I’ve Got What????

Bron Hatton is one tough Aussie! Both she and her son have ITP but they are not letting it get the best of them....

I was diagnosed with ITP in early 1998. I had been having heavy periods and felt really flat, so I thought I was anaemic so went to Docs for some bloods at 10.00 am one morning 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.

Next morning, after all the drama had settled and they got to examine me was when we noticed all the patechiae (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), told me I had this thing called ITP, put me on 150mg of prednisone per day and sent me home after 3 days. Let’s see how you get on they said and for the next months it was weekly blood counts and bruises like I was in a fight with Mike Tyson.

 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 offey 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, I got the shakes, heart palpitations, joint aches like I was 100 years old and moods that could kill a horse with a single look.

This went on for nearly 2 years, with each taper of prednisone dropping the counts. At this point after finally being referred to a hematologist in Sydney (I am 250 km’s away), I was given IVIG for the first of 13 times over 3 years. I had crappy veins, not helped by the pred, so each time a cannula went in it was a nightmare. It got to the point that you got two goes at my arm 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 3 months in hospital over one year and ended up seeing a counsellor suffering from deep depression associated with all that goes with ITP.

My heamo team at RPA decided to try dapsone and dexamethasone as well , it just didn’t work so splenectomy was the next move. Again a far less educated me said yes, 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 on line. 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 and I also planned a wedding and we got married. 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.

He was dismayed to hear I had had my spleen removed and knew just by 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 pred gave me 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.

I was induced at 37 weeks because they thought Will was going to be huge. 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 heamo team were ok with it all.) It was uncomplicated and a waste of all the blood and IVIG they had on hand. 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 when I had my next round of rituxin with a new heamo 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.

I manage my own ITP so to speak. My heamo 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).He has agreed in the future that because Rituximab is successful and pretty much the only thing that works that I can have it again.

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.

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, 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 next year.

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 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.

We have a team for me and a team for Will.  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.

Advice I would give to folks 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 etc. Focus on life and get on with living it.

Hello Everyone! My name is Erica and I have ITP. I've had ITP for 26 years. 

My parents took me to the hospital because my nose was bleeding and I was bruised from head to toe. The doctors had told my parents that I had a rare blood disorder. At the time the doctors didn't know how to treat it. As life went on, I would experience flare ups, some minor, others major. I was never allowed to play sports or do any kind of physical activity. Playing with my brothers and sisters was limited because they were very active. Sometimes all I could do was watch from a window.

As I got older into my teen years I had an episode where my platelet count dropped to three...yeah not three thousand but three. Doctors immediately wanted to remove my spleen (apparently that was the “cure”). My dad refused as he thought there would be a better way. After meeting with numerous doctors he finally found someone who would listen. Her name was Dr. Goldberg, and she started me on prednisone and IVIG. I was in the hospital for two weeks. My platelet count would go up and down but eventually stabilized.

As a child with ITP, I went through a lot of teasing because I had the "moon face". I learned how to laugh it off and move on but more importantly, this is where I formed my relationship with God. For the next few years, I would experience a couple of flare ups but nothing major. At that time I had an excellent Hematologist who kept up with my platelet count.

I later on became a preschool teacher (one of my dreams), but I kept getting sick because of the germs and viruses that was going around. One day, I was outside with my students and my nose began bleeding. My first thought was that I had another drop in my platelet count, so I was pretty scared. That day, I was walking home because I lived around the corner from my job. My husband happened to be on his way home and saw me walking. He described my walk like as if I was about to pass out. But I told him I was okay.

Later in the night as I was getting ready for bed, I looked at my legs and saw they were covered in bruises. I immediately called my husband in the room and said we have to go to the hospital. We went to the hospital and doctors ran some tests. I overheard some of the nurses talking and I heard, "do you want me to tell her or do you want to tell her." I started crying right away because I knew my platelet count was lower then I thought. One nurse came in and held my hand and said, “I don't know how you survived the day.” Confused, I didn't know what to say. She continued to tell me what my platelet count was.

One.

So I had to quit my job (a job I loved) as a teacher and my husband gave me the option of not working altogether. He felt that it was more important for me to be as healthy as possible. He knew that it would be difficult for us financially, but he told me that he would figure it all out. Thankfully, there wasn't that much to figure out.

