Calciphlyaxis Diagnosis

[Trena]

I have been diagnosed with a condition not many dialysis patients even know about. Calciphlyaxis. It is a disease that is very difficult to treat. When your phosporous and calcium levels are out of balance, calcium builds up in your body and forms deposits in the soft tissues and smaller blood vessels. This leads to loss of blood flow to the tissues and the tissue dies causing sores that can easily become infected and are very slow to heal. There is not much that can be done to treat Calciphlyaxis other than keeping your phosphorus levels down, protecting your self from injury, and using a meication called sodium thiosulfate (which is not paid for by Medicare/Medicaid). My doctor is trying to find out if there is any way to get the med paid for through private sources. I really hope they can because it will slow the progression of the disease and help keep deposits from forming. There is no cure except to treat the symptoms. Calciphlyaxis has an extremly high mortality rate, above 80% mostly due to infections.

[Sax-O-Trix]

Wow.  Sorry to hear this diagnosis. I hope you can get the meds you need and will be able to slow the progression of the disease.  How expensive are the meds?  Can you appeal to Medicaid/Medicare to reconsider paying for the meds?

[greg10]

Welcome to the forum.  I am sorry to hear about your condition.  Sodium thiosulfate has been shown to be effective in at least one case of calciphylaxis (calcific uremic arteriolopathy), presumably by increasing the solubility of deposited calcium.  Sodium thiosulfate should not be an expensive drug because it is a commonly available chemical compound, but you could never know how much of a mark up these drug companies can make out of a common compound.  Medicare/Medicaid probably won't pay for sodium thiosulfate because there is probably insufficient studies on its use with calciphylaxis.  The following is a case study published in 2005 of what is apparently an effective treatment of calciphylaxis with sodium thiosulfate in combination with hemofiltration with low or zero calcium replacement fluid.

Rapid resolution of calciphylaxis with intravenous sodium thiosulfate and continuous venovenous haemofiltration using low calcium replacement fluid: case report
Correspondence and offprint requests to: Edward A. Ross, MD, Division of Nephrology, Hypertension and Transplantation, University of Florida, Box 100224, Gainesville, FL 32610-0224, USA. Email: rossea@medicine.ufl.edu
Received January 10, 2005.
Accepted March 15, 2005.

calcific uraemic arteriolopathy calciphylaxis thiosulfate

The pathogenesis of calciphylaxis (calcific uremic arteriolopathy) remains to be fully elucidated, which makes treatment of this often fatal disease quite challenging. While controversial, vascular calcium deposition may play a secondary, if not primary, role in ongoing tissue ischemia. In cases that would not benefit from early parathyroidectomy, alternative strategies have been proposed to control calcium and phosphate homeostasis. The use of non-calcium based phosphate binders and intermittent haemodialysis with low calcium dialysate [1,2] has been of benefit in some but not all patients. One of the most recently suggested therapies is the use of sodium thiosulfate to increase the solubility of calcium deposits [3]. With its reported success in treating both nephrolithiasis [3] and tumoral calcinosis [4,5], Cicone et al. [6] described its efficacy when given after haemodialysis treatments in a single case of calciphylaxis. We hypothesized that the benefits of solubilizing calcific deposits in patients with ESRD would be severely limited by calcium clearance being dependent on intermittent dialysis sessions. To optimize removal, we devised a regimen combining intravenous sodium thiosulfate treatment with continuous venovenous haemofiltration (CVVH): a constant state of mild hypocalcaemia was maintained by a regimen involving zero-calcium replacement fluid, regional citrate anticoagulation and protocol-driven calcium repletion. We report here a case of progressive biopsy-proven calciphylaxis that responded rapidly to this new approach and which had an excellent outcome.

[AguynamedKim]

Im sorry, Trena. I hope you can get the medicine and that its effective for you. Thank you, greg10, for providing some more insight that I hope helps Trena.

[greg10]

There are no current clinical trials of thiosulfate on calciphylaxis, but there have been some studies of thiosulfates on HD patients.  It seems to be a well tolerated drug and seems to be effective for calciphylaxis in very limited case studies, however there doesn't appear to be any clinical trials on it so Medicare/Medicaid will be unlikely to pay for its off label use (thiosulfates is normally prescribed only for cyanide poisoning).

http://www.clinicaltrials.gov/ct2/show/NCT00568399?term=thiosulfate&rank=1

Patients who are at high risk for having coronary calcification (history of MI, ischemic heart disease, peripheral or carotid artery disease) will be selected to undergo testing. We will recruit 60 HD patients receiving treatment in our units to undergo MDCT scanning along with non-invasive testing of PWV and CIMT. Assuming that 60% will have a CAC score of ≥50, 36 patients will be treated with sodium thiosulfate. We will administer 25% sodium thiosulfate solution (American Reagent Laboratories, Shirley, NY) at a dose of 12.5-25g/1.73m2 per over 15-30 minutes after each hemodialysis session for a total of 60 treatments (5 months). Assuming a 35% attrition rate, 23 patients will complete the entire protocol and undergo a repeat study of the initial battery of tests.

Rationale for treatment with sodium thiosulfate: Sodium thiosulfate, used as an antidote for cyanide poisoning for more than a century, is also an anti-oxidant, and binds with calcium to form a highly soluble calcium thiosulfate salt. The solubility of calcium thiosulfate salt is 250-100,000 fold higher than calcium oxalate or calcium phosphate salt. It has been used to treat recurrent calcium kidney stones and tumoral calcinosis (ectopic calcification usually around joints). It has also been used successfully in treating calcific uremic arteriopathy, a disease of small artery and soft tissue calcification, in several studies of dialysis patients and in our own experience of 5 patients. By 2 months there is radiological evidence of reduction in soft tissue calcification. Unpublished data also have demonstrated regression of established aortic calcification in uremic rats.

Sodium thiosulfate is a FDA approved medication for the treatment of cyanide poisoning. It is classified by the FDA as "generally recognized as safe". There are no known contraindications. The only side effects reported during intravenous (IV) administration in ESRD patients are nausea, vomiting and hyperosmia during the administration, which can be alleviated by pre-administration of anti-emetic medications. Sodium thiosulfate is slowly given through the dialysis venous line toward the end of HD treatments. The selected dose for this pilot study is the same as that used for the treatment of calcific uremic arteriopathy.

[aharris2]

Trena,

My brother is a calciphylaxis survivor so remain hopeful because it can be beat.

He had that slice biopsy adjacent to a lesion done that is necessary for a definitive diagnosis of calciphylaxis. It hit him in 2003 and, sorry to say, it took 4 years to fully heal all of the lesions. At that time the morbidity rate was 90%. Sodium Thiosulfate was barely experimental back then so we were not able to use it. We bought it (it was very inexpensive) but no one would administer it.

Be prepared for the pain. Calciphylaxis lesions are excruciating. Make sure you have a doc on board that is willing to aggressively treat the pain.

As you said, it is infection that claims most all victims of calciphylaxis. It is important to be in the care of a wound care specialist. Keep the wounds clean and covered and head to the emergency room at the smallest sign of trouble - a color change, an odd smell, a slight fever.

You will find that not only have few dialysis patients heard of calciphylaxis, there are very few medical professions that know of it.

Best of luck Trena, we are here for you. Feel free to PM me, my brother and I will help in any way we can.