More Than the Number

CK is oddly still higher than I would like- still in the 900's. It was also in the 900's when we left the hospital which was a little over 2 weeks ago. I am not sure why this is but I feel good and need to wait it out. I really don't know what my baseline was before all of this but normal is 23-300 or so. As I try to ignore the numbers I instead focus on the fact that I can once again see some definition amongst the free flying flab. So I am gaining muscle along with some needed holiday fat and surely don't miss my old hairy Italian self.

Also, ovaries are still working:)

Being able to hold up my part a little better during pool wrestling sessions with Graham has brought back some good memories.

Since I didn't get around to a holiday card this year...here is one for you now.
Cheers to a great New Year!

Energy

I am pleased to report that I have energy! I don't need to take a nap in the middle of the day and I can actually stay up past 9pm. My workouts are actually enjoyable and I don't get slammed with fatigue after them. I notice such a difference...My muscles are still weak of course but I can tell that it isn't weakness from active Myositis. It is weakness from muscle loss/damage that can hopefully be built up. Going to physical therapy was pretty exciting since I was able to show off my improvements. My balance is much better as well.

Graham is very intrigued by my bald head even though he doesn't really see it. I think we will do some Henna on it soon.

I will do a CK today and hopefully get good news. I have waited a bit to let things even out and am trying to focus more on how I feel instead of what the numbers say.

Moving On

So I was looking back and realized how much Plasmapheresis and Rituxan really did help me. There was a point when I couldn't get out of that chair, when I couldn't walk alone or roll over, when I would fall continuously. I never documented that phase and never want to revisit that 90 year old body. That is why I went forward with HiCy.

I do not know if this will put me in remission and for how long. I do not want to think about it actually. I am done being consumed with health and ready to move on; to experience life instead of watching it pass me by.

Everyone has something it seems and all I know is that pushing to get the treatment you deserve is your right. I am so much more thankful now. I started this in such denial, not realizing that running isn't the goal anymore but just functioning. Step by step, day by day.

Having a supportive family and community has been critical. Your positive energy wherever you have been has helped more than you know. Now let's talk about something else!!

Welcome Home!

Strength Comparisons - 3 Weeks After HiCy

Art Therapy

"This is the war between the white blood cells and bacteria. Don't worry the blood cells will win. Get well soon." Thanks Adrian!


"This is a picture of Rapunzel. Get well soon." Thank you Ellawyn!


The latest saber-toothed tiger from Graham.

Rollercoaster Continues

Good to be home to clean air but we were a little disappointed leaving the hospital to find out that my CK jumped a bit from 658 to 910. All the nurses say not to worry since counts fluctuate around after treatment and to wait it out. I am a little concerned that we were tapering from 15 to 10mg of Prednisone and now are going to sit tight at 15mg.
Coming home to an empty house was not ideal, but I will take the time to rest and go through the mounds of papers in my office. I won't do too much holiday prep since I need Graham's help for most of it. When Granny started to explain that Mom took a new medicine to help make her stronger, he replied, "Will she be able to run?"
Slowly but surely Cracker Man.

Home again, home again . . .

Walked in the door about an hour ago. Sure is nice to be home.

Jen spent her last morning in Baltimore visiting IPOP for blood work and getting her Hickman line taken out. She is getting to be such a pro at this that I spent the morning loading the car up with our stuff. The MRI they did earlier in the week didn't show anything new, Jen might have some more detailed information on that. Her white blood count was over 8000 and her platelets were 70 something - high enough that she didn't need a transfusion before the surgery (removing the line.)

Thanks to everyone for sending emails, phone calls, cards, boxes, and all that good stuff! She is upstairs now unpacking her bag and jamming out to the Allman Brothers, which is a good sign!

Going Home Early

We found out today that we could be discharged from IPOP today since my white count is now 2230!
I still have to go in tomorrow for an MRI of the brain to double check some calcium deposits they found in the CT of the sinus. Friday we get some final labwork and some platelets before I get the line out and off we go. Counts should be in the normal range by Friday for sure.

So for those that are thinking of doing this treatment, I must say that the hardships are few and benefits great. Aside from one night of vomiting, one night of a high fever, and one night of some joint pain, it really was not as bad as I was expecting. We will still have to take precautions to stay healthy as one would with a newborn.

We can't wait to see Graham and be together getting ready for the holidays. I did this for you little man!


I hope everyone has a simple and peaceful holiday with lots of singing and dancing!!!

Day 13 IPOP

Today we got more good news.

Firstly, my white count is steadily rising and is 470 (doubling almost every day!) Dr. Brodsky said today that it should jump pretty quickly in the next day or so and we may be out of here by the end of the week! It may get tricky and actually be early next week since we wouldn't be able to remove the cathetar on the weekend. Also, my CPK today was 658! It has not been below 1000 in almost 2 years. This again is the indicator of my muscle enzymes seeping into my blood. So the low numbers means the inflammation is indeed going down!

There have been small changes that I have noticed in my range of motion. The left leg hamstring definately has improved and lifting my arms outstretched from my side to above my head is surely more fluid. The mental fatigue/fog has lifted. Rebuilding the muscle damage will surely take awhile, but now I have some of the energy back to climb that mountain.

Hopefully we will be mask-free soon and the joint pains won't be too bad.

Well the joint pain has started now in the evening as expected in the hip area.

