Marion's Story Type B AD

It took me a while for me to get round to sharing my story, writing it down seems somehow to give my experience substance, but if we don’t talk about it who will. I am a successful 52 year old woman living in a small village in the north of England; I have 4 children, 6 grandchildren and hopefully no more on the way.

I woke up on June 6th 2008, life was good my new business was growing and after years of working hard to bring up my family, my husband and I were on our own and looking forward to the new chapters in our lives. I was enjoying the beautiful view of our garden from the bedroom window when a felt like I had been hit in the chest by a heavy weight boxer, I fell back on the bed and felt a tearing sensation move into the top of my thigh. My husband Richard was downstairs, he always brings my first cup of tea in the morning, I lay still and waited; I think I’m having a heart attack I said to him as he walked through the door, he phoned for an ambulance, I waited for other symptoms to appear but, no, just an excruciating pain in my back. The ambulance arrive and paramedics gave me aspirin checked my heart and blood pressure which were both “normal” but suggested I should go to the local hospital to be checked out the time was 9.30 am.

I arrived at the local hospital where I was seen in A&E, had another ECG and a chest x-ray by this time the pain had almost gone, but the A&E doctor still sent me to the ward for observation and we now both felt like it was just “one of those things” I sent Richard home. My blood pressure began to rise and the pain returned, I was given a drug for angina but nothing was helping the pain in my back which had returned with a vengeance I got out of bed to seek help; Luckily for me a young Registrar at the hospital, suggested that I may have suffered an AD, something I had never heard of, and arranged for me to be taken to a hospital with a CT scanner to check, by this time Richard had returned to be greeted by me being put in an ambulance to travel blues and twos to a hospital about 20 minutes away.

I arrived at the hospital at 7.30pm that evening where a Consultant Radiologist was waiting to do the scan, he told Richard and myself that we should prepare ourselves as I probably wouldn’t survive the next three days, I had a Type B descending dissection which had travelled from aortic arch just below the left subclavian into the top of my left thigh. Frantic phone calls were made and I was taken to the ward where the fight began to bring my blood pressure down below 140 and keep it stable. My day ended with me hearing the staff nurse and pharmasist who were trying to bring my blood pressure down with a cocktail of drugs, they came backwards and forwards it seemed to go on for hours until eventually I remember them saying, "nothing seems to be working just give her everything" at which point I remember throwing up; before I drifted off I heard them saying "thank god it’s working her blood pressure is coming down."

If you can be lucky in these circumstance I certainly was, although my aorta had dissected the dissection had torn back into the true lumen in my thigh so there was no aneurysum. Tests during the first week confirmed that none of the major organs had been compromised, I had good kidney function my heart was not affected, in fact when my heart was examined I was told there was little damage it was slightly enlarged but that was to be expected with the high blood pressure and would return to normal once that was under control, so the major issue for me was to stabilise the dissection by lowering my blood pressure which at some point had been well over 200/120.

I spent a week in the heart unit in that hospital, however because there was little experience at the hospital of treating dissections, they were constantly in touch with Professor Scott and his thoracic team at Leeds. Exactly a week after the initial crisis I suffered a further problem when I nearly died drowning in my own body fluid; at this point I was transferred to the specialist unit at Leeds, where after more CT scans it was decide that if the dissection could be stabilised. My treatment would initially be medical. I spent a further weeks, tied to the bed by drips and feeds, the drug leaving me confused and hallucinating often, everything from flying beds to butterflies; eventually it was decide my medication could be managed orally; my lovely man took me to the showers where I sat in a chair having to be helped with the menial task of washing; I couldn’t do anything for myself but I was still alive. I return home after 3 weeks on a cocktail of medicines whose affects would have pleased the most demanding junkie

Living with aortic dissection has been an extremely lonely path, you don’t meet others who have suffered any type of AD and survived, if you do research on the internet, you become even more scared, and when you tell people what has happened to you because AD is not well published they really have no idea, even health worker haven’t come across it; so you fall between the cracks of our health care service, everyone keeps talking about my heart condition, and in my case it isn’t a heart condition, in fact people never stop telling me my heart, kidneys and liver are working really well!; so it been hard to find the aftercare that someone who had a heart condition would, on the other hand once I found myself at a Major Teaching Hospitals I have been under the care of one of the leading thoracic surgeons in the UK and could not have wished for better care, amazing, cutting edge is the phrase but outside that environment!!!!!! as we are all only too aware we are indeed the lucky few.

My GP has admitted that she has no experience of caring for someone with my condition and insists that I go directly to my specialist if things go wrong. I resorted again to the internet for advice but the outlook on most sites is so bleak, I gave up!

