What Can You Do?

Hydroneprosis is a scray situation, but you don't have to be a doctor to take some extremely helpful measures. I have found that the very best way to be swelling-clear is to be Claritin Clear! � This is a simple, but amazing rememdy. If you prefer, taking a Claritin (loratidine) allergy pill a day does wonders for keeping or bringing kidney swelling down, since many cystinurics have a frequent state of at least slight swelling that comes and goes unpredictably. (especially if you have had one or more severe attacks already.) The successfulness of the Claritin � rememdy suggest an antihistamine reaction when the kidney swells. This would explain also why hysronephrosis tends to act up during colds, when membranes are swollen. Diphenhydramine (Benedryl�, Tylenol PM �) remedies will not work as they are a different type of antihistamine. Muscles relaxants (Doan's �) can have a similar affect for swelling. Notice that drugs of this type ("anitpyretics") suggest a putting out of a fever or fire- anti="against" py- ="fire" or "fever". While Claritin may interfere with the effectiveness of other medication, it is safe to take with nearly all medications, so you may want to add it to your regimen, occasionally or routinely. The plain kind (Not D) is best. Loratidine is a mild antihistamine, especially safe for sensitive people, such as young kids or the elderly.

Click Here to Be Claritin Clear

Renal Failure

To keep this in simple terms, renal simply means "kidney" or "kidney related". There are 2 basic kinds of failure- 1) acute (immediate, not lasting) and 2) progressive or chronic. In rare cases, chronic kidney failure may result from the complications of stones and the stress they cause on the organs. But in most cases, if there is renal failure, it will be acute. There are several words doctors and others may use to describe acute failure, including blockage, shutting down, etc. How Can I Tell If I Have Acute Failure? If both kidneys are failing, this is of course, a medical emergency. But in most cases of the stone patient, only one kidney will be blocked, which makes it a little less serious, but still serious nonetheless. Acute failure is usually caused by a stone that has become stuck in the ureter, and this will not allow passage of stone or urine. Symptoms included severe pain (or no pain at all), or an alternating between the two, swelling of the belly/side/back, pressure felt from this swelling, little or no urine output, nausea, vomiting, fatigue, malaise, and strange mood changes. Your brain is telling you something is wrong, and when the kidney shuts down, the area of the brain which controls your kidney stalls, resulting in the mood changes or confusion. You may feel as if something had "died" in you, because you will feel no activity in that kidney as usual. Hydronephrosis may also be present, as the blockage will cause urine to forced up in the wrong direction, resulting in swelling. If you are in the hospital for a stone, the doctors may discover this failure, if they detect a blockage on an x-ray. If you are at home when you think there is a failure, you can probably take care of yourself at home, as the stone will eventually move downward, and your kidney will come back "to life", within a few hours or days. This "awakening" that you feel is most likely do to normal blood flow returning to the kidney. Important Note: Keep VERY well hydrated during this latency period, as you will probably feel very dehydrated. Gatorade � or Powerade � works GREAT for keeping the electrolyte balance and hydration. It moves very fast thru the body,bringing relief. Should you feel you are becoming too dehydrated, tho, do not hesitate to see a doctor.

Chronic Failure

Chronic kidney failure (chronic renal insufficiency), has many causes and is the complication of many diseases, not just kidney related. When in advanced stages, the damage is usually irreversible, and the kidneys will failure completely, resulting in death. The damage occurs over a period of years, is "silent" in occurrence, and may be discovered incidentally during routine testing. Symptoms include weakness, edema, shortness of breath, nausea, tiredness, and commonly, intense itching of the skin. How does this relate to you? Depending on the level, number of stones, physical stress, and trauma of one's Cystinuria, there is a small possibility that progressive kidney failure will result. But rest assured, this is VERY rare, and, even in patients with severe cystine cases, chronic failure will never....crystalize.

Dehydration

As mentioned, dehydration is common in kidney failure. But many cystinurics experience a sense of dehydration often, whether the kidneys are actually failing or not, known as the notorious "unquenchable thirst". There may be unknown reasons for this, or it may be due to a slight or moderate drop in the salt or water balance that chronic dysfunction of the body brings. More than one cystine patient has reported a feeling of dehydration after drinking Coca Cola-just Coke, not other soft drinks, specifically. A feeling of dehydration may be especially present early in the morning or upon waking, or with constipation or intentionally holding in the next bathroom break. (Many cystinurics will avoid the urge to pee or poop at a given time as an unfortunate way of avoiding a pain attack. This is not a smart practice because the best way to avoid excess stones is to void. But unfortunately, it is a reality.) An unquenchable thirst can leave you feeling very dry, drained, and tired, occur after eating salty foods, and may be accopanied by a headache. Excess water alone will probably not help in this case. One of the best remedies is to keep drinking the Gatorade� to get things back in balance. A variety with a high sugar content is especially good.

Infection Section

Due to inbalance and chronic disease, this may make the patient more prone to infection, and we're talking urinary tract infection. This usually includes any or all of the following: ureter, bladder, urethra. Kidney infections are more dangerous, and usually classified as a separate entity. Not only movement and the presence of a stone, but infection may occur from blockage, failure, or hydronephrosis. Infections can be can be one-time, occasional, or chronically reoccurring. A tendency towards reoccurrence is NOT FUN on top of already painful stones. If the occurrence is occasional, you may want to pick up some Uristat � and be treated by a GP with an antibiotic. But it may not be the last...and it's not hard to see why. With the body continuously out of whack, infections can crop up as often as once a month or more, even a never-ending infectious state that lasts from day-to-day. In stubborn cases such as these, your urologist can prescribe an antibiotic such as () that you take on a continual basis, probably every day. This is a lower dose and more of a prevention method that a one-time cure. Note your side effects, but they are usually mild a few, such as diarrhea.

Warning! Is There Such a Thing As Cystine Poisoning?

We spoke about antibiotics earlier, and the standard antibiotic of choice for UTI's is Bactrim, a HEAVILY sulfur-based preparation, and obviously that's why it's in a class called sulfa drugs. And cystine contains ALOT of sulfur. If there is such a thing as cystine, or sulfur-like poisoning, i think iv'e come as close to it as you can get. If you are a cystine patient, PLEASE strongly consider never using the sufer based drugs. The results can be horrible, since it's the equivelant of putting more CYSTINE into an already overloaded body! I have used Bactrim many times in the past, and never had a problem with it. Until just recently, and it produced a very scary and bizarre reaction. I should have known better. Not only did it bring on a mass production of stones in the days to follow, i experienced (for the next several hours i might add)a repeated shaking a trembling on the right side of my head and body, that sometimes extended to my kidney (not a seizure, tho) a strange mental state, extreme anxiety, and reduced ability to perceive color! Greys and light blues were the most noticable, it's the closest i've ever come to seeing a "black and white" world for myself! ( a passing form of color blindness, you might say). Needless to say, i'm glad to be alive and it was the scariest day of my life! Along with the shaking it also produced extrmeme, involuntary muscle tensing and rapid growth and hardening of the nails (since cystine affects finger and toenails), and a rash of red bumps. I want to spare all of you the scare that i went thru, so the next time you visit a GP, please put down Bactrim as one of your "allergies"! (It's easier for some doctors to understand it this way than going into a lengthy explanation about cystine and sulfur.) Either way, you won't be prescribed a cystine hell!

Notice how much a sulfur rock looks like a cystine stone. Click on the pictures.

Picture 1
Picture 2
Picture 3
Picture 4
Picture 5 (with Calcite)
Picture 6
Picture 7
Picture 8
Picture 9
Yellow Tinted Bactrim (indicating sulfur prescence)

Next we'll look at some pictures of actual cystine stones to make a comparison. The bottom picture shows the actual cystine chemical formula. The "S" stands for sulfur. It's spelled "cys-S-S-cys" in letter form. As mentioned, cystine is sometimes called "dicystine". This is because cystine forms from a disulfer bond. Notice also the prescence of sulfur in the methionine bond. This is because cystine (and cysteine) synthesizes from methionine. Also notice the abscence of sulfur in lysine, arginine, and orthinine. This would explain why these actual chemicals are not part of the stone's make up, which was touched on earlier. (On a note, "sulfur" is the correct spelling. The spelling "sulphur" is used in the U.K. and abroad, but scientists have made the Americanized version the technical term.)

Pic 1
Pic 2
The Famous Quarter Pic!
Cystine Formula
Cysteine Formula
Methionine Formula
Lysine Formula
Arigine Formula
Orthinine Formula

I can't emphasize enough how dangerous sulfur is for you. Need help understanding? Sulfur, who's name literally means "mineral of fire", is found in volcanoes, meteorites, hot springs, and is repsonsible for the colors in Jupiter's moon, Io. In addition to antibiotics, some things to avoid would be MSM (organic sulfur) supplements, and the acne medications Acnomel, Fostril and Rezamid.

