Alison - Dual Transplant
ADPKD - I live in the UK and received a dual transplant, both liver and kidney in the UK
My name is Alison. I had a dual transplant, both liver and kidney transplant in the UK. At present I am 56 yrs old; I have two grown children and 5 wonderful grandchildren. I live in London and was diagnosed with PKD & PLD in 1987. My daughter was also diagnosed in 1987 with PKD & PLD, she was 6 yrs old and my son was negative for both, he was 4 yrs at the time. Also one of my brothers has PKD/PLD. The picture to the right is of my PLD cystic liver. Below this is a photo of me in the hospital recovery from surgery.
My story begins with " How did I get to the diagnosis " well it was the break up of my marriage, which led me to seek some counseling. During one of the sessions I was asked about my father, he had died at an early age, 41 yrs (1959 ). So I was asked if I could find out, which I promptly did, my mum wrote down what was written on his death certificate Bilateral Polycystic Kidney Disease. She also wrote down that my uncle's were troubled with kidney problems, alarm bells started to ring in my head. Something is not quite right here. So off to our family GP, went for ultrasound scans and results were myself & Jodie positive for PKD. Nicholas was negative. I was in a state of shock, had never heard of PKD or PLD. All sorts of things were buzzing around in my head: what is it ? why me ? what will happen to us ? and even though you are told " nothing will happen to you for quite some time " it does not stop your mind from working overtime. So I thought to myself " stay strong and deal with it ". Next step renal consultants.
Jodie and myself were both then referred to Guy's Hospital, she saw Prof Chandler and I saw Prof Cameron. I must say that they were very good and informative. Again we were told nothing will happen for a long time and that we needed to be seen once a year, also to stay healthy, drink plenty of water. It was at this point that my consultant stated that my Liver was not a problem as it would not fail,and that was the only time my Liver was mentioned. So now its regular checks once a year for life, which was fine, the only thing which irritated me, was that every time you went to the Hospital you saw a different doctor and you would have to repeat all of what you have already stated, that drove me mad. This has changed now, thankfully.
Over the next 10 years which takes us to 1997/98. I removed salt from my diet and I also did a family medical history, it was amazing to find out who had PKD, it ran right through lots of cousins with it, some were lucky and were transplanted; others unfortunately were not. However, I remained positive. In 1990 I developed an ovarian cyst. It was drained but there was a possible malignancy so had to have the right ovary removed, one question that dominates here is, is there any links to PKD & PLD they told me no, my instincts tell me maybe. During these years my blood pressure started to go up, and of course high BP and kidney disease are not good, so I started medication just a small dose to start with, regular checks with GP and more meds plus an increase in dosage, I think I was on about six tablets a day, which did increase to 12 a day, it was 4 of this and 2 of that etc. And yet I felt absolutely fine. Then I came across a photo that someone had taken of me it was a sideways view, I thought hmm looking a bit fat around the middle, and I had put quite a lot of weight on at the time, and the PLD didn't even enter my thoughts, however, I was asked if I was pregnant and when are you due ? how embarrassing. I have lost count on how many times that has happened. So I guess I did what many others have done and that was to hide my abdominal bulge under baggy T-shirts, I also would not look at myself in a full length mirror. As the following years rolled by my bulge was getting bigger, and my renal consultant put me on Furosemide ( diuretic ) my weight went down as I was storing too much water, unfortunately it made my bulge look even bigger. I am sure that I am probably no different to any others that have PLD, will do anything to cover up, even if it is baking hot outside, I must say at this point that my PLD was not causing me any pain or discomfort.
From 1998/2008 is the time when things started to develop, but only slowly. T-shirts were not as baggy as they had been. I had to start wearing & buying maternity clothes. My upper arms and upper body became very skinny and I lost muscle. And yet I was still trying to hide my enlarged liver, my kidney function was decreasing, but I felt fine, a little tired at times but I was not unduly worried. To be honest I don't think anyone was concerned at this point, I used to see my GP every 3 months for a check up and medication, no mention of PLD would only ask when I am next at Guy's. To be fair he didn't
know much about PKD/PLD which he freely admitted. On one of my visits to Guy's I didn't do a good job of hiding my bulge, my consultant happened to catch sight of it, he wasn't happy that I hadn't spoken to him about it, and so he wanted me to have CT scan. The results were lots and lots of cysts very little normal liver, also found a kidney stone in my left kidney. Now you would think that he would then refer me to see a Hepatologist, oh no it wasn't his field however, he did refer me to a urologist for the kidney stone, and that was a waste of time as they couldn't do anything because they did not want to cause any further damage to my kidneys, so they suggested an x-ray every 6 months which I did for 18 months, then I decided that there was no point to the x-rays, the stone looked as if it was stuck between cysts, well that was my opinion. From about 2005 my Liver seemed to have a sudden growth spurt, my daughter would often say " your getting bigger mum " not what I wanted to hear to be honest. There were times when I did get fed up with my Liver size, and I had started to get some bleeding from my kidney cysts plus an awful backache. I didn't complain I just got on with it.
