If this is your first time visiting my blog and wondering what is going on, here is the first 4 parts.
part 1,
part 2,
part 3 and
part 4.
Some parts are what I heard from my mother-in-law and her experience, when I did a mini interview with her; I refer to her as mom throughout this post.
After Dr. S referred Dr. E in Massachusetts General Hospital, mom called his office. Although Dr. E was out of the country at that time, mom was able to explain everything to his secretary. She told mom to gather all the medical records, and send to them so when he gets back, he can take a look at it.
I was still very ill, but we didn't know what to do next. Since I had to deal with pain all my life, I am pretty good at that, but not knowing what the future will hold (if there is a future) was the part that was putting my spirits down, to be honest, I thought maybe, this time I am really going to die.
One evening around 9:00 pm, the phone rang at my in-law's house. Mom picked it up, and to her surprise it was Dr. E himself. If it was 9:00pm in Utah, that means it was 11:00pm in Massachusetts... Mom and I talked about this; Dr. E probably came back from overseas, saw the medical records, and heard how urgent this was and decided to call her right away instead of waiting till the next morning. Seems to me, that is someone who truly cares, a doctor who I desperately needed to find...
Near the end of the conversation, Dr. E said this to mom, "You explained very well (including all the medical records that were sent to him too), Sachiko does not have Kasabach-Merritt syndrome, she has Venous Malformation."
WHAT??? All my life I thought I had Kasabach-Merritt syndrome, but I didn't? I will actually get more of an explanation from Dr. E later on, but at that time, I was still skeptical, but wondered if the doctors in Japan misdiagnosed me.
Also, Dr. E asked mom how soon we can fly to Boston and come see him...
All I could do at the time was just lie in bed and deal with the pain. Although, everything was falling in to place, there were many times in my life where I have met amazing people that have helped me through difficult times. This was another experience that taught me that there are angels among us. My in-laws, Mr. TRH worked everything out so that the travel was doable. We were still young, and just purchased our first home; we didn't have enough savings to cover the cost of the trip. Without my in-law's generous help we would have had a much harder time going to Boston. Mr. TRH had to get a week long time off from work and school. We were able to reserve a seat on the airplane with wider leg room. I was very nervous going on the trip, because I have been lying down for so long, I didn't know how long I could stay sitting with pain. We thought with the extra leg room, I could even lie down on the floor if I needed to. The people at Mass General as well, they worked everything out so I could come to see the doctor and possibly to have a procedure done to help me.
The trip to Boston was one of the fond memories that I think about often. It was indeed uncomfortable to sit, but I made it through. When we got to Boston, I was surprised to see the city has similarities to where I grew up in Japan. The gray sky, rain (living in Utah, there is quit a difference), a river that runs through in the city... from inside of the moving car I was eagerly looking and observing the scenery and I had plenty of time to do so because we got lost! We rented a car, and from the airport to our hotel we got lost...well, we were lost for almost 4 hours! The roads in Boston are more complicated compared to Utah and there were many "one way" streets, and we had to go all the way somewhere to go back and such. I didn't mind though, looking at unfamiliar scenery, joking with each other back and forth really lightened my soul. Not to mention that meeting the doctor who might be able to help me was giving me hope.
We stayed at a nearby hotel to the hospital, and went to see Dr. E the very next day. The meeting and talking with him was such a life changing moment for me. He explained that the Kasabach-Merritt syndrome and venous malformation are VERY similar in terms of conditions. At the time I was born, there was no way of knowing which was which. He said that the patient who has Kasabach does not live a long life; in fact a lot of them die at a very young age. He also told me that sometimes, Kasabach can change into venous malformation too. So, either I had venous malformation all along, or I am the extremely lucky one where Kasabach changed forms and became venous malformation.
He also reassured me that there were no treatment back then, so my parents and doctors made the right call of amputating my leg. He has been a specialist of this condition and has seen so many patients, but he has never seen a condition so extensive as mine. Because of it, there is no cure for me, but with a certain procedure it is possible to control the symptoms.
I had more questions, and he had more answers. This was the first time in my life that one doctor knew everything I wanted to know. Dr. E was kind, compassionate, and not arrogant at all... (Dr. D and Dr. T- In your face! :) ). After talking to him, I felt like a cloud was lifted and I had a clear view of part of my life!
