Hypermobile Ehlers-Danlos Syndrome with a side of Fibromyalgia

New year, new diagnosis and still coming to terms with what that means for me.

Who would of thought that a thumb surgery would result in my life being turned upside down in my 40's.

So lets recap...

This past decade of diagnoses for me (during 6 clomid cycles and 3 rounds of IVF) between the ages of 30 and 42 are as follows: Hypothyrodism, Renal agenesis, Pelvic congestion syndrome, Bicornuate bicollis OR Unicornuate Uterus (they havent decided yet), Spondylolisthesis, Scoliosis, Acrocyanosis, Bradycardia, Mast Cell Activation Syndrome, Postural Orthostatic Tachycardia Syndrome, Irritable Bowel Syndrome, Fibromyalgia and Hypermobile Ehlers-Danlos Syndrome. 

To think I was labelled a hypochondriac. Growing up I don't ever remember much about the effects the above conditions had on me as I was too busy being a kid. I do remember my night terrors, my panic attacks and nausea at bedtime, my breathlessness, my chronic growning pains, my constant constipation. Anxiety, headaches, visual problems, brainfog, fatigue... I thought I was normal enough? HA! little did I know I was far from normal.

10 years into my journey of discovery and I feel like every now and then we do a little rewind. What a decade its been. There has been some really great and positive things that have happened. There has been tragedy. I feel like that is life and for every dark cloud I find a silver lining. 

My journey to EDS has probably been one of those silver linings. With diagnosis comes understanding, not only of my own challenges but those of others. It has reinforced my goal of becoming an advocate and I've been welcomed into the EDS community by a dazzle of amazing zebras that are all unique in their own way and have so much compassion for others. After successfully completing a qualification for Adult & Teritary Teaching as well as fostering two amazing young ladies and working full time I'm hoping to join the dazzle officially in some capacity to bring awareness and support to where its needed the most. I will be completing a Community Leader and Advocacy course with the Ehlers-Danlos Society starting in few weeks and look forward to where that will lead.

Meanwhile I was contacted about my Rheumatology follow-up a few weeks back and found out the Rheumatologist who diagnosed me has retired and I will be seeing someone new. I have been without support for a while after stopping my specialist physio appointments as could no longer afford them. I actually think I met him on my Dads AI journey and I actually look forward to meeting him. Will be interesting to see his view on my diagnosis and treatment plan. I'm refusing to up my Amitriptyline as its already having an affect on my health. It's working tho! my REM and Deep sleep have improved. I'm still waiting up sore but able to still function and drive which is what I wanted. Next will be looking into my BP, Heart and Immune side of things. I've managed to put on and maintain 4 kgs with the meds and trying to eat more in the evenings. All bloods NORMAL for a change, so have never been healthier. That has come at a cost however as my intolerance for food in general has not been easy. Eating is not something I enjoy and hope that will be something i can get a solid plan in place for. Finally I have the energy to exercise though, being able to do 5 kms on my exercise bike is a crucial step in recovery and management, as is my strenthening exercises to sort out my Proprioception issues. First things first tho, sorting my chronic pain and stress will be a key element of that. I'm on the list for a Brief Pain Management program which is spoken very highly of. When staffing issues at the hospital are over I should be starting that too. Everything is slowly lining up.

Oh, and we all got Covid. Will be interesting to see if any Long Covid signs pop up during the next stage of this journey.

Onwards and upwards... to be continued.

An update of epic proportions!

The gap was tremendous.. It feels strange not to have blogged in over a year.

So much to report, I don't know where to start...

The past 24 months has been a roller-coaster ride to say the least! I think we left off after my last specialist appointment and probably before my car accident. Yes, I wrecked my car Xmas of 2019 and suffered a bad whiplash and concussion and had a rough few months recovering from that while dealing with work and family. Funningly enough i wrecked it on a hot summer day, racing in 5pm traffic taking my father his weekly groceries while on the phone to him (hands free of course). Caring for my family is all I have known this decade and its what i love doing... but sometimes it was at the expense of myself... I think that day was one of them. Mum came to my rescue and took me for a neck x-ray. Nothing broken and off to the physio I went. She worked on me for a number of weeks and ive slowly come right.

