Nutrition

Just like everyone else, it’s important that people with cystinosis get the right nutrition to stay as healthy as possible. Cystinosis will affect your nutritional needs differently throughout the course of your lifetime, from onset and diagnosis through adulthood. Depending on factors like age and disease severity, different kinds of foods, drinks, or eating habits may be recommended.1

Here you can find some advice on how to handle some nutritional challenges particular to cystinosis. Always consult your doctor or healthcare professional when making a nutrition plan. Your doctor may also be able to refer you to a trained nutritionist.

Dehydration and nutrient loss

Cystinosis is the most common cause of Fanconi syndrome, a kidney disorder. If you have Fanconi syndrome, your kidneys don’t reabsorb important nutrients, which are instead passed into the urine. Without the right amounts of these nutrients and minerals, your growth may be slowed, your bones may not form properly, and you might experience excessive thirst, frequent vomiting, and poor appetite.1,2

Tips

In general, if you have Fanconi syndrome and cystinosis, it’s important to always have easy access to fresh water, because you will need larger quantities to make up for what is lost through urination.1 Do you crave salty foods? That’s normal because you’re losing so much salt through urination. In the absence of other health issues, such as dialysis, there is no need to restrict the amount of salt you consume. Potassium and phosphate supplements may also be recommended.1

Growth impairment and eating problems

Some people with cystinosis find mealtime difficult or uncomfortable because of such problems as gagging, choking, and vomiting. You might have acid reflux or find that it takes a long time to digest your food.1 If not properly managed, these difficulties can keep you from getting enough to eat. And if you don’t eat enough, chances are you won’t get enough calories, protein, and nutrients.2

Tips

Even if mealtime is uncomfortable, it’s important to find ways to get the nutrition you need. To make mealtime easier, consider replacing the standard three big meals a day with smaller, more frequent meals. Your doctor can talk to you about supplements, appetite stimulants, feeding tubes, or occupational therapy. Growth hormone may also be appropriate.1

Gastrostomy tubes (G-tubes)

If getting enough nutrition becomes a serious problem, a G-tube might be inserted into your belly.1 A G-tube allows food and medicines to be delivered directly to the stomach.

Dialysis and diet restrictions

As cystinosis progresses, dialysis may become necessary. Dialysis helps to clean waste from the blood when kidneys can’t function properly. Dialysis presents its own dietary challenges. A diet designed for a person on dialysis limits the amount of waste that enters the body so that less needs to be removed by the procedure.3

In general, if you are on dialysis you will need to:1,3

• Eat more high-protein foods
• Limit salt, potassium, and phosphorous
• Learn how much fluid is safe to drink

Tips

A key to managing a dialysis diet is learning correct portion sizes. For example, you may be instructed to limit potassium. Fruits and vegetables contain potassium, but some may be safe to eat within certain limits. The right portion size will let you eat foods you like and receive important nutrients without compromising your treatment goals.1

While dialysis is doing the important work of cleaning the blood, it can also remove important vitamins. That’s why it’s important to take vitamins and supplements, under the advice of your doctor or other healthcare professionals, such as a dietitian.1

For more information on dialysis diets, visit www.Kidney.org.

Kidney transplant

It’s common for people with cystinosis to have a kidney transplant. After a kidney transplant, many people with cystinosis begin to feel better. They may start eating more and growing at a more normal rate. However, it is also very common for patients to gain more weight than is healthy in the year or so following a kidney transplant. Gaining too much weight is linked to many other health complications, so it is best avoided.1 Talk to your physician about how to monitor post-transplant weight gain.

Tips

Patients with cystinosis who have had kidney transplants are often advised to follow “heart-healthy” diets that prevent obesity. Heart-healthy diets include lots of fruits, vegetables, and whole grains, and they limit portion sizes and unhealthy fats like butter and margarine.1

References

  1. Data on file, Horizon Pharma plc; 2016.
  2. Gahl WA, Thoene JG, Schneider JA. Cystinosis. N Engl J Med. 2002;347(2):111-121.
  3. The New York Times. Kidney diet: Dialysis patient overview. Available at http://www.nytimes.com/health/guides/nutrition/kidney-diet-dialysis-patients/overview.html?print=1#Food-Sources. Accessed July 21, 2015.