Struggling to Get Diagnosed - GCA

[Alihughes25]

Hi all, newbie here. First off, I'm so incredibly grateful to have stumbled across this forum last night. My journey these past three months has been extremely hard, lonely and frustrating. Here's my story: I'm a 46 y.o. female who developed a new headache in late October. It started with a very sharp pain on the top right side of my head, almost felt like a knife stabbing me. Over the course of the next few days, the pain became duller and extended to my right temple and ear and base of my neck. It progressively got worse over the following week to the point that I was crying each night by bedtime. Ibuprofen didn't touch the pain. After a week of worsening pain, I went to an urgent care. In reviewing family history, I mentioned to the doctor that my dad has GCA. She said my headache was presenting with many symptoms of GCA and to be abundantly cautious, she started me on prednisone immediately until I could get a follow up with a neuro and rheum.

I started 60 mg the next day and immediately felt like a new person. All of the headache symptoms disappeared. At that point, I was in denial that GCA could be a possibility so I started to taper the steroid dosage on my own over the following week. As soon as I did, the pain was back so I called to get an appt. with a rheumatologist. All of the docs with good reviews in my area were booked for six months so I took a cancellation appointment with one who had bad reviews.

At my appointment, the rheum was very dismissive and told me that the urgent care shouldn't have started me on steroids, that she had never seen anyone my age with GCA and that now that I was on steroids, it wouldn't show up in my blood work or a temporal biopsy even if it was the case (which again she said there was NO possible way). We did blood work anyway and my sed rate and CRP were both normal. She sent me home with a tapering program for the steroid, which I followed over the next few weeks. When I got down to 20mg/day, all of my symptoms came back with pain on my left side as well. I called her office to let her know and she was extremely rude and dismissive and told me again there was NO WAY I could have GCA and that I needed to go to a neurologist. She also sent me a letter saying that she would no longer be able to treat me at her practice.

I found a new rheumatologist and started seeing him in early December. He also did blood work and my sed rate and CRP came back normal with him but my ANA was positive. I was still on steroids at the time of this blood work. He referred me to neurology as well. I went to the neurologist who ordered an MRA but could find "no known cause" for my headache. She recommended that I do a nerve block, which I declined.

I went back to my new rheum for follow up after seeing the neuro. He said he could still send me for a temporal biopsy but it's highly likely to be negative at this point since I've been on steroids for so long. He told me he could treat me for GCA based on symptoms but also thinks I'm "too young" to actually have it. He gave me a prednisone prescription with instructions to self adjust it to combat symptoms and give it a few weeks to see what happens.

I've adjusted my prednisone slowly from 25 mg to 40 mg but am still having symptoms. Although not as bad, I do have pain on both sides of my head and a new symptom this past week - the top of my scalp hurts to touch and burns all day. I have neck pain when I wake up in the morning and occasional tongue and throat pain, as well as pain deep in my left shoulder joint. The right side of my jaw hurts and tires quickly when I chew. I have a follow up appointment with my rheum tomorrow morning to decide on next course of action. I plan to push for going to the full 60 mg course and hope he will be on board.

I feel a bit hopeless right now with doctors refusing to believe this could be GCA, even given my family history. I keep pushing and advocating for myself but it's been really discouraging. I almost wish I'd never gone to the urgent care because the initial steroid dosing masked any evidence for diagnosis. Yet if this is GCA I'm happy that they were proactive and took it seriously enough to prevent vision loss. I guess the important thing is that I am taking steroids and still pushing for answers. It's been a long, lonely, frustrating road but I've found comfort in reading many of your stories here. Thanks so much for reading and listening. - Ali

[DorsetLady PMRGCAuk volunteer]

Hi Ali,

And welcome.

Very frustrating for you - but as you say, good that steroids were started even though they may have muddied the waters in getting a diagnosis that your Rheumy would accept.