Come February 2020 during the stress of working Xmas and shopping for a new car we were faced with a family related issue during that time and my stress levels skyrocketed. I was coming up to my 40th birthday with no real answers to my health concerns, getting used to our life of childlessness and caring for a very ill father who made it through sepsis, toxic shock and C-diff and was trying to get his life back with assistance from his children. It was a Monday night and we got a call from a friend of my nieces who asked us to come pick up my grand-nieces from their home and the police would meet us there, just for the night so my niece could seek emergency mental health support. Little did we know that night and the weeks that followed, our world was about to turn upside down. The girls who were 5 and 7 at the time, have now been with us nearly 2 years (24 months) and the story of our journey is a massive one and might be a blog of its own one day. DH and I are still in it together even though it has been incredibly hard and we sometimes battle through everyday.

Come March 2020 and my 40th birthday, I had an amazing month with my family having a reunion in Akaroa on the same weekend that I had a roller disco and was able to provide the girls with a fun time with their friends and mine, then have the opportunity to meet all my family in one spot for the first time in forever, including meeting my sweet 2nd cousins from Bali for the first time ever.

The following Monday came Covid lockdown #1. I was now a full time worker with my husband going into the battle zone (CDHB) most days, a home school teacher, grocery delivery person and trying to make sense of not being able to see my family for months on end. I think they were not only the hardest 7 weeks of our lives, its now seems so surreal... watching it unfold around the world. It was crazy.

During that time before winter set in we were getting outdoors a lot and really feel like pain was one of the things i was worrying about the least... that is until my tendonitis in my thumb started to get worse. You know i've always battled with De Quervain's tenosynovitis, carpal tunnel and other nerve issues, well this ended up being Trigger Thumb and yes another referral to a specialist. This time a hand surgeon. While waiting I attended a few appointments with a Hand Therapist and we started to talk about my tendonitis and chronic pain, taping, splinting all the things and he agreed the chronic side of my day-to-day pain was just something I would have to learn to live with and medicate and plan better for times I really need it, like gardening, lifting and cleaning.

I finally got to see the Hand & Wrist Surgeon in September 2021. Her consultation was very thorough. She was very quick to say "you have a very complex family history.. here I was thinking you would be a very quick 30 min Trigger Thumb, but it doesnt even trigger the right way, everything about you is complex". Yes, not a surprise to me, not even that the bone in my right thumb is larger than the other. Just add it to the list of right-sided anomalies. I was what she said next that threw me for a six... "has anyone every assessed you for a connective tissue disorder". I just about laughed. I told her that I was under many specialists that have never found links to my long list of ailments. She gave me a good look over and said "your appearance is very alike Marfans Syndrome and EDS (Ehlers-Danlos Syndrome), clearly you are hypermobile so you should look into that. I bet you can press your thumb to your wrist, bend your pinky 90 degrees and touch the floor without bending your knees? Ummm yes, among a few other weird and wonderful tricks I had performed as a child. It turns out my feet and hands are very hypermobile, hence my early-onset basal thumb arthritis which I have been able to sort splints out for. She prompted me to see a Rheumatologist for tissue biopsy and to see my fathers one if possible, if not I had a private option also. I was booked in for surgery 2 weeks after that, in the private hospital just incase due to my low blood pressure and complex nature. 

I went home and google Marfans and EDS, these are both terms I had looked into before, having known a few people with the conditions, I went straight to the source to ask advice. My friend whos daughters both had EDS, straight away said "dont take this the wrong way but you do have the look about you". Then my google search began, which I knew I shouldnt do. DH and I printed out the criteria for a condition called Hypermobile Ehlers-Danlos Syndrome also known as hEDS (this is one of 14 subtypes, and the most common of the rare conditions and the only one without a gene to type)... before my husband could get back from the printer, he had read it, looked at me and said "omg this is you." I read through the criteria and it was then my stomach dropped... all these years, all these specialists... is this even real? I promply joined the FB group my friend was in and started asking questions. First one, where next for diagnosis? They suggested a lady who specialises here in Christchurch in these conditions and had recently opened her own Specialist Clinic. It turns out not many specialise in NZ due to it being a rare syndrome and our low population numbers. 

