music diane is such a great person the most selfless person i've even met in my entire life nurse talking never complains never not once did she ever complain the words of quit fail and can't are not in her vocabulary well
she is probably the strongest and one of the biggest fighters that i know in when we now when we found out it really um shook us all up because di isn't just my sister my best friend she's the family rock story begins with purple eyes my family are hockey players so most people most people just assumed i took a hockey puck in the eye but a biopsy showed i had a very rare non-curable disease hello and welcome to themassachusetts school of law educational forum produced by themassachusetts school of law at andover
i'm diane sullivan you're host for today's program being irish i have the curse of very fair skin so each year i visit my skin doctor doctor finkel was concerned over my bruised eyelids but i explained to him they had been previously checked and a biopsy showed nothing abnormalthis is just how they are i said no diane said dr. finkel, this is not normalthe biopsy showed nothing i responded again sometimes he said it depends on who reads ita few days later doctor finkel was on the phone it was then i heardabout the disease amyloidosis
the first time and i quickly learned this was not good though the amyloidosis diseases are actually a set of diseasesthat can be blood or bone marrow disorders in themost common form but there are also genetic diseases and diseases that occursecondary to other conditions like chronic infections and chronicinflammatory diseases so they really span a broad spectrum of medical care and in fact we've developed a multi-disciplinary program to take care ofpatients with amyloidosis but my interest is in what really is themost common type
amyloidosis in the united states withwhich is al amyloidosis also known as primary amyloidosis and the same appliesto doctor sanchorawala so we are hematologists andbecause that particular form is a blood disorderover many decades research here and elsewherewe learn that these diseases can be treated with chemotherapy to affect cells in the bonemarrow and we consider the disease to be acousin of a more common blood disorder multiple myeloma so ourtreatment approaches are
based on once used for myeloma and overthe years we've developed a program here that includes chemotherapy and stemcell transplantation as a major modality for treatment for mechemotherapy proved unsuccessful so after careful thought and deliberation upon my doctor's recommendation i decidedto undergo a stem cell transplant since it provided the best chanceof a durable remission stem cell transplantation can be usedfor many many hematological diseases as well as some autoimmune diseases um the stem cells
are parents which reside in everybody'sbone marrow we stimulate them so they come out fromthe bone marrow into peripheral blood and we can harvestthem from the peripheral blood we can also harvest them from directlyfrom the bone marrow and that is known as bone marrowtransplantation when the harvest them from theperipheral blood it is known as peripheral blood stem celltransplantation the donor can be either a patient or a sibling or a relative or a donorfrom the national marrow registry
when donor is not the patient himself it is known as allogeneic stem celltransplantation when the donor is patient himself it isknown as autologous stem cell transplantation the disease that we are very very expert in here at boston medicalcenter is amyloidosis and we usually use peripheralblood autologous stem cell transplantation thedonor in that case its patient himself or herself thepurpose of stem cell transplantation is to able to deliver high doses ofchemotherapy
safely and effectively and the use of peripheral blood stem celltransplantation makes delivery of high doses of chemotherapysafe and possible you know she's really the solid personthat we all depend on so when we found out she was sickit really just rocked all our worlds to know that she's sick and we know that she's veryindependent and she doesn't like to ask for help or ever expect anybody
to give she's she the giver notreceivers so we know that we were gonna have to arguewith her fight with her just do have her allow us to help her she no independent and it's a struggle to tell her di we're here foryou you know she knows we love her and thatshe's our world but she doesn't like to take the help she likes to give the help so we know that that would be astruggle with her just having allow us in to help her out
so it was it was tough i was working at the prison which isa stressful job in itself and then with that stress on on top of it knowing my sister was sick it it did a lot to me you know i got really stressed and depressed in it was a battle psychologicallyworking through you know my sister has this disease that you know taker the first process thefirst ultimately could you know take her so the first process, the first phase of the treatment isevaluation making sure that the patient
is eligible to receive high doses ofchemotherapy and stem cell transplantation once the patient is eligible deemedeligible to receive this aggressive form of treatment then thenext step is to make sure that patient undergoes a catheter placement which is usuallya big line that is placed in the major vesselof the chest which is close to the heartand
then the process of stem cellmobilization begins which involves administration of agranulocyte colony-stimulating factor which is a cytokine the kind which is usedto stimulate stem cells which are presentin the bone marrow the stimulation of stem cells in thebone marrow leads to you know moving the stem cells fromthe bone marrow into the peripheral blood the patient'sthen undergo local leukapherisis or harvesting of the stem cells which isdone usually in the clinic done over 1 to 3 days through a