Yes it is unusual get get GCA during your 40s - but it should not dismissed - many papers now recognised that more and more 50 years olds are getting it - and the old adage of you must be in your 70s’ is slowly being phased out. Although, obviously not in some people’s minds.

Your Rheumy is correct that now you’ve been on steroids, a biopsy is pretty much useless, but it very often shows a false negative anyway when no steroids have been taken.

It’s symptoms, symptoms, symptoms all the way really - and let’s hope your Rheumy takes that on board tomorrow - and if he is hesitant please impress upon him that you are still very concerned about your sight. As you are on 40mg and symptoms are increasing that just proves to me it’s not enough. Make a few notes of exactly what you had before, and now and take it so you can refer to them at appointment.

Good luck, and let us know the outcome.

[Alihughes25 in reply to DorsetLady]

Thanks so much for your kind words and support DorsetLady. I'm a bit scared to be having these symptoms at 40 mg but it's definitely a motivation to push for the full treatment. I will keep you posted on what my rheumy says tomorrow. Thanks again!

[PMRpro Ambassador]

I do wonder why when something looks like a duck, walks like a duck, quacks like a duck it appears to be impossible for some people to admit it COULD be a duck ...

GCA can occur in patients under 50 - it is just less common, doesn't mean it doesn't happen.

You mention rheumies and neuro - what about ophthalmologists? Though after all this time on pred they probably won't see much. But they might be willing to listen and think outside the box.

[Alihughes25 in reply to PMRpro]

Hi PMRpro! I totally agree with you and have been frustrated that my docs haven't been more open minded.

I went to an ophthalmologist last week and everything checked out as being normal. Grateful for that. Now to get the rest of these symptoms under control...

[SheffieldJane]

This is an awful story of medical arrogance and then abandonment. It is possible to have GCA at your age and I am glad you went to urgent care as this action probably saved your eyesight. You seem to be experiencing symptoms which indicate that your GCA is very active and progressing. You would be safer on the higher dose until these symptoms settle. I sincerely hope that your efforts to be an effective self advocate continue to pay off and your Rheumatologist agrees to start again with the only effective treatment for GCA which is high dose steroids. Your Rheumatologist may even find that 60 mgs is not enough to contain the inflammation because you have been messed around so much. Some patients need as much as 80 mgs. It is unusual but not unheard of to develop this disease at 46. I would say particularly with your family history. I am keeping my fingers crossed that you encounter a doctor who can think outside of the box and give you the treatment you so urgently need.

[Alihughes25 in reply to SheffieldJane]

Thanks so much Jane. Yes it's been absolute arrogance on their part. I decided to adjust my pred to 60mg today after reading your post. This slow progression upward isn't helping and seems to actually be allowing the disease to progress. I'm hoping to not have to go up to 80 mg to control it.

My plan is to let my rheum know tomorrow that I've decided on this course of action. Crossing fingers that he will be on board and support it.

[PMRpro Ambassador in reply to Alihughes25]

Creeping up the dose never works - and is tantamount to medical negligence, especially if you were to develop visual symptoms or visual loss.

[Alihughes25 in reply to PMRpro]

I'm worried now that the jump to 60 may not be enough. Today was my first day going from 40 to 60. It took care of the burning sensation but I still feel some pain on my right side. Trying not to panic but admittedly quite scared.

[PMRpro Ambassador in reply to Alihughes25]

Give it a couple of days - often nothing happens for 48 hours or more, instant changes are unusual.

[SnazzyD]

Jeepers creepers. The lack of thought is staggering. If I was feeling brave I’d give them a list of all your symptoms and ask for confirmation in writing that their rejection of a GCA diagnosis, in light of good response to 60mg, is sure enough to say that blindness is not a risk. I wonder if they would.

Anyway, if not GCA, why not some other vasculitis?

[Alihughes25 in reply to SnazzyD]

Absolutely staggering. The first rheum sent me the letter telling me she would no longer treat me in large part due to the fact that I did ask her to provide that confirmation. She said she felt I was going to threaten her with legal action. It's been quite a journey.

