today's endo appointment

Just an update on today's clinic appointment. 
So.... my endo has completely ruled out FHH. she said that it wouldn't be causing the bone thinning in the dominant forarm. And my dad's calcium in 2012 of 2.5 rules it out also.

We spoke about his labs and says there could be a connection or may just be a coincidence.  As his vit d was low she thinks it would be useful to supplement it and see what happened to his calcium. Although it may cause it to rise.

I had all my hormones tested as I had a low oestrogen and missing menstrual cycle for over a year and a Half. I also has the usual tested of parathyroid and calcium ext. ... to see what happening now I've doubled the cinacalcet dose. She told me she's trying to gather as much info as possible to present to the surgeon.  Also she said if my calcium is more stable and my menstrual cycle has returned then this is more evidence that this problem is having an effect on my metabolism. 

She also spoke about MEN and how this could be a risk for me as they think it's  4 gland problem.  As both scans were negative even though sometimes the adonema doesn't show up. 
So she is going back to the surgeon to ask too see me. And I have to email her next week to find out my latest labs. 
She said that they don't want to operate if they don't think it will make me feel better As if it's a 4 gland problem that could leave me hypo pt.  If my Calcium levels were in the 3.0s then there would be no question.
she said it's her job to provide all the necessary evidence to the surgeon to prove that an op will make me.feel better.  
She wants me too see him again in the joint clinic to discuss things. 

I know things are moving slowly but it's all getting on top of me and I feel like my life is hold. 
I'm worried this won't get sorted and it will be going and back and forward for ever!


Rachel xx

Some positives then and another step forward.

You definitely don't want to end up hypopara as that can really be a nightmare, so I can see why they want to be really sure before diving in, not sure how your Endo can prove an op will make you feel better

I think you have to go with the flow for the time being, difficult I know but at least your Endo is doing her job, it does all take a long time but a decision will be made at some point I'm sure.

Nelly

Thanks Nelly

I really appreciate your response. I understand that being hypo would be a great move. 
I'm just still confused and don't see how this is really going to end, I know I have to be patient.  But I can't get my head around all these symptoms and how I feel. 
It seems being hypo hyper or normal thyroid don't make a difference to my calcium levels they still come back elevated. Although cinacalcet has stabled it to some extent but I will have to wait and see what's happening with the double dose and if that's made a difference. 
I'm also interested in oestrogen and if that can effect calcium levels. Or that's causing the oestrogen problems.

I am also worried about the MEN issue as a parathyroid problem has been confirmed. Bone loss in forarm predominantly, low phosphate and high calcium.
surely that makes it all necessary for me to have the op.
I really don't want to continue to have bone loss and feel the way I do. But there is nothing else to do but wait. 
My endo is going to speak to Mr M again but I'm holding out any hope. Although she did say the main issue stopping them going forward was the possibility of FHH which has been ruled out as my calcium creatinine was borderline high.

It's just hard trying to get in with life and pay the bills and keep tings ticking over with this all going on, I was hoping I would see an end in sight but it seems to be getting gut her away  

Rachel

Hi Rachel - so glad to hear you have had your appointment with the endo and that she is really on your case.

I agree with Nelly, as difficult as it seems they have got to be careful how they proceed, and it looks as though that is exactly what they are doing.

It seems to me that they are looking to rule out the Multiple Endocrine Neoplasias. I would allow them just a little more time to see what the surgeon's opinion is at the end of all of this. Then it might be an idea to ask if you can be referred to a MEN clinic.

This seems to be the path I am on at the moment as 3 of my glands were hyperplastic which might also point to MEN needing to be ruled out.

I have had a lot of useful information and helpful recommendations regarding where the best MEN clinics are the website of AMEND. You can download their information booklets, but if you sign up with them (free) they will also send them in the post - and it's very useful information.

I think most people with MEN do end up having parathyroid surgery, if they are able to have it, so don't worry about that.

I think Londonanna has more experience of MEN1 and I hope she will chip in here for you. I'm on the same path as you (wanting MEN1 ruled out, but looking at the possibility of it more seriously now) so we can buddy up on this.

My GP has referred me at my request to the Endocrine Genetics Clinic at Addenbrookes. I didn't really want to go back there, but they can't all be as bad as the endo's I've seen for PTH (I hope). The main MEN man (and I will let you know his name when I can find it) works out of Oxford at the John Radcliffe Hospital, I believe.

Just hang on a bit longer, Rachel, see what your endo comes up with - just run with her now as she is the best you've got for the moment and seems to know her stuff and is on your side.

Love and Hugs from Tigerlily xxxx

Hi tigerlily,  

Thanks for the reply,  
I can't help feeling disappointed as usual But I know I have to be patient.

my dad's gp is finally on the ball. He saw him today and has spoken to an endo. He's started vit d replacement with a blood test in 2 weeks. Then another in 4 weeks. Then if calcium comes back high he will then have a 24 hour urine test. They have been so much more on the ball with his case in Devon than where I am. I had to wait for a referral to an endo before any vit d or urine tests. Seems too much of a coincidence to me with this going on with my dad.

