Bone Marow Biopsy and stuff...

We have to be at the hospital by 6 am for the 7:30 am bone marrow biopsy. Having had one before with only a little local freezing and going into shock afterwards, I was pretty stressed about having to experience that kind of torture again. However, my Hematologist is knocking me out completely. SO, the procedure will be not only painless during it, but the team of doctors at St. Michael's will take great care not to have me in pain (as it's a Masto trigger...)

I just talked to the Charge Nurse (who won't be there tomorrow) but she KNOWS about latex allergies (a friend of hers has one that is as bad as mine) and says she will flag my chart so NOTHING goes wrong. She asked me if I carried an Epi pen. "I carry 4," I told her. She told me to bring them. Just in case. They should be prepared but JUST in case...this might seem silly in a hospital, but after my last knee surgery in March in Parry Sound (the procedure was fine, but I didn't do so good coming out of anaesthetic and they weren't prepared...) I'm not willing to take any chances either. I'm glad they aren't either.

So, we're heading over to the inlaws for dinner shortly, and to drop the kids off. We have to leave here at about 3 am to get there for 6 am tomorrow. The Charge Nurse told me to make sure the nursing staff are aware and check my chart AND if anything looks even remotely suspicious in the waiting room or anything, to tell them and they'll move me to somewhere secure asap.

Feel like I'm in good hands, overall. It'll be good to get this c-kit mutation over and done with.

Yes it is...Yes, yes it is & Bone Marrow Biopsy

My Immunologist, in Barrie, sent me to St. Michael's Hospital in Toronto a few months ago. February, to be exact. Since then (yes, same calendar year) I have seen Dr. Peter Vadas (Dept. Head of Allergy and Immunology) several times, and he's referred to me to Dr. Lisa Hicks in Hematology who's now referred me to Anesthesiology where I had the pleasure of meeting Dr. Chen yesterday. (For those of you unfamiliar with Hematology, it's the study of blood which encompasses quite a lot, including cancer and (it seems) Systemic Mastocytosis. ) Dr. Vadas, Dr. Chen & Dr. Hicks are incredible doctors. Aware that they are not Gods but being as learned as possible, they are in pursuit of excellence in their respective fields, they are compassionate, and are trying to help me.

They are in agreement that another bone marrow biospy is needed to do the c-kit mutation testing. This is sort of the FINAL diagnostic test they have for SM right now. While a final diagnosis won't change the course of treatment that they have me on currently, it will offer some peace of mind that no-one's missed anything and that THIS is what is going on with the insanity that has become my body. I haven't mentioned much that they are also testing me for some other disease that is so rare that it doesn't really have a name but is referred to with a bunch of numbers and letters only. This disease is even rarer than Systemic Mastocytosis BUT is curable. No-one has mentioned what the cure is, but apparently it's curable.

If my c-kit mutation test reveals I DO have Systemic Mastocytosis then, the prognosis for cure is grim. However, life IS possible even in the face of feeling like absolute crap. Just have to avoid 'shocking' and anaphylaxis at all costs...easier said than done with triggers like latex, dust, dust mites (those things are EVERYWHERE!!!!), massive list of food that is growing steadily (all the tasty stuff too!), pain, hot, cold, extremes or quick changes in temperature, medications, perfumes, cleaners (we're a Dr. Bronner's Hemp soap, hydrogen peroxide, tea tree oil, lemon, vinegar and water household now), some animals and some silicone products., etc. Especially "etc.". The list is all together too big to note here right now.

If my c-kit mutation test reveals I DON'T have Systemic Mastocytosis, there is a fear that I have some "orphan" disease of my own...because SOMETHING isn't right. I've asked if it's possible that this is all in my head. Apparently, it's not. Not with what my body has been through and experiences. (So,...I'm NOT crazy?!?!!??!)

If my bone marrow biopsy reveals I have this other disease, there's a cure.

So, that's where I was at when we headed south to Toronto yesterday to the pre-op department at St. Michael's hospital. The real meal deal here is that there is a TEAM of doctors working together to help me at this hospital.

