Rubella Network

Nancy O’Donnell reports:

These days, it is rare to find an entire book dedicated to rubella. “Dangerous Pregnancies: Mothers, Disabilities and Abortion in Modern America” by Leslie Reagan, Ph.D., is a recently published book about the impact of rubella from a historical perspective.

I met Dr. Reagan in 2008, when she visited the Helen Keller National Center library as part of her research for this book. She was looking for information about the effects of rubella on families, politics, and society as a whole, and on mothers in particular. Her book brings us back to the 1960’s, the period of the rubella pandemic. It walks us through its impact on society, health care, laws, educational policies and human rights, up to the current day. It is a must read. (University of California Press, ISBN 978-0-520-25903-4).

Stan Munroe reports that the Canadian Deafblind Association (CDBA) will be conducting a follow up survey on adults with Congenital Rubella Syndrome (CRS). This will be an update to the 1999 Canadian survey on the “Late Manifestations of Congenital Rubella Syndrome”. This new project will include distributing a new questionnaire to families and facilities to learn more about the ongoing impact of these late emerging medical and behavioural conditions believed related to Congenital Rubella Syndrome. Nancy O’Donnell

CONTACT DETAILS:

Trees van Nunen

E-mail: t.vannunen@kentalis.nl

The Rubella Network had its first official meeting at the DBI conference in Perth, Australia, in September! Our session was only about an hour long, but it was just enough time to get the fire going in the group. The room was filled with professionals, parents and consumers with CRS from all over the world. The consensus of the group is that we must not let CRS disappear from global consciousness. Because the incidence of rubella is slowly decreasing or even being eliminated in some parts of the world (thanks to better immunization policies), there is a tendency to think that rubella no longer needs to be addressed. But as we say in the U.S., rubella may be eliminated, but adults with CRS are alive and thriving. Late onset health issues are still a concern and we need to do a better job of figuring out the long term impact of CRS. Our work continues!

At the network meeting, we handed out a short survey to try and get a better idea of immunization policies, numbers of people with CRS worldwide, information and resources that are available, and how the network can be most helpful.

We received a heartfelt request from Africa for support of national immunization programs throughout the continent. This is a country with many needs and few resources. As a group, we need to take a look at what we can do to help.

There were 3 presentations on rubella at the conference, including the very exciting report by Trees van Nunen and Anneke Schoenmaker of the Netherlands who reported medical and psychological data of approximately 65 adults with CRS born before 1985. Joff McGill of the UK addressed MMR/autism debate. His full presentation, and the latest on rubella and CRS will be available on our webpage.

CONGENITAL RUBELLA SYNDROME – HEALTH AND WELLBEING

Sarah Butler and Joff McGill report:

This joint Sense and Sense Scotland event, which took place between 6th-7th March in the UK, brought together fifty practitioners, as well as scientists, families and people with CRS to share our latest knowledge about congenital rubella syndrome and its ongoing impact on individual’s health.

Sense has produced a full report of the information gathered; visit their website.

An overview

In much of Europe and the Americas rubella is now almost absent because of comprehensive immunisation programmes, though elsewhere rubella is still a significant problem. Even where new cases of CRS are rare, adults continue to live with CRS: so we need to understand the on going health concerns of individuals with CRS and how to support them.

Though scientific research into the effects of CRS on adults is still limited, we do know that people with CRS are more likely to suffer thyroid conditions and diabetes than other people. We also suspect that CRS may cause other conditions to develop earlier or more often.

There are a number of studies of CRS, often carried out by deafblind organisations. There are difficulties in comparing these studies because of differences in sample groups (type and size), different people responding to the study, and different timescales (when the study happened, the age of those studied and the year in which they caught rubella). None the less, they give us useful indicators as to the health indicators we need to look for in our work.

More importantly they point to a clear role for support staff and professionals, that of health detective. Staff have a crucial role to play observing and recording what they see, reflecting on this and finding out what health issues people may have. It doesn’t stop there though – we need to identify what we can do to help and find ways to be taken seriously when working with health professionals.

Supporting healthy living is vital – we all need to make friends, keep active, eat and drink well, discuss our feelings, have a break, do things we are good at and have a sense of purpose. As professionals and support staff we need to find ways to support well-being. The seminar chose to focus on the following:

• Endocrine problems: thyroid conditions and diabetes
• Vision
• Hearing
• Circulatory issues
• The Brain and behaviour
• Sharing different cultural ways of using and viewing the outdoor environment.

ENDOCRINAL PROBLEMS:

The way that hormones work in our bodies is extremely complex and not yet fully understood. We do know, however, that people with CRS are more likely to develop certain hormone-related problems, in particular thyroid problems and diabetes.

The thyroid may become over stimulated, which causes the body’s systems to run at high speed. Or, if the thyroid is underactive the body’s systems slow down. Both conditions can be treated with medication.

