 16 and one at age 18. Several parents wanted to make sure that we include an alert to other parents as to the increased risk of their children developing- glaucoma as a result of cataract surgery. Late onset glaucoma has also occurred in children who never had cataracts and in those whose unilateral cataract was never operated on. Please be aware of this and have your sons and daughters tested for glaucoma on a regular basis!

Detached Retina

There were 12 cases of detached retina (13%). For those who reported an age of onset, there was one case diagnosed at each of the following ages: 2, 8, 10, 12, 16, 17, 18, 19, and 39.

I could not find any article which documented the incidence of detached retinas in individuals with CRS or in the general population. However consider that 35% of our cases were reported to engage in self-injurious behavior at least once a week. Some of this behavior (head banging, eye poking) could undoubtedly contribute to retinal detachment.

Glaucoma

Thirty percent (30%) of our respondents were reported to have glaucoma. This rate of occurrence is very high, when compared to an incidence of 0.5% in the general population. Our survey also asked whether particular medical conditions had been tested for in the past year. Only 32% responded that they had been tested for glaucoma in the past year. We strongly recommend that glaucoma testing becomes part of the annual physical examination. This precaution should be applied as well to CRS adults who are deaf with no previous vision problems, as they have also been found to be developing glaucoma at much higher rates than the general population. (12) Glaucoma prevention is critical in order to try to maintain whatever vision an individual has, particularly in light of additional handicapping conditions.

We have had at least one report of glaucoma that is difficult to control by medication. One parent described a 22-year old daughter (who had had nine eye operations since birth) whose glaucoma was notresponding to treatment.

C. Cardiac Problems

Several studies cited the incidence of heart problems in the CRS population:

“35-76% of rubella youth have heart disorders. Patent Ductus occurs in up to 58%; Ventricular Septal Defect occurs in up to 18%.” (1)

“Hypertension can appear later on due to renal artery or aortic stenosis and may be associated with further occlusions of damaged vessels…these vascular lesions are potential causes of coronary, cerebral and peripheral vascular disease in adults.” (4)

“Heart defects occur in 30% of children infected between 25 to 93 days of gestation.” (2)

“van Dijk, citing Menser, Dodds and Harley, 1967, states that of the 50 high functioning persons born with CRS in 1940, twenty-five years later, three were found with systemic arterial hypertension.” (9)

Fifty-two percent (52%) of our respondents indicated the presence of a heart condition. Patent Ductus, Atrial or Ventricular Septal Defects and Pulmonary Stenosis were the most common. In many situations, the condition was coffected surgically in infancy. Twenty-eight out of 88 participants (32 %) answered affirmatively regarding whether or not cardiac testing has been done in the past year.

Parents did not have a lot of concerns about heart problems until August of 1990, when it was learned that one of our 39 initial participants passed away due to congestive heart failure. In a low incidence population, such as ours, one case like this could have serious implications. However, it was later determined that this 26 year old male had had structural repair to his heart in infancy. Although devastating to the family, this was not a medically unexpected outcome for someone with a long history of complicated cardiac disease.

Another case which created interest was a female, now 28 years old, who was part of an ongoing study on congenital heart defects conducted by Johns Hopkins Hospital. As part of the research, she was put on a Halston heart monitor for a 24 hour period. Surprisingly it was found that her heart stopped beating several times, missing up to 5 beats at a time. In 1987, she received a pacemaker and at the time her mom was interviewed (February, 1990) all was well. At the beginning of 1991, the mother called to report that her daughter is now experiencing tachycardia (abnormally rapid heart rate). She is also retaining fluid in her feet and she is on medication for both of these conditions. A previous occasional problem with gagging has recently gotten worse, to the point where it now occurs on a daily basis. No explanation is known.

We present these two isolated cases as a portrayal of the wide variety of medical conditions and challenges being faced by this population. We are continuing to review this information to try to identify trends and further areas for study.

