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Porphyria Cutanea Tarda (PCT): Any experiences?
My sister is in End Stage Renal Failure and has developed PCT. She is is so much pain with the blisters and it has become completely debilitating. For the past 5 weeks she had undergone aphresis to cleanse the iron from her plasma. Prior to the aphesis, her ferritin level was at Ferritin 1596, 56% sat, with Total porphyrins: 251 mcg/dL, and specifically Plasma--Uroporphyrin: 118.3 mcg/dL. Her most recent test results show her totally porphyrins at 212. Does anyone know at what level the porphyrins have to get to before the blisters cease forming? Does anyone have any suggestions on pain management and treatment of the blisters...especially on the ones that have formed under her fingernails?
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I have only been diagnosed with PCT for a year I was advised to use SPF 100 sunscreen always. And I am to reapply it every two hours. The only skin I have exposed are my hands and face including my ears which have been affected by the sun. I have had four phlebotomies so far which have brought my ferritin level down. I am scheduled for more. I'm trying to adjust to a different lifestyle
which is difficult at times but I hope to adapt and remain happy.
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2 Reactions@chriseliz, I moved your post to this existing discussion:
- Porphyria Cutanea Tarda (PCT): https://connect.mayoclinic.org/discussion/porphyria-cutaneous-tarda/
I did this so you can read previous posts and connect with members like @donnasa @jfmaddog @ouchelp220 @griffin2 and others who have experience with porphryica cutanea tarda.
Chriseliz, what treatment helps you manage PCT?
Is anyone dealing with porphyria cutanea tarda?
Yes, she is on Plaqunil and the blood letting has been helping get the iron count down. She was over 1000 on her iron count and after 3 months, was down to 400. The dialysis center no longer will allow it at their site. She made arrangements to have it done at a cancer center and just today has decided to give up on that. The pain has won.
She was referred to the Mayo Clinic and was told they wouldn't take her as a patient. We had plans to go to the University of Utah but has decided she has had enough.
She is working with the wound clinic, a dermatologist and infectious disease. The first picture is of her hands last August 2022. The eye was from February 2023. She has 2 fingers with bone infection and want to amputate. She said, NO.
What treatment are they giving her has she tried hydroxychloroquine?
They are trying through my dialysis treatments. They are throwing away part of the blood at the end. The numbers have gone down, but so small it’s not noticeable.
They have done 10 treatments like that so far, and my numbers only gone down 10 points. It’s still above 900.
Just today, on my way into town, a blister broke out that covered my entire thumb. It’s hard to describe the pain.
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1 ReactionIt is very treatable IF you have functioning kidneys. Dialysis for ESRD does NOT filter the iron. My sister has been blessed to see Dr Herbert Bonkovsky - one is only a handful of specialists on PCT. He is located in Winston- Salem NC. Phlebotomy is a challenge with ESRD. My sisters hands are indescribable. They are severely infected from the blisters and when she recently bumped her wrist and the skin peeled back. She will eventually lose all fingernails. She is being treated with meds to help reduce iron through bowels rather than kidneys. Basically, her hope is in getting a kidney transplant - until then, it is a daily struggle with pain and wound management. She is currently in the hospital (for over 2 weeks )on IV antibiotics to try to get the infection under control again. Pain is being managed with Tylenol and tramadol and on dialysis days which horrible pain days ( dialysis exacerbates the pain) dilaudid via IV is the only thing that helps. PCT is rare enough without being complicated with ESRD. I have wondered if there are even 10 people with both.
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2 Reactions@ouchelp220, I'd like to add my welcome. Can you share why phlebotomy is not a treatment option for you?
Thank you for your suggestions. I have seen that website and read all the things that would be helpful. I made an appointment with my doctor to discuss Plaquenil and its benefits. I was put on a very low dose of it and have since been taken off of it. I need to ask them why.
I wish you the best in all you are going through.
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2 ReactionsWelcome to Connect, @ouchelp220 Your symptoms of PCT sound awful with the blistering and pain. I’m so sorry you’re having to go through this and not getting any relief!
I was looking online for a little more information on Prophyria Cutanea Tarda and the type of the disease you’ve acquired is supposed to the most treatable form by having phlebotomies. Unfortunately for you that’s not an option.
I did find on Mayo’s main site that another treatment for people who are not able to to have a phlebotomy. Taking a medicine used to treat malaria, usually hydroxychloroquine (Plaquenil) can help absorb excess porphyrins and help your body get rid of them more quickly than usual. The medicine is generally used only in people who can't tolerate phlebotomy. This may be a real of hope for you!
Here’s the article that talks about the types of your disease so scroll down until you find PCT. The next page, discussion diagnosis and treatment
https://www.mayoclinic.org/diseases-conditions/porphyria/symptoms-causes/syc-20356066
I’m also giving you the link to Mayo Clinic in case you’d like to get a second opinion. http://mayocl.in/1mtmR63. You can click on there to request an appointment.
If you’re not having any success with your current doctors, are you able to seek treatment at a larger teaching hospital or visit a Mayo Clinic at one of the 3 campuses?
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