Diverticulum

In rarer cases, there may be a diverticulum ( a small, unnatural pouch or pocket of skin) on/in a male or female urethra or bladder. You may be born with this, (congenital) or it can be caused by stone trauma or other trauma. As a result, an area of skin and tissue will weaken, resulting in a pouch or fold. Squeeze a portion of a small, hand-size balloon between your fingers like you did as a kid. This is what a diverticulum looks like. A diverticulum is usually asymptomatic, and may not even be discovered on an x-ray or test, because it appears invisible. There may even be multiple pouches, esp in the bladder. A diverticulum can lead to infection and other problems. Surgery for this is available, but it is somewhat difficult, due to inflammation of an infected diverticulum. God forbid you should ever get a stone stuck in one of these! Take the guy in this link, for example. A Brazilian man has a giant stone in diverticulum. Giant Stone You know, all jokes aside, men really shouldn't give birth ... OUCH! Let's hope none of us ends up that unlucky....

Calyceal Diverticulum

A rare and speicalized form of an outpouching that may occur is a Calyceal diverticulum, or a diverticulum of the kidney itself. A calyceal diverticulum is a cyst-like pouch that deveolps on the wall of a calyx (division of the kidney pelvis)- congenitally, from blunt trauma, or obstruction. This kind of diverticulum occurs in any number of individuals, not just cystine patients, and are found in both males and females, children and adults. The pocket will most likely spawn stones, infection, and collection of urine inside it. About 36% of patients will present calcium oxalate, calcium phosphate or carbonate apatite stones in the pouch. The situation (if present, but rare) will only be complicated for a cystine patient, as more stones and cystine concentration will increase with this problem. Kidney diverticula are related to other kinds of kidney cysts and similar dysfunctions.

Section 2:What To Do About Stones: Therapies and Procedures

Now that we know what Cystinuria is, what it does, and how it affects you, Do you have it, and what the heck can you do about it? Cystinuria brings distress, both immediate and long term. This section will cover what can and will be done to bring relief, health, healing, and answers, both immediate and long term.

Testing, Testing

From the boring to the beneficial, you will want to be familiar with the various tests and methods that go along with being a cystinuric. Hopefully these next few paragraphs will increase your knowledge and ease your fear!

What to Expect From A Hospital Visit (Besides the Bill !!!)

A stone has hit without warning. You are in panic, and on the way to the hospital. What's gonna happen (or not happen) next? When you arrive, a receptionist will want your info. If you have a friend or family member along with you, they can give the info, since you will be in pain. If the stone has gotten out of control, you will be hollering and moaning constantly, maybe to the irritation of others waiting around. Don't let that stop you! (As if you could help it.) You might wait what seems like an eternity, while that ER takes forever to call your name. And when you get back there..more waiting! They will want your vitals, a urine sample, then take you to your room. Depending on what your hospital is like, they may or may not give you your pain shot right away. AHHHHHHH!!!! Nope,they won't take the chance of a one-time shot til they prove that something is wrong and you're not a doctor-hopper. Proof ? They seem oblivious to your screams heard 'round the world, as they're milling around out there in the hall, making jokes amongst themselves. They may think your carrying on is weird or even funny. But hopefully they'll be sympathetic to your face. When a doctor FINALLY comes back and gets your IV water-drip in, then it's time for the tests. They'll do an IVP (see paragraph below) or plain xray, or maybe even a CATSCAN (CT-SCAN) or MRI , later on. And finally...drum roll please... when they see that stone, it's time for a shot. Some still use Morphine,but a lot of hospitals use Meperidine (Demerol �). In fact, it's the most widely used pain medicine in American hospitals. Once they see their proof, their faces will go from comical to caring in a hurry. Something was wrong...you were telling the truth after all ! I'm not saying all hospitals will be this way. Some may have you back in and taken care of in a hurry. But just as a friendly reminder...
Note: Once you can think clearly another day, you'll know you can't really expect doctors, nurses or anybody else to "feel your pain" if they've never lived it. We all know their methods are standard, as there are many fake visitors, and they treat you no different then they would anybody else. But in a desperate moment, you will be ready kill anybody in your path.

Tests at the Hospital Or Doctor's

Remember, this is ONLY a test! :o) The following are standard tests performed for a first-time stone patient at a hospital, or as part of routine check-ups.

Urinalysis

A urinalysis is your standard pee-in-cup test. We are all familiar with it, as it is done for most doctors visits, even when you are there for a cold or flu. For a stone patient, you will be asked to do one at nearly every visit. You may use sterile wipes with a red dye. After leaving your present ;) in that cute window or on a tray, it will be checked for basic findings. These include levels of protein (when in balance, are healthful), and signs of bacteria or blood. Or maybe even...surprise! A small stone or two. Your doctor may tell you there was blood. But i didn't see anything! That's because it's too small to be seen with the naked eye. If there was infection or other abnormal findings, the appropriate treatments will follow.

*Note: When this routine starts to get old, or for a quick reading, there are always at-home tests that you can buy at your local drug store. These are one time use and disposable. There are a few brands, and they vary a little in the way you take them, but a good one to try is the Azo Test Strips �. They check for protein and nitrites. You can find them here: Test Strips Then you'll be ready to...um...go with the flow.
**

IVP'S

An IVP or intravenous polygraph is a "dye test in the veins". This is a very common procedure used when dealing with a stone patient. It is used to clearly see a stone(s) that would otherwise not show up on a non-dye xray. Radioactive dye, also called a contrast, is administered into the veins with a prefilled pump and needle of dye. The dye is dark blue in color. Cystine stones (especially small ones) can be very hard to see on normal xrays, they appear almost translucent or invisible. (This is also why it is so hard to convince medical personnel that you truly have a problem.) Once the dye is in the veins, it reaches the urinary system within minutes. The dye then illuminates the kidneys and related organs when they appear on the xray. This can be compared to a camera flash used in a dim room. The doctors and nurses can then look at this "neon" image and quickly pinpoint the stone. A word about the dye tho, for first timers to this test, when injected, it can make you feel very sick, like gagging and throwing up, as it rushes to your blood and brain, but this is short-lived. Your xray technician may warn you of this. When i had this test for the first time, a friendly nurse brought me a cool wash cloth to hold over my face and neck to keep from gagging and to use if i threw up. I really appreciated that, i hope all your nurses take the time to do a nice gesture!

Note: Another inspection method is to insert the dye directly into the urethra, known as retrograde urography.

Diagnosis

An xray or IVP can see a stone, but it can't determine what type of stone it is. Cystinuria cannot be confirmed unless these in-depth tests are performed.

The Good Doctor

You will want to find a reliable urologist or nephrologist or team. You might want to look for one who takes an interest or specialty in your type of stones or stones in general. Be clear and specific with your symptoms and concerns. Your doctor will work with you to provide you with the appropriate amount of pain medication, antibiotics, or anything else needed for your treatment, such as stone therapy medications, which will be discussed later. Although not all patients may be presented with this, routine blood tests are sometimes done to make sure the kidneys are not going in to failure.

Straining to Pass...

And when those stones have finally made their appearance... save those babies! It's very important to save as many stones as possible, to have tangible proof of your disease and its pain, and for testing purposes. Your doctor or hospital will provide you with a strainer, a plastic funnel with a mesh bottom, with holes so small it catch even the smallest stones. Don't mistake stones for table crumbs! Tiny stones need storage. Here are some collection tips for the newbie stone maker: Use your strainer, rinse them in the sink in litely running water (so they don't spill from the strainer! ) and lay them out on a cloth or piece of TP to dry. Once dry, you can keep them in small plastic jars/vials/or other see-thru containers. Or here's a cute idea! :) Buy one of those plastic bead kit containers and organize them by size, shape ,or color (whether you use them to make jewelry or not is up to you ;) )

24-Hour Urine Test

After visiting a doctor or hospital, you may be required to do a 24-hour urine collection when you return home. This is a fairly simple test, and you will be given a sheet of instructions on the time, methods for collecting. It measures the current level of cystine concentration in your urine. This is very important for evaluation by your doctor, as it can help in diagnosis and treatments, as well as follow-up monitoring. Some docs may require a sterile water jug that YOU provide from home, but usually, you will be given a jug with a spout or large mouth. A word of caution! While some aren't, many of the jugs are lined with a poisonous, burning substance like hydrochloric acid. I learned this the hard way too.....when i thought i'd take a "little" sniff, and got a nosefull that i about choked on ! It does fume or "smoke" when the lid is removed, so stand back! This acid makes it possible to interact with your own cystine. The jug i received was lined with acid from top to bottom, so another word of caution...you will NOT want to put this near any delicate skin, as it can cause burns. I recommend collecting your pee in another sterile container or fresh cup, and pour it in the jug immediately. Important Note: If a young child will be doing the collection, it's especially important to keep the jug away from their skin and eyes.

