Submitted by deb on Mon, 06/27/2011 – 22:24
Life is a daily challenge for the child with periodic paralysis.
Periodic Paralysis poses unique problems for school-age youngsters. Because young PP patients usually look perfectly normal, and may even look extremely muscular and fit, it’s hard for school staff to understand what a challenge daily life can be for them. But these children’s strength may vary widely from day to day, even hour to hour, which is not just frustrating for teachers to cope with but extremely difficult for the child as well.
“Inflexibility of the school staff is one of our biggest challenges.”
Because the child’s strength may vary so much it is very difficult to set out guidelines which will work for every child, or even which will work for one child every day. Parents of these children stress that inflexibility of school staff is one of the biggest challenges they face, because at times the school staff may read a description in a journal or text, or a physician’s report, and are determined to make the child fit the pattern described.
It’s best for everyone to remember that the child is the one who knows best how they feel. There are few entirely ‘typical cases’ of periodic paralysis. There are more than 30 currently identified mutations, all with subtle differences in symptoms and triggers, and it is unlikely that the child you are dealing with fits exactly and neatly into a textbook article which may describe one mutation.
Allow the child to adapt their activities
It is extremely important for school staff to be flexible and allow the child to adapt his or her behavior as needed to avoid episodes. It is actually possible that what triggers episodes in a child one day may relieve them the next; i.e. sometimes a child needs to move, and sometimes he needs to be still. Sometimes she needs to eat now and sometimes she needs to put off eating until her medication takes effect. One time a child may complete a task with ease and the next he/she may not be able to do it at all. Poor strength may lead to clumsiness, lack of coordination, and a general inability to keep up with one’s peers when NO CHILD wants to be perceived of as different.
In some children potassium shifts bring hyperexcitability or emotional lability. One man in our group with HyperKPP/PMC (whose native language is French) was called by the nickname, “Jean qui rire, Jean qui pleure” (John who laughs, John who cries) as a child.
There is no doubt that living with PP is hard on child, parent and school. But there are many ways to support the school-age child with PP so that their daily life at school is easier. Several of the important issues to consider are: Diet, activity, hydration, warmth and adequate rest.
We stress that the school must work with the parent in regards to the diet of the individual child. These guidelines are meant to give school personnel some understanding of the general needs of persons with periodic paralysis.
Snacks are essential:
Depending on the type of periodic paralysis, high potassium foods should be emphasized or avoided. But some dietary principles apply, regardless of which form of PP the child has. The diet should, as far a possible, strive to keep the blood glucose levels stable. The child should never be allowed to become over hungry, or to eat too much at one time. This is best done by supplying small snacks to be eaten between meals.
For HyperKPP – High Carb Snacks:
For HyperKPP youngsters snacks should tend to be high in carbohydrates, though not strictly sugar treats. Sometimes a high carbohydrate treat is interpreted to mean that the child should be given a steady diet of candy and soda pop. Though this may actually make a child with HyperKPP feel good, and may be useful for aborting episodes at times (see “Food as Therapy” below) it can have repercussions in other areas of health. An example of a good HyperKPP snack might be three crackers with 1 tsp of peanut butter spread on each, topped by a dollop of jelly and second cracker. This has protein, carbs and a bit of fat.
For HypoKPP – High Potassium, High Protein Snacks:
For HypoKPP youngsters the snack might be cheese and a handful of strawberries, or unsalted nuts and half an apple. In other words, high in protein, with some fat and with an excellent potassium source. Salty foods must generally be avoided by HypoKPP patients, though a few HypoKPP patients need salty foods.
Children with PMC walk a tightrope:
If the child has Paramyotonia Congenita a high carbohydrate snack (like candy or pop) may drop the potassium level low enough to trigger paramyotonia (which is sensitive to drops in potassium). For the child with PMC the snack should be low/moderate in potassium but high in protein. However, these children often go through swings when the potassium shifts up or down and they need a higher or lower potassium snack, depending on the phase they are in. They may carry both kinds of snack and eat whichever they feel is appropriate. As a general rule the child should eat something every 90 minutes without fail during waking hours. This means portion size at mealtime must be reduced accordingly or weight gain will become a problem.
Food as Therapy:
In HyperKPP episodes may follow a daily pattern, occurring at the same time each day. A high carb snack taken just before the attack is anticipated can often avert or lessen the attack. The HyperKPP child should at all times carry a can of sugar-sweetened soda. If they feel themselves beginning an attack they should drink the soda, even if in class. Hyper attacks develop very rapidly and must be treated without delay. Getting up and going to the Nurse’s office may simply take too long. Patients with HyperKPP can degenerate from perfectly normal strength to ‘too weak to speak’ in 30-60 seconds. HypoKPP doesn’t respond as quickly to food, but it is vital not to allow these children to go hungry. Snacks carried to school must be appealing to the child, so they will be eaten, not discarded or swapped for chips and candy bars. If this means adding a few M & Ms to the mixed nuts, then compromise slightly. Chocolate is high in potassium.
It is important that the child stay more than adequately hydrated. Every time the child has a snack they should also be drinking at least eight ounces of fluid. Not fruit juice, which i too high in potassium for most of those with HyperKPP and too high in sugar for most of those with HypoKPP. Plain water is the best drink. Some people with HypoKPP find that caffeine makes them stronger, so if your child is adamant that cola drinks make them feel better it’s probably better not to go to war over it. However HypoKPP youngsters should be drinking diet soda, not the sugar-sweetened type.
