Emma's Story (Aug 11, 2013)
I was diagnosed with SLE formally in 2008 and my case seems to be extremely rare. As you can imagine its very frustrating and confusing at times.
I should probably go back July 2007, when I approached my doctor about feeling unwell. For the previous 2 years or so I had noticed a hormonal like weight gain, particularly on my face and stomach area. Also the skin on my face mainly my chin area, became increasingly worse with spots although my GP said it was not enough to determine as Acne, which I had never suffered from before. My cycle was also very irregular but they had always been this way and were controlled by an oral contraceptive pill. I complained of tiredness and joint pain particularly in my wrists. Unlike most people at the start of becoming Ill, I already knew I had SLE (Systemic Lupus Erethamatosis) more commonly known as Lupus, an uncommon disease. My mother had been diagnosed with this in 1994 but is completely under control with no side effects with medication. However my GP referred me for an ultrasound on my ovaries as she thought I may be suffering with polycystic ovaries, this happened late Jan 2008.At this time I also gave bloods.
On the 17th Feb 2008, my father died very suddenly and unexpectedly at 46 years of age from Sudden Adult Death, it was a very stressful and painful time and a time where my emotions were very much uncontrolled and all over the place. The following week I visited my doctor for the results of the ultrasound and blood tests. While yes there were some cysts on my ovaries there were not enough to classify as polycystic ovaries. However, I had a positive ANA (blood Type) and with the family history of Lupus my doctor felt it was wise to see a rheumatologist. The following week Weds 5th Mar I attended a Rheumatologist in a local private hospital. He went through the 11 signs of SLE and on the basis of family history; tiredness joint pain and blood results diagnosed SLE and put me on plaquenil.
By the following Friday I began to feel very unwell. On Monday 10th March I had a strange taste of blood in my mouth, overwhelming tiredness and headaches. The following 2 days I became much worse, vomiting and sleeping almost 24 hours solid. The morning of Thurs 13th Mar I woke at 5 am, my bed and floor was covered in blood. I made my way to the bathroom and what greeted me in the mirror was like a scene from a horror film. Massive blood clots were coming from my mouth, nose and eyes. My body was completely covered in bruises and I had little red spots all over my skin, I mistook this at the time for bruising, when in fact it was my blood had completed become clots and were trying to push themselves up through my skin. When I opened my mouth I could not see my teeth as my gums had swollen so much they were completely covering them. I got straight into the car. On arrival at the accident and emergency in Naas General Hospital, I deteriorated even further. First doctors thought it may have been an allergic reaction to the plaquenil medication, however about an hour later a doctor approached myself and my mother. My platelets had dropped to around 12,000 (Normal Platelet count 350000) and continuing to fall, my HB was 3.2 (Normal 12) and my kidneys were going into failure, LDH and Creatnin were fast shooting through the roof. The A&E doctors were baffled, they were looking up online my symptoms and they rang the haematology Department in the Adelaide and Meath Hospital in Tallaght for consultation. I was then diagnosed as being a critical Thrombotic Thrombocytopenic Purpura patient (TTP), a condition neither my mother nor I had ever heard of. Naas general hospital was not equipped to deal with me but right then I was too ill to transfer, they said had been placed in an ambulance I was a massive risk of a fatal stroke or haemorrhage at this stage. After 24 hours , 2 units of blood and 4 bags of platelets I was transferred to The Meath and Adelaide hospital (Tallaght) where my nightmare was really only about to start. I had a central line inserted to a main artery in my heart though my neck where I underwent treatment called plasmaphereisis. After 17 plasmaphereisis and 4 weeks fighting I was finally allowed home. This was all done under the guidance of the Irish Blood Transfusion Board. Plasmaphereisis is a procedure in which a machine is attached to your body very like Dialysis, all your blood is removed from your body and “cleaned”. My bad platelets or plasma was being removed and I was being given donated platelets. At the time platelet stocks were very low in Ireland and my platelets were being flown in from the USA every day. The donated platelets I was receiving was called Octoplas.