Not understanding what you are going through, with the drugs and the pains and twinges is very frightening and even after surviving the first ten month I was still surprised that some morning I managed to make it through the night. Depression and desperation in the early months seem to be the companion many of us have during the bleak times, as we fight with our blood pressure and the side effects of the treatments. At one point I lost feeling in the left my left arm and side but after 48hrs it returned, everyone shook their head and had no idea! My husband has been fantastic; his positive attitude has kept me going through many of these dark days.

I have also trod another path that I haven’t heard anyone talk about, I have seen a Homeopath for the last 20years and their holistic approach have helped me with the side effects of my treatment and helped in managing my emotional well being, which in turn has support the doctors who in reality are focused on my physical well being which has been of great help to me. At Christmas I was having a great difficulty walking on my left leg, it became exceptionally weak, I began having some “Reiki Healing” which was also extremely helpful - as my lovely husband says “if it help you, that’s all that matters”

In January 2009 I was told that the size of the false lumen was growing and I was told that they would like to enlist me for surgery to mend the tear. There were two procedures which needed to take place to give me the best opportunity to survive without serious side effects the odds weren’t good about 20%. My surgeon had decided that I was a good candidate for stent repair surgery as they were unable to stabilise the dissection and I had been turned down for open chest surgery as it was felt that the risks were too high, my options were very limited. My surgeon was planning keyhole surgery, and in order to reduce the risks associate with stents being place on the aortic arch he planned to do some preparatory surgery to reduce the risks.

On the 20th May I returned to hospital and where they performed a double carotid bypass as MRI scans have showed that the Circle of Willis in my brain was incomplete therefore in order to secure blood flow to the left hand side of my brain and my left arm during the stent surgery they are going to secure a secondary flow, once completed six weeks later they would perform the stent surgery, stopping my heart and re-starting it to ensure the location of the stent in the aortic arch, they will then decide if any further stents were needed, this was the first plan which changed dramatically before the surgery.

This first procedure is took place because my surgeon was cautious and was determined to achieve the best outcome for me; the tear in my aorta is exceptionally close to the left subclavian artery and therefore they were concerned that the stent, if they were able to put it in place, might be insecure and need repeating at a later date. This way they will be able to place the stent over the left subclavian artery and even if the blood flow to the carotid artery was interrupted there would be no loss of blood flow to the brain and the left hand side of my body, this would also achieve a tight seal at the point of dissection, while still ensuring an unobstructed blood flow to both my brain and my left arm, does that make sense?

I hate hospitals, I know I needed to go but the memories of June last year loomed large! I also knew this is only the first time, in six week time I would return for the stent surgery which causes me a small amount of anxiety, not so much the surgery but the after effects.

I had grown use to the Idea and felt really positive, I think in many cases it’s harder for our loved ones to cope with than us.

The surgery should have taken about 2.5 hours and recovery in hospital about 5 – 7 days; in fact it was the other way round the surgery took seven hours and I was home in two.

I have a scar from ear to ear almost, but miraculously I suffered none of the side effects that we had endlessly talked about before the surgery, these included:
• Loss of facial movement
• Short term loss of speech
• Loss of tongue movement
• Eye nerve or muscle damage
• Loss or reduced use of the left arm
• Damage of nerves to the diaphragm
I still had to go for tests but everything looked good, and the way was now open for the sent surgery at the beginning of July.

I know we often get frustrated with both our health and the long involved tests, treatment and surgery we all have to undertake in order to hang on to this mortal coil, I am once again I am amazed by the skill and expertise of the surgeons who care for us. My surgeon sat on my bed the day after the surgery and I was in no doubt that this man is doing everything possible to ensure that I have both the best quality and quantity of life available to me. I thank God that these people exist and that through luck or good management we arrive in their care.

My left arm was still very weak and my core strength much reduced but I felt remarkably well. As always I saw the looks on my family’s faces and wished I could do more to take away the fear and worry they endure, I am truly blessed to be loved in this way. Since I came out of hospital we celebrated my daughters 30th, my lovely husbands 46th and our second wedding anniversary, (we have been together nearly 20 years but, decided to marry two years ago on Memorial Weekend in Hawaii, and I’m so glad we did!)

Two week after my first operation I wanted to reflect on the positive effects of these first changes. I was told that this operation was in preparation for my stent operation at the beginning of July, and that there were no apparent health benefits, the graft surgery was to improve the chances of the sent surgery being completely successful by lowering the risks involved. So when I returned home and my family started to say how much better I looked and how the light had returned to my eyes I was a little surprised, the thing that I noticed almost immediately was that I seemed to be more awake, and my brain seemed to be working better! The second more surprising element of the closure of my left subclavian is the reduction in pain, I used to get almost constant pain from my dissection but now it’s only very occasional and takes me by surprise as I become used to living without it!

