How My Body Tried to Kill Me

This article is from the Feeling Fit supplement in the August 9 edition of the Charlotte Sun newspaper published in Port Charlotte, Florida.

The article was found by Tom Schilling's sister who lives in that community. She passed it on to him and he passed it to me. Bob Massey graciously gave his permission to share his story with all of you.

ITP is everywhere and it is great to see information being passed along to raise awareness....

A blood disorder started as a nosebleed – and almost ended in death

By BOB MASSEY

Feeling Fit Correspondent

       Three years ago, for reasons I (or the doctors) could not fathom, my body tried to kill itself. Not intentionally, mind you – but I came very close to being dead all the same.

       Here’s how it could have went down: Sunday morning. My then-10-year-old boy wakes up to spend another father-son day with dad. What he finds is me, unconscious or – more likely – dead, lying in a pool of blood.

       And it started with a nosebleed.

Seeing red

       I didn’t think much of it when I blew my nose into a tissue and noticed some blood. After all, it wasn’t running or dripping. But as the morning progressed, it was getting thicker, which turned out to be from blood drying on this inside of my nostrils.

       At lunch time, I happened to glance at the back of my hand, only to see a red striated rash, under the skin. My son, Patrick (who even then wanted to be a neurologist and often corrects me on medical points) was worried. He told me a clot could be causing my symptoms, and was no longer enjoying our day together. He wanted me to go to a hospital. For goodness’ sake, I reasoned, it’s a nosebleed and a rash. How bad could it be?

       I was about to find out.

When blood doesn’t count

       It took a fair amount of convincing to get me to the emergency room at Charlotte Regional Medical Center in Punta Gorda. It’s a good thing I went when I did. The staff got hold of infectious disease specialist Dr. Mark Asperilla – who raced to the ER when he heard the results of my blood work.

       The platelets in human blood are what allow it to clot. A normal platelet count is 150,000 to 400,000. Mine was 3,000. What I had mistaken for a rash was actually a series of tiny bruises called petechiae, caused by blood leaking from my capillaries.

       I was slowly bleeding to death.

       Asperilla later confessed that when he realized what my platelet count was that Saturday night, he was doubtful I would live. He said few people in that condition actually make it.

       I was supposed to be given transfusions of platelets – five units all together – but the hospital didn’t have any.  Some were sent from Orlando, which only had three. It took another day to receive the final two.

       The question Asperilla needed to answer was: Was my bone marrow failing to produce platelets, or were they being produced but destroyed before they could function?

       But he had already suspected it was the latter. He had actually diagnosed the illness immediately, but waited for test results to make it official.

       It’s called ITP. (More completely, it’s idiopathic thrombocytopenic purpura – which is why I went right for the acronym.) The name means an abnormally low platelet count (thrombocytopenia) marked by bruising (purpura) due to an unknown cause (idiopathy).

       There is little information about ITP because it’s still such a mystery – although there is a push for more research in the United States and in the United Kingdom, since the disease can affect children. Fortunately, it was mine alone, and can’t be passed from one person to another.

The treatment roller coaster

       Asperilla called in a hematologist and oncologist, Dr. Antonio Gabarda, who prescribed a bone marrow aspiration, in which a 2mm cylinder of bone is extracted and examined. It is rumored to be one of the most painful procedures you can have, and I won’t argue.

       As it turned out, my spleen was destroying my platelets – treating them as foreign invaders and sending antibodies with express instructions to annihilate them.

       True to its idiopathic nature, the cause – how I got ITP in the first place – is something the physicians were never able to figure out. The usual suspects were being an alcoholic or an illegal drug user, or suffering some kind of body trauma (such as would occur in a car wreck). I failed in all of those. That left only two other possibilities: a virus – or cancer.

       Now 28,000 platelets strong, Gabarda put me on a regimen of steroids, which were supposed to bond with the platelets to protect them. Except the next day’s count had gone down again to 24,000 platelets.

       Not only that, the meds shot my blood sugar sky high, making me semi-diabetic. I was getting my finger pricked for a blood glucose test every hour for two days, then before every meal. Another doctor put me on medication for blood pressure – which had been on the high end of normal before going into the hospital – in order to keep me from losing my vision.

       I was beginning to feel that whatever cosmic warranty I had on my body was beginning to run out.

       There was good news, however: Tests for cancer came back negative. The best Asperilla could figure is the ITP was triggered by a virus. That was his best guess, anyway.

A costly cure

       Short of having my spleen removed (which was the next step), Gabarda prescribed a medication called WinRho. I had a dose and a half over the course of two days. For about 10 minutes after the first dose, I had a reaction – violent chills and teeth-jarring shaking as well as radiating aches. But that was nothing compared to my reaction to the price of the medication: $8,000 a dose.

       But my platelet count went up to 26,000 – so the WinRho did the trick.

       I had been admitted on Saturday and was discharged on Thursday. Things could only get better. Or so I thought.

       My first post-hospital blood test was glorious – a platelet count of 260,000! The original plan was to wean me off the steroids in two weeks, but Asperilla decided to keep me on it for another two months. It puffed up my cheeks and caused to live as if I were diabetic: blood glucose tests, insulin shots if necessary, 2,000-calorie diet, the works. I also had to watch out for illness, since the steroids were suppressing my immune system, making me vulnerable to a host of problems. If I developed an infection, for instance, my body would probably not have responded in a predictable manner. It might have bypassed warning signs such as a fever, and I wouldn’t know I was even sick until I was really sick.

       As it turned out, Asperilla’s decision to keep me on steroids was a wise one. A blood test taken two weeks after my discharge showed a platelet count of 134,000 – slightly below normal, though not dangerous. Unless it became normal, the spleen removal strategy was still on the table.