line which is pleased in the chest anda usual dose of stem cells that is required isabout 2.5 to five times million stem cells perkilogram and once the stem cells are harvestedthey are frozen in the blood bank placed in liquidnitrogen and get viable and alive for about 10years after harvesting of stem cells is done following this phase of treatment patients receivehigh doses of chemotherapy and stem cells are given back to thepatient which are
in the freezer of the blood bank abouttwenty-four to forty-eight hours after completion of chemotherapythe third phase of treatment is the period of myeloablation or bonemarrow suppression and the side effects from chemotherapywhich can last from anywhere from seven to 10 to 14 days thestem cell start to regenerate and produce new cells and this is called engraftment phase and that is when patients start to feel betterthe discharge from the program is usually one to three weeks after stem cells havebeen grafted
and the blood counts have returned to normal i'm a very type a personality and ialmost had to create a separate calendar that just put everything together and iknow that sounds almost a little bit inhuman that it wasn't feeling but i had to put together where i needed to be andwhat times for my sister but then also for otherfolks because this is like you know i watched my parents go through cancer treatments and chemo treatments and everything that goes with that and it isoverwhelming
um this was just entirely somethingdifferent cuz for the period when she went in for for stem cell it was a matter ofdiane needed 24/7 support all the time and it had to be someone and realistically even ifi didn't have this job that i have and if i didn't have my familycommitment it's not a one-person job so between a group us coordinating i put together a calendar that had alldiane's appointments in it had days which marked what pill she needto take all the
all the different things that went intoit but then also who's gonna be there what time relief was coming and whenpeople were switching this kind of sounds crazy but i it's like i scheduled in sleeping um so for me that was the hardest partwas just coordinating all of the effort initially in 1994 westarted the program as an inpatient program in 1996 we moved the program tooutpatient setting when the patient is in the hospital it is you know you spend about six to eightweeks maybe even four to six weeks in
the hospital and you are in a private room isolatedfrom rest of the patients and it is very veryhard for somebody's psyche to really really you know undergo a massive treatment withno support or limited support from familymembers and nurses and their you know other coworkers however and i thinkthe risk of infections also in the hospital
are significantly higher when we movedthe program to outpatient setting we were a bit courageous at that pointhowever we found that you know the patients really did extremely wellmajority of the patients start the program as outpatient and they day do end up in the hospital eitherduring their period of low blood counts because of a feverinfection dehydration or bleeding complications but the length of stay is usually onlyfour to seven days and i think that really truly helpsthe patient's
and you know they are in their own homesetting as well as you know with their own family members and we seethem in the day hospital which is you knowthey spend the whole day in the clinic or the day hospital from eighto'clock in the morning til 5 o'clock we could provide intravenousfluids blood transfusions platelet transfusions antibiotics and other symptom management in the dayhospital and i think you know we take great pridein actually having this as outpatientbecause i think the patients
do much better and as far as even deconditioning is concerned that tends to happen in the hospital in she's going in to um have her stem cellsharvested and there's this hurricane that supposed tohappen and i'm a little concerned because i'mnot really a big city driver but you know she'sgonna take me through and show me the way toget to the hospital she still in horrific pain early morning we leave really early in the morning youknow they're talking about
maybe shutting down boston for for theday but i'm not telling her what myfears are we just get to the hospital she got a little sick along the waydropped her off at the hospital parked the car met her inside they didtheir thing with her as the day progressed i'm looking outthe window and i'm seeing the wind just picking up she's inthere a little longer and a little longer it's getting to be 3 o'clock and i'm lookingoutside i'm seeing things flying outside the window
twirling outside the window and i see a few less people outside walkingand now i'm thinking okay i'm gonna be driving over the zacim bridge i am deathly afraid of bridges and ihave like panic attacks going over a bridge ingeneral but now it's we we're having thishurricane so get on the road she tells me where togo which is good she's very slouched overshe's just not feeling well but she's keeping it up for me because she
gotta show me how to get to her houseall of a sudden here we are at the entrance of the zakim bridge and i'm holding on to the wheel with both hands and my car's going this way and then it'sgoing that way and i'm like oh it's a little windy out herediane no big deal yup we can do this it's no big deal and there's a truck huge truckbehind me and just about go over on his side ofthe road but i i said anything but going over the water or over the other side of thewater
i'm like oh my god how am i gonna do this sofinally we get through that bridge and then isee things flying in front of me poor diane is sick as adog and we're on this highway i'm not veryfamiliar with and i see tree limbs flying in front of melike the wizard of oz we finally make it tothe house after this whole big ordeal i'm gonna continue going home and i'm driving home it was horrible igot home and i immediately text her i said heythelma
