Where we're at now

Since I posted last my platelets have continued to increase. My visits to the hematologists have gone from weekly visits to monthly ones. With no drug treatment for the last three months my results have been in the 300s, which has been more than amazing!

So it seems (for now at least) that the splenectomy has truly worked.

Celebrate the Little Things

When my platelets dropped off again rather sharply after the op, it left me concerned as to what options I had left. I began to wonder about what kind of therapies they might suggest to me next and if I had many options at all.

I became active in the two Facebook ITP groups which are a wonderful support for people trying to make sense of - and live with - ITP. There people were discussing the different therapies and their experiences with them.

One lady shared a story about one of her family members who had a count of zero and didn't respond to any treatments. She eventually died. Stories like this are scary to hear about, but eye opening too.

The problem with ITP is that each person afflicted by it will present the disease in a slightly different way. Some will suffer from a lot of bleeding, bruising and intense fatigue where others will have the petechiae and fatigue, or no symptoms at all.

In the same way each person responds to the treatments in a different way. One person may have great success with a treatment, yet it may do nothing at all for someone else. One person may experience no side effects to a drug that others react very strongly too. So in many cases our Drs simply have to try different things and hope they'll find a perfect match between patient and treatment, and hope the results will last. This can be challenging and disheartening for patients and their hematologists.

The frustration I have is that in New Zealand (as in Canada) we don't have full access to all the therapies that are available to UK and American patients of ITP. For instance the two options I was presented with were surgery or Azathioprine - and I was cautioned against the drug.

If I was in the UK or America they would likely give me a range of drugs to trial and, in many cases, surgery would be one of the final options.

Most of the time having ITP is little more than an inconvenience, a specter that hovers in the back of your mind. When things are going well it can be easy to forget that you're unwell and that ITP is a dangerous condition to have.

On a lighter note, things at my end have continued to improve.

I recovering from the virus I fell prey to a couple of weeks ago and the double dose of penicillin seems to have prevented my catching any secondary bacterial chest infections.

Two weeks ago I was sitting on platelets of 36. That rose the following week to 66 which was a great relief. Imagine my delight today when I found out I'm now sitting on 184!!!

I couldn't believe it. I'm so stoked!

I said to the hematologist, "So it looks like the operation has worked after all."
He gave me a funny look. "Well, things are looking all right, for now. They did drop very low though. It may not last long," he said in a voice that implied he wouldn't be surprised if the crashed again in the near future.
I said, "Yes, but I was told they'd probably drop off after the surgery and then go up again."
He just looked at me. "Not that low."

Still, I refuse to let his pessimistic prediction get the better of me. I won't let myself worry about what will happen if/when I crash again.

I'm determined to celebrate this and think of it as a victory. Long may it last!

Let me off the Roller coaster

Last time I blogged here my platelets were sitting on 380 and things were looking sweet. It seemed the operation had been a great success and I was thrilled to be getting off the Prednisone.

Unfortunately since then things have, once again, got a bit complicated.

We had expected my platelets to drop a bit and settle. Mine, however decided to drop a lot more than "a little".

It has been five weeks since the splenectomy.

On Monday this week as I was getting really for bed I noticed patches of petechiae on my chest. This is red flag for me and I was instantly concerned even while trying to convince myself it was probably nothing. The following morning (3rd May) I went and had a blood test.

That evening my GP rang me and told me my platelets had dropped to 38!!

I told him I'd ring my hematologist the following morning and see what she advised. As luck would have it she rang me early the next day.

I asked her "Are we worried?"
She said, "That depends."

She asked if I'd been unwell with a cold or viral infection. No on all counts. I haven't been sick. She suggested I get another blood test that same day so we would have an indication of what my platelets were doing.

I did as she requested and she rang me again later that afternoon. My platelets had dropped again to 30. A small drop, but a drop none the less.

She suggested that I go in for an outpatient appointment on Friday and have another blood test prior to that appointment.

In the interim I've developed more patches of tiny petechiae and small bruises.

Friday (6th) I went in for my appointment. My platelets had risen very slightly to 36.

So where does that leave us?

It was suggested to me that it might be possible the platelets might stablise around the 30-40 mark, which is not ideal but not uncommon for ITP patients.

I declined the offer to put me back on Prednisone. I have no desire to go back on the steroids if I can avoid it.

I'm to go back for an other blood test and check up next Friday to assess where we're at and if necessary plan any steps going forward.

So now we're in the midst of a waiting game.

Will my platelets hold steady, go up again or plummet?

I hate this uncertainty. Every time I discover new spots or new bruises I can't help but worry that it means my levels are dropping again.

Just when you think the roller coaster ride has come to an end life straps you back in and throws the switch. "Life. Just so you know, I'd like to get off now. Thanks."

On the Up and Up

I was back at the hospital on Tuesday for an out patient appointment at the Hematology department. My platelets were at 380, so up very slightly from the previous week.

This is the high end of normal so the hope - and the expectation - is that once I'm off the Prednisone my platelets will drop a bit. Apparently it is not uncommon for them to be high-ish and then drop off somewhat as the Prednisone is tapered off.

What we don't want is for the platelets to continue to rise because then we are looking at the opposite problem thrombocytosis, the risk of blood clots. However, according to Wikipedia platelet counts over 750 are "cause for investigation" and I'm are a good way off this.

Ideally we want to be sitting somewhere in the normal range of 150-300 (though some websites put the top end of normal around 400-450).

The plan is that on Sunday I'll drop the Prednisone to 5mg and the following week take 5mg every other day (unless I begin to feel unwell as a result), ceasing to take it all together the following week. I image it will take a few weeks to get the steroids out of my system all together.

It seems incredible that after six months I'll finally be off the Prednisone and free of it's side effects: the sweats, the hot flushes, my hair falling out, the indigestion, the tingling in my feet, my "buffalo hump" and my "moon face," the facial hair, being tired in the middle of the day and wide awake at bedtime.

