Calciphylaxis, or something oddly similar

[kennube]

Hi --

I don't have ESRD, but my sister-in-law does.  Quick history: she's 32; ESRD since she was around 15; Type 1 diabetic since she was a toddler.  Two years of hemodialysis; then a transplant from her brother which lasted 3 years; 10 or 11 more years of dialysis; and another transplant about a year ago (or maybe two years).

This past summer she developed a strange and very painful bump on her leg, which was diagnosed as "maybe a bite from a brown recluse spider, or maybe something else".  After months of treatment with no improvement, and with the new addition of a matching lesion on her other leg, some of her doctors are convinced that it is calciphylaxis.

In many ways it does sound like it, including the appearance of the lesions and the excruciating pain.  She fits many of the criteria often associated with calciphylaxis: female, white, diabetic, ESRD with transplant.  I've seen "obesity" in the list of common factors as well, but she is not -- she's pretty tiny.  But the thing that has everyone baffled is that normally, calciphylaxis is preceded by very high calcium, phosphorus, and parathyroid levels.   Hers are just the opposite: her calcium and phosphorus levels are extremely low, and she had a parathyroidectomy several years ago.

They've decided to treat it as calciphylaxis, because they can't think of what else it could be.  But it seems that the only treatment is wound care -- and they've told her it could take three years for the wound to heal.  They've reduced some of her immunosuppressant meds to lower the risk of infection, since right now an infection in that area is much riskier than the possibility of rejection.

From everything she has read, the mortality rate is 60 to 80%.  She's looking at the positive side of that, though, saying that that means that 20 to 40% of patients survive it -- and she is wondering where those people are, and how she can get in touch with them to find out what they did to recover from it.

While I was doing Google searches on the subject, I saw a post on this forum written by Alene, about Rolando, who was fighting calciphylaxis in 2007.  And now that I've joined here and have done another search, it appears that he did manage to beat it (although at the cost of a leg).

So I'm posting here with the hope of getting any kind of information I can about how my sister-in-law can get herself into that group of calciphylaxis survivors.  Any help is very much appreciated!  Thank you.

[thegrammalady]

another thing to remember is diagnosis is nothing more than an "educated" game of russian roulette, the doctor's could be oh so wrong. i don't have any answers for you but i'm sure aharris2 (alene and rolando) will have something helpufl to say, they are wonderful people  and this board is the best place to find answers. best of luck to your sister-in-law and keep us informed on what happens.

[nursewratchet]

Sounds impossible to be calciphylaxis, given the labs.  Even if it is, I've never seen anyone die from it.  After LONG bouts of very high Phos, have seen amputated limbs, blindness, etc.  It can also show up on the heart muscle, as well as on the eyes.  But that is with labs al elevated.  Has she had her medications chaged, and the dialysate bath changed.  If her phosphorous is low, she needs to take no binders at all, and should probably be taking a Calcium supplement since that is low as well.  The low Calcium is much more worrisome than the calciphylaxis, if that is truly what it is. 

[Meinuk]

We lost Susie (goofynina) last year to complications from calciphylaxis.  http://ihatedialysis.com/forum/index.php?topic=1965.0

It was a tragic loss, and the deposits grew and grew, until finally they were so advanced that the disease won.

Your SIL's team is doing the right thing by treating aggressively.  Labs are one indicator, buy if she is presenting with lesions, the whole of the patient needs to be treated.

[paris]

Susie's seemed to progress so quickly. I am glad they are treating it agressively.  Labs don't always tell the whole strory.  Susie was in a lot of pain also.  I hope things get better for your SIL.   I am sure Alene would be glad to answer any questions you may have. She is an amazing woman and knows more about kidney failure and complications than most doctors.  She may be a great source of support for you.   

[aharris2]

1. Susie did not have calciphylaxis. She had calcium deposits - very common in the ESRD population. Calciphylaxis is RARE.
2. Nurse Wratchet, if you have never seen anyone die from calciphylaxis then you have most likely never seen anyone with calciphylaxis. As I said, it is extremely rare. Many medical professionals have no idea what it is. Rolando's nephrologist had only seen it one time prior to Rolando's case. He has encountered it since, but Rolando is the only survivor. The cause is still unknown! Labs do not give a clear idea of who will get it and who won't.

Kennube, I am sorry you found us this way. Don't despair though, calciphylaxis can be beat. First, you said that the mortality rate is 60 to 80%. It is not a mortality rate, it is a morbidity rate. Calciphylaxis does not kill you. Infection does. The morbidity rate used to be 90%. So things are looking up.

The way to survive is:
1. Prevent infection.
2. Stop the formation of additional lesions (more on this later).
3. Prevent infection.
4. Encourage rapid healing.
5. PREVENT INFECTION!!!