Back to IPOP - Day 12

We are glad to be back with the IPOP crowd again and happy to report that my energy is better than it has been in awhile. We are waiting now for IV antibiotics to finish up and have to continue them for a few weeks or until counts are back to normal. Spacing out when I administer them at home is challenging since they have to be given 6 hours apart and I want to get as much restorative sleep as I can. As far as counts are concerned, WBC are slowly going up- 200 today.
All done...time to go.

Thank you for all of the wonderful letters and packages of warmth, support,and laughter. The snacks, games, and books have been put to good use and we very much look forward to using our Send A Meal gift certificate. Thank You Appalachian Lab!

Hospital Day 3-Back to Hotel

Up early since I got some good sleep and am glad to report that the fever is down to 98.9. I am getting one unit of blood right now and am anxiously awaiting breakfast and news from the cultures. Hoping that all of this was just a neutropenic fever, meaning a natural response to having no white counts.

I hope others took part in the National Day of Listening and recorded someone on StoryCorps. Although I wasn't much in the mood for an interview yesterday, I hope to sit down with my grandfather in the near future and gather the many stories.
http://www.npr.org/multimedia/2008/11/ndol/ndol.html

More later...

Fever is back to normal and we are heading back to the hotel tonight!
We have to continue with the IV antibiotics ourselves and found out that white count went up a bit to 130. After seeing our excitement, the nurse reminded us it isn't quite time to be rolling around with runny-nosed kids.

Hospital Day 2

I am glad we are here now and covering all of our basis to rule out infection. They did many cultures to see if there is infection in the line or in my bloodstream and we are waiting for those results. I do not have the flu. One of the doctors told us that 10% of the patients that have to come back because of a fever actually have an infection.

The chills and aches have subsided a bit as we are letting the fever do its thing without taking Tylenol. I did get to finally get some sleep after taking Benedryl at 6 this morning.

The hair loss was getting to be a mess so Andrew just shaved it and it feels so much better.

I am hoping this fever goes down sometime soon. It has been around 100 or so most of the day. I am in good hands and will hopefully be back to the hotel in a few days.

Back to the hospital

Jen's fever went above 100.4 so she called IPOP and they told her to come in and be admitted to the hospital. They are worried there might be some infection somewhere in her body, so they have started her on IV antibiotics and did a CT scan of her chest and sinuses (which was negative). She had the chills and was feeling uncomfortable (tight throat, achy) but they gave her a Tylenol and she feels much better now - she is even hungry again. They did a throat culture this morning and a blood culture just now. It will be 48 hours before they get the results of the blood culture, but if there is something nasty in there they will know in as little as 10 hours. Jen's plattlets are below the threshold, so they are setting up to give her plattlets. I guess the red blood cells are ok right now, but I haven't seen the numbers. Chances are she will need red blood cells in the next day or so.

Although we were hopeing Jen wouldn't have to go back to the hospital, many HiCy patients do have to be readmitted. Jen feels fine now that she has a Tylenol in her so it almost seems silly to be here, but the risk of infection is very high so it is best to be careful. She will probably be here for 2 or more days, or until her fever goes down. There are various scenarios that would cause her to be here longer, but nobody seems overly worried about her. They are all just following the protocol.

Happy Thanksgiving!

Today has not been one of Jen's best days, but nothing much out of the ordinary. Jen woke this morning feeling achy and tired. When we got to IPOP she had a mild fever (99 something) and complained of fatigue and swollen glands in her neck. The nurses at IPOP are always worried about the flu so they did a nasal flush and throat culture. We will hear a preliminary result from the culture tomorrow and will continue to take her temperature, which seems to be hovering around 99.4. We have to call IPOP if we get to 100.4. Also, Jen's heart rate raced a bit when she stood up and the nurses thought it might be due to a bit of dehydration. So they put her on the drip again for about an hour.

In other news, Jen started to notice more loose hair on her pillow and clothes. I expect she will need to shave her head tomorrow but she remains recalcitrant to arguments that she shave a moment before she needs to. Her red blood cell and platelet counts continued to decline (indeed she might simply be tired because of her low red blood count), but neither were low enough to justify a transfusion. Oddly enough, her white blood counts bounced back up above the detectable level. No one can say if this is the reboot or just random fluctuation.

Day 7 IPOP

Counts today are lower than yesterday but no need for transfusion yet. We are curious to see if they will go any lower tomorrow or will start to rebound. I had my dressing changed to the fancy Obsetra 3000 model that you don't have to change for 7 days and had the upper stitches removed from entry of the catheter. The stitches close to where the line is are supposed to come out 30 days after, so we have some time on that. The AST and ALT counts always come back elevated and nurses are always alarmed about my liver function but I always reassure them that my ALT and AST have been always elevated with Myositis. They are reading up about Myositis more now and learning why I want my CPK drawn.

Cravings have started for red meat!

Day 6 IPOP

Nothing much to report today which is a good thing. My potassium level was just a bit low, my appetite is back, and my energy level is pretty good considering I have no immune system. My packed cell volume is 34.1 and anything lower than 25 and I will need a transfusion. I checked out the exercise room here at the hotel and it was perfect, no on in there and with new clean equipment. The main obstacle now is the monotony. We are turning into an old married couple watching old reruns on TV and reminding each other to take their meds. Soon we will have matching jumpsuits.

A child's perspective

This one is inspired by the son of one of my good friends who has MS and is thinking about HiCy. When she mentioned that she may loose her hair, her son said it was OK as long as she looked like Asajj Ventress from Star War's Clone Wars. So here is a reminder to all the bald moms out there of how cool they really are.