[SnazzyD in reply to Alihughes25]

Too right. So she couldn’t explain her non-diagnosis to the best of her evidence based knowledge and be happy stand by it in court. Rather punt you on to somebody else. Speaks volumes.

[Alihughes25 in reply to SnazzyD]

Exactly!

[piglette]

It really was very lucky you had heard of GCA because of your dad, otherwise due to the total lack of competence you could nave been left blind. It looks like you are now on the tight path in spite of a load of arrogant doctors.

[Alihughes25 in reply to piglette]

Totally agree and thank goodness for the urgent care asking the right questions!

[Alihughes25]

Wanted to post a quick update from my follow up today. I told my rheum of new symptoms, including scalp burning and tenderness and pain now in left temple as well as right. Immediately he asked if I was planning to go back to the neurologist. I told him that I did have a follow up scheduled in a few weeks but also that I wanted him to treat this more seriously. He started to examine me, then asked "are you sure you're not just reading about symptoms online and thinking you have them?" I kept my wits about me, looked him in the eye and said "I NEED you to believe me about this".

Long story short, he agreed to treat me at 60mg for the next month and then start to taper and see what happens. He is also requiring me to get a temporal artery biopsy next week but added that the vascular surgeon would probably "laugh at him" for requesting it.

I'm trying to keep things in perspective and remember that the important thing is that I am getting treated. The unfortunate epilogue to this is that so many medical professionals have dismissed me, refused to believe me and have been so arrogant. I don't want to be in pain every day, I don't want to be on high dose steroids, I just want to feel better. Why have I had to fight so hard for it?

Thanks for allowing me to vent. This has been such an amazing community already and I so appreciate you!

[PMRpro Ambassador in reply to Alihughes25]

I know - anyone would think we LIKED being on pred!!!!

[Jeanfkelly]

My story is very similar to yours - I was told that I was "too young" to have GCA.

I was 49 and a half. Apparently I needed to be 50 before I could have GCA !

I was admitted to hospital and languished for 9 days being fed paracetamol every 4 hours with no effect and tested for strange conditions I'd never heard of.... all of which came back negative and still the consultant refused to believe I had GCA, despite my GP's letter insisting the evidence was compelling.

Some time later, I got a reluctant diagnosis and was given Prednisolone.

I changed consultant and 2 years later was off the Pred and looking forward to being well again.

Unfortunately , I had a relapse 8 months later and a 2nd opinion confirmed Refractory GCA.

5 years down the line, after unsuccessfully trying Methotrexate & Azathioprine I'm now on Tocilizumab & have just started to reduce Pred again.

Positive attitude to the fore - this feels like my last chance to significantly lower the Pred -currently 20mgs daily.

It's exhausting when you have to keep a track of things and "fight" your corner. Howeverr ' this is what you must do in your circumstances,

Eventually, you will probably know more about GCA, treatments, protocols, current research and thinking than the consultants who think they know best and who have NOT

kept abreast of current developments and thinking.

Arrogance and patronising attitudes only make our journey more debilitating, but you must stick with it.

Organise a 2nd opinion if you can and make sure you have a GP who is clued up on all things GCA!

I was very lucky that my GP was/is and supported me through some very difficult times.

For some consultants, patients who don't fit the text book descriptions and recommended

treatments/tapering protocols fail to be considered as individuals - we're all different and very few of us match the text book description of GCA and recovery.

Join PMRGCAUK and read as much of their literature as you can.

Show it, if necessary, to your consultant.

I wish you all the very best.

Don't hesitate to contact me if you want to.

[Alihughes25 in reply to Jeanfkelly]

Thanks so much for your kind response. I'm so very sorry to hear that you had to fight your way to diagnosis too. Sounds like you have an incredible GP.

My father's doctor is a few hours away but I'm considering switching to him for ongoing care after everything is under control. He is familiar with the family history and is giving him great treatment for his GCA, PMR and RA.

I so appreciate your support in this and will check out all of the resources you recommended.