Stone Composition Analysis

This is da man. An actual stone analysis is the only test that can really diagnose a cystine stone, or any kind of stone for that matter! This is why saving your stones is so important. Your doctor will take your stone, or if have more than one, a prime specimen ( probably a large one ), and if it can't be tested locally, he/she will send it off to a lab for analysis of its make up. Once at the lab, there will be various methods of testing used to determine its chemicality and structure. In addition to inspection and photos with a microscope, there will be a cross-section, a determination of its surface, and an inspection of the core and composition using "infrared spectroscopy". This procedure is one of passing electromagnetic radiation thru the stone, as it relates to it's molecular energy, producing a reading. Analysts may also use special software, that categorizes stones, to help in the findings. Once its composition has been confirmed, the results will be returned to you in 1-2 weeks, in the form of a lab sheet, with a photo of your stone(s) on it and a "gross" description of the findings. (and they are, too ;)) It will tell the size, weight, texture, color, shape, whether or not a core (nidus) is present, and most importantly, its make up, which would be in most cases of this, 100% cystine. However, another 20-40% of patients may present stones that are a mixture of cystine and another substance such as calcium phosphate, calcium oxalate, or magnesium ammonium calcium phosphate. It is unclear why chemicals other than cystine present themselves, but it may have to do with the varying levels of cystine-for example, a patient with a gene pattern programmed to make lower levels of cystine may present this finding.

Medical Intervention

The tests came back positive. The diagnosis in is. You have Cystinuria, more specifically, a stone that is caught. And you need help-fast! These methods and surgeries will be used at those times when disease escalates to a level where medical intervention is mandatory or even lifesaving. This isn't the most fun part of the site to read, but neither is the thought of not knowing how to get help!

Cystoscopy

If a stone is suspected, your doc may want to first of all see where it is located, so the best possible course of action can be taken. A cystoscope is one of two instruments, either a flexible or hard tube, used for inspecting the bladder. It is not so much a retrieval instrument (as in the Ureteroscopy, discussed below), its primary intent is for inspection, as it contains a light on the end. You will be put under local or general anesthesia, and the physician will slowly insert the scope up your urethra. He/She will then look thru the scope to find the stone. If a stone's location is determined, surgical action may be recommended.
The cystoscope is also used to detect other signs of abnormality in the UT, including infection, bleeding, or tumors. A doctor can then insert tiny surgical instruments onto the scope for a biopsy sample of the tumor or the bladder lining.

Kinds of Surgeries

The following are your basic stuck-stone-surgeries. All sound risky and primitive, and not very pleasant, but they can be necessary in a moment of crisis. It goes without saying that these removal methods apply to all types of stones, not just cystine.

Basket Case

Ureteroscopy or "Basket Retrieval" uses a fiber-optic, catheter-like instrument called a ureterscope with a small cage or basket-like device on the end of the scope, to retrieve the stone. In fact, it is the preferred method of lower ureteral stone removal. It is often done as an outpatient procedure, not only in the hospital, but may even be simple enough to do in a doctor's office. No incision is required, and will usually be done under general anesthesia. (or local ). The surgeon inserts the scope up thru the urethra, and locates the stone thru medical imaging, such as an ultrasound, etc. He/ She then grabs the stone with the basket, or shatters it with a small shock wave using the scope. Risks~ Risks include perforation of the ureter and stricture. There is a greater chance of stricture if the stone has been imbedded within the ureteral wall for more than 2 months.

Mini Fact: Before fiberoptic imaging came in to use in the 1970's, extraction was done was by the "blind basket" method. This was the same approach, except that the surgeon used blind probing to locate the stone. This was too risky, doing ureteral damage, and is not in use anymore.

Beam Me Up, Scotty!

Laser surgery is a popular, modern method of stone removal, that is similar to cystoscopy. It is less invasive than traditional surgery. You may hear your doctor mention this option, but it can sound a little iffy if you're not familiar with it. But now we'll take the time to understand it. Laser surgery can be performed on an outpatient basis in a local doctor's office. A scope that contains a laser fiber is inserted into the urethra. When the laser is activated, the beam pulverizes the stone. A surgery such as this is also called "lithotripsy", which means breaking up a stone into passable pieces. In some medical circles, this method is slowing replacing shock wave lithotripsy, ( which will be discussed next ), because it is less risky.

Shocking

Extracorporeal shock wave lithotripsy (ESWL) is one of the most talked about and possibly one of the most used methods when stone surgery is needed. While it may seem like the way to go when a stone is unpassable, i would really consider all your options, as this procedure can be quite risky. This is one time the risks may really outweigh the former. This process takes about an hour, give or take. Mild anesthesia/sedatives may or may not be used, prior to, and during the procedure. The patient lies in water-filled tub, with a pillow for back support. The machine is then aimed at the area of the body where the stone lies. Millions of powerful sound waves pass thru your body, shattering the stone. As in the case of a hard, stubborn stone, (such as cystine), it may take several rounds of blasting before the stone is actually crushed. And the more shock waves, the more risky it is. So, what ARE the risks? Many websites and doctors make it sound simple by emphasizing the benefits and failing to mention just how risky it is. These are true sound waves, and they have such a high frequency that the patient is required to wear ear protectors that look much like DJ headphones. There can be slight to moderate injuring of the body at the "shock" site, such as bruising, internal bleeding, post-bleeding a few days or hours later, or post-infection.

Traditional or Open Surgery

This is the standard surgery that tends to come to mind and is called nephrolithotomy. It doesn't need much explanation, as it is like anything else, first, being put to sleep, then the surgeon makes an incision in the skin, to remove the stone either from the kidney or the ureter. For alot of people, tho, this procedure isn't used or preferred as it is more invasive and a little more risky. This method is usually chosen for extreme stones, very large ones or staghorns that are rendered unpassable. This may be done after the stone cannot pass after any reasonable amount of time, or after a very quick amount of time for a painful stone. A whole staghorn can obviously not pass thru the ureter, so alot of the open surgeries will be performed on the main kidney head alone. It is a preferred method for very obese patients, too.

Back Door Options

PNL or percutaneous nephrolithotomy is a less-invasive surgery of choice for some stones. A small slit (about one centimeter) is made in the back, with the surgeon guiding a small wire into the kidney. A passage is made around the wire thru dilators (wideners) to access the kidney. These wires are called "percs" in kidney patient circles. A similar procedure is that of placing a catheter or catheter-like device directly into the kidney in the back, when severe blockage prevents urination or insertion of a catheter in the normal way.

Temporaries

A temporary catheter is just that-temporary. It will be used during your hospital or doctor's visit by the doctor or a nurse. A small, (6 inch) flexible catheter of this type is used to take a mini urine sample or maybe even remove small stones in the process, oftentimes for a patient who's urination is temporarily blocked. It is inserted only in the urethra, and removed within a few seconds. Some people can't handle the feeling of plastic in a small space, even for a fews seconds. For a stone patient who's urethra is scarred/narrowed, pain is likely to be a problem.

Stents

A stent is a flexible tube the diameter of a spaghetti noodle, that can be placed in the ureter by a physician for a variety of reasons. As mentioned before, it helps with widening when a stone is trying to be passed. The stent runs from the bottom of the kidney to the inner bladder. When the stone is in the process of passing, the stent can successfully drain the urine around the stone, while the stone(s) is the only thing that moves thru the ureter. Stents are used after surgery, such as basket retrieval. There can be swelling and irritation to the ureter, and the stent can be placed to leave the pathway "open" as the trauma subsides, and normal healing can take place.They can also be used for non-stone reasons, such as to relieve the blockage of a tumor. But remember! The pain and irritation of a stent can be ever worse than a stone, so hopefully this option will never be necessary!

Is Removal Ever An Option?

In those extreme incidences when kidney damage (from stone stress, etc) is beyond repair, kidney (and ureter) removal may unfortunately, be necessary. But this should only be a LAST resort, as the result will make things more complicated than the present state. Say only one of your kidneys produces stones. As soon as it is removed, the remaining kidney will compensate for the "loss" by making the cystine stones, even if it never did so before. As we can see from this example, only one kidney (depending on the person) is apparently responsible for the filtration of cystine. So it would be better to keep both kidneys, than put the stress of the stones on one alone. And kidney transplantation is just as much of a gamble. Regardless of whether you are a stone patient, a kidney transplant is risky-it involves finding a suitable donor, and anti-rejection medication will probably be required for the rest of your life. Procedures with the actual kidney should be the LAST alternative of the surgeries, and unless life-threatening, only you (not a doctor or anyone else) can have the final say. It must be your OWN decision, and a long-thought out one at that.

Therapy,Man

You've had your surgery. But here comes another stone, and another, and another, and another. What can you do when the doctors are thru? Relief is in sight! This is the therapy section, and will deal mostly with at-home therapies and self-help techniques, growing more personal as it goes along, going from the temporary help of a pain killer, to the possible long-term modifications of a diet. The therapy section is a lead-in for Section 4, which contains even more suggestions for personal modifications, that are based on gender, climate, lifestyle, and other considerations related to your friends, family, and YOU as an individual.