My call to teh specialist physio was the best decision I ever made. I told her my story and its one she had obviously heard a thousand times before. Come on in as soon as we hit Level 2 (we were staging out of our second lockdown at this point)... and I did. Little did I know that it was just the beginning of a journey that would change everything.

It turns out I fit the criteria for hEDS like a glove. I got 5/9 on the Beighton Score and 8/12 on the Brighton Criteria.  I dont have hypermobile knees or elbows but I do have hypermobility in my hands, feet, shoulders, neck and hips. She could only however give me a provisional diagnosis. I still had to meet with a Rheumatologist to confirm the diagnosis by ruling out that my symptoms and list of ailments werent being caused by an auto immune disorder. We tried public with a pretty sound referral letter from my GP, my specialist physio and my hand surgeon. They declined me instantly. I have since learnt from the EDS Society and through a lot of research that some countries do not have specialist clinics to diagnose and treat, so finding a medical professional here in NZ that believes in it, let alone specialise, is very hard. I went to a private Rheumatologist as suggested by her and managed to get an appt 2 weeks after I called.

The appointment with the Rheumatologist was very indepth, going through my family history once again. My list of 'what is wrong with me' I think surprised her, the journey I had been on thus far and that there was 3 rounds of IVF in there too! We finally got to the physical exam part after a bit of standing, bending measuring... there was not much discussion and just answered the 'is this tender' questions with, well yes, everywhere hurts all the time! 

We sat back down and went to summarise the discussion and exam we just completed. She gave me a diagnosis of Fibromyalgia! 

Fibromyalgia is a disorder characterized by widespread musculoskeletal pain accompanied by fatigue, sleep, memory and mood issues. Researchers believe that fibromyalgia amplifies painful sensations by affecting the way your brain and spinal cord process painful and nonpainful signals.

OMG what? It was a bit surreal. I had been researching Fibromyalgia for many years as myself and my father believed it could co-exist with other disorders or diseases and we were right. Once I recovered from the initial shock, I asked what does that mean for the confirmation of the EDS diagnosis...? She said "well i agree with Anita, so today you get two for the price of one!". There was a tense moment when I asked how to explain the lack of familial links for these disorders and syndromes and she asked my why I wanted to know? I didnt have children so what did it matter? It turns out a paper is being written at the moment as I may end up being able to test for the hEDS defunct collagen gene at some point, but for now its possible I am what they call a spontaneous gene mutation. Typical!

I left the office for DH's office which happened to be next door to tell him how it went. Shocked still I tried to explain to his workmates i had just been diagnosed with a connective tissue and a central nervous system disorder and finally gone full circle to be prescribed Amitriptyline (a tricyclic). That is the one I had been I had been avoiding for some time. 

So, what has been happening since? Well my specialist physio and I have talked more as there are co-morbitities that come with both conditions, I'm yet to find out if I do infact have two of the main ones, MCAS (Mast Cell Activation Syndrome) and POTS (Postural Orthostatic Tachycardia Syndrome). I do fit the criteria for some of these, or dysautonomia in general, but definitely not on the severe side, now I can give my symptoms a name! I had heard of MCAS before as my gynecologist was the first to suggest it. I have since found that Tourettes tics and muscle spasm's are also part of the autonomic system dysfunction. Who knew? all this time? 

Next was a visit to my GP to talk about the medicine before taking it. I asked why it was prescribed and not others, why a natural approach wouldnt work and we ended up coming to the same conclusion as the Rheumatologist and I had, im so fatigued and battling a sleep disorder caused by my chronic pain that I dont have the energy to exercise to meet my increased cardio requirements to get me fitter and doing better. So reluctantly I started the meds on 1.12.21. Its time to look at the over all picture, connect the dots and plan for the future.

What needs to be addressed from a care plan point of view will be my next blog post, as lots has been happening in the time i have been away.

