Crystals deposit in various organs of the body. In the
kidney, they deposit in tubular cells. One of those functions is to control
water excretion (how much one urinates).
The kidney works by filtering
about 50 gallons of water per day (in the adult) and then reabsorbing 49
gallons of water back, as it passes the tubular cells. One can, therefore,
imagine that if the tubular cells are damaged even just a little bit, one can
lose a lot of extra water. Children with cystinosis, therefore, cannot conserve
water. When we drink a lot we "pee" a lot, and when we don't drink so much, we
don't "pee" so much. Cystinotics "pee" a lot no matter how much they drink,
although they will urinate somewhat more, given more fluids. Cystinotics,
therefore, need almost unlimited access to fluids and should rarely, if ever be
restricted from water ( and then only under a doctor's strict supervision). One
cannot punish or reward these children using fluids: it is too dangerous.
Parents often relate to me how young children will drink out of toilet bowls or
lick water off windows, especially in the winter. This is a sign they need
water they can get to and easily, especially at night.
Cystinotics will
usually drink so much that they won't eat, and this is a real problem in
getting adequate calories for growth. I try and substitute high calorie drinks
for as much water as possible, i.e.. milk, milkshakes, root beer floats, etc.
Milk is a super source of good amounts of calories, proteins and fat.
Another problem is that drinking so much really puts the bladder to
work. Usually cystinotics will not be toilet trained for urine, especially at
night, until late, sometimes not until ages four to seven. School peer pressure
usually helps, but can be a problem if the child is made fun of when he has
poor control of urine during the day. Remember they don't have good control and
that should be understood. Parents need to walk a fine line between
understanding and not being manipulated.
If anyone has trouble dealing
with this (most parents do) or any related problems, it is necessary to discuss
it openly with your physician.
Your child is an individual and
individual medical problems should be discussed with your physician.
Sheldon Orloff, MD Sub-Chief of Renal Medicine/Kaiser
Hospitals