September 24, 2010

Today is the first day of the rest of our lives. How we cope with the challenges that face us will be the focus of this blog. I created this blog as a outlet to express my inner most feelings and for therapeutic reasons. The tensions and reactions of today made me realize that I need to let it all hang out someplace. If this is too honest and raw for anyone, please realize that this is my way of coping since all that I do is for Joe's well being that I am forgetting about mine.

I will start by giving some background of Joe's blood disease and when it all started. The truth is that we do not know when it started since Joe's primary doctor at the time did not notice that Joe's annual blood tests were abnormal for at least 3 years. It was Joe's neurologist for Joe's nocturnal seizures that advised us to see a hematologist. We started with Dr Allan Freedman at Emory Eastside in Snellville and also met with several hematologist before settling on Dr Freedman because of his no nonsense approach and a well oiled run office. Freedman's confidence was reassuring. Joe's first Bone Marrow Biopsy (BMB) was done by Freedman at Emory Eastside. It was fast and relatively painful. Joe could tell that Freedman was very experienced by his fast technique. Next we had to wait till the BMB was analyzed and Joe was diagnosed formally with Polythemia Vera (PV) which is a Myeloprolifeative Neoplasm Disorder. It means that the bone marrow is overproducing red blood cells and platelets making Joe's blood thick like molasses. The danger can mean strokes, clots, heart attacks without treatment. Thank God, he did not have that happen during the undiagnosed years. There are various methods to treat PV and that was a hard decision to make. First they had to get his blood counts down by taking blood out by what they call phlebotomies where Joe sits in a chair and instead of getting blood they take it out and throw it away. We decided to have Joe take INTRON A (interferon) which was by injection twice a day. Freedman's office taught me how to do it by testing on an orange. Joe hated INTRON A since it made him feel like he had the flu all the time and all he wanted to do was lay on the couch. After giving it 6 months, Joe and I decided to have him quit that and take Hydroxyurea (HU) orally which is a type of chemotherapy which we read could cause leukemia after 10 years or so but had not been proven. Joe said that he rather that chance and have a good quality of life and that is what Joe did for 11 years along with regular plebes which got to be 4-6 months intervals which Joe was very pleased.

In winter of 2008 Joe started have a steady low grade fever, great fatigue, sweats when he was horizontal, loss weigh quickly but had a huge stomach. Joe would check in with local hematologist Freedman. Joe being Joe would tell him that he was just fine. Both Joe and I were getting ready to retire on 7/1/2008 so I decided to go with him to Freedman's office to get his blood tested (CBC) and then meet with Freedman. Sure enough Joe told Freedman he was fine and I rolled my eyes. Freedman caught that then asked Joe what was really going on. Joe told him to ask me. I told Freedman and Freedman asked Joe if that was true and Joe said Yes and Freedman asked Joe why did he not bring this to Freedman's attention himself. Joe just shrugged his shoulders. Freedman stated that he needed to take a closer look. He stated that Joe's spleen was enlarged and sure enough that Joe did lose a bunch of weight. He scheduled Joe for his second BMB within two days. We were heading on our retirement adventure to Africa on 7/5/08 so we would not know the results till August 2008 when we came back. He gave Joe his blessing to go on African Safari.

The first week of August 2008 we checked back with Freedman who was so excited about the clinical trial at MD Anderson Cancer Center (MDACC) in Houston and that he had communicated with Dr Verstovek (V) on a general basis and looked as of Joe would benefit from the trial. Joe's BMB locally showed progression to Myelofibrosis (MF). MF is one of the myeloproliferative but neoplasms where Joe's bone marrow has grown scar tissue where the bone marrow is not creating healthy red and white cells. Joe's genes had mutated and he has the JAK2 positive gene which 95% of PV patients have. Fatigue and sweats can knock the socks off of most people. The INCYTE drug trial attempts to block the JAK2 gene. It treats the symptoms and improves the quality of life (QoL) but does not cure the disease since there is no cure. It is said that once someone has been diagnosed with MF that their life expectancy is 3-5 years. There has been some progress made on mini stem cell transplant since full stem cell transplant odds were horrible.

It was important to Joe to have a good Quality of Life so we decided to join the INCYTE trial when Joe was offered it from Dr V. Joe started the INCYTE trial on 9/12/2008 in Houston the day that Hurricane Ike hit Houston and Joe was stuck there for 6 days. He was miserable but survived and has great stories to tell of his experience. Maybe it was a sign from God that Joe's experience would be a great storm. It has been and the storm continues.