Im parenting two traumatised children, working full time, studying and my father died this year in the most terrible way and I have a sick brother. Some how in all of that I remain calm, mostly collected and still manage to get things where they need to be, no matter the cost. Not only do I need to treat the body, but check on my mind... so im awaiting a reply for a psychological assessment as part of my future care plan. Hunted high and low and for a someone who specialises in Chronic Fatigue / Pain syndromes... so the journey is really only just beginning!

Post-op recovery and ongoing follow-ups

So its been a while ... I'm shocking when it comes to sitting down and starting a post ... I think about what I need to post then don't get around to it, now I have so much to cover I'm going to be here a while ...

Last time I posted I had just come home from hospital after my surgery, it was a sad week that week, so much more pain than my last lap surgery, it was mostly due to the trauma my rips experienced, gas trying to escape still, the constipation I was suffering from the anesthesia, swelling and codeine, so took a bit to get back into swing of work, driving and all things housework... you know me, I dont sit down and have trouble doing what Im told.

Apart from contact dermatitis reaction from the iodine solution used during my surgery and one of my op-sites that has overhealed, I think all-in-all the healing process has gone significantly better than I expected. Im thankful for that.

So ... follow ups, where do we start? I revisited my GYN at the 6 week mark to talk through the surgery, the photos and the whats next. Was a pretty short appointment as I had healed fine, no infections, no worries. We talked about the surgery and what she thought, what she saw. She confirmed that they didn't find the Hydrosalpinx which is really unfortunate, she confirmed that she was unable to confirm the mass but was happy with the ovary removal and happy the other ovary is now in right place. Got to be happy with that right? We talked about the pics she took of the inside of my bladder and confirmed my referral had been put through to see her Urology colleague, same building different centre. It turns out referral letter never received once i called to chase up appointment, we got that sorted and a referral confirmation received with a date, I was able to get into see her before the silly season started.

The Urologist had be do a urine sample and a bladder diary prior to our appointment, she greeted me with a medical student and made sure he was ok to attend, I said absolutely as my case is always a complex one I like the students to put that in their experience folder, will help them think outside the box haha. He was just a young guy, looked more nervous that I did! 

Results were in, I had a UTI. Geez what timing! We think I might have developed that post surgery as I definitely was experiencing irritation and a little pain but put that down to having the cystoscopy during surgery. So my tests were delayed (she was planning a follow-up cystoscopy). 10 days antibiotics and off home to rest. 

She decided with all that aside she was quite interested still to know what the mass in my lower right pelvis was knowing it did not have a connected ureter, so sent me for a CT with contrast during that time too, she would have one more follow up with me where she wanted me to do a Flow Test (measure your volume when voiding and then ultrasound to see if voided properly)... so I booked in for the CT and that appointment thereafter for the test.

Well, the CT was not helpful at all, and I had an allergic reaction the iodine they put in me! It pretty much just sent us in circles again, I still get a copy of ALL my reports.

Once I was able to re-attend the appointment for my cystoscopy we got to see the inside of my bladder! I took DH with me this time ... just in case it was bad news. I could visibly see the roping my GYN was talking about after surgery, not as bad as I thought. My Urologist confirmed 'only x1 ureter' and showed be only the one opening for my left one. She decided that for my 'complex urogenital anatomy' what she was seeing was 'normal for me' and that she has no concerns at this stage that I have cancer, IC or any major bladder conditions. My bladder does overfill tho so I just need to be very conscious and she suggested to set an alarm every 4 hours as a reminder to go. Re the CT, we both just stared at each other as if to say WTF. At the bottom of the report it said to get an MRI hahaha. She sent me for a Hi-Res MRI, we were determined to have all the answers.

The Pelvic MRI that I went for was lengthy, they obviously had a lot on the list to look at. I was given a Muscle Relaxant injection about 15 mins in, which is good cause normally my bowels cause issues. Then they pulled me out at the 45 min mark to inject contrast after consulting their Radiologist, this told me they either found something and weren't sure what It was perhaps?

