Prevention

Drink gallons and gallons of water.

This is perhaps the most common prevention method used. Nearly everyone you come into contact with will tell you to drink, drink, drink.

To find out how much you need to drink you need to get a 24hr collection done. When you get your 24hr cystine level, check to see if you have the total amount in mg and the total literage. By dividing the total amount of mg by the liters you get your untreated concentration . You need to get this rate down 200-250mg to prevent stones forming.

For example: 1500mg in 24 hrs with 3 liters of fluids = 500mg/liter. You would need to double your fluid intake to get to your optimal dilution i.e. drink 6 liters of fluid in 24hrs.

At least a third of this should be drunk at night. However this is not always possible so the aim should be to drink as much as possible during the night and the rest during the day.

Or another way to look at it is that the conversion factor for mg to millimole is 240 mg of cystine = 1 millimole, or 1mg = 0.04 millimoles. Thus, production of 1000 mg of cystine per 24 hours is almost exactly 4 millimoles. The rule of thumb is to drink one liter of liquids for every millimole of cystine to keep the average concentration below 1 millimole. If the 24 hour cystine were 1500 mg (about 6 millimoles), one would need at least 6 liters of water.

With regards to what you should drink, there are many theories. Some people swear by water and water alone. Others say that it doesn’t matter what you drink, providing there is plenty of it. Others try to avoid liquids such as tea, coffee, soda and alcohol. Anything, in fact which has a dehydrating effect or would make the urine more acid.

Medical opinion is also divided. However the most sensible advise would be anything in moderation, providing it is in liquid form and the majority of which is a fluid such as water, which doesn’t dehydrate.

Some, however, cannot face alcohol at all, others feel that their kidneys have suffered enough, others that it is vital in keeping stones moving. It is one of those things that is a personal decision.

For some, especially those who suffer a lot with stones, having a PICC line inserted is considered. This is a semi-permanent IV line where fluids can be hooked up and run through the night, increasing fluid intake at a time when the kidneys are most likely to produce stones. This is not a permanent line, is visible and definitely not for most people. Certainly someone who is controlling their hydration, pH levels and medications would not normally consider this option. It is a drastic measure but one which has been successful for some people.

Follow a methionine free diet.

Methionine is the amino acid from which we get cystine. To reduce the amount of methionine ingested would also mean a reduction in the cystine produced. Methionine is found in animal protein.

The diet is simple. Exclude animal protein by not eating any meat, limited milk, yogurt, cheese and other dairy, no eggs and no fish. Stock up on green vegetables and other forms of vegetable protein such as nuts, beans and pulses.

It is a very hard diet to follow strictly, however, there are some people who do it successfully. The value of the diet has not been proven scientifically, however it makes logical sense to reduce the amount of cystine we eat to reduce the amount of cystine available to form into stones.

Many people follow a modified form of the diet and just do the best they can not to go overboard on animal products. Some people have been advised that eating fish at all is worse than eating some meat.

Alkalization

This is raising your unrinary pH to a level of 7.5 – 8.0. Having a high pH means that the environment in the kidneys is not conducive to forming stones.

You can alkalize your urine by using an alkali such as Urocit-K, Polycitra, sodium citrate, potassium citrate or sodium bicarbonate. To work out how much alkali is necessary just take 24 hour urine profiles to find your low spots and alkalize accordingly. A persons pH is dependent on what they eat. This is why some people follow a low meat, high green vegetable diet, even if they are not following the low methionine diet. Green vegetables and lemon juice naturally raises the pH of the urine. Meat, fish, sodas and alcohol naturally lower it.

Remember that a pH of 8.0 and higher is likely to encourage calcium stones to develop.

You could also use lemon juice in water to a dilution which is drinkable. Although lemon juice is acidic it acts on the body like an alkaline and raises pH.

If you are using a sodium based alkali, you may be advised to reduce your salt intake. You may choose to reduce your added salt, avoid processed foods and foods which have a high salt content.

Medication

Tiopronin (Thiola)

This drug prevents the formation of kidney stones when there is too much cystine in the urine. It is the medication of choice for many people. It is not available yet In the UK., however it is available in the US and Ireland. However it can have some severe side effects such as skin rashes, itching skin, mouth sores, mouth ulcers and a condition which decreases the elasticity of the skin. It can also cause abdominal pain, gaseousness, diarrhea, nausea and vomiting. It has certain unwanted affects on an unborn child and should not be taken until the mother has discontinued breastfeeding. The good news is that hydration increases the effects of Thiola and it is often effective in controlling stone formation.

d-Penicillamine (Depen, Cupramine, Distamine)

This drug combines with cystine to prevent cystine stones. It helps the solubility of cystine. This is the drug that is widely available in the UK. Although it is slightly less effective as Thiola, it is effective enough to stop stones from forming. It is not commonly used in the US as Thiola is found to be more effective with fewer side effects.

The user may have side effects, the most common of which are a rash, itchy skin, swollen lymph glands, appetite loss, nausea, diarrhea, vomiting and impaired taste. It can also cause a sore throat, fever, unusual bruising, swollen feet or legs, bloody or cloudy urine, weight gain, fatigue, weakness and joint pain. It can also cause double or blurred vision, pain, ringing in the ears, ulcers, sores, white spots in the mouth, difficult breathing, coughing up blood, jaundice, abdominal pains, skin blisters and peeling skin. Not a pretty list!

The user must be monitored with regular blood tests to ensure compatibility, for example white blood count, platelet count, red blood count, hemoglobin and heamocrit, kidney function and liver function. When someone first starts to take Penicillamine, it is generally started slowly and gradually increased to minimize the side effects. The side effects are the main reason that this is not the drug of choice for many people. In addition to all this, it may be advisable to increase your B6 vitamin intake by taking a supplement as a B6 deficiency is common with this drug.

It is not advised that a patient gets pregnant whilst taking this drug and the drug carries over into the breast milk. However for some it is the only choice and is used effectively by many people.

Captopril (Capoten)

This is the drug of choice for those who are intolerant to Thiola and d-Penicillamine. Although it is not as effective as the drugs above, it can be useful in severe cases where the drugs above are no longer tolerated. It is the drug of choice for those with poor kidney function, for whom the other two drugs are not suitable.

It is more commonly used as a treatment for high blood pressure and for patients with congestive heart disease. Its side effects are fewer than with the other two drugs. For example a user may find they have a rash and loss of taste. It can, however, cause a severe anaphylactic reaction for those who are intolerant. Other side effects are swelling of the mouth, face hands and feet, dizziness, fainting, chest pain, fast or irregular heartbeat, coughing, confusion and nervousness. Also diarrhea, headache tiredness, sore throat, cloudy urine, fever, chills, nausea, vomiting, indigestion and abdominal pain. It must be stressed, however, that generally this is a safe drugs with few side effects presenting themselves.

You should not get pregnant on Captopril and it passes over to breast milk.

Prolonged use may cause a decrease in white cells and proteinuria (protein in the urine). Regular blood tests should be taken to confirm continued compatibility and the drugs should not be discontinued abruptly.