As of today I'm cutting down the amount of codeine I'm taking so I've been a bit more uncomfortable today, the nerve has been pinching a bit off and on, but over all it has been tolerable.

The sleeping pills have been working wonders at night, but I've decided to try to cut back on this as well. We'll see how I go. Tonight will be my first night on 1/2 a tablet.

Back Home

During my first few days at home I needed help to do the most basic things, like getting into bed and putting on my shoes, and I spent most of my time sitting in our little down stairs lounge while my Mum ran around after me.

It wasn't until Friday evening that I let Glenn help me up the stairs to join my family for tea for the first time since the operation.

On Friday I went to the medical clinic and had my incisions dressings and my dressings changed. One of them had been looking a little dodgy but apparently the wound itself looked fine upon inspection. So that was good news.

The next morning (Saturday) I was quite unwell. I felt really spaced out. I was really nauseous and sweating. I felt (and looked) awful. We all began to worry that I was coming down with an infection of some kind.

Luckily I had been discharged from hospital with a few anti-nausea tablets (among other things). I took one and within an hour I started to feel much better, the fever symptoms passed and I was fine. I still have no idea what set off that episode but am very relieved that it all came to nothing.

By now I was beginning to suspect that they might have damaged a nerve near my hip when then made or repaired the largest of the incissions, the one on my side. I have a numb area above my left hip, which sometimes stings or pinches and at times, especially at night, burns. It's can be incredibly painful and at other times its little more than a twinge.

On Monday I went for my weekly blood test and on Tuesday I went back to the medical centre to see my GP. My platelet result was 375!!!!

This would seem to indicate that the operation was successful. Even though I was still on 15mg of Prednisone this is an off the chart count for me and high into the "normal" zone.

My GP will be overseeing weaning me off the Prednisone. So the plan is now to stay on 15mg for a week and then drop to 10mg and so on until I hit 5mg.

It'll be interesting to see where my platelets are sitting on Monday and where they will finally settle once all the steroids are out of my system.

I also discussed the nerve pain problem I was having and my GP suggested a medication I might try. More commonly used as an anti-depressant, it's also used to treat nerve pain. She said there are a number of different drugs we could use if the first one didn't work for me. She also advised that I wasn't to take this and my sleeping pill at the same time, rather I should take one or the other as this drug should dope me up enough to help me to sleep anyway.

As it turns out we may need to try something different. The first night it seemed to work. The burning sensation, which is often at its worst at night, eased but I had the weirdest dreams and it wasn't until I took my painkillers at 3am that I actually slept properly. I tried it again the next night but again had strange dreams, slept very poorly and it seemed to have very little impact on the nerve pain this time.

On Thursday night I decided to go back to using my sleeping tablet to see if it worked any better. The result? I slept like a baby. I didn't wake until morning and didn't need need to take any painkiller in the middle of the night.

Perhaps I should have given the drug more than a two day trial, but the odd dreams were just disturbing. Shrug. I'll check in with my GP again on Tuesday to see what she advises, in the mean time I think I'll go back to using my sleeping tablets.

I also needed my GP to refer me for a medic alert bracelet. This will have my personal medical alert number and "post-splenectomy," engraved on the back so should I become ill medical/ambulance staff will know straight away what infections I am most at risk from. I still need to check back with my GP as to whether or not I need to have "ITP" detailed on the back as well.

I'll send my application off to medic alert next week. I'm looking forward to wearing my new bling!

Post Op

On Friday the 25th I had an appointment with my hematologist. She was pleased with the fact that my platelets were at 226. She told me to stop taking my garlic and fish oil supplements as these thin the blood.

My folks finally arrived on Sunday afternoon after an extended stay with my brother on their way south. I had to go into the hospital that evening for a blood sample to be taken (in case they had to give me a blood transfusion) and I was also to have an injection to thin my blood.

When we got in to DPH they told us they were no longer going to give me the injection. We couldn't help feeling a little peeved. We'd driven all the way into town and they were only going to take a blood sample which they easily could've taken the following morning.

My hubby dropped me off at the hospital just before 7am and I headed up to the 4th floor trailing my little purple suitcase. I was given a lovely set of hospital pjs, a stylish gown and boxers. "One size fits nobody," the nurse joked as she handed them over.

I was first on the list for surgery. As I'd requested I was given a little pill to help me relax. At 7.30 was taken down to theater and asked to climb on to the operating table. My memory at this point is a little hazy, no doubt the de-stressing drug was beginning to take effect. They gave me a mask to breath into....

.... and the next thing I knew someone was saying, "You have to wake up. You're in the recovery ward. The op has been done, it went well and you need to wake up."

The next thing I was aware of was pain.

I admit I moaned and grizzled a fair bit after this.

I think they gave me a few oral pain killers. Then I remember someone saying it wasn't enough and they were going to have to give me something else. Another someone started giving orders. A pain pump was pressed into my hand and I was told to pump it as much as I needed to. The pain eased quickly after that and I think I went back to sleep.

My next memory was waking up on the ward with my Mum sitting beside me. Apparently I'd been awake before this and had a conversation with her and my Dad when I'd first been taken to the ward, but I don't remember any of it or that my Dad had even been there.

The pain pump was brilliant! It was my very good friend for the next 24 hours.

I was very unsteady on my feet, and pretty spaced out for much of the first day. I remember being fairly proud of how coherent and with-it I was, but apparently reality was a little different...

The next morning (Tuesday 29th) the surgeon and his team came round first thing to check on me. This was the first time I'd seen what they'd done to me. I think I was a bit scared to look before that. I had four wounds/incisions. Two small ones just below the line of my lower left ribs, one more towards the center of my tum and another larger one on my side.

Because my platelets had been so high there had been no need for me to have any infusions of platelets. The surgery had gone well with no complications, he was pleased with how it all looked and thought I could probably go home the next day if I was strong enough.

My platelets were are at 230.

My Prednisone had been given intravenously during the op and increased to 30mg (up from 15mg) to cover any post-operative stress my body might experience. I was then given 30mg orally on Tuesday and it was dropped to 25mg on Wednesday. The plan was to decrease the Prednisone by 5mg every two days until I hit 15mg.

