Caring for a Child with Fragile X

Approximately one in every 4,000 boys and one in every 6,000 to 8,000 girls is affected by fragile X. However, one in every 259 women is a carrier of the fragile X gene.

It's normal to go through a painful period of adjustment upon learning that your child has a special need. Here are some guidelines that may be helpful:

• Grieve the loss of the child you expected or dreamed about and begin to develop new dreams for the child you have.
• Recognize your child's strengths, as well as his or her limitations.
• Educate yourself about your child's condition.
• Focus on helping your child to be "the best he or she can be." Avoid comparing your child with others.
• Seek help for emotional issues, finding a caring health care provider, and creating an educational program that meets your child's needs.
• In addition to health care professionals and various therapists, other parents in your situation may be wonderful resources, either through providing one-on-one support, in support groups, or through personal stories about their adjustments and coping strategies.

Joining one or more of the support groups for parents sponsored by these organizations should be helpful.

• The National Fragile X Foundation
• Fragile X Research Foundation
• Daily Strength Fragile X Syndrome Support Group
• Fragile X Syndrome Online Support Group

Many children with FXS syndrome are affectionate, empathic, good at imitating others, and funny. Often they have sweet temperaments, enjoy humor, and have excellent memories. They value interacting with others, although they tend to be shy and may have social anxiety.

A fragile X diagnosis usually leads to a family seeking Early Intervention.

Early intervention for FXS involves an assessment and then therapeutic interventions depending on the results of that assessment. The therapy can be given by a teams comprised of an occupational therapist specializing in sensory integration disorder (which is common in people with fragile X), a speech therapist, a mental health therapist, a behavioral therapist, tutoring in social interactions, and a physical therapist, depending on the specific needs of your child.

Become your child's advocate.

Learn everything you can about the disorder.

Demand the services that will help your child.

Challenges occur both out in public and within the home.

Out in public:

• Learning how to feel included in the community without apologizing for your child's behavior can be a difficult lesson.
• Trips to the dentist or doctor can be traumatic for all concerned. Kids may become anxious and develop hypersensitivity in either office. They could act out in ways that disturb personnel or other patients. Going over exactly what will occur in advance, and in what sequence, may mitigate some problems.
• Any family activity (such as dining out or attending church and cultural events) becomes difficult and requires special preparation. Some families prefer to have one parent stay home with the child who has FXS while the other goes out with the other child or children.

Within the home:

• One mother is grateful for the support of her husband when so many other parents of children with special needs get divorced. The stress of the situation can strain family ties, both between parents and between children and parents. Other children might not understand why the child with FXS receive so much attention and why they can't control him.
• Many children with FXS have unusual sleep patterns, difficulty falling asleep, hypersensitivity to fabric which makes them uncomfortable wearing pajamas, and may wander about the house at night or wake up at an unusually early hour. As a result some families install gates or half-doors to prevent their children from wandering. Others find that establishing a bedtime routine and creating a soothing environment can help.
• Many babies with FXS have trouble with both bottle feeding and nursing due to oral motor weaknesses which make sucking difficult.
• Due to problems with inadequate oral sensations, some children with FXS can't tell they have too much food in their mouths until they choke or gag, and can be messy eaters.
• Some find that silverware is over-stimulating and so prefer to eat with their fingers.
• Dressing can also be a problem due to poor muscle tone and extreme sensitivity to clothing.
• Many boys and men dislike washing and brushing their teeth due to hyper-stimulation when they do them.
• Due to poor bodily cues, many kids with fragile X have problems with toilet training.

Within Oneself:

• Some parents ( particularly carriers of the FXS gene) find it difficult not to blame themselves for their child's condition.

 

An occupational therapist can help your family adapt your home environment to meet the needs of your child by:

• Recommending minor changes such as avoiding fluorescent lights, which can irritate some children, or how to lower noise levels in certain rooms. So that your child can feel calmer and less upset by noises.
• Purchasing equipment that will calm your child, such as a swing, trampoline, or rubber balls.
• Teaching you how to calm your child with massage or deep pressure.
• Facilitating your child's use of computers and pencils by finding a mouse with the best shape a pencil with a certain grip.
• Helping your child develop fine motor skills.
• Designing both an environment and a daily sequence of activities that would best foster your child's ability to learn.
• Assessing which kind of work would be best for your child age 22-and-up, and then visiting the workplace to set up helpful adaptations.
• Helping your children to enhance their work skills.