Now I am 30 and haven't had an episode since. I am always checking and I get my blood checked periodically. Though my hematologist told me I would have this for the rest of my life and it is something I am going to have to keep my eye on, I try to stay positive. I started working out, eating healthy and taking my vitamins. I didn't want to spend a lot of time on the couch so I started my own photography business where I have control over my schedule. I also spend a lot of time with my son who is my little hero (ever since I had him my count has been wonderful).

I must say I owe everything to God because He gives me strength. He gave me my husband and my son, who are my support. He has shown me that I may have this disease but I am not defined by it. ITP does not define me, and I jump at any opportunity to tell others the same.

Stay positive...keep smiling...and I am sending hugs to you all. God Bless.

2013 - What will it bring....
           More bleeding, bruising,                   treatments or remission? 

Have you come to terms with ITP? Has it become just a part of your life now and not so scary anymore or do you still worry about your platelet count? 

Can we ever come to terms with ITP? Do you have days when dealing with ITP, family, friends, work, etc. seem to overwhelm you
like it did with this person.....

It is such a harsh reality to see how our families just don't get it. They see it all....the vomiting, the headaches, all of it and yet some of them just don't understand. My sisters and brother don't even call anymore because they think all I want is money. I am expected to listen to all of their complaints and problems and pray for them but where are they now? Where are they when I need them? I think it is harder on my kids because my husband and I both are dealing with illness. The difference with my husband is his basically controlled with pills. He had the stroke because he didn't listen and yet I did nothing to get this crap and somehow I am over reacting when my teeth are bleeding or I have bruising everywhere. Both of us can't get private insurance because they don't cover ITP (unless you had prior coverage and were diagnosed while covered), so we have to rely on medicaid share of cost (you have to prove that you need medical care). I was told today that when my son turns 18 we will lose that coverage too. I have my husband telling me that I am worrying over nothing. NOTHING!!! Maybe to him it's nothing but to me my life now depends on getting these shots. I missed three weeks and went down to 19,000. I think it hurts more when your husband and children don't seem to understand. Four years of this....I think it is getting to be too much for them. I think they just want it to stop but it does not look like that is going to happen. I have faith and I pray and I push myself to believe but today, I am just having a bad day. We can't be strong 24/7/365 like everyone expects us to.

What are your thoughts? Leave a comment and let us know how you feel. Is it possible to ever come to terms with ITP?

I hope everyone had a wonderful Christmas!

We are coming up on a new year now and my Christmas wish is for everyone to have love, good health, happiness and lots of platelets in 2013!

What is your Christmas wish? 

Comment below and let us know. If you do not have a google account, you can comment as anonymous. Thank you everyone for supporting our ITP blog and remember you are more than welcome to send a guest post any time you want!

Please share your Christmas wish....

This week's question is again stolen...er....borrowed from one of the ITP groups I am in.

We often discuss all the negative aspects of having a chronic disease but developing a positive attitude can be beneficial to our mental, psychological and physical health.

Today, I would like for everyone to list the positives of having ITP.

Some of the responses in the support group were:
     -- If I get Lupus, cancer or any other medical condition, it will be diagnosed earlier.

    -- I have met some wonderful people through medical services.

   -- Strengthen faith.

  -- Make me slow down and take care of my health.

  -- I've learned to live life to the fullest since I can't change things.

What are your thoughts? Has anything positive come out of having ITP. Please leave a comment. If you do not have a Google account, use the drop down box and use "Anonymous".

What Do You Think II

Anthony Heard came up with our ITP question of the week...

Despite what your doctor or specialist may have said WHAT DO YOU THINK MAY HAVE BEEN THE CAUSE OF YOUR ITP, WHAT DO YOU THINK MAY HAVE TRIGGERED IT ? 

Anthony commented, "I would just be interested to hear what other ITP folk think. I have my own theory about why my ITP started but my specialist says no. For the record I think my ITP started as a reaction I believe my immune system had to a bereavement (that is what I think but who knows, it's as good a theory as any). Would be interested to hear what other folk think.

Give us your thoughts by leaving a comment about this question. 

Greta Burroughs

I think mine was triggered by stress. My parents were both undergoing chemotherapy and I lived too far away to help them. At the same time, my husband had three stents implanted around his heart resulting in his aviation career coming to an end. I was worried sick, hyper and lost a bunch of weight. After a year of self-induced stress, I was diagnosed with ITP. 