The INCYTE did not shrink Joe's spleen which is now 30 cm in length which is caused by the storing of immature white blood cells (lymphocyctes) that do not want to die. Joe had a full lab day in February 2010 and when I reviewed the results on line, I noticed that the BMB pathology report mentioned CD20 and CD5 and the words consistent with leukemia and lymphoma but slightly atypical for CLL. I contacted Dr V in March why he had not mentioned this to us and he said not to worry about it and it changes nothing but the words just scare patients. Well heck, yes! I trusted Dr V and we went along traveling and enjoying life as we had. Looking back over the last 12 months Joe had four nasty respiratory infections and long lasting deep coughs. He had several minor shingles outbreaks. Some enlarged lymph nodes in Joe's groin. He had three silver dollar sized cold sores above his lip that took forever to go away. He had several basal and squamous cells removed off his face, ears and back. His night sweats were coming back stronger and his stomach was getting bigger whereas his muscle mass was decreasing. Since he had MF I have been tracking his blood counts closely and whenever they got out of whack I would notify Dr V and the INCYTE would be stopped, then started, and adjusted several times. During the last 6 months Joe's WBC starting rising quicker. Before we left for Alaska in July, Joe had full lab work done once again at MDACC in Houston as part of his clinical trial check in. When we were in Alaska, Joe got another horrible respiratory infection and cough. When we got back to Atlanta mid August, he went to get his CBC blood test locally as he usually does. Joe's WBC was 83 on 8/19. I tried to go onto mymdanderson web site to review the results of his July visit but was unable to get on line to see the results. Next CBC on 8/31 his WBC jumped to 98.6. I was now able to get on line to see the reports. On the pathology reports for the July MDACC visit it showed that Joe's Bone Marrow Diagnosis as: Persistent Primary Myelofibrosis, MF-3, Chronic Lymphocytic Leukemia (CLL), Small Lymphocytic Lymphoma (SLL).

CRAPPO! I stated why the heck am I finding this out all on my own for Joe. I sent an e-mail to Dr V to request that he look at Joe's last pathology report and get back with me. I had stated nothing else. Dr V e-mails me back that nothing has changed and to continue going what Joe has been doing. YOU HAVE GOT TO BE KIDDING ME. I could not believe that response.

I then e-mailed back Dr V that there sure had been a change a drastic change and also went over all that Joe had gone through the last 12 months. I requested that we get a second opinion from Dr Michael Keating and that we would like to see Dr Keating on our next scheduled visit to MDACC on 10/12/2010. From my immense research on the God wonderful web, I knew Keating was the top expert of CLL in the US and he was located right where Dr V was located. Dr Keating agreed to meet with us on 10/12. Next CBC blood test on 9/15/2010 showed Joe's WBC now at 120, increasing rapidly. Thanks to the nudge of Joe's Family and Friends they suggested us to get a faster appointment. On 9/16 I e-mailed Dr V if we could see them both sooner rather than later . They agreed to see us on 9/21. It was wild getting air reservations, hotel and car reservations for Monday 9/20 to get to Houston. Thanks to frequent flyer Delta mileage I was able to use those to avoid the $767 round trip fare for each of us. Priceline came through with a 4 star hotel at the Galleria for an amazing $60. The rental car deal was not so great but it was the lowest I could find with Dollar. It is obscene the add on charges that rental card companies charge. Do you know there is even a charge if you turn a car in early? REVOLTING.

So this past Monday Delta was not ready when we were since the brakes were bad on the plane we were on but three hours later we were on our way to Houston on a new plane. The car and hotel were terrific which made things less stressful . There were little kids next to our room making lots of normal kid noise. The hotel was gracious and efficient in moving our room from the 7th to the 12th floor . Now we had quiet so Joe could get a good night's rest. He did.

Up at 6AM and we drove over to MDACC. Thanks to the iPhone for directions. We checked in on the 8th floor which is the Leukemia Clinic where Dr V and Keating work out of. Joe got his vital signs and blood drawn then we wait till the blood results are done. Finally we get called at about 10:30 to see the doctors and the clinical nurse. I guess we saw at least 5 people before we got to Dr V. We got a copy of Joe's CBC for that day. His WBC is now up to 137. Climbing higher so quickly and so scary. When Dr V came in, there were 2 others docs and Cathy, the clinical nurse. He agreed that I did not over react when I brought to his attention the rapid rise in the WBC and lymphocyte count. Again I asked him why did I have to find out about the CLL on my own on line. He said that we had discussed that in March ( not really). I knew that I was getting no where. Now we had to wait to see what Dr Keating says but the appointment was not till 1pm so we headed to lunch on the first floor of MDACC where there is a wide variety of food where one can always find someone that fits their taste buds.