The wounds will heal in their own good time. Clean them with plain saline and keep them covered. Watch like a hawk for signs of infection. Any such sign is an emergency - don't wait, go to the emergency room! The doctors didn't take the strange little wound near Rolando's toe seriously just before Independence Day in 2004.Two weeks later he lost most of his leg to it.

The lesions will eventually be covered by eschar - dead black leathery skin. Most of the doctors wanted to remove the eschar. The wound care center did not - called it a biological bandage which protected the wounds. We also kept these covered. Eventually they broke away along the edges - due to new skin pushing the edges out. At this point we started using gentamycin ointment and packed the edges. Four years later, the last of the lesions healed.

Kennube, I could write at length but I won't. Please feel free to pm us and I will share all that we know and all that we experienced. How large are your sister-in-law's lesions? The greater the surface area, the greater the risk.

Other comments:

A. While lesion formation is ongoing, there is a treatment: Sodium Thiosulfate. It was not available in 2003, when Rolando's bout began, but I understand that it was standard treatment in Britain a year ago.
B. Calciphylaxis, though still rare, is becoming more common. It has been suggested that it may be because of the use of Vitamin D analogs in the dialysis population. Rolando had just been started on Zemplar when the pain which precedes the lesions began to occur. Your sister-in-law is post transplant so I am not up on whether or not such drugs are used, but if so, get her off of the Vitamin D analogs and if binders are still used, get her off of any which might contain calcium (such as Phoslo). Again, though, the cause of calciphylaxis is unknown.

A and B may help to stop the formation of additional lesions.

C. Consider hyperbaric oxygen for wound healing. Do the research - I don't remember a positive correlation between hyperbaric oxygen and wound healing, but it can't hurt. (My strongly held opinion is that time is necessary for healing these wounds because time allows the body to revascularize the areas.)
D. No one would provide us with sufficient wound care supplies. We always had to supplement ourselves. Surf the net. There are sources of bulk supplies - bandage rolls, gauze sponges, sterile saline, etc.
E. Ease her pain! Calciphylaxis is immensely painful. It is tissue death - the same pain as a heart attack. Make sure she has a doctor who is as aggressive as necessary with the pain meds.

[nursewratchet]

Good info!  You're probably right, I have seen calcium deposits, and seen the pictures of calciphlyiaxis.  the Dr.s always threaten it, in response to lab values.  Thanks for the info...

[thegrammalady]

i knew aharris2 would have answerws

[kennube]

The lesions will eventually be covered by eschar - dead black leathery skin. Most of the doctors wanted to remove the eschar. The wound care center did not - called it a biological bandage which protected the wounds. We also kept these covered. Eventually they broke away along the edges - due to new skin pushing the edges out. At this point we started using gentamycin ointment and packed the edges. Four years later, the last of the lesions healed.

The second, newer lesion does have that leathery covering -- it has not been removed, for the reason you mention (protecting the wound).  I have not heard her mention gentamycin ointment, but I'll pass that info along.

Kennube, I could write at length but I won't. Please feel free to pm us and I will share all that we know and all that we experienced. How large are your sister-in-law's lesions? The greater the surface area, the greater the risk.

The first one is more like a big hole now, as they cut the original lesion out and then have since cut away more necrotic tissue.  Maybe 2 or 3 inches across, going by her mother's description of it.  The second one is smaller -- somewhere between the size of a quarter and a silver dollar, depending on who is describing it.

Other comments:

A. While lesion formation is ongoing, there is a treatment: Sodium Thiosulfate. It was not available in 2003, when Rolando's bout began, but I understand that it was standard treatment in Britain a year ago.

Another little tidbit of hope -- thanks!

B. Calciphylaxis, though still rare, is becoming more common. It has been suggested that it may be because of the use of Vitamin D analogs in the dialysis population. Rolando had just been started on Zemplar when the pain which precedes the lesions began to occur. Your sister-in-law is post transplant so I am not up on whether or not such drugs are used, but if so, get her off of the Vitamin D analogs and if binders are still used, get her off of any which might contain calcium (such as Phoslo).

She had been on Phoslo for part of the time she was on dialysis, and then Renagel.  I don't think she has been on either one since her transplant.  As for the vitamin D -- I know that she used to take some prescription vitamins; I'm not sure if she still does.

Again, though, the cause of calciphylaxis is unknown.

And that is the most maddening part -- the not knowing!

C. Consider hyperbaric oxygen for wound healing. Do the research - I don't remember a positive correlation between hyperbaric oxygen and wound healing, but it can't hurt. (My strongly held opinion is that time is necessary for healing these wounds because time allows the body to revascularize the areas.)

Interesting -- I'll pass this along to her too.