I'd also been given my first dose of antibiotics intravenously, but now I started taking them orally. Pencillin VK 500mg twice a day for the rest of my life.

The pain pump was taken off me at about 10am and I was given oral pain relief; panadol, codeine and tramadol. I'm not allowed to take ibuprofen or asprin because they thin the blood.

I was encouraged to eat, though I really didn't feel like it. Mum came in with Dad in the morning and spent the day with me, chatting, drinking coffee and generally just hanging out. Dad went off to potter and do a few odd jobs he'd managed to find that needed doing.

Mum helped me shower in the morning (the nurses were so busy there was no way they could have found time to assist me) and read a book while I snoozed in the afternoon. My Dad brought the kids in to visit for a short while after school and my hubby dropped in again after work. A couple of friends also popped in.

I slowly began to regain my strength and by the end of the day I could hobble to the bathroom and back to my bed without assistance as long as I had something to hold on to.

On Wednesday morning my platelets had dropped a little to 219. I was given the all clear and told I could go home. My wounds were cleaned and dressed and by the time the house surgeon had got all the paperwork together it was after lunch. I was wheeled down to the entrance and left the hospital at about 1.30pm.

A Bit of Good News

As I suspected, once my platelets had broken through the 200 barrier they'd start to drop again.

On Monday 7th my platelets were at a very comfortable 188, by Friday when I had my pre-op they'll cruised to an all time high of 223, but on Monday 14th they'd fallen back to 196.

Still 196 is nothing to sneeze at. It's nicely above 150, which is the minimum my Hematologist wants me to be sitting on when surgery rolls around in a just over a week and a half.

After getting the result from the nurse this morning my Hematologist rang me and confirmed that I was allowed to decrease the Prednisone again by 10mg. So tomorrow I'll be down to 20mg.

Already, as I've been decreasing the dosage, the side effects are easing. I still have a lot of neck and facial swelling, but the pain in my neck and shoulders has eased and Panadol is usually enough to deal with it. The tingling in my hands and feet and the numbness in my face have gone. The indigestion has eased so much I very rarely need to take Mylanta, the Omeprazole twice a day is usually enough now.

So I'm excited and celebrating this small victory.

Now if they will just hold steady and if I can avoid getting sick for the next week and a half I'll be a happy camper.

Pre-Op

On Friday 11th I was back in Dunedin Public hospital for my pre-operation appointment.

I met with the House Surgeon and her attendant student, the Registrar and the Anesthetist. They examined me, tested all my vitals and took more blood tests. Feeling like I was signing my life away, I signed off on the op.

I also had an MRI, which was not related to the upcoming op but to problems with ovarian cysts I had last year, the appointment just happened to fall on the same day. I've heard terrible things about MRIs. About how loud and clostrophobic they are. I coped with it pretty well. I just kept my eyes shut and pretended I was having a nap. It was really loud though...

All up it took about three and half hours.

I will have to go in briefly the Sunday evening before the op to have an injection to thin my blood to prevent blood clots. Now you'd think that having a condition where low platelets (trouble with clotting) is an issue would make this unnecessary, but apparently it doesn't work like that, go figure.

At the same time they'll take blood from me in case I need a blood transfusion after the operation. Apparently they would normally have done this when I was there on Friday, but something about my condition means the blood will only be good for 48hrs rather than the usual 20+ days.

I have to present myself to ward at 7am on the Monday morning (28th March).

I will be given platelets before the op (though the Anesthetist thinks this is a waste of time and will have a discussion with the surgeon about it. The theory being that those platelets will end up in the spleen and be removed with the spleen so you may as well not have had them. I'll let them argue it out...) and then they'll give me more during the op, once the spleen has been clamped off.

The Anesthetist asked me if I wanted to be given something to dope me up and help me relax before going into theatre. I unashamedly said, "Yes, please!"

So my hubby will drop me off first thing, on his way to work. My folks will get the kids off to school and then come in to the hospital so that someone is there with me when I come around afterwards. Not that I'm going to be very with it, but (big baby that I am) I didn't really want to wake up alone.

All going well, the op will be laproscopic and will last about three hours. I'll have four small incisions in my tum, mostly on my left side, as the spleen is under the left ribs. One will be next to my belly button where they will insert the camera.

The reality is slowly sinking in...

I phoned the hospital yesturday to find out the results of the bloods they took on Friday. I couldn't believe it when the nurse said I was sitting on 200+. I was so taken aback I actually can't remember the exact number.

Once I hung up the phone a false, inner voice began whispering, "See you're fine, you don't need this op. It's pre-mature. You've had so many people praying for you; you're all better. You don't need this op. There's still time to get out of it." My inner Golem had awoken ... and has been tormenting me ever since.

Of course I know it isn't true. I know the platelets are high because the meds are working and without them I'd be a mess. As much as I don't want to go through with this I know I have to. This is the best and the most sensible medical option for me right now.

I didn't think my platelets had been this high since I was diagnosed with ITP but when I was talking to my Mum last night she reminded me that I'd broken the 200 mark once before when I was first on the Prednisone. I'd got up to 210 before the level started to drop off.

If I needed a warning about being sensible and taking my antibotics after the op I got one on Friday. One of the many people I spoke to said her friend's daughter had recently had a splenectomy but she'd stopped taking her antibiotics and now had peritonitis (fluid around the heart). Point taken: Don't be a git and stop taking your antibiotics, they give them to you for a reason!

A Surgery Date

I got a call from the DPH Surgery department today to tell me that even though I haven't received anything in the mail as yet, my surgery date is Monday 28th March. Just under three weeks away.

My pre-op appointment will be this Friday at 10.30am and I should anticipate that this could take 4 to 6 hours! I will be meeting with the anesthetist, surgeons etc and having whatever tests they think necessary.

I admit to being taken a bit unawares by the call. It shook me a bit.

