The Medicine Cabinet

Notes on Hyperparathyroidism and ALS

It is most important to realize that I am not a doctor, and do not have a medical degree. The following page is based on what I have read and been told by my doctors. I believe all information included in this page to be true and correct. Should you find any errors, please bring them to my attention.

Since Hyperparathyroidism and ALS can have similar symptoms, and these problems have struck very close to home, I have written the following based on what I have learned.

My Problem

I have been diagnosed and treated for hyperparathyroidism. I have passed over 188 kidney stones since 12-24-86. My condition was complicated due to the fact that I was not properly diagnosed for many years until I had changed doctors. My new doctor was able, after a few tests, to determine that I had kidney stones because I suffered from hyperparathyroidism. In April of '92, I had a single parathyroid adenoma removed, and responded well to the treatment. Blood calcium, phosphorus and parathormone levels returned to normal. In December of '94, blood calcium levels again went "through the roof", and tests later confirmed a similar problem. Surgery in September of '95 to remove 2 more parathyroid adenomas has so far looked positive. A biopsy on my one remaining parathyroid gland showed normal results, however, I still have many more kidney stones that have formed from years of having this condition.

After having a 15mm (.590") stone get stuck in my ureter, I had Extracorporeal Shock Wave Lithotripsy (ESWL) twice. This is the procedure in the tank of water, where they direct sound wave at the stones, in order to break them up. Both treatments were unsuccessful. Later, my urologist performed a Cystoscopy and Hydraulic Lithotripsy. A much more invasive procedure. A scope was inserted up the urethra, to the bladder, a second scope was inserted through the first scope, and up from the bladder to get to the stone, which was in the ureter. A small piston in the second scope was used to literally hammer at the stone, in order to break it up. My stone wasn't giving up so easy, and the surgeon worked for over six hours on a procedure that was originally estimated to last just a couple of hours. The fragments were removed after the stone eventually broke, so I do not include these in my current count of the number of stones I have passed. On average, I have passed more than one stone a month since this has started in 1986. I have passed as many as five stones at one time, and as many as ten in one day. The largest stone that I have passed was just over 7mm (.275"). Here are a few photos of one that I caught in one of those little strainers they give stone sufferers.

kidney stone

The hypercalcemia that this condition causes has led to other problems including calcium plaque in my heart and arteries, joint pain, partial kidney dysfunction, loss of muscle tone, and slow nerve to muscle reactions.

After about two years, of less pain, and less passing of stones, things would change. In the fall of 2002, my kidneys seemed to be losing a lot of function. Protein levels in my urine were over 10 times normal. More tests were ordered. In November 2002, I was diagnosed with blockages in both ureters (which also explained why I hadn't passed a stone in a while). There were two stones in the right (one over 1/2" and one smaller), and 4 in the left (one about 5/8" and three smaller). There was also still one large stone (1/2") in the left kidney, as well as smaller stones in both kidneys. The left kidney was grossly distorted, and barely functioning. Both kidney have severe Hydronephrosis (urine not draining from them properly) and thinning of the renal cortex (the working part of the kidneys). Several doctors, including my Nephrologist (kidney doctor) couldn't imagine that I was not in a whole lot of pain. I had been experiencing my normal dull pain, but not the sharp pains of kidney stones. Pain, would have been the heads up signal, that I had a problem, but it wasn't there. This caused both me and my doctors to miss the real cause of the problem, at first. It was determined that since my problems have been so chronic, that my body was not responding to kidney pain, the was it used to (or should). Now, that we knew the culprit of the problem, it was time to do something about it. In Dec. 2002, I had right ureter Cystoscopy again. This was to preserve my better functioning kidney from deteriorating any further, and also to remove the stones if possible. The plan was to attempt to remove the stones using a new laser, and install a stent (drain tube) between the kidney and bladder. That would keep the urine flowing temporarily, even if he was unable to remove the stones. Surgery was successful, and both stones were busted up, and removed. The stent was placed, so that any scar tissue would not block the ureter while I was healing, and it was time to start working on my left side.