How about you? What do you think triggered your ITP?

TO I.T.P. AND BEYOND

Ros and her daughter Calley in Perth.

Ros Bryan lives in Australia. She has had her ups and downs with ITP but support from her ITP friends and her family has kept her strong through it all. Here is her story.

Hi everyone, you are about to get a warts and all story from me, in fact, honestly I don’t even know if now is a good time to write it.  For all of you that have ITP, or know someone with it, you will know it can be a default line in your life. Right now for some reason, probably because it’s coming up to the one year anniversary, of the most dreadful time of my life, I’m going through a really big downer. This is where living in Australia, is not ideal, lol, because right now I’d kill for a hug from one of my ITP friends.

My story began maybe late Oct, early Nov 2011, I noticed I had a rash on my stomach. Being the laid back person I am, I didn't really care. I figured it may be washing powder, fleas from our animal’s, heat rash, there was a million things I put it down to. I did promise myself next time I went to the doctor I would ask. Meanwhile I was also spitting up and blowing blood from my nose, lol, still it didn't bother me.  A couple of weeks later when I did see the Doc, he immediately gave me a referral to a lung specialist, thinking maybe I had lung cancer.  I forgot about a rash, until I was on the way out, so I then ran back in to show him, he put it down to viral. So I was none the wiser, lung cancer did concern me, but not overly.

 

A couple of days later, the rash appeared on the back of my hands, this did freak me out. I decided to go to Mum’s doc, to get a second opinion. He immediately sent me for a blood test. This all happened on a Friday, since I had not seen him for goodness knows how long, he didn't have my contact details.  Being a Friday night about 7 pm we were just about to walk out the door to my daughter’s netball. I had a phone call from my Mum, the doc had contacted her, because he couldn't get me, to tell me to get to hospital immediately, I only had 2000 platelets. I remember telling her sure, I’ll go after netball, that’s when mum had to relay the message if I didn't get there immediately I may die, oh yeah, and don’t fall over or bump your head on the way!! 

I think my legs gave out from under me, and my head certainly spun, as I was hysterically   crying so much I couldn't see.  My son took my daughter to netball, and myself, my hubby, and Mum went to hospital.  I knew it must've been bad, no doc, ever rings at 7 pm over here.  A little detail I do remember is the nurse shoving, and I mean shoving a canula in, while coughing all over me, I was a bit calmer by then, but  besides babies, I had never been in hospital before, and I was certainly not staying there this time!! LOL, or so I thought. After me giving them a lecture about how I was going home, so hurry up and fix me, the Doc, had to tell me if I walked out that door now I would die (one of my greatest fears), so rest assured I wasn't going anywhere, Mum was with me, trying to small talk, gee, I was horrid, telling her to shut up etc. I was in over night before meeting a Hemo the next day, who told me I needed a transfusion because I had ITP. After asking questions, I still had no idea what it was. That day over 6 hours I had a hemoglobin transfusion, (I think, see I still don’t know)!!! My platelets came up very slowly and I was released about 5 days later, with 2700 platelets, and a 2 page brochure, about the blood disorder. One page, was actually pictures, that’s how well informed I was!!! I was a nightmare for my hubby, poor thing.

I had to go back for an outpatient 2 weeks later. I was on 130 mg of steroids so my platelets had gone up. I also not wanting to whinge mentioned to my doc that my arm I had the transfusion in  was still really sore, probably nothing, and I wasn't worried. She insisted on getting it checked, but forgot to give me a form for an ultrasound. Meh, I didn't care, I was sure it was nothing, but she posted one out, and told me to go. For that I went to a different closer hospital. Of course I had a blood clot in my arm, superficial, so it should be okay but it seemed every time I went to hospital I was taken into an emergency room and given more bad news, and was booked in too see a doctor there. That’s where the memory of the Nurse came back to me. I reported that to the doctor, I have never seen one so angry. Well I didn't make it because the blood clot had moved to my lungs. A PE man, I couldn't catch a break. 