I was ready to get the letter in the mail, to sit down and take my time to open it and get my head around it; getting the call took me off guard. I'd pretty much convinced myself the surgery would be delayed, but the date is exactly what the surgeon indicated when we met with him.

I think I'm still getting my head around it. It feels a bit surreal...

I also got the results of my latest blood test today. My platelets are sitting happy on 188!!!! (which is amazing) I will be able to drop the Prednisone a further 10mg to 30mg tomorrow.

Friday Follow Up

On Friday 5th March I had a follow up visit with my Hematologist.

She was very pleased with my last platelet count of 151 on the previous Monday. But she took one look at my "moony" swollen face and announced that we would halve the dose of Prednisone, dropping me from 80mg to 40mg.

The hope and the plan is to reduce the Prednisone as much as possible over the next few weeks and still keep my platelets above 150. A nice safe level for surgery.

Each week I will have my bloods taken and will check in with my Hematologist before adjusting the dosage for the next day.

I have been on the 40mg dose since Saturday (only three days) and will get my blood results tomorrow.

It will be interesting to see what happens. Will I be able to maintain platelets of 150 on 40mg?

Another Step towards Surgery

On Monday 28th Feb I popped into the local medical centre to have my weekly blood test before work. While I was there I checked in with the nurses to see how they were getting on with ordering in the immunisations I needed.

They still had one or two they were waiting on. Not knowing how I would respond to the immunisations I had hoped to get them out of the way early in the week so I could spend my days off work dealing with any side effects, but that wasn't to be.

On Tuesday I called the hospital to get my blood results. Wait for it....

151!

My platelets had continued to rise and I was sitting on 151!

So while I've struggled with the nasty side effects of the Prednisode it hasn't been for nothing. The drug was working it's magic. I was back in the "normal zone."

On Wednesday, 2nd March, I checked in with the nurses at the medical centre again. They had managed to get four of the five immunisations we needed. The fifth, Meningoccal B, could not be sourced - apparently they aren't manufacturing it at present.

It was decided that I'd go in the next day.

However when I got there on Thursday they took my temp and discovered it was raised (37.6), and so the doctor had to be called in to check me over. Eventually it was decided I was fine and could go ahead and have the jabs. Crisis averted!

I really had been dreading getting them done. I hadn't had an immunisation since the rubella jab when I was 12yrs old! In the end only one of them was really uncomfortable ... though I couldn't tell you which was the ouchy one.

I was patched up with four Barbie bandaids, two on each arm. My girls were very impressed when I came home sporting these brightly coloured momentos of my bravery.

I haven't had any adverse reaction to the immunisations. The ache in my neck and shoulder did increase and I had a bit of stiffness too, but nothing that a good dose of painkiller couldn't deal with.

So, I have been immunised. It's done. I've taken another step towards surgery.

Toughing it out

After my trip to hospital for a transfusion of antibodies in the middle of February and my unfortunate reaction to it, I came away better but with a sore shoulder. The pain in my shoulder initially receded, but on the Tuesday night following my discharge it returned with force.

Over the next few days it continued to flare and extend into the side and back of my neck. By Friday I was taking Panadol and Codeine which helped but I still suffered break through pain, especially at night.

On Saturday night as I was getting ready for bed I noticed my shoulder and the back of my neck had begun to swell. On Sunday I noted that the swelling on the back of my neck had traveled around to the front, destroying the definition at the base of my throat, and it was spreading up my neck giving me a double chin and swelling my jawline.

The pain was continuing. I had been experiencing tingling in my right foot which had become continuous. I had occasional tingling and burning sensations in my palm. On the Sunday my face began to tingle and I experienced a patch of numbness in the area of my left cheekbone which came and went. I began to worry that whatever was causing the swelling in my neck was causing pressure to my nerves or something.

On the Monday I visited a GP. She thought that some of my symptoms could be caused by the stress of my illness and upcoming surgery and speculated that the swelling I was experiencing might be due to an allergic reaction to a medication. However, since I was taking no new medications she decided to send me in to ED to rule out any other potential issues.

So, on Monday afternoon I was back in ED. They took full bloods and x-rays of my chest and neck.

The bloods came back clear. My platelets had risen to 70!!! The x-rays were fine. After some discussion with my Hematologist they concluded that the most likely cause of the swollen collar around my shoulders, my "buffalo hump" as the doctor called it, was a reaction to the high dose of Prednisone I'm on.

Obviously my body has decided to protest the sudden jump in dosage. But I was otherwise responding well to the Prednisone, as evidenced by my blood results. My platelets had jumped from 18 to 70 in a week and my doctors are reluctant to mess about with the dosage and risk another drop in my levels. They advised that I keep up the panadol and codeine for now and they'll review the Prednisone dosage next week.

So I'm sucking it up. I'm taking painkillers when the pain flares ... and trying to avoid any and all mirrors.

Symtoms of ITP

The symptoms of ITP tend to vary in intensity between those effected by it.

I tend to have a fairly mild response to a low level of platelets, compared with some of the nasty images I've seen on google.

According to the US NIH;

Having a low platelet count doesn’t cause symptoms. However, the bleeding that a low platelet count can cause may have the following signs and symptoms:

• Pinpoint red spots on the skin that often are found in groups and may look like a rash. The spots, called petechiae, are due to bleeding under the skin.
• Bruising or purplish areas on the skin or mucous membranes (such as in the mouth) due to bleeding under the skin. The bruises may occur for no known reason. This type of bruising is called purpura. More extensive bleeding can cause hematomas. A hematoma is a collection of clotted or partially clotted blood under the skin. It looks or feels like a lump.
• Nosebleeds or bleeding from the gums (for example, when dental work is done).
• Blood in the urine or stool (bowel movement).

Any kind of bleeding that's hard to stop could be a sign of ITP. This includes menstrual bleeding in women that’s heavier than usual.

Bleeding in the brain is rare, and the symptoms of bleeding in the brain may vary in severity.