how are you doing thelma she immediatelytext me back i'm doing really great louise so the chemotherapy that accompanies thestem cell transplant is usually given massive amounts massive doses meaningthat this chemotherapy is hopefully able to ablate and eradicate any microtumor cells or any small number of tumor cells orsmall number of disease cells and the doses ofchemotherapy are very very high and the complications are associated with
the aggressiveness of the high dosesof chemotherapy and the complications are starting fromfatigue hair loss nausea vomiting mouth sores low blood counts requiring blood and platelet transfusions immunesuppression leading to infections fevers bleeding loss of appetite obviouslyinfertility and things like that but the you knowthat is the mainstay of treatment is the high doses of chemotherapy and ithink the stem cells
they make it possible for the deliveryof high-doses of chemotherapy day one you will come in as you did previously we draw on your blood through your central line just to check your blood count to make sure the white cells okay nothing has changed your potassium your electrolytes are okay after that we'll start the pre-hydration thatwill run for about two hours probably about a half an hour before fluid is to end i will come in and i will give youantiemetics
usually thats kytril decadron and atavin and those are used to prevent nausea during the chemo about 15 minutes before the chemotherapy is to start i'll be giving you ice what you'll have to do is pack your mouth with ice 15 minutes before during thechemotherapy a mouthful which will one about 15-20min and after the chemotherapy which will be about 15 minutes so altogether you're looking about anhour-and-a-half the purpose of the ice is to decrease the blood flow to the mouthbecause we don't
really need the chemo going there and what that's going to do is cut down on the blood supply there and therefore you won't have mouth sores because if you are to get mouth sores that couldbe devastating you could end up your mouth cavity from your mouth oral cavity all the way down your um digestive system your mucosa and once that becomes compromised you could bleed you could have a lot of pain end upin the hospital you could actually end up on a morphine drip because you won't be able to swallow and it will be very very painful
so the night before she called me andsaid you know mike this is it i i can't go through with the treatment i can't go through with the process and you know i didn't sleep that night i wasnauseous at home with my wife i remember laying inbed just thinking this is is it's not gonna happen umm but you had to get through it and so for her itwas the biggest obstacle for me that second day having her not see me lose it like i am about tohere well it was it was the worst thing havingwatched you know my parents go through
chemo it just words can't explain how tough it was but to her credit she got through it we survived and i sure as hell hope we don't ever have todo it again i'll give you an idea what's gonna happen so obviously the room is going to be busy and crowded you''l have cynthia come in she's your one to one nurse and she's going to sit over here and put you on the monitor the monitor allows us to watch your oxygen levels it allows us to watch your heart rate and your heart rhythm and it allows us to monitor your blood pressure
along the way all of those things can be affected by the dmso that your stem cells have been preserved in so we really want to monitor them carefully the other thing is that we give you premedication as we did when you had the high dose malfulin but some of those medications make you a little bit sleepy on when you're a little bit sleepy you don't take such deep breaths and your oxygen levels may drop so don't and this is for you too so you know don't be fearful if we
wake you up and say you know dianeoxygen we're going to put a little oxygen on you it doesn't mean that anything is going wrongokay it just means you're sleepy not takingdeep breaths we're either going to poke you and keep telling you to do sookay or we're just going to give you a little bit of oxygen so we can let you sleep we've already started iv fluids so you get pre-hydration and then afterwards you'll getpost hydration in a little while the blood bank will come up and they're gonna come up with a bath that kind of warms up the stem cells so they're still frozen right to the minute they come into the room for you we're gonna give you all of the bags back you collected five bags
or you know we process them in five bags you collected 9 million cells per kilogram so thats a really good yeah that's a really good collection so what the blood bank will do is will thaw out your bag one bag at a time they'll thaw it out to your body's temperature because you don't want anything super cold and you certainly don't want anything super hot right as they thaw it out one bag at a time then they'll hand it off to me we do double checks so we'll talk toeach other about
the bag the volume the amount of stem cells who you are and when your birthday so often you'll hear me saying even though you know i know you'll hear me saying diane what's your birthday okay okay its not because i don't remember it's because we're doing the double check so that will happen for each bag okay and when we're halfway done with one bag they'll start warming up the next bag you'll probably be sleepy through this okay you already had some benadryl prior to the
chemotherapy and it really didn't make you fidgety or anxious which sometimes it does so i anticipate for you that your going to be more sleepy than anything okay alright but easily i'll be able to wake you up and talk to you okay if you wanna stay awake during the process and you want to ask questions you're more than welcome to but don't fight the sleep okay okay one thing i'm certain theaudience dr seldin is thinking if patients are ill with some abnormal protein in their blood and you do a stem cell transplant whereyou're freezing that person's blood