I don’t know what interest this is to anyone else but if we don’t have options to talk to our surgeons about or be able to describe other treatments that we have heard about, we as patients are in a vulnerable position.

As you all know by now I was due to have surgery the first week in July but I was actually called into hospital on the 30th June and my operation took place on the 2nd July, and I return home after a little over two weeks.

I don’t know where to start really because I’m still in shock, I had been living with my AD for thirteen month and I woke up on July the 2nd at 7:30pm after 7 hours in surgery and the tear in my aorta had been mended. I never thought I would see this day and I really can’t believe my luck so forgive me if I seem a little disconnected.

I know you all want to know how, so I will fill in all the details but if anyone wants to know more I will be happy to explain further. The scan they took three days before my surgery revealed that the tear was increasing in size and was making its way further down the left subclavian artery and so to ensure a complete seal to the stent they were about to fit they further closed off the aorta branch to the left subclavian which had already been diverted by graft surgery to the left carotid artery. Then they carefully set about managing the pressure in my spinal fluid to avoid paralysis during placement of the stent. The sent which I believe was specially made, was inserted into the acceding aorta in the right thigh passing through the heart and placed directly over the dissection closing further blood access to the false lumen.

The blood in the false lumen started to clot immediately and I was taken to a HDU so I could be fully monitored for 24hrs.

I woke in the HDU my surgeon, god bless him, at my side again slowly going through a check list of questions to ensure I hadn’t suffered any permanent or temporary neurological damage during the operation. I must admit the first thing I did on waking was wiggle my toes to make sure!

24hrs later I return to the ward, over the next two weeks I suffered from temperature spikes, and was treated by a micro biologist who confirm that I didn’t have any infection, the temperatures were caused by my body dealing with the large amount of clotted blood in the false lumen, which must have been quite a large quantity as it was decided on day seven to give me two units of blood to support my body’s return to health.

I was still extremely weak but I’m home, breathing better and I can sleep on my left hand side!!!
Mentally I was all over the place, I couldn’t wait to really begin to feel well but in my last meeting with my surgeon he told me in no uncertain terms that it will take about 12 weeks for my body to recover from this massive attack and almost a year to reach full recovery . I had great difficulty believing that the surgery has been so successful a CT scan taken before I left hospital showed that the repair was in their words “perfect”; I have spent the last thirteen month coming to terms with living with AD it almost too much to bear to believe that I have been given this fantastic gift.

Three months since my stent was first fitted I returned to see my surgeon, I had a CT scan about six weeks after surgery to ensure the stent had embedded it’s self firmly in place, it is titanium and designed to hold the internal walls of the aorta open under slight pressure. I was told at the stage of the CT scan there was slight pooling at the end of the stent that they were keeping an eye on, but they were hoping it would be absorbed, and that I didn’t need to be seen by him for six months!!

What I think is really worth mentioning is that within three weeks of the stent being fitted by blood pressure dropped dramatically and I don’t just mean a few points, after taking the prescribed meds I had been taking for the last 13 months, I was shocked that these same meds now induced loss of vision for a short period of time and spent hours in a zombie like state, I knew I had to do something before my appointment with the cardiologist at the beginning of October and so with the help of my Doctor I began to reduce my medication substantially, over the next few week I took out two types of medication and reduced a third by half. My blood pressure was still under 120/60 most of the time but we both feel it is important to get the cardiologist input before we took it any further. My surgeon has also recommended that I attend a cardio rehabilitation program as private fitness centres are reluctant to help me get back in shape!

I already knew how lucky I was, within two week a shower, where I had to wash and dry my hair and get ready, would have taken me an hour with my poor husband being call to towel dry my hair, I found I could manage in half the time and didn’t need to lay down and rest once, it is all too good to be true!

The appointment with the cardiologist came and went with a plan to reduce my medication even further, wow I thought this is great, I’ll be on just blood pressure meds in no time, but oh how wrong I was – coming off beta blockers has been one of the hardest things I’ve ever done, even a small reduction in potency was met by sweats, shakes, palpitations, anxiety attack which blew my blood pressure off the scale for a while- very frightening but I have at last taken my last tablet and I’m hoping that the side effects will disappear over the next three weeks.

I am now felling much better, in fact I can’t believe I’m saying this, but sometimes I even forget, but twinges and short comings quick bring me back, but wow. I know I will never return to my former life style (nor would I want to) my career as it was is also beyond my present capabilities, but I can at least get back to my correspondence and my endeavours to support research into our condition, I am also looking forward to spending time with the British Heart Foundation helping to improve awareness both within the medical profession and the general public.

As for earning money I know I’m going to have to do something soon but I also know that there are much more important things and that the balance I didn’t have before, maybe I needed this wakeup call, well let me tell you I heard it loud and clear I won’t make those same choices again.