Exhaustion
Some places on the internet deny that ITP should cause you to feel tired and leave you battling with exhaustion (maybe this is true if your platelet levels are above the critical zone) but my Drs are never surprised I'm exhausted. This is one of the first things I notice and the one symptom I find the most difficult to deal with especially when it leads to lethargy and the sensation of limb weakness.

Headaches
I sometimes get sharp, short lived headaches, this is probably due more to the weariness factor than ITP itself.

Mild Paraesthesiae
A tingling or burning sensation in the skin like pins and needles.

I have increasingly been getting this, especially when I'm in bed. It's not as uncomfortable or as intense as pins and needles but more like a random tingling in the toes/foot and fingers. Lately I've also been getting a sensation in parts of my hands that I can only describe as a burning, stinging feeling, as if I'd recently scolded or burnt it.

This may be a reaction to the steroid treatment rather than a symptom of ITP.

Petechiae (Blood Spots)
Flat roundish red spots under the skin which are caused by blood leaking from the capillaries in to the the skin. These can be anywhere on the body including inside the mouth.

I tend to get them most commonly on my chest, arms and legs, especially around my ankles. They can be quite scattered or in clumped in 'crops'.

Purpura
Are larger, darker spots or areas of bleeding that become purple or brown bruises caused by bleeding under the skin.

Bruising
I get a lot of bruises when my platelets are low and I couldn't tell you where I'd get most of them from. They're ususally the yellow or brown variety but occasionally I get a lovely purple one. The legs seem to be most vulnerable for this.

Bleeding
[note: If you have ITP and you hit your head go to your GP or ED as it may cause bleeding on the brain.]
Some people maybe have blood in their stools or urine or related spotting. Women may have much heavier periods. You maybe susceptible to nosebleeds and bleeding gums when brushing your teeth. Blood filled blisters may appear in the mouth and on lips. If cut, bleeding will be much, much slower to stop. "In general the more bleeding symptoms you have, the lower your platelet count" according to the PDSA.

When my platelets are low I get bleeding gums on doing my teeth and they are sometimes quite raw. I don't tend to get nose bleeds, though I sometimes have small blood clots on blowing my nose (what they call mild epistaxis) and spotting. This was the case even when my platelet count was 2!! unless I'm cut or punctured I don't tend to bleed.


I hope this gives you an idea of what to look for if you think you, or someone else you know, has ITP. Remember some people will have a much milder set of symptoms than others.

Meeting with the Surgeon

We met with the surgeon this morning. It went well ... or as well as such an appointment can go. He has a very good reputation and seems very careful and competent. He was very clear about the risks involved and what I should expect in terms of recovery time etc. Much of what we discussed was stuff I'd already gleaned from the internet but it was good it have in confirmed and clarified.

All things going well they will perform the procedure laproscopically. However given my condition there is the risk that if I bleed too much they will be forced to resort to open surgery (they simply can't see what they are doing otherwise). This will require a much longer recovery time and I'll have a nice battle scar just below my left ribs.

If my platelets don't come up on their own they'll give me another infusion to try to bring them back to a safe level. If this is still insufficient to get my platelets back to at least 50 they'll give me an transfusion of platelets both before and then again during the procedure.

I have a tentative date of Monday 28th March for the operation and should know for sure in the next week when it'll be happening.

I'll have to go in to the hospital about a week before for a pre-op appointment. This is when they'll do all their tests and I'll get to meet the anesthetist. I've been warned that this usually takes 4-6 hours. It's going to be along day!

Yesterday I took the immunisation information around to my doctor's clinic. I rang in again today after I'd seen the surgeon to confirm that we would be going ahead. On Monday they'll set about ordering in whatever immunisations they don't have and sometime next week I'll go in and have needles stuck into me in multiple places. I'm not looking forward to this! Blood tests I can handle with nary a blink, but jabs are something else.

Without a spleen I'll be more susceptible to bacterial infections, especially those of the chest infection kind. So there are four or five vaccines I'll be required to have. [note: depending on where in the world you come from this list may vary]

Pneumococcus
(this protects against pneumonia)

Haemophilus influenza type B
(protects against Hib or Hib Meningitis)

Meningococcal ACYW
(protects against a group of bacteria that can cause Meningitis)

Influenza
(protects against the flu)

Meningococcal B
(protects against Meningococcal B)

Once this is done I'll have to repeat the Pneumococcal every 5 years, Meningococcal every 3-5 years and the Flu vaccine every year during the normal autumn flu season.

I've been snatching rests when I can and picked up some sleeping tablets yesterday and so managed to get a decent sleep last night. I had a better day today.

I haven't declined as rapidly as I'd anticipated since finding out that my last platelet result was 18. I'm very slowly getting a few more spots everyday, but haven't had a drastic increase in symptoms, so perhaps the Prednisone is working to slow the decline. I'm assuming my platelets are still on a downward trend since the petectiae (the little red blood spots) are increasing rather than disappearing as they usually do when my platelets go up.

Thanks for all your support, encouragement and prayers. It really does help!

"Stop the world I wanna get off!"

I got a call on my cell while I was meandering along the road to catch the bus into the hospital today. It was the Surgical Outpatients department saying they'd received a referral from my Hematologist and had an appointment for me at 10.30am this Friday. Yes, you heard right. In just two days.

I was a bit surprised since I hadn't actually told them I'd decided to go ahead with it yet, but my hematologist is the proactive sort and is obviously keen to get the ball rolling. Besides if I'd decided to go the other route it would have been easy enough to cancel it.

I dragged a friend along to my appointment since hubby couldn't get the time off work and got in there to discover my doctor was away but that she'd asked someone else to meet with me. Her replacement was lovely and answered all my crazy questions.

My platelets have dropped since Monday (when they were 31) down to 18 yesterday. So, as before, I'm to go for another bloodtest on Monday unless I deteriorate in the meantime, in which case, I'm to go straight into the ward.

