Welcome to Bron's ITP Story
A little bit about me…
I was diagnosed with ITP in early 1998 at aged 27. I had been having extremely heavy and long up to 22 day periods and felt really flat. I thought I was anaemic so went to Docs for some bloods at 10.00 am and went home. By 12.00 pm, my mother and doctor both had started banging on the door of my flat telling me I had to go to hospital as soon as possible. I had a platelet count less than 1000. Hmm, okay, freak me out folks. When I arrived, there had been a horrific accident on the highway with 5 people killed and a couple of kids needing air lifting. So I was put on a bed to the side, told they had to check but it could be leukemia and left me for 6 hours. A very anxious night indeed thinking I was going to die pretty terrified to say the least.
The next morning, after all the drama they got to examine me and noticed all the PETACHIAE (I get dry skin so had put the dots down to that), then of course inside the mouth and then my nose started to bleed. I had lived the previous year in the UK and had a really bad dose of the flu that they think may have triggered it. So they did a bone marrow biopsy from my chest (the single most painful thing I have ever experienced), they told me I had this thing called ITP, put me on 150mg of PREDNISONE per day (I am a big girl hence a high dose ) and sent me home after 3 days.
‘Let’s see how you get on.’ they said and for the next few months it was weekly blood counts and bruises like I was in a fight with Mike Tyson.
So many treatments…
Never send a patient home not telling them the possible side effects of a drug (bare in mind this was a while ago so Dr Google was just emerging and I wasn’t so computer offay at the time). I put on nearly 40 kilos, would go 72 hours without sleep, my skin was so thin nothing would heal, I grew hair like a nanny goat all over my face, I got the shakes, heart palpitations, joint aches like I was 100 years old, memory loss, fog head and moods and I was a mess.
This went on for nearly 2 years, with each taper of prednisone dropping the counts. In and out of hospital each time the counts were under 10,000 and or a bleed or felt really off.
- I was referred to a haemotologist in Sydney (2.5 HOURS AWAY)
- I was given IVIg for the first of 15 times over 3 years (a big body mean a big dose sometimes taking up to 3 days).
- I had crappy veins, not helped by the prednisone thinning my skin. So each time a cannula went in it was a nightmare I would be crying and in in a lot of pain as they dug deep to find veins. It got to the point where nurses had two goes at my vein then the anaesthetist was called to do it, no matter how good they thought they were, it was rarely successful.
- I had 3 pic lines (a tube threaded up through my chest) by this point in order that the infusions would get through.
- I had a total of nearly 6 months in hospital over one year and ended up seeing a counsellor suffering from deep depression associated with all that goes with ITP.
Luckily living in a smaller town I knew a lot of the staff and or they got to know me. Having low neutrophils as well often meant I was in a room by myself which was good at times but terribly isolating at others. It was the nursing staff who called in the counsellor and I ended up on antidepressants. I had so much time off work as well it wasn’t a great feeling.
…Splenectomy is the next move
My haemo team at RPA in Sydney decided to try dapsone and dexamethasone pulses as well, it just didn’t work so a splenectomy was the next move (as was often the case more than 10 years ago). Again a far less educated me said ‘Yes’. I wish I hadn’t.
I had the dubious honour of being filmed for the TV show RPA (medical documentary TV programme in Australia) whilst having my operation and all the pre- and post filming it involved. Picture me laying on the trolley in prep room, central line coming out of one side of my neck and a microphone on the other. They filmed my follow up visit 4 weeks post-surgery with a count of 4000 to find it hadn’t work. Again, watch and wait and have more IVIG when needed. No way was I ever ever going back on prednisone again!
This was 2000, the same year I first met my husband Nick online. He was in the UK . The next few years included getting the first of 3 port a caths inserted in my upper chest to make infusions easier and more IVIG to hold me until I was given my first go round with rituximab (rituxin, mabethera etc. many names it has)
My first port broke so they had to go back in and cut out some tissue and stitch it to my breast bone, fun. The first Rituximab infusion leaked and I had quite the reaction, we soon learnt that I had to have a very slow rate of infusion and pre-treat and mid-way treat with antihistamine and panadol.
Yay, it gave me 3 years remission though, so I was thrilled…
During this time I would often get infections and when having my counts checked regularly it appeared I had also developed immune neutropenia (the white cells that attack bacteria), so started to have GSCF Granulocyte colony-stimulating factor injections which I gave to myself daily. These shots gave me serious bone ache almost like shin splints which was my bone marrow working hard.