As the months went by, and one year merged into the next, I was becoming more tired. It's a tiredness that seems to come out of nowhere, and I was finding it difficult to get up from a sitting position, or getting out of the bath among other things. I was really getting to the point where I would have to do something, wasn't sure what yet, t I knew that I couldn't carry on for much longer, things were only getting worse. Plus I had found out that I had got osteoporosis and apparently a lot of kidney patients get it. So I searched the internet came across some articles on PKD/PLD found them quiet interesting, most of the PKD I already knew about, and the PLD articles mentioned de-bulking,fenestration,resections and transplants. Time to have a chat with my GP, he was very understanding and said that he would support me with whatever path I choose.
I asked my GP for a referral to Kings College Hospital, unfortunately it was the wrong department, having said that they did do a CT scan, but it was decided that they could not do anything as I had very little normal Liver left. Now you would think that they would have suggested that I see an expert in the PLD field and there are a few at Kings, but no it was never mentioned. As you can imagine I was bitterly disappointed, I felt let down, but I knew someone somewhere could do something, so back to the internet spent time searching, came across the Mayo Clinic and Dr Torres, found it fascinating spent a long time reading whatever I could, even tuned in to a Webinar by Marie Hogan. I decided that I was going to give it one more shot and fight if I had too. Got another referral to Kings but I decided who I wanted to see this time.
My appointment was with Prof N Heaton, he was excellent, he suggested Kidney and Liver to be done at the same time so long as Guy's were in agreement, so then I went on the assessment list. During my this time My symptoms were getting worse, I was so very tired, my legs were covered in black and blue bruises my Liver would itch so much that I just wanted to rip it out myself, and at night whilst trying to sleep my liver would push against my bladder, so I would get up many times during the night, and if I laid on my right side it would push up against my ribs, for four years I tolerated this.
Its now assessment day and what a day that was full of appointments, people to see, question after question it was so tiring but necessary. Now its the waiting as the board decides, thankfully they said Yes what a relief that was, so its all systems go now. Have to attend an educational day, they tell you everything you need to know, show you all the tubes that you will have etc. Its also your opportunity to ask questions and its the day that your name will be activated on the transplant list. So now its pack a bag and wait. I waited ten weeks and got that call, was warned it would be a shock and it was. Once I got to the Hospital I was fine felt quiet calm considering what lay ahead, although time did drag. Its 2pm June 1st 2011 time to go, as I laid on the operating table I heard voices in the background and that mask heading towards my face , that's me gone. I believe that I went into intensive care at about 10.30pm. I don't remember anything till Friday evening, which is probably not such a bad thing. Recovery was quick and not too painful the worst thing for me was the catheter, I absolutely hated it and found it very uncomfortable. Came home on day 13, spent the next few weeks at my daughters, wound got infected, so had to have it flushed out and redressed every day for the next 5 weeks. However, I am doing fine at 8 months post transplant, had some pain on the right side still and my sleep pattern seemed to be improving, so all is pretty good really. I know that I'll never be 100%, but life has improved and I thank all those that have supported me through all the tough times.
My story has not quite ended yet, my brother had a kidney Transplant in 2005 after being on dialysis for only 5 weeks, his PLD was not severe, and he is doing great. As I have said previously my daughter has PKD & PLD and she is doing very well at the moment, what we don't know yet is what her liver is like, so I will be making sure that she has a CT scan at some point soon. Then there are my 5 grandchildren, two of which have already had an ultrasound which was negative and the other 3 have yet to be tested. Here in the UK they review children of a PKD/PLD parent every 5yrs. I sincerely hope that my daughter does not have to go through what I went through. And her story has yet to be told.
I would like to say to all those people who have PKD & PLD to remain positive and fight the fight if you have to. Don't ever give up no matter how long it takes. To all of you that are about to undertake liver surgery, I wish you all the very best. There are times when you maybe worried, concerned or just a little confused about something; or you may just need to discuss issues with someone who has the same condition you have, my advice is try this PLD Support Group, you will find this group very helpful. And they will always be there to support and guide you.
For Your Reference
Liver Resection [352 KB PDF]
Summary of Dr. Nagorney's article [553 KB PDF]
Octreotide Clinical Trial First Year [573 KB PDF]
Summary of Octreotide [164 KB PDF]
Alkaline Clinical Trials [168 IB PDF]