Pain Medications

I can't think of any situation where narcotics are needed more. Stones cause super-strong pain, so you need something super-strong to kill it! If you come to the hospital with your stone attack, you will be given the strongest available, like Morphine or Meperidine , in shot form. For other times, your doctor can prescribe any of the various narcs in pill and/or liquid form (for kids, or those who can't swallow tablets), on an as-needed basis. To kill the pain of a stone, anything from a small does of Codeine, to one guy who needed 4 shots of Morphine, could be needed. You may take quite a few of these prescriptions over the course of a lifetime. But don't let anybody raise an eyebrow at you! While tolerance/addiction is possible in anyone, i wouldn't worry too much about it your case, as dosing can be monitored. Tho it may seem like an inconvenience, stone pain is responsive to very little else, and given the acuteness (and unpredictability) of the attacks, immediate relief is the only choice. The pain also can be quite chronic and SEVERELY chronic at that. While this seems hard to believe, it can get very old being "stoned" after a while lol. Let me give you my personal tip: The pain is worse than the pleasure is good, if that makes any sense. And if you've dealt with many stones, believe me, it will start to make alot of sense.

An Interesting Thought: For those who don't know, how necessary is it to kill the pain of a stone? This example is good. Pain killers like Vicodin � and Percocet � are prescribed standard for teeth pulling and other dental work. While i understand alot of people need that for a tooth, in my personal opinion, it's unnecessary. I can hear you stone people agreeing with me. The pain of a pulled tooth is VERY TOLERABLE compared to a stone! Once you have experienced a stone, it's a whole other ballgame. Believe me, after a stone, you'll be begging to have your whole mouth of teeth pulled unmedicated. Ahh, ain't the *pain* of normal life grand?? :D

Ok! Let's look at some of your options for blasting that pain and their explanations.

All the narcotics, of course, have a morphine-like action. Some are true derivatives of poppy, while others are lab-induced "imitations". But they all have basically the same effect. Some side effects, beside sedation, may include dizziness, nausea, constipation, and intense itching. Due to possible complications, like tolerance and intensity, meds are mixed with an over-the-counter, usually Tylenol� , and not given in straight form.

Codeine: A true morphine-derivative, supposedly the weakest of the narcotics, often given to children in a liquid preparation, labeled Tylenol w/ Codeine number #2, #3, #4, etc.

Hydrocodone (Lortab �,Vicodin �, in various strengths) Tylenol+Hydrocodone strengths: 7.5/500, 7.5/650, 7.5/750, 10/650, time released (sprinkles) or standard (plain).

Oxycontin (Percocet � ) Oxycodone+Tylenol, time released or standard, in various strengths

Vicoprofen � -Ibruprofen+Hydrocodone

Hydromorphone (Dilaudid �) Hydrocodone + Morphine

Meperidine ( Demerol �) (usually given in a Promethazine combination-to help with the nausea-of stone and drug)

Morphine

Ultram �-Tramadol Here's an interesting item. Ultram is a non-narcotic with a narcotic-like action. An "imitation of a synthetic"- whatever that means. It's the weakest in the line up, and has a slightly lower effect than codeine or hydrocodone. But all in all, it's still powerful for a less-severe pain.

Ketorolac-friend or foe?

Ketorolac (Toradol �) is an alternative prescription pain killer offered in the place of narcotics. It is an anti-inflammatory much like ibuprofen. (Advil � or Motrin �). It is given after various surgeries( not just kidney related) and is used for glaucoma problems. Many people report much success with this, as its action is to soothe the inner organs, like the ureter, relieving the stress of small stones. If given in intravenous form, it can actually help propel a stone to the nearest exit! In pill form, tho, it can take hours to work to its full effect. While its sedation effects can be strong, this is NOT an effective pain killer when it comes to stones. In your case, it will probably help nothing more than a headache.

Less Is More

With prescription pain killers, you'd think, the stronger, the better, right? Not always. As you go up the scale in strength, you may find that the real knock-you-outters have a back firing effect. For some reason, the more powerful ones dim everything...but the pain! While they may leave you intoxicated, some pain is still felt...this is a scary situation! Your doctors will want to help you by giving you something strong enough to kill pain. And for good reason! But if this happens to you, ask your doctor for a lower strength.

OTC's-Is That Possible?

Depending on your level of pain, it is! But since stone pain is rarely "mild", they will come in handy as a supplement. A good suggestion i got from my own doctors was to take something such as Advil or Tylenol in between times to help ward off pain. I have found this to be a very good idea, as it provides added relief before the next dose. Try it!

*But remember, things such as pain meds are only a MASK to the real problem. Don't let it get to this point! Or you will always be living in fear of your next pain episode. Get help! *

The Closest Thing To A Cure

Let's be brutal. Unfortunately, at this point in time, (August 2005) there is no cure for Cystinuria. There has been talk of possible gene therapy in the future. But while we are waiting for that on some distant horizon, there are stone therapy medications available thru your doctor, that can actually modify the amount of cystine in the urine. These will NOT cure you, and different people experience different results. They can help tremendously, or maybe not at all. They may be your ticket to freedom or just another possibility to check off the list. Usually, an individual will hit the middle ground ( not completely sick or well, periods of wellness, which may include occasional relapses.) Severe cystine concentrations are harder to tackle. Here are you options:

1)Thiola � :

Tiopronin or alpha-mercaptopropionylglycine (whoa!), is manufactured by Mission Pharmacal � of San Antonio. Their name says it all. Mission � is on a quest to continually to provide cutting-edge medicine for rare diseases such as this. Just as Cystinuria is called an orphan disease, this an "orphan drug" due to its rarity. Thiola is a white crystalline, that works by going thru an exchange with your own cystine to theoretically lower the amount, and thereby lowering the chance of forming stones. When prescribed, they come in glass medication bottles, and they must be kept in their original bottles for protection from light, and for freshness, etc. Your pharmacist should inform you of this. It's hard to say exactly what side effects to expect, as they are varied, and the drug has been used and tested on a relatively small number of patients. (that's because of the rarity of the disease, of course.) Some common complaints include skin-related problems, such as jaundice or peeling. Another person reported tiredness. Other symptoms may include easy bruising, stomach irritations, muscle aches, or loss of taste. Thiola may have all or none of the same side effects as Penicillamine (as discussed below). Routine monitoring and tests may be done to watch for side effects and check your progress. For those interested in further understanding its properties and possible complications, this is the most comprehensive link i have found on Thiola, from the Mission Pharmacal � site (a note to anyone on dial-up, this is in Adobe Reader � form)Thiola

So, how well will Thiola work for you? Some people report reasonable wellness, many have had at least some level of sucess with this product. But patience really does pay off: Thiola taken consistently for a few months straight may really start to show results. When i first started writing this website, i could not report on its findings because i was just starting Thiola for the first time. But about 6 months into it, i could notice a decrease in the size of the stones. What a blessing! But let me warn ya, the pain and number of stones has remained the same. Most still take a long time to pass and feel no different than larger stones. But any little change is in itself a miracle. Mild-moderate cystine cases will respond even better to its use.

What about dosing? According to the Mission company itself, dosing should not be arbitrary. While an unsafe upper limit is not really known, the dosing should be dependent upon the level and severity of the cystine. With 100 mg to a tablet, 800/mg (8 pills a day) may be too much, or 1,200/mg + (12 or more pills a day) may be too little. Your doctor will does based on the readings of the extensive urine test.

And for our overseas friends outside the U.S., Thiola is known by these brand names:
Capen�
Captimer�
Epatiol�
Muscolysin�
Sutilan�
Thiosol�
Tioglis�
Vincol�

Interesting fact: Where does the name Thiola come from? A thiol is a compound in which sulphur is present, and its chains are found in cysteine. Or, according to our friend Miriam: Etymology: International Scientific Vocabulary thi- + 1-ol 1 : "any of various compounds having the general formula RSH which are analogous to alcohols but in which sulfur replaces the oxygen of the hydroxyl group and which have disagreeable odors".

One of the most commonly talked-about side effects of Thiola is skin wrinkling. If you've never used Thiola, this can sound scary and you'll wonder if you'll come out looking like Dumbo :D But the wrinkles are localized ruts or creases that look like scars, not "elephant skin". They are likely to occur where natural bending and creasing takes places, such as your elbow or crook of your arm. And for the most part, they disappear on their own.

2)D-Penicillamine (Cuprimine �, Depen�)

Penicillamine is the more outdated and risky option, and was the "classic" drug used to treat Cystinuria, starting in 1963. Penicillamine was first discovered in 1954, while studying skin sensitivity with penicillin. The drug is a mixture of penicillamine and cysteine, and just like Thiola, works with your own cystine to lower the amount secreted. Both are chelators or ones that "mix together".

