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.

Stop the Rollercoaster - I Want to Get Off...

Jill Steinberg has had the ride of her life on the ITP rollercoaster for the past year. Here is part one of her story.....

Prologue- I had not been to a doctor in over 5 years…. I should have but I hadn’t gone…

I had just been to Wine Country in California the middle of July 2011.  Hubby and I took a second honeymoon and traveled from San Francisco to Silicon Valley and then toodled through wine country.  We had over two weeks in California so we took our time and went sightseeing.  We had bid on a wine country tour at a charity event and had won.  We visited six wineries that were on the tour and about 10 more.  I was having the time of my life.  Until, hubby decided to drive through the mountain range from Napa to Sonoma.  I had tremendous headaches but didn’t think much of it because it could have been due to allergies.  I have always suffered from pollen allergies. 

But during the latter half of the vacation, my legs started to get sores that would just bleed a little spontaneously and then would go away.  Ok I thought- something strange but it cleared up.  Must be I’m tired, did too much. I’ll need a vacation from this vacation.

We get home and I go back to work, however, I am really exhausted.  A couple times, I had to take a nap at work. I started to break out in black and blue marks and didn’t remember where they were from. Nothing unusual for me, I always have bruised easily and been a slow healer.  But these bruises were different.  They were a different color and no pain. I got a spot on my eye that I thought was a sty but it bled…

Then the spots broke out. I have red hair, fair skin, light eyes and lots of freckles. I felt like a red headed leopard. I started to sleep 20 hours a day, couldn’t eat, or concentrate but for a few minutes.  This went on for three days and finally hubby says that’s it, you are going to Urgent Care. That was August 8, 2011.

 We get there and they said, “She needs to go to the Emergency Room right way.  She needs testing that we can’t do here and it has to be now.  Either we call an ambulance or you can drive her and we suggest a hospital that has little to no waiting time….”

Thus begins my saga at South Miami Hospital ……..

I walk into the emergency department and hubby and my dad get their badge. From the way I look, I’m surprised, they didn’t ask about domestic violence….

We get to triage and they get the information quickly and march me into the emergency room.  They take my blood and go to do the blood tests.  That’s when they tell me, “You have 1000 platelets. If you had waited to come in any longer or had hit your head, you would not be alive…..”

Needless to say, I am the immediate emergency at that time…  Lucky me…

Round One ITP (1) – Jill (0)

They immediately ordered a platelet transfusion and an IV because I was dehydrated.  Before they started the IV, they wheeled me in for a chest X-ray and a CAT scan of my brain.  When I got back they started the IV. 

All this time, they still hadn’t told me anything except for the fact that my platelets are 1000K.  And that I needed to lie still….. 

They finally wheeled me up to a room about 11:00pm, after 6 hours in the emergency room.

They get me settled in the room and the nurse writes on the board her name, phone number and in big RED letters – catch the significance- BLEEDING RISK

To make life more interesting at the hospital, I have rolling veins so they had to get special technicians to take my blood and to start an IV.

The next morning, the on call doctor and the hematologist come in and start asking a million questions…. Have you been out of the country? No. Have you had a virus or been real sick lately? No. Do you drink tonic water? No. Do you have HIV or Hepatitis C?  No.  And on and on……

Round Two  ITP (2) Jill (0)

To be continued…...

ITP and My New Life

My name is Linda Eschen. I am a wife and mother of four: Chris 22, Sean 16, Tony 13, Jessalyn 11.  I work full time as the librarian at a K-8 charter school in California.  My two youngest, Tony 8th grade and Jessalyn 6th grade go to the same school. 

My ITP story goes quite similar to many others, I started having bruises and nose bleeds, but what actually got me in was when the knuckle on my ring finger turned black…. I thought it was going to fall off…..  My doctor, who I had not seen in years, looked at me and said….. “Have you ever heard of ITP?”  I asked him what it was and he said it was low platelets…..  Well, I HAVE heard that before…… this brings me back to 1998 when Tony was born…

Tony had to be induced, because my body was not happy and Tony was not growing inside as he should. My doctor induced my labor a month early….. Tony came with quite a large amount of bleeding… after staying overnight in labor and delivery being watched very closely, my bleeding finally got under control.  My gynecologist came in and told me that I have low platelets, this was something I was born with, but I looked too good to receive a transfusion and he felt the stress of pregnancy had caused this problem (ITP is common in pregnancy)…..   I forgot all about that as I enjoyed my third son. 

I proceeded to have another pregnancy, did not hear anything about platelets and went about my merry way enjoying my GIRL!!!

