Therapy is probably one of the most important things you can offer for your child. Not only physical
and occupational but there are many other varieties and ranges of therapy available.
Lets start with physical therapy::
Most likely started long before your actual diagnosis of Sandhoff, this leads to one of the best activities for your child. Looking back on it all now, I feel so thankful for each and every therapist that worked with our family. Some feel as if it may not be important because a Sandhoff child can not be rehabilitated, however it is so beneficial. Therapist sit down and work with each and every muscle in the child's body. One of the most common problems with children who have Sandhoff is Spasticity - PT care is vital to help fight this, here is a description.
Spasticity
Spasticity refers to feelings of stiffness and a wide range of involuntary muscle spasms-sustained muscle contractions or sudden movements. It is one of the more common symptoms of neurological disorders. Spasticity may be as mild as the feeling of tightness of muscles or may be so severe as to produce painful uncontrollable spasms of extremities, usually of the legs. Spasticity may also produce feelings of pain or tightness in and around joints, and can cause low back pain.
There are two types of severe spasticity:
In flexor spasticity, mostly involving the hamstrings (muscles on the back of the upper leg), the hips and knees are bent and difficult to straighten.In extensor spasticity, involving the quadriceps (muscles on the front of the upper leg), the hips and knees remain straight with the legs very close together or crossed over at the ankles. Spasticity may also occur in the arms, but is less common.
Spasticity may be aggravated by extremes of temperature, humidity, or infections, and can even be triggered by tight clothing.
Treatment With Exercise and Medication
There are a number of therapeutic approaches to the management of
spasticity. Because spasticity varies so much from person to person, it must be treated on an individual basis and demands a true partnership between the person with physician, nurse, physical therapist and occupational therapist. Treatment begins with the physician recommending ways to relieve the symptoms, including medication, exercise, changes in daily activities, or combinations of these methods. The physician will track the progress and make referrals to other health professionals such as occupational and
physical therapists. Daily stretching and other exercises are often effective in relieving spasticity. If drugs are also needed, there are two major antispasticity drugs that have good safety records. Neither, however, can cure spasticity or improve muscle coordination or strength. Baclofen, the most commonly used drug, is a muscle relaxant that works on nerves in the spinal cord. Common side effects are drowsiness and a feeling of muscle weakness. It can be administered orally or by an implanted pump (intrathecal baclofen). Intrathecal baclofen is used for severe spasticity that cannot be managed with oral medication. Tizanidine (Zanaflex®) works quickly to calm spasms and relax tightened muscles. Although it doesn't produce muscle weakness, it often causes sedation and a dry mouth. In some patients, it may lower blood pressure.
Other, less commonly-used drugs, include: Diazepam (Valium®) is not a "first choice" drug for spasticity because it is sedating and has a potential to create dependence. However, its effects last longer with each dose than baclofen, and physicians may prescribe small doses of Valium® at bedtime to relieve spasms that interfere with sleep. Dantrolene (Dantrium®) is generally used only if other drugs have not been effective. It can produce serious side effects including liver damage and blood abnormalities. Phenol, a nerve block agent
Botulinum toxin (Botox®) injections have been shown to be effective in relieve spasticity in individual muscles for up to three months.
Other issues that the PT's work closely to help with is maintaining the child's muscle control for as long as possible. There are wide varieties of exercises for this. PT's also work with using balls, laying the child on the ball face down and urging them to lift their heads off the ball with help to maintain head control for as long as possible.
You may also receive AFO braces for your child's feet. Most children are prone to keep their feet more pointed downward, thus causing clonus ::
Clonus is a reflex that is a spasmodic alternation of muscular contraction and relaxation., usually seen in the calf muscle reaction when the foot is sharply bent upwards towards the thigh and held in mid position. Can also occur when the child's legs are rested and he or she is sitting, ie: even sitting in a stroller, or in a rocker. It will start while the child is sitting and pointing the feet downward, using the AFO braces will cut down on the child being able to point those feet and cut down the attacks of clonus.
Another area of muscle problems the PT will work against is hypertonia:: Spastic hypertonia involves uncontrollable muscle spasms, stiffening or straightening out of muscles, shock-like contractions of all or part of a group of muscles, and abnormal muscle tone. It is seen in disorders such as cerebral palsy, stroke, and spinal cord injury, and other neurological disorders. Dystonic hypertonia refers to muscle resistance to passive stretching (in which a therapist gently stretches the inactive contracted muscle to a comfortable length at very low speeds of movement) and a tendency of a limb to return to a fixed involuntary (and sometimes abnormal) posture following movement. Now onto Hypotonia:: Hypotonia, or severely reduced muscle tone (the amount of tension or resistance to movement in a muscle), is seen primarily in children. It is not the same as muscle weakness but it can co-exist with muscle weakness.
Hypotonia may be caused by trauma, environmental factors, or by genetic, muscle, or central nervous system disorders. While most children tend to flex their elbows and knees when resting, hypotonic children hang their arms and legs by their sides. They also may have substantial weakness and little or no head control, giving them a "floppy" appearance. Typical symptoms also include problems with mobility and posture, breathing and speech difficulties, lethargy, ligament and joint laxity, and poor reflexes. When hypotonia is caused by an underlying condition, that condition is treated first, followed by symptomatic and supportive therapy for the hypotonia.
Physical therapy can improve fine motor control and overall body strength. Occupational and speech-language therapy can help breathing, speech, and swallowing difficulties. Therapy for infants and young children may also include sensory stimulation programs.
One last area that the PT may include in her program is swing therapy. The therapist normally sits with the child on a large platform swing, this is so wonderful for the child in so many different ways.