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The Snow Foundation would like to inform its readers of the importance of monitoring sodium levels in Wolfram patients and of the life-threatening effects sodium could have on a patient if not properly regulated.
Electrolytes and Sodium levels in Wolfram Syndrome
What are electrolytes and sodium? Electrolytes are “salts” in our blood and cellular fluids. The difference between the concentrations of these salts inside and outside the cells regulates the contraction of muscle cells and the signal transduction in brain cells (neurons).
Sodium is the major salt outside the cells. A decrease in the sodium level causes water to enter the cells, causing them to swell. This may occur in the brain, leading to a condition known as cerebral edema, where the skull restricts expanding brain cells. A normal range for serum sodium is 135-145 mmol/L.
It seems like some patients with Wolfram Syndrome (WS) experience “low sodium.” Our body regulates sodium levels by balancing water in the body with the use of a antidiuretic hormone. DDAVP is often prescribed for patients with WS because they tend to produce less antidiuretic hormone and produce excess amount of urine. DDAVP is a synthetic antidiuretic hormone, regulates the body’s retention of water, and decreases the volume of urine. The challenge for WS patients is that they tend to have bladder problems and may need to go to the bathroom often. This is not because of the excess production of urine, but they may increase the dose of DDAVP, which increases the body’s retention of water and may lead to low sodium levels.
Symptoms of low sodium include restlessness, weakness, fatigue and muscle spasms. In addition, serum sodium levels may not be reliable when patients have poor renal functions or have severe hyperglycemia.Wolfram patients should consult with their endocrinologists if they feel their sodium levels are low.
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Rafaela Ungaretti, 18, Brazilian founder of Wolfram Inside, was highlighted in Mundo da Inclusa (World of Inclusion) magazine as an activist in search of a cure for Wolfram Syndrome. Rafaela created the website to share her knowledge and experiences of the disease. Mundo da Inclusa magazine and Wolfram Inside highlights The Snow Foundation as the world’s largest Wolfram Syndrome organization supporting medical research. The Snow Foundation is thankful for the financial support and recognition Rafaela and Wolfram Inside provides. 
