Hello My name is Riccardo,
I am 25 years old and until November 2005 was told that i was not even a Thal carrier, after a tiring child hood and even leaving my professional cooking career I work in Telecom and was in the company three years, when the fatigue was just to much , I went on sick leave my family pyhsician saw something wrong and did not know what so i sought a hematoligist who after doing numerous tests and getting numerous resultd told me i was perhaps causing this condition mentally and that there was just some minor anemia. So i went on at this point i was getting depresses, Soon after my mother had to undergo a historectomy and she enrolled in a study since the Royal Victoria hospital is auniversity hospital here in montreal, When the study team arrived my mother having a great maternal instinct asked the Chief hematologist to look at me, Imeadiatly she told me there was something wrong and we began testing, I was so overjoyed that perhaps it was not in my head! Dr Margaret Warner the chiefe of hematology/ oncology at theMonteal University Health Centerand director of thalassemia here in montreal became my friend and gardian angel, After a battery of tests we discovered the Thalassemia to be , Homozygeous Sicilian Gamma delta beta thalassemia intermedia-major still unsure, untill now i was never transfussed ever, at this point my ferritin was 700,
I was introduced into the thal center and recieved my first transfussion, which changed my life for the next 2 weeks i regained so much energy, at ths point my spleen was 15 cm , after the two weeks i developed fever and felt terible rapid heartbat, no strength and became green in color, i went to see Doctor Warner and we mesured bloods my Hb was at 38 I was immediatly admittted to the hospital an given 3 units of blood, o which i had no response at all ,I also developed an infection and fever, and became nutropenic and was placed in isolation, this was february 14 2006, The doctors did not understand we did bone marrow aspirations which is one of the most painful things in life, as we suspectd luckemia and all came in negative, i was reiving 3 units of blood per day and there was no response at all, my spleen was reaching 27 cm it was as if it was injesting all my blood, Dr. warner decide spleenectomy was a wise choice as nothing seemed to work Ipprex, igg, nupogen nothing at all, I signed my consent form and had liver transplant surgeon that was going to perform the operation my hemoglobin was 40 and i had a severe infection due to auto immune antibodies , the relese forms and doctors told me my condition was innacceptable to opperate and it was a 60/40 risk but I had no choice and i had my faith as well so We went in on march 1 and i was succesfull i was in ICU for 3 days fully intubed and recovered well, 44 stiches , myspleen was 36 cm when removed and weighed about 3 pounds, my hg started to go up and i reached 50 with prendizone etc but no more blood.
Theproblem still was the infection i filled over 80 blood culture vials and nothing was ever found, all of a sudden we stopped vancomicin and other anitbiotics an he fever broke, After numerous panel discussions across canada Dr Warner decided to treat autoimmune antibodies with Rituxan a drug used in nhl, the drug went well no side effets with me, and i was released from hospital after 38 days, However his was the tip of the iceberg, we soon discovered massive osteoporosis t score of -3.2- -4.1 and so i began carbo cal and vitamin d as well as Fosamax, folic acid for life, Panaloc for the stomach,cortef to help me as my cortisol levels returned after long use of prednizone, also my vision was affected i had losg a considerable ammount of my vison after the surgery and was seen by ophtamology and found out i had retinal hemorages or roth's spots which is very rare bu occures in severe anemia especially in drops of blood pressure , after a while this cleared but my vision has not been the same so now after ding a Pet /Ct t o rule out any tumors since i had numerous hadachs and then an mri we founf Extramedullary hematopoesis in my skull pressing on the optic nerve Very Very rare, so i begin Hydrea 2000 mg per day in an effort to slow down my bone marrow as it is working about 60-80 times more than a normal human , On an Indium chloride bone marrow scan every bone in my body was pithc black indicating exteme dense marrow concentrations in every crevvase of my body, so toda i saw a neurosurgeon who explaind how to remove this mass pressing on my optic bnerve and a radiation onclogist who gave me another option of doing radio therapy , for now we will wait 1 more month to see what he next mri says on the progression of the mass or see if hydrea is working,
I also have developed severe fatigue despite a nontransfussed hg of 130 stable, but this they say is hyper mtabolic my body is working constantly to produce new cells, and as well i have extreme joint pain which is unexplained nothing is shown in blood work mri x ray so perhaps fibromyalgia i take dilaudid for the pain about 20 mg a day ! so this is my story My doctors are presenting my case to the american journal of hematology because it is so rare if you have any input feel free to respond or write also if any questions or insight, oh and mentally im doing great it is moremy family and fiancee that are suffering as i am in great spirits and keep positive! To all of yo u out there Keep positve and you will overcome anything ! Also I just want to mention A special thankou To Dr. Margaret Warner my gardien ange frien and another mother who actually had me pulled out from the or when i was getting operated just to hug me and reasure me good doctors are everyeher let us learn to appreciate them and value, them.
I keep myself busy now as i go to hospital about 2-3 times a week i am getting married in July so that is exciting I often come here and read and it reassures me knowing i am not alone and that there are other graet stories like mine, even though we are in the unknown right now withmy doctors we are lways thinking ahead perhaps more rituxan we'll see if radiaion or brain surgery is abetter option for the extramedullary hematopoesis and we are always talking stem cell transplants and the berloni insitute in Pesaro Italy he world leaderin Thal treatment and most succesful stem cell transplant center in the world,
venthough it may seem life sucks for me I neverfeel it does and many people tell me its remarkabl how im not affected or depressed by the constant new developmets like the brain mass and the vision problems along with the severe pain , What I tell them is that i hav learned to appreciate life day by day , although my doctors tell me im an intermedia i sometimeds wonder if life expectancy is lower , my ferriin is about a thousand and droping so i am fortunate, the doctors say because i only produce hemoglobin f(featal) it sort of protects me in a way and my body has compensated by producing so much marrow to produce so many cells, the human bod truly s a marvel!
Keep Positive and ou will win any batles keep me in your thoughts as i go through the radiation or surgery as i will keep all of you in mine and dont hesitate to write or ask me any questions or send soem inpit!
Riccardo Camarda
A Rare of Rare of the Rarest ......well at least i am unique...
