Category Archives: Symptoms

Don’t give up. Don’t ever give up.

dont give up

My friend Dale shared this video from the Jimmy V awards last week, thinking of me. ESPN anchor Stuart Scott got the Jimmy V Award for his battle with cancer. He was in the hospital just the week before he got the award with liver and kidney failure, as well as surgery….the same as me back in March.

Please take 15 minutes to watch this.

This video epitomizes me. I don’t want to leave my kids without a dad. I love what I do and I want to keep working hard. It’s what keeps me going. “That’s how I win.”

I don’t know how he got through that speech without crying, because I didn’t. Stuart Scott is my new idol.

Funny how little things like this hit you in a very timely fashion. I needed that. Thanks, Dale.

Even though I don’t have cancer, aplastic anemia is up there with rare and deadly diseases. Stuart Scott had a fantastic quote about cancer that applies to anyone with a life-threatening disease:

“When you die, that does not mean that you lose to cancer. You beat cancer by how you live, why you live and the manner in which you live. So Live. Fight like hell.” Stuart Scott.

As I feared, my numbers plummeted yesterday. The doctors at NIH stopped my cyclosporine chemo last week since it was doing a number on my kidneys and causing ankle swelling. While my swelling is gone, now my numbers have dropped to levels I had 2 months ago. My ANC dropped nearly half, to 700, as well as my platelets that had been rising on their own.

All the progress…
All the hope…
All the hope…
All the optimism…
All the prayers…

This is not good. It probably means that my marrow really hasn’t started making the cells on its own, and it probably won’t. The chemo was just suppressing the t-cells that were attacking my marrow. Without the cyclosporine, something continues to stop my marrow from producing cells. The cyclosporine was not meant to be a long-term solution, and I was due to stop it next month.

We don’t really know what’s next, and won’t know until my visit back to NIH on August 12. I don’t think there’s much else they can do without a full match bone marrow donor. All we can do is pray for a match and pray that I avoid infections.

roller-coaster-life

I’m on an emotional roller coaster right now and trying to remain strong. It felt as though they had given up on me. Anxiety, fear, and depression were lurking around the corner.

But I’ll tell you this…I’m not quitting.

I’m just coming from the bottom of the coaster and making my way back up.

slow-motion-rollercoaster

It’s How you live, why you live, and the manner in which you live.

I have a huge base of support from family and friends; even people I don’t know are praying for me.

I wanted to share something from someone else that inspired me. I’m so happy to hear almost every day that my story and my blog have been so inspirational for others.  That’s what I set out to do after my diagnosis…so I can’t quit.


 “Don’t give up. Don’t ever give up.” Jimmy Valvano

Aplastic Anemia Q&A

While I have a few extra days here at the hospital, I thought I’d tell you exactly what’s going on with my disease and treatment. I still have many people who aren’t totally clear (as well as myself), but I’ve spent a good bit of time reviewing the literature like a good little nerd. So here goes.

What is Aplastic Anemia?

Your bone marrow produces all the blood cells in your body. This is happening constantly. All these cells start out as “stem” cells which then evolve into specific types of cells. The 3 major types are Red Blood Cells, White Blood Cells, and Platelets.

With Aplastic Anemia, the bone marrow stops producing these cells, and is replaced by fat in the bone. Because the marrow stops working, they call the marrow ‘plastic’.  They refer to this as “bone marrow failure.”

aplastic anemia cells

Aplastic Anemia is characterized by a significant reduction in those 3 types of blood cells: reds, whites, and platelets. This condition is known as “pancytopenia”. That’s what the doctors saw first on my blood work.

Aplastic Anemia is characterized by its severity. I have “Severe” aplastic anemia because my counts are so low. I believe when I came here I was actually “very severe.” The biggest risks from aplastic anemia are infection and bleeding. People can have “mild” or “moderate” anemia and continue onward, but no one survives without treatment in severe aplastic anemia.

While anyone can get Aplastic Anemia at any age, it’s more common in teenage males.

What’s with the blood counts?

Blood counts are critical to measuring my response and progress.  Steady (yet slow) increases in these numbers are good signs of response.

snoop as count

The red blood cells carry oxygen in the blood. The oxygen is attached to something called ‘hemoglobin’. When the hemoglobin is low, I get fatigued and out of breath. The low normal red blood count is about 4.5.  Today, mine is 2.7. Not too bad. My hemoglobin is 8.2, and the low normal is about 14. Again, not bad, but they tend to transfuse red cells when hemoglobin hits 8. I’ve been exercising regularly and hope that it’s been keeping my hemoglobin up. These cells live the longest in the blood, up to 120 days.

The white blood cells help fight infection.  There are several kinds of white blood cells. The most important cells for me are the “neutrophils” since they fight bacterial infection. Normally, they should be around 1700. But 500 is a good number to be at to reduce the infection risk. My biggest problem is that I’m only at 40. That makes me “neutropenic” and leaves me very susceptible to infection.  There’s nothing to do to increase the neutrophils unfortunately. We just have to wait. These cells unfortunately live in the blood only about 24 hours.

The platelets help stop bleeding.  They are normally about 150. When they are low (<10), I start to notice things like petichia (little red dots on my skin) or more easy bruising, or even sores in the mouth.  When that happens, I need to get platelet transfusions. Right now, I’m running at 32. These cells stay in the blood around 6 days.

petichia
My petichia before coming for treatment was pretty bad.

What causes Aplastic Anemia?

