Ignorant, Stupid, Chronic

Through my three episodes of Thrombotic Thrombocytopenic Purpura (TTP), I think I have passed through three phases:  ignorant, stupid and chronic. I guess what I'm really saying is I have learned quite a bit through the last 6 years and now hopefully know my status.

Ignorant

In January 2012, I started to experience many symptoms, but thought I just had a cold. I had dark urine, sniffles with blood in one nostril, headache, shortness of breath for sports, and a large bruise on one leg. After about a week and a half, I talked to my doctor about a different issue. I could not find words or finish my sentences. The doctor talked to my wife and recommended I be taken to the hospital. Luckily, the hematologist on staff determined TTP immediately, so I was sent off to another hospital that has the plasmapheresis machine.

I call this the ignorant phase as we knew nothing about TTP. Never heard of it. Why would we as it only impacts 3 per 1 million people per year. But we also did not respond fast enough when the number of symptoms started to accumulate. We were waiting for the "cold" to go away.

Lesson learned, pay close attention when you are not normal and have behaviour changes.

Stupid

Just over two years later, I started having symptoms again. It started with dark urine, then a headache, bruising and shortness of breath in the gym. It took me about five days to go for a blood test. Stupid.

I'm not sure why a TTP survivor would not be off to the hospital immediately. In fact, I decided to spend the long weekend moving my sons into their apartment, then setting up our recreation property for the summer.

I suppose the thought was the first episode was a freak of nature. Remember, TTP only impacts 3 per million. Unfortunately, I didn't remember TTP survivors have a 30 percent chance of having TTP again.

Luckily, we did go to the hospital much earlier than the first time. The sequential issues were fewer and I was able to go back to work within 6 weeks. TTP was treated with 10 plasmapheresis treatments. Rituximab was administered after plasmapheresis had been completed, so it wasn’t removed from my system. I was cured! Stupid.

I didn't even not know what the words for TTP. Couldn't say it, couldn't spell it.

Chronic

Now, just over three years later, I have TTP for the third time. Within just over half a day my wife and I determined that I wanting a nap, not having supper, having lower back pain through the night, diarrhea, throwing up and bruising, meant TTP was back. We were off to the hospital for a blood test within 30 minutes and were and in the right hospital for treatment within 4 hours.

I have accepted my TTP condition is chronic. I have to live with the consequence; I will have to battle TTP every few years. I'm doing what I can to get prepared.

I also now know how to spell and say Thrombotic Thrombocytopenic Purpura.

Bruce Fights TTP

I have decided to start a lifelong, that's a big word, campaign called Bruce Fights TTP. The goal is to provide information about TTP, encourage blood donation and raise charitable funds to help find answers as to why TTP occurs and how to prevent repeat occurrences.

Thanks, Bruce.

@BruceFightsTTP

Chemo! I Thought You had TTP?

I have had Thrombotic Thrombocytopenic Purpura (TTP) three times over the last 6 years. I have been treated with prednisone, plasmapheresis, red blood, Vincristine, Rituximab and Cyclophosphamide.

A couple of weeks ago, I was told we are getting ready for my chemo.

"Chemo? No, I'm getting Cyclophosphamide."

“D'oh, same thing“

Chemotherapy is the treatment of disease by the use of chemical substances, especially the treatment of cancer by cytotoxic and other drugs. Chemotherapy is also used for TTP.

Chemotherapy drugs are used to combat the autoimmune dysfunction. In order to stop TTP, we need to stop the platelets from being destroyed. I don’t know the percentage, but many TTP patients get chemotherapy.

I suppose the good news is TTP patients can combat the disease using therapies which were developed for cancer. The TTP patients may also be assisted by associated support groups.

The downside is there is little research to help find a specific solution to TTP. Using chemotherapy is like using a bazooka to combat TTP. The drug may destroy cells which have no relationship to the TTP autoimmune deficiency. My current chemotherapy actually reduces my platelet growth. This is very depressing to a TTP patient, where one measures progress as an increase in platelet growth.

The search for a specific TTP solution is why we think it is important to help a charitable organization which supports TTP research. Answering TTP Foundation has committed over $1.6 Million to research grants through 2019.

Bruce Fights TTP team will support Walk to Answer TTP Together on September 16, 2017. Please support.

Thanks, Bruce.

@BruceFightsTTP

For TTP, What Should I Watch For?

I have had Thrombotic Thrombocytopenic Purpura (TTP) three times. Each time the symptoms built up slowly. Normal symptoms you would get with cold, such as a headache, dark urine, stomachache. Symptoms from a bad day such as fatigue or a backache. Everyday symptoms such as a bloody nose or a bruise. 

The issue is you may get many symptoms over a few days. The involvement of many symptoms, may be a buildup of a bigger issue such as TTP. The reason is the blood is bad, the platelets are gone and organs start to have consequences. You need to watch for what is REALLY not normal. 

For my latest trip down TTP lane, I started on vacation with my cousin. We were boating, tubing, swimming, maybe even drank a beer or two. At the end of the day I was tired, sunstroke? Thought of having a nap. Skipped dinner, had back lower pain all night. In the morning, had diarrhea four times and threw up toast from breakfast. All of these items were not normal. My wife arrived later in the morning and saw bruising on my elbows. I lifted my shirt and saw bruising on my stomach and we immediately knew TTP was back. 