The really ironic thing was that during my time in hospital, it was concluded the SLE diagnosis was a wrong one, that in fact I wasn’t an SLE sufferer. This was something I wasn’t entirely convinced about. TTP didn’t make sense to me, it was so rare and unusual I kept thinking, why me? Having been told that Lupus and TTP was even more rare together, and there was only about 6 known cases ever recorded in Ireland so there was no way I could have this. I resigned myself to the fact that I may live with this TTP and try and learn about it. I think because my mam has such mild Lupus and I knew something about it I would have been a much happier person had I been told it was an attack of Lupus. I understood Lupus to a certain degree but TTP was completely alien to me. I was also told at the time that in Ireland there may only be less then 1 idiopathic TTP case a year, this was information I couldn’t handle at all. On top of that I had every Doctor and student Doctor wanting a piece of me to see this TTP in real life. My medication was the dreaded prednisilone steroid, aspirin, folic acid and blood pressure tablets. As it is with pred I ballooned up, I felt fat frumpy, ugly, I started getting horrible hair on my face and I felt like my face was bigger then 3 footballs, if I fell over you could roll me like a barrel. After 4 weeks in Tallaght hospital I was discharged on the condition I had to attend the outpatient cancer unit every 3 days. There was no specialised unit to look after what I was suffering from.
During the summer months I gradually built up my strength and my platelets remained stable, always around 220 - 280. Sometimes I would get an unbearable pain in my wrists, like they were broken and very bad headaches. I attended the Haematology clinic where they found my platelets to be 149 which being at the lower end of normal they weren’t too worried about it. I spent hours online, searching every available TTP website or information I could come across. There were no support groups in Ireland, the nearest group to me was in the UK. One woman from Cavan contacted me to say she had been searching for years for another TTP patient in Ireland. Her sister died of the condition in July 1998 and she was desperate to speak to someone who understood what it is. I felt very alone.
Early November I began to feel very tired all the time. I put this down to the fact I had just recently increased going back to work from half days to full days. For the duration of 2 weeks I had a constant headache. I was living on solphedeine. On the 21st Nov I attended a 30th birthday party and had maybe 5-6 drinks, not a huge amount for me!!!!!! The following 2 days were hell, vomiting and I couldn’t stay awake. On the 23rd Nov I woke up still sick and found my legs completely covered in bruises , immediately I phoned the hospital and informed them I was 99% sure I was at the start of a TTP relapse as I had been warned previously the chance of a relapse was quite high. Admitted to hospital I spent the next 5 nights waiting for some answers on what was happening to me. My platelets had dropped to 87 but remained stable there. My appetite was gone and I was vomiting but not, it seemed having a relapse of TTP. I began to feel a lot of pressure like feeling at the back of my head and more bad headaches. After an MRI scan it revealed I had inflammation on the Brain, at this stage I also had a bit of inflammation of the kidney and substantial amount of protein in my urine. Doctors were baffled as it was clear TTP was not making a return here. After a 24 hour urine sample and some more blood results I got the news I had known for the previous 2 years. Fri 5th Dec it was concluded I was in fact a SLE patient and this was an attack of lupus. They conducted a lumbar puncture to see had I an infection in my brain fluid. I was started on an IV steroid and I went to sleep in hospital in good spirits and relieved that now I knew I was a "LUPIE".
I always knew myself I had lupus and finally the doctors were listening to me and diagnosed it. This made me so happy as I could deal with Lupus. My views on Lupus were very distorted at the time. I had lived with Lupus through my Mam for 14 years. Lupus to me was not a deadly disease, it was “a problem” that caused joint pain. In my eyes it was a very mild disease. How wrong was I? None of us, me, my then boyfriend (who is now my husband) my mam or all my friends and family, realised at that time just how serious it would get. Sometime that night I suffered the first of 5 brain seizures. I woke to find I had gone completely blind. Forgive me because from now on I don’t know all the details and my memory of the time is almost nil. My Blood pressure rose to the very high Hundreds and they could not get it under control. The brain seizures had left me in a coma like state, I was immediately transferred to the intensive care unit in Tallaght and my family and boyfriend told I only had a 10% chance of seeing through to the end of the day. They worked all day to control the seizures and blood pressure and to try and get some kind of kidney function back. Nothing was working.
