Symptom Management
Head, Eyes, Ears, Nose and Throat
Head
Eyes
Ears
Nose
Throat
Dental
For several reasons, many children with progressive neurological disorders have enlarged heads. An increased head circumference, which is generally noticed during a routine physical exam, may be one of the first indicators that something is wrong.
The first year and a half of life is the period of greatest head growth, because the bones of the head are not yet fused. Head growth begins to slow down as the sutures (lines between the bones) in the head begin to fuse and the fontanelles (soft spots) close.
As the size of the cranium grows, the head becomes increasingly heavy. Holding the child for extended periods becomes tiring because of the weight, especially if your child tries to “extend” (arch) backwards. You may find yourself shifting your holding positions and accommodating for the head size when you position your child for sitting or laying down.
Some positioning problems exist because of the weight of the head and the child's inability to move. When your child lays on his or her side, the ear must be checked periodically for pressure areas and soreness. Using a sheepskin under your child's head, with a soft cloth over it to absorb moisture is helpful (see Dermatology section).
When buying clothing for your child with an enlarged head, you should look for shirts, sweaters etc. with large or stretchy neck openings to comfortably accomodate the head. It may be helpful to remind well-meaning friends and relatives about this special need if they are buying
gifts for your child.
| “There were so many things that had to be done for my child: medications, tube feedings, exercising and othernecessities of daily care. Then there were all the ways in which I could‘stimulate’ him that the therapist suggested. There was so much that I had to do, when all I wanted to do was to sit and hold him and love him.” |
One of the earliest signs of a potential neurological problem is the presence of nystagmus which is a rapid, exaggerated, involuntary eye movement. Although this may be present to a lesser degree with a healthy newborn, exaggerated movement is an indicator of a neurological abnormality.
The presence of a “cherry red spot” on the retina (back of the eye) is a diagnostic sign in Tay-Sachs and some of the other progressive neurological diseases. This can be seen by a trained professional using special equipment for examination of the eyes.
Parents may begin to have concerns about their child's vision, especially if the child is not tracking (following with the eyes) by a certain age, or does not seem to be focusing on a familiar face or object.
Many children with progressive neurological disorders may be visually impaired. They may have partial vision or may progress to a complete loss of sight. A test called “Visually Evoked Potential” can help to diagnose the type of visual impairment your child has. In this test, brain waves are measured while a strobe-type light is flickered in front of the eyes.
Since it is difficult to measure precisely how much vision a child has, it is important to provide lots of visual stimulation for your child
(see Stimulation, Play, Communication section). Many local voluntary and governmental agencies provide wonderful services and support for visually impaired children. You may want to investigate the services in your area.
The most common eye infection in all children is conjunctivitis (“Pink Eye”), which produces yellow mucus and a red eye. An eyelash can also cause redness, so you may consider looking for a foreign body irritating the eye before you assume it is conjunctivitis. Conjunctivitis may be an early signal of an ear infection, so it is important to have any persistent redness of the eye evaluated. Be aware that the discharge may be contagious, so precautions need to be taken not to infect others. Antibiotic drops or ointment are generally used to treat conjunctivitis.
Ear infections are very common in all children. While these infections are not necessarily more frequent in our affected children, they do occur and require attention. Middle ear infections (otitis media) have the potential to cause hearing loss. It is especially important to maintain optimal hearing function in our affected children since so many of them are or become visually impaired.
The more obvious signs of an ear infection are fever and discomfort, but with our children it is helpful to learn some of the early signs. As mentioned above, an ear infection may first present as a conjunctivitis.
Other early signs may be: the persistance of a cold with discolored nasal mucus for more than one week; unusual fussiness; loose stools; and vomiting. Middle ear infections are treated with oral antibiotics.
External infections of the ear canal (otitis externa) are less serious, but are perhaps more frequent with our children because of chronic drooling which can enter the ear. It is important to change moisture laden cloths under the face and ear frequently. These infections can be painful; they may or may not cause fevers. External ear infections are treated with ear drops.
Because these children cannot blow their noses, they often have thick dried secretions in the nose. Carefully using a nasal or ear syringe with some saline solution will help to remove the discharge. If your child has persisting nasal drainage which is discolored, he or she should be evaluated for an ear infection, as mentioned above. This may also indicate a sinus infection, which is treated with an oral antibiotic.
Tonsillitis should be considered if your child is uncomfortable, has a foul odor on his or her breath, or more pronounced drooling and fever. When the tonsils are greatly enlarged, they have the potential for blocking the airway in children who have little or no head control. One family used a neck brace to support their child's head during episodes of tonsillitis. Suctioning may be indicated to help to handle secretions. Tonsillectomy is sometimes indicated in cases of recurring tonsillitis. Tonsillitis may be caused by the streptococcal bacteria (“strep” throat) which requires antibiotic treatment.
Regular dental visits and good oral hygiene are very important. Try to find a dentist in your area who has experience with special needs children. The Americal Dental Association may be able to provide you with a list of dentists with this specialty.
Teeth and gum problems are common in children who do not chew or use their oral muscles in a normal way. Oral care should include brushing the teeth at least twice a day with water or mouth wash. If you decide to use toothpaste, use it sparingly. Try to avoid foods with sugar if your child is eating by mouth. Dental flossing should also be attempted on a regular basis, if possible.
If your child is not being fed by mouth, you should regularly moisten the mouth with a toothbrush or swab. Children often enjoy chewing on the toothbrush; if they are not orally fed, this may provide the only opportunity to chew. A child can chew on a cold wet washcloth as well, and the cloth can be rubbed on the gums, tongue and teeth which can be especially comforting during teething. Stimulating the mouth on a regular basis helps to maintain the swallowing reflex, which enables the child to handle his or her own secretions for as long as possible.
One common problem among these children is the presence of a high arched palate (roof of the mouth). Secretions and food can get caught in this area and are not easily removed with the tongue. Care should be taken to see that the area is clean and moist, as dryness can cause fissuring (cracking) of the palate, which is painful and can be difficult to heal.
Lemon and glycerine swabs or Toothettes are useful in cleaning the palate. If there are dried crusty secretions, you can try a solution of half hydrogen peroxide and half water on a swab to clean the mouth and palate.
Enlargement of the gums (gingival hypertrophy) may occur if the child is on long term Dilantin therapy (an anticonvulsant). Gentle brushing of the gums is important to keep them healthy.