Thiola and Cuprimine are specially formulated just for Cystinuria, and are not a "cover-all", as most drugs. They are used for a couple other diseases, tho, including Wilson's disease ( an overload of copper in the body),and certain types of arthritis, tho i should mention they are NOT anti-inflammatories, just to clear up any confusion.

As you can see, these stone drugs are a relatively new concept, just as the knowledge of the disease is.

Baking Soda and Buffers

One of the most promising and inexpensive options for a stone-blasting regimen is baking soda! Baking soda (sodium bicarbonate) is a buffer, or a chelator, making the urine less acidic, and actually binding to the stone and "eating" away at it! The baking soda option is most often used for dissolving cystine and uric acid stones. Baking soda will not always work, as conditions must be right within the body, but other times, it will work like a miracle! Begin by taking anything from a teaspoon to a tablespoon, mixed in an 8 oz glass of water or other liquid (to mask its salty flavor). It may start to work in a few short hours, but usually baking soda will take a few days to reach its desired affect. And it's no secret that's its working~baking soda comes right out in the urine! If it has successfully dissolved a stone, the baking soda will cling to the pulverized pieces, resulting in a mushy clump of stone/baking soda in the urine. Amazing!

For those who can't stomach plain baking soda, what other options are there? Try an Italian twist. After spaghetti for dinner one night, i noticed the next day this same crushing happened to my stone. Was it in the sauce? No ! It was the parmesan cheese! Try it ! Its alot more fun and yummy than baking soda.

I wish i could say that baking soda always works as the miracle cure, but it seems to have a mind of its own. When i first started that regimen, stones started to pulverize. But after a while, (even with taking more) NOTHING HAPPENED! Talk about a teaser! Go figure that one.

Dye Medications

As you probably know, dye meds aren't just for urinary tract infections! These are a true lifesaver for a stone patient. They are unique in that they can do something no other medicine can--and that's hit the pain right at its source...on the skin!, both inside and out. Here are the kings of pain killing: Uristat �, Azo �, and Cystex � Note: {This isn't the same as the dye found in your IVP'S ;) }. I would recommend Uristat (Phenazopyridine Hydrochloride), as your main man, Azo (also Phenazopyridine Hydrochloride) second, and Cystex (Methenamine), third. Cystex is not really a dye, but has a similar action. It is the only one of the three that does not stain the urine. All 3 preparations are over the counter. They can be a bit expensive, but you'll be glad you did! I have been told that even as close as 30 years ago, dye meds like this did not exist (except maybe by prescription). As for the prescription kind, I had some once for a bladder infection. These are..(insert) name) and will turn your urine pale blue or green. A word of advice! It really hurt and stung, and that blue-green funk turned out to be worse than the bladder infection! If this happens to you, that's def not that way to go...

~Water Logged~

While water has been STRONGLY preached as a necessary "drug" for the cystine patient, every person is different, so don't take this recommendation as the gospel truth. The one reason to maintain a healthy water consumption is to make sure all systems are running smoothly, and plenty of urine is being made. As far as stone formation goes, that's another story. While it has helped decrease the cystine concentration of many patients, it's no guarantee. Cystine stones are a hard nut to crack no matter what approach you take. A person can make the same number of stones, even with more water. So take this or any suggestions on water with a grain of salt, and tailor it to your own needs and how much your body can comfortably hold. Kinds of Water: Again, speaking from my own experience, some waters are better than others. City water is a gamble, and really a personal choice, depending on the conditions in a particular city. As for bottled water, i have found brands like Dasani � and Crystal Springs � to be really "mild" and gentle on the kidneys. Most off brands are fine too, i really like the WalMart Sam's Club brand, its really soothing. But i do NOT recommend Aquafina �. Aquafina ,for whatever reason, whether it's the minerals in it (or lack thereof), seems to make MORE stones, esp small, gritty ones, even within a few minutes of drinking it. Do a little research of your own- check the labels, or go to the websites to see how a particular brand of water is filtered or what the ingredients are.

While a large water consumption probably won't do much in the long run,it's important to keep alot in you for another reason. ACID PEE BURNS! And it can be torture to feel that as it comes out, because cystine urine is extremely thick, even when no crystals can be seen. While it won't be thinned out enough to stop stone production, it may thin it out enough to pass peaceably.

*Alert!*

While i know i'll get bashed for this, i think this next thing is the WORST piece of advice i've ever heard for kidney stones. Many, many sources suggest that you repeatedly wake yourself up during the night, at least 3 times, to drink more water. I'm sure this is well-meant but it's just not smart! While i can't argue that there are many things worse than a kidney stone, this is just unreasonable. The idea behind this is that cystine production will continue to mount, even in your sleep, and you must continue to thin it out, 24/7. Yes, it could, but i don't think these midnight water raids are the way to go. As with any disease, you need all the sleep you can get, and as you know (and believe me, i do) enough sleep is lost as it is trying to deal. I have had hundreds of sleepless or mostly sleepless nights trying to deal with pain or other symptoms. Well, i guess if you're up at THOSE times you could drink your water lol. Cystinuria knowledge in some areas is still in its infancy stage, so just use your common sense when looking for advice.

What's Diet Got To Do With It?    

In most schools of thought, a low protein or more vegetarian-like diet is recommended for the patient. This is because of a concentration of methionine and the related cystine-amino acids found in products like meat, pork, eggs, milk, cheese and so on. Again, this may help some people, but it must be taken with a grain of salt and catered to your own needs. If it helps, super, you're on the right track, but the foods you take into your body are not the root of the problem. It's the same concept as with cholesterol. Modern findings show the cholesterol that you take IN to your body has little to do with the cholesterol that is actually processed inside you. It is more due to internal processes, or heredity. Same with cystine and its family of amino acids. No matter how much of these "bad" amino acids your body consumes or absorbs, it has little bearing on what your body will actually make or pass thru urine. Since the error is built in to produce too much cystine, this wrong "signal" will continue to do so, regardless of what you eat. The focus here should be good health and diet in general.

What Foods Contain Cystine?

The foods that contain cystine are similar to methionine, and include bananas, meats, milk, and pistachios. I once read a long list that included almost all the main and well known (and liked) foods as containing cystine! So that doesn't help much, especially when you get the munchies. I'd recommend eating whatever and in whatever way you always have, because, like we said, the cystine taken in (in small food amounts) won't have much bearing anyway. If you try to avoid everything under the sun, you'll drive yourself crazy. And that's not good for your health!

Of course, there are exceptions when choosing food that could mess you up big time....

Moo Juice

A note about milk-in writings, you'll find that milk is one of the dreaded methionine culprits and is said to be avoided. I have not found any satisfactory proof about milk one way or the other. It may sometimes contribute to the stones, but maybe more because or its texture and thickness than the aminos. If you enjoy milk, take it slow and monitor yourself closely, as with any food or drink you think might be offsetting a stone attack. The food reactions are as unique as your own body....

Should Your Ride the Porcelain...?

Sorry folks, but alcohol and cystine do NOT mix. Even trace amounts (like in medications or cooking) can cause large stones. Trust me on this one, be careful!

Should You Do the Dew?

Rest assured, Dew lovers, your regular, basic yellow dew (or Mellow Yellow � ) doesn't seem to be a problem. But there's a scary (and very yummy) orange monster known as LiveWire.... watch out! I've made a bad stone from this and so did somebody else i know ! I'm thinking it's that creamy, dreamy vegetable oil that's added... ^*^*^*^*^Enter at own risk.....^*^*^*^*^

*Note: Vegetable oil is an ingredient in many soft drinks, popcicles, and the like. You may want to avoid it if possible, especially if in the process of passing a stone.

The #1 No-No

What is the worst thing you can do for a Cystinuric? Put more cystine in the body! Cystine and related supplements are all over the internet, offering an array of benefits. And for a healthy person this is just fine! Lysine supplements have long been used to prevent and treat cold sores and other things. Wanna break the record for the worst cystine case in history? Yeah, that's what i thought. But with your personal cystine supply, you have a built in immunity! Read the surprising benefits under the "Genetic Pros and Cons" header of Section 3.

Did They Say Cure?
Here is something very important that i need to clarify. I have read that Cystinuria can be cured by removing the offending kidney(s) and receiving a transplant. This is absolutely FALSE. As talked about, Cystinuria is not a disease of the kidney, but in the way that the chemicals are routed. It is prone to cause confusion because the kidney tubeles are not able to filter properly. But the defunct is built into the GENES, NOT the kidney itself. The wrong command is coming from an unchangebale chromosome,if not for this, the kidneys are otherwide healthy. With a built-in command, cystine will not filtrate whether with your old kidneys or brand new ones. Note:With the stress that the offending stones exert on the kidneys, Cystinuria has been called a type of kidney disease. While it is accurate to say this since the stones and cystine have a direct effect on the kidney's well-being and even functioning, a cystinuric's kidneys are otherwise just as healthy as the next person's, minus the stones.

