Thirty-Seven Uninterrupted Years of Hemodialysis: A Case Report

Stephen I. Rifkin, MD, FACP, FASN

Disclosures

October 07, 2008

A patient is presented who was on uninterrupted hemodialysis for 37 years. She did extremely well for much of her time on hemodialysis. Significant issues that occurred included multiple vascular access problems, cardiac arrhythmias, osteodystrophy resulting in a fracture of her femur after light trauma, the incidental finding of a renal cell carcinoma, and the development of hepatitis C positivity. The literature of extremely long-lived patients on uninterrupted hemodialysis is reviewed, and the clinical characteristics and complications of these patients are discussed.

Chronic hemodialysis has been in existence since 1960 when Belding Scribner initiated a chronic hemodialysis program in Seattle, Washington.[1] One of Scribner's original patients, Clyde Shields, lived until 1971, and Harvey Gentry -- another of the same group -- received a renal transplant in 1968 and lived until 1987. However, in general, the patients of that era did poorly. Alwall's chronic dialysis program, started in 1960, initially had no survivors beyond 5 months. In 1966, there were 157 patients in chronic dialysis in Sweden; 53 of them died before the end of the year, and the longest survival time was 3 years.[2]

Long-term hemodialysis first became a reasonable possibility in 1966 when the arteriovenous fistula was described,[3] and there is now a small group of extremely long-term hemodialysis survivors. Unfortunately, much of the information about these individuals is either anecdotal or appears in the lay literature. A report of a patient who was on hemodialysis for 37 uninterrupted years is presented, and the literature of extremely long-lived patients on uninterrupted hemodialysis is reviewed.

The patient is Philippe Smith. She was first diagnosed with chronic renal disease, probably poststreptococcal glomerulonephritis, in 1963 when she was 18 years old. Regardless of this diagnosis, she endeavored to lead a normal life. She married her high school sweetheart, and, in 1969, became pregnant and -- against all odds and against medical advice -- continued the pregnancy and delivered a healthy boy who is now 38 years old. The following year, on November 13, 1970, she initiated chronic hemodialysis. She and her husband were trained to do home hemodialysis and then she later switched to in-center hemodialysis.

She became a schoolteacher in 1976 and rose to the position of principal. She retired in 1994. Her marriage failed after 15 years, and she later developed a relationship with another dialysis patient. She felt well enough on dialysis to refuse consideration of a renal transplant. She neither smoked nor drank alcohol.

She had a parathyroidectomy with a forearm implant in 1976. Most of her medical problems revolved around blood access. She had at least 2 fistulas and 4 grafts placed in her upper extremities. In 1996, she had Tesio (Medcomp, Harleysville, Pennsylvania) catheters placed in her left upper central venous system, and these, remarkably, functioned until the time of her death in February 2008.

She was noted to have acquired cystic disease of her kidneys in 1995, and in 2000 she underwent bilateral nephrectomies because of an increasing mass in her right kidney. She was found to have a less than 1 cm renal clear cell carcinoma, which was well differentiated, and she had no further difficulties from it. She also had a cholecystectomy for cholelithiasis at the same time.

She became hepatitis C antibody-positive, but was noted to be hepatitis C antigen-negative by polymerase chain reaction (PCR) in 1997. She had an elevated serum cholesterol and had a mild peripheral neuropathy involving her feet. In 2003 she had bowel obstruction requiring surgery with partial small bowel resection. She had a right carpal tunnel release in 2004.

She was noted to have a supraventricular tachycardia in 2002, and over the ensuing 6 years she had intermittent problems with paroxysmal atrial fibrillation, which was treated with cardioversion and medication.

In January 2008 she slipped and twisted her body while getting into a car and fractured her left femoral head. Prior to this incident, she was community ambulatory with a cane. A cemented hemiarthroplasty was done. Postoperative rehabilitation was difficult, and she then developed another bowel obstruction. At that point, regardless of her love for life, she simply had given all of the fight that she had had and could not bear the thought of having to deal with bowel surgery again. She thus refused surgery and decided to terminate dialysis. She died on February 15, 2008 after 37 years on hemodialysis.