how is it that when you up put thecells back in the patient the disease doesn't just regrow that's a great question but it seems like when we collect the cells which doctorsanchorawala described with mobilization process we get healthy cells and the abnormal cells that areresponsible for the disease are for the most part left behind in thebone marrow and then they get taken care of by thechemotherapy that the patient goes through the cells that are put back into thepatient as part of the transplant
appear to be healthy cells and in thebest of cases don't provide any problems in the future staying at the hotel she did good we were in two rooms and iknow when i'm sick i don't want people around and at theworst part of her life you know getting sick you know not wantingpeople to see her the way she was she really sucked it up she did a phenomenal job she you know talked a lot when we weretogether
about her two nephews ryan and corey which i am so into that with her becausethey're also my nephews we share the same two kids that we watched from the time they were born till you know present time now we did a lot of talking a lot of laughing we did some crying so boy did we do somereminiscing and i think a lot of that lifted her up are up she would just gofrom you know you could see her being tiredand she would all of a sudden talk about cory and ryan and she would be all wide awake with it
we had that we had a good thing to talkabout things you know like her parents you know different things her brothermichael and this is funny and it will bring you into the part where michael helped her with the ice chipswhen i first met michael i remember pulling into the driveway at their house on appleton circle the lamy house and i looked up and i thought i was seeingthings i saw batman on the roof well it was michael
so when michael helped diane through the ice chip situation where she had to hold these ice chips in our mouth we figured ok this is where super hero comes into play and it brought me back to when michaelwas just this little kid out on a roof dressed as batman so you know we did a lot of talking about family things and through theyears and and like i said it was always a good notediane hit some pretty sick times every single day we gotback and i would watch her
just getting into bed to take thatlittle snooze before we would do our walk every day it was like i saw a differentpart of her it would change it was just it took its toll every daysaw it it was a little bit more a little bit more and and it got a little hard for me emotionally and and i kinda hid it from her it brought me back to when diane'sfather had cancer and towards the end of jack
i'm going to cry we kept jack home as long as we couldhe had hairy cell leukemia which is rare and it was tough i did his dressing changes and this was a man this will go to show you how diane's like her father he would be so sick and i would go over to do his dressingchanges in the morning before work and then wheni get out of work and he would call me at work every singleday to see what i wanted i wanted for supper he'd give me a you know ia handful ofthings to pick
as sick as he he still made me dinnerand i would have dinner with him every night this is a man this is where i look at dd and see herfather the words of quit fail and can't are not in theirvocabulary they're very uh a wonderful familythey're very strong great family values and just seeing dd go through
things that we would have seen towards the end of her treatment while we were staying in boston it did get emotional it did bringback a lot about about her dad but nothing stops her she's like like the most driven woman i have ever met in my entire life what are the advantages on using somebody's own cells to do thetransplant versus having a relative or a sibling donate theirtheir
their stem cells the advantages ofautologous stem cell transplantation meaning that the patient is the donor are using their own stem cells is a rapid engraftment meaning that the period of of low blood counts is only for aboutseven to 10 days whereas if stem cells are used for from another person or a donor thenthe then the period of myeloablation or period of low blood count is for almost 20 to 30 days furthermore
i think when you receive blood stem cells or stem cells from a donor there's always a risk ofgraft-versus-host disease meaning that you're immune system yourbody is not able to work with the stem cells from a donor so the graft versus hostdisease is a major complication of allogeneic stem cell transplantation which is usually not the case inpatients who received their own stem cells are there any major disadvantages tousing someone's own stem cells
well the disadvantages would be that thedisease is not that you are infusing stem cellsfrom a patient which are contaminated stem cells withtumor cells or contaminated with the same proteinproducing cells so i think that is the majordisadvantage that there is you know and that's moreof a problem for other diseases that would be my next question i'mcertain it would depend on the type the disease somebody suffering from as you choose what is thevery best type of
stem cell transplant there are blooddisorders and some types of leukemia where stem cells are actually the problem and giving back the same stem cells wouldnot be a good treatment and we and then we definitely go to look for other donors and what about ifthe ill person is a child would you still consider using their own stemcells are do you look for a donor children actually are very goodcandidates for transplants and they in many cases can even get a transplantfrom a parent children are more tolerant to foreign stemcells
then adults are so they have a lowerincidence of the complications of graft rejectiongraft-versus-host disease so although kids tend to have good responses to chemotherapy for blood diseases they also are very good candidates fortransplant from other donors dr. seldin i wonder ifyou might comment on the ice treatment that accompaniesthe chemotherapy that most patients arehorrified of so thats an interesting question so in medicallingo we call it cryotherapy which means