The results from the MRI were in quite quickly and I had a good read through before attending my Urology follow-up. Oops should of mentioned I had my right ovary removed few moths before... wow, there was some new information this time, there are a few new things on list now, possible rudimentary horn, fibrous tissue in uterine wall and biggest of all being that MASS that has had us stumped for past many years is actually a tangled mess of varicose veins in my pelvis!! The term Pelvic Congestion Syndrome has been used but yet to have this confirmed, if that is indeed what the case is, the list of symptoms matches most of my current ailments, back pain, irritation of bladder and bowels, edema in the legs, increased risk of DVT, varicose veins in the legs etc... there is quite the list. My Urologist had met with my GYN re these results and also her Radiologist and decided that they would probably not look at treating it ... hmmm not sure I agree but will leave that to the experts to decide. 

I contacted my GYN for comment on the results and she wants to see me in February, so will have another update then, in a week for so Im seeing my GP so will be interesting to see what she makes of all of this as she has given up a little, with all these specialists I now see she is reluctant to comment.

So there you have it, closure is what I was looking for - before my 40th birthday - now we will be cutting it fine, this may open a whole other chapter? we will find out soon... fingers crossed for no more surgery!

All things unexpected

You wouldnt believe me...even if I told you it was true.

The month leading up to surgery has just been CHAOS, utter chaos. Normally we have little stress pockets here and there, but just seems this month it was all on. Work projects, family commitments in all directions, trying to do it all, not that I mind as I love being there for those closest to me, but it was a juggle, a struggle and I was sure I could handle it. Which I did, in my normal stride, until 5 days from surgery when I got sick, just a head cold but all the same, it coincided with my father developing pneumonia and just about ended up in ICU again. That was the straw that broke the camels back I think. Again, I was fine, I could handle it, but my body said "enough is enough". It was time to ask work for a little bit of leave to mend myself pre-surgery. The anesthesiologist was ready to pull the plug if needed. Not what I had planned at all.

Sunday night pre-surgery was a lot calmer than last time I think as I felt ready, bit nervous still about my head cold, but ready.  My surgery was a Monday morning with a reasonable pre-admission time of 8.30am. IU chose a morning surgery as we were not sure of what outcome by surgery would have this time and thought recovering in hospital would be the right call, oh and it was! DH and I arrived early as they had wanted to see me a little earlier if they needed to perform a health check and just so happened that surgeries were on time that morning and I was called upstairs to see the pre-admission nurse.

My temperature was fine, I was so relieved as last time I had surgery I remember it was slightly elevated (both surgeries in midst of winter) so that meant we were full steam ahead. they took my height, weight and talked through all the paperwork. The anesthesiologist came by to check in and talk about all his important stuff then it was tim to get my gown, robe, stockings etc on. We were lead down to the 'waiting room' which you each get your own individual one, and given your 'relaxing' dose of x3 paracetemol. Meanwhile i had to do a pregnancy test as it is mandatory for women, results were negative, DH and I had a chuckle at that, wondered what we would do if it miraculously came back as positive! haha.

My Surgeon OB/GYN popped in to check on me prior and seemed pumped. That made me happy, we had talked about this moment for a long time! only question i had for her was "you reckon you can do all of that in 1 hour!" maybe that was the meds talking. IT was funny. She left us to wait it out until it was my turn.

I was called. This was it. The familiar walk down the hall to the operating theatre. Such a serene space with the best mural on the wall, lots of smiling staff prepping in there (haha wonder if they knew what my file looked like). Climbed on the table and went through all the procedures of anesthesia with the team, totally calm, I actually like the part of drifting off as weird as it sounds... I was not afraid.

So my operation went good and FAST! I woke up in recovery very out of it this time as think they had given me a good dose of pain meds before I woke up. My pain scale was still at a 6 though so was given a good dose of Fentanyl and IV Tramadol. Wow. wasnt in pain anymore but man, was I out of it. They gave me some water, IV fluids and checked my wounds, i had bleed quite a bit so they had to change my gown and sheets but gave me a chance to see my tummy. Very bloated and 5 entry sights! I knew then at that point that she had got it. They confirmed for me in recovery that they were able to remove my right ovary. After all these years and talking about it, it had finally happened!

