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.

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.

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?

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.

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