Diabetes is extremely common, and is caused by a lack of insulin. This means that the body cannot absorb glucose, and so cannot derive energy from food. Though a small proportion of diabetics need to inject insulin, most can control the problem by managing their diet, exercise and lifestyle.

VISION:

A defining feature of CRS is the eye problems that people are born with. Many babies are born with cataracts – which nowadays are operated on, and replacement lenses inserted. Some may be born with small eyes and most have some vision problems.

As people with CRS grow older it seems that they are more prone to other eye conditions than the general population. The most common conditions are cataracts, glaucoma and retinal detachment. In addition, because more people with CRS develop diabetes, they are also more likely to develop diabetic retinopathy.

It is therefore crucial that everyone with CRS has regular eye checks. For some people these eye checks can be difficult but they are worthwhile for everyone. The key to successful eye checks is to build good relations with the eye clinic and to prepare well.

It is also important to be aware of the signs of deteriorating vision such as a person becoming clumsier, or finding it hard to judge the height of steps and curbs. Staff need to watch out for these symptoms and to seek help urgently as they can indicate a serious problem that needs immediate treatment.

HEARING:

Ear problems are the most common effect of CRS and are present from birth. Because both the inner and middle ear may be damaged this may cause problems not only with hearing but also with balance and movement. As people get older they may also experience age-related hearing loss and loss caused by damage to the ears by loud or persistent noise.

The most common ear problems experienced by people with CRS are tinnitus, recruitment (where certain frequencies are very loud), loss of balance, auditory processing disorder, and problems with integrating the senses.

It is often hard to tell the effects of damage to the ear as clinical tests are at best a rough guide. We therefore also carry out extensive functional tests to see how someone hears, and how different situations affect this.

HEART CONDITIONS:

The most common heart problems in people with CRS are patent ductus arteriosus (PDA) and pulmonary artery stenosis. In PDA the child has a hole in the heart that has to be closed by surgery or some type of plug: very rarely an adult needs treatment for PDA if it was not dealt with in childhood. Pulmonary artery stenosis describes a narrowing of an artery which causes reduced blood flow to the lungs and puts a strain on the heart. The artery can be widened by inflating a balloon via a catheter.

When a deafblind person receives treatment in hospital they will need support: preparation (such as familiarising them with the room where they will be staying) will make this much easier.

CRS, THE BRAIN AND BEHAVIOUR:

Rubella affects the developing brain, which may in turn affect a person’s relationship with the world and their behaviour. The rubella virus can disrupt the development of the neural paths in the sensory, motor and cognitive and integrative systems. Early damage may not have observable effects straight away – we may only see the effects when the affected part of the brain is called into action as the person develops.

While we can use standard checklists to assess a person with CRS’s behaviour, looking for possible neural impairment, we must always bear in mind the powerful effect of deafblindness itself. So while a person’s changing behaviour may be due to impairments in the brain, it may also be due to the way that deafblindness affects their ability to communicate and understanding of the world.

We all behave in challenging ways from time to time – we all lose our tempers, for example, and would hate to be labelled by this behaviour. It’s only an expression of the way we feel, of communicating our feelings or needs. People with CRS are no different – the only difference is that we label some of their behaviour as challenging because it carries risk and because we can find it hard to cope with.

There are many possible causes of challenging behaviour – a particular incident will be caused by many factors. Though it can be a long and complex task to unravel why someone uses challenging behaviours, it is worth the effort as by doing this we can build effective support.

Supporting communication and providing meaningful experiences that form the basis of conversations and shared memories is vital in this.

SUMMARY:

The incidence of CRS is low enough that many non-specialist health professionals know little about it. This can be enormously frustrating for people with CRS and their families. So we have a duty to learn more, to support better and to listen properly. As delegates from all over the world reflected on the seminar, three themes emerged:

• the importance of understanding the initial and ongoing medical effects of CRS and their implications on individual’s health
• the importance of communication between professionals and the person with CRS
• the importance of collaboration between professionals.
• sharing different cultural ways of using and viewing the outdoor environment.

All too often our work in this area focuses on congenitally deafblind people, but if we are truly to understand the on-going health issues that people with CRS face we need to listen to and learn from individuals with CRS who have fewer disabilities, but as many health worries.

More than anything we have to remember the role we play in supporting the health and well-being of those we work with. Being a reflective health detective is as good a start point as any.

There are two groups online where you can share and learn more about CRS. Both are moderated by Nancy O’Donnell at the Helen Keller Centre in New York. One group is for people affected by CRS (many were at school together in Boston, USA): it’s busy, and has two key professional contributors, Nancy O’Donnell and Pam Ryan from Perkins school. The other group is for worldwide professionals working with deafblind people. To find out more about joining, email Nancy O’Donnell.

CLICK HERE for updated information and resources.