The next day, I went back to the hospital to have my first sclerotherapy. Basically, they will inject this formula directly in to my veins that are too big and wide and try to shrink them. Of course during the procedure I am completely asleep... when I woke up from the procedure, I had a really bad reaction to the anesthesia, but that wore off and I was able to go back to our hotel the next day.
The next few days, I was recovering at the hotel, except when we went out to eat. We couldn't do much sightseeing, but we had a great time. We watched many movies in the room, one night we watched this B-movie called "The Tick". A giant tick was attacking people and killing them. We were laughing our heads off at certain lines, and situations. I LOVED the lemon sorbet at Legal Seafood too! I promised myself that someday I am going back to Boston to do sightseeing and visit many museums and shops.
Dr. E also located a Doctor in Utah who can do the exact same procedure for us. After the first procedure, I saw an amazing difference for the first time in a few months. The pain in the leg was reduced, I was able to sit and move around on my crutches around the house, even though I still had many areas that needed the procedure. I had the procedure done every 6 weeks for the next year and a half. Because of sclerotherapy, my quality of life has greatly improved. I was able to have three beautiful children without major complications. I was able to have a hip replacement (oh yeah, I was also born with abnormal hip dysplasia, and it was giving me awful pain all the time).
Now, when I notice the pain or anemia creeps up on me, I know where to go, and what to do to make it better before it gets really bad. I have some control over my syndrome, and that is an amazing feeling of freedom and security.
On a side note, I had the pleasure meeting another doctor at the U of U. She is my heroine Dr. B a Hematologist. She was helping me to control my anemia, and also she finally discovered why my body kept rejecting the blood I was given.
Test after test, there were still no answers. She even sent out my blood samples to labs in different states. The people in the lab at the U of U were also trying to figure out this mystery.
One day I was at the hospital (I don't remember why I was there, but I was waiting for something) and a man came in to the room. He seemed very excited and said, "Hi! I am so and so (I can't remember his name, and that is the only time I met him), first of all, can I shake your hands?".
I was puzzled about his request, and thinking to myself, "OK... Who is he? Why does he want to shake my hands? So...odd..."
As he was shaking my hand, he started explaining to me that he finally solved the puzzle of why my body was rejecting blood transfusion. Basically, my blood is missing little antigen-e. When I receive blood with little antigen-e, my body doesn't recognize it, so it starts attacking. So, as long as they clean the blood and remove little antigen-e then give it to me, I am fine.
Usually when they run the test, they do not run such an extensive set of tests. It cost more time and money. Then Dr. B and the man from the lab were determined to find out why and their determination paid off!! I was so excited to hear the news. There are no more bad reactions to blood transfusions!
He also said that he heard about a condition like that, but he has never met a person with that blood type in 10 years of working at the lab. He was so excited to finally meet one.
I went through a whole bunch of frustrations and sadness, but in the end (well...at least with this story. I have so many more medical stories) it was well worth it. I discovered so many things about my conditions, found a procedure that will help me and the love and respect towards Mr. TRH increased by 200%. I knew I married a nice person who loves me, but the love, support and devotion he gave me during that time was way more than I could ask for. And sometimes I wonder, if I can do the same for him if he is the one that gets sick. Whenever I think of that, it makes me wanna be a better person.
Sometimes, people tell me that I am a very special. Yeah... I was born with venous malformation, Abnormal Hip dysplasia, missing little antigen-e in my blood and top that all off with allergies to most pain meds. I am special all right. I stop asking "why's" a long time ago, but I can't help but hope that since I feel like I had my share of medical craziness, I will not get any more scary stuff.
Speaking of hope, one of my good qualities that God has blessed me with is that I am always very hopeful. When my boys were littler, one of them said; "when I get big, I will become a scientist and invent a seed to grow your leg back!". That made me smile.
But hey, we can put a man on the moon, or select genders of babies, why not a seed to grow my leg? I will never say never... I only never give-up. :)
Thank you all for reading such a long post! I am really thankful that there are people interested in my story and who take time to read them. If you are going through a tough time, hang in there... I will be thinking of you.
Love & hugs
Sachiko