freezing your mouth basically and itturns out that if you do that you prevent blood flow tothe mouth and chemotherapy doesn't get into the mouth and you have a much lower incidence of mouth sores so its probably the cheapest and most effective thing in all medicine cheaper thantaking couple of tylenol or anything in terms ofwhat helps patients get through the side effects of these kinds of treatment because the alternative would be mouth sores right all kinds of complications and very painful people
have trouble eating people gethospitalized and at least that complication from their transplant process is pretty much behindus and people are much better from our point of view grateful when it's over true dr seldin talk about some ofthe clinical trials that you're using here at boston medical you know one of the things that's been atremendous advance in the field and we've gone from a period where the the only treatment we had waslow-dose oral chemotherapy which was
minimally effective for this disease as we talked about beginning in themid-nineteen ninety's we started high-dose chemotherapy and stem celltransplant and gotten much better results in termshematological remissions and actual improvement in the function oforgans we were finally able to see that kidneysand hearts and livers and could heal up from damage caused by the amyloidosis the protein depositiondisease however as doctor sanchorawala had
mentioned the patients have to be selectedvery carefully to go through aggressive treatment they have to have good cardiac function other parameters that we look at veryclosely because we don't wanna take patients through atherapy that's gonna be highly morbid or have a high mortality rate so only less than half the patients we seeactually are candidates for that and we've been very pleased that in thelast 8 to 10 years a number of additionaldrugs have come along for treatment of plasma cell diseases like amyloidosis and myeloma
and we've been able to taketremendous advantage of that for our patients offer them kind of a menu options and really tailor the therapy to the patient in a very personalizedindividualized based on their condition their symptoms their preferences for treatment and through trials to test those drugs we'vereally we've been able to identify the right doses to use safe doses and
combinations of drugs that work best for our patients it's wonderful that at least the patienthas some input into to the type of treatment that they're ultimately goingto undergo you know i think you know the reason that we focus a lot onclinical trials is it's really a way to test and look for more effectivetreatments for disease we greatly appreciate patientsparticipation in clinical trials we don't want patients to fear clinicaltrials they're not getting things that have not beenextensively tested
in other settings and animal and mostlikely other patients and clinical trials thesedays are very very rarely making use of a placebo so almost everybody that gets treated on a clinical trial at ourcenter for this disease or other diseases is almost always getting some formeffective treatment it's not that patients are getting sugar pills or waterinfusions for treatment but it really is a way forpatients themselves to get new treatments and also
really to benefit others and help uslearn what the best treatments are for patients diseases you are both involved in a studythat was done here at boston medical center on 421 patients who underwent the stem cell transplant combined withthe high dose chemotherapy i wonder if you would share what youthink are some of the pertinent findings of that study that studyactually you know and all patients from 99 ona different clinical trials in all it was a
combination of different clinical trialswhere four hundred and twenty-one patients were in enrolled from 1994 to 2011 and these patients were treated withwith amyloidosis were treated with high-dose chemotherapy and stem celltransplantation and what we found was that the responserates complete hematologic response rates were about forty percent about thirty to forty percent ofpatients received a partial hematologic response in addition to complete metalogicresponse and the survival
was significantly better then you know what used to be in the past of about allsix years or so and majority of these patients who haveachieved remission remained in remission with very very smallnumber of patients relapsing even you know as long as 12 years laterjust a wonderful contribution doctor seldin i wonder if you mightexplain the disease of amyloidosis and how it goes to different organs and your findings in the study withrespect organ response so that's the big challenge for patientswith amyloidosis not only do they have a
blood disorder but they actually end up with impaired organ functionbecause proteins produced in the bonemarrow circulate through the blood and get deposited in organs and damage them and that generally is what leads to the diagnosis because the patient is beginning to develop develop symptoms of heart failure or kidney failure or liver problems or neurologic problemsand that that's kind what brings them into thedoctor and generally how the disease is
found when we take the patients through stemcell transplant or other treatment for these diseases weretrying to kill off the factory inside the bone marrow and we're hoping and expecting thatorgans will respond and heal up afterwards and if the disease is diagnosed early enoughthat is the case patients can go back to normal organfunction normal activities and a normal life after that treatment one of our