As a last resort it was decided to give me a few plasmapherises sessions to boost my body, the first time this was ever done in Ireland for a lupus patient. They hoped it may kick start some reaction. They pumped my body with anti seizure medication, steroids, antibiotics, blood pressure medication and ACE inhibitors. It was very touch and go for 3 days; they were finding it extremely difficult to control my blood pressure. However thank god after what must have been hell for my family, I started to pull through and show signs that the seizures had not left me permanently damaged.
At that moment I was still in the acute stage. A renal biopsy returned that I had between level 4-5 kidney damage (this classification is normally people who are at end stage renal disease) and will have significant scarring for the rest of my life. None of the medication they were pumping into me was working, nothing was kick starting my Kidneys back into life and they could not find a way to get my blood pressure down from a critical high. It was then the decision was made that I started on intravenous drug called cyclophosphamide. The Doctors came to my room late on a Monday evening and explained about this immuno suppressive treatment they felt was the only thing that would save my life. I was nodding along not thinking anything more. It was only when my Mam asked “when you say immuno suppressive are you talking about Chemotherapy” that I actually realised what was going on. This frightened the life out of me. I only ever thought chemo was for cancer patients. That night we were told I had to give my consent, I had to sign forms to agree to the chemo, it could affect my fertility, I may never be able to have children and they weren’t sure if this would even work. In my eyes I didn’t have a choice, if I hadn’t gone down this route I wouldn’t have survived this attack of lupus. Other medications included intravenous steroids, blood pressures meds, anti seizure meds, aspirin, folic acid, antibiotics and the usual SLE meds.
It was 2008, nearly Christmas time, my Dad was only dead 9 months. I remember looking at that purple bag in the critical care unit and just breaking down at the thought of me, a 24 year old on chemo, for something I had no control over, they WHY ME started.
I was so ill from side effects of chemo and all the other meds. I went down to about 8 stone, had no energy, couldn’t wash, dress myself, feed myself. I couldn’t go to the toilet and had to use a catheter. The next 6 weeks in hospital were up and down, one day id be doing really good, the next it was the docs telling my things weren’t so rosy anymore.
I woke up on Christmas day in Tallaght hospital and I thought my life couldn’t get much worse. However my wonderful boyfriend proposed to me that day so for many reasons it will be a Christmas I will never forget.
The next few weeks were crazy. On week 4 of the chemo treatment my whole body broke out in a red blistering rash. It was agony to lay down, agony to put my bed clothes anywhere near me, I felt like my whole skin was burned raw. I felt I could not go on with the treatment anymore, the only thing that made me continue was the fact that it seemed to be working. Then the best news ever, I was eventually let home. I still had to travel up and down to tallaght hospital every 2nd day and I was still receiving chemo.
Eventually in March 2009 I was given the great news that I could stop the IV chemo and replace it with a tablet form, TABLET I WOULD BE ON FOR THE REST OF MY LIFE!!!! More why me.
After more tests and biopsy’s and poking and prodding all my consultants started putting the pieces together. I have a double stranded DNA, while they say lupus is not hereditary it’s very coincidental that my mam has it and gran aunts have history with arthritis. I am under a team of 5 consultants including Nephrology, Neurology, Cardiology, Rheumatology and Dietician. My main consultant is my Nephrology Consultant who is fantastic.
Unfortunately in my case lupus attacked me in the worst possible way. I have both systemic and discoid.
My skin can get so ugly, my face is red and blotchy all the time, im a big fan of make up and sally Hansen spray on legs!!!! I have grade IV Lupus Nephritis, my neurologist insists that lupus is active on my brain hence the brain seizures and in the last 3 years new black spots on my brain are evident on MRI scans. My memory is effected and my husband is always slagging saying I never told him things when I think I have (I reckon this is just an excuse though)!!!!
Overall im happy with life, I survived and im glad I lived to tell the tell. I have had such good times the last 2 years, getting married, learning how to cope better with my illness and try and accept the fact that I do have this and its not going away.
Its very frustrating knowing an attack can happen again at any time but that’s part of my life now. I have the most wonderful team of doctors looking after me. My main consultant is a wonderful person. They do not advise people like me to have children but she is fully supportive of myself and my husband taking this rocky road but has asked us to wait a couple of years. I am in the hospital every couple of months and my medication list is unreal with a cocktail of 15 different tablets up to 3 times a day.
