2 Reds Straight Up, with a Platelet Chaser

Now that I have my new Port in place and can no longer partake of my favorite wine and beer, the wine analogies have taken over my impoverished brain.  If someone tells you, "Don't even THINK about apple pie!" then all you'll see are apples, pies, pie a la mode... I'm sure there is a psychological term for that, but I digress.

Monday's blood work showed Low's & High's:
Hemoglobin: 7.9  (low)
Hematocrit: 24.9  (low)
Platelets: 62  (low)
Whites:  3.1  (low)
Neutrophils:  33 (low)
MCHC: 31.6 (low)

High are Bands, Metamyelocytes, Myelocytes, Anisocytosis, Poikilocytosis, Polychromasia, and Schistocytes.  (I'm still looking up all these from the Manual Differential test).

Next thing I know, I'm here at Gwinnett Medical Center's Out-patient Transfusion Center.

Good to the last drop!

2 units of platelets

My appointment is for 7:00 am EST. 
Yes, you read that right! 7:00 AM
Hells bells, I wasn't a morning person on my best, healthiest days!
Kudos to my wonderful daughter who brought coffee to my bedroom this morning ~ at least it got one eye open.

I'm getting two units of Red Blood Cells and 2 units of Platelets. This is the first time I'll receive platelets and third time receiving RBCs. 

I must give a shout-out of THANKS to the blood donors from Gainesville, Florida who made their precious B negative blood available to me. Thanks also go out to the platelet donors who are saving me today. 

 Thank you! Thank you! Thank you!

Close up look at the port in use.

My last blog entry shows what the port looks like (please excuse the leftover betadyne stain on the skin. Here are pics of the port in use. With great advice from another patient, I applied a dollop of lidocaine 5% on the port and covered it with a clear sticky sheet the nurse gave me the day before when I went in for the blood match and cross. I put the lidocaine cream on the port site about 20 minutes before my scheduled appointment. 
The nurse, Amy, was fastidious in her preparations and the IV hook-up. We both wore masks during the process. I barely felt the needle go in through the skin! Yay me!

Turns out she worked in the transplant unit at Northside Hospital (where I will be). I was quick to recognize her knowledge of blood stem cell transplantation and peppered her with questions throughout our day together. Her candor was professional and refreshingly frank. 

When I asked about how to best prepare for the big fight (other than eating well, avoiding illness, and exercise), she recommended that we prepare for the enormous physical and emotional toll it will take on me. It can be frightening to someone who doesn't understand that it is part of the process. Others have told me that the intense chemo takes you to death's door, leaving you to crawl back to the land of the living.    

Well folks, I'm not sure we'll be sharing photos of that grandeur (but you never know).

While the platelets were dripping in, the hospital chaplain stopped by. Now, I don't know why, but when an official person of God shows up in my hospital room my first reaction is, "Why am I the last to know? How long do I have? Holy crap! What sins should I be confessing? Is eating ice cream out of the carton (double dipping of course) and sticking it back in the freezer a deal breaker?"

Platelet Pole Dancing

We talked for awhile, while I was under the influence of Benadryl, and I think I confessed to a lot of anxiety about this whole silent sickness / need a transplant / need an unrelated donor situation. The speed at which this seems to be progressing is really throwing off my well-intended plans to hit some Bucket List items before the transplant. She listened attentively, likely wishing she picked another room. 

As I talked, my eyeballs got a bit sweaty and I decided to lighten the mood. I asked the minister to take a picture of me pole dancing with my platelets. She kindly indulged me as you see here.

I'm home now and feel exhausted. Time for a nap!

Marina's Port ~ Reflections on the Experience

It's in! A PowerPort is now a part of me.

Goofy grin courtesy of anesthesia! Glad the head cap matches my shirt, too.

I was relieved to learn that they would give me anesthesia before cutting into my chest to place the port and guide the catheter through a large vein (superior vena cava).  While I have a pretty high pain tolerance, I have a low blood and gore tolerance. I don't want to see or hear them cutting into me.

It was an out-patient procedure done in the Radiology department of the hospital. That seemed odd to me until I learned that the doctor uses technology to guide the placement of the port and the catheter into the superior vena cava. We don't want them poking around hoping for the best, do we?!

The port is the raised spot beneath the red stitches.

There are 2 cuts ~ the one on top (on my collarbone) is tiny and was used to thread the catheter into the vein. The larger one (about 2" below) was made to insert the PowerPort. I have no idea how the actual installation process occurs - it's a macrame or crochet technique beyond my pay grade.

I don't remember much of my time in the procedure room. I do recall a couple of occasions where I said something or started to turn my head (from looking left to right), the nurse would say in a soothing voice, "Everything's okay. Close your eyes and relax, baby." as she injected sleepy juice into my IV. Those are words worth remembering.