In the mean time I feel a bit stuck I know my endo is doing everything she can for me. But part of me is scared I'm going to be left like this. My endo said if it's a 4 gland problem then I may not be able to have the op so easily as the risk of hypo for the rest of my life could happen.  But I can't stay like this forever.... can I? 

So 60mg of cinacalet a day and we shall see what's happening......

With regards to the MEN testing tigerlily is it all genetic ? I've had my pituitary tested etc. ..all seems fine bit I know this may not present itself now. 
I'm really confused as to why nothing seems to be coming to much. ... is there something they are not telling me :/

Howas is MEN ruled out ? 
and I can't seem to think why they defiantly think it's a 4 gland problem just because of the negative scans.... surely my endo would know it may have been missed? 

Thanks for the info I will check it out. Can't help feeling a little frustrated and thinking if this will ever get sorted ??? 


Rachel xx

Hi Rachel - so glad to hear that your father is getting some action at his end as well. That can only be beneficial for him for the rest of his life - and he can't actually be that old.

No, you can't stay like that forever. If it's a 4-gland problem then your present surgeon might not be the one experienced enough to operate, and that might be why they are being so careful.

If your present surgeon cannot operate, you still have the option to get referred to Fausto Palazzo at the Hammersmith and his team. So don't worry too much - you still have other options.

But your endo seems really skilled in taking this forward, even though she may not know much about 4-gland hyperplasia herself. If that turns out to be their final diagnosis in a few weeks time, then you can take another tack and get referred to the right person to treat it. I think you will still be able to have an operation. They usually remove 3.5 glands and leave you with one whole one left or half a one, and you can live with that with frequent monitoring to ensure that the remaining part of the gland functions well.

Your endo is right, though, that hypOparathyroidism is a much worse disease than hyper, so they don't want to make any mistakes with you. FP pointed this out to me when I saw him for my post-surgery check-up in February.

Regarding the MEN testing: the gold standard is a genetic test, but there are various other blood tests they can do to get an idea if the gene test is necessary. I've had the pituitary tests done, too, and a gut hormone screen (to check to see if any of them are over-producing hormones). But all my hormones are low, rather than high, so the blood tests are not clearly indicating MEN1 for me at the moment. I do however have other symptoms like various lumps and bumps (other endocrine and non-endocrine cysts, including quite a large ovarian cyst) so I need to get these checked out as well - which I hope is what will happen at the MEN Clinic when I get there.

Go on over to the AMEND site for further information on MEN1 - it is presented really well, and there is a closed Facebook group that you could apply to join.

I will also let you know how things progress for me on the MEN front.

Chins up! as we say on here, as a number of us have quite a few more than one! Go with the flow for the moment, but you do have other options, so try not to worry too much.

Love and Hugs from Tigerlily xxxx

Thanks tigerlily

Yes it all seems very complicated!  
I have faith in my endo and the surgeon and like you said I think they are treading carefully because of cetane things. And I understand this completely.

I see with the genetic testing and that must be very costly. I'm trying to put the pieces together with my dad's case and mine and that may explain that I have inherited something and I did mention to my dad perhaps it could be a vearient of something. ... she wasn't too sure and didn't seem to know if it was connected or not but we shall see how his case progresses.

Maybe he will get sorted sooner than me!  At least that's one down and one to go 

I never thought at the start of all this it would take so long but I've a lot to learn when it comes to being patient with all this !


I read that some familial cases are to do with a faulty casr gene so it still releases pt hormone even when the calcium is elevated. I don't think they will do genetic tests on me with my pituitary and other things being okay and I'm guessing the results would take Ages. 

I hope it's not possible for my diagnosis to be taken away and float into thin air ??? This petrifies me !!

Thanks for the info tigerlily and I would be facinated to know what's going on at your end too !

Rachel xxx

Hi Tigerlily and Rachel

I haven't been on here for a while as I felt I did not have any more useful comment to make on quite a few of the problems people are still experiencing.  However, you are both now expressing an interest in finding out some more information on MEN.   I have recently found out that there are now five strains of MEN (new information being discovered all the time) which have been diagnosed.   As you may know I have MEN 1.

It is true that between 95 and 99% of MEN patients go on to develop hyperparathyroidism.   The two other areas most people develop tumours in MEN 1 (almost always benign and therefore fall into the category of 'nuisance') are piturity and/or pancreatic tumours.   There is no evidence that people who have had hpth go on to develop the other two types of tumour or any other.