Now, On To Some Healthful Options

We've covered what to avoid. Now, what should you be taking for cystinuria? What would best benefit your body? Here are some easy options to include in your new lifestyle.

In severe cystine cases, diet modification alone will not help. But it is still worth a shot no matter what level your disease stands at. You may have heard the term "alkalization" of the urine. Chemistry enthusiasts will understand the Ph process in better detail. But for the rest of us, it's simple. Ph scales or reports measure the concentration of certain substances in the urine. At one end of the Ph scale is acid, at the other, alkaline. The urine of a cystine patient is extremely acid. Therefore, one would want to make it more alkaline, to prevent stone formation. Foods with a high alkalinity would include orange juice, gratefruit juice, and all fresh citrus fruit. But if the alkaline in them doesn't work, Vitamin C is said to be good for the stone patient. And for those who tire of citrus stuff ,or for kids, there's always pineapple juice. It's chock full of vitamin C. Also, cranberry juice has long been recommended as a cover-all remdey for most types of stones. This is because of the high Vitamin C content and alkalizing effects. While this is a good place to start in your Vitamin C regimen, it's no guarentee to be helpful when it comes to severe cystine stones. As far as vitamins go, the best bets are Coline & Inositol and Vitamin B-12.

Hippy Remedies

Good Ol' Mother Nature. She's ready to provide you with an array of remedies right from her own garden. You know what i'm talking about: Herbs and natural remedies. 3 important things to remember when you're considering herbs as part of your health regimen. 1) Almost ALL drugs and "unnatural" remedies go back to the earth, their chemicals come from nature ,therefore making herbs a separate classification as "all natural" inaccurate. 2) Treat all herbs as you would anything else, with caution and moderation, "natural" does equal "safe" and many herbs can be deadly, even in small amounts. 3)They are not for everybody. Now that you know the precautions, what are the benefits? The key thing to remember here is , they are not a cure-all, as toted by many fad companies. They know herbs are the in thing in some circles, and are eager for your dough. So be mindful when choosing. They should be incorporated as part of your healthful diet and lifestyle, adding positive health benefits. A good place to start would be the label. Do they tell the ingredients? All or most of the ingredients? These are a safe bet. You know what you are getting and can go from there. Or are the ingredients kept "secret" in fear of the magic potion being revealed? These are not a good bet You do not want to deal with shady products, number one for your own health, and what if there was an ingredient in it you were allergic too? Just because an ingredient is safe for one person does not mean it is for another. So proceed with caution. Say you're in the throes of a bad pain attack. This is just not bright-- do not reach for some mystical arabian root and hope he'll work his magic. That genie's not gonna work his magic! XD

But,to give them a fair run, there are some herbal products that could be a really helpful supplement to urinary and stone health.Again, the key is not so much healing, it's prevention and supplementaion. The herbs recommended for urinary, stone ans diuretic health are: Dandylion, Wormwood, Gravelroot, Marshmallow, Parsely, and Uvi Ursi.

External Therapies

We've got the inside of the body covered.But you can really help kill your pain with these simple and easy to do tips!

Heating Pads & Ice Packs

I can't tell you how much i appreciate the inventor behind these ingenious babies. Iron-filled , body-heat activating pads come in a variety of brands, shapes, and sizes, and can be bought at your local drug store or just about anywhere else. They come in the shape of small rectangles, ovals, or full body wrap-arounds. These disposable pads with a sticky back can be applied to the skin or over clothes, and last about 12+ hours. They are sold as relief for minor back, knee, menstrual cramps, or other body pain, so of course, they were not originally invented for stones, but man do they work! When you need a great way to help soothe stone pain, just apply one to you back or over your bladder.They really work to help soothe the muscles, and may even relax it enough to help push a stone thru with its heat! I recommend the ThermaCare first, and the BodiHeat. The BodiHeat are the least expensive, and surprisingly, one of the most effective. I also recommend the body wrap style with the velcro, because they are very soothing and stay in place better and longer than the pads.
Find Them Here!
And Here!

But i have to warn ya! Do NOT fall asleep with one on! I did one time, by accident, and it left painful water blisters on my skin.

Even better than a disposable heating pad, for home use, is an electric heating pad. It can cover all bases no matter where a stone is causing the pain. This one heats up super fast: Dunlap

Another good pain killer is the homemade water bottle. Take the label off of a plastic water bottle, fill with hot water, and apply. There ya go!

Ice Packs

When a heating pad just won't cut it, an even better option is an ice pack! Take crushed or cubed ice from your freezer, and fill a small Ziplock � bag. You can also make mini ice packs with the 2" tall mini-bags.

Tub Time

Nothing helps ease the pain and tightening muscles of a stone attack more than a hot soak in the tub. Hot water or a shower spray can even help move a stone out. And don't be afraid to use the tub as a toilet! While in plain water (with no soaps, of course) you can try passing the stone or stones. This can help ease the pain and make passage easier. And no, that's not just a wacky idea of mine. This is oftentimes how it is done after shock wave surgery, with the patient still sitting in the tub.

And...

Hottubs!! These things are AWESOME for a stone attack. I happened to be near a hottub one time when a stone hit. They are very relaxing. Get a strong water jet against a sweet spot, and who knows...it might just put enough pressure on it to loosen a stone! :)

Section 3: Life With Stones: Adjustments, Lifestyle, and Helpful Tips

Doctor Availability

There are doctors (including pediatricians) who specialize in Cystinuria. But they are few and far between. Why aren't there more doctors who specialize in Cystinuria? The answer is for reasons of convenience. Suppose a doctor became a urologist, with Cystinuria as a side interest. No matter where they decided to set up practice, there would be little demand for something of this sort. Or suppose a doctor wanted to specialize in Cystinuria ONLY. He would have only a few patients in a given town or even state. And when those people leave, where is the biz? The doctor would be forced to keep moving from town to town, in search of cystine patients. Even if there were 100 patients in one place, this would never be enough to make a living on. But the docs who take a side interest in it are ABSOLUTELY NECESSARY. Doctors and scientists who take a serious interest in this field is where it's at. Or most of our knowledge on the disease would never be known today. All urologist have to have a basic understanding of stones of most kinds. So, when you are treated, you can still find most of your help from these general stone urologists. Most doctors would not choose our speciality because, 1) It is of little use or interest to the general population 2) And, even after years of extensive study, portions of Cystinuria are still poorly understood, and they may feel it useless since there is no permanent cure. But just because something is rare, does not make it any less important. There are thousands of suffering people looking for help for something that is underacknowledged. Even if 1 person were suffering (suppose it was your Grandma or a good friend), that would be reason enough to find them help. Don't you agree?

The Info Train

If doctors are that scarce, you can only guess what i'm about to say next. No big surprise ending here! Cystinuria information (even a good size chunk of it) is just as few and far between. As i mentioned at the top of the website, there are some cystine articles on the web, and they can be nosed out. But, like we also talked about, knowledge is in its infancy stage. When punching Cystinuria into your basic search engine, you will come up with about 900 results. But don't be fooled. Most websites, have a section on the disease, and many are duplicates-compare websites and they say the exact same thing, word for word. Some website info is as puny as this-"Cystine are a rare type of kidney stones. They are inherited". That was a *big* help, but who can blame 'em?! So, how many sites are dedicated exclusively to Cystinuira in humans? Only a couple, i'm afraid. The most extensive web article to date is by Shekhar Biyani on eMedicine.

Besides internet articles, are there any books written on Cystinuria?

Thru my searching, i haven't been able to find ANY books written solely on the subject. The only one is a study of Swedish school children, written in 1966, plus a pamphlet from 1936! Here's where i saw the book:Rare Book

Reality Check

Now, we'll start to discuss the realistic side of your disease as it applies to everyday life. Cystinuria isn't a "rainy day disease"; something to be considered only when a major flare-up occurs, or is it a disease of convenience. What will life be like for you? Your stones can be anything from a minor irritation, to a moderate setback, to a living nightmare, or maybe all of these at once ! You will need to make realistic adjustments to fit this new part of your life. These will no doubtedly (but unfortunately) include work schedules, sleeping schedules, social activities, and how you function every day and interact with people. There are new (and possibly more) doctors appointments to consider, sleepless nights, and changes in appetite and energy levels, which will no doubt put a damper on routines and any semblance of normalacy. The main rule of thumb to remember is SLOW DOWN! This is an absolute necessity, one you will be forced to do anyway at one point or another. For you, this may mean cutting down to a part-time job or not being free to schedule things as you please. None of this is a sign of laziness, but just part of the reality. It will be a confusing time for you and those around you, and hopefully, you will have sympathetic friends and family, as will be discussed more later.

Exercise

Is exercise good for you during your disease? Yes and no. If you aren't in too much pain and a stone needs passing, walks are a good idea. Just moving the body around may help to loosen the stone and relax tight muscles.