D. No one would provide us with sufficient wound care supplies. We always had to supplement ourselves. Surf the net. There are sources of bulk supplies - bandage rolls, gauze sponges, sterile saline, etc.

She is currently going in to  -- somewhere -- hospital, clinic, wound-care center? I don't know -- three times a week for them to change the bandages.  I would imagine that at some point they'll start having her do that at home, at which time supplies would become a big issue.  She spends so much time battling insurance companies and their bizarre and contradictory policies!

E. Ease her pain! Calciphylaxis is immensely painful. It is tissue death - the same pain as a heart attack. Make sure she has a doctor who is as aggressive as necessary with the pain meds.

She's on some pretty strong pain meds, but there are quite a few days when it's just not enough...


Alene, thank you so much for all of this information.  Finding even one person who has gone through this rare condition and survived it is tremendously helpful and a huge cause for hope.  I am going to encourage her to sign up on this site and to contact you directly -- she could certainly answer your questions much better than I can, and I think that you and Rolando could provide her with a lot of useful information, not to mention being able to understand exactly what she is going through better than I ever could.  Again, thank you so so much for your helpful (and hopeful) response.

[paris]

Alene really knows so much and always has a good answer.  She needs her medical degree!   Thanks Alene for informing all of us.   

[boxman55]

. Consider hyperbaric oxygen for wound healing. Do the research - I don't remember a positive correlation between hyperbaric oxygen and wound healing, but it can't hurt. (My strongly held opinion is that time is necessary for healing these wounds because time allows the body to revascularize the areas.)                                                                                                                                                                   

I had hyperbaric treatment for 30 days straight. If you have good blood flow to the and at the wound area, it is very effective. If you don't, then it is a waste of time and energy. There is also a machine called a Wound Vac that preforms the same type of treatment directly over the wound and it is portable..Boxman

[etoile26]

I am new to this site, but was encouraged to join because "Kennube" is my sister-in-law.  I am the aforementioned patient, struggling with the diagnosis of calciphylaxis.  As I typed the previous sentence, I thought for a second how easily I spelled and typed out that weird word... calciphylaxis.  It's almost as if my fingers know it so well now, it doesn't seem odd to me at all, even if my spell check thinks otherwise.  It is late tonight, and I have plans with some of my family very early in the morning but I was so eager to finally get the email verifying a screen name that worked, I had to post at least something before heading to bed.

Many things have changed since Kennube's initial post.  I was finally given the "official" diagnosis of calciphylaxis in late December when the second wound showed up on my left leg, significantly parallel to the first, classic sign of calciphylaxis.  Doctors stopped guessing and started calling it a name.  It didn't necessarily help with treatment, but it definitely stopped some of the ridiculous testing the tried to encourage me to undergo.  I have stayed with the same doctor that began treating it back when it was the mysterious wound, and he and I have both learned a lot about the way my body heals, what works, and what to avoid (the WOUND VAC, for CERTAIN!!!).  Many of you have mentioned hyperbaric treatments.  The center I visit is actually called Wound and Hyperbaric Treatment Center.  However, hyperbaric treatments are only protocol for wounds meeting very specific standards, most commonly "diabetic wounds on the lower legs that have not healed after significant attempts have been made using a variety of bandages, prescription, and lifestyle adaptations."  As fantastic as the possibility of hyperbaric treatments seemed to hold, with such little effort on my part (coming from a former hemo patient, on for 3 1/2 hour treatments 3 times per week, I think 2 hour sessions five times per week, sleeping on a bed, WITHOUT needles is NOTHING to cry over!), I was not a candidate.  Not only that, but an initial test had to be conducted, testing the bloodflow to the area.  The extent of the damage was so great, hyperbaric would have been a waste of time, since my circulation levels were so far below standard.

My doctor continued to follow my wound, and I continue to see him and his staff of very caring and supportive nurses three times per week.  In December when I was admitted for the infection, I asked to be put on provalactic Bactrim (since I had been on that immediately after the transplant, I figured that would be a safe antibiotic to choose), and my doctor agreed so quickly it was as if I had read his mind.  My transplant team also temporarily reduced my immunosuppression, keeping a close eye on my creatinine levels.  Together, these med changes helped the healing process.  I also used a special dressing called WIC cavity filler immediately over the wound, another poly-mem piece over that, and ABD pad to keep it in place, and an ACE bandage to keep it from sliding down my leg.  Now, 5 months later, the wound is back to 1/3 the biggest size, and is bleeding a LOT (which is a GOOD thing, showing circulation) and healthy pink tissue.  Two weeks ago I had (we hope to be) the final surgery on this leg to remove nearly all the necrotic tissue so as to speed along the healing process by taking away some of the necrotic tissue that this dressing had been dong, but all at once rather than the slower rate it had been going through.  About half the wound has new skin completely covering it now, and even has hair growth on it.  As a woman, I never thought I would be so happy to see hair on any part of my legs as I noticed there!

