Something New For ITP Awareness

Last month, I was sitting here in my cluttered office debating on whether I should start cleaning this place up or do something else.

Cleaning is my least favorite activity so the "something else" option was open for any clever ideas that might pop into my head.

Earlier that day, one of our fellow ITPers, Tom Schilling, had asked about where to find decals his auto-racing buddies could put on their cars and equipment to promote ITP Awareness Month. PDSA had some decals but they were too small and custom-made decals were too expensive.

Ah ha, maybe I could make something with my handy-dandy Paint.net program. That lead me to making several designs to put on various items in my Zazzle store and now I'd like to present my efforts to you.

I'm a firm believer that we need to spread the word about ITP Awareness, not only in September but all year round. So I made some coffee cups, tote bags, key chains and bumper stickers that would make great conversation starters in our quest to inform our friends and colleagues about our blood disorder.

 Take a look at the items I created and give me your thoughts, constructive criticism and any other ideas you may have on how I can accomplish my goal of "Spreading the Word."

I'm going to make more designs and try to add some T-shirts and posters to my Zazzle store as time goes on. 

Take a look, and see if there is anything you'd like to purchase. A portion of the royalties will go to PDSA.
Here are the links:
US 
UK                                    
Canada 
Germany 
Spain 

France 

Sweden 

Netherlands

Norway

Switzerland

After you get to my home page, click on this icon to go to the ITP store: 

Thank you my friends and may you all have high platelets.

Happy ITP Awareness Month

Hello, my fellow ITPers.

Below is an email I received this morning. It is directed to the parents of children diagnosed with ITP.  I'll leave it up to you to research MedQuery and decide if you want to participate.

Disclaimer:

I do not know the company or the person who sent the email. I am not endorsing this company and am not receiving any compensation for posting this on our blog. 

Hi Greta,

My name is Della and I work for a company called MedQuery based in Chicago, IL. MedQuery is a medical market research company and we are hired by pharmaceutical, medical device and biotech companies to conduct paid market research interviews and surveys for them. The reason I am reaching out to you is because we have a market research opportunity and I hoping your organization could help us reach the parents of children diagnosed with ITP. This is in no means a sales approach or solicitation. We are simply interested in hearing from the parents to understand the day to day impact this diagnosis has made on their child’s life/on their life and any areas of unmet needs whether it is referencing pharmaceuticals, educational information, support groups or even physician familiarity etc.

The interview would be a 60 minute telephone interview on the topic of Latest Developments for Chronic Idiopathic Thrombocytopenic Purpura. In appreciation for participating, we are paying respondents $150 and they would need to complete a short homework assignment 1 week prior to the interview. The interviews are being conducted from January 10th - January 24th so we are hoping you can send this out as soon as possible.

I was hoping you would be able to post this on your blog and if anyone is interested they can directly contact us at MedQuery. Please call Jackie, recruiting manager, at 312-241-1649 to see if you qualify to participate. WE REALLY NEED YOUR HELP and we look forward to hearing from the blog members.

Thanks in advance!

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.

Samuel Lollman has to be one of the most cheerful kids around. He has had ITP since he was a wee lad but that beautiful smile never disappears from his adorable face.

His mom, Julie shared their story of bruising, hospitals, treatments and determination to live a full happy life in spite of ITP....

We jokingly refer to Samuel as our million dollar baby. Being a preemie and spending a month in ICU will earn you that title.

A year after he was born there was little sign that his life started out fierce and fighting. Cruddy ventilator lungs was the only lasting sign. Samuel was  your average 3yr old who loved life and left nothing unexplored. 

The first bruising we noticed was in 2009. I remember telling my older son that he needed to not be so rough while they are playing. ( awful, I know) It was winter and the boys had been playing in some unexpected OK. snow. Later that day he developed huge bruises across his lower back that seem to get worse and spread out by the day. I was at one point even worried the school would think we were beating our child! I still never had any alarms in my head going off and chalked it up to great outside fun and a clumsy boy. 

A couple months later, I watched Samuel lean over to pick something up and barely hit the corner of his eye. He didn't even flinch and I thought nothing of it. Moments later, I look at him and there is a huge ball on the side of his eye that is black and blue. Bleeding inside his eye and blackened the other side of his face and corner of his other eye. Then the blood looked almost like it was slowly moving down his face. I had never seen anything like it! 

Finally some alarms went off. His brother was going to pediatrician the next day for a followup and I decided I should probably take him with me and just

let him take a look at his face. Our awesome pediatrician did bloodwork just to be safe and when he came back he said that Samuel had only 5k platelets and normal was 150k and above. He then proceeded to tell us that he is at risk for inter-cranial hemorrhage and that he could not rule out leukemia. He told us to head to the hospital and that they would be waiting for us. Ugh. In my head I am thinking, ok, what is a platelet? What did they do? How did a followup appt. end up with us going to the hospital fearing for our child's future? We head home to pack a quick bag and drop off our older son.

