Home         Forums  

Go Back   Marrowforums > Bone Marrow Failure Diseases > PNH
Register FAQ Search Today's Posts Mark Forums Read

PNH Paroxysmal nocturnal hemoglobinuria

Reply
 
Thread Tools Search this Thread
  #1  
Old Sun Feb 20, 2022, 04:25 AM
Andy S Andy S is offline
Member
 
Join Date: Oct 2021
Location: Loughborough UK
Posts: 11
New PNH diagnosis

Good morning.
I was referred to haematology in October, 65% PNH clone, referred to Leeds. I had a phone assessment on Monday and I'm attending for bone marrow, echo and other blood tests this Friday.
They have been lovely as regards patient care.
I'm a bit jumpy in myself, I'll be glad when we're into the treatment phase.
Thanks for reading. I've found forums like this very helpful over the years.
__________________
Andy, 68, retired GP, husband of Charlotte for 45y. MDS diagnosed 2001, treated with daclizumab in 2004. Now dveloped into PNH.

Last edited by Andy S : Sun Feb 20, 2022 at 04:31 AM. Reason: Duplicated info
Reply With Quote
  #2  
Old Sun Feb 20, 2022, 12:42 PM
Neil Cuadra Neil Cuadra is offline
Owner
 
Join Date: Jul 2006
Location: Los Angeles, California
Posts: 2,556
Andy,

I'm glad you'll have an in-person appointment. There's only so much they can do with video appointments.

I'm also glad that you've been treated so well by your health team. From your forum signature, it's clear that your wife is an important part of your support team too!

As a former GP, you presumably understand the tests, the reasons for them, and will be able to work with your physicians to interpret the results and consider the appropriate treatment.

PNH is the concern now, with your long-term MDS lurking in the shadows. PNH is much better understood now than when you were first diagnosed with MDS, and of course there are now drugs for PNH treatment.

Please let us know how it goes.
__________________
Founder of Marrowforums and caregiver for my wife
Reply With Quote
  #3  
Old Sun Feb 20, 2022, 05:32 PM
Andy S Andy S is offline
Member
 
Join Date: Oct 2021
Location: Loughborough UK
Posts: 11
Thanks Neil.
Yes, it's possible that the dismissal of my first attack of haemoglobinuria was due to the lack of effective treatment at the time.
I'm very aware of the privilege of living at a time when illnesses are treatable. I think back to the care that was available to my parents' and grandparents' generations and feel quite humbled.
Best wishes
__________________
Andy, 68, retired GP, husband of Charlotte for 45y. MDS diagnosed 2001, treated with daclizumab in 2004. Now dveloped into PNH.
Reply With Quote
  #4  
Old Wed Feb 23, 2022, 01:41 PM
Andy S Andy S is offline
Member
 
Join Date: Oct 2021
Location: Loughborough UK
Posts: 11
Assessment postponed until March 15th to enable echo, abdominal ultrasound and bone marrow to happen on the same day. It unsettled me a lot (perhaps I get unsettled more easily these days) but I'm levelling out mercifully. One isn't always master of one's self as one would wish.
__________________
Andy, 68, retired GP, husband of Charlotte for 45y. MDS diagnosed 2001, treated with daclizumab in 2004. Now dveloped into PNH.
Reply With Quote
  #5  
Old Sat Feb 26, 2022, 01:25 PM
Andy S Andy S is offline
Member
 
Join Date: Oct 2021
Location: Loughborough UK
Posts: 11
I seem to be sleeping a lot of the time. Slept all last night and 3 sleeps during today. Plus more breathless on minimal exertion.
We're up for the weekend with my son and his family in Castleford. I think I'll ring Leeds on Monday morning and see if they can see me on Tuesday for the bone marrow and get treatment under way sooner rather than later.
Does that sound reasonable/feasible?
I've never been like this.
__________________
Andy, 68, retired GP, husband of Charlotte for 45y. MDS diagnosed 2001, treated with daclizumab in 2004. Now dveloped into PNH.
Reply With Quote
  #6  
Old Wed Mar 2, 2022, 01:15 PM
GoodDay5150 GoodDay5150 is offline
Member
 
Join Date: Sep 2014
Location: Centennial, CO
Posts: 150
Hi Andy. We are all sorry to hear that you may have PNH, but as you know modern treatments enable many patients to live reasonably normal lives. I am just curious as to whether or not your MDS is in remission or has it being controlled by meds? If you had MDS in the past, then you are accustomed to all of the testing and appointments associated w/ bone marrow failure diseases. Being a GP, you are aware of transplantation for some PNH patients, and there are a few of us here who have been successfully transplanted. I have many posts on this site regarding my experience, and I would be more than happy to answer your questions. Of course, me being a patient and you being a GP as well as a patient. I had never heard of PNH until I was diagnosed in 2011, and w/ the internet there are a lot of great resources I have used. We all wish you luck in your treatment.