Robert, my marvelous husband/best friend/partner in life, was my driver and companion for this particular procedure. I really wanted him to be with me for this one and he was able to arrange his work travel to depart late that evening (after getting me home and settled). 

Yet, as we sat in a sterile exam room waiting for the procedure, I wanted him to leave. I didn't tell him that, of course ~ that would be rude. At the time, I couldn't even articulate to myself why I wanted to be alone. 

Now, a week later, I think I get it. Sitting in a cool hospital room in a no-frills "gown" that is open in the back (nothing you would see at Victoria's Secret), I felt small, weak, and vulnerable. The IV was in place (for the fluids and anesthesia) and I signed several information and consent documents.  The magnitude of the illness hit me like a ton of bricks. Tears started streaming down my face, silently, like a slow, quiet leak at first.

All of a sudden it hit me: I'm sitting in this room, about to have a port implanted in my body, because my veins are shot from years of phlebotomies, infusions, and blood draws. I need the port because I require blood from other people to stay alive until another donor enables me to receive a blood stem cell transplant. 

I have a deep belief that the stem cell transplant will not only save my life, it will restore my health to great condition. I will use this gift to do great things for others, although I do not yet know what direction this will take.  

Even with this belief, I still get frightened. Yet I don't want my momentary lapses of fear to add concern for my loved ones. They have enough to deal with - my everyday limitations, doctor visits, the many uncertainties of the disease process...

My husband has the added burden of being our sole provider of financial and insurance security. To distract him from his work seems counter-productive to me...especially hours before he departs for a business trip. Perhaps it's my feeble attempt to protect him. He has been nothing but kind, supportive, and patient with me through this process. 

The only thing worse than being a sick patient is being a loved one of a sick patient. Watching any of my family members suffer with no ability to "fix it" would make me crazy. 

In the end, I did not ask him to leave. I did not hide my tears. I did not bellow out Boo Hoo's or break into heaving sobs, either. I told him that I get scared sometimes and this is one of those times. He took it all in stride. I am so glad he was there with me.

A Port for Weary Veins

Port of Pireaus, Greece

When I think of a Port, I first imagine a place near land for boats to find shelter from rough seas or load and off-load goods and passengers.  

The second thing I imagine is a glass filled with a dark, heavy, rich dessert wine made in Portugal.

This coming Wednesday, I will experience another kind of port - up close and personal. I'm having a port installed in my chest. But it is for loading something other than libations.

When I went in for my second transfusion of Red Blood Cells (RBCs), the nurses told me it was time to get a port installed. My good veins for IVs are shot due to phlebotomies and Remicade infusions over the last few years. There is scar tissue and some veins have flattened out. Other veins are covered by lipomas (benign fatty tumors) on my arms and legs (fat isn't limited to cellulite, my friends!).  

The port installation is an out-patient procedure with local anesthetic. I do hope they will give me something to calm my nerves, because watching people run tubing through my chest will trigger some of my natural anxiety.

IV Educational Moment:
In preparing for this, I've learned that there are three types of intra-venous (IV) catheter mechanisms: Ports, PICCS, and Lines. Each is used for different medical purposes.

Ports:  Ports are funky-looking things that are implanted just below the skin's surface on the upper chest. A tube connected to the port is fed through a central vein towards the heart. Ports can stay in ones body for years if necessary. Ports are good for chemotherapy, transfusions, and delivering medication and other necessities. A special needle is required to connect to the port. Ports have lower infection risk because there are fewer points of entry to the body than the tubes from PICCs and Lines.

How Does A Port Work?


PICCs:   Peripherally Inserted Central Catheter 
I had one of these when I had the whopper hospitalization in 2007. They can draw blood, give medication and liquid nutrition without sticking me every time. A purple tube went from my right arm into a vein that led to my heart. I was unconscious when it was installed, but I do remember that when the nurse pulled it out it was no big deal.
PICC lines are not used for chemotherapy. 

Lines:
There are 2 types of lines, tunneled and not tunneled. Tunneled catheters / lines are used for chemo, blood draws, delivery of nutrition, medication, etc. They are not for long term use.

An on-line friend shared this brief summary of these 3 types of IV catheter approaches.


I'll post a photo or two of Marina's Port (sounds nautical, doesn't it?!) on Thursday or Friday.

Blood Stem Cell Transplants in the 21st Century

When I first heard about bone marrow transplants, I envisioned the donor getting drilled in the hips for bone marrow extraction (like the bone marrow biopsy and aspiration) and then a surgeon drilling into my large bones to implant the donor marrow. I am happy to say that I was way wrong!


In most cases, blood stem cells are harvested from the donor through a process called Apheresis. It is like donating plasma (some of you may remember college students doing this for weekend money).   