At the hospital we waited half the day and night. They gave us a diagnoses and said they were pretty sure it was not leukemia. I've never been so relieved. Samuel began his first of what would be 30 plus infusions of IVIG. The infusion lasted 8 hrs and nasty side effects followed with fever, vomiting and migraine.  

We were given all the odds and percentages for ITP and we were hopeful as the remission rates for his age were great. Only 4% will go on to be chronic as children. 

A week later his numbers were in the 400k range and our hemoc. told us she thought he was in remission. Yeah!

Two weeks later we went for a followup. I noticed bruising coming back and after a CBC, his body had already chewed through most of the 400k platelets and was down to 60.  This became a pattern over the next year. Every two-three weeks he was in hospital getting IVIG infusions. It was definitely a lifestyle change. Weekly blood draws and missing out on fun things because he was in the danger zone. As a Mom and friend, I became the most unreliable person. We could never truly make plans at school or otherwise because we simply did not know what our week would entail. 

After a year, we realized that Samuel is part of the 4% who's ITP is chronic. We explored another treatment option called mercaptopurine . This was an oral chemotherapy  pill given  to leukemia patients as a maintenance drug. Scary, but it was shown to have good results with long remissions. It would be the first time Samuel learned how to swallow a pill and he did it like a champ. He took it at night at certain specific  times. He did not have many side effects, mostly a nauseated stomach. After 4-5 months I began researching the drug and found many disturbing facts about secondary leukemia risks while taking this drug. It was not helping his numbers and his doctor took him off. 

We began discussing splenectomy and vaccinated him and prepared. We decided on doing a bone marrow biopsy to be sure prior to surgery to make sure we were not missing anything. That was tough and glad it was done. During this time we started noticing bruising and bleeding with higher counts. I would assume he was low, take him in for labs and numbers would not match the symptoms. This began a whole series of testing putting splenectomy on hold. Aggregation testing and platelet function testing were coming back abnormal. So not only did his body fight off his platelets, the ones he did have were not functioning. Many, many tests were done for months, specialized tests sending as far as as Wisconsin with no real answers.

 

In Feb. of 2012 I was getting Samuel's bath ready and I could hear him commenting on how his pee looked funny. I turned and looked and there was a stream of dark brown. It didn't even register what it was at first. You get that familiar pit in your stomach and I knew we were in for a long night. I called our doctor and she said to head to children's and they would be waiting to admit him. This began the scramble of packing bags for the hospital and getting our son Ethan somewhere. We are good at not panicking at this point, but the unknown of "what now" is what can drive you crazy.  He was in the hospital 3 days until the bleeding stopped. He had CT scans, MRI and ultrasounds. His platelet count? 80k! Samuel for sure got everyone's attention. The bleeding was from the kidney so a nephrologist was called in. Due to the bleeding, he could not do a kidney biopsy. He did other testing for kidney function that was normal. The hematologist blamed the kidneys and the nephrologist blamed ITP. At this point we were scared that we did not have a clue at what we were dealing with nor did anyone else. 

We decided to try Rituxan. IV chemotherapy was given once a week for a month. This also unfortunately did not nothing for his counts and was a very long month and rebuilding of his immune system and side effects.  I had already been researching, trying to find a platelet specialist. Boston kept coming up and before long we were headed there for a second opinion.  They were super excited to meet Samuel and the first conversation was spent discussing our approval on putting Samuel on their new study for testing. Although very insightful, our options were still limited. Both Boston and his local doctor are concerned with the possibility that there is something underlying going on that has not surfaced even though he has been tested for everything under the sun. He is an awesome doctor who I am thankful we were lead to. He has discussed Samuel's case with rooms full of oncologist / hematologist around the world who met at oncology conferences for their opinions. How awesome is that! 

So now we are in somewhat of a holding pattern. We have had to stop using the IVIG because of the chemical induced meningitis he was having. The most recent treatment he had is Winrho. It was nothing short of a nightmare. He was a very

sick little man and we had some scary moments with this treatment. 

This brings us to our current date. We will be going to consult about starting NPlate. There is positive information regarding this drug. Although I am very skeptical to keep experimenting with these new drugs. It was shortly after stopping the 6mp that he began the weird bleeding symptoms. Coincidence? I am ready to pull out his spleen and be finished but his doctors are still worried there are other issues. There is actually a test that can help determine whether it is spleen or liver that destroys the platelets. Of course this test is only offered outside of the U.S.  Why? Well there are two thoughts:

1. Not enough need for the test and the isotope that is used in the test is very hard to get and the lab has to be certified to get it which is very expensive and hard. This was concluded from a Red Cross research facility that used to perform the test but can no longer because of the isotope. We had an interesting conversation for sure. 

2. The test just is not a good tool. 

That brings us up to date! 