D. Endocrine Systems

Diabetes

“Research in programs at the Maryland School for the Deaf (1980) and previous findings from Australia indicated that diabetes mellitus is starting to appear among rubella deaf teenagers at an alarming rate of 15-40%.” (1)

“ The most frequent delayed manifestation of CRS is diabetes mellitus. Studies reported by Menser, et al, of patients from the rubella epidemic in 1941 in Sydney, Australia showed that 9 (20%) of 45 of their patients had overt or latent diabetes by the time they were 35 years old. An additional nine patients had abnormal insulin response…The high prevalence of diabetes in this small number of patients is remarkable, since diabetes mellitus develops in the general population in only ~0.1% of individuals younger than 30 years of age…A history of maternal rubella, when known, indicates that infection occurred between the fourth and sixteenth week of pregnancy.” (4)

“A high incidence of overt diabetes mellitus is seen among patients with…(CRS)…who reach adolescence (10%) and an almost equal number of young adults with stigmata of CRS show abnormal glucose tolerance responses.” (2)

O’Dea and Mayhall cite Rayfield, Kelly and Yoon, (1986) “the incidence of diabetes…in association with CRS has been reported to be between 12 and 20 percent.” (6)

Our survey indicated 5.7% (5 out of 88) of our participants are diabetic. The ages of onset were reported as follows:

Age of Onset, # of Cases

2,1

5,1

14,1

24, 1

Unknown, 1

Although our percentage is lower than those indicated in previous studies, it is approximately twenty times higher than the incidence found in the general population. (12)

We must also consider that the bulk of the population in our survey were in the 25-26 year old age range. The Australian study on diabetes included patients up to 35 years old. It will be interesting to see what the next 10 years brings to us here in the States.

In terms of testing, 16 out of 88 respondents (18%) indicated that their son/daughter had been tested for diabetes in the past year. We should keep in mind the symptoms of diabetes include excessive thirst, frequent urination, rapid weight loss, nausea and vomiting.

Please note that several parents also mentioned that their child's blood sugars fluctuate a great deal. This has been observed by Fedun, as well, in her ongoing study of the relationship of diabetes and CRS. (12)

Thyroid

“Hypothyroidism, hyperthyroidism and thyroiditis have all been reported as delayed manifestations of CRS…” Thyroid dysfunction has been identified in 5% of patients with CRS in one study. (4)

“…thyroid disease has been relatively rare…” (10)

“Once again, infection followed by an autoimmune response has been indicated as a possible cause of thyroid disease.” (6)

E. Degenerative Conditions

This section contains some of the most puzzling and disturbing descriptions of changes occurring in individuals with CRS. The following comments written by parents will give you a glimpse into the types of degenerative symptoms some children are developing. The first case describes a 26 year old male.

'Over the past four years, (our son) has gradually lost his ability to walk. In the past year, he cannot stand by himself nor walk at all without totally leaning on another .... (He) was always very hyper ... also very aggressive. He still butts his head quite often and pinches if he is near us. We were recently told that (he) is on a down-hill slide and will eventually lose all control, including muscles, swallowing, etc...The doctor does not know another patient with (this) condition. The doctor advised us to remodel our house to accommodate a wheelchair. Do you know how long a time between wheelchair and bed?"

Another parent described her 24 year old daughter:

'... about six years ago (she was 18) things first changed. She had one seizure She became much more irritable, broke lots of windows, would actually swing at people close to her, wasn't as willing to do things she had always loved and was generally terrible. Her tantrums were frequent, violent and long-lasting. This went on for 2 to 4 years to some degree. About 2 years ago she calmed down some but in November all at once she became very unsteady on her feet. I thought she may have had a stroke. Her gait is much like that of an individual with cerebral palsy. She looked 'drunk' all the time. She had a great deal of trouble walking three feet without hanging onto something. She appeared real confused over this change of events and began to really depend on us to help her get from place to place. But the tantrum stopped.