Most of the time, the cause is unknown. In fact, they really only find a cause in about 25% of cases. These causes can be genetic, environmental, viral, or even nutritional! But all those causes have been ruled out in my case. Therefore, I am among the 75% of ‘unknown etiology.’ This makes it much more difficult to fix.

They believe in most of these cases that it’s an ‘autoimmune’ disease. In this case, my own immune system attacks my bone marrow for some reason, thinking it’s a foreign body. During this attack, my specialized white blood cells in my bone marrow, called, “T-cells” are thought to destroy the bone marrow.  It’s similar to what you may have heard about rheumatoid arthritis or lupus, except that my immune system attacked my bone marrow rather than my joints or skin.

In my case, it happened relatively quickly. I had normal counts a year previous, and really didn’t have symptoms until about November, but wasn’t diagnosed until January.

What’s the treatment for Aplastic Anemia?

The treatment for severe Aplastic Anemia really depends on several factors, including age. Younger patients usually go straight to a bone marrow transplant. Older patients like myself start with “immunosupression therapy.”  Even if I needed a bone marrow transplant, the immunosupression therapy is considered a ‘conditioning regimen’ for the transplant.

Immunosupression therapy starts by killing off my immune system that’s attacking my bone marrow. As you many of you have already read, they start with an infusion of horse-serum (ATG) for 4 days in the hospital. That kills all the white cells. Then, they provide a drug called “cyclosporine” which suppresses the action of the new T-cells I’m forming. The cyclosporine lasts at least 6 months. Cyclosporine is commonly used to prevent rejection of organ transplants.

Unfortunately, high blood pressure and subsequent seizures are side effects of the cyclosporine. They are having trouble managing my blood pressure, so I have to wait a little while on the cyclosporine. That’s what’s keeping me here.

As you know, I’m participating in a clinical trial here at the NIH. Dr. Neal Young, my physician here at NIH, actually ‘invented’ and researched the ATG-Cyclosporine protocol. As part of my clinical trial, they are using a drug called eltrombopag for 3 months in addition to the cyclosporine. I started that a few days ago.

Eltrombopag is approved by the FDA to increase platelet levels. Dr. Young and his colleague Dr. Townsley were using it for treatment after unsuccessful initial courses of ATG-cyclosporine. They began to see 40% of the patients increase their blood cell levels. So, they wanted to do a clinical trial on its effectiveness as a ‘first-line’ treatment.

Other medications are an important part of treatment to prevent infections and ‘serum-sickness’.  The most important part of my treatment moving forward is to take the medications correctly (a major task within itself!) and to prevent and monitor infections.

pills

What’s the prognosis?

Untreated, severe aplastic anemia is fatal. But, there have been overall survival rates reported as high as 95% after 3 years!

Younger patients do better than older patients. There’s generally a 60-70% response rate with my treatment. Because I’m over 40, my chances of success are a little less than a younger person.  I basically have a 50/50 shot that my current treatment will be successful.  That number might be increased with the addition of the clinical trial drug they are giving me now.

Even if I do respond to this treatment, it may not be fixed. Based on the literature, 1/3 of patients ‘relapse’, and 10-15% evolve into a more serious condition, including leukemia.

If I don’t respond to this treatment by 3 months, the options are to provide another round of immunosupression therapy similar to this one, or if I have a perfect sibling match for bone marrow (which would be only a 30% chance), then I would be a candidate for a bone marrow transplant.

There’s not much else to do but be careful, calm, and pray.

keep-calm-and-fight-your-disease

 

 

 

 

Beating a dead horse

horse head-sittingWell, I hope you didn’t get too grossed out by my recent reactions to Horse Serum.

The doctors and nurses checked me out and said it was a temporary side-effect, and should be gone in a short time.

horse-phillipa
My nurse Phillipa (yes, that’s her real name!) checks on me

But before we let that horse go, there are a few questions I have to answer for some of you.

Question. I’ve heard that Horse Serum may also cause other parts of your body to grow larger. Is this true?

Answer: True. But of course, these effects are very temporary just for Valentines Day:

horse-in-bed1
Larger teeth are noted enlargements with Horse Serum

 

Question: So now that you’ve gotten Horse Serum, what type of Horse do you feel like? A “Stallion”?

Answer: While it would be nice to say “stallion,” I’d say it’s more “clydesdale,” but in reality, it’s probably:

dead-horse
Glue Horse

 

So now we await the (true) possibility of “serum sickness” that can occur 7-10 days after starting treatment: muscle & joint aches, rash, fever. Just what I’m looking forward to. Luckily, my name Phillip actually translates to “Lover of Horses.”

But hopefully by this weekend, I can officially say that we beat the horse:

In the mean time, the next step of treatment is the chemotherapy. I’m starting cyclosporine, as well as a cocktail of other meds to control things like blood pressure, etc. This is what my morning pills look like:

pillsUnfortunately, I’ve just been put on isolation because I’ve developed a cold. This means limited visitation and anyone coming into the room has to wear a gown, gloves, and a mask to prevent cross-contaimination with other patients.

With a very low white blood cell count (low immunity), I’m more prone to infection. So the cold virus promptly found it’s way to my system.

The good news is that my white count has actually doubled (from 70 to 150) in one week. I’m still in the danger zone until the 250 level, so keep the #MOJO flowing that I continue to trend up…

austin-powers2

White counts are probably the most important for me because most aplastic anemia problems result more from infection.

On a good note, Angela is very excited because they are predicting 6 inches of snow here tomorrow.