I was wheeled up to my room before DH was called this time, I think because last time i was quite emotional and asked for him repeatedly. This time I seemed to cope fine but once im my room, i wanted him there so asked them to ring him. I didnt want to be wasted as in my room alone. It was a beautiful day outside so when i could open my eyes i could see blue sky and treetops. that was nice. DH arrived and was able to hold my hand while we did obs and got water into me and got me comfortable. Thank goodness for electric beds as MAN I was uncomfortable. i could not sit up though, or sleep, or eat, they ended up giving me anti-nausea via my cannular as that tramadol knocked me all over the place. All he could do is hold my hand while I rode that woozy rollercoaster, patient man that he is. They topped me up with codeine (instead of Tramadol or Ibuprofen) mid afternoon as i was ready to get up for my first pee, I had not been catheterised this time as very short surgery, thankful for that too! With help from the best nurses Ive ever had, I was up to the toilet, eating and drinking by mid afternoon and ready to sit up for some dinner by 6pm.

My surgeon popped up to see me not long after I had been in my room as she couldnt wait to tell me what she got up to while i was asleep and show me the photos! 

Laparoscopic removal or right ectopic streak ovary and tube 
(salpingo-oophorectomy) + adhesiolysis of left ovary and relocation

I was still completely out of it looking at the photos but could not believe what she was saying, DH, the nurses none of us could believe all that time my right ovary and fallopian tube were 'together'. A deformed structure that reached all the way from the base of my liver, down to by pelvic brim (hip bone) then through to underneath my uterus. All that time... there will be photos posted at some stage soon. We are awaiting results from pathology as its currently being dissected, im sure they are finding it very interesting, was a first for my clinic, not sure if a first in my hometown, hope it helps with research.

The next surprise was that scar tissue from my previous endometriosis excision had somehow adhered my left ovary to my abdominal wall, no wonder i was starting to get pain again, not sure why that wasn't picked up on ultrasound prior to surgery, but thankful she discovered that.

My endometriosis in all of this is infact pretty stable! the saving grace there was there was very little on my ureter but loads scar tissue, so she decided to leave it alone as the complications that could of happened with my only ureter were not worth the risk at this stage. It is clearly very slow moving so now I just need to meet with her at 6 week followup and decide on having the Jaydess in place, or waiting to see how long I can remain pain stable with it.

That was not the last surprise tho, on asking about my cystoscopy also performed during my surgery so confirmed there was no 2nd ureter which means the mass in my pelvis isn't piped through to my bladder, she did however discover a very unusual lining of my bladder that mimicked 'fibrous plasterboard' was the only was she could describe it. I am now being referred to a Urologist, as its about time we got my bladder and kidneys looked into properly and the possibility of that ovoid mass being a pelvic kidney (non functioning).

So recovery in hospital was to be expected overnight, i actually slept 8 hours in 2 hour lots just on paracetemol. They gave me some more codeine and morphine in the morning to get me up and through the shower and it was time to get packed up to go home. Silly as it sounds tho i made my husband take me via the mall on the way home for supplies and then push me into a homewares shop to buy new pillows (I blame the drugs but i love the pillows!). Home to try and get settled in, more meds on board and figure things out.

I was nothing like i had planned! Moving was hard, peeing was hard, eating was hard, breathing was hard. The first nights sleep was hard, especially seem we were both nervous as I had started bleeding a little. I did not expect to wake up unable to breathe either so phoned into the nurse at clinic who confirmed the bleeding was probably my next cycle that was brought on (with vengeance) which was right, but ended up down at the GP office that afternoon after struggling to clear my airways, she confirmed I had a sinus infection but my lungs were clear... to just watch my temperature and do inhalations and rinses and I would eventually come right. The pain with breathing ended up being severely bruised ribcage which is improving, and distended bowels pushing against my diaphragm until the 'gas' situation sorted itself out, but it was worth getting checked out. needless to say im still on 4 hourly Paracetemol and 6 hourly codeine until it all resolves to get me through the worst of it. Not ideal as the codeine is no good for my chronic IBS but beats the tramadol alternative!

