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.
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.
4 comments:
Really appreciate all you are going through! If not SM, what about Idiopathic Anaphylaxis? Good luck!
I intially went to see Dr. Vadas for IA, but he thinks it's mast cell related. But who knows, it could totally be IA. And then what???
Thanks. It helps to know we aren't floundering out here in left field all alone, eh? Thank GOD for the internet...in this respect.
Sweetie - according to Dr. Castells - they are close to a cure. She thinks they will figure this horrible disease in our lifetime.
If you can get to Boston - see her. I really think she could give you some peace of mind.
Just want to add that I'm so happy you were treated so well! You deserve it so very much after all you have been through. I hope it continues!!!!
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