The Stone Belt

A new rock group? Or something else. The stone belt refers to the southern part of the united states, from Texas to the East Coast, where, because of the heat and humidity, kidney stones of all kinds are the most prevalent and hard-to-treat. Even tho Cystinuria is inborn, humid weather (no matter what part of the country you live in), can contribute to stone formation. I have noticed in my own experience that larger, more stubborn stones seem to form in the summer.

Life to Its Fullest

For a Cystinuric, life will undoubtedly take on a whole new richness and meaning. All of you cystine sufferers out there know exactly what i'm talking about. Life will not be viewed as the same drudgery that so many healthy people seem to take for granted. Because any time NOT spent in pain for the cystine patient is a real blessing, nothing short of heaven on earth. But how can a person with so much pain keep such a positive outlook? Once you have lived with a merciless disease, your priorities will get straightened out in hurry. Mundane tasks will be an exciting victory to a stone patient, who does not get to experience the freedom of going here and there whenever the spirit moves. When time is "rationed", you realize how important life is. Yes, there will be times when you are depressed. You will hate the whole world and hate your cursed body who hates you. You will want to die, give up, escape this senseless suffering. Unable to accomplish much at times, you will feel useless, nothing more than a chunk of dead weight to your loved ones and society. Cystinuria not only affects one physically, but emotionally, mentally, socially, and personally. All of these feelings are natural! But its hidden blessing is, the suffering paves the way to the good times.

Dead at 21?

A disease brings weakness, but a diseased person is a STRONG person. Ironically, these same stones that bring grown men and women to their knees are what make them stand the tallest. Millions of people, every year, commit suicide for a whole array of reasons. But i have NEVER heard of a Cystinuric committing suicide! A life full of pain seems like the best reason to die, but most have chosen the high road! I have seen many cystinurics, especially teens, who have a positive outlook and uncommon bravery. They are an inspiration to all of us! And they know the secret: problems are to be faced, not run from.

Genetic Pros and Cons

Is there an upside to such a painful disease? There is! And good thing! What could possibly be good about having stones? Well, nothing, but when cystine comes into play, there are some added benefits. Cystine is the building block for hair, skin, and nails, and you may notice harder nails at certain times or changes in hair or skin. People with an elevation of cystine tend to have more immunity to common cold and viruses, as cystine helps to ward off these invaders. While not proven, your supply of cystine and lysine could help ward off viruses of the herpes family, such as cold sores and mono. Even if a cold or virus is caught, its duration tends to be shorter for this reason. And if you're worried about all the Tylenol in your narcs, get this~shots of CYSTINE are given to people who have Tylenol poisoning of the liver! And who said this disease was all bad, huh? :o)

But there are downsides to this condition. Let's check 'em out~

One not-so-obvious sign is that of bone fuser. Due to cystine's effect on the growing body, there may be bones that never fuse completely by adulthood, such as a knee. These will be discovered incidentally thru x-rays, possibly from pain or pressure exerted on a joint. I had this happen on my own knee, and, even as an adult, the bone near the kneecap has not completely fused. Many people with the disease also report short stature. Whether this is due to genetics or disease, or both, is hard to say, but studies from way back have reported the short stature phenomenon. The reasons for this defunct growth are poorly understood, although similar growth haults have been associated with other diseases such as Cystic Fibrosis, Angleman's Syndrome, and Prader-Willi, due to a poor function of cystine. It's amazing how many rare diseases, although not a specific cystine malfunction, display signs of poor growth or related problems due to cystine not working as it should.

Do Stones Follow Formation Patterns?

For unknown reasons, yes. Maybe you have found your worst attacks tend to be on certain days or certain times of the day. Stones (and pain) tend to get worse at night for alot of people. Stones are usually passed with greater ease and in greater quantity in the morning, after sleep. Mine seem to be most prevalent on these days: Friday, Saturday, Sunday, Monday. ( i can't tell you how many stones i've passed on a Saturday!) What's your prime day for passing? You might want to keep notes on it to see if there's a pattern.

The Ladies Room

Heads up, ladies! Here's some tips 'specially for you to help with um...the daily grind of passing. Periods and stones are two separate things, right? Guess again. Of course they are, but since all body systems coordinate, whether it's for good health or bad, your monthly cycle (and not just the bleeding stage), can have an effect on stone formation. First of all, there seems to be a pattern at times, with the bigger and more painful ones occurring around ovulation and the actual bleeding stage. So it apparently has to so with the rise and fall of hormones (estrogen, progesterone,testosterone), tho i can't say which for sure, as i'm sure this has never really been studied. I would think it was the rise in estrogen, as it rises at the said times. And together, they probably interact with cystine production or distribution. Want to compare stones to your own rhythms? I recommend mymonthlycycles.com. When there, you use a clickable calendar to record all aspects of menstruation, and you can use the "notes" section to record your stone patterns. This is not only handy for yourself, but also if you want to have this info ready for a doctor sometime.

As discussed before, cystine pee is VERY acid, throwing the body off, making it prone to infection. But even when no infection is present, acid pee and stones can irritate the vaginal skin, and you may mistake this for a yeast infection or urinary tract infection. Or remaining acid urine may cause imbalance, leading to infection. When in doubt, ask your doc.

Just for Guys

Guys, we all know your internal unrinary systems are longer and more complex. Therefore, one can imagine that passing your stones could be even MORE difficult or painful than for a woman. Nurses have confirmed this too me, also. The male urethra becomes narrower in diameter, at certain points, compared to the female's. Men have described it as passing a basketball, and "giving birth thru that thing!" Similar to female hormone cycles, there are rhythmic rise/fall patterns of testosterone throughout weeks/months for men. This would be a good thing to consider when monitoring stone formation. Certain levels of testosterone may be interacting with cystine production and the intricate balance of the body systems.

A Note to Guys and Gals-This is a little talked about thing but must be said. Sometimes, after passing many sand stones (small grit in one sitting) , these tiny stones will cling to your skin, and you might not even know it! This can cause MUCH pain and irritation, you may think some stones still needed passing, when in reality, they are already out of your body. Guys, if you are uncircumcised, microscopic stones can get caught. Rinse your penis in warm water to remove any debris. And for ladies, any "aftermath" can be removed with your fingers or by rinsing in warm water. This all sounds pretty funny :D, but it happens! The last thing you need is for a stone to get stuck outside your body rather than in ;) Also, men or women may be able to pass stones (especially small ones) voluntarliy or involuntarily thru ejacualtion. This process also allows stones to soften or break up when they wouldn't normally otherwise.

What About Baby?

Now that you know you have it, you'll wonder if your kids will get it. Should you be worried? Maybe, maybe not. It depends. It's recommended that your prospective partner should be tested for mild cystine elevations, if they haven't done so already. On the brite side, since relatively few people are carriers, it's unlikely that they will be, but possible. If you are the only carrier(diseased partner),the chances of passing it along to your kids is close to zero. If your partner carries the gene, the outcome will be almost certain, or 100%. If there is more than one kid, the chances are between 25% and 100%. Or, the kids may be born with "silent", mild-moderate cystine elevations, that mirror the "silent" parent. From the beginning of modern findings ,Cystinuria has probably been seen between siblings more than any other family grouping, such as a more distant connection, like a said person and an uncle.

Caution in pregnancy: It should be noted that, if you are thinking of/or have started Thiola or Pennicillamine, these drugs have a possibility of causing birth defects, such as cleft lip or more serious complications, when used during pregnancy. It has been recommended that Thiola only be taken in those extreme cases when stones are unbearable, even in pregnancy (and knowing stones, it's doubtful alot of cases would be minor ). It can also cause miscarriage, maybe due in part to the fact that Thiola causes muscle weakness/limpness, and therefore may not be able to adequately "hold" a fetus in place. It should also be noted that taking Thiola many months or years before a pregnancy can marginally increase the chance of causing birth defects. Now THAT's scary!
On another note, Thiola may interfere with fertility and the start of pregnancy. Depending on your mood, this could work for you or against you! XD

And Sex?

Can you have a normal sex life with a kidney disease? Well, according to one website, you can! One informative kidney website states that even a dialysis patient can continue a normal sex life. So get it on!

How to Deal with Your Naysayers

When faced with something of this sort, we'll all have our naysayers who, whether their intentions are good or not, can leave us frustrated for their apparent lack of understanding. And, after all these years, i have realized even more that you can't expect somebody who has never been thru an unusual situation to understand. It's just unreasonable. But let's make a distinction here. We aren't expecting the royal treatment. We wouldn't want people dropping everything like we were the only thing that mattered, waiting on us hand and foot. While this disease doesn't suddenly make you all-important, it doesn't mean your friends can't be sympathetic. Hopefully, they will treat you like anybody else, with the exception that you may need a few minor adjustments. You are still a person, and hopefully you won't be either avoided or fawned over.