So recovery is going well. Today I managed a day at home on my own which is good as DH already had to book an extra day due to my 'state'. Today I washed my own hair and got dressed on my own. Today is was worth it and im grateful. Today I'm thankful for my DH, for without him I would not have made it through. <3

The road ahead is unknown, im sure there will be more updates soon, but right now I marvel at the human body, modern medicine and the minds ability to just push on through even when you think you are at the hardest point, almost like it knows best.

The unknown

Today was surgery consent day and marks the countdown to my second Laparoscopy surgery. Will be 5 years to the day since I prepared for my last one. Spooky. This one will be different tho as we will possibly be saying goodbye to parts of me that make me that little bit unique, parts that zigged instead of zagged, parts of me that to my knowledge haven't done anything to hurt me, but now it's time. Time while I'm healthy, time now we've closed the IVF chapter. I don't know how I feel right now, little bit excited for another opinion, little bit scared because DH is, fearful of the unknown outcomes? My GP is confident we are making the right call and I just need to trust in that.

My full surgery is a bit of a mouthful... Diagnostic Laparoscopy with excision of Endometriosis with possible removal of Right Ectopic Ovary, with possible removal of Right Hydrosalpinx, and a Cystoscopy.

Long and the short of it is until my OB/GYN gets in there for a look we wont know what's going to stay and what's going. I have opted out of having a Jaydess placed as think I will have enough of a recovery without it to be honest and once we know what my Endo is up to we can make informed decisions from there.

I look forward to finding out the results, especially if she can access and confirm the adnexal mass on my right side as that is what is troubling me the most to be honest. Nobody likes a solid ovoid mass living inside them not knowing its origin or name?

Time will only tell. I just need to keep healthy and focused for another 35 days!

More updates to come, prior approval received. Paperwork underway... it's happening!

Mortality & Morbidity meeting

It had been some time since i posted last, that is really because i didn't have much to say other than the same old, same old. 

Recently I booked in my final fertility appointment to close that chapter for good with my OB/GYN and I wasn't quite sure how it would go, how I would feel and what the outcome looked like. It was meant to be sad? I was meant to be devastated and heartbroken? No, it felt right. It felt like my DH and I spent 2 years finding our feet, looking at what our future would look like and how we felt in our hearts. Its ok for us to be childless. 

So the visit to clinic was to talk about the 'what next'. There are specific anatomical issues that we have put off looking into, its been 5 years since they excised my Endo and since then I had x3 IVF cycles and over stimulated on each one so there has to have been some significant growth since then? Its hard when it comes to looking at the list of symptoms for Endo when it comes to me as I have chronic pain anyway, on any given day it could be spent too long on my feet, too long sitting down, ate the wrong thing, slept in the wrong position or lifted something to heavy. There is only one way to check and that is Laprascopically... again. We talked about this time looking into the contradictory scans and ultrasounds ive had over the years, specifically the one my GYN did recently that said unlikely for my right ovary to be ectopic (finally a diagnosis for that thanks to my extensive research) and focused solely on my 'adnexal mass'. 

So what is it? Adnexal mass, we know it has its own blood supply? From what i had read it cant be an ectopic kidney (kidney in the wrong place), its not an ovary as you cant have 3 of those... if you read the link above it can be all kinds of things, possibly even a tumor but you would think it would of killed me by now if it was malignant. All i know is that one of my MRI's from 2014 called it an ovoid solid mass which isnt necessarily a good thing. My previous GYN didnt want to go there during my last surgery, he ran out of time i think as my endo severley infultrated my ureter and took some time to deal with that. He did however take some spectacular photos of my streak ectopic ovary and lucky he gave me photos as then proceeded to lose them so I was able to scan and send a copy to my GYN just the other week. She thinks she might be able to take it out, depends on how it is attached however. 

So the long and the short of it is I meet with her next month to discuss and consent to my next Diagnostic Laparoscopy with possible Endo excision, possible removal of what is believed to be a Hydrosalpinx in my retro-peritoneal cavity and the possible removal of the streak ovary sitting up by my liver. There will potentially be a urologist on call incase my ureter is comprised. Nah not nervous at all?? Gulp....