Dr. Chen opened the door with my file in hand, introduced himself and said he had read the lengthy file on me, and sat down. He looked at me with pity. Nice pity, if you know what I mean. And then told me that Systemic Mastocytosis is "life-changing". Er....yes, yes it is. I was confused. This doctor KNOWS what my life/our life has become.?! Different. Changed. Altered. Other. Uncomfortable. Painful. "You know Masto?" I asked, confused. Was I really not going to have to try and explain this disease to this doctor and hope that he would believe me that he really needs to call my Immunologist and speak to him about what's going on with me? "Horrendous disease," he quipped, "Life-changing." Er....yes, yes it is.

"I thought I was here to test to see if I have this disease, not that I have it." I replied.

He said he's not an Immunologist or a Hematologist but he's read the file, chances are good I will test positive but we'll see and that Dr. Hicks (it notes in the file) has already spoken to me about this. Yes, yes she has. He told me that Dr. Hicks is a wonderful doctor and she knows what she is doing. I agreed. He said her notes were pretty strong and we need to behave as IF I HAVE Systemic Mastocytosis for this test and anaesthetic because, chances are good this is what ails me,. So precautions, markers and preparations need to be made because this is rare, and serious. Yes, yes it is.

He then said it's an insane disease because it's one disease where getting upset can actually kill a person because sufficiently triggered, mast cells behave in these insane ways in a person with Systemic Mastocytosis. Add this, he went on, that for me, latex is a massive trigger, so that combined with getting stressed or upset can be detrimental in an O.R. SO, they are having me first thing in the morning and will prep the room without latex and let it sit and 'clean' overnight. First patient of the day in that room. SO, their job is to make me as comfortable as possible; I am not to be stoic and am not to permit myself to be in pain (they can help me) because pain is a trigger as the body has it's own stress-response to pain and THAT is massive in and of itself. And, bone marrow biopsies are painful. No two ways about it. Unlike other people who've had or can have lidocaine or local freezing to assist with the perception of that pain, I cannot. All terrible triggers for me. And, have hurt me/created reaction in me before. So, he said sedation and pain management post procedure were crucial to pulling through this well and without incident. At the end of the day, we want no incidence. Agreed.

It hadn't occurred to me that pain IS a trigger.

He said he had a good idea about what medications we could use and not use to sedate me but would check with Dr. Hicks/Dr. Vadas. I told him that Dr. Vadas has a list of medications not tolerated well by Masto. patients. The list comes from Michigan and Dr. Cem Atkin's office. I coudn't find my copy of it (think it might have got left with my Emerg. Room file or Patient File when I was in my local hospital last month for 4 days (well 6 days, if you count the two and half days in and out of Emerg.) So I pulled from the Mastocytosis Society in the USA's website Dr. Maria Castell's Emerg. Protocol for Systemic Mastocytosis and/or Mast Cell Disease and the printout on Anaesthetic on the same webpage. He asked if he could keep them. I said I'd brought them for him. The pages noted their sources. I was relieved he wanted them. He also told me that there's a good chance he won't be my actual Aneasthetist next Tuesday, June 9th, when I have this bone marrow biopsy done, BUT would write a detailed letter outlining what needs to happen with this/with me. He trusts his colleagues implicitly, he said.

It was so nice NOT to have to convince a doctor of the validity of this disease.

I feel so incredibly lucky to be living in Ontario right now, with this care team. St. Michael's Hospital and the University of Toronto have every right to be proud of three of their doctor's. They are doing their best for me. And, Systemic Mastocytosis. I appreciate it.

I was really worried about having this bone marrow biopsy because of what happened with the last one, in October, in Parry Sound. I've written about it in a previous post, so I'll spare you the details. But, I was not looking forward to this test without freezing or painkillers. Wasn't actually sure I would be able to go through with it. I don't say that about much in my life. Never have. But THAT was doing my head in. Dr. Hicks says it would be "inhummane" to do a bone marrow biopsy like that. Dr. Chen said he once had a patient, with Systemic Mastocytosis say that a bone marrow biopsy was like the torture machine on the movie The Princess Bride when Weslely is being, well, tortured and he lets out that primal scream that is the scream to end all screams because it's the cry of man who's lost his true love...THAT is the kind of pain a bone marrow biopsy without freezing, sedation or painkillers is. And even with sedation, Dr. Chen said it would hurt coming out of anaesthetic because there is no freezing in the area. He said that drawing out the bone marrow is like that Princess Bride scream because it's the "core of a person", just like the heart...to touch those things is to "mortally touch or wound a person" and it is excrutiating. So, it's going to hurt real bad afterwards so, I'm not to be stoic. No body or emotional stress over pain. He would order epi, benadryl, ranitidine and steroids on order for post procedure too. He knew I would need an iv line for all of that going in. I am to take my ketotifen, cromylyn and meds with the tiniest bit of water and let them do the rest.