priorities one of our goals and one of the reasons we appreciate your programmingabout this is that it's extremely important that patients get diagnosed early so doctor sanchorewala i and othermembers of our program travel around the country we talk todoctors we talk to patient groups and we really try to promote early diagnosis so that patients have options for treatment before they suffer from organ failure due to proteins accumulating
in the organs you must then see patients that are so advanced in the disease that it limitstheir options that is the case sometimes patients cometo us where their options are very limited we actually have a very innovativeprogram with colleagues massachusetts general hospital wherevery selected patients can actually be considered for heart transplantation if their hearts are badly damaged by the disease and if that works out they can come back to us for a stem cell
transplantation afterwards so its a very effective collaboration between our twohospitals we talk every week we send patients backand forth again try to help patients get to thebest treatment for their disease how many stem cell transplants does boston medical for example do over the course of a year or a weekhowever you wish to answer that so you know boston medical centeryou know the program has been in existence since 1994
and we have done about forty toforty-five stem cell transplants per year here onaverage in 1997 which was the highest number ofstem cell transplant which was 56 all patients we had done and majorityof the stem cell transplants are done for the disease al amyloidosis eighty percent of the stem celltransplants are for al amyloidosis and 20 percent are for other diseaseslike lymphoma multiple myeloma hodgkin lymphoma things like tell us a little bit aboutyour medical team
those that work with you on thistremendous undertaking there'll many many individuals who arededicated and involved in taking a patient through stem cell transplant the multidisciplinary evaluation that occurs in the initial phases oftreatment involve an internist who is dedicatedto amyloidosis center a hematologist which involves myself doctor seldindoctor sloan a pulminologist cardiologist nephrologist which is arenal specialist
and a neurologist then the patientundergoes evaluation by a nurse practitioner who works with the stem celltransplant program who's on my team once the patient isdeemed eligible for stem cell transplant and once they return for stem celltransplant many many individuals who are you know helping us getting a patient through stem cell transplant that starts from medical assistant to many many nurses
who work in the solomon center forcancer and hematology blood disorders anutritionist nurse practitioner dina works with thetransplant program clinical research nurses from theclinical trials office as well as fellows who are in training and some of our other colleagues all themulti-disciplinary team members are available during stemcell transplant for any complications that occur duringthis process the staff at boston medical wasfantastic
i remember thinking in this processthank god we're an hour from boston and i'm a bit of a northeast elitist is what i've learned with this i work inhigher ed in massachusetts and an hour from thebest medical facilities in the world and i just remember this isgroundbreaking what they're doing here and thank god this is you diane's housein 25 minutes from her door to the front of boston medical and i was thankful for that because the staff from beginning to end i mean literally talking when you checked in the first day at the amyloid clinic in the moakley building
to these world-renowned physicians whoare specialists in amyloidosis which is a disease two years ago i had never heard of and i remember thinking to myself thatboy even if these people were had a horrible bedside manner you could tolerate it because they'regoing to save your life but they were some of the most decent just great human beings ican't say enough about dina dina was kinda my rock through the process where i had her work number i think her personal cell phone numbers just where if i
have any questions or concerns i couldget in touch with her she just was every bit as vested in seeing a great outcome for diane as i was just to me it was incredible this place that has so many people that they're taking care of and such a burden on them that they were so good as human beings too i remember a moment walking into one of the procedures procedures for diane and dina and i each had a coffee and she raised it up we clinked it as if to toast and said here we go
and it just that's the kindasupport you need as a family member going in when it just you know your world's crashingaround but to know you have these people that are amazing and that they actually care about you is just incredible dr seldin anything new on thehorizon you see as an alternative or supplement this type of process there's a lotof research using stem cells going on in fact one of the things that we'redoing with stem cells is
studying some the other amyloid diseasesand we're starting to develop models using stem cells for the type of amyloidosis that comes from the liver not the bone marrow there's a veryactive research program at the boston university medical campus developing stem cell models for manytypes of diseases we work closely with that research groupwe work closely with many different groups at the medical campus to improve treatment one of the thingsthat actually in terms of the al amyloidosis
is really thinking about how we go afterthe proteins that are deposited in theorgans itself so the things that we've spoken aboutso far really are targeting the factory inside the bonemarrow with chemotherapy we also would like to target the proteindeposits themselves and so we work with biophysicists and biochemists on the structure and folding and misfolding those proteins and small moleculeapproaches to attack them we've developed an animalmodel using