So, let’s get back to the doctor visit and ITP…… My doctor sends me to the lab, this being Friday, I asked when I would hear from him, he said “Monday”.  Saturday morning, 7:00a.m. I get a call from my doctor telling me that I have very low platelets (4 thousand) when the normal range is 150 thousand to 400 thousand….  He tells me that I am going to be admitted and that I needed to get there as soon as possible….   When I arrive, my doctor is waiting and starts checking me to see if I am bleeding into my brain.  He tells me that I am going to be receiving a transfusion of IVIG (Intravenous immunoglobulin) and platelets.  And I got my first introduction to steroids….  After receiving my first IV steroids my blood pressure started to rise…. It continued to go up….  During my two day stay, my nurses would freak at how high my blood pressure was, but the doctors knew that the steroids had caused it to go sky high.

Over the next week, I met with my new hematologist at the cancer clinic…. Talk about scary!!!  My numbers which got up to 100 thousand came down in spite of the 60 mg oral steroids.  My hematologist told me he will be doing a week long IVIG where I come to the clinic each day for 6-8 hour transfusions…..  When this was not successful, I came in for a bone marrow biopsy and my hematologist said we were going to wean me off the steroids which were not working.  I was going to be getting four treatments of Rituxan (a cancer medication). 

Rituxan…… my body did not like this medication.  My numbers continue to go between 14 thousand and 28 thousand…  I became very tired and had no appetite.  My hematologist told me that he felt I need to go with the splenectomy because my body was not reacting to stimulus.  By the time I went in for my third transfusion, I was down to 10 thousand and my hematologist said enough!!!

I had my spleen removed, April 3rd this year, my numbers went up and stayed in the normal range.  I am feeling very, very good.  But through all those months, my blood pressure continued to be VERY high.

June 15th, I went to see my General doctor because of my concern with my high blood pressure.  He prescribed blood pressure medication.  I took my very first dose and two hours later, I woke up with the worst chest pains imaginable.  I rushed to the emergency room --- yes….. I was having a HEART ATTACK!!!!

Within 20 minutes of arriving to the emergency room, I was on a procedure table receiving an angioplasty and stent.  What was found was a blood clot that completely blocked my main artery to my heart.  I ask,  “Is this connected to my ITP?”   Well, I am learning that yes, it was connected… 

So, now I am currently recovering from a crazy year of diagnosis of ITP, splenectomy and now heart attack at 51 years of age…. But, through this all, I am going to just grow healthier, smarter and stronger!!!

I Have What?

Hap Hapner has been a part of our ITP family for a while and has shared some of his experiences with us via the Facebook ITP support group pages.

Now, we get the rest of the story....

I Have What?

That was the question that kept running through my head over and over. The doctor standing at the door to the room in the E.R. was talking to my Lady saying something about removing my spleen. REMOVE WHAT? Wait a minute, what the heck is going on here. Things were moving way to fast. So where did this all start?

The Lady in my life had been suggesting that I should be getting all those 50+ year old test done while I had the time being unemployed. So off I go into the public health care system. It all started the way any new patient going to see his/her doctor for the first time. Fill out ALL the paperwork. Who had what, did you break that, bla bla bla. Off to lab to get blood drawn. I had an appointment set for 2 weeks to get all the follow-up stuff going. The next morning I receive a call from my doctor’s nurse asking for me to come in as soon as possible. Yes, ALL of the “BAD” thoughts came up. “Great Gods, am I going to be alive next week?” What would cause the doctor’s office to call first thing in the morning asking me to come in.

My doctor goes right past “Good Morning” straight to “You need to go to the E.R. and have a platelet infusion.” I do have to give Dr. Garcia proper respect, he said all of my blood work looked great except for 1 (one) very important part, platelets. He hands me a copy of the lab report and he is right. My blood looks fine, everything hitting almost mid point on any of the scales. All but platelets.

“OK Doc, what the heck causes that and is there a pill or something for it?” What Dr. Garcia said next floored me and still does today when I think about it. He said, “I have only read about it, but I believe you may have ITP.”

“I have what!” He gives me a rundown as he understands it. Well, what he understands and what I heard are two very different things. So off I go to the house (not the ER) to look ITP up. There is a lot of stuff out there if you Google ITP.

My platelet count was 3,000, way to low to be doing anything other then lying down. Using the computer, I found that almost everything I had listed on that “how do you feel” part of the doc’s form was listed with ITP; bruises, nose bleeds, fatigue. That explained a lot, but now what to do about it. Again enter my Lady. I’m told (by her) we are going to the ER and that was it. So armed with my new knowledge I got into the car. More paperwork and another blood draw and waiting. Then after hours of waiting, the ER doctor comes to the room and all I heard was “set up to remove his spleen.” Being half a sleep I freaked out! Wait a minute, remove what! I got up and left the hospital.