We jumped in the car and headed for the hospital. I quickly texted my cousin to advise about TTP. In our following conversations he asked "were there any symptoms we should have seen?" 

Here is my list of symptoms we are going to watch for: 

• Fatigue 
• Eye floaters 
• Dark Urine 
• Diarrhea 
• Bruises 
• Backache (indicating possible kidney problems) 
• Headache 
• Bloody Nose 
• Petechiae (pinpoint-sized red or purple dots on the skin) 
• Paleness or jaundice (a yellowish color of the skin or whites of the eyes) 
• Shortness of breath 
• Speech changes or loss of words 

Thanks, Bruce.

@BruceFightsTTP 

Don't Go to the Eye Clinic for TTP!

I thought I was an all-star! In my third edition of Thrombotic Thrombocytopenic Purpura (TTP), I had 9 plasmapheresis appointments in 9 days. My platelets went from 10 to 205 and I was checked out of the hospital. We went into the weekend, checked my platelets and they were up to 212 -  all-star!

On Saturday, we were off to celebrate the 150^th year of Canada and went to see the MosaiCanada with the spectacular plant sculptures. We even saw the plant version of Paul Henderson and Yvan Cournoyer from the Summit Series of 1972 - all-stars! Sunday, we were off to our vacation property, boating, skiing and swimming, even stopped at a craft brewery for dinner. We had a great weekend.

Monday, I am still waiting for the next part of the plan from the hematologists. I have the day off to keep recovering, but I then start to lose eye focus. Should I sleep if off or go back to the hospital? I decided to go back to the hospital. I called my wife to take me and called the Apheresis Clinic to let them know I was coming back. The nurse advised to go to Emergency to get the process started.

Arriving at Emergency, we advised I am a TTP patient, have been checked-out last Thursday and I am now having problems focusing with my eyes. We are advised, since I have an eye issue, I will be examined right away. The doctor understands I have TTP, is very happy my platelets were 212 on Saturday. They do an eye exam and set an appointment with the hospital eye clinic. They take no blood.

After waiting a couple of hours or so at the eye clinic, the head Apheresis nurse calls me.

"Where are you?"

"Eye clinic"

"Emergency has checked you out and I have no blood work. I'm on it!"

Within five minutes the head nurse was at the eye clinic and took my blood in an eye examining room - all-star, hero! My platelets had dropped to 42. It was TTP which was affecting my eyes.

Lesson learned, you need to take care of yourself. Emergency thought there was no issue with TTP as my platelets were 212 just two days before. No one in Emergency had any experience with TTP and how it affects others areas of the body. We have experience. We knew TTP was the issue. We should have insisted on an immediate blood test.

Patient supporters of any disease need to bring their experience to the table. The patient might already be impacted and may not be able to support themselves.

Thankfully, we had called ahead to the Apheresis Clinic and our all-star, hero, head nurse got me back on track.

P.S. Don’t go to the eye clinic for TTP.

Thanks, Bruce.

@BruceFightsTTP

Blood, Plasma or What?

I often get the question, why are you asking for blood donations when you are receiving plasma?

Plasma is the liquid portion of blood. A protein-salt solution in which red and white blood cells and platelets are suspended. Plasma constitutes 55 percent of blood volume and is 92 percent water. 

The blood can be separated into red blood cells, white blood cells, platelets and plasma. This allows donations of specific blood parts. It also allows replacement or treatment to those parts. 

With Thrombotic Thrombocytopenic Purpura (TTP), the platelets are destroyed due to antibodies in the plasma. The body's bone marrow is still capable of generating platelets. The issue is the new platelets will also get destroyed. Eventually, the body will not have enough platelets, bringing on symptoms and consequences. 

Part of the TTP solution is to replace the bad plasma with good plasma using plasmapheresis. The process is done by removing the blood, separating out old plasma, adding in new plasma, and returning the blood. The new plasma will allow platelets to increase and better blood markers should result the next day.

Plasma can be provided in the following versions: 

• Fresh frozen plasma (FFP): FFP comes directly from a few blood donors and can be used for plasmapheresis 
• Cryosupernatant: a version of plasma with reduced levels of factor VIII, von Willebrand factor, factor XIII, fibronectin and fibrinogen. Cryosupernatant plasma can be used when replacement of FVIII is not required, and is indicated for plasmapheresis for patients with TTP or HUS. 
• Solvent detergent plasma (S/DP): a version of plasma pooled from thousands of donors and the processed with solvents and detergents to remove viruses and reduces the risk of allergic reactions. I call this the Tide plasma. In many cases TTP patients may have allergic reactions to FFP or Cryosupernatant; S/DP may resolve this risk.

In my case, I am special, special! I have chronic TTP which impacts 3 in one million people a year. I am also anaphylaxis to plasma transfusion. In other words, I am allergic to FFP and Cryosupernatant, so I need S/DP.  

My apheresis nurse just reminded me, in 2012, she was the first nurse in Canada to provide S/DP and I was the first patient. S/DP is now available for all patients who require it. 

Thanks, Bruce.   

@BruceFightsTTP