Ben’s Story

Written by Ben Lokos

I have been trying to compensate with this disease for 18 years. My first run-in with Cystinuria was on my 16th birthday, while playing golf. My left kidney shut down and the doctors threatened me with open surgery unless I could pass the stones, which I subsequently did.

Eight years later at age 24, I had developed a huge staghorn on the left side that created a blockage to my left kidney. A percutaneous was performed at this time but I needed to stay in the hospital for four more weeks in order to dissolve the 30 smaller stones that remained after the surgery.

Five years later, at 29, I again had developed a staghorn on the left side. This go around it took two percutaneous and one lithotripsy to get rid of all the stone material. This time I was sidelined for only two weeks in the hospital, but I had become very weak.

Four years later, at 33, I again developed a staghorn on the left side but this one was smaller than the other one had been. This time I had a ureteropyloscopy performed which was successful in getting to all the different calyxes in the kidney. I also developed a small stone in the right kidney which caused intense pain in the right ureter. This stone passed on its own, followed by another.

Unfortunately, no matter what I seem to do I still develop staghorn stones. However, during the last 17 years I have developed a “learned helplessness attitude” toward this illness. Every time I seem to be slowing down the production of stones, they seem to return. Do any of you feel this way?? I know that some Cystinurics have experienced greater hardships than I have described. Yet let’s face it – this is NO PICNIC.

I now treat the condition very seriously as it has also caused me to have extremely high blood pressure. (180/130) All along I was drinking 1-2 gallons per day, as well as taking 60 mgs of Polycitra-K crystals during the night.

After the last surgery was done, I discovered a natural way to alkalinize my urine, by drinking wheat grass juice, and chlorophyll. I am also taking d-penicillamine which I gradually adjusted to. My nephrologist (Dr. Zackson) recommended a desensitization schedule until I am able to tolerate a therapeutic dose. The reason for the desensitization to Cupramine is because I am allergic to Thiola and Captopril and developed hives with Cupramine when I originally took it. An allergist recommended taking a tweezer-full at a time for two months until I built up to one pill. After 2 more months I built up to three pills.

In closing, I have discovered that by drinking the wheat grass juice, I am better able to adjust to alkalinizing the urine and actually derive tons of energy. I know that I will beat this condition and not let it get to me, and I think we all need to maintain a positive attitude!

The High Cost of Denial

Written by Carolyn Arrington

This is a personal account of my own struggles with cystinuria, and while it is always difficult to recall the traumatic events involving my past medical problems, I hope many other cystinurics can relate to my experiences.

I’d like to tell you I’ve been courageous in my endurance, vigilant in following doctor’s orders, a cooperative, docile patient — but that is not true. Some of my resistance to initial treatment came from my own emotional difficulties surrounding the particular time of my diagnosis, as well as a myriad of complex factors. Suffice to say, I was a troubled young adult, mainly from unresolved dysfunctional family issues, and the first few small stones occurred in my late teens. Though painful and requiring hospital emergency care, I passed those stones, and the G.P. who treated me did not suspect that those isolated incidents were connected.

After I was married a year or so, I had episodes with larger stones which required hospitalization and one cystoscope. Fortunately, I was under the care of an excellent urologist, and he had laboratory analysis done on a stone, told me I had cystinuria and sent me to a nephrologist.

At this point there occurred an almost fatal mistake on my part, and both the urologist and nephrologist. What happened? In retrospect, I tend to think it was simply a profound lack of open communication, almost a misunderstanding, complicated by my own emotional frustrations, fears, worries which were never addressed at all. The urologist was a talkative, demonstrative individual; he would speak at length about the urological aspect, even showing me small-scale models of the kidneys, etc. I’d become accustomed to his articulate, engaging manner and expected the same treatment by the nephrologist.

But, as I eventually learned, these two specialists were utterly different in their personalities, their medical approach, their go on and on with the medical saga, but I think every cystinuric knows all the various details about major kidney surgeries, the other procedures that helped me survive. It was a long, difficult two years of endless rounds to doctors’ offices, medical labs and occasional hospitalizations. I’d love to tell you I was brave, but I was not; I was a thoroughly depressed, miserable woman.

If I could change one thing about that ordeal, I wish I’d had professional counseling — not only for my diagnosis of cystinuria, but also for other troubling emotional difficulties that took me many years to resolve. I never met another cystinuric during that time, but the few people I happened upon with kidney stones, either while in the hospital or while in a doctor’s office, were suffering so much that it seemed impossible to discuss our medical problems.

Perhaps in an effort to correct his earlier casual attitude about my condition, the nephrologist now gave dire, scary warnings and instructed me to take penicillamine. And I didn’t question him; I did what I was told. Frankly, I didn’t see I had a choice, and though I was depressed, I did want to live. When I broached the subject of having children, there really was no need for the doctor to say anything: the look on his face said it all. It was not a primary concern for me, therefore I willingly decided against ever having children…something I’m sure many women might not so readily accept.

I attribute my survival now to the penicillamine, which I took for fifteen years; but when side effects occurred, I had to stop it. Looking back, I personally feel that the starvation diet along with overwhelming stress brought about my acute attack when both kidneys were blocked. Prior to that, the early diagnosis apparently gave no indication of cystine levels being high enough to cause such a devastating consequence.

I’d love to end this by saying I always take care of myself, always drink enough water, eat right and see a specialist regularly. But the truth is, while I do much better now at taking care of myself, I am far from the perfect patient; I am always looking for alternative treatments, hoping that a genetic breakthrough will solve the riddle of cystinuria, somehow give us all better prospects for treatment/prevention in the future.

The good news is, though, I no longer DENY being a cystinuric — because I learned the hard way it is a costly mistake to do so.