~But You Look So Healthy!~

What a classic! Of course you look healthy! Cystinuria is an internal disease. On certain days, others may see you happy and "normal", and think you look so energetic, that you must be exaggerating. This is due to the unpredictable nature of the disease-one can go months without a stone or problems, or there can be a problem every day. That's when you'll have to gently remind them that you can't judge a book by its cover... and hope they'll take your word for it.

~Is this an excuse to get out of school or work?~

A definite No.

~C'mon, the pain can't be that bad!~
If THAT won't make the pain worse, WHAT will?

~Uh-Oh, Here Comes the Hypochondriac~
Hypochondria:"Centered on imaginary physical ailments". Imaginary? Don't you hate this one? I think there's enough evidence from the tests (AND in the toilet!) to prove this is no game of the imagination. How could you possibly make up something this complex? The stones are so horrifying in themselves that there's no need for exaggeration. All your energy is taken up in trying to make it thru, there's none left to put on a front.

~We All Have Problems, Get Over It~
OoooO, such lack of understanding! Who has encountered this classic?

Section 4: Funky Factoids!

Did i cover all the bases? here are some mini facts for inquiring minds who want to know..... :)

(For those who can't read the green text, please highlight with your mouse)

Related Conditions

A related condition to cystinuria is cystinosis. This is a condition in which cystine accumulates in body tissues and other parts of the body, leading to more severe complications, especially in early childhood. Cystinosis is more common, and its knowledge is more widespread than that of Cystinuria.

What other kinds of stones are there?

A few basics kinds and many mixtures. Some common types include Calcium, Uric Acid ( goes along with gout ), Struvite, and Xanthine, which come from the VERY rare condition (enzyme defect), Xanthinuria. Click here to view many kinds of stones from humans and animals! All Kinds of Stones

We tend to think of Cystinuria as the classic stone "disease", with other kinds of stones being random flukes. While some occur as flukes, such as after a large alcohol consumption, a bladder infection, or presence of bacteria, the many kinds of stones you hear about are associated with some type of condition, that the person has been born with or otherwise acquired. These conditions operate on the same principle, of having a certain chemical or element raised to an unhealthy level. The difference is, alot of these other conditions appear to not to be as severe or as chronic as Cystinuria, which leads us to believe they are occurring as flukes, with the person making a relatively small number of 1-10 stones in a lifetime. While they may not be as severe, several of the other kinds are actually the most common. Calcium stones are the most common stone, occurring in 1-2 million people annually. "Textbook" knowledge says that common stones occur more often in men, and in people between the ages of 20 and 40, with an average of 2 stones per lifetime.

All In The Mix

Can you have more than one kind of stone at the same time? Well, yeah. Apparently, there have been cases of people making several kinds of stones, each a separate kind. OR you can have a mixture, usually with 2 kinds to a stone, which might include cystine.

Other Stones.

We all know what gall stones are, but can you get stones in other parts of your body? Yes. Liver Stones, Pancreatic Stones ( from chronic inflammation of the pancreas, due to large alcohol consumption or thyroid problems), and Vaginal Stones can occur. Don't believe me on this one? Click Here: Vaginal Stones . Only 26 cases have been reported.

It's Gone to the Dogs!

Did you know...? That not only humans can get it, but animals as well?! The most common breeds w/ cystinuria are Mastiffs, Newfoundlands, and English Bulldogs as well about 70 other breeds of dogs.It has also been identified in cats, and the Maned Wolf or "Guara", a fox like-creature of Brazil. I'd hate to be the dog who has one and can't talk. *Ruff* *Ruff* What's wrong Lassie? Did you pass one at the hydrant again?!

Our Buddy Ben

Ben Franklin invented the first flexible catahadar (in America) to bring relief to his brother, who was ailing with kidney stones. A similar cathadar was in the worx in Europe before this. Thanx Ben! :)

No Hypocrite

Stones were metioned in the Hippocratic Oath.."I will not cut for stone, even for patients in whom the disease is manifest; I will leave this operation to be performed by practitioners, specialists in this art."

Oddities...Doin'A Double x Take

As promised, we'll talk about duplications of the urinary system. There can be duplications of the kidney, ureters, bladder, or urethra. But it doesn't stop there- There can be more, with as many as 3-6 ureters in the body! (up to 2 extra pairs.) Of course, this many is rare, but ureter duplications occur in about 15% of the population. They are 10x more common in women. These duplications are not a disease, and are found in healthy individuals or sick individuals. They are genetic flukes and the tendency towards this may run in families. What causes it? Again, this is due to faulty gene coding. The gene has the "command" to make only 2 kidneys, 2 ureters, etc. But sometimes, the gene portion makes an extra copy of itself, resulting in a duplication, much like an identical twin, if you will. Kidney and ureter duplications are probably the most common of the urinary organs. If there are double bladders or urethras, this will probably require surgery, as the funky system will not drain urine correctly, and other complications. There are two kinds of ureter duplications, a partial, and a true or ectopic ureter. A partial occurs when a sprout of a ureter buds or breaks off (before you are born), and grows connected to the other one, much like siamese twins. The bud may be shorter, sometimes weaving in and out of the other ureter, and usually (but not always) connected. A true duplication results from the double copy of the gene. It is ectopic (in an unusual place). It grows at another point in the kidney, completely separate of the other. It usually drains into the bladder, but its end point may be ectopic too, ending in a wrong place, like the uterus, for example. This may require surgery to correct. Also, each ureter has its own renal pelvis. No, this is not a bone. A renal pelvis is the internal network of the kidney. Typically, there is one to a kidney, but there will be an extra pelvis to accompany its ureter. So what does this mean for you? If you were born with a double ureter, you may not know it for a long time, as it is often asymptomatic. (producing no symptoms.) Again, i speak from experience. I was born with a true double ureter on my left kidney. Mine looks like the one in this drawing: Double Ureter . This has been a great help to me, and can be a blessing in disguise for anyone who has stones. Only my right kidney makes stones, and when the bad one has failed a few times, the extra system on the left has been a tremendous help, doing the job of two kidneys, taking the stress off one when the other is "gone" for the moment. For others tho, it may cause a little trouble, as the funky kidney is prone to infection, reflux of the urine, irritation, and minor pain. In non-stone patients, a duplication may be found during xrays or in childhood.

More Mini Facts...

Sly Stallone and wife Jennifer Flavin have a daughter named Sistine Rose.

The Holy House

Common Blooper!- it's "Sistine Chapel" not "Cystine Chapel" ( ! )

~A Special Message~

Now, you may be wondering, how do you deal with a disease like this? Where does my strength lie? For me, there's only one ultimate answer~ and that's my faith. Being a Christian since i was little, Jesus has been my protector, and now He's my refuge and strength to help fight this scary battle. I hope you will want Him to be your strength, and "very present help in trouble" as your journey continues. Remember, Jesus can bring comfort in sickness, but he isn't a magic genie to grant immediate requests if the time is not right. If that were the case, all the world's problems would be gone with just a few prayers. But He is a Loving Father. Although He can, He does not choose to bring the instant "healing" that so many people are looking for. He works a different way in the modern world, thru people, knowledge, and the power of prayer. Although mountains might or might not tumble into the sea, He works many "mini-miracles" that we can see in our everyday lives. Sometimes, He doesn't work around hardships, but thru them, to accomplish a higher purpose, since He knows all. As iv'e struggled to make sense of my disease, i have found this Bible verse to be the most comforting:

"And as Jesus passed by, He saw a man which was blind from birth. And His disciples asked him, saying, Master, who sinned, this man or his parents, that he was born blind? Jesus answered, Neither this man sinned, nor his parents, but that the works of God should be displayed in his life.
John 9:1~3

I've always liked the song "Doctor Jesus" by Randy Travis. I think it's really fitting. I'm sure you'll like it too.

You know, I've got so many problems
And lately, I've been feelin' kinda down
I hear you're the One I should talk to
I hear you're the best healer around

Doctor Jesus, will you help me?
Make me better, make me whole
Doctor Jesus, Lord I need you
To mend my heart, and save my soul

There's so many out there who need you
Do you think you could work me in?
You see I'm in the worst of conditions
But mostly, I just need me a friend


         

~Thank you for visiting my website. It has been very healing for me to write this website, as well as alot of fun. Organizing all this information has given me a chance to really refelct on, and come to terms with what's happened over the years. Hopefully, i have helped at least one of you find the answers you need. Remember, Never Give Up, Never Surrender.~

"Prayer does not require eloquence, but earnestness"





Loving Borrowed from: Chronic Pain Support

A Short Dictionary Of Terms~
urologist:a physician who specializes in the urinary tract
nephrologist(not to be confusd with neurologist, a nervous system doctor):a doctor who is concerned, more specifically, with the kidney alone, rather than general urinary health.
Cystinuric : a person with Cystinuria
intravenous urography : the process of using in-vein testing methods, such as the intravenous polygraph.
uti: abbreviation for urinary tract infection.
amino acid: a building block of protein
Hematuria: blood in the urine

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