Being a mechanic this made no sense to me. If a part is broke, you look at it, try to fix it, and if all else fails, remove and replace. Well, no one could say “Your spleen is broken.” They could not say it’s only working at 50%. NOTHING! It took an Assistant Professor of Clinical Medicine to help me understand just what my body was doing to itself. I’ve been told at times that I can be a bit “self destructive,” but this takes it to another level.

I have discovered that depending on which doctor you’re talking to, which treatment they believe works. There is not one kind of treatment or cure for ITP because it affects each person differently.

So after being reassured by Dr. Harrington that they would try all they could not to remove the spleen, I headed back to the ER to get a steroid injection to get my count to a safer level than where it was at 3,000. So they get the count up to 27,000 after 2 injections and set up LOTS of doctor follow-up appointments & blood draws and send me home.

I’m still learning something new about ITP every day. My body does something different or I read it here on the internet. So far Prednisone seems to be the only thing to keep my numbers up, but the price the body pays for that are HUGE! I’ve done IVIG and did get a nice boost (short term) in numbers. Right now I’m going thru Rituxan treatment, one treatment a week for 4 weeks. Then it will be weekly blood draws for 4 weeks, then twice a month and see where thing level out.

It’s all “Wait & See!”

I'm not ill, just a bit faulty!

Since I'm reaching another turning point with my ITP, I thought I'd contribute my story so far to this blog. My name is Caitlin, I'm 21 and a student from sunny Wales, UK. My ITP story goes back to the spring of 2007, when I was 15. My first symptoms were petechiae on my arms and chest, but I put it down to getting too hot in rehearsals for a musical I was in. I also trained 4 nights a week in karate and competed at a national level, so bruises on my arms and legs were normal to me, I didn't even notice them!

            That little pinprick rash soon turned into lots of big angry looking red spots all down my arms and across my back that didn't disappear when my Mum pressed a glass to them- cue panic! I was at the Dr's office that very day being poked and questioned and having blood taken, but feeling absolutely fine. I carried on with my day, went to my Saturday job as a waitress, and was looking forward to a party for a friends 16th birthday.

            Needless to say, my night didn't go as planned. My Dad picked me up from work an hour into my shift, I'd had to cancel on my friend and go straight to hospital for a whole load of tests. By midnight, I had the diagnosis- "You have ITP. It means your platelet count is low, so you bleed easily." My Dad lost a sister to leukaemia when she was a toddler, so my diagnosis was something of a relief to him, as, unhelpfully, all the phonecall had said was "Your daughter's blood counts are very abnormal." However, I was terrified. I knew what a platelet was, but getting a major diagnosis of a disease I'd never heard of at 15 years old was horrible. 

            Being a nosy, scientific type, I threw myself at the internet and tried to research the best I could, but all that achieved was convincing me that I was going to die of a brain bleed! Then we found the ITP Support Association, a UK charity who send out factsheets and newsletters to patients and families. I remember they had a leaflet for kids and teenagers that helped me with a lot of basic information and reassurance that though the condition is rare and scary sounding, it was by no means a death sentence. I also got some cute fluffy toy platelets!

            For that first 6 months, as I was still a child, we adopted a watch and wait approach, and hoped it would sort itself out, which was fine for the most part. I've never been bothered by needles, and apart from a huge nosebleed on an exam paper (oops!) I didn't have too much trouble and passed all my exams with flying colours. However, my ITP soon became more of a problem. With counts below 10k I was always tired, and had huge bruises, and I missed school a few times because my nosebleeds were so bad. It was around then I started on high dose Prednisolone. I'd taken it before for asthma, but never had it been so much (70mg, for those in the know) Teenagers can be cruel, and as if the huge weight gain didn't get me enough grief, my bruised arms and strange behaviour (the side effects manifested as mood swings, depression and sleep disruption) attracted the kind of whispers, pointing, glances and questions no one ever wants, never mind a 16 year old girl. No one wants to be asked "Is everything ok at home?" when you know full well you have the most wonderful family in the world who would never ever hurt you, but even when I'd tried to explain the condition people didn't believe me, and they talked. I found out who my friends were pretty quickly. That was some of the toughest stuff to deal with for me, even to this day.

            It became clear that I couldn't stay on the Prednisolone forever, even though it did help my counts. I had Rituximab treatment in January 2008. My counts began to climb after only a couple of weeks, and I was declared in remission in June 2008. I was still under the watchful eye of my haematologist, back in and out for check ups for most of the rest of my school life, but with counts of 200k+ all the time, it was hardly a chore. I finished high school in May 2009, got good grades and spent the summer saving money and hanging out with my friends before heading to university in September, just as any 18 year old should! But was this the end of my ITP story? On the advice of Greta (I'm a terrible editor and have a long story!) *tune in next time* to see what happened next!