At this point we had a CT Scan done, took her off her Phenobarb, took her to a heart specialist, otorhinolaryngologist, neurologist and just about everyone else we could think of – no one knows what's going on. Recently I requested an MRI. That did show a large 'scrambled egg' appearing thing. The report ruled out a brain tumor, mentioned in passing and rejected MS so now we know there is something in/on her brain but don't know what it means. They also don't know if it is something 'new' or has always been there. How I wish we had been able to have tests like this when she was a baby.

Right now she has been “off balance” for a year and a half. She has adapted her once springy gait to a wide stance, is sometimes amused, sometimes frustrated over her new limitations (above I observe from behavior and facial expressions - she can't tell me these things) and does not seem to have regressed any more during the last year and a half.'

A third mother provided this account of her daughter, now 26 years old:

'When (she) began her weight gain in 1982, almost overnight after being so thin since birth, I felt we had a problem. Along with this extreme weight gain, she began tantrums, etc. Also (her) beautiful, smooth complexion turned to pimples and acne...(her back was also like this). (Her) behavior became so aggressive, I could not handle her (1986). With all the above, she developed facial and chin hairs, and cleavage hairs. Hirsutism was diagnosed. Ever since 1982, there has been one thing and then another. Diabetes -diagnosed 1988, losing control of (bowel) and bladder (what appears to be aging faster on inside than outside), eyesight declining due to congenital glaucoma, even though the medication is present, it doesn't seem to be effective. On the other side of the coin, we have seen the most miraculous advancement in (her) mental abilities. This has been since 1988. It is almost scary sometimes because she has these conversations with you that are so 'adult' and completely normal. (She) talks with wisdom and great reasoning abilities!"

During the course of this survey, I have spoken to many parents and professional and listened to questions about these degenerative processes. Often, the discussion would turn to a condition called Progressive Rubella Panencephalitis (PRP). To gain additional knowledge on the condition, I wrote to Dr. Jerry Wolinsky at the University of Texas Health Science Center in Houston. Dr. Wolinsky is a neurologist who has been involved in research, and published articles, on PRP. The following are excerpts which describe the disease process of PRP:

“Progressive rubella panencephalitis (PRP) was recognized as a separate disease entity in 1975 when two groups described patients with slowly progressive neurologic deterioratoins…(PRP) is a rare disease with only about twelve known cases. Seven of these have been well documented; five are with the known stigmata of (CRS) and two with postnatally acquired rubella.” (2)

“A relatively stereotyped clinical syndrome has been defined based on …ten cases. The syndrome is not geographically restricted and, to date, appears only in males…Between the ages of 8 and 19 years, neural deterioration becomes apparent. Deterioration in school performance and behaviors are early symptoms, sometimes associated with seizures. With time (there is) a progressive global demential…The gait becomes clumsy early. Ataxia becomes prominent and progresses to involve all limbs…The syndrome evolves slowly but relentlessly over several years.” (3)

“Intellectual function is dramatically impaired…All known cases of PRP thus far have proven fatal.” (5)

It must be emphasized that PRP is a very rare disease which has only been well documented in 5 cases of individuals with CRS. There are very specificand complex tests, as described in these articles, that are necessary in order to come up with a diagnosis of PRP.

So, if not PRP, then what else could explain the deterioration that is happening to some children? In their article, published in 1985, Sever, South and Shaver surmised that “new neurologic abnormalities may occur that are not followed by the relentless deterioration that is characteristic of PRP. These abnormalities include behavior disorders and seizure disorders, but are not associated with the ataxia of PRP... Such abnormalities probably represent another delayed effect of earlier acute rubella encephalitis." (4)

We will continue to follow these phenomena and encourage any insights and information that the readers can share.

F. Esophageal/Gastrointestinal

This category has been created in order to highlight reports of gagging, and vomiting. These symptoms do not seem to fit into any type of pattern which we could identify. They first surfaced during conversations with several parents who participated in the telephone survey. In two cases, it involved young men who also have cerebral palsy. The young woman described in the section on Heart Problemsalso developed a problem with gagging. Phase II of the survey brought us more examples of this behavior.