All the medication are my new best friends for life. Sometimes I wonder why I need them as days I feel so fine, then im reminded im only feeling fine because of these tablets!!! The joys.
I have constant pains in my hands, arms and shoulders. I don’t know what it is like to be free from headaches. I can’t ever just go away on a spur of the moment, everything has to be planned weeks in advance. I will never be able live in another country as I am tied to the Health system in Ireland for the rest of my life.
I reacted to such a life changing thing in a very strange way. As I got stronger physically I started to push the severity of my situation to the side. I looked at what had happened to me as just another normal thing. I buried myself in organising our wedding and moving into our new home. I also became extremely focussed on work. I pushed myself to the limit. If anybody asked me how I was doing the reply was always the same, grand. I never talked about it, I never said when I wasn’t feeling well. In fact I never even let myself think about the days when I wasn’t doing good. It was all pushed aside. I started to block everything out and pretend I didn’t live with this disease. I was afraid to talk about anything as I felt people would think I was only looking for sympathy. Again I felt extremely alone.
In January 2012 my husband came home from work and said he had heard a woman on our local radio station talking about Lupus and this new group that had recently been formed, Lupus Group Ireland. I searched online and found their Facebook page. From the very first moment I contacted them I was so extremely overwhelmed. Here was a support network, a whole family of both women and men who also suffered from Lupus. I gave a brief outline of what I had gone through and the support and words of encouragement was unbelievable. Here was a place I didn’t have to hide or feel ashamed. I found out that another lady Jessica O’Bryan had set up the group. She had been diagnosed with Lupus in 2011 and like all of us had searched many times online and found no real support or advice for people with Lupus in Ireland. She set up a Facebook page and in weeks it had exploded.
I began speaking to Jessica more and in May 2012 she asked me would I publish my story for the group as part of Lupus Awareness Day. I agreed and put pen to paper. My story was published and the reaction floored me. From complete strangers to family and friends, their heartfelt messages of love and support left me in an emotional tumble dryer. Shortly after this a lot of what I had blocked out started to come flooding back. I couldn’t sleep, I couldn’t think straight. My thoughts were all over the place. I was starting to find it difficult to leave my house. I didn’t feel safe or secure anywhere unless I was at home with my husband and dog. Things came to a head when I was away for one of my best friend’s hen party. The first night of a 2 night trip I had a panic attack. A near emotional breakdown. Under the guise of not feeling well I went to my bed early that night, rang my husband and cried on the phone for a solid hour. We both agreed I needed to talk to someone and get this sorted before it got any worse.
After another emotional breakdown with my consultant a few days later, she referred me straight away for intensive counselling. Looking back we all agreed I had been dealing with everything that had happened far to well. A reaction like this was always going to come sooner or later.
Counselling was an experience to say the least. In the space of 4 months I went through feelings and emotions I had never dealt with before. I grieved for my Dad. I grieved for the life I felt I lost for myself, I grieved for a future life I will never have. I learned that my body had gone through something so traumatic physically that I was lucky to be living such a normal life now. We delved so far into me, my mind and my thoughts. I had been channelling all my emotions and drive into one area, work. For the first time I recognised that I had to spread out priorities for my own well being. I accepted the fact that I nearly died. My counsellor gave me the confidence to say it out loud. He showed me that is something to be proud of instead of shy away from. Today I am in a much better place but my journey is far from over.
Lupus Group Ireland gave me the one thing I could never give myself. Courage. I often wonder now had Jessica not set up the group would I still be living in a state of denial. One of my proudest moments was when Jessica asked me to become a committee member for the group after it had been given charity status. I was both honoured and humbled. I am now on the committee in the capacity of Spokesperson and PR Manager. I feel I can give something back. We are determined to raise more awareness and acknowledgment for the many Lupus sufferers in this country.
Our main aim is to support newly diagnosed Lupus patients and be an ear and shoulder to cry on for any Lupus sufferer who needs someone there. We want to create awareness for this deadly disease and make the government stand up and listen, get Lupus recognized as a Life Long Medical Condition which will entitle patients to Health Service Benefits that are only available to a select few. We have recently got on board with a panel of doctors and scientific researchers and we hope to raise some much needed funds to develop more on this. We will succeed on bringing this to many people’s attention.
Lupus nearly took my life but now I am taking it back.