Following are a few sample questions we have selected - you can use the category listing to the left to find more questions and answers relating to your areas of interest. Click on a question to see the answer.

What is Sandhoff's Syndrome?

Joshua Alexander, MD: Sandhoff?s Disease is an inherited, worsening neurodegenerative disorder caused by low levels of hexosaminidase A and B proteins. This leads to the body?s inability to process and rid itself of other proteins called GM2 gangliosides. These proteins build up in the body, especially in neurons (nerve cells) and cause problems similar to those seen in Tay-Sachs disease. Weakness begins in the first 6 months of life. Startle reaction, early blindness, progressive mental and motor deterioration, doll-like face, cherry red spots, and macrocephaly (large head) are all seen. Death usually occurs by age 3 years. For more information about Sandhoff?s Disease, try the website of the National Tay-Sachs and Allied Diseases Association at http://www.ntsad.org/pages/sandhoff.htm

What is the Tardieu Scale?

Joshua Alexander, MD: The Tardieu scale, developed by a French clinician in 1954, is a way to measure spasticity ( A velocity-dependent increase in tone) that compares how a spastic muscle ?catches? at low and high speeds. It measures the intensity of the muscle reaction to being stretched, the angle at which the catch is first felt, and how this changes when the muscle is stretched slowly and quickly. Some clinicians feel that this is a better measure of how velocity contributes to spasticity compared to other measures (like the Ashworth Scale) which don?t take speed of muscle stretch into account. After ranging a joint slowly and then quickly, the spasticity is assigned one of the following scores: 0 No resistance throughout the course of the passive movement. 1 Slight resistance throughout the course of the passive movement with no clear catch at a precise angle. 2 Clear catch at a precise angle, interrupting the passive movement, followed by a release. 3 Fatiguable clonus, less than 10 seconds when maintaining the pressure, appearing at a precise angle. 4 Unfatiguable clonus, more than 10 seconds when maintaining the pressure, at a precise angle.

Can You Give Me More Information About Salivary Gland Ligation Surgery to Treat Drooling?

Joshua Alexander, MD: Drooling is a common associated condition in children and adults with cerebral palsy. In my opinion, partial ligation of salivary glands is a good option for those children whose drooling is not treated sufficiently by oral motor therapy, and medical management (with scopoplamine or robinul). Botulinum toxin injection management is also a useful, but temporary, intervention. For a more permanent solution, the surgeon will partially ligate (close-off) either the parotid or the submandibular glands (the glands that make saliva). When successful, this operation can significantly reduce the amount of saliva (and thus, drooling) the child exhibits without making the mouth too dry. If the mouth becomes too dry, it can impact eating and swallowing and increase their risk of tooth cavities. While I hope that this provides you both with a little more information, the best resource to answer this question is the surgeon or the surgeon's nurse, who can discuss the specifics of the surgery, the recovery (based upon their prior experiences), and the potential side effects.

We have a 4 year old child that has beeen diagnosed with Autism. He has frequent bowel movements, loose at least 2 times a day (from 8:30 a.m. to 2:30 p.m.), they seem to stick to his skin and the smell is very, very strong. Could this be a trait of Autism?

Joshua Alexander, MD: Autism is a spectrum disorder with a wide variation in abilities and deficits. Its major diagnostic criteria include impairments in social interaction and/or communication, and the presence of restrictive, repetitive patterns of behavior, interests and/or activities. There is nothing in the diagnostic criteria that mention stool patterns. While there has been much controversy about the gut-brain connection in autism (Dr. Wakefield and others have proposed a relationship between autism, bowel inflammation, and measles vaccine), there have been no scientific connections proven between autism and stool patterns. Still, many (like the Autism Society of America) feel that ?Individuals with autism may exhibit low tolerance of or allergies to certain foods or chemicals. While not a specific cause of autism, these food intolerances or allergies may contribute to behavioral issues. Many parents and professionals have reported significant changes when specific substances are eliminated from the child's diet.? In addition, the ASA writes that individuals with autism may have trouble digesting proteins such as gluten (found in wheat, oats and rye) and casein (found in dairy products). They believe that incomplete breakdown and the excessive absorption of peptides may cause disruption in biochemical and neuroregulatory processes in the brain, affecting brain functions. You should also check in to see if a child has any unusual food preferences (as is commonly seen in autism) that might be contributing to his diarrhea. While most pediatric gastroenterologists do not feel there are direct relationships between the gut and autism, many are willing to run tests (stool, urine, blood, breath) to see if there are other gut problems causing symptoms like chronic diarrhea. I would suggest that, if a child has a pattern like this for more than 6 months, or if a child is failing to grow at a normal rate, a visit to a pediatric gastroenterologist might be worthwhile.