Mario
__________________
MARIO, 52, DIAG IN 2011 W/ PNH, MUD IN DEC 2011. MINI TRANS PSL DENVER/ SOME MILD GVHD. CURRENTLY TAKING JAKAFI FOR GVHD.
Reply With Quote
  #7  
Old Wed Mar 2, 2022, 10:18 PM
Matthew42 Matthew42 is offline
Member
 
Join Date: Jul 2021
Location: USA
Posts: 130
This is interesting. I've never heard of someone with MDS developing PNH. Would it be primary or secondary PNH? They are not all the same. Secondary PNH is much milder, I believe.

Often times aplastic anemics have small PNH clones, and so they are at risk of developing secondary PNH. My mother has a small PNH clone, by the way, but she has classic aplastic anemia. When I asked my mother's hematologist about it, she said that having a small PNH clone means that her aplastic anemia is strongly immune-mediated, first and foremost. She wasn't that concerned about my mother getting secondary PNH, although possible.

@Andy: I'm wishing you all the best.

Love and peace,
Matthew
Reply With Quote
  #8  
Old Thu Mar 3, 2022, 03:48 PM
Andy S Andy S is offline
Member
 
Join Date: Oct 2021
Location: Loughborough UK
Posts: 11
Hi, and thanks for posting. I'm in the pre-assessment limbo phase, searching all over for information, and it's nice to be in touch with old stagers who have levelled out.
My MDS was very hypocellular, strongly auto-immune, daclizumab worked by targeting the activated T-lymphocytes. The auto-immune process was still there but my levels rose.
PNH stem cells are apparently not affected by the auto-immune response so they have an evolutionary advantage and increase.
My clones are around 80% atm. My main symptom is lethargy, though I have had 3 episodes of intense haemoglobinuria.
If I'm treated with ravulizumab, which I think is likely, I'm hoping that I'll have more energy.
I don't think BMT is used for PNH in the uk as ravulizumab is so safe.
Best wishes to all.
__________________
Andy, 68, retired GP, husband of Charlotte for 45y. MDS diagnosed 2001, treated with daclizumab in 2004. Now dveloped into PNH.
Reply With Quote
  #9  
Old Thu Mar 3, 2022, 04:26 PM
Matthew42 Matthew42 is offline
Member
 
Join Date: Jul 2021
Location: USA
Posts: 130
Quote:
Originally Posted by Andy S View Post
Hi, and thanks for posting. I'm in the pre-assessment limbo phase, searching all over for information, and it's nice to be in touch with old stagers who have levelled out.
My MDS was very hypocellular, strongly auto-immune, daclizumab worked by targeting the activated T-lymphocytes. The auto-immune process was still there but my levels rose.
PNH stem cells are apparently not affected by the auto-immune response so they have an evolutionary advantage and increase.
My clones are around 80% atm. My main symptom is lethargy, though I have had 3 episodes of intense haemoglobinuria.
If I'm treated with ravulizumab, which I think is likely, I'm hoping that I'll have more energy.
I don't think BMT is used for PNH in the uk as ravulizumab is so safe.
Best wishes to all.
Very interesting, Andy.

Yes, I did read that "hypocellular MDS" (much more uncommon kind of MDS) was more auto-immune, and that's why you might have PNH. Most MDS is not auto-immune (immune-mediated). That sort of makes sense as to why you may have PNH.

Aplastic anemia and PNH are more related to each other (both immune-mediated) than typical MDS is to aplastic anemia or PNH.
Please give us updates.


Love and peace,
Matthew
Reply With Quote
  #10  
Old Thu Mar 3, 2022, 05:11 PM
GoodDay5150 GoodDay5150 is offline
Member
 
Join Date: Sep 2014
Location: Centennial, CO
Posts: 150
Yes, it is more typical for patients to have AA that can be treated and come back later in life as PNH, and some persons present w/ both AA and PNH. I have met and have been told abt other patients by hematologists and rare disease specialists. In respect to stem cell transplants, it is my understanding that the usual age cut off date in the U.S. is 70. Obviously, someone's overall health absent of their disease is important. A transplant is a huge commitment, but having a good attitude helps.