Day 5 IPOP

Neutrophils, the body's white blood cells that attack infection and my muscle are down to zero. We had the growth factor shot and it wasn't as spectacular as Andrew's rendition of old comic book heros healing miraculously on the spot with their "growth factor" magic powers. I will take this for 2 weeks, or as long as my neutrophil count is below normal.

I looked at the calendar and from what others have told me, you lose your hair 12 days after the start of chemo so that joyfully leaves me bald on Thanksgiving. I am ready and Andrew suggested I go for the Sinead O'Connor look but we may need a new wardrobe to pull that off.

Energy level is not as bad as I was expecting and morning nausea has stopped. We had some Indian food last night which was so good. I do like the flexibility of walking to the store and to dinner, but I am supposed to be very careful now that my neutrophil counts are at zero.

My CPK numbers are 1140, down from 1864 10 days ago. Normal is about 300.

Day 4 IPOP

Another day....and counts are down to:

WBC: 430 (Normal: 4500-11000) (Fight infection)
RBC: 3.96 (Normal: 4-5.2) (Carry oxygen to body)
Platelet: 128 (Normal: 150-350) (Blood clotting)
Neutrophils : 258 (Normal: 1500-7800) (The lymphocites/neutrophils are the body's attack cells).

We were reminded that we need to bring our meds with us in case we needed to stay longer for some reason. I also figured out that I was supposed to take 2 and not 1 each morning of the Fluconazole. We went over what to expect tomorrow when I get the growth factor that triggers the reboot. I think I will be at nader or below detectable levels for 3-4 days. I am expecting some pain or discomfort in the bones and joints and it will be a longer day than the normal vitals and labwork.

I have noticed some spotting and we will monitor it. Bleeding of the bladder is one of the main risks but I don't think this is related. I think it is carrying over from my cycle now that platelets are low.

Nurses are great and are finally figuring out how to pronounce my name. One almost was recruiting other nurses to take us home for Thanksgiving dinner!

Day 3 IPOP

Well 4 days after chemo my counts are steadily dropping to:
WBC: 1140
RBC: 3.83
Platelet: 138
We still have a few days before they get to zero and are now going to IPOP daily.


Andrew has gotten into a grove as scientist/caretaker and can go from twirling around the room with excitement that I defied gravity with my hamstrings, to then settling down to write proposals and reports in waiting rooms. He probably has all the vegetarians on our floor in a tizzy since our whole floor wreaks of tasty Mama Dot stew. Of course you have to have Mama Dot stew with a side of football. He explored the exercise room since we are now urbanites and he can't go cut wood or hike around. I wasn't feeling up to joining him since just doing stationary exercises is fine for now.

So seeing some improvement today in one little way was all we needed to remind us of what will be!

Morning, Day 3

Woke up this morning and started breakfast before Jen woke up. A few minutes latter I heard her yelling from the other room, went in there, and found her doing her exercises. She was laying on her stomach in bed and lifing her feet off the bed (by bending at the knee). . . . this is something she wasn't able to do with her left leg for the past several months!

We are going to IPOP at 9:30. Jen's WBC must be low if she is making this kind of improvement. Thank goodness muscles can heal if you just give them a chance. Keep your fingers crossed.

Day 2

We have a day off from IPOP today and glad for it since I am beginning to feel some fatigue. Diarrhea has also hit yesterday and today which was expected and not too bad. So the honeymoon is over for now in terms of walking around and eating out like we did yesterday. We wanted to head out while my counts were still sort of up. It was pretty unusual for me to eat so slowly and have such small portions since I love food, especially Japanese. Strolling around a city just the two of us was a reminder of how we are lucky to at least have the time to spend together.

Being around the hospital, the nurses, seeing the other patients, going through chemo, reading the literature etc. has made me so much more aware of what a Cancer patient really goes through. I am having some sadness in that regard in how absorbed in my own life I was as some of my family and friends underwent this. We are all human I guess and only know from experience. Their strength and grace is something I hope to follow.

Andrew commented today how excited he was to hear I was wired last night. He said he couldn't rememeber the last time I was wired at night. I don't know if it is the meds or my old self coming back?

Here are some numbers from yesterday:

White Count: 2150 (Normal 4500-11000)
Red Count: 3.95 (Normal 4-5.20)
Platelet: 181 (Normal 150-350)

I will do CK on Tuesday and will pick up packages today at other address. Also, sorry to say but we are not allowed to have plants or flowers. We will have friends take care of the ones we have gotten until we can have them again- Thank you!

Day 1 IPOP

We had our first night at The Tremont Park last night and are settled with all our stuff. It is smaller than McElderry but it is nice to not have to deal with stairs to and from the kitchen. Also, I can wipe down a smaller space easier to keep clean. We do have daily cleaning if we want it, but we may just do it ourselves.

I had some crazy dreams with the meds and a little nausea during the night, nothing bad. My appetite is almost back but some smells do turn me off. So now we really are trying to remember the little details like to take my temp 3 times a day, when to take the many anti-biotics, to wash my mouth after meals, wash hands, wear mask, keep excercising, oh and see some of Baltimore!

I will post my labwork which we should get back today. My white, red, and platelets should be going down here within a few days. Oh and I was reminded I need to look at my vitamins because they can't have any iron in them. You don't want to have and iron build up if /when I get a blood transfusion.