So...that's the deal. I had intially thought going all the way down to Toronto for a pre-op would be a big waste of time and an emotional drain. It was anything BUT that. The nurses who did my physical were gentle, awesome and incredible (had to get nasal and anal swabs because I've been admitted to a Canadian hospital in the past year...swine & avian flu and SARS...thank you. The nurse gave me the swab and said I could go to the loo and do it myself if I wanted. Yes please. So no embarassment, discomfort or lack of privacy. Thank you.) I walked out of there with a sense of calm and trust. My husband remarked, "that went well." Yes it did. Aside from the anal swab. Yes, it did. Worth the trip.

C-kit Mutation testing et. al.

The call came yesterday. My Hematologist is going on mat. leave in early July (with twins, it turns out) and wants to do the c-kit mutation testing herself. Apparently, I'm an 'interesting' case...oh joy. So pre-op will be June 2nd. And they have to call back to confirm the June 9th procedure date. They need me in first thing in the morning for the procedure to make sure NO LATEX is in the operating room. I'm being put under for this procedure. With my history of shocking to local anaesthetic and the post-op hypothermia and anaphylaxis suffered in March with my right knee surgery...they don't want to take any risks.

I'm vaguely relieved that I won't have to be awake for another of these bone marrow tests. Equally relieved that my Hematologist/Oncologist (Dr. Lisa Hicks of St. Michael's Hospital in Toronto) thinks it's inhumane to do a bmb on someone without freezing or painkillers. She also wants a really good sample, so this is the best way to get it. We were hoping to co-ordinate my impending hysterectomy (not exactly thrilled about having to have this either...) with the bmb but Gynaecology at St. Mike's is booked up. The earliest consult I could get in for was June 22nd. It's gonna be a busy Toronto or St. Michael's month...

I'm scared to write the next bit. I want to jump for joy that it's been almost exactly a month since my last serious series of anaphylactic reactions (and subsequent 4 day stay in West Parry Sound Hospital - who totally didn't know how to handle me...sigh...) but I'm scared I might jinx myself. Everytime I've ever written that I've made it to such and such time landmark for no reactions, I have one within hours of writing it and then end up saying, "I spoke too soon..." So for now, I will write that I'm reserved about writing such things.

Overall, it's been a pretty good month. A few 'flare ups' we call them in Masto world. Not bad enough to be full anaphylaxis but enough to be uncomfortable, painful or debilitating. Had to use my new epi-spray (available over the counter in the USA but to be used with caution) about 4 times this month but it's better than using epi pen and calling in the big guns. The spray got me over the hump, enough time for drugs (Benadryl, Ranitidine, Reactine, Cromylyn, Ketotifen, etc.) to kick in and works REALLY quickly. So, I consider this a plus.

Have also kept myself on a super strict diet this month. This morning, I broke down and asked for a Tim Horton's French Vanilla, Half Decaf BUT my only deviation. It's been dairy free, mostly gluten free, meat free, cruciforous vegetable free, and been napping and resting sufficiently. Here I am with my period and doing ok so far. Usually I'm in hospital with my period because I flare up SUPER bad during this time of the month. Not unusual for Masto women, apparently.

Having said all that, I've not escaped the dire bone pain that comes with this disease. IF this is the disease I have. Ergo the c-kit mutation testing in the next 2 weeks. Dr. Vadas, my Immunologist (also at St. Michael's) and I have agreed that KNOWING for sure, is the best way to go. Because on the off chance this ISN'T SM...we need to know so that we can start looking elsewhere or realize that I'm the first of some sort of something else...however, I present like SM, so it's probably that. We'll see...In the meantime, I've been in incredible pain this month. I don't moan about it much (except to Antony) but it's bad. Night time is the worse. Sleep is...difficult.