Over the years, the stem cells that get transplanted have many names:  

bone marrow, stem cells, hematopoietic stem cells, peripheral blood stem cells, and pluripotent stem cells. These are the stem cells that become red blood cells, white blood cells, and platelets. 

What's Involved in the Transplant Process

For me:    Patient and her caregiver must live near the transplant center for 6 months.
The week leading up to the transplant, I get blasted with chemo that will kill all the cells in my bone marrow (among other things) and also receive immune-suppressants to prevent my body from rejecting the stem cells.  I’ll lose my hair, get mouth sores, feel like crap…

For my donor: The week leading up to the transplant, my donor will have labs updated (to ensure no new infections) and get a growth hormone shot each day for 5 days.  Then will have blood drawn out of one arm; the stem cells are separated in a bag for me, and the blood is returned to the donor in the other arm.  Generally takes 4-6 hours. S/he will likely feel fatigued for the next couple of days. 

For me: I will receive the donor’s stem cells by IV.  Then I sit and wait for those brave stem cells to engraft in my bones and begin producing blood (takes 2-3 weeks).
I’ll also continue to receive anti-rejection medications for the first 6 months post-transplant.

At day 100: a bone marrow biopsy will be done to see if the transplant is successful.

Major Risks of SCT

Transplants for secondary Myelofibrosis are very different than transplants for other leukemias and much more intense than organ transplants.

The risks are highest in the first year of transplant.
1. Risk of Graft Vs. Host Disease: 30-50% with sibling donor.  Of those 20% will not be responsive to steroids (aka steroid refactory).  MF patients have more GVHD than other leukemia patients.
2. Risk of Death: 15-20% within the first year.  Organ failure is most common cause.
3. Relapse Rate: 20% within the first 1-3 years
4. Graft vs. MF effect:  treated with donor lymphocyte transfusions

These are odds I'm confident I'll beat.

When a Match = Life

I see lots of ads for matching companies -- match.com, e-harmony, christian singles... 
They sell the dream of happiness for life by promising to find your compatible, perfect match.

Here is a way to help someone (like me) find a perfect match that saves life -- and there are no monthly membership fees:   Bone Marrow/Stem Cell Donation 

First Fact:
A bone marrow or stem cell donor is determined by HLA (Human Leucocyte Antigens), NOT blood type.

Why match the HLA?
HLAs are protein markers which sit on all our cells. The immune system recognizes them and therefore does not attack them as foreign or infection. 

When the donor and recipient have matching HLAs, the likelihood of stem cell rejection or the new stem cells attacking the host body (Graft vs. Host Disease) is decreased. 

The ultimate match is when the patient and donor share 10 out of 10 HLAs. Most transplant centers require a 10 out of 10 or 9 out of 10 match for a standard transplant.

"Haploid match" is when the patient and donor have half of the HLAs in common. Biological parents and children of the patient are haploid matches. 
These transplants can also be successful with special care to decrease risk of Graft Vs. Host Disease (GVHD).

Get more info here:  HLA Match education

HLA Match Priorities

The priorities for my HLA match are as follows:

1.  Full-match sibling  

2.  Full-match Unrelated Donor (10 out of 10)

3.  Partially-matched family member 

4.  Cord blood

All four of my siblings did the cheek swab test through bonemarrowtest.com, as I did.
It cost $175 each, yet we got the info quicker than waiting for the doctor referrals and insurance approvals.

There is a 25% chance that a biological sibling is a perfect match. With 4 siblings, we figured that we had a good chance. One brother and one sister are haploid (half) matches with me and full matches to each other; the other two are identical matches to each other.

The doctor said that my son, Alexander, would be a haploid-match with me, and his stem cells would be preferred to my siblings because of Alex's youth. 

They also prefer younger male donor to younger female donor, so Katrina is excluded if Alex is willing to help his mom. In light of his upcoming car repair bill, I think he can be persuaded (wink, wink... both kids asked if they could be donors when this first came up).

We are now in the process to search for a 10/10 Matched Unrelated Donor (aka MUD) through the National Marrow Donor Program. 

The process takes 2-3 months, and the doctor believes that we will find a donor.

If you are between 18 and 44 years of age and interested in being a Bone Marrow/Stem Cell donor, check out these options:

bethematch.org

deletebloodcancer.org

They both contribute to the national and international donor databases. 

In the circle of life, I'm reminded that we receive what we put out in the world. Donation of bone marrow/stem cells, blood, plasma, and organs are the ultimate Random Acts of Kindness. Give with love and know that your gifts are received by the patients (and their families) with love and immense gratitude.

peace & love,

Marina

How We Chose the Transplant Center for Me

After an absolutely wonderful escape to Sicily, Robert and I returned home ready to tackle the next big adventure.  Where should I go for the stem cell transplant?  Who will be my donor?