Can you give us any information about using tegretol for behavorial control/mood changes?

Joshua Alexander, MD: While most commonly used to treat seizures and some types of pain, carbamazepine (Tegretol.) has been used as a mood stabilizer in bipolar disorders and rage outbursts. It has also been used to decrease agitation and self-injurious behavior, and it has been used to lessen aggression in children with attention deficity hyperactivity disorder (ADHD). Other examples of seizure medicines that have been used to control severe mood changes include Valproic Acid (Depakote, Depakene), Gabapentin (Neurontin), and Lamotrigine (Lamictal). In most cases, rigorous scientific studies to demonstrate the efficacy of using tegretol in these conditions have not been performed, and the long-term consequences of medication use in children with behavioral/mood disorders are not well known.

An orthopedist has suggested my 8 year old daughter with hemiplegic cerebral pasly get surgery for heel cord lengthening. Where do I get information on the different varieties of this surgery and their respective success rates?

Joshua Alexander, MD: The advantages of a surgical lengthening of a heel cord include: * Better range of motion * Better ankle dorsiflexion (bringing toes up to your nose) * Smoother walking The disadvantages of a surgical lengthening include: 1) Surgical risks 2) Over-correction leading to crouching on the side that's had surgery. In cases where there is only an Equinus ("toe-down") deformity, the usual procedures focus on lengthening the tendons to the calf muscles (the gastrocnemius and soleus). These can be done either under general anesthesia or as an outpatient surgery using a method called percutaneous (through the skin) surgery. In most cases, the child who has a heel cord lengthening will wear a short leg cast for 2-6 weeks afterwards to allow the tendon to heal in place. Most heel cord lengthenings work well, but research has shown that about 1 out of 5 children who get them need to have them done at least one more time. This recurrence rate is higher for children who have surgery before they're five years old and lower for children who are 8 years old or older. Since every surgeon has different approaches and results, it's best to ask your surgeon what they would predict for your child's outcome.

My daughter has problems aspirating her secretions and it's hard to brush her teeth without her strangling. Do you know of any toothbrushes or equipment that could help?

Joshua Alexander, MD: Ask your dentist about using a Plak Vak. It's a toothbrush that attaches to a portable suction machine. At http://www.trademarkmedical.com/personal_use/plakvac.html you can find more information about this product and order a demonstration video.

My child uses cisapride for his reflux, but my pharmacist told me that it's going to be taken off the market. Is this true?

Yes, cisapride (Propulsid(R)) is going to be taken off the US market in July, 2000. For more information, go to TelAbility's Medications of the Month archive (located in the What's New section).

How Can I Toilet Train a 3 year old Girl with Cerebral Palsy?

Joshua Alexander, MD: As you know, potty training a child requires the child to be cognitively and physically ready. You can tell that a child is cognitively ready when she is able to tell you when she has to go to the bathroom, doesn't like having a wet or soiled diaper, and can follow simple instructions related to potty training. A child is physically ready when you can tell when they have to go to the bathroom by what they say or how their face and body look. Another important factor is that they have regular, soft stools that are not painful to pass. Children with cerebral palsy can have problems with communication,constipation, neurogenic bladder, muscle coordination/control, balance, and functional deficits in transferring and mobility - all these factors can contribute to difficulty with toilet training and need to be taken into account when devising a program. A special potty chair and other adaptations (like a stool so that feet don't dangle) should also be considered and a timed (shcheduled) voiding trial can be used for children who have not yet developed good bowel and bladder control. I'd suggest that, if she is cognitively ready and willing, you first make sure she doesn't have any constipation problems. If not, I'd suggest contacting an occupational therapist who has expertise in adaptive equipment to help evaluate the child's potty chair and positioning needs. Once this is all in place, either regular potty training or timed voiding training can commence under the supervision of her local doctor, therapist and, if needed, a pediatric psychologist. For more information, the American Academy of Pediatrics publishes a book about toilet training http://www.amazon.com/exec/obidos/ASIN/0553381083/ref=sib_rdr_dp/103- 0182930-5288618 that includes a chapter on training children with special needs. Above all, readiness, preparation, patience and celebrating success all help to make toilet training a succesful venture for all involved.