My gut has been up and down too. But overall somewhat better. Could the Pantaloc be making some sort of difference? I'm on that now too since giving myself a hiatus hernia and stomach bleed through all the allergic coughing and puking.

I've been taking FLORADIX liquid iron and B-vitamin supplement too. It's the least aggressive of all iron supplements (in my humble opinion) for the body. There's NO constipation with it. It's made from natural sources, and doesn't taste too bad either. So, I'm wondering if this is making a difference in my overall energy levels? The dark rings under my eyes are improving. Hoping to have those mostly gone by the time my cousin Heather gets married. I'm supposed to be MC'ing the reception and I don't want to look like a bag of death. Am trying to keep myself as healthy and as strong as possible for this trip out West to Winnipeg at the end of June/beginning of July because this wedding is very important to all of us. As all family weddings should be. I don't want to let Heather and Julian down. That means taking precautions now, and then.

My hair continues to fall out. Anyone else suffering from this? I used to have such a thick and lucious head of hair. Now it's brittle and falling out far too much. Advice? Comments? Thanks.

It's nice to have a 'normal' sized tongue in my head for once. So for this, and not being in the hospital and flat on my back in bed ALL day, every day, I am utterly grateful. xoxoxoxo

Bone Marow Biopsy and stuff...

First, may I please ask that you continue to keep Dawn and Gavin in your thoughts, hearts, minds and prayers. Gavin's big sister, Chelsea is having an understandably hard time with her Mom being at the hospital this past two weeks and wants them all to be home together. Dawn...I just want to wrap Dawn and her family in a big love blanket, take away all this health crap and enjoy a 'normal' day together...one day....

Also, one of the most amazing people I know and love is coming to terms with her father's suicide/death right now and, while I can't possibly know what it's like to lose my parents (her Mom died of cancer a scant couple of years ago...), I want to ease her pain and suffering and loss. Drea, I love you. xoxooxoxo

It's really odd when you become your own administrator. Two weeks ago I saw Dr. Vadas in Toronto, and he says that he is not in receipt of the abnormal tryptase/24 hr catecholamine (elevated levels of histimine) results that were done in Winnipeg by Dr. Hicks's Immunology office. Weird. All my other stuff had been forwarded. It has taken me two weeks to get a hold of an actual person...and they wouldn't forwarded those results to Dr. Vadas because I have to sign a medical release form, apparently. I've signed one for Dr. Fischer's Immunology office in Barrie...so I asked if they could send it there. I called Jane, Dr. Fischer's INCREDIBLE administrator, and she said she'd watch for it, and send it on immediately to Dr. Vadas. Once again, she had my file on her desk. She says I'm a 'special' patient. We joked, NOT the kind of 'special' a person really wants to be. So...FINALLY, that should be sent on...

I see Dr. Vadas again tomorrow. I've had one Epi-breakthrough reaction in the past two weeks, and days of facial and throat swelling. This subsided yesterday FINALLY. I feel almost human today...which is amazing!!!!

Bone marrow biopsy with another Dr. Hicks in Toronto, at St. Michael's tomorrow morning. This time for the c-kit mutation. No-one has told me whether I need to be on or off meds for it; no-one has told me anything except they know about my severe latex allergy and to show up. I'll need to tell them I can't have lidocaine or xylocaine or painkillers...as I go into shock. Mind you, I guess if that happens, I'm in the right place???? Ugh, NOT looking forward to this. I have managed, pretty much to put it out of my mind. But, it's looming, getting closer....I don't ask for people to think of me very often, but please, if you could tomorrow morning...that would be grand. I'm scared. This test hurts soooooooooooooooooo bad; and is super unpleasant (excrutiating) and...I wouldn't wish this on anyone.

Systemic Mastocytosis

I saw Dr. Vadas in Toronto yesterday. If I may comment, he's compassionate, thorough, informed, caring and patient. He specializes in idiopathic anaphylaxis and is Head of Immunology & Allergy at St. Michael's Hospital in Toronto. I was there for almost 3 hours with him. He says he's 99% sure I have SM but the only way to tell for sure is to do the c-kit mutation test...which is another bone marrow biopsy. He thinks I've been through enough already, including a bone marrow biopsy (bmb), and it can wait because quite frankly the only thing it would do is tell us is what we think we already know and he said it would be sort of like just finding the answer and closing the question door.