I believe that it is important from a number of perspectives to be aware of these long-term survivors. It is important from a historical perspective. It is important from the perspective of hope to appreciate that at least a small number of patients could live through an era of relatively primitive equipment and a less sophisticated understanding of the dialysis process to survive and function for an extended period of time. Patients on hemodialysis who, for one reason or another, are unable to receive a transplant will certainly take comfort from this knowledge. It is also important from a medical perspective to appreciate what nephrology has been able to accomplish, and to be able to appreciate the problems that occur as a result of being on extremely long-term hemodialysis. It is also important to appreciate that a number of the problems that these patients had to deal with can be much more effectively treated in the modern dialysis era.

Because there is no registry of extremely long-lived hemodialysis patients, information on this group is scattered, found mostly in the lay press and the Internet, with some data in the medical literature. The Table describes a small group of European and American patients who have been on hemodialysis continuously without any significant dialysis-free period for at least 33 years. This table is certainly not complete, but it does provide what is probably a significant representative sample of this patient population.

Piccoli[4] described a group of 56 patients who had been on uninterrupted dialysis for at least 20 years. None of the patients were diabetic; 82.1% had severe vascular disease; 46.4% had severe cardiac disease; 89.3% had severe bone disease; and 48.2% of the patients had had a parathyroidectomy. One patient had renal cell carcinoma. Seventy-five percent were hepatitis C virus antibody-positive, but only 3.6% had severe liver disease. Of interest, only 7.4% were hypertensive. An observational study by Owen and colleagues[5] of 17 patients on dialysis for more than 10 years also demonstrated a lack of diabetes and a relatively low incidence of hypertension at the initiation of dialysis. Golder and colleagues[6] identified 6 patients who had been on dialysis for an average of 16.7 years. All 6 had symptoms and signs of amyloidosis. Bazzi and colleagues described 24 patients on hemodialysis for 16-23 years.[7] None were diabetic, and blood pressure was elevated in only 6 patients. Bone histology was abnormal in 19 of 20 bone biopsies, and signs of dialysis-related amyloidosis were frequent and disabling. Rehabilitation was described as fair. The number of vascular access procedures varied from only 1 to more than 10.

Otsubo and colleagues[8] reported on the characteristics of 16 patients who were on hemodialysis for more than 30 years at Sangenjaya Hospital, Tokyo, Japan. None of the extremely long-term survivors were diabetic; 25% were hepatitis C virus-positive; and hypertension was less common in the long-lived patients. Chronic complications were said to be the focus of their next study. When evaluating the long-lived Japanese patient population, one needs to keep in mind that transplantation is rare in Japan and that, for uncertain reasons, the Japanese hemodialysis death rate is considerably lower than that of Western Europe and the United States.[9]

In addition, 2 patients from Alwall's program, each with greater than 35 years on hemodialysis, but with dialysis-free periods of 15-18 months, have been described.[10] The first patient started hemodialysis in 1968 and has been on hemodialysis since, with the exception of a dialysis-free period of 18 months after 2 renal transplants. She has hepatitis B and C and suffers from significant joint problems from dialysis-related amyloidosis. She underwent parathyroidectomy in 1977. She has had multiple surgeries for carpal tunnel syndrome. Her blood pressure had been difficult to control, but is now acceptable without treatment. The second patient was started on hemodialysis in 1971 and has only had a 15-month dialysis-free interval since then. She has hepatitis B, had aluminum intoxication diagnosed 18 years after initiation of hemodialysis, had a myocardial infarction at year 31, and has profound osteopenic bone disease regardless of undergoing a parathyroidectomy in 1980. Neither patient is diabetic, and neither patient has had substantial access problems, with the second patient having had the same arteriovenous fistula since 1971.

On review of the information on these patients, a few details stand out. First, there are no diabetics. Whether this is because the selection process many years ago was biased against diabetics and/or whether diabetics generally do not fare as well on hemodialysis is not clear. Second, severe hypertension is not as common in this group. Third, having a long-lived dialysis access is helpful, although not mandatory. Fourth, hepatitis B and C are very common in this group, presumably as a result of the many blood transfusions that they received prior to the use of erythropoietin. Fifth, bone disease in one form or another is very common and has caused significant disability. This has occurred despite treatment with parathyroidectomy in many of the patients. Sixth, dialysis-related amyloidosis has also caused significant disability.

These patients were all initiated on dialysis at a time when aluminum hydroxide (a cause of aluminum bone disease) was the preferred phosphate binder, before vitamin D analogs were in use, before cinacalcet was available, before the widespread use of statins, and before more efficient and effective dialyzers were available. It would thus appear that many of this group's problems could be expected to be either relieved or obviated in the era of modern dialysis.


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