eyes
DJ doesn't blink often and rarely cries. We have to check to make sure his eyes do not get irritated and red. DJ also 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.
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oral care
We try to brush DJ's teeth on a regular basis. It also helps to take some of the mucous off his gums and tongue each day.
We use a washcloth dipped in warm water or a rubber finger cover with ridges (made by First Years) to clean his teeth.
A couple of times each week, we use an extra soft toothbrush dipped in Listerine (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's lips are dry. We use a lip balm daily. He likes flavored lip balm - especially mandarin orange by The Body Shop.
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skin and hands
Children with Tay-Sachs have the softest, smoothest skin that we have ever seen.
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 tape to keep the NG tube in place on his cheek. DJ wears a baseball cap to 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 have noticed some patches where he has a tendency to develop eczema - behind the ears, his shoulders, his ankles, and on his back where the diaper ends. We usually use moisturizing lotion for these rough patches. If it gets worse, we sometimes use 1% hydrocortisone cream for a couple of days until it clears up.
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| body temperature
Children with Tay-Sachs are largely immobile. DJ's hands and feet are always cold, so we rub them frequently to restore circulation. He wears thick socks most of the time. In contrast, DJ's head is almost always hot. Sometimes his head becomes very warm when his seizure threshhold is low.
We have noticed some variation in DJ's body temperature. It is not unusual for affected children to be 96 degrees F (36 degrees C), or even lower in some cases.
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"the scoop on poop": constipation and elimination
Constipation is a major problem. 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.
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 drank nectar-like juices, and his favorites were carrot-apple and sweet-potato juices. He also drank milk, yogurt, and Pediasure. DJ needed fruits and vegetables every day such as strained prunes, pears or plums. Adding karo syrup or adding a 1/2 teaspoon of canola oil to his food also worked well.
When DJ did have a problem with constipation, we gave him the PLUMBER'S SPECIAL. The plumber's special consisted of a mixture of stewed prunes, prune juice, and a little ground bran cereal. It worked like a charm.
As DJ became older, we found that adding fiber to DJ's diet helped to move the stool down to the lower bowel. While DJ ate by mouth, we added fiber to his diet through a tablespoon of ground bran cereal. Now, we give Pediasure with Fiber through the NG tube.
If DJ has not had a bowel movement in two days, we occasionally have to use more extreme measures such as glycerine suppositories, or manual extraction with a rubber glove and vaseline.
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sleeping
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.
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.
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| egg crate mattress
Children with Tay-Sachs are largely immobile. We frequently change DJ's position to prevent pressure sores from developing. He lies on an egg-crate mattress. The egg-crate mattress is made of foam and has ridges and bumps along one side.
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positioning
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. We are also careful to position his hips squarely into the seat of his wheelchair or special needs car seat.
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 partially out of the socket. We try to do range of motion exercises and move his knees a couple of times each day.
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