My son's school wants to do a program on spina bifida. Where can they find more information about it and about prevention?

Joshua Alexander, MD: It's wonderful to hear about the interest in spina bifida from your son's school! For more information and informational packets about this condition, I'd suggest you contact the Spina Bifida Association of America at http://www.sbaa.org/ the Spina Bifida Association of North Carolina at http://sbanc.home.mindspring.com/ and/or the UNC Center for Maternal and Infant Health at http://www.mombaby.org/ and, for informational packets about prevention, contact the North Carolina March of Dimes at http://marchofdimes.citysearch.com/

My daughter is 8 years old and has spastic diplegic cerebral palsy.What do you think about Smooth Move tea to help with her constipation?

Joshua Alexander, MD: As per some website information provided by the company that makes this tea, he only "active" ingredients are the Sennosides A&B and the Senna leaf. This is the same active ingredient found in Senokot (we reviewed this as a recent TelAbility Medicine of the Month - go to http://www.TelAbility.org and search for SENNA). However, the tea also contains other "natural" laxatives like licorice and fennel seed that may increase its effectiveness and soothe the stomach from some of the side effects of the sennosides. Keep in mind, though, that while the makers of Senekot state that their pills are standardized (every pill contains the same amount), it would be hard to do the same with a blended tea bag. As always, I would recommend that you discuss use of this tea with your daughter's doctor. Even though it doesn't need a prescription, it can still cause effects in the body that need to be considered.

I "follow" a CP chat group on facebook and one woman asked about phenol injections. no one had really heard of them. What is this? Who it is suitable for?

Joshua Alexander, MD: Phenol injection is another option for spasticity management in a specific muscle (unlike baclofen or other medicines taken by mouth that decrease spasticity throughout the body). Here's an edited part of a description from www.WeMove.org (with my comments in parentheses- JJA) "Phenol and alcohol are injected onto nerves or into muscles to destroy them. Phenol and alcohol can effectively weaken a spastic muscle, which reduces spasticity and allows improvements in range of motion. These agents are effective in treating spasticity that occurs in large, powerful muscle groups close to the trunk, such as those of the thigh. Alcohol or phenol may be used in combination with BoNT (botulinum toxin- JJA), and all may be combined with other antispasticity treatments. The duration of effect is quite variable, from less than 1 month to more than 2 years (I usually hear it lasts from 4-8 months- JJA). The cost of either phenol or alcohol is much less than that of BoNT. When cost is a major concern, phenol or alcohol may be used to treat muscles in which BoNT could also be effective. (but this does not include the cost of sedation/anesthesia which always has to accompany phenol injections to allow the child to tolerate the electrical stimulation/shocks that are used to make sure the phenol is injected in the right place - JJA) Unlike BoNT, phenol and alcohol do not provoke a reaction by the immune system. There are several significant disadvantages to the use of phenol and alcohol. Phenol and alcohol may produce more troublesome initial side effects than BoNT, such as burning and odd sensations called dysesthesias. Damage to nearby sensory nerves may cause temporary or permanent pain, which may require medication to control."

My family wants to go to the beach, but we can't move our son's wheelchair on the sand and he's too heavy to carry. What can we do?

Joshua Alexander, MD: In North Carolina, we're lucky to have a program that can help your whole family enjoy the beach. Beach-ability, run by Easter Seals of NC http://www.esnc.org provides beach accessible mobility aids at most of NC's coastal areas. Natural Access, a company in Santa Monica California , markets an all-terrain wheelchair called the Landeez that can roll over sand, gravel, snow, and soft soil. More information about this product can be found at http://www.natural-access.com/