I told him that I just want my quality of life back and to be well. He laughed nervously and then leaned forward and said to me, "Fiona, dear, THIS IS your life. This is it. All we can do is try to make you comfortable. There is no cure for this disease at this stage, we're looking, but we have to try to minimize the anaphylaxis reactions and keep you comfortable. So we are going to try a combination of drugs..." Plus, I have to do all the things I'm already doing.

So, I'm on Gastrocom now. On top of everything I already have. And, am to continue following protocol for the anaphylaxis when it hits. And to continue to be isolated; keep safe. He says it's a good job I'm already on Long Term Disability because I need it.

He says there are three types of this disease. The leukemia kind; the kind where you have a positive bmb and uticaria pigmentosa, itching, etc.; and the kind that I have with no positive regular bmb (because it's not about having TOO MANY mast cells, so much as mast cells that don't behave properly) repeated anaphylaxis, skin flareups, digestive disorders, severe osteo-arthritis (which my orthopedic surgeon found last week when he did my knee surgery), joint swelling and pain; bone pain, flushing, itching, severe fatigue, etc. And then there's the day to day feeling like shit that comes with all of this. We talked about that too.

I hadn't realized until recently HOW itchy I am a lot of the time. Not all of the time though. I think I've spent an inordinate amount of time being 'out' of my body so that I can't feel it. Because when I permit myself to be REALLY present in this skin, I'm itchy. Especially during flareups. Dr. Vadas says I'm not crazy that the shower totally aggravates this too!

We talked a LOT about triggers for me. Heat and cold, especially the extremes. We've ascertained that it's definitely aggravating or causing flareups. We had a joke about living where we live, and how not good for this disease it is. But, this is where we live...

Then there's foods, alcohol, drugs, emotions, latex, chemical sensitivities, bees/wasps...we talked about that too. I'm not to get any venomous snakes any time soon.

He gave me some handouts on Dr. Cem Atkin's work on this indolent form of Masto. He told me it's a super rare disease and that it will take some time for my primary care providers up here in Parry Sound to start understanding what I'm dealing with. He thinks, like I said, that doing another bmb (especially because I go into shock with lidocaine and xylocaine, so I can't have any freezing for it) wouldn't change how we manage me, because it's obvious that I have some sort of mast cell degranulation issues. So, we are going to assume it's SM because, as I mentioned, the treatment and management are the same. All my other tests results and my clinical history point in the SM direction. Glaringly so.

I go back to see him in 6 weeks, am to ring him if I have anaphylaxis in the meantime, and we'll talk about the bmb then. Like I mentioned, he said it's more a question of "I have a final answer", as a patient, that will change for me with the bmb, not a change in management. He thinks it's too much pain and personal suffering for a final answer when we have this much info. already. And, he urged me to come to terms with this disease. It's real. It affects me. It's changed my life. It can kill me. I'm not crazy. It's time to start living WITH this disease instead of hoping it will go away, because it won't. There is no evidence to suggest that anyone has spontaneously corrected themselves, and there's no cure.

So, that's 4 doctors/specialists who think I have SM; and 1 who doesn't (she was only looking for the leukemic form of the disease.)

Ironically, stress and emotional upset/joy can cause flareups too. So, Ican't even get upset about all this! ha ha sniffle ha ha.

Bone Marrow Biopsy

It's been a while since I've posted. Haven't been feeling so hot.

Had a bone marrow biopsy done yesterday. The nurse told me right before, "don't worry, it's not as bad as people say" and then following the procedure said to me (with tears in her eyes), "I'm so sorry, I feel like I've lied to you, that was awful for you..." Apparently not all bone marrow biopsies go this badly. Although the doctor got what he needed, so it was ok by all counts. Hope I don't have to do this again. I'd be a liar if I said it didn't hurt like hell. My husband said hearing my muffled (face in the pillow) screams down the hall was really hard.

For me the worst is over. The test itself was the thing I was worried about the most. Not the results.

Will I/Do I have excessive numbers of mast cells in my bone marrow? I don't really care. I know how I feel.