We figured out how to talk with Graham with Ichat so we could torture ourselves. Maybe Skype will work better with him moving around so much. He had fun plastering his face close to the camera and shining his flashlight at us. I showed off my painted pasta necklace he made for me and tried to get descriptions of the many pictures he sends us.

I am glad we are in a better area so we can venture out to some shops if we want to. I also don't have to worry about Andrew walking alone to the store=)

Fresh Baltimore Air

I made it! I am up, holding food down, and actually clean too. They are giving me potassium before we go and then we will pick up the meds at the pharmacy downstairs. I am eager to be unhooked from the beast of tubes to get some good Baltimore fresh air=)

I can't tell all of you enough how much it has meant to have your letters, calls, etc. You really don't realize what you are truly going through until you see everyone gathering closer. We were so used to doing this and plugging along, and now with others along I sure hope to have some good news!

We now enter bubble mode...

Sleepy time

The nurses have been giving Jen anti nausea drugs left and right. They are helping, but Jen still has not had more than a cracker in the past 18 hours or so. The side effect of the anti nausea drugs is sleepiness. So she has been sleeping most of the afternoon. Not sure if she will be able to sleep tonight or not after all these Zs. But as long as she isn't vomiting I doubt she will mind much.

The fourth and final dose of Cytoxan was this afternoon at 1:00 and prompted all the anti nausea drugs. If Jen is feeling up to it we will leave the hospital tomorrow morning. There was some chance we would leave this evening but I don't think we will now. Jen just isn't up to it. Her white blood counts are just starting to show signs of going down, which is what we want to see.

We have decided to stay at the Tremont Park Hotel rather than the McElderry. We should be able to pick up mail at the McElderry for a few days.

Rough spot

Well, we have reached the point where the Cytoxan is of high enough concentration in Jen's blood that she is experiencing side effects. She was up all night vomiting, including all her pills. So they will start delivering all her other meds (no Cytoxan until noon) intervieniously here in a minute. They are also going to start her a stronger anti-nausea drug.

I feel pretty pretty helpless. All I can do is hope this passes pretty quickly. Unfortunately, it can't really start to pass until after the fourth dose at noon. Jen doesn't seem to want company at this point.

HiCy Day 3

The morning was uneventful, I felt good and did some laps around the halls and some exercises in my room. Took a nap, talked to both Dr. Christopher and Brodsky and then got my 3rd dose. Right during my 3rd dose Dr. Brodsky asked if I wanted to be filmed in a Hopkins video about HiCy. I guess the filmmaker was interested in the rarity of this and I managed to speak somewhat coherently even though I had just gotten Ativan and HiCy. Luckily, I waited an hour later after they left to vomit in the trash can. Oh the life of a movie star=)

HiCy Day 2

Yesterday went smoothly, just a lot of down time and was fighting a bit of a headache. I didn't take anything because I didn't want to be sleepy all day.
Not sure if it is the Anzemet (the anti-nausea drug) that is working or if the nausea just hasn't hit yet. Maybe it won't? Keep your fingers crossed. I keep saying I am going to eat light and then I dive into all the meals.
We did some laps around the halls yesterday which was interesting with the rolling poll of chaotic tubes which I have named something I can't repeat =) Dr. Christopher will hopefully stop in today and we will discuss the tapering question. I think doing 15 to 10mg for awhile will be best. All the nurses have been wonderful and they still come to my room even after I hit the call button repeatedly by mistake.
If all goes well, I will be out and to the townhouse by tomorrow night!
I will start HiCy today at 4pm and then Tuesday at noon.

HiCy Day 1

Yesterday was mostly a day of waiting. We arrived at 10am to be admitted but were sent home to be called back later. I took advantage of the time and took a nap and then we came back to IPOP at 12 or so. They changed my dressing and I had them take a CK which ended up being 1864.
When we finally got into our room they did height, weight, EKG, a cognitive test. I had to follow multiple directions, remember words, draw pictures, and write a sentence. "I will run again."
Nurses and docs are nice even if they have wake me up every 4 hours to do vitals. We were a little concerned when one of the fellow oncologists told us that he wanted to taper my Prednisone to 10mg now. I just went down from 20-15 last week with my doctor's OK and do not really like doing so without her ok. I guess they do this all the time and the adrenal will make 7.5 mg on its own, so we are safe.
Joyful addition to this is I started menstruating which I thought would be an issue with the low platelets, but those numbers won't start going down for a bit now and I will hopefully be in the clear. If not, they will give me something to slow it down.
Andrew has transformed into my errand boy/nurse and is doing a great job. I get to vicariously hear about the outside world and he keeps me smiling.
No nausea, just a headache and tired and trying to block out the machine that I am hooked up to that sounds like an on-going slide machine. We brought in the fan last night which helped with noise.
So after a whole day of waiting for the doctor to come in, do a check up, write the order, get the OK from the pharmacy, etc. we didn't start chemo until around 11:30/midnight. The pharmacy was questioning this treatment since it is not usually done and we were trying to have the on-call fellow doctors send articles. They couldn't be reached, but by the time the on-call physician came, it was fine with just our verbal ok and his signature in the morning. I was given the anti-nausea drug, Mesna to line my bladder, Ativan, and then the HiCy. I actually fell asleep when they gave the chemo for my 4 hours of peace.
We will start HiCy tonight at 8pm I hope.

Goodbye white blood cells

I am sure, or relatively sure, Jenny will want to post more details tomorrow, but I wanted to post that she is doing well and is starting the chemo (Cytoxan) now. She is very tired and might actually sleep soon because they gave her something to help her sleep.

