Treatments for Cystinuria?
Treatment starts with doing things to keep stones from forming. For adults and children, this means drinking more water, reducing salt, and eating less meat. If these steps are not enough, you may also need to take special medicine to help keep stones from forming.
- Drinking more water. Drinking lots of water will lower the ability for cystine to form stones in the urine. Ask your healthcare provider how much water you should drink each day to help keep stones from forming.
- Changing your diet. It is easier for cystine stones to form as urine becomes more acidic. Eating more fruits and vegetables can make the urine less acidic. Eating meat produces urine that has more acid, which can increase your risk for cystine stones.
- Reducing salt. Eating less salt can help keep cystine stones from forming. Try not to eat salty foods, including potato chips, french fries, sandwich meats, canned soups, and packaged meals.
- Medicine. Some people may also need to take prescription medicine to help keep stones from forming. Different medicines work in different ways. Some types help to keep your urine less acidic. Other types help keep cystine stones from forming by not allowing crystals to come together. Your healthcare provider can explain these different options and help you find the right medicine for you.
Self Help
Pain Relief
- Soak in a hot bath, use a hot water bottle or heat pad. The heat is a good form of pain relief.
- Keep busy, talk to people, use diversionary tactics. This may sound easier than it is to do but for long term or persistent pain this is a good habit to get into.
- Keep drinking. This is a recurring theme. Although it may increase the pain in the short term, it will make the pain go quicker.
- Go for a run, walk round the block or go swimming. Again, although you may not feel like doing this, if you are able it helps to relax the muscles and reduce the muscle spasms.
Some Suggestions For Moving Stones Quickly
With all stones it is important, before you make the decision to encourage the stone to move, to ensure that the stone is small enough to pass through your tubes and not to cause an obstruction. For stones that are too large to be passed naturally other forms of stone removal are probably best. Take advice from your doctor before trying any of these methods.
- Get and use pain relief promptly. Don’t wait until it is really bad. The quicker you take the medication the more use it will be.
- Keep drinking. This may be the last thing you want to do with renal colic, but it is the only way to keep those stones rolling.
- Go for a run or walk round the block. Again, probably the last thing you want to do but it gets the muscles in that area moving.
Medications
The following information is only meant to be helpful. Read the medical literature on any drug you take and discuss with your doctor. Always ask your provider what medications may work for your situation.
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. However it can have some 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 very 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. 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.
Evaluation and medical management of patients with cystine nephrolithiasis - cystine consensus guidelines J Endourol 2020.pdf