Unfortunately, if my history is anything to go by, now that my platelets have slumped this low it's unlikely they'll come up again by themselves. I'll be monitoring myself closely over the next few days for signs that things have deteriorated further. However, because of my bad reaction to the Immugam transfusion last week they're now reluctant to give more unless they have to. As the doctor said, "If we can avoid putting you though that again, we will."

I had my suspicions yesterday that my platelets were dropping again. The site of my bloodtest was still seeping when I went to take the plaster off five hours after the test! And then this morning I noticed a few new tiny red petechiae on my upper right arm and shoulder. So I wasn't really surprised to hear the low result.

Of course if I can't maintain my platelets at the moment on the highest dose of Prednisone they can give me then we have other problems.

A surgeon isn't going to want to operate on someone with a low platelet count. After all, they don't want me to bleed out all on the table... that would be very inconvenient!

Ideally they'd like my platelets to be 80+. It's unlikely they'll do it if I'm sitting under 50. And that feels like miles from where I am now!

So how am I aside from all this? I'm knackered!

I've been very stoic and practical though out the process of making the decision to have the surgery, but the exhaustion is getting to me now and undermining my calm. One of the draw backs of being on a large dose of Prednisone is that no matter how physically, mentally and emotionally exhausted I am my brain won't shut up and let me sleep.

I'm so tired right now all I want to do is curl up in a corner and cry. Let life carry on without me, I don't want to know about it anymore!

It sucks, but that's not the way life works...

I'm trying not to think too hard about the fact that I'm working Friday through Monday. I can't afford to take any time off and we're going to be working short-staffed this weekend anyway. So unless my platelets crash though the floor and I've no choice but to go into hospital, I'm be soldiering on.

What Happened Next

9th February: So back to hospital I went. I packed a small overnight bag on a hunch I'd end up being in overnight.

All three of my hematologists, including my Consultant and Registrar, were waiting for me in ED when I arrived and I was out of the waiting room, processed and into a ward within 20 minutes (which has got to be some kind of medical miracle, they were certainly pleased with themselves).

They did the usual processing in ED, examined my bruises and spots and questioned me about any illness or medication or health products that might have triggered the crash in my platelet levels.

The thing was, other than feeling tired much of the time I'd been fine. Which had them scratching their heads and frowning. I'd had nasty UTI in the middle of January but it was deemed to have been too long ago for the antibiotics to have caused the crash.

And that's when all the apples fell off the cart and my hematologist said, "I think we may have to consider surgery. Otherwise you would have to be on least 10-12mgs of Prednisone dosage, and that's too high."
Surgery was something that had been mentioned when I was first diagnosed. I nodded and asked, "So how long will we wait before we have to think about it seriously." Thinking that if I didn't improve in a few months we'd give it due consideration.
She said, "I think we could get you in in the next two or three weeks."
I stared at her. I'm sure my eyes bulged and my mouth dropped open in shock because she added, "There is a drug you could take instead but it carries a cancer risk and you're too young to take that risk." Seeing it was all a bit overwhelming for me, she gave me a hug. (I have lovely doctors!)

They soon had me up in the ward and hooked up to an IV line. I was given a large dose of Immunogobulin Intragram (antibodies) to quick jump me out of the critical zone and get my platelets back up above 10. It takes about three + hours to take on board the full dose.

Each time I've had an infusion I've had different side effects. The first time it gave me mild joint and muscle aches and an annoying dry cough that lasted for weeks afterwards. The second time I had almost no reaction - possibly because it was within days of the first dose - I just felt a little queasy. This time, I was fine (aside from the annoying cough) until the last hour when I started to feel queasy. By this time it was almost midnight, they were almost done and I was too tired to complain.

By the time they unhooked me I was feeling quite nauseous and headachy and was given some anti-nausea drugs. I tried to sleep.

I semi-woke later shuddering and shaking but too exhausted to even roll over and press the bell for the nurse.

Later when the nurse came in to do my observations I woke enough to ask for another blanket and tell him I was feeling cold and confess my quaking. The nurse promptly whipped out a thermometer.

I had a fever. He took my blankets away, turned on a fan and plastered a cold cloth to my forehead. I was too miserable to argue. My head was sore, the light hurt my eyes, my joints ached and I felt stiff everywhere. I had many of the flu-like symptoms of meningitis.

A doctor was brought in to check on me and they decided to cover their bases and give me IV antibiotics in case (though it was highly unlikely) I had an infection on the brain.

By the time my doctors came by about 8.30am the next morning I could tolerate the light and the headache and most of the stiffness was gone. After a bit of discussion with me and amongst themselves it was determined that I'd experienced meningism like symptoms as a delayed reaction to the Intragram. I'm still, almost a week later, troubled at times with aching joints (and the cough) but otherwise the symptoms have fully resolved.

My bloods showed the Intragram infusion had worked and my platelets were back up to 25. Because I "was a sensible person and tuned into my body" they were happy to discharge me.

I would have to go home and discuss with my hubby the next step in my treatment.
I would have to make a difficult choice.
The choice between surgery to remove my spleen or the drug Azathioprine.

I was given information about the drug and about post splenectomy considerations to help me make up my mind. We considered this information, studied abstracts from studies of ITP patients who have been treated by both splenectomy and Azathioprine, and read pages and pages of info on the internet about the risks and success rates of both these treatments. I talked to people who had taken this drug (though for different conditions to my own). We prayed.

On Sunday night we gave voice to the decision we'd made. I would have the splenectomy.

On Monday I updated my boss and discussed the time I'd have to have off work as a result, then trotted off to have another blood test. My platelets had increased to 31.

I had another test today and will find out the result tomorrow when I go into hospital to discuss our decision and find out what happens next.

Things go Pearshaped

By the 10th of January my Prednisone had been reduced to 10 mg and we had a plan in place to ween me off the steriods. My blood results showed my platelets were holding steady and the levels increasing. I seemed to be sitting pretty.

I was to take the 10mg dosage for 4 weeks then, all things going well, drop the dosage to 9mgs for another four weeks and so on.

