Living With Rett Syndrome as an Adult: Challenges and Care

Adults living with Rett syndrome face many medical challenges and typically can’t communicate verbally. However, many remain alert, aware, and ready to socialize or learn. In addition to a robust treatment plan and help with activities of daily living, many people with Rett syndrome require more autonomy and independence as they age. This article will outline the clinical challenges associated with Rett syndrome in adulthood and discuss practical tips for caregivers.

What Does Adulthood Look Like for People With Rett Syndrome?

The International Rett Syndrome Foundation describes adulthood as a stage of “stability and calm” for people with Rett syndrome. For some people, certain symptoms like seizures and hand movements may happen less frequently in adulthood.

Symptoms

On the other hand, this life stage comes with new concerns, like an increased risk of osteoporosis and bone fractures, a tendency toward social withdrawal, and a potential worsening of gross motor skills like walking. Depression, pain, and gallbladder issues can also occur.

Here are some of the most common symptoms documented in adults with Rett syndrome:

• Epilepsy
• Low bone density
• Constipation
• Scoliosis
• Breathing irregularities
• Sleep disturbances
• Irritability, agitation, or screaming/crying
• Gastroesophageal reflux (GERD)
• Recurrent urinary tract infections

Complications

Pneumonia, difficulty breathing, abnormal bleeding, long-lasting or frequent seizures, and a refusal to eat are some of the most common reasons adults with Rett syndrome are hospitalized. Pneumonia often occurs when swallowing problems cause accidental inhalation of food particles. Breathing abnormalities in Rett syndrome can sometimes make it harder to recognize symptoms early. A Rett syndrome specialist can recommend dietary changes that may reduce the risk of aspiration (food inhalation).

In total, there are about 15 areas of assessment that healthcare providers use to gauge the well-being of adults with Rett syndrome. It remains a challenging condition to manage, but a knowledgeable healthcare team can help prevent or treat complications. Visiting specialists every six to 12 months is recommended.

Pushing Boundaries

Although the symptoms of Rett syndrome tend to take center stage of any discussion, it’s important to remember the bigger picture. Symptoms often include significant intellectual disability (learning disability), but regression often stabilizes over time. Many people with Rett syndrome have learned to communicate through assistive devices, and about 10 percent can walk completely unaided.

Many adults with Rett syndrome can make friends, laugh, have a conversation, read a book, learn new skills, and even go to work. Everyone with Rett syndrome is different, and some may face greater challenges than others. But adulthood with Rett syndrome doesn’t always look like endless appointments. It can look joyful, hopeful, and connected.

Tips for Rett Syndrome Caregivers Navigating the Transition to Adulthood

Helping your young adult transition to a more autonomous, independent lifestyle may seem scary at first, but there’s a practical purpose. Adults with Rett syndrome tend to have a life expectancy into middle age. Your child may outlive you, or you may struggle with their daily care as you both age.

It’s completely natural for children to want to strike out on their own, but the journey and end goal can look a little different with Rett syndrome. Here are some practical tips to help you get started.

1. Prioritize Autonomy, Consent, and Privacy

Growing up means learning how to share our opinions and make decisions about things that affect us. It’s normal for young adults to want to take more ownership over their lives as they transition to adulthood, and it’s important for caregivers to step aside and make space for newfound autonomy and independence.

That doesn’t mean leaving your child to fend for themselves, but rather asking for an opinion, giving guidance, and respecting their final choice.

Start asking healthcare providers to speak directly to your child during appointments and to obtain their consent for procedures, even if you’re legally allowed to make decisions on their behalf. Offer to remain in the waiting room to respect your child’s privacy. You may find they still welcome your participation and guidance while appreciating your show of respect.

Think about other ways to honor your young adult’s autonomy and independence. Here are some examples of questions caregivers can consider asking:

• Would you like to live in alternative housing or remain with me?
• Do you want to continue your education or train for a job?
• Do you feel like any part of your therapy or treatment is ineffective?
• Do you want to make more decisions about day-to-day things like groceries?

Once you ask, be willing to honor their response.

2. Consider Legal and Financial Necessities

Some people with Rett syndrome may not be able to safely make their own decisions or consent to treatment. In order to continue directing your adult child’s care and benefits, you will need to ask a judge to grant you guardianship status. In some states, it’s called a conservatorship.

You may also want to consult with an estate lawyer about drawing up documents like a special needs trust, a letter of intent, and a will. These documents ensure your child will continue to receive government benefits and care in the event of your death. You can also name a new guardian in your will.

3. Re-Evaluate Communication Tools for Independence and Socialization

Many people with Rett syndrome have been taught to communicate with assistive devices. Make sure these tools can adapt to a young adult’s growing need for independence and new supportive relationships.

Consider upgrading to an eye-tracking device that not only speaks out loud, but also lets someone use mobile apps and send emails, texts, and social media posts. This can open new doors for socialization and fun. Teens without Rett syndrome often get their first smartphones or social media accounts as they transition to adulthood, and your child can participate in the same rite of passage with a few technical tweaks.

If your child’s assistive device is image-based, take time to update the image library with age-appropriate concepts and ideas. If you’re not sure where to start, consider the results of a 2018 survey of more than 200 people with Rett syndrome. Their median age was 18 years, and some of their most common interests included people, music, animations, books, and things to see. Expanded vocabulary around a person’s specific interests may help uncover new interests and increase quality of life in adulthood.

4. Discuss Day Programs and Other Activities

Adults with Rett syndrome are at an increased risk of muscle and bone disorders. Staying active may help to strengthen bones, improve flexibility, reduce pain, and improve balance. Medical experts recommend sticking with a long-term program that offers opportunities for social interaction and physical activity.

For adults, this may look like formal occupational or physical therapy sessions or possibly a day program designed specifically for people who are transitioning to a more independent lifestyle.

Because autonomy is important, research the options and discuss them with your young adult. Ask if there are any other activities they’ve been thinking about, like aquatic therapy or horseback riding therapy.

Join the Conversation

On MyRettTeam, people share their experiences with Rett syndrome, get advice, and find support from others who understand.

What tips do you have for parents of teens with Rett syndrome? Let others know in the comments below.