MEN is caused by part of a chromosome - a gene -  (No. 11 in MEN 1) being switched off.   No one knows why this is but it is ALWAYS a hereditry genetic condition and does not miss a generation.   Rachel, if your doctors suspect that you might have MEN then you will have to undertake genetic testing and counselling to tell you what a positive result could mean in your particular case (it is different for everyone).   This can take up to three months.   I can also confirm Tigerlily that you are not 'too old' to have MEN.   This was poorly explained to you by your doctor because even though, if confirmed, you have had the condition from birth (as I have) it has remained undiagnosed.

As far as your para's are concerned Rachel, you could have four gland disease but that does not necessarily mean you would have to have all four glands out.   It would be your doctor's decision depending on what he finds.   However, I have had four gland hyperplasia.   When I was 21 I had two taken out and for the next 35 years I had excellent health.  Three years ago I got sick again and finally, after finding the excellent team at St Mary's in London, had the other glands removed - almost all.   They left one third of one gland in place so that it would continue producing hormones.   This is working absolutely fine as I write. 

Its hard not to worry and be scared but really, whatever is found or not found, you should receive really good advice and support.   I am hoping that you both have a negative diagnosis eventually though.   The AMEND site is a fantastic source of good, accurate and non-hysterical information.   I hope this has been helpful but if I can help any further, then let me know.

HI londanna,

Thank you for all the useful infomation.  I see you were of a similar age in your twenties as me when you were diagnosed.  I haven't been offered genetic testing and may not be, but my endo has mentioned MEN a few times. It all seems a little up in the air at the moment. 

I would love to ask you some more questions of that's okay. For example what we're you symptoms and levels in your twenties ? And how long did it take for things to get moving. I understand when there could he 4 glands involved at a young age it's a difficult situation for Dr's decide what to do. 


thank you

Rachel x

Rachel

Yes, please ask me any questions you want to and I will do my best to answer.

I lived in Oslo, Norway when I had my first removal operation.   This followed an emergency operation to remove an enormous kidney stone (big operation at the time with large scar on my right side).   This was undertaken successfuly and I left hospital after about 8 days.   They did take bloods and I heard nothing else until out of the blue some four months later I received a letter asking me to return to hospital - they wanted to discuss another operation.   When I got there it was just explained that I was producing too much calcium and it was necessary to take out some glands found in my neck after which I would have no more kidney stones - ha ha ha.

With the innocence of youth I readily agreed.   I didn't really question it.   I was just relieved that as far as was explained to me and I thought I knew, I would be 'cured'.   To be fair, genetic testing had not yet been developed so that was not possible.   Anyway, the operation went well and I was discharged two days later (after an agument with the surgeon because I was due to go to Finland and the medical glue they had used to stick me back together again was a very, very new invention).   On return to hospital a few weeks later my blood readings were 'normal'.   That is all I was told.  

It was only later (several more kidney stones and a translation of my medical documents) that I learned the two glands they had removed were hyperplastic, with the two remaining ones in 'a good, normal condition'.   This remained the case (with periodic blood tests) until three years ago.

When I presented at St Mary's in April last year my surgeon took one look at the notes my doctor had written and declared that 'something was going on' and he suggested genetic testing straight away.   The rest you already know.   I had my operation in September and so far everything is good.   I will be monitored for the rest of my life which is a good thing although a bit of a nuisance.   My eldest daughter has also been diagnosed with MEN 1,   She is about to undergo IVF and is being fast tracked for the process to screen out the faulty gene before implantation (this is a little bit of a consolation for me)   We are currently waiting for my younger daughter's results.   My sister has been cleared though so her family are all OK.   I have also been told that I have a very, very rare and mild form of the disease.

Hope this has been helpful.   Its interesting to actually see medical progress in diagnostics and treatment.   I understand also the current operation for hpth does not involve leaving a scar on the neck.

Londanna

Thanks so much for this information, Londonanna.

I'm sure Rachel will be helped by it and I have been, too.

If I don't get the help I need from Addenbrookes in order to fully rule out MEN1, then I shall ask to be referred to St Mary's.

I think I've had this condition all my life and that my father may have had it, too. I am in the process of trying to access my father's medical notes to see if there are any clues in there. He died with myelofibrosis in 1996 and I have seen recent papers that indicate that myelofibrosis can be one of the end-points of long-standing untreated primary hyperprathyroidiam. My father had a brother so I have a number of cousins plus their families to whom information about MEN1 might be relevant, although I shall not bother them with it until I have been diagnosed or the condition fully ruled out.

Please don't be a stranger to the forum, Londonanna! I'm sure that Rachel will be pleased to have someone here who can help by answering questions - and I certainly am!

Talking of the scar in pHPT operations: mine was quite a large one because I insisted on a 4-gland exploration, but the original 6cm has now shrunk to about 4cm and it is well hidden in the existing crease in my lower neck. Some of my bulky under-chin area has also disappeared with the sort-of reverse face-lift - not how you would choose to have it reduced, but a definite bonus!!

Thanks again for your comments and advice, Londonanna. I really appreciate it.

Love from Tigerlily xxxx