I had gone from 5 days a week to 3 days a week in the childcare centre where I worked as the whole steroid thing and hospital stays just wore me out and I wasn’t coping with full time work.
In between this time and in remission, I travelled back and forth to the UK several times to see my soon to be husband and places like Morocco, Spain and Portugal, all while spleen less, though healthy and happily in love.
In 2005, I had a 4 week top up of rituximab after my counts started to go downhill fast, I also planned a wedding and got married that year. I ended up spending some time on my honey moon in a hospital with a low count, severe bruising, and patechiae. We had finally worked out that stress was a big trigger for me and PLANNING A WEDDING with people from overseas will do that to you.
Things settled down for a while and we got on with life. Then the next Christmas, I thought I had pulled a muscle in my shoulder, unable to lift it at all, so went for an x-ray to find that a 10 cm piece of my port tube had broken off between my rib and collar bone and travelled down to within 3 cm of my heart. Rushed down to RPA with a count of 40,000, I had to have an angiogram and have it removed through my thigh under twilight sedation. Then they had to go in under local to have the other part removed because they could simply not get a vein.:-( There went any easy access for the future.
We knew we had to wait 12 months before trying for a baby after the rituximab so we did and fell pregnant only to MISCARRY early on in 2006. I had more drops in my count and symptoms, but was keen to have a baby and was told to go cautiously and in 2007, I saw a CHINESE HERBALIST to help me fall pregnant.
Pregnant with ITP…
He was dismayed to hear I had had my spleen removed and he knew just from looking at me and feeling my pulse. In his words, “Some think spleen idle organ till everything else has to do the work” My bone marrow and liver were working overtime and he had a lot of work to do in supporting my blood, but within 3 months of drinking HORRID HERBAL TEA, I was feeling the best ever with great counts. I was pregnant. We were elated.
At 28 weeks my count was 83,000 and they started me on a low dose of prednisone much to my dismay but it didn’t help so at 32 weeks, I was sent to RPA in Sydney to the high dependency unit to have IVIG with a count of 28,000. They wanted it boosted before delivery. The prednisone induced gestational diabetes so had to have that monitored introducing even more needles as well as blood counts. Imagine how divine my black and blue body looked. I FELT LIKE A JUNKIE and people kept staring at my arms. My hormones were off the chart as it was let alone all the other feelings that came with the situation.
I was induced at 37 weeks (in a hospital far from my hometown friends and family) because they thought Will was going to be such a big baby. It didn’t work, so 4 days later with a huge team, I had an emergency ceaser (they were in a bit of a panic over the ITP thing though the haemo team were ok with it all.) It was uncomplicated and a waste of all the blood and IVIG they had on hand (but glad at that point they did). Will’s count was fine, I was fine but later I developed a clot in my leg and had 2 weeks in hospital. Happy girl with new beautiful baby, unhappy girl with ITP and clexane (blood thinners after surgery) injections.
Enjoying motherhood and married life, my counts were steady until 2010 before they crashed again with all the usual symptoms. I had my next round of rituxin with a new haemo after my adored Professor had retired. I live in a country town where I had my past treatments but my new Doctor didn’t have admitting rights to the local hospital so it came to budget and politics. I did have to go to Sydney to have it this time which didn’t impress me but RPA hospital was paying so that’s where I went. One day a week over 4 weeks and it did the trick again. That was December 2010 and I have been as low as 8,000 and as high as 282,000 during this time.
In May 2011, my husband and I decided to start our own computer business. I was working part time at my job in a child care centre and Will was growing fast. My hubby was feeling ill for a few weeks in August and was tested for Celiac and Chrons disease. It wasn’t that. By the end of September he had been diagnosed with Duodenal adenocarcinoma at age 38. He died in the January 2012, leaving me devastated and alone with Will, then 3.
In June 2012, Will was diagnosed with Autism which we had inkling had been coming and he had been attending early intervention but otherwise was a very healthy little boy.
In late July the same year, after a day at day care, I put Will in the bath only to find his little legs covered with more than the usual bruises and patechiae all over him. I put back on his clothes and took his straight to emergency.
My heart sank, I knew what is was. Over the next 2 weeks he was admitted and monitored, his count going back up to 53,000 then dropping again and being admitted again with a nose bleed. He was given Tranexamic acid (to stop the bleeding) He ended up throwing up blood so we were transferred by ambulance to Sydney Children’s hospital. Will had had a mild cold a few weeks before and I had been struck down with the flu. It was such a torment.