One parent described these symptoms in her 19 year old daughter:

"The past year (especially 3-5 months) we have had some strange behaviors come and go with (our daughter). Your late onset manifestations of CRS was very insightful as (she) is definitely experiencing changes, whether physical or emotional is anyone's guess. She has had some weight loss, decrease in appetite with intermittent crying spells at times, when crying she does a strange throat sound prior to this - then just sobs. During these times she gags violently as if to vomit but doesn't. If anything it's only mucous. She has had most exams done - blood tests, urine - complete dental checks under anesthesia and we have found no obvious physical ailment."

This was written by the mother of a 24 year old daughter:

'The doctors are unable to determine the cause of her recurring vomiting (every 3-9 weeks for past six years). '

The Canadian survey verified this finding in their population. (Monroe, 1999) Twenty-four percent of their respondents indicated a problem with either swallowing, gagging or vomiting. Most of these occurred in early childhood, while nine reported first onset from the teen years or later.

G. Miscellaneous

Many parents had questions/theories/suggestions that they wanted us to pass on to other parents. In some cases, we have been able to include these in specific sections of the report. The remainder are presented here.

'Please let other parents know that these children have smaller than average extremities. Their feet are small. This poses a problem for those who have put on extra weight. There could be pressure on the ankle area and problems supporting all that body weight.'

'Medic Alert is creating a special registry for patients who have had implants (such as pacemakers) in case there is a product recall. Contact Medic Alert for more information.'

"In my (CRS) son's group of peers at school, the siblings born after the rubella child seemed to have more than their share of their own problems. My daughter had a condition on her retina which looked like a 'rubella' retina. Other children had siblings with learning disabilities, vision or hearing problems. Is there any medical basis for this, or is it just a coincidence?'

'I am glad you are doing this survey ... I am am interested in psychological factors /symptoms which seem to change - compulsive behavior, aggression, anxiety, "ticks, ' etc. What can we blame on rubella and what can we blame on our child's 'gene pool?' Good Luck!'

"We are interested to know any further developments with the late onset of CRS. Anything we can do to prevent these problems from developing?'

"Are other parents having as difficult a time getting medical insurance for their child as I am? My son has been turned down several times, even with the suggestion that they put a 'rider' on his eyes.

"Are these children aging more quickly because of the rubella? Will that mean that they have a shorter life expectancy? What are the implications for planning their future?"

"My son has been experiencing seizures since he was a young boy...not the commonly known types of seizure, like gran mal' and 'petit mal. For example, have you ever heard of abdominal seizures? When he was young, he would throw up and then fall asleep right after that. After being put on seizure medication, this stopped. He later outgrew them. At age 18, seizures started again. Recently they have become more frequent. Many of the symptoms of his seizures could have been mistaken for 'bad behavior.” ( Author’s note: These seizures were eventually controlled through medication.)

General Conclusions

By studying all of the data and listening to the parents who voiced their concerns, an interesting picture is emerging of the adults with CRS who are represented in our survey. Almost a third of our respondents indicated nosign of late emerging medical problems. The problems that were reported ranged in severity from individuals who "only' developed glaucoma to one extreme case of a woman who developed every late onset symptom on our list except diabetes. (Author’s note – in 2000, this woman’s mother reported that her daughter developed diabetes as well.) Researchers still do not understand the mechanism which triggers the development of these conditions. Three possibilities are autoimmune response, reactivation of the virus and persistence of the virus. The probability of any one person developing a particular condition is unknown as is a time frame in which these things might occur.

We can expect that individuals with CRS will be susceptible to the same ills that the general population develops. We can only guess as to the effects of the various stresses on their lives, health and behaviors. If we look at the survey respondents as a whole, we see a severely involved group of young men and women, many profoundly deaf and totally blind or with significant vision problems, with limited communication systems, sometimes with limited opportunity to communicate, They are undergoing, or have the potential to experience, significant changes in their medical condition, yet are very limited in their ways to tell us about their symptoms. They are often subject to changes in their living and/or working situation, yet are often unable to be prepared for these changes because of their own limited communication or that of the people around them. If we feel any sense of urgency in relation to this population, it is, of course, in regard to their medical status first. However, one simply cannot ignore the red flags surfacing in the areaof "communication." At least a third of the parents with whom I spoke on the phone specifically mentioned their concerns about the lack of communication instruction/intervention/opportunity that their child had, especially those who are out of the educational system.