Inactivity brings on attacks, so sitting still in class is one of the most complained of triggers for youngsters with periodic paralysis. This is the factor which forces many youngsters into home-schooling. It will be helpful if the child can sit in the back of the class, so they are able to get up quietly and walk as needed. The teacher can help by making use of any opportunities to send the affected student on quick errands, or by finding ways to get them out of their seat and moving a little.
Adults report that chewing gum is enough activity for some to stave off attacks. Attacks happen when (in HyperKPP) sodium ions ‘stack up’ in the T-tubules of the sarcolemic membrane, and (in Hypo) when the Na+ -K+ pump keeps pumping potassium into the muscle cell. In both cases the end result is ion imbalance and temporary paralysis. Steady, gentle activity keeps this ion imbalance from happening. So, even though gum chewing in class is probably forbidden, it might be just enough activity to keep young PP patients from suffering attacks during class time.
At any rate teachers should discuss with the child and parents the child’s tolerance level for inactivity. Activity breaks must be built into the schedule, so the child never sits longer than their tolerance level allows. Let the child know there are stations to go to in the classroom when he or she is feeling weak. For example: a mat at a certain place in the room, a bean bag chair, a rocker, your own desk. If the child isn’t able to verbalize the need to shift positions, the teacher can prompt the child to change positions.
In schools where classrooms are on two or more levels these children may have difficulty climbing stairs on days when strength is low. There is a risk of falling or being knocked off balance. If the child changes classrooms they may need to leave class a few minutes early to have time to negotiate stairs, and they may need a friend to carry books.
By law the School must accommodate children with disabilities. Period. Whether the Gym teacher thinks your strong-looking son or daughter has a problem or not, the child with PP must be accommodated. Most children with PP can be moderately active, some can be very active, but there are some who have very little tolerance for exercise, and these children may experience paralytic episodes if they are pushed beyond their physical capacity. This does not necessarily mean the child will become paralyzed in gym class, but when time comes to get out of bed the next morning the child may be unable to move and miss one or more days of school.
Children with PP should not be expected to ‘keep up’ with unaffected children. Doing laps, sit-ups or pushups or other intense bursts of physical activity followed by rest can trigger rapid-onset paralysis in some children. In PMC muscle rigidity grows worse with exercise, not better. In other words these children do not ‘loosen up’ with warm-up, they tighten up. Exercise for a child with PMC must be extremely gentle and they must be allowed to set their own pace and stop when they feel they have had enough. Although it’s not wise to overdo, physical activity and active play should be encouraged, as episodes seem to occur less frequently when patients are physically fit. Consistency in the level of daily activity appears to be a key factor. Activity should be as consistent from day to day as is practical.
Kids tend to pack things back and forth from school like mules. Localized weakness (an arm or hands) can result from carrying weight with one hand or arm, or carrying unevenly distributed weight. Some families have solved this problem by having two sets of books, one which is kept at school and one which is kept at home.
These children should not be playing outdoors in very cold weather and should be warmly dressed at all times. Even in warm weather they may not be comfortable in shorts and tee shirts. They should be seated in a warm place in the classroom, and near a heater on the bus if they are cold-sensitive. Long waits at the bus stop during cold weather should be avoided. Breathing cold air may induce breathing difficulties (which are often misdiagnosed as asthma) due to rigidity of the chest musculature. On the other hand, some children with periodic paralysis are as heat-sensitive as others are cold-sensitive, so in hot climates they may require protection from overheating. Some more affected children may require a rest period mid-day where the muscles can be relaxed and rested. This may mean a nap in the nurse’s office, or quietly wrapping in a blanket or throw and curling up in a large supportive chair or sofa in the classroom.
If neck myotonia/pain or weakness is a problem, there is a great device that attaches to the school desk, called a slant board. It can lie flat, or raise up at an angle, so that the kids don’t have to look down while writing or reading material. A timer is a great device for monitoring work, especially when organizational skills are waning. It is large enough to go on the teachers desk, or put on the wall, or can be put on the child’s desk. It can time things from 1 – 60 minutes. It can also be used for timing sitting intervals (time for a stretch, etc.) It can be used as a visual clue, that there is only 10 minutes for this assignment, so stay focused. It makes no noise but has a bright red section that gets smaller as the time goes by. It can be used by the whole class too! Additional helping aides may be a large note card taped on the desk of things to remember (i.e. hand in your homework, empty mailbox, or other daily routines). Additional modifications may also be needed depending on the day, time of day, etc. For example, some children may need note takers or a scribe on certain days when their hands are not up to par. This may be done by a paraprofessional (i.e. aide) or by another student if possible (in older grades).
Additional therapies to be done in the school setting which can be very helpful. Consult with Occupational Therapy…for ordering specialized equipment, ensuring proper posture, desk and seat size, organizational tools/input, and or help with finding necessary modifications for fine motor areas. It is vital that the child be in a desk which does not stress weak muscle areas.
(For some children this is invaluable. ) The paraffin wax treatment for hands and the back of the neck has proven helpful for relieving myotonia, and is very soothing. The PT can also set up a daily stretching program. For a child with PP, stretching helps avoid stiffness. It is important that parents meet with the PT and continue treatment, especially the stretching regime, daily, at home. One parent in our group reports that gentle massage with essential oils, (a mixture of lavender and sage) meant to help with discomfort (pain or muscle cramping) is helping ease the discomfort of her seven-year-old’s myotonia. Her son cannot tolerate deep tendon massage. The PT also has been doing cranial facial release therapy with this child, which has also been beneficial. Passive Stretching has been of benefit to some with PMC. We hope this has given you some ideas about managing your child’s daily activities. Thanks to the parents of the children in our group for their help and advice with this article.