Never did I anticipate being the subject of discussion among highly skilled specialists, researching my rare anatomy in their Mortality and Morbidity meeting. It had ended up being quite a complicated situation now with the emphasis put back on me to decide between a permanent IUD device and high risk surgery. I had a really good appointment with my GP about it recently and she agreed we were taking the right  course of action. 

So the countdown is on. I should have more updates in a few weeks time. 

Complex Urogenital Anatomy and Neutropenia

Yup I now have a label. Still not entirely sure what that means but know its definitely not conducive to fertility and going to be costly for me. Outcome of my follow up with my OB/GYN was that she agreed my latest ultrasound didn't tell us anything new and really nothing at all but was a good conversation piece when we were talking about IUD vs Surgery. Now that I have finished treatment there are things we need to decide and one is my path moving forward for Endo, my other random anomalies (ectopic ovary, hydrosalpinx and my kidney remnant and if they create risk, surgery is risky, a Jaydess (smaller form of Mirena) may pose its own problems. My OB/GYN was also curious to see if I was entering early menopause by running fertility bloods...my AMH came back at 42 (was 44 in 2015) so has dropped but is still double what it was when I did IVF?? Everyone keeps telling me that is fine, but I don't actually think that is based on what I have read, for my age that is what we call higher than average... can be a precursor for PCOS or granulosa cell tumors  so lets hope its just really that I'm abnormal haha. She is getting her ducks in a row so she could take my 'complex' case to a multiple disciplinary meeting the following week. Also wanted to wait to see if my Neutropenia has improved, currently i don't have an immune system ... now that is a whole other story!

Neutropenia is a blood condition characterized by low levels of neutrophils. Neutrophils are a common type of white blood cell important to fighting off infections — particularly those caused by bacteria. For adults, counts of less than 1,500 neutrophils per microliter of blood are considered to be neutropenia. 

Mine are borderline but at this stage we wont worry too much unless they dip below 1,000. Latest results from past 12 months.

Based on the SCL range of 1.9 - 7.5:

24.01.19 = 1.7

11.01.19 = 1.5

30.11.18 = 4.3

07.06.18 = 2.1

24.05.18 = 1.6

20.05.15 = 1.9

07.08.15 = 2.1

So situation is that we don't know what is causing the issue, whether it is chronic or if it will get better on its own so we will be starting 3 months tests at least till it has improves, obviously its not low enough to make me really sick but if if heads that way i might need medication, not much i can do to improve it at this stage. I'm getting some bloods tomorrow to see if we can figure out a cause? the test will be for the below (don't stress about some of those, I'm not! I think they are just routine)...

Anti-nuclear antibodies (ANA)
Rheumatoid factor
B12
Folate
Serum protein electrophoresis (SPE)
HIV
Liver enzymes
Hepatitis B

In the meantime I saw the GP today to talk about my low BP (Blood Pressure/Hypotension). We have decided that the number isn't as much of an issue than the symptoms... so will be keeping eye on my dizziness, fluid intake etc moving forward. Its been ranging from 88-110 systolic so that is now my new normal.

What's considered low blood pressure for you may be normal for someone else. Most doctors consider blood pressure too low only if it causes symptoms. Some experts define low blood pressure as readings lower than 90 mm Hg systolic or 60 mm Hg diastolic. If either number is below that, your pressure is lower than normal. 

We also talked about the reply from my OB/GYN that we happened to get during the appointment, she is still not entirely happy with making a decision at this point until they have researched a bit more. I am the first one they have seen at their practice with a Unicornuate Uterus with Renal Agenesis and an Ectopic Ovary. I was discussed at their Collegial Morbidity Meeting with my previous OB/GYN and even he said that he would like to research more. They are unsure they will be able to safely remove my right ovary, whether it has a risk for being malignant. We may be starting with a Jaydess sooner rather than later and seeing how we go, its the pain that Im worried about, I could do with less of that.

So - there will be a few more updates to come, especially once these bloods come in, who knows where this journey will take me next.