genetically engineered mice and we'restarting to look at treatments that will go afterthe proteins themselves that would be a huge benefits to patients if we could kinda do two things at once or in somesequence go after the factory go after thedeposits and really have a chance of really clearing the whole disease from the body wow dr sanchorowala i think that you know the newertreatments or the novel agents which have been developed in the treatment of myeloma
also have been shown to have significantefficacy in patients with amyloidosis and i think those treatments are goingto be either a single agent or in combination with otherchemotherapy agents are going to be you know i think very very beneficialand you know step forward in the treatment of this disease doctor seldin something you brought up a moment ago the union as i see it between bostonmedical center and boston university school of medicinetell us a little bit about that from a patient's perspective it's a wonderfulthing well from from every perspective it's a wonderfulthing we have a
campus in the south end of boston we've got a hospital we've got a school public health we've got a dental school we've got a medical school we've got a graduate school and we have very that geography facilitates collaborationbetween basic researchers and clinicians and the hospital i think that's really one of the basics of success of our program that you know the research component of this started you know in the laboratory at the medical school and oncebreakthroughs got to a
certain place where we could start tooffer treatment to patients it was very easy for us to set up a clinicalprogram a transplant program and we hope it is seamless to patients there's a lot goingon behind the scenes to develop new treatments and supportpatient's through their treatment and think it's a great organization that both the school and the hospital work together so well supporting ourprogram
it is a huge undertaking now i wannaturn focus and look at you personally tell us aboutyour career choice how do you end up here you want the truth i want the absolute truth so i came yet as a fellow as a after i completed my residency as a training in hematology/oncology in 1994 and it was july first nineteenninety-four when i'm on the floor in the hospital ireally do not even know where the
bathroom is i do not know what where to start where to begin and one ofthe nurses says oh this is your patient you go and seehim i didn't really know what the diagnosis was i'm going into the room and they say ohyou can't go and like that you have to put a mask a gown booties and gloves to really reallyenter the room i did get dressed and entered the room and i said so
what do you have and he says i haveamyloidosis i said how do you spell that disease he was very very sick i took him through transplant the gentleman came from new jersey to undergo treatment with high-dosechemotherapy and stem cell and during my that rotation of you know three months you know he wasstill in the hospital for three months and was extremely extremely sick his family members he had three childrenthey were
in new jersey his wife was a flightattendant and was able to come to visit him onlyon the weekend and i thought that was a challenge forme i thought that was a you knowsocial challenge academic challenge and i took it uponmyself saying that i don't know the disease idon't know the treatment but i would make sure that thisgentleman gets through and that is how my career started ifinished my fellowship and stayed here stayed on staff
since 1997 and you know i think i likechallenges as you have known and this is a challenging disease i've seen many patients undergo thismassive aggressive treatment and get better you know thatshow i got involved in this disease and got involved in the career andhow fortunate your patients are i must say i become part of their family yes you have and doctor seldin what about you notsure i can top that you know i came to boston university you know around when this program wasbeginning in in 1995 and
and you know was asked to become a part of it as a someone arriving with a background instem cell transplantation for other diseases like dr sanchorawala i was incredibly intrigued by the disease i wasincredibly rewarded by the responses that our early patients had and the way that they got better and i developed a stronger and strongerinterest over the years and have the privilege to be third director of whats now the
amyloidosis center at boston university i really follow on the shoulders of giants our program wasbegun by doctor alan cohen a rheumatologist who started research the research side of this in the nineteen sixties dr. skinnertook over the program martha skinner and she really had the foresight to not only to continue to expand the laboratory side of the program but to reach out
to clinicians and to assemble a team to beable to start the treatment components of theprogram and to build disciplinary program and she is internationally-recognized figure in this disease she's been president of the international society of amyloidosis and it's really privilege for all of us to continue the work that she and dr cohen before her began
the day we finally got to go home we actually talked about it the daybefore and the doctor said well depending on what your labs back at you know we'll probably let yougo home and in the history of amyloid cell transplant patients she made history she went home a week earlier then what they said would be her earliesttime to be able to leave and let me tell you when we found out the day that there was a chance that she could leave she had me packing up that car the night before cause the
second they told her she could go home we were in that car and that's exactly what happenedthere wasn't a crumb in that that was left in the hotel room to pack i had that car packed to the hills the night before and if she could have ran to the car she wouldhave done it you would have done it so that was uplifting and and i have to say it was onmy birthday that we were able to come home
