skin and hands
In this section we are trying to give our family and friends an idea of what daily life is like with DJ since his second birthday. This page was not meant to be a manual for home care for a child with a neurodegenerative disease. Rather, it is a list of our ideas and experiences with DJ. Each child's illness and each family's circumstances are different.
Constipation is a major problem. At first, DJ's doctor prescribed a high-concentration sugar called lactulose, but it was not very effective. Laxatives such as Senokot (senna concentrate) tend to cause excessive gas and often make him uncomfortable.
We decided to control the constipation through diet rather than medication. Extra liquid really helps the constipation. DJ drinks nectar-like juice, and his favorites include carrot-apple and sweet-potato juice. He also gets milk, yogurt drinks, and Pediasure. DJ needs to have plenty of fruits and vegetables in his diet, including strained prunes, pears or plums every day. Other remedies for constipation include: a small bowl of freshly grated apple, adding a tablespoon of ground bran cereal or adding a 1/2 teaspoon of canola oil to his food.
When DJ does have a problem with constipation, we give him the PLUMBER'S SPECIAL. The plumber's special consists of a mixture of stewed prunes, prune juice, and a little ground bran cereal. It works like a charm.
The constipation is exacerbated by DJ's inactivity. We use passive exercise to compensate. We move his legs and arms a lot, and try to change his position frequently. We massage his lower tummy lightly every day, following the movement of his intestine.
If DJ has not had a bowel movement in two days, we occasionally have to use more extreme measures such as manual extraction with a glove and vaseline or glycerine suppositories.
DJ does not like to be prone all the time. To sit up, we prop him up on pillows and a chair rest. We also place him sitting up in his high chair, stroller, or KidKart wheelchair. We use the stroller indoors as well as outside. The stroller can recline flat so he can sleep comfortably when we are at a friend's house. During the long Chicago winter, we often put him in the stroller facing the glass doors to the garden so he can get a change of scenery, enjoy the view and bask a little in the sunshine.
To keep DJ straight in his stroller, we wedge his body with small stuffed animals like Beanie Babies or Pillow Buddies. Other kids like the stuffed animals which they think are "cute" and adults tend to stare less openly.
As he gets older, DJ's muscle tone has decreased significantly. When lying on his back, DJ's legs fall into a "frog" position. We have to be careful to position his knees and hips so the joints do not "slip" out of the socket. We try to do range of motion exercises and move his knees a couple of times each day. We are also careful to position his hips squarely into the seat of his wheelchair or special needs car seat.
Our main priority when DJ sleeps is to minimize the violent rocking produced by the startle reaction and seizures. When DJ sleeps, we use rolled-up towels and pillows on each side to wedge his arms close to his body. If he sleeps on his side, we place a rolled-up towel or a stuffed animal behind his back. We place a folded towel or small pillow between his knees to make him more comfortable.
DJ is too weak to roll over by himself anymore. We moved him from his crib and we let him sleep between us when his seizures started. We get up 2 to 3 times every night to roll him over or make him more comfortable. We also frequently change the burp cloth under his face to absorb the extra moisture from his mouth, and minimize facial rashes. The suction machine is also close by.
skin and hands
Children with Tay-Sachs have the softest, smoothest skin we have ever seen. However, they are largely immobile. We frequently change DJ's position to prevent pressure sores from developing. We also have him lie on an egg-crate matteress when he sleeps.
DJ has delicate, porcelain like skin on his face. DJ has a lot of secretions, so we need to change the burp cloth under his face fairly frequently. The burp cloths absorb the extra moisture from his mouth and help to minimize facial rashes. We also use Tegaderm to keep the NG tube in place on his cheek. DJ also wears a baseball cap when we go out in the sun.
DJ tends to keep his hands clenched. We smooth out the fingers several times during the day. We trim his fingernails frequently, to keep them from digging into his palm and scratching the skin. The hands are usually moist from being clenched, so we wipe them after each meal with a damp cloth or clean them with antibacterial gel.
We try to brush DJ's teeth on a regular basis. We use a washcloth dipped in warm water or a rubber finger cover with ridges (made by First Years) to clean his teeth. It also helps to take some of the mucus off his gums and tongue each day. A couple of times each week, we use a soft toothbrush dipped in Listerine, an antibacterial mouthwash, to get the plaque off his teeth and give him a more thorough cleaning.
We have noticed that some of DJ's teeth are a little uneven. He sometimes grinds his teeth during seizures.
DJ doesn't blink or cry very often. We just check to make sure his eyes do not get irritated and red. DJ has extremely long and thick eyelashes, so he can easily have a stray lash lurking in there. We have used artificial tears to lubricate his eyes when they get too dry.