The surgeon told me, that if I had a perfectly functioning right kidney, that he would have probably recommended just removing my left kidney, but since I had damage to both kidneys, he wanted to attempt to preserve as much function in both, as he could. The plan was to do a Percutaneous Kidney Surgery. This involves going in through the back, and into the kidney. He would work in the kidney and down the ureter from there. Percutaneous Kidney Surgery was done in January 2003. A guide is placed through the back, into the kidney under x-ray. This is dilated during surgery, to allow access to the kidney. I talked to the technicians the whole time they were placing the guide, which surprised them, as the drugs they had given me, should have had me quite knocked out. After the guide was placed, there was a two hour wait, for surgery. The surgeon was able to go in, and grab the large stone in the left kidney quite easily. He had no plan to attack the smaller stones in there, as they were embedded deep in the kidney tissue. The then worked down the ureter, to the first stone (the large one). This obviously had been there for a while, and was really adhered to the ureter. He was successful at breaking it up, and removing most of the pieces. Further down, closer to the bladder, was a cluster of three stones. He also was able to remove these, and placed a stent between the left kidney and the bladder. The stents were removed at a later date, when I'd healed. He also removed some small residual stone pieces with the Cystoscope when he did that.

I still have stones in both kidneys....but hopefully they will either stay there, or pass with ease. I don't want any more blockages, that's for sure.

If you are, or think you are, in a similar situation, please feel free to write to me. Sometimes it helps to talk to somebody else who's been there, and understands what you're going through.

Questions, comments, or just want to chat? My E-Mail address is tabco@comcast.net.

My Family's Problem

My Grandmother, her brother and her son (my uncle), (all deceased) have been diagnosed with ALS. It has been determined that we have one of the 5 to 10 percent of cases that run in families. Since my closest relative currently diagnosed to have this problem is a grandparent, I run a 1 in 4 chance of having inherited the gene that causes this disorder.

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Hyperparathyroidism

Hyperparathyroidism is a condition where an abnormality of/or tumor on, one or more parathyroid glands, causes the glands to produce too much parathormone (parathyroid hormone). This hormone regulates the way our bodies handle calcium and phosphorus. Hyperparathyroidism leads to a rise in blood calcium and a fall in phosphorus. Calcium is removed from bones, resulting in increased fragility, muscular weakness, reduced muscular tone, and general neuromuscular hypoexcitability (slow nerve to muscle reactions). Generalized osteitis fibrosa and osteitis fibrosa cystica (decalcification and softening of bone, and nephrolithiasis (kidney stones), cysts form and tumors may develop) are also associated with this condition.

This condition causes hypercalcemia, where the body leaches calcium from the bones and puts it in the bloodstream, where it coats the arteries, heart, joints and kidneys. Left untreated (or undiagnosed in my case) the calcium is able to do a lot of damage to the heart, arteries, kidneys, joints, and make LOTS of stones (...not only kidney stones. Stones of the salivary glands are also common for people with this condition).

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ALS

Amyotrophic Lateral Sclerosis (ALS)

Motor Neurone Disease (MND)

Lou Gehrig's Disease

maladie de Charcot

ALS is a progressive disease of adults caused by degeneration of upper and lower motor neurons (skeletal muscle nerve cells), resulting in muscular weakness and the progressive wasting of muscles that have lost their nerve supply.

The early symptoms of ALS include slight muscle weakness, clumsy hand movements, and/or difficulty performing tasks that require delicate movements of the fingers and/or hands. Muscular weakness in the legs may cause tripping and falling. People with ALS may have difficulty swallowing, and speech may be slowed. Other symptoms of this disorder include progressive weakness of the lips and impairment and/or loss of function of the tongue, mouth, and/or voice box. Leg cramps may occur during the night, most frequently in the calf and/or thigh muscles. ALS may progress quickly or slowly, and gradually additional muscles become involved.

Other symptoms of ALS may include the uncontrolled twitching of muscles, stiffness in the legs and/or coughing. People with this disorder may also have exaggerated deep muscle reflexes. Severe weight loss occurs in approximately 5 percent of cases. As the ability to move becomes progressively impaired, people with this disease are at increased risk for respiratory failure and abnormally low levels of oxygen in the blood. People with ALS are also at increased risk for acute inflammation of the lungs, caused by the inhalation of food or stomach contents. An overall decrease in the ability to move may also result in inadequate nutrition.

There is no known cause; however 5 to 10 percent of cases are inherited. Treatment is symptomatic, and involves multi-disciplinary efforts of physicians, physical, occupational, speech, respiratory and nutritional therapists. The mean survival in ALS is 3-4 years, but 20% of patients live longer that 5 years and 10% live more than 10 years.

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