and i remember saying to diane it was thebest birthday of my entire life and i was grateful to beable to share that way her you know a lot happened in such a shortperiod in three weeks as far as you know things that were emotionalfor the two of us and just watching her deteriorate day after day after day so towards the last few days of being inboston when her counts were coming up and youcould actually start
her coming back to herself looking wise she you know as far as her spirit it didn't take her down well its forty days post-transplant a few days before christmas and i guessi'm doing pretty well now by comparison at least it's been a really it's been a really interesting toughordeal when you consider the week afterthanksgiving
was not that long ago i had a standing blood pressure of 71 over 45 which meant i couldn't stand more than 90 seconds icouldn't even stand for two minutes to enable them take my standing bloodpressure or i would hit the ground it is remarkable how well i'm doing now not to say that i don't have some minor issues but i am very excited at how well i'm feeling i had come close togiving up i felt that bad i was unable towalk my dogs i was unable to concentrate most importantly if i couldn't stand for more than
ninety seconds how was i ever going to go back to the classroom and be a law school professor how was i ever going to stand at thepodium i couldn't even stand i couldn't climb stairs i'd climb two stairs and i'd have to sit i couldn't talk anybody to my friends to my colleagues because i was close to giving up and i would have just sobbed you know my life as i knew it i thought had come to a close this went on for a number of weeks so as each day passed i became more fearful that i wouldn't have my old life back but i had the most wonderful team at boston medical
stem cell transplant team and the amyloid doctor are second to none and the interesting thing is they'renice people and they worked at it work at my blood pressure through heart medication and finally i started to feel better and i was able to stand it was a good day when my blood pressure hit 90 i think we were all ready to celebrate so although my blood pressure is low i'm able to function i'm able to walk my dogs we don't take the hills although i must admit yesterday i tried and we did pretty well we take the easier routes a little shorter distance
but i'm back to functioning i'm back to up and down the stairs and so a few days before christmas i'm optimistic the one story i remember is you know where in downtown boston where its not too far from where the boston marathon bombings took place and it didn't dawn on me that i'm in a hooded sweatshirt in ahospital in boston i've got a backpack over my back and i've a got face mask on and because i can't take a chance atgetting my sister sick
and all of a sudden my sister looks up at me and she starts laughing and i was like it hardly seems like the time or the place to be laughing but she tells me mike you look like a terrorist who's going to blow up the building and i rememberthinking gee that's really nice diane but it was that kind of comic relief that made going through this a lotjust a lot less painful than i guess what itcould have been what happened in such a short period oftime in three weeks as far as you know things that were emotional for the two of us
just watching her deteriorate day after day after day so towards thelast few days of being in boston when you could hercounts were coming up you can actually start seeing her coming back to herself and looking wise you know as far as her spirit it didn't takedown during that time we were there there were three other patients going thru the sametreatment
and you get to know them you sat in the same waiting room with them every single dayand and it was the running joke because the three of them were attorneys i mean go figure she was the only female and one of theattorneys that was there he made it like it was a joke like oh i'm not letting a girl beat me doing this oh my god i gotta suck it up more youknow she's never complained i'm gonna stop complaining to my wife
and that was his biggest thing that he wasn't gonna let 'a female beat him you know i had this done during the fall and i live in a house with lots of old oaktrees and i have over a hundred bags that i that i do every year and mycolleagues came out and they cleaned my yard people could not do enough for me my friends at work my personal friends how lucky am i it'sinteresting because i needed 24/7 care and people came out people gave up parts in their life to staywith me my sister
hasn't been home in months so i'msurprised and i'm lucky and i learned that family closefriends and colleagues care about you is really really so crucial and it was really crucial to me pulling through the whole procedure so that surprised me and that my dogs could make it without me was the biggestsurprise of all a little disappointed with that but they did just fine so that's really what was good what reallysurprised me i'm telling you some of the symptoms that i had before are starting to
dissipate so as they say it's all good i admire her for really not giving up i admire her for for yes i'm gonna go through it you knowwhatever it takes i will do in the end the ice treatment was a horrible thing but you know what she did get through it she knew in her mind she was gonna finish it and she was not not gonna do it so i think what she how she youknow how she handled it
sol that's our show and my story on stemcell transplants at the end of the process i had a partiallyfavorable response and now i've moved on to a new type oftreatment as eleanor roosevelt said you gain strength courage confidence byevery experience in which you really stop to look fear in the face you are able to say to yourself i livedthrough this horror i can take the next thing that comesalong so until next time be well
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