Who is Samuel? He is not the poor 6yr old that has ITP. He is the most happiest, energetic, kindhearted child. He takes every needle and treatment in stride and can be sick as a dog and wake up smiling. We are not dramatic in speaking with him about his disease and after three years he does not really know any different. We find something fun out of every hospital stay and doctor visit. ITP  is not who he is and to speak to and see Samuel, you would not even know he is sick and that's our goal everyday. God gives his toughest battles to his strongest soldiers and Samuel is a survivor and a fighter. 

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.

 -

Zane's Story

           Ronda Green is sharing the story of her young son, Zane with us today. In the picture to the left, the youngster jumped off his bunk bed and hit the frame of his brother's bed. That led to his first ambulance ride and CAT scan. Zane has had a rough road with ITP but he still has a beautiful smile on his face...

        Zane was born May 10, 2007, in Lawrence, Kansas. Thinking he was a just a normal active 2 ½ year old, we thought nothing of his bruising.  In November of 2009 after noticing bruising on his legs he was taken to the doctor, and had a CBC done. 

            The next day I (Ronda) received a phone call from the doctor and was told Zane needed to be taken to the Cotton O'Neil Cancer Center to be seen by a specialist because his platelets were critical at 12,000.  A normal range is 120,000 to 400,000.  Dr. Jakica Tancabelic, M.D. diagnosed Zane with Idiopathic Thrombocytopenic Purpura (ITP).   The symptoms of ITP are similar to the symptoms of other diseases that can cause platelet counts to drop.  ITP symptoms can include, small red dots on the skin caused by broken blood vessels (petechiae), purple spots on the skin (purpura), large bruises, especially on the arms and legs, resulting from seemingly minor bumps (ecchymosis) and bleeding from the gums (gingival bleeding). If a person with ITP has very low platelet counts, it is possible to have internal bleeding or, in rare cases, bleeding in the brain. 



Zane's first stay in the hospital for
IVIG and steroid treatment.



         He was sent directly to Stormount Vail for intravenous immunoglobulin (IVIG) treatment.  That was the first of many 3 day stays at the hospital.  The first day consisted of the IVIG treatment and his first shot of IV steroids, very harsh and very hard on his little body.  The second and third day in the hospital was to watch for dehydration, and the 2^nd and 3^rd shot of IV steroids.  This picture was the harsh reality that he may never be able to be a “normal boy”.

            After several IVIG treatments, Zane's body quit responding to the IVIG.  Therefore we decided to use the predisone treatment.  After 9 months of predisone, Dr. T (as we call her) decided it was time to take more proactive step.  In November of 2010, Dr. T sent Zane to a specialist to consult for a splenectomy, which is a treatment as well for Chronic ITP. One of the spleen functions is to remove old red blood cells. In most patients with ITP, it is the main site of platelet destruction.  In chronic ITP, surgery to remove the spleen is an option if the platelet count remains too low or doesn't respond to treatment. Only about 10 per cent of children with acute ITP have the operation.  The surgeon said that Zane had a spleen and mini-spleens, therefore we were excited this would “cure” him!  Zane also has to  take a lifelong antibiotic to prevent infections.  Zane came through the surgery with flying colors, but after a week his platelets went back down.

            In May of 2011, Dr. T thought it might be a good idea to get his tonisles removed because of infection.  Well, the surgeon took out infected tonsiles and the night of his surgery we had to rush him to the ER because his throat was swelling shut.  He spent 3 days in the hospital for a simple tonsillectomy. 

            Since May of 2011 we have tried to keep Zane out of the hospital and continue bi-weekly blood draws.  If Zane gets a simple cold or infection his platelets drop, which caused him another hospital stay in February, which consisted of IVIG and steroids.  This instance Zane's gums started bleeding, which is not a good sign for ITP patients.  It is kind of like a few months ago when Zane bit his tongue and it bleed for 24 hours.  His platelets were 87,000 when he left the hospital and were back down to 20,000 one week later.  

          Dr. T has now determined that since Zane cannot stay out of the “critical range” that we should consider the treatment “Nplate” (http://www.nplate.com/).  Nplate® is a man-made protein medicine used to treat low blood platelet counts in adults with chronic immune thrombocytopenia (ITP), when certain other medicines, or surgery to remove your spleen, have not worked well enough.   We have to wait 30-days to see what the insurance company is going to say about the Nplate treatment, because of the cost.  We have been told that the Nplate treatment will run approximately $3,000 a week.  The Nplate treatment requires weekly CBC and the Nplate injection for an unknown amount of time, it all depends on Zane and his reaction to it.

Zane's biggest dream right now is to play baseball, he talks about it everyday, but the only way Dr. T will let him play is for his platelets to be above 50,000. 

Pure happiness.... Zane's platelets rose high enough to go for a ride
with his brother, Wyatt.

What a couragous kid! I'm looking forward to seeing Zane on TV playing professional baseball.

Greta :)

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.