Cystinosis (Fanconi’s Syndrome) Level 3

Cystinosis (Fanconi’s Syndrome)
What is it?
*Heredity disorder -of amino acid metabolism, fructose intolerance,
-may be caused by use of heavy metals, chemical agents,
Vitamin D deficiency, kidney transplantation, multiple myeloma
*Taking- outdated tetracycline (antibiotic)
*Cystinosis- occurs when cystine crystals are deposited throughout
the body
*Infantile form leads to renal disease
*Neurological symptoms- toxic accumulations of copper in kidney,
brain
*Can be caused by Lead Poisoning; cannot be Cured
*Rare disorder of tubule function- excessive amounts of Glucose,
Phosphates,Bicarbonate, Sodium, Potassium
Signs and Symptoms:
*Child- secretes large amounts of urine; weakness, bone pain
What can I do?
*See Physician- discuss prevention of Bone Demineralization,
correct Hypokalemia, adapt to Swallowing Dysfunction,
Supplementation of Vitamin D, calcium, Sodium, Potassium
 replacements; Correct Fluid and Electrolyte Imbalances,
Consistency changes in Liquids, Solids as needed
*See FFF RD on line- or meet with RD for information on Na, K