Robert, Mom, and I met with Dr. Lawrence Morris of Bone and Marrow Transplant Group of Georgia in Atlanta. This group is under contract with our health insurance provider.

Here's what we learned:

About the Blood and Marrow Transplant Group & Facilities at Northside

Dr. Lawrence Morris came into our appointment well-versed in my condition and situation. Right off the bat he noted that I must be tired of hearing doctors say that I have an unusual and complicated case. 

The transplant group did 186 transplants in 2012 and their success rate exceeds the "Confidence Interval" (the expected success rate is determined for each center after controlling for the diseases transplanted, age of patients, co-morbidities, etc.). He said only 10 centers in the US exceeded their confidence interval. 

This center is well-within the top 10% of transplant centers in terms of scale. It's not a mega-center (like MDAnderson, City of Hope, Hutchinson, or Sloan-Kettering...)

Transplant Protocol
The transplant process would be managed mostly on an out-patient basis. This is very different from the Mayo process (5-6 weeks in the hospital). The chemo and the transplant would take place in their out-patient transfusion center. About one week after the transplant, I'd be admitted to the Transplant floor at Northside for 10-20 days, depending on how the engraftment is going, my vitals, any signs of rejection, etc. After release from the hospital, I will still go to the out-patient center daily and then every other day for check-ups. He said that at any point in the process, I could be admitted immediately to the hospital's transplant unit should that be necessary. 

They have better outcomes with the out-patient process, and I'd rather barf in my own toilet anyway. 

About My Situation

Dr. Morris explained the reasons why a stem cell transplant (SCT) is appropriate for me and the additional risks that I face. The Behcet's auto-immune issue is a bit of a wild-card. The portal vein thrombosis is also an issue; since my body has created new varices to deliver blood from the liver, we need to look and see if a similar thing has occurred in the esophagus (treatment would be required).

Transplants due to Myelofibrosis

He also identified the issues specific to transplants due to myelofibrosis: there is additional stress on the body due to years of medications, fibrosis in the bone marrow, and concern for stress on spleen, liver, heart, and lungs. That was a huge piece of helpful information for me because Dr. Fauble at Mayo said that there are unique post-transplant issues for MF patients but didn't specify what they are.

Given the stress on my body, he expects that the preparation regimen will be something between a full-in ablative chemo and the reduced intensity protocol.

Decisions, Decisions

After our Mayo and Northside appointments, Mom, Robert and I talked about the big, burning question: where to have the transplant. We want to go to the center that will have the best chance for a favorable outcome. 

The primary considerations: quality of care/experience;  quality of life for me & family;  insurance/financial considerations.

In the end, we are in agreement to have the transplant here in Atlanta.

Here's our rationale: 

Quality of Care/Experience:

• We looked at outcomes data and experience at Northside, Mayo-Scottsdale, MDAnderson, and others. Since there are very few transplants for MF, the disease specific data is hard to get. None of them do more than 2-3 per year for myelofibrosis. 
• Northside does enough transplants and participates in research; it's not a side business. It also does a great job - the docs have outcomes that exceed expectations and the transplant unit is state-of-the art.  
• They know the risks and issues associated with my particular situation. 

I don't believe we are compromising the care by staying here.  This group would be my local post-transplant support regardless of where the procedure is done. 

Quality of Life: 

• I really want to be close to Alexander during his Senior year in college. Whether I'm in the hospital, an apartment near the hospital or at home, he will be able to come visit regularly.
• Robert can do his job and he won't have to choose whether to stay with Alex on weekends or fly to see me somewhere. 
• Staying local will also help me stay out of the depression zone because I won't feel as isolated from my loved ones. I do better keeping my game face on when I've got good reasons to do so. 
• The out-patient protocol appeals to me a great deal. I recall going stir-crazy the last few days of my 2 week hospital stay back in 2007. Being confined for weeks is not appealing to me at all.    
• We have many friends who are willing to form a 'care team' to help out as the journey continues (it will be a marathon, not a spring). 

Insurance/Financial:

• I was initially inclined to go to Mayo-Scottsdale (as you all know). After looking at the data and meeting with the Northside doc, we'd have an uphill battle to get insurance to pay out of network.  I don't think we can make a compelling case that I'll get superior care elsewhere based on the data. 
• I don't want the added stress of insurance and financial burdens.
• Kaiser Permanente has excellent coverage if we do the procedure here. 

I'm glad we got the diagnosis and recommendations from Mayo first. Not only did it give us time for the info to settle into our minds and hearts, it gave me time to do more research, both data and anecdotal, before meeting with the folks that our insurance will cover.

We ran through several scenarios and the biggest question: If things go poorly here at Northside, is there going to be any shoulda/woulda/coulda discussions? 

There really isn't a stand-out, slam dunk transplant center that we dismissed. So we are in agreement.

So on we go... no regrets!