As the wound on my right leg healed, we continued to test the theory of "let it scab, dry up, and fall off."  The wound stopped spreading, so this seemed like a good idea, and seemed a lot less invasive than all the surgeries I had endured on the right.  However, no PROGRESS was being made, and suddenly my LEFT leg was more painful than the right.  About 3 weeks ago, I started complaining that something had to be done.  I was told "avoid weight/pressure on the leg."  I didn't need a doctor or nurse to tell me not to touch the painful wound.  The following week, I asked again, 'please, anything to stop this pain, it's just like when I fist got here with the right leg," and again I was told that we would be playing with fire opening the new wound.  My immunosuppressives had been returned to normal doses after a consistent increase in creatinine scared me into weekly blood draws, and calls to and from the transplant team to find the right dosing to protect the transplant.  Finally last week, when the wound was more red than usual and still no progress had been made, we weighed the risks and benefits of surgery to debride the left leg.  This would mean potential for infection once again, but it could be the only way to rid myself of the pain of the necrotic tissue constantly eating away at my leg.  I am scheduled for surgery on Wednesday the 18th to open the left leg.  With the experience my doctor has in operating on my wounds, he has decided not to remove further than the boundaries already in place by the wound, and plans to remove whatever necrotic tissue is located centrally within the wound, leaving the outside rim to heal as the right leg wound did, using the WIC dressing.  I will try to keep this site updated on my progress as to inform any patient with the threat of this immensely frustrating, PAINFUL, scary, emotional roller coaster of a disease.

To all of you here who offered support/advice/words of kindness to me, without knowing me or my sister-in-law, just on the basis of being a kidney-disease related setback, thank you for your generosity and time.  Before I became an official member, I was able to read a few postings as forwards from Kennube, and I look forward to hearing more and possibly helping others as I have been helped during these past few months.

[kitkatz]

Good luck on your leg surgery this week.

[Wallyz]

Welcome, good luck, an prayer about the surgery.

[aharris2]

Since your wound formation is still ongoing,has there been any mention of using sodium thiosulfate? Like I said in the earlier post, 5 years ago it was experimental. It is standard treatment now in Britain, and for all I know, here too. Ask about it.

Painful - more than most can imagine. I hope that your surgery relieves your pain and that your doctors are as aggressive with pain meds as you need them to be.

WIC cavity filler is something that we never used. It looks interesting and encouraging. Hopefully it is sufficient to bring you full healing.  As time goes by and your wounds heal (and the area revascularizes - yes, bleeding is a wonderful thing, gotta love that beefy red granulation tissue), you may find that you do become a candidate for hyperbaric treatment. It is not something that we used, but late in the process, a wound vac was used on one of Rolando's foot lesions and it did the job quickly. Another effective treatment also late in the process was something called an Apligraf. It is a thin layer of tissue placed into the wound that delivers growth factors to encourage skin formation.

Scary - we knew that we had just been handed a death sentence at diagnosis (we were just simply not going to go down without a fight). Etoile 26, there are survivors, it can be beat. I attribute Rolando's survival to good treatment, aggressive pain management, and his very strong faith. Another survivor writes me from time to time behind the scenes. And, it sounds like you too are on your way to beating it. It is unfortunate to see people having to suffer through calciphylaxis, but encouraging to see progress in beating it.

Patience, calciphylaxis teaches you patience. The wounds can and will heal, in time, if you can "just" stave off infection. Best wishes Etoile26.

Alene

[paddbear0000]

Etoile26, I have no experience or even knowledge of calciphylaxis, so there isn't anything I can offer except lots of hugs! 

I must add though, that as a life-long type 1 diabetic hoping for a transplant, I am now freaking out! This is very scary sounding!

[birdy]

AHarris-

I want to thank you for posting about calciphylaxis. I was worried it would be a death sentence for me now I know I may have a chance. So far I've only had one spot that has turned black about the size of a nickle. I have had to have half my stomach removed because the diagnosed it as Panniculitis. Upon pathology report is when they made it clear as to what I have. I've declined vitiamin D and Iron which seem to aggravate it and now on Renegel.

I started Sodium Thiosulfate this week but it makes my whole body burn in pain. I'll stick with it though for as long as I can. My husband has decided my life is worth more than his job so we are moving to Columbus, Ohio so I can go to the University for treatment. His family lives there so we'll stay with them until he gets a new job.

Where I live now my doctor has only had experience on one other case than mine so I don't feel confident enough in their treating me. I was told I would DIE from this and I've been very depressed ever since.

I might die from it but your post gives me hope that I can beat it.

Bless you,

Birdy...