It is 40 minutes past the end of visiting hours, but they haven't kicked me out yet. I will see how this goes for a bit longer and then hit the sack myself.

Checking In

Having the Hickman catheter placed yesterday was no problem. I only wanted a local anesthetic since last time I didn't enjoy the side effects of the sedative. It was nice to be aware of what was going on and to leave sooner. I had my headphones on and did some good breathing and visualizations to stay relaxed. I didn't fill the pain med script they gave me and just took some Benedryl to help sleep with the discomfort. Since it is a smaller catheter than pheresis and I am stronger this time, I am not in as much pain and I can move around a little better. With scheduling issues, what pain med would be acceptable, questions for doctors etc., I was reminded that I don't want too many sedatives. You really have to be your own advocate with everyone so busy and yes, I may be a bit of a control freak.

Funny how this time reminds us of our travels to West Africa. We are in a hotel without Graham, eating only cooked food, and thinking about everything we touch.

Graham is doing well in his new school and says he could stay with Granny and Grampa for a year. He is having fun feeding the llamas, going to the sand dunes, a dairy farm, building great llego ships, soaking in the hot tub, and more. Can I come? The other night he told us that in his new school he doesn't have to clean his own plate after snack, that they have a bunny, a crab, and a fish, and that they have a maze like his school at home. We played simon says even though I can't see him and good old twenty questions where he changes his person, place, or thing half way through. He has no idea how much he makes us laugh.

OK, off to check in by 10 and saying good-bye to my wacky immune system....

Small Change in Plans

For some reason a bed didn't open up as it should have and they are going to delay the start of Jen's treatment by one day. She will still have the port put in today (waiting for that now), but won't start the HiCy until tomorrow.

Details

Today was a day that put us in much more positive spirits knowing that another Myositis patient has benefited from HiCy. I was also happy to hear that having visitors that are healthy is OK. Anyone thinking of suprising me- you must go through these questions.
Any live vaccines in the past 28 days (live flu?) Had chicken pox in the past 28 days? Had nausea, vomiting, diarrhea in the past week? Any runny nose, cough or cold? Cold sores, blisters? Skin rash?

I also was pleased to be given such thorough information slowly. We were given a large binder of everything to expect about getting chemo both in the hospital and out. - Treatment information, Managing Side Effects, Nutrition, Exercise, Coping, Care at Home, etc. There is some good info about what to do with certain symptoms, anemia, mouth care, taste changes, fatigue, naseau, food safety, etc. I will ask about getting a better oral thermometer. I don't trust the air flow with my digital ear one. Anything over 100.5 and you must contact the doc immediately.
We will wear N95 masks that have better filters in them than the regular ones.

What to pack:
clean clothes for each day- yeah don't have to wear a gown!
soft toothbrush and mild toothpast- flossing only if you do already
moisturizing soap
can't bring your own meds to the hospital- they will provide them with hospital pharmacy
no razors- you have to watch out for bleeding since your platelets that control clotting are wiped out

Meds:
I will get HiCy for 4 days and start tomorrow night.
Mesna will be given to protect the bladder wall from the side effects of HiCy.
After 4 days I will be given Norfloxacin, Fluconazole, Valacyclovir, Amoxicillin.
6 Days after HiCy I will be given the growth factor to speed along the "reboot."

I will do CK weekly to track and will hopefully do some physical therapy too if I am up to it.
I get line placed at 1pm and then we start with hydration. I will try to write or call in between bathroom runs....

First day in Baltimore

Jenny and I arrived in Baltimore today at 11:00. Parking was the typical dizzying experience of driving around those parking garages we used to run up for exercise in Indiana (few hills). But I dropped Jen off at the front door and eventually met her in the HIPOP center. A few minutes later they had drawn some blood from Jenny (about 10 vials) and we were talking with Dr. Hesdorffer in one of the examination rooms. Dr. Hesdorffer explained the procedure, which was exactly as Dr. Brodsky had described it, so no news there. But it was good to have a refresher.

The big news was that Dr. Hesdorffer knew of a guy from WA who had Myositis and came to JH for HiCy treatment! I don't know why no one mentioned this to us before. . . I could have sworn we specifically asked if there were examples of using HiCy to treat Myositis but maybe not. Anyway, this guy was in really bad shape, bound to a wheelchair and couldn't really feed himself. But 2 months after HiCy he was skiing black diamonds in Washington state! (Dr. Hesdorffer made it clear that he had known how to ski before getting Myositis!) So that was good news. Apparently this patient has been in remission for a year now.

We learned that the greatest risk of infection comes from Jen's own gut. Apparently there are bugs in there hanging out that get out of control when the HiCy supresses her immune system. That is why she will take 4 different antibiotics in about 4-5 days. It also means that although she needs to be careful about infection from others, the doctor wasn't AS concerned about it as we thought he would be. We did wear masks in the hospital, which is oddly comforting. We also learned when and where we were going to go tomorrow. At noon Jen will have the line put in and then will be transfered to the HiCy area (floor 4b) where she will begin hydration and eventually the chemo.

We then checked into our hotel, which is fine. We walked down to an indoor market that sold lots of different types of food. Jen got a salad, but I couldn't resist a phatty borrito. In a bit here she is going to wipe herself (whole body) down with sterilized wipes they gave us in the hospital. She will do that again tomorrow morning before going in to the hospital. It will be good to get this started after so much planning. Jen has been a trooper and, although nervous, is also looking forward to getting started.

