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Questions about PAH/PH (LFT, ECHO, ICD and rheumatology)


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#1 Amanda Thorpe

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Posted 13 December 2015 - 05:35 AM

So it's been a long time since I posted a topic, let alone an update but having had a full and eventful day at the Royal Free this week, I've a few questions... 

 

To recap, diagnosed 2007 diffuse systemic sclerosis, serious heart failure from myocardial fibrosis early 2010, bi ventricular ICD implanted late 2010, chronic leg ulcers from 2011ish, completely wheelchair dependant 2012 having used one from 2007. Main problems are now crippling pain and fatigue. 

 

This week's appointment and my LFT was normal, ICD functioning well, ECHO was puzzling because the tech suggested it was all good but the rheumatologist wasn't sure. During the ECHO the tech checked my JVP (I think that's why she put the doppler on my neck) and when I asked if the pressures in the heart itself were normal, she said yes. The rheumatologist wasn't sure as the reading was 27-42 and whether that wide of a reading was an error so I'm being referred back to cardiology.

 

Now, I went a'googling, as you do, and see that some sites say 25 and above is pulmonary hypertension territory so here's my question(s): 

 

-are readings above 25 considered to be PH 

-can you have such a wide reading as above 

-can you have a normal LFT and PH  

-can you have a bi ventricular ICD and develop PH (anyone knows that and I'll happy dance for ya!) 

 

Having survived myocardial fibrosis, an out of the blue hit with a wet fish diagnostic experience, that at the time reduced my life expectancy to 2 years, I can't help but wonder if PH is going to be the other shoe, landing squarely on my head, stiletto heel positioned downwards.

 

With this disease, you just never know when the floor is going to drop out again. In 2010 my life expectancy reduced to two years without successful treatment ( 30% of people never get any benefit from pacemakers) and I had gotten used to thinking I was way passed all of that and now I know that I let myself enjoy a false sense of security. The numbers from my ECHO may be nothing and nothing mat have changed and then again I could have PH although I know that right heart catheterisation is required to officially diagnose it.

 
I'm also being referred to gastroenterology, the rheumatologist thinks, based on symptoms, the valve between esophagus and stomach is faulty. My gastro symptoms used to come and go, about 2 months ago they came and never left, leaving me difficulty swallowing, reflux, constantly feeling something is stuck and bringing back up recently eaten food whilst still eating. Bad table manners, bad, bad table manners!

 

I'm also off to physiotherapy to see if I can be enabled to stand and even walk, pain allowing (post pending about pain management procedure). Finally, I'm giving hydroxychloroquine another try but a lower dose this time, gotta get on top of this fatigue! Anyone find it causes headaches and do they go? 

 

Take care.

 


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#2 Shelley Ensz

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Posted 14 December 2015 - 07:13 AM

Hi Amanda,

 

Wow, you are being put through the mill!  I'm sorry you have so many complications, and all at once, no less.

 

I don't know the answer to most of your questions. PH can be very hard to diagnose. And it can be misleading, because some tests can appear to indicate PH, others can appear to be perfectly normal, but only the right heart cath can sort everything out properly. And, that is quite a procedure to undergo. When is your next cardiology appointment?

 

Be sure to read up on Nissen fundoplication before you visit gastroenterology, in case they suggest that surgery to fix it.

 

For hydroxychloroquine, my rheumatologist had me take my entire dose at bedtime, so that I would sleep through any side effects. That worked like a charm and I never had had any bothersome side effects, except for the good ones like a very noticeable and welcome reduction in pain and fatigue, and fewer flares of all sorts of things.

 

Keep us posted as things develop. It's certainly going to be a cute balancing act to manage all of this at once!

 

:hug-group:


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#3 Joelf

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Posted 14 December 2015 - 12:01 PM

Hi Amanda,

 

I'm so sorry to hear that you've discovered even more complications; as if you don't have enough to contend with!  :sorry:

 

Thankfully, I don't suffer with PH and I'm afraid I can't give you anymore info than you got from the Royal Free. At least you have got them dealing with it for you, although I realise that's not much comfort when you've got the prospect of more procedures to be done.

 

However, one thing I can do is wish you the very best of luck with your tests and keep the fingers on my other hand firmly crossed for you.

 

Best wishes,


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#4 greypilgrim256

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Posted 16 December 2015 - 02:59 AM

Did you get your DLCO score?  



#5 Shelley Ensz

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Posted 21 December 2015 - 08:21 AM

Hi Amanda,

 

Have you had any more tests or results in the past week?  There is so much you are juggling. And right at the holidays, too! 

 

I'm thinking of you, and sending lots of good thoughts your way.

 

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Shelley Ensz
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#6 Amanda Thorpe

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Posted 27 December 2015 - 01:30 PM

Hello All

Still waiting for follow up appointments and ditched the hydroxycloroquine.

Something was causing rapid weight gain despite maintaining the same eating regime I had been all year. I know that the information leaflet says that weight loss is a side effect (much more attractive than weight gain) but the lupus forums, my friend who has knowledge of it and Michael all confirm weight gain not loss. Having worked all year to lose over a stone, and keep it off, nothing is worth putting it back on. Remember I can't exercise/cardio and without controlling my diet, I go up a dress size every year.

It will take a while to lose this weight gained which I am not happy about but I have to see it as a set back and move on.

My DLCO was normal GP, the results were perplexing for sure, don't get me wrong, good results are great but often confusing!

Thanks again and will keep you posted!
Amanda Thorpe
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