Since boys with FXS often have language delays and learning disabilities, speech therapists can help with speech comprehension and articulation. Also researchers have reported many auditory processing problems in kids with FXS. Issues with attention, memory, word retrieval, and auditory sequencing often affect comprehension and production of speech and language. And speech production of kids with FXS may be characterized by imprecise articulation, a fast rate of speech, unusual voice characteristics, and cluttering, all of which affect intelligibility. Speech therapists can help work on all of these factors.

Depending on the severity of your child's fragile X, you may want to alert the airline well in advance that you will be traveling with a child with special needs. In addition, here are some things you can do:

• Discuss with your child the sequence of events that will happen when you leave your home -- you will park at the airport, walk to the terminal with your suitcases, check in, and wait in line to go through security, then go through the security check point, get on the plane, etc.
• When alerting the airline in advance, discuss how being searched with a metal wand might stress your child.
• Be sure neither you nor your child wears or carries anything metal that could make the detectors go off.
• Bring your child's favorite books, tapes and other activities to help pass the time. CDs also help block out stimuli that could overload your child.

It is important to remember that FXS itself is the underlying cause of the depression. Therefore, standard treatment for depression (or for bipolar disorder) doesn't work as well in fragile X patients, because the treatment doesn't address the underlying genetic condition.

Often, children with FXS are sweet and friendly, but they also can be impulsive, distractible, and hyperactive. In fact, 80 to 90 percent of boys with FXS also have attention deficit disorder (ADD). Many have low frustration levels. Here are some things you can do:

• Devise a behavioral plan.
• The behavioral plan can include calming techniques and modification of your child's environment to remove anything that triggers an outburst.
• It helps to create very concrete plans that have relevant cues and rewards, such as stickers, that can lead to prizes.
• Time-outs to punish unacceptable behavior can be implemented. Time-outs should be short. While one minute per year of age is a good amount of time for other kids, a shorter time-out for kids with FXS is more helpful, since they have shorter attention spans. If a child does something wrong, such as bite another child, the best response is to say simply, "No biting, time-out." Then, wherever you are with the child, implement the time out immediately after the bite, turning the child away from others, and blocking the child's exit. Don't pay attention to the child during this time, as your lack of attention is the main punishment. If the child tries to get away, just repeat the phrase, "No biting, time out."

Transitions have to be planned carefully for those kids with FXS who don't like changes.

• One helpful option is to place pictures of the schedule for the day on a board or on a card.
• Write out or place pictures of schedule changes and insert them into the proper sequence.
• If you know that one of the activities will be disturbing to your child, see if it can be followed by a calming activity. An example would be a loud assembly, which would disturb your child, followed by time on a beanbag chair, or another activity that your child finds soothing.

Interventions that work for children with ADHD can also help those with fragile X. These include:

• Seating your child near the teacher and far from distractions
• Allowing your child to use a private work area at times
• Giving your child brief tasks
• Offering visual cues for sequences of events
• Implementing active interactive lessons, in which your child doesn't need to just sit still and listen.

One solution is to help your child to anticipate the situation.

• Go over the sequence of what will happen, how many people will be there, what the restaurant will look like, how long he will have to sit still, what will happen if he needs to get up, etc. Bring home a menu so you child can anticipate the options and what he would want to order.
• If going to church or a film, try sitting in front to see if it helps your child pay attention. If that doesn't work, try sitting in the back to enable an easier escape if your child can't sit still.
• Many children with FXS find medication for ADHA, anxiety, depression, or aggressive behaviors helpful.

Some people have found that the aggressive behavior exhibited by kids with FXS is often a cry for help -- a way to get out of a situation when they feel overwhelmed. The kids know that by exhibiting that behavior, they'll be separated from other people. So it is important to keep a written record of exactly what happened when each aggressive behavior ensued. Then you can look at see if there are any patterns in the situations that led to that behavior. Some emotional states that have been found to lead to aggression are:

• Anxiety
• Fear
• Agitation
• Confusion.