       Eventually, it did – my blood has remained normal, and the symptoms never returned. And I’m grateful to Asperilla and Gabarda for saving my life, not to mention the wonderful care I received at the hands of the other medical professionals who attended to me.

       Perhaps one day we’ll understand more about it. As a near-victim myself, I’d like to know.

Rachel Pagano Chahir is a very familiar name to many folks. She has been a voice on many of the ITP Support Groups and has helped us to see this disease through her eyes.

Rachel is also very busy spreading the word about ITP and made "GOT PLATELET HATS" to share with us. Through this endeavor, she also set us a contribution page through PDSA to raise funds during ITP Awareness Month. If you would like to contribute to this worthy cause, check out her donor page at http://pdsa.donorpages.com/HelpRachelChahirFightITP2012/

Another project under way is the 

St. Gennaro Festival BLOOD DRIVE & ITP AWARENESS

Rachel and Linda Guy McGuirl are going to have a table set up at the festival with information on ITP. 
It will be on Sunday, September 23 at St. Patrick's Basilica Youth Center, 268 Mulberry Street (between Houston and Prince) New York City.

The American Red Cross will be there for folks to donate blood. Blood is in short supply. Call 1-800-Red-Cross to make an appointment or to get information sent to you. All donors will receive free cannoli! 

Join in with the fun, food and festivities at the San Gennaro Feast, learn a little something and donate some blood while you're there too.
https://www.facebook.com/events/348196801939778/

A MESSAGE FROM RACHEL:

My name is Rachel Pagano-Chahir, I was born in Long Island to an Italian-American family and then by two years old we came back to Brooklyn where our relatives lived and we could have a real sense of neighborhood. From my early childhood I always knew I wanted to help, so I decided I was either going to become a Nun or go into medicine. I chose the later and became an RN in 1981. This was a dream come true cause I had a job that I really loved and gave me great pleasure. I found great comfort in helping others get well, understanding their conditions and being able to to heal. As I kept a very busy pace working 3 days a week 12 hour shifts, caring for a disabled husband, a mother who had cirrhosis of the liver and my father who had early dementia and raising my son, while volunteering for McDonald house foster home program & Covenant house, it wasn't unusual that at times I felt very tired and fatigued.

What surprised me the most was when in December of 2002 I was hanging my decorations for the Christmas season, when I slipped off a ladder and slid down, I didn't think much of it. Getting dressed the next morning my entire leg was black not on one spot you could see my skin, thinking I must have had bad fall then, I continued on my day. As I brushed my teeth, blood started to squirt out from the inside corner where your jaw meets, again I said what? and in a hurry I dealt it off to a period problem..Later that day at work I felt completely cold especially my hands and feet. It went on for days like this and the bruise on my leg wasn't clearing. I went my GP who was a co-worker and friend and she said, of course you are runned down, look at the schedule you keep, the fall you took, you really need to eat better and make sure you take your vitamins. Well we left it at that.

After months of me becoming a walking "poster woman for domestic abuse" because of all the bruising I finally said I think its time for a second opinion and looked to get in with a hematologist. Much to my surprise his first reaction was I think you have "ITP" but I would like to run more test and do a Bone Marrow to rule out the "L' conditions. My world and the life that I had lived changed from that day forward...I didn't get diagnosed till 2006 andstarted treatments IVIG, steroids. I had an TIA and partial loss of vision in my left eye, I was depressed, weak and fatigued. I spent most of my days laying down or sitting and could only do things for a short time. I had brain fog. 

How can this happen to me I said? I'm the one that takes care of everyone, now I'm on the other side and this wasn't my life anymore, it was an ITP life. Trying to gain some control I opted to have a spleenectomy 8/15/12 at age 52. Well after 3 months, it didn't work I'm back to being refractory. I will not give up, but if ITP is what I have to live with I am going to direct my anger, my sadness, my caring nature to spreading awareness to those that might have and don't know and to the world and doctors that need to learn and push for more research.

Thus for September ITP awareness month, my son who does graphic design and studying Architecture helped me put together ITP Awareness caps "GOT PLATELETS?" So we can spread the word and bring the nation of rare blood disorders. They are my way of giving back to PDSA & fellow ITP'ers and support groups for their unending hope, knowledge and support.

September is ITP Awareness Month

September is ITP Awareness Month. Many of the members of the ITP and Me support group are truly in the spirit of showing off our purple for this month.

What can you do to help spread awareness? 

These suggestions are from PDSA website. (Platelet Disorder Support Association.) http://www.pdsa.org

• Write a letter to your congress person. A template is provided here.

• Contact your local media to be interviewed about ITP. A press release is provided here.

• Distribute PDSA Brochures to doctors' offices, pharmacies, hospitals, libraries.

• Attend a local support group meeting – bring a family member or friend. 
  See a list of support groups and meeting dates by clicking here.

• Participate in our 3rd National Walk/Run: Pump it up for Platelets ~ for a world free of ITP.
  See a list of participating sites by clicking here.

• Organize a walk or other fundraising event. For ideas, check out our “50 Ways to Fundraise”.

• Contact local civic clubs and offer to speak about your personal experience with ITP.

• Coordinate a display at a health fair, store, or hospital.

• Ask your city government to publicly acknowledge ITP Awareness MonthSM.

• Share information about ITP Awareness MonthSM with your contacts on Facebook, Twitter and other online sites.

• Download the ITP Awareness Ribbon and share on your pages or make your profile picture.

• Join PDSA or give a gift membership.  More information can be found by clicking here.

• Make a donation.

PDSA has done so much for ITP awareness, research and recognition. They worked very hard to get ITP Awareness Month recognized.

Their website has invaluable information for ITPers, caregivers and anyone who wants to know what ITP is. 

Thank you PDSA for all you have done and continue to do for us!!

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.

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