You know when you go to a public hosp and report in, and they chuck you on a bed and rip off your clothing, that something is really wrong!! So I had to stay in the hospital for a couple of nights observation, as the Cat Scan had shown a lesion on my lungs. They had to be wary in case it was cancer!! I was a freaking mess, by this time. Home from hospital though, cry enough and they’ll let you go!! They started me on a needle called Clexane, a Warfarin type drug, but couldn't actually give me warfarin itself in case I needed a biopsy, and of course I would probably bleed out. So for about 6 weeks I was on needles given by nurses at home 2 times a day, a great service we are blessed with. I couldn't sleep in my bed, I was frightened to moved off the lounge, I love my daughter with all my heart, but didn't want her cuddles, I had just lost the plot!!

Anyway, now I can cut it short, the lesion came down in size with every fortnightly cat scan, so they were confident it was only a clot, not the dreaded “C” word. However my doc, told me he would continue to pray for me, man that totally freaked me out. I couldn't at the time see it as a nice gesture to me. It meant I had to be dying.

Eventually after 6 months I am off the warfarin, however I still have to have another cat scan, that I am putting off until I go back to the hemo on Nov 12, so I will arrange it then. I am being weaned off steroids, I think this is my 3^rd time. Of course, then I crash and go back on them. Thanks to all you out there, I am aware of different meds and treatments. Short term I have had no trouble with steroids, except extra sweating, and not being able to see 3 feet in front of me. I was told by my Hemo that steroids cause a thin watery lining over your pupils and basically seeing is really hard, and it is. I do know from previous experience it will go away though. 

I'm certainly going to inquire about the other drugs and treatments though, I think steroids is just the easy option. He must dread me coming, with my list of questions, that makes me sound really clever and knowledgeable.  Of course they are just stolen from all of you, but it keeps him on his toes. I do share care with my doctor though, because mine is fairly standard !! I’m hoping this time will be THE time, that it works, and ITP just disappears, I really don’t want it anymore.

One thing that I do know for absolute certainty, that without my ITP GROUP, that I accidentally found, I would be nowhere. Further, I have gained so much knowledge, love in abundance, and friends that will last forever. I'd love to name you, but you know who you are!!!  My hope is to start something like this in Australia. Without my group, I wouldn't have had the confidence or support I need to be even able to think that. I don’t have a rash, I don’t bruise, I just don’t have platelets, so I guess really it doesn't or shouldn't effect my life the way I let it. I don’t all the time, just sometimes like now!! 

Somewhere in between this, we have moved state for my husbands job, I know absolutely no one in our new State, but it’s kinda cool. I don’t have to meet someone and say, "Hi I’m Ros, and I have ITP." I have 3 children and one has gone back to our old state, because he is at the end of schooling and the curriculum is just too different between states. He is happy and living with my parent’s. I try to tell myself its just like boarding school, but just thinking about him not being here makes me cry, I need him, lol. My hubby has been fab, taking me to my appointments etc, but truthfully I think he may be sick of hearing about it.  I think maybe I am suffering a little from PTSD, maybe, but I will cruise through it,(reading what I've just written, I guess I'm entitled though). Well that is my plan, but I'm not silly. I will get help if I need it. Like I mentioned earlier though, I really would give my right teeth, to have a hug and cuddle with Greta right now. xxx

Ros, here's a big hug from me and all the other ITPers reading your story. Thanks for sharing!!

A "Normal" Life with ITP

Alison is from the UK and her son has ITP. He is a typical teenager and does not want to be different from all his friends. They have been very fortunate in that his wish to be 'normal' has been a reality. 

I 've been really touched by reading the stories on this blog for the last couple of months.  But I have sometimes found them a bit scary, so I thought it might be useful to show a less worrying ITP case.

We live in the UK, and my son was diagnosed with ITP aged 10 after we discovered he was suddenly covered in bruises one Saturday in May 2009.  We were told to take him to Accident and Emergency, where 4 hours later we finally got to see a doctor (those in the UK will probably recognise this as a typical Saturday evening at their local A&E Department!).  He was admitted for the night, and in the early hours of the next morning, a doctor came to tell me he had a platelet count of 5 and they thought he had ITP.  I was told it would probably go away quickly, not to worry too much, but he would have to take care for a few weeks.  Doctors in the UK generally treat ITP in children on the basis of symptoms and not counts - my son was fortunately only covered in petechiae and bruises with no active bleeding at all and so he didn't receive any treatment. The next morning his count was 9 and he was discharged at lunchtime and we went home to watch and wait for things to improve......