On Wednesday 2nd of Feb a friend and I were out and about, making our way slowly to the book launch of a local writer. We were sitting in her car and I was doing my makeup in the sunshade mirror (as you do) when I noticed a rash of tiny petechiae on my neck and creeping down across my chest.

Up until then I'd not noticed anything so it had come on pretty quickly. I mentioned it to my friend and she offered to take me in to the hospital for a blood test. This we did and I was in and out in about five minutes.

Forty-five minutes later my cell phone rang. My heart leapt into my throat when I recognized the distinctive accent of my hematologist. Obviously something was very wrong.

My platelets had dropped from 185 to 23 in little over a week.

As calmly as I could I asked her what we should do. She advised me to double my Prednisone to 20ml and to go for another bloodtest in a week. It would take my body about five days to process and respond to the increase in my steroids. However, if I things got worse (unstoppable bleeding or very nasty and unexplained bruising, etc) I was to go for a bloodtest or to go into ED.

In the days that followed I had a little gum bleeding but nothing major. By the following Monday I had smatterings of tiny red petechiae all over me, especially down my calves and around my ankles. I was also becoming dotted with small brown and purple bruises on my legs, none of which I could account for.

On Tuesday evening I discovered a patch, about the size of an old fifty cent coin, of closely placed bright purple petechiae, each about the size of a pin head. I found another much larger purple patch on my chest. I'd never had anything like this before and it startled me.

I wasn't due for my blood test until Thursday but it seemed to me that I wasn't improving so the next morning, Wednesday, I took myself off to the lab for another test.

When I phoned my hospital nurse later that day it was to discover that I was right. My platelets had continued to drop. I was now sitting on 5!

Within 45 minutes my hematologist was on the phone instructing me to come in for an infusion of Immunoglobulin (a blood product of platelets).

It seemed we were back to square one.

Home again...

Once I came home from hospistal, rattling like a small chemist, I was off work for a few weeks.

The dosage of Prednisone prescribed by the hospital was 80ml, the highest recommended dose. My body was trying to recover from the shock of everything, I was struggling to get my head around the fact they my body was attacking itself (for someone who is almost never unwell this has been difficult) and to adjust to the side effects of the Prednisone.

I was tired, physically weak, emotional and hyped all at once.

My GP prescribed some sleeping tablets to be taken as needed as the Prednisone had been making it difficult for me to sleep. She also advised me to increase the Omeprazole to two tablets (80mg) due to the increasing and persistent pressure I was feeling in my diaphragm and advised me to take Mylanta when needed.

I was exhausted, felt weak and crying and my Dr insisted I take more time to recuperate before going back to my physically demanding job and provided me with another medical certificate. I was also trying to adjust to the side effects of the Prednisone, which made me tired, emotional and hyped all at once.

A strange pressure on my diaphragm continued to cause me discomfort, a bit like having a rock sitting at the top of my stomach, and I began notice a tight sensation at my throat, like someone had their hands around my neck or were pressing at the base of my throat. I wasn't having trouble swallowing or breathing so the discomfort was thought to be due to gastric reflux, which can be a symptom of Prednisone. The hematologist advised me to take a low dose of Mylanta 4x a day and I was too take an other Omeprazole tablet before tea.

My bloods were monitored by a weekly blood test. My platelets steadily increased and had jumped as high as 211 by the 23rd of November.

Over the next few months my Dr began to wean me off the Prednisone. My platelets fluctuated slightly but held steady, usually sitting around 100.

As the dosage dropped the side effects of the drug eased and once I was taking only 10mg the pressure in my throat had disappeared completely. However, as the dosage reduced so did my platelet levels until they were hovering more around the 70 mark. My Drs weren't overly concerned though and we settled on a plan to continue to reduce the prednisone with the view to wean me off it completely, though it was going to take a while. Now that I was on 10ml the dosage would only be reduced by 1mg every four weeks.

Still, it looked like I was stable and, all things going well, I'd be able to manage living with ITP without drugs or medical intervention, for the most part.

But it wasn't to be...

How my Story Began

This is an account of how I came to be diagnosed with ITP.

Please feel free to share your own diagnoses experience in the comments following this post.

I'd been feeling tired, which wasn't anything new for me, but on Monday 11^th October I noticed I had a few petechiae or blood spots on my right forearm [note: The arm in the picture isn't mine]. I didn't think much of it at the time.

However, the following day I noticed the spots had spread so I had more around both wrists and up onto my forearms, a few on my chest and more down my legs, especially around my ankles.

On Wednesday, while at work I asked a few of the the nurses if they had ever seen anything like it and what they though might have caused it. I was tired and headachey, but other wise felt fit. They wondered if it was a reaction to a multivitamin I had just begun to take and it was suggested to me that I cease to take it and see how I was in three weeks, after which time, if it hadn't improved I should visit a doctor.

That night while on the phone to my Mum I happened to mention the strange spots. She expressed concern, pointing out that they likely meant my skin cells were bleeding, and told me to go to A&E. I had a meeting that night, I felt more-or-less fine and I wasn't about to drag my family on a half an hour drive into Dunedin for something so trivial, but to allay her concerns I agreed to visit the doctor the next day.

On Thursday, as promised, I visited my doctor. She quizzed me thoroughly, examined me and took photos of my spots. Then she had a colleague examined me and together they consulted their medical encyclopedias.

They agreed that the most likely scenario was that I had ITP or Idiopathic thrombocytopenic purpura. This seemed to most closely fit my symptoms. They then contacted Dunedin Public Hospital (DPH) and consulted the hematologist and general surgeon there.

ITP is fairly rare (about cases 100 per million people a year) and initially when talking to my doctor the hematologist was very reluctant to concede that I might be suffering from this condition. Instead she seemed convinced that my doctor was miss-diagnosing meningitis (much to my doctor's chagrin). Other than the spots I had no other indicators of meningitis.

Samples of my blood were taken and I was sent home to await my blood results.

At 5pm that afternoon the doctor called. My blood work showed that my platelet count was extremely low. Normal levels should be around 300. I was sitting on 2! I was to go to hospital ASAP.