Mario
__________________
MARIO, 52, DIAG IN 2011 W/ PNH, MUD IN DEC 2011. MINI TRANS PSL DENVER/ SOME MILD GVHD. CURRENTLY TAKING JAKAFI FOR GVHD.
Reply With Quote
  #11  
Old Thu Mar 3, 2022, 08:30 PM
Sally C Sally C is offline
Member
 
Join Date: Dec 2010
Location: Chesterfield, Va.
Posts: 470
Early on my husband's hematologist drew 3 overlapping circles, representing PNH, AA and MDS. At the time she said it was possible that his diagnosis fit where the 3 circles overlapped. A very strange disease for sure.
All the best to all you brave Warriors. Keep the Faith.
God Bless,
Sally
Reply With Quote
  #12  
Old Fri Mar 4, 2022, 01:03 AM
Matthew42 Matthew42 is offline
Member
 
Join Date: Jul 2021
Location: USA
Posts: 130
Quote:
Originally Posted by Sally C View Post
Early on my husband's hematologist drew 3 overlapping circles, representing PNH, AA and MDS. At the time she said it was possible that his diagnosis fit where the 3 circles overlapped. A very strange disease for sure.
All the best to all you brave Warriors. Keep the Faith.
God Bless,
Sally
Yes, it is a very strange disease, so much so that even my mother's hematologist gets puzzled by it, saying how unpredictable it is. It's not like cancer or standard autoimmune diseases.

You are right: Although my mother has classic aplastic anemia, she could develop secondary PNH (25%) or hypocellular MDS (10-15%). But, again, the doctor said, there is good chance she will only ever have had aplastic anemia. At any rate, the hematologist told my mother that her aplastic anemia is highly immune-mediated (Dr. Young confirmed this, I believe), putting her at risk for PNH most of all. She said that sometimes aplastic anemia is less immune-mediated in some people, requiring treatment that is less immuno-suppressant.

Love and peace,
Matthew

Last edited by Matthew42 : Fri Mar 4, 2022 at 03:03 PM.
Reply With Quote
  #13  
Old Sun Mar 6, 2022, 02:52 AM
Andy S Andy S is offline
Member
 
Join Date: Oct 2021
Location: Loughborough UK
Posts: 11
Thank you everyone. It's very helpful to hear people's stories as well as me reading learned articles and getting worried!
I'm currently counting the days to my assessment. Abdominal ultrasound, echocardiogram and of course bone marrow. I'm on warfarin due to a previous embolus in my leg so there's Fragmin to start injecting to bridge my anticoagulation during the procedure.
Then to start ravulizumab and hopefully feel less fatigued! There's so much to do?
Strength and peace to all.
__________________
Andy, 68, retired GP, husband of Charlotte for 45y. MDS diagnosed 2001, treated with daclizumab in 2004. Now dveloped into PNH.
Reply With Quote
Reply


Thread Tools Search this Thread
Search this Thread:

Advanced Search

Posting Rules
You may not post new threads
You may not post replies
You may not post attachments
You may not edit your posts

vB code is On
Smilies are On
[IMG] code is On
HTML code is Off
Forum Jump

Similar Threads
Thread Thread Starter Forum Replies Last Post
PNH and Cancer Diagnosis Catarina M Tell Your Story 3 Wed Feb 13, 2019 09:55 AM
Two PNH Drug Trials Going Well:Update Susan PNH 0 Sat Jul 7, 2018 10:52 PM
PNH mentioned in TV show The Good Doctor Marrowforums PNH 2 Tue Apr 10, 2018 01:01 PM
Possible Cure for PNH Tom Cramer PNH 16 Tue Aug 16, 2011 09:41 PM
PNH Webinar, September 2009 Marrowforums News and Events 0 Mon Sep 14, 2009 04:43 PM


All times are GMT -4. The time now is 01:23 AM.


Powered by vBulletin® Version 3.6.7
Copyright ©2000 - 2024, Jelsoft Enterprises Ltd.
Forum sites may contain non-authoritative and unverified information.
Medical decisions should be made in consultation with qualified medical professionals.
Site contents exclusive of member posts Copyright © 2006-2020 Marrowforums.org