His count didn't go over 20 for the next six months so he had a bone marrow test - very uneventful, he was cycling his bike down to school to pick up his little brother a few hours later.  It didn't show anything abnormal. After about 6 months his count suddenly went up to about 120 and we all thought it was over ............ for at least a few weeks until it plummeted to under 20 again!  

This was followed by a fairly good year countwise - counts went between about 5 and 50 but they were mostly around 30.  About 18 months in though, his count went down to about 10 and it has mostly stayed somewhere between 5 and 20 ever since.  He still has very few symptoms - petechiae on his legs if his count is below 20, bad bruising and bleeding gums when he brushes his teeth if it is below 10, and an occasional heavy nosebleed. He's had a few accidents and sports injuries where ITP complicated things but nothing serious. He tried steroids for a few weeks after a run of nosebleeds (didn't do much) and IVIG once (worked, but gave him the headache from hell).  If he has a few nosebleeds now, we give him a couple of days of tranexamic acid which seems to work.  Fingers crossed, he is about to start the Eltrombopag trial, and he hopes that this will allow him to play rugby.

I sometimes read about children (and adults) with ITP who have very restricted lives and a lot of medical intervention. I feel very lucky that, three years in, my son has a very normal life. He sees the haematologist every couple of months and misses school to attend these appointments, but otherwise has missed only a couple of days of school due to ITP in the last 3 years.   I think his doctor is very relaxed compared to others that I have read about and he is happy for him to do pretty much anything if his count is over about 20.  This helps to keep things normal and keeps the worry to a minimum. The doctor has told him to limit activities that may result in head injury, but he is a typical teenage boy and only hears what he wants to hear! 

I 'think' he has understood the message on some of the more risky sports and activities, but I have also resigned myself to the fact that he does pretty much what he wants to do otherwise, and just doesn't tell his father, myself or the doctor about it.  It probably helps that we keep restrictions to the absolute minimum, so he knows that when we say no, it really must be too risky.

He plays basketball, tennis and football (soccer) with our permission, although when he is particularly bruised he doesn't play competitive football matches.  He goes to scout camp where I am told he climbs trees with the best of them.  He playfights with his brothers and friends and he rides his bike (with a helmet on if I see him first!).  He goes paintballing with friends as well as and bombs down hills on a sledge on the rare occasions that we get enough snow. We have just been to a theme park where he rode all the most scary rollercoasters and the photo above is him jumping off a cliff into the beautiful Greek sea a few years ago - his count was about 20 at the time.

Through reading other people's ITP stories, I realise how lucky my son is, and I am thankful every day that he only has ITP.  Every time I take him to the haematologist, I think that my son must be the 'wellest' person that he sees - I even feel like a bit of a fraud taking him at all!  Yes, ITP is a real nuisance and a bit of a bind sometimes, but that's all it is at the moment -  in fact, my son tries his upmost to ignore it! I often hear people describe themselves as ‘fighting’ or ‘suffering from’ ITP and I don’t think those are the right words for my son at least – ITP is just something he has, a bit like I would describe him as having brown hair, or beautiful green eyes!

With luck and a following wind, I am hopeful that it will remain that way.

 -

ITP Three Little Letters

We have had several posts from adults with ITP, now we have a mother explaining the anguish of watching her child deal with this blood disorder.

This family resides in the UK but this same scenario is repeated worldwide. I applaud all parents who cope with dealing with their children having ITP.

 It was supposed to have gone away within six weeks, yet three years later those three little letters, ITP, are still a huge part of our lives.

It began with a bruise which appeared overnight on our two year old son’s arm.  It was vivid purple, hard to the touch and raised up—we were not even sure it was a bruise at first.  It was the weekend so we trekked off to the minor injuries unit at the hospital, and within an hour of seeing the doctor there, we were at the acute assessment unit of children’s hospital and our lives were changed forever.

The diagnosis of acute ITP is one of elimination: we can’t identify it as anything worse so it must be ITP.  We were given an appointment to see the haematologist and went home to google Idiopathic Thrombocytopenic Purpura.  The haematologist told us that 80% of children spontaneously go into remission, more so if they are male and young.  Our 2 year old son had good odds.  We should watch him carefully and wait for him to cure himself.  Watch and wait—how hard could that be?