Hospital

Once at ED I was reassessed and re-examined. The placement of petechiae and a number of bruises of unknown origin, mostly of my arms and legs, were noted on a chart. I'd had some spotting on a tissue when blowing my nose and bleeding of the gums when brushing my teeth and these symptoms were noted too.

A lure was put in my arm and more bloods were taken. My family history of auto-immune issues were recorded; both my grandfathers had diabetes, coeliac disease, autoimmune liver disease and autoimmune hemolytic anemia (AIHA) and acute ITP.

At about 8pm I was admitted to the Haematology and Oncology ward on the 8th floor. I was given an infusion of antibodies IVIP (a blood product). I was also started on 80mg of Prednisone (a steroid and commonly used therapy for ITP) and because there is the risk of stomach ulcers and osteoporosis when taking this drug over an extended period of time, I was also put on medications to protect my stomach and my bones.

I was also put on a "soft foods" only diet, in case anything I ate scratched my mouth, throat or gut and cased it to bleed, and wasn't allowed to drink anything too hot or two cold. I was give little sponges on a stick (often used for mouth cares of the elderly) to brush my teeth to stop my gums from bleeding.

As I'd been suffering from headaches an MRI was done of my head to rule out any aneurisms or bleeding in the brain, which is a very real danger when suffering from very low platelet levels. The results were negative.

On Friday bone marrow was taken from my hip to verify ITP.

I was a bit daunted going into this procedure because I'd seen it done once of TV and it looked incredibly painful. My Mum had had this done before. She suggested asking them to increase the dose of "dope" that they give you so you aren't really aware of what's going on, because one dose hadn't been enough for her. When I didn't respond as well as expected to the initial dose they did increase it. And while it was a little uncomfortable, it didn't actually hurt.

The drugs definitely worked to wipe any clear recollection of it from my mind and may recollection of it now is very hazy. My hip was sore for the quite a few days following the procedure, though nothing a good dose of codine couldn't deal with.

The bone marrow results "indicated likely ITP". After the infusion the night before my blood work now showed my platelet levels had increased to 25 and while my doctors were pleased with this jump in my levels they decided to keep me in for observation.

Saturday my platelets had dropped slightly to 21. I was deemed to be out of the critical zone and allowed leave from hospital to visit with family in town, so we took the kids to McD's for a happy meal and play in the playground. There was talk that I might be able to go home the next day.

However on Sunday my platelets had plunged back to 3. I was transfused 1 pool of platelets followed by more Immunoglobulin IVIP therapy (antibodies).

On the Monday I was reviewed by the eye clinic over concerns that spots in my vision were due to bleeding in the eyes. This was determined to be due to severe myopia and not related to ITP.

Following the second infusion of platelets my levels rose to 50!

The following day they had risen to 57 and though they dropped very slightly to 53 on Wednesday, I was deemed to be stable and discharged from hospital.

On the Friday I returned for a CT scan of my chest and abdomen to rule out the ITP being a secondary condition to some other issue. Scans showed no secondary conditions or abnormalities.

For apparently no reason at all, my body had turned on itself and I now had ITP.

What is ITP?

ITP or Idiopathic (or Immune-mediated) thrombocytopenic purpura is a blood disorder where a person has a low platelet count due to an unknown cause.

Idiopathic = they don't know what causes it
Thrombocytopenic = platelet count
Purpura = the petechiae or bloodspots that are a symptom of low platelets.

In most cases (and in my case) the antibodies attack the platelets in the blood and destroy them.

My consultant explained it to me this way:

Your antibodies are your bodies policemen. When the body is attacked by disease or infection the policemen lock on to the invaders and take them down.

Now some people have lazy policemen and so have a low immunity and frequently fall ill.

Other people (like me) have over zealous policemen. They're are a little too enthusiastic and for some unknown reason have given my platelets "enemy" status. And so they are working really hard to purge my body of these so called nasties.

It would be nice if there was some way the little antibody policemen could simply be "retrained" so that they would cease to attack the platelets, but at this stage as far as I'm aware this can't be done.

The problem is, of course, our platelets are very important. We need them for blood clotting without them we bleed a little too freely. This puts those of us with ITP at much higher risk of bleeding to death in a car accident, for example, or a brain aneurism from a blow to the head.

According to Wikipedia,

The mortality rate of chronic ITP patients varies but increases as a function of age. In a study conducted in Great Britain, it was noted that ITP patients experience an approximately 60 percent higher rate of mortality than gender- and age-matched comparison subjects without ITP. This increased risk of death with ITP is largely concentrated in middle-aged and elderly patients. Ninety-six percent of reported ITP-related deaths were patients 45 years or older. No significant difference was noted in the rate of survival between males and females.^[9]

It would be nice if there was some way the little antibody policemen could simply be "retrained" so that they would cease to attack our platelets, but at this stage, as far as I'm aware, this can't be done.

A healthy adult will have platelet levels of 150,000-450,000 per microlitre of blood. Anything below 150 might be considered thomboytopenic. Less than 10,000 (10) is considered critical and in my experience usually involves hospitalisation and therapy to bring up the level of platelets in the blood.

ITP can be brought on by illness or as reaction to something, such as antibiotics. Sometimes, as in my case there seems to be no rhyme or reason for the sudden on set of ITP.

Children who develop ITP often go into remission. This is less common in adults who are more likely to develop chronic ITP. More women seem to develop ITP than men.

To find out more follow these links.

Wikipedia

US Department of Health

Welcome

Hi and welcome to Living with ITP.

I hope this blog will act not only as a personal journal but will also be a support and encouragement to others living with ITP (Idiopathic/Immune Thombocytopenic Purpura) and other similar blood disorders.

I will share my highs and lows as I come to grips with living with ITP, the tough medical decisions I must make and my responses, physically and emotional, to the therapies I undergo.

I am not a doctor or specialist.

The opinions expressed here a purely my own. This is a journal of my personal experiences living with ITP.