In looking at behaviors as communication, our data depicts a higher frequency of negative behaviors in those who have the least opportunity to communicate at work and at home. These individuals are not "bad" ... they are frustrated, sad, confused, bored, angry, not feeling well, etc., and they are trying to tell us this in the only way they know how. We who are communicatively "intact" need to do a much better job of figuring out what these individuals are trying to say and helping them to say it in more appropriate and effective ways.

If you are looking for a good resource book for yourself or for those who work with your son or daughter, I would like to recommend two books: Enhancing Nonsymbolic Communication Interactions Among Learners With Severe Disabilities by Ellen Siegel-Causey and Doug Guess. It is an excellent introduction to understanding basic communication attempts. It gives concrete examples and suggestions on how to develop a communication program, including guidelines and strategies. Remarkable Conversations: A guide to developing meaningful communication with children and young adults who are deafblindby Barbara Miles and Marianne Riggio is another excellent resource. It is available through Perkins School for the Blind at a cost of $35. Contact their Publications Department at 617-972-7328.

As you can see, we don't have all "the answers." It is obvious that there is a tremendous amount of work to be done and the information gathered must be shared. If you have any comments or questions after reading this report, new information you want to add to the survey, or if you have not yet participated in the survey and wish to do so please write or call: Nancy O'Donnell, HKNC, 111 Middle Neck Road, Sands Point, New York 11050, (516) 944-8900 ext 223 or HKRegistry@aol.com.

Bibliography

(1) American Annals of the Deaf, Vol. 125, No. 8, November 1980. "Handicapping Conditions Associated with the Congenital Rubella Syndrome." Vernon, Grieve, Shaver.

(2) Neurologic Clinics, May 1994, Vol. 2, No. 2, "Rubella Virus and Its Effect on the Central Nervous System." Waxharn & Wolinsky.

(3) Handbook of Clinical Neurology, Vol.12, (56) Chapter 22. 'Progressive Rubella Panencephalitis.' Wolinsky, Jerry.

(4) Journal, of Infectious Diseases, Vol. 17, Supplement 1, March-April 1985, "Delayed Manifestations of Congenital Rubella." Sever, South & Shaver.

(5) CHWeal and Molecular Aspects of Neurotropic Virus Infection (1990). "Rubella Virus and Central Nervous System Disease.' Slagle & Wolinsky.

(6) Journal of Visual ]Impairments and Blindness, November 1988. "Delayed Manifestations of Congenital Rubella Syndrome." O'Dea & Mayhall.

(7) American Annals of the Deaf, Vol. 125, No. 8, November 1980. "Deaf-Blind Children with Maternal Rubella: Implications for Adults Services." Lockett & Rudolph.

(8) American Annals of the Deaf, Vol. 125, No. 8, November 1980. "The Demographics of Deafness Resulting from Maternal Rubella.' Trybus, Karchmer, Kerstetter and Hicks, W.

(9) Persons Handicapped by Rubella: Victors and Victims. A follow up study (In Press,1990) "Current Status of the Rubella Problem." Jan van Dijk.

(10) "The History and Medical Consequences of Rubella". Conference of Infectious Disease 1985. Louis Z. Cooper, St. Luke's-Roosevelt Hospital

(11) Enhancing Nonsymbolic Communication Interactions Among Leamers with Severe Disabilities.Ellen Siegel-Causey & Doug Guess. Paul Brookes Publishing Company, P.O. Box 10624, Baltimore, MD 21285-0624.

(12) Observations - Ongoing Study of the Relationship of Diabetes and Congenital Rubella Syndrome. Barbara Fedun, St. Luke's-Roosevelt Hospital 1991.  


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