On our second appointment with the haematologist 8 weeks later, we were told that our chances of complete remission were still great. Our son’s count was still under 10k (the normal range is 150k to 400k).  We didn’t let him have a play centre birthday that year.  We went home to watch and wait some more.

Over a year later, we were still watching but mostly waiting.  We had a lively, active four year old and those restrictions we had enforced in the early days had mostly gone by the wayside.  We had to let him live his life. He had bruises and petechiae but no more serious symptoms. He was about to start school and for me the thought of him running around the playground with 200 other kids was terrifying!

Our haematologist supported us in feeling that our son’s quality of life could be improved by medical intervention and we agreed to try Rituximab in November of 2009.  This is a drug (originally developed for chemotherapy) that would basically destroy his immune system and allow him to rebuild it; the analogy we were given was that it was like rebooting a computer to fix a problem.  Our son was 4 and his platelet count was 9k.

Watching your child get hooked up to an IV for the first time is hard to take.  He was fascinated by the process, though, and happy to spend the day on a hospital bed channel-surfing his own TV and playing Nintendo.  The first infusion was slow, the second saw his count rise to 14k  - double figures for the first time since diagnosis.  It was looking good.  Then he contracted chicken pox—a normal childhood disease but possibly serious for someone with a compromised immune system.  The morning scheduled for his fourth infusion, he woke up with a severe nose bleed.

We headed for the hospital and were put in isolation because of the dangers of chicken pox for the oncology patients on the ward.  The bleeding did not stop and he started vomiting the blood that was going down his throat.  The consultant gave him two units of intravenous immunoglobulin (IVIG) and a unit of whole blood.  As soon as the IVIG hit his system, he stopped bleeding and was able to sleep.  We spent two nights in hospital and were then sent home.  For a while he lived on the borrowed platelets and started to look great: no bruises, no petechiae.

Then they started to reappear. He had his last dose of Rituximab but sadly his platelet count did not go up any further. Shortly before Christmas of that year we were back in hospital with another unstoppable nose bleed. More blood products given by anonymous donors helped to keep our son alive. 

In Jan 2010 he started school.  Together with the school we put a care plan in place for him.  His consultant gave permission for him to take part in all aspects of school life including PE, but clearly he would need close monitoring.

Two more nose bleeds, two more rounds of IVIG, and spring finally arrived.  The cold season was hopefully over and we would be once again free of snotty noses leading to bloody noses.  With his count running consistently under 10K we were advised not to fly long haul.  So our family visited us last year instead!

Autumn approached and he wanted to do tag rugby—we got medical permission and he loves it!  But every club, sport and outing involves an explanation of his disorder.  Every glance at his bruises when he’s out and about makes me feel sick.  Once at the swimming pool, a ‘concerned’ mother suggested to me that if my son had chicken pox he should not really be swimming.  His body was covered in its usual fine petechial rash.  My son stopped her in her tracks by confidently saying ‘Actually I have low platelets and my blood vessels burst on their own and that’s what you see; I’m not contagious!’

This last winter was mercifully kind—we visited hospital only 4 times and each time the nasal bleeding stopped without any medical intervention.  His count, however, stayed mostly below 10K. We tried a short burst of steroids but he did not respond to them.

He turned 6 in the spring and had his play centre party, and my hair turned a little greyer!  We are now starting the process of entering him in a drug trial in the hope of raising his count to a safer level. It’s a long road and there are many mountains we have to climb before we are even guaranteed to get him the drug. Weekly visits to the hospital for blood draws and he has to learn to swallow tablets!  Even then it will not cure him just hopefully get us through next winter without any hospitalisations.

Three years on, we are still mostly watching our son grow and waiting for his body to correct itself. His most recent count was 18K: low for most people but a cause for modest celebration for us.  We’ve learnt not to put too much emphasis on his numbers though, rather to look at his symptoms.  He gets very tired and is often emotional and has a tendency towards always expecting the worst possible outcome.  What he doesn’t know about germs isn’t worth knowing!

Complete remission is still possible for him although it feels as if it is getting further and further away.  Those three little letters still play an enormous role in our lives but our story is not over yet.  In our family we have tried to put back into the system at least as much blood as we’ve taken out since his diagnosis (even though the IVIG is made from thousands of different donations). We do what we can.  If you can donate blood or platelets, we really appreciate it and lives do depend on it.