Sir,
Your step-daughter's disease is considered one of the rarest of rare diseases. There is no set diet. I highly suggest you see a qualified Dietitian so a diet can be individualized for her.
In general she should probably be limiting her sodium intake to 1500-2300 mg/day. You cannot tell her hydration level by the color of her pee because her disease will discolor the urine anyway. The sodium restriction will help with fluid retention and ease the work on the kidneys.
Although this disease affects other parts of the body, it is similar to a form of
glomerulonephritis or kidney disease. Since each person is individual in their needs for phosphorous, potassiuim and protein restriction, a Dietitian should be involved. And, these needs will change if
dialysis enters the picture.
Her protein intake should be of high biological quality, but limited to about .8 grams/kg body weight. High biological quality means "complete proteins" which, again,the types and amounts will depend upon whether the diet is vegetarian or if meat is consumed.
Oral
calcium supplements (1 g/day) and vitamin D (400-800 IU/day) are recommended for prophylaxis against
osteoporosis in glomerularnephritis, but check with your doctor first. Dietary treatment for Dense Deposit Disease frequently follows the guidelines for the glomerularnephritis (kidney) diet.
Fluid recommendations, defer to personal physician. Frequently fluids are limited but whether the doctor wants input/output records is a question. You want to avoid
dehydration as well.
Good Luck. Regards, Kathryn Shattler, MS,RDN