I was so ill, my mum had to go with Will in the ambulance as riding in the back made me ever more ill. Finally arriving and him still bleeding, I was a mess. Bless, he was just plain exhausted and had a terrible colour. Imagine my torment thinking I had done this to him, I had handed down this awful disease and made my baby so sick. We stayed for 4 days and he had his first round of IVIG and more Tranexamic acid. We came home with a count of 64,000 and all was fine for the next 12 months, phew.
Maybe he was going to be lucky and it was just an acute case.
Despite the stress and my counts down to 14,000 with some other symptoms, this year the rituximab has held its own. Thank god really because in late September 2013, Wills counts dropped to 2000. Again the same symptoms but this time his count stayed low for 2 weeks and he bled on and off but they wanted to watch and wait. He ended up with a severe nose bleed of 12 hours so we were transferred back to Sydney, more Tranexamic acid and a week in Westmead with 2 infusions of IVIG.
It has been hard to try and back off on Wills ITP because it is handled so differently in children, so much of what I knew or would have expected treatment wise doesn’t fly with him. There was talk of cauterizing vessels in his nose at one point and doing a bone marrow biopsy whilst he was under GA in the end they didn’t do bone marrow but they do feel there may be a bigger immune picture in our family. They shrug their shoulders and say it is a watch and wait and they trust me to do just that…watch and bring him in if I am worried.
He now has a MEDICAL ALERT arm band and all his emergency plans are in place for preschool and day care and for primary school . He has now gone nearly 2 years without another episode although we check every night for “spots and bruises” They did end up doing his nose in 2014 due to continual small bleeds as a precaution.
Then we get to June 2014 and my numbers bottom out again and symptoms return. I hover at about 16,000 and had no choice but to go onto prednisone again even though I swore off it. It had ot be my stop gap whilst they applied to have rituxin again. This was to be my 4th lot in 10 years and with it comes its own complications. Not an easy decision but I was still pretty terrified of the clot scenario with TPO’s being offered. Luckily for me the drug committee agreed for me to have it again because of the success I had had in the past. Yet another picc line another month of day long visits to the cancer clinic ( they are like family now) and now back in remission.
My dear old haematologist was a Professor at RPA (Royal Prince Alfred Hospital) and had been involved for a lot of years with ITP research. He was always the calmest and easiest to talk to person I had met, not to mention a gorgeous Scottish accent.
He always maintained that it was the symptoms not the count that mattered and that the numbers could often cause far more stress than was required, so that’s pretty much how we treated and how I went about with life.
I manage my own ITP so to speak…
My haemo and I have a good understanding. (Although we did butt heads over n-plate as he wanted me to try this new sparkling drug because of the costs of Rituximab but I refused due to clotting issues).
We treat reactively, not proactively. I have a count every 3 months because I also have neutropenia so we watch the cycle. If I get a few bruises or patechiae on my abdomen then I watch for a week. If I get any wet patechiae (i.e. blisters in mouth) then I get a count done. I call the lab direct or bless, Ian my pathologist will call and say, “Bron, your numbers aren’t great today. I can see a bit of a down turn you may want to check in.” I can bounce around from 8,000 to 240,000 in one week so we don’t jump on treatment, and after all these years, I am kind of glad. I just don’t balk at the numbers anymore and you soon learn that each machine reads a little differently and what’s on the film might be different as well.
We have a team for my son and a team for myself. I really trust them and know I can challenge and ask questions of them and feel comfortable this way. They know I read a lot (perhaps too much sometimes). They are always willing to listen to what I do and don’t want and know that I am a very big advocate for my son. I am blessed with an amazing family and close friends and I have learnt to say yes please to offers of any help which can sometimes be hard to do. So we just get on with living life the best we can and deal with the ITP bumps along the way.
I have learnt that each and every person I have met with ITP presents slightly differently, reacts to meds differently and is handled differently. They call it idiopathic. I call it idiotic myself.
Advice I would give to folks…
who are new to this thing called ITP is be informed. Ask questions and find a medical team that you feel comfortable with. We are all different, there is not a single person who will be dealt with in the same way.
So don’t freak out if somebody else says you should be doing this or that, or is horrified by your response, and don’t be shocked by their treatment either. Try not to focus on all the negative stuff that you read about what you can’t do, eat , drink, smell, etc.
Focus on life and get on with living it.