Go Pats!

Evaluation Day 2

Everything went smoothly yesterday and today. Today I had the MUGA scan, sinus CT, and chest x-ray. I feel better knowing my way around now with registration, parking, and housing. Our housing is very close but I was a little wobbly walking on the uneven streets. The woman at the security desk asked if I wanted a wheelchair and I quickly replied, "No thanks, we are fighting that!" I will bring my walking stick during all of this since I figure if I'm not confident walking now, I will be less so when I am tired and weak.

Glad to find out that we can have our townhouse cleaned more than normal. They are used to this request with others having bone marrow treatments. I was also pleased to see how many hand-sanitizing dispensers there are around the hospital. They are everywhere!

I have started to mentally prepare for being like the other vulnerable folks stuck in hospital gowns and booties. Luckily it won't be long and I am used to it. I am hoping we can find some green areas to sit in but I have a feeling it will just be wishful thinking.

I popped into the blood center where I did pheresis because I wanted to give them a follow-up. I am not sure how much they know about their patients progress after they leave. Since not many Myositis patients get pheresed, I wanted them to know that it did get me out of a rough spot. The nurse agreed that being aggressive with this condition now is the right thing. She has seen so many patients who wait too long to take any serious action.

The hardest part of all of this was saying goodbye to Graham. I know he is happy with family and being in a new school for a month, but we may need to set up the video cam so I can actually see his silly self.

Thanks again for all the well wishes and packages!

Evaluation Day 1

Jen has finished her first day of evaluation. Today she did a pulmonary function test (passed), and EKG (passed), physical/family history/drug review (no red flags), blood was drawn, and urine was sampled. Jen also stocked up on parking coupons.

Tomorrow they will conduct a heart scan and CTs of Jen's sinus and chest. We also learned that the hotel we will be staying at will take mail for us. There is also a grocery store near by and a shuttle to get there.

Thank you to everyone who has been calling and sending best wishes. Keep checking the blog for updates!

Almost There

I saw my Myositis doctor the other day so she could have me fill out some paperwork, do a strength test, take some blood for research, and to answer any questions. We discussed that I did not try regular dose Cytoxan and I again told her I don't wish to. I have seen the severity of this and don't want to play around any more. I owe it to myself and my family to be knowledgable and agressive with this right now.
I was starting to think that I was improving when I noticed some little changes here and there. But after seeing the doctor, we agreed that yes, things are still in need of improvement.
I will go down to 15mg Prednisone pre-treatment. I will take an acid reducer during treatment to help the fact that I will be taking Prednisone with little food. I will take Diflucan now and during treatment to control any yeast issues. Rituxan does not interfere with treatment we were told.
The case manager reminded me of our status as pioneers, both in terms of some snags with insurance and in terms of the excitment for remission and future research. I don't want any praise, just some muscle will do.

Counting Down

The reality has hit and so has some underlying anxiety. I am staying mentally busy with my many lists of what to bring- I even went out and got antibacterial spray, hand-santizers etc. because they don't sell those in Baltimore=) Also trying to keep up with physical therapy since I am afraid of losing muscle during all of this. Doing reps of what is most difficult is the best thing to do- like lying flat on stomach to all fours over and over, getting out of chair, etc. Good news on that - PT folks were happy with my progress since they last saw me in August. Not sure if it was Rituxan kicking in or Plasmapheresis, but I did improve from the summer. They noticed my ability to get out of a chair better and my torso is not so far back when walking. So I have gotten to a more managable level, but not what I could be. I got some adjustable ankle arm weights even though gravity seems to be enough some days.

I will be seeing Dr. Christopher before I start treatment so she can get a baseline of strength, labs, etc. and track my progress.

Andrew videotaped me the other day and it was pretty hard to watch. I didn't realize how I throw my legs, how my hips move all over, and how contorted I move when trying to get up from the ground. It is better than it was, I must remember that. Never underestimate the strength of your big toe=)

Schedule

Well insurance cleared much faster than expected and the coordinators have been wonderful in trying to get me in so we can spend the holidays together.

We were given our housing options and will be staying at the McElderry townhouses which is in walking distance to IPOP.

We are not going to do evaluations locally since that will take up too much time waiting for results. The schedule for treatment is as follows:

Nov. 10 & 11 - Evaluations
Nov. 13- Sign Consents
Nov. 14- Line placed, start HiCy
Dec. 15- Hopefully home

Preparations

I got a call today from a Blood and Marrow Transplant Coordinator. (Reminder, I am not getting a transplant). I was relieved to finally get a call to move things forward. I will soon get a call from a case manager to discuss the insurance side.

We discussed what to expect with this treatment and what I will be doing in the upcoming weeks.

-I will hopefully be able to do some evaluations here with my local primary care physician. Things like Sinus CT, Chest X-Ray, MUGA/Cardiogram. I will fax the results/disc to them. They will need 4-5 days to review and then I will go to Hopkins for the rest of the testing.

-Hopkins testing will include one day of a physical, history, and bloodwork. Next day will be for me to sign consent forms, and the 3rd day I will have my line placed. I will start treatment the following day after the line is placed. I am glad that treatment does not start the same day the line is placed since last time it was pretty uncomfortable.

-HiCy will be given for 4 days and then I will spend 30 days coming in daily to their IPOP (Inpatient/Outpatient Center). I will soon be connected with a housing coordinator.