Once the triggers are identified, then attempting to intervene before the behavior occurs -- by teaching coping skills -- can often reduce the aggressive episodes. Intervening soon enough to prevent the behavior is important, as you don't want the aggressive behavior to become habitual. In addition, medication can help to reduce aggression.

The following interventions have helped to reduce aggressive behavior:

• Seat the child near an exit
• Encourage frequent breaks and build in down or calming time
• Schedule activities that release stress and energy
• Act out consequences to certain behaviors
• Reduce noise and sounds
• Limit classroom size
• Structure the environment with a predictable sequence of events
• Use signals and visual cues to warn of transitions
• Avoid crowds
• Use natural lighting and avoid florescent lights

Sensory integration is the ability to organize light, sound, movement, and texture without even thinking about it. Not having that ability requires a great deal of effort to manage sensory input. Many children with FXS -- mostly males, but some females, too -- also have sensory integration disorder. Some examples of the disorder are:

• Some children are hypersensitive to light, sounds, odors, touch, or textures of clothing.
• Some children object to being held.
• Some children find it difficult to be in crowded or visually complex surroundings, to be exposed to crowds or surprises.

Due to their difficulty screening out stimuli, situational overload can provoke emotional outbursts or tantrums in kids with both FXS and sensory integration disorder. They may react to the overexposure by flapping hands, biting, hitting, rocking, or having tantrums.

Children with sensory integration disorder often benefit from a combination of occupational therapy, speech therapy and educational therapy. The occupational therapists help with arousal levels, tactile defensiveness (high sensitivity to touch sensations or being easily overwhelmed by ordinary daily experiences and activities), fine and oral motor weaknesses, and can design environments with the most helpful combination and timing of various sensory stimuli planned out for each child's individual needs. Finding the right balance of some but not too much stimulation is an ongoing issue.

These behaviors associated with fragile x are similar to those of autism:

• Hand flapping
• Hand biting
• Poor eye contact
• Tactile defensiveness (high sensitivity to touch sensations, or being easily overwhelmed by, and fearful of, ordinary daily experiences and activities)

However, while some symptoms of fragile X syndrome overlap with those of autism, one strength of males with FXS is their great sociability and friendliness, which is not shared by children with autism.

Many children with FXS have good visual perception and memory. As a result, techniques that teach based on visual cues may be more effective, as opposed to presenting information orally. Some children best process information with whole pictures, or by learning sign language, and some best learn math or language with computer-based visual themes.

Parents of children with special needs have to plan well in advance for the day their child turns 22, at which point the state no longer provides for the child's education. What happens next, of course, will depend on how severe the child's condition is. Options include:

1. Continuing to live at home.

2. Living in a group home, with live-in support.

3. Living in a group home, with periodic support.

4. Living in a shared environment, less structured than a group home, with or without supports.

5. Living in an independent situation, but with periodic care.

6. Living in communities created specifically for people with developmental disabilities (such as a Camphill model, where people live and work in alternative communities, with lots of support).

7. Living in a larger facility with more residents and more assistance.

8. Living in a state institution, for those at risk of harming themselves or others.

Most facilities have waiting lists, and as a result some parents join together to create appropriate living situations for their children.

Here are some things you can do:

• Find or create a living situation near where you live, so that you can easily visit and make sure things are going well.
• Arrange to speak to your child each day so that you can assess the situation.
• If you aren't able to frequently visit to check things out, find special needs care. First interview the caregiver carefully to make sure this is someone you can trust with your child. Arrange for weekly conversations so that you can get a full report on how things are going. Make sure your each have emergency numbers for all involved.
• Help devise a balanced schedule for your child, one that includes activities, socializing, work, and time to relax.
• Expect that this transition will be a time of intense emotions for you. Do something special for yourself.

Care.com is a website that lists caregivers throughout the United States who provide assistance to people with special needs. You can search for caregivers near you and review caregiver profiles including photos, references and background checks, and work history. For specific listings of special needs caregivers, go to Care.com.