We went back upstairs to the 8th floor and waited. After one and half hours we get called back to the exam room where again we meet with several doctors who get info and do some examination of Joe. Dr Keating comes in and gives me a bear hug and shakes Joe's hand. He has a vibrant smile and personality. He hails from Sydney, Australia and I love him already since I never have met an Australian that I did not love. He says that he needs to wait on some more blood test results before he recommends the therapy for Joe's CLL. It is a big concern that his WBC is increasing so rapidly and tells us that he will see us the next morning.

That night we meet with our adopted Chinese family who are now living in Houston . Yan is enrolled to obtain her advanced degree in Public Health. We met Yan and Hang through the AMIGO program when Yan was a student at Emory University. We had invited Yan and Hang to Thanksgiving Dinner 2009 and also another Emory Physics Major, Xia to learn more about American Culture. We adored them all and became friends. Yan became pregnant and we got to meet both her Mom and Dad for dinner at Red Lobster. It was so special. Even though her Chinese parents could not speak English we bonded with them since we loved Yan and Hang. Yan's Mom stayed in the US to help Yan with the baby's birth in June along with the move to Houston, What a MOM!!! We met them for an amazing authentic Chinese dinner where we had our first Peking Duck and other new dishes. I tried them all (so much food) and only did not like the cold fried fish. I got to hold Yan's gorgeous BIG baby girl. I had always thought Chinese babies were tiny and petite, not this one. It must be the air in the USA. Yan's Mom was there and she just glowed and could not keep her loving hands and arms off her beautiful granddaughter. Thank goodness, Yan is breastfeeding her or she would never get to hold her bundle of JOY. They treated us to the dinner in spite of Joe wanting to pay our part but they would not hear of it. I gave the baby a soft blue and pick stuffed elephant as a small gift. Yan and Hang gave me an amazing string of pearls from their Chinese village. Both Yan and her husband are so smart and loving. What a gift God has given us with this unusual friendship! They are our adopted Chinese family.

Now it is Wednesday morning we checked out of the hotel and headed back to MDACC to meet with Dr Keating and his professional team. Keating recommends the golden CLL regimen of three drugs abbreviated as FCR and that we should start ASAP. BUT we have to wait to see what Joe must do to get weaned off the INCYTE trial drug since we know that there have been some dire cases of people who came off of it too quickly and passed away. I have now heard from others who weaned off of it without any issues. Dr Keating states that Dr V will contact INCYTE and get their input. Since I have not heard anything yesterday or today I e-mailed Dr V and he stated he was waiting word from INCYTE. An hour later Cathy, Dr V's clinical , called Joe and I back that they were waiting for Incyte and that Joe could be weaned off the Incyte before he starts the chemo of FCR on 10/12. Joe will get chemo for three days and they will watch him carefully for adverse reaction since the reactions would happen in the first couple of hours but they have proven ways to offset any adverse reactions. After that Joe will have 5 other cycles of Chemo which can be done in Atlanta. I do not know how and where that will be done.

Joe stays very active to keep his mind off of his disease. He has had very little patience with everything especially me. I need to keep him positive but he can sure aggravate me that I need to hide away for while. It is what motivated me today to start this blog.

Lynn Scott and her sweet sister, Lucy, stopped over for a visit today and bought us some coffee cake and we chatted for an hour. Yesterday, sweet wonderful Sally Jackson stopped by with her home made bread and chatted with Joe while I was getting physical therapy for my right aching shoulder. Unfortunately I came home in tears when she was here. I just became overwhelmed with so much advice and trying to assist in making the best decisions for Joe.

Oh, I also sent an e-mail to Dr Tefferi at the Mayo Clinic in Rochester, MN to get his input on how to get Joe safely weaned off the INCYTE. Dr Tefferi is a medical genius and is the head US doctor in charge of the INCYTE trial. Joe and I met Dr T in New York City last November at the Symposium and found him honest, funny and straight forward. Thank you to Annette fellow MPD CML INCYTE chat person for Dr Tefferi's e-mail address and your valued advice and pep talk.

Thank you also to Joe's prayer army and my family reaching out to me when I need it the most.

Ciao, on another adventure!
Bonnie

Please ignore spelling and grammar since this is NOT an English assignment.