-I was reminded again about hair loss and that most everyone will lose their hair.

- I will need some kind of mouth care. The HiCy attacks any rapidly growing cells ex. hair, mouth, lining of the stomach. I will need a note from my dentist stating that I do not have any dental issues since any cavity/infection could spread throughout the body. Luckily, cavities are not much of a problem for me but I am due to go in for a cleaning before treatment begins.

-I was reminded that I am not to be on any blood thinning drugs for at least 7 days before treatment. I guess fish oil is included here as well.

-I will be connected with a social worker to assist with paying for meds and who knows what else.

-Infertility is not much of a risk for my age and for having just one treatment, but needed to be addressed.

I will get an email of everything that was discussed on the phone and then she will call me on Wednesday to figure out what evaluations I can do locally. We discussed scheduling a bit and I confirmed that I want to do this as soon as possible and preferably before the holidays. We are not so concerned about this interfering with the holidays since we will make our own holiday when we are all reunited again.

Waiting

We are waiting now for insurance to clear and for a case manager to contact us. We should hear something by the end of next week, hopefully. In the mean time we are preparing/researching things like housing in Baltimore, head scarves/hats, and gathering our books/movie lists.

I found out that the Tremont Park Hotel has studios and one bedroom suites that are reasonable with the Hopkins discount. I tried to work the AAA discount too but didn't have any luck. I think we may go with the one bedroom with a door, so I can sleep and Andrew can work.

On November 4th I will talk to my OGYN about doing the BRCA2 test that other family members have tested positive for. This is a genetic test that reveals whether I carry the gene that often leads to breast/ovarian cancer. Some people choose not to know this information, but with a young child at home, I feel it is best to know.

CK yesterday was 2920. Feeling OK, I still get slammed with fatigue in the middle of the day and have to rest. Weakness is most noticeable in the usual Myositis areas: getting up from chair, floor, stairs, rolling over, neck, raising arms overhead. What I cannot do: lift head from lying position, lift legs from sitting or lying position, lift arms when overhead from lying position, sit up from lying on back, hamstring curls. What I can do: although contorted, get up from a chair/floor/etc., slowly climb stairs with a railing, and walk, walk,walk. I used to be an athlete, a runner, biker, skier, hiker, practice yoga, pilates, etc. and all of that has helped me know my body and its potential. Walking, stretching, meditating, and using the therapy pool is key for the stiffness and key to calming the racing Prednisone mind.

It's crazy how comfortable we get with our limitations, accept it, and call it normal. So many of us forget what we used to be like and carry on. Although hard, we have to continually remember and strive to get it back!

A few additions

I thought I would add that I found Dr. Brodsky's discussion of the difference between HiCy and a Autologous (patient stem cell donor) or Allogeneic (second person donor) Hematopoietic stem cell transplantation very interesting. He made a very convincing case that HiCy was not only more effective but much safer. Autologous BMTs are problematic because some of those pesky white blood cells are always getting in there with the stem cells harvested from the patent. This means that the body doesn't forget that it thought Jenny's muscles were an enemy. Allogeneic BMTs are problematic in that they often cause graft-versus-host disease. The more I learn about HiCy the more I think this is the way autoimmune diseases will be treated in the future. All other drug options just treat the symptoms and attempt to slow the rate the immune system does damage. HiCy works by actively restarting the immune system.

Another item we learned is that Copaxone (question #7 below) is only used on MS patients and Dr. Brodsky didn't see any reason to use it on Jen. It would have meant daily injections following the HiCy so we didn't argue with him.

I know this procedure sounds horrendous but compared with what cancer patients go through this is going to be much easier on Jenny. The faster we can get Jenny off the drugs and into remission (no matter how short) the better.

The figure shows Jen's CPK (aka CK) numbers for the past 2 years. As I mentioned in my first blog this is a measure of the rate Jen's muscles are being broken down by her immune system. Normal is typically below 300.

Meeting Dr. Brodsky

The consult went well today at Hopkins with Dr. Brodsky. He was very knowledgeable, patient with my questions, open to ideas, and most importantly, confident about this treatment for autoimmune conditions. Whether or not my body stops attacking my muscles permanently is an unknown but something we will find out with time. Doing HiCy again is a possibility but that would only be if I was in remission for say 4 years, not if I flared again in a few months. Bottom line...50% or more of MS patients have improved from this and Dr. Brodsky didn't see any reason why I wouldn't follow this trend.

Other good news, with my age, 34, there isn't much risk to ovaries (5-10%). Other positive parts, were that if all goes well and with Dr. Christopher-Stine's OK, we could taper my dose of Prednisone following the HiCy treatment. There is more risk for me in terms of bone loss, cause for cancer, etc. with all the drugs I have been on than with this treatment. The mortality rate with HiCy is less than 1% (among 140 patients none have died), nothing more than what I have dealt with with already.

Good news on the family front, seeing Graham the first month I come home is fine and no problem. My immune system will be able to handle most normal things, but I heard that I have to stay away from people or babies that have just been vaccinated with live vaccines. All those vaccines I had for Africa travel will be long gone.

Also good news, it is OK to have raw fruits and veggies as long as they can be peeled- this means we can continue juicing instead of eating hospital/hotel food etc. OK, I probably won't be doing my normal wheatgrass shots right away, maybe Andrew will be able to bring in some real food. Also, catheter will be smaller than the pheresis one.

Dr. Brodsky went over what to expect: Hair loss, nausea, immunity will go to zero and will come back with the help of meds, and I will probably need a blood transfusion. I was aware of all of this. Hair is nothing in comparison to not moving, being nauseous I can handle, if I get a fever they will give me antibiotics, and I just have to accept the fact that I may need a transfusion. Also, we will have to stay in the Baltimore area for 3-4 weeks and found out that housing is not covered by insurance. Joyjoy.

We then went over polymyositis triggers and how it might affect this treatment if one of the triggers was still unidentified in my body. There are many viral and environmental triggers, and if there was an infection in the body which triggered all of this, I would have cleared it by now. Doing this HiCy would actually introduce tolerance for such infections, meaning if I still had some bacteria in my body my new immune system would be better able to fight it.

We did a strength test and he was surprised by my weak hip flexors and upper arm strength. I did well on balance! But I didn't have a moving child, toy, or dog to walk around=)

So now we wait for Dr. Brodsky to get the verbal OK from Dr. Stine and then we can set up an appointment to do some testing to make sure once again that we can go forward. ex. blood work, pulmonary function (which I have already done), heart/MUGA test, and probably more. So after that day, we set up a day for the line to be put in, and we start treatment. It did not sound like a lot to go through to get started. The longest wait may be for doctors to talk and for insurance to go through. He did mention that this protocol is used so much now that insurance is covering it with less hassle. He said we could possibly start in a month, which is what I would prefer. I am anxious to get this over with before the holidays and before flu season.

I will try to do some video of my graceful self so I can have a strength comparison.

Thank you all for your thoughts, wishes, and offers to help. We have such amazing family and friends and are so grateful.

Questions for Consult

Since I am a fan of asking too many questions... I know I will gather many more after Tuesday's consult, but here is the short list.

1. What other myositis patients, if any, do you know of that have done HiCy (Cyclophosphamide)?

2. How much will you and Dr. Christopher-Stine be talking during my treatment?

3. Will you be available throughout the treatment, when will you be away?

4. I know the risks for infection and damage to ovaries, but what are the percentages?

5. When would a blood transfusion be neccessary?

6. Do I continue with same amount of Prednisone throughout treatment? (I am at 20mg now. CK is 3137).

7. How does the drug Copaxone work in training the immune system to hopefully stay in remission?

8. If I have a history of cancer in my family, what are the risks for this treatment causing cancer?

9. Are there any neurological risks?

10. What precautions do we take for the first month home? When do you recommend re-vaccinating?

11. What are all the medications that I will be offered? How do they interact with one another?

If you have any others questions for me or for me to ask, please let me know.

Introduction

If all goes as planned, this blog will keep everyone informed of my wife Jen's successful treatment for Myositis. Myositis is an autoimmune disease where Jen's immune system mistakenly thinks her muscles are an infection. The symptoms are extreme fatigue and muscle loss. Untreated, Myositis would continue to weaken Jen to an extent we don't even want to talk about. For more on Myositis I recommend the Myositis Association's website and related links: http://www.myositis.org/

Jen's treatment started almost 2 years ago with prednisone, which continues today. She has gone through all the "standard" and "slightly more rare" drug options and has received the best care in the world from Dr. Christopher-Stine at Johns Hopkins Medical Center in Baltimore, MD. I can't go through all the details right now, but rest assured that we have tried everything. Jen's doctors are all great; Jen has just been resistant to every treatment. Her immune system continues to attack her muscles at almost the same rate it was 2 years ago. Her CPK numbers (Creatine kinase; a measure of muscle enzyme levels in her blood that indicate the level of ongoing muscle damage) have fluctuated some, but today are only about 50% of where they were 2 years ago. This despite some very strong immunosuppressant drugs. Jen can still walk, but watching her I am continually amazed at how little strength you need to walk once you are standing upright!

Earlier this summer we learned that Jen tested positive for the antibody SRP (Single Recognition Particle), which means she has "anti-SRP positive" Myositis. Folks with this usually get hit hard and fast and are usually much worse off than Jen was at the start. Anti-SRP positive Myositis patients usually respond well to steroids in the beginning and then nothing but Rituxan. Jen took Rituxan (even before the antibody test) but it hasn't done much good. Rituxan was supposed to be the magic drug, but Jen's immune system continues to be in high gear and the muscle weakness continues to progress.

So this brings us (very quickly) to the present. On Tuesday of next week we are going to Johns Hopkins to visit the famous Dr. Brodsky. Dr. Brodsky is famous for developing (with colleagues) a technique called high-dose cyclophosphamide (HiCy). This is a chemotherapy whereby the drug eliminates your entire immune system but leaves stem cells in your bone marrow and blood intact. Stem cells are apparently resistant to cyclophosphamide due to an enzyme that breaks the drug down before it can destroy the cell. These stem cells eventually (beginning about 7 days or so after treatment) "reboot" your immune system. The "new" immune system will hopefully forget that it used to think Jen's muscles were an enemy.

So, on Tuesday we hope to convince Dr. Brodsky that he should try this technique on Jen. As far as we know, no Myositis patient has ever received this treatment. However, Myositis patients have received bone marrow transplants and this is only slightly different (and possibly better.) I think the main reason no one has tried this treatment is that Myositis patients are usually responsive to one of the other treatment options. There are several blogs out there of MS patients who have received HiCy treatment from Dr. Brodsky. Please see Got HiCy? for more information. Also, Chris had MS (and links from there) has been a big help figuring this all out.