In 1996, just one month after i got married my sister Kimberlee died of undefined collagen vascular disorder. In her 26 years alive she battled multiple issues with a club foot she was born with. She had 28 surgeries to date on her legs, ankles and calves alone prior. She also had a 5 year old daughter at the time, so when i received a call at work that Kim was in the hospital and was possibly not going to make it thru delivery i rushed to be by her side. The guilt i felt was horrible. She just spoke to me the evening before asking for a candy bar and crying that she didn't want surgery or any more scars. You see i was 4 1/2 months pregnant myself and was very sick to my stomach and i did not go. My whole family was so scared and upset. My sister is pregnant; why is she dying? No one knew the answer to that. How did this happen? She has seen so many doctors over the years. Why did this happen?
You see, the doctors took my nephew via c-section because my sister thought the pain she was having was contractions when in reality it was a huge blood clot the size of a grapefruit shifting all of her organs to one side. When they cut her open they realized that her vitals were very low and had to stop and that decision kept her from being sewn up. So they shoved and packed towels inside her stomach and placed a film of plastic wrap over that. She was bigger than when she was pregnant. Kim had taken a fall about a month earlier on a cleaning job and at the time was taken to the hospital with blood vessels popping in her legs (thigh area). It almost looked like she had been raped. The hospital sent her home on bed rest. (Cape Coral Hospital, Fl.)
When i reached the hospital to be with my sister we were all told that Kim probably wouldn't make it through the surgery to sew her back up because they said her tissues were like cooked spaghetti where ours are like uncooked spaghetti; they were waiting for her vitals to stabilize. They didn't exactly know what was going on at the time. All they knew was she was losing a lot of blood. Kim had drained two local blood banks of blood and platlets. You could see the blood dripping from every needle in her neck and arms. We were so scared, we haven't lost anyone in our family in years aside from just being old. What was happening? My sister has been through so much as it was. It was like a dream; a nightmare. We asked my sister who she wanted to take care of the kids in a round about way making it look like she would be in the hospital for a while.....she couldn't speak because of the tubes. The nurse went and got a board for her to point at letters but it was too blurry to read. So as i stand there watching my sister struggle for breaths and bleeding from every needle point on her body, she starts to sign to me. As kids we taught ourselves to sign letters. I saw her bring her hand up and sign these letters to me: A....M......I......G.....O......I....N......G......T......O......D.....I.......E ?
I just started to ball my eyes out and left the room for my brother to answer her......how could i tell my sister the truth when she is already fighting for her life? He went in and told her that she was going to have a serious surgery and that we just wanted to make sure the kids were where she wanted them to be. My sister answered the question with my mom and the father of her daughter. The doctors let us all walk her back; all 20 of us right to the operating room doors. I was so upset and sick to my stomach, i had to eat. My oldest sister and i went to get a bite downstairs with the priest. About 20 minutes later as we sit down to eat my sister passed thru me. I felt this woosh inside me. I grab my sisters leg under the table and said we need to go back upstairs now. I really didn't want to say what just happened in front of the priest as he may look at me funny. I don't know why i felt that way he probably deals with that all the time. It wasn't 20 minutes later when the doctor came out to tell us that Kim passed away. I CAN'T POSSIBLY TELL YOU HOW THAT MADE ME FEEL, HOW I'M FEELING NOW AS I TYPE THIS......EMPTY.
So many people wanted me to sit but i couldn't. What just happened? MY SISTER DIED HAVING A BABY???? All i can say is that day was probably the last day that our family was ever whole. That night i started to bleed lightly. I only told my husband and best friend. Later that night they would rush me to the hospital with heavy bleeding and vomiting. For three days my husband stayed by my side because we were both worried what happened to my sister was happening to me too. I wouldn't let him call my mother because she needed to grieve for my sister until the third day when they wanted to give me blood; i lost so much already. I told my husband to call my mom who was a private nurse. The doctor stopped my contractions for approximately a day, then at 1:20 a.m. i remember looking at the clock thinking "oh god, they are starting again". I looked at my husband laying asleep on the sleeper sofa when i felt a hand rub the top of my head with the softest stroke. I looked in back of my bed and no one there. It was then and there i didn't worry about losing the baby because i knew my sister would be taking care of it. I woke my husband and told him what was going on, about 2 hours later i lost the baby. We had no clue it was a boy until then. We swapped sons!!!! I was so worried at the time that i wouldn't make it to my own sisters funeral.
My husband and I raised my nephew as our own son along with my own 5 year old daughter and later another boy and girl. My son (nephew) has shown many of the signs my sister had. Easy bruising, bleeding, tearing of skin, light rash on cheeks and thighs, burns easily; right down to the mysterious rash that looks like ringworm my sister had on her arms and his are on his neck. We have had him tested repeatedly over the years for lupus as most doctors advised he probably wouldn't test positive until his adolescense. We are now in the process of seeing a specialist at shands hospital in gainsville. I think we are finally getting somewhere. I don't want my son being lost in the crowd and shoved aside like my sister. When I found the exact picture on the internet for EPS or the rings on his neck and it stated it was associated with Ehlers-Danlos type IV and I read up on it; I knew I was on the right track. Finally a diagnosis! Read Londons story below.
UPDATE 4-9-09: As much as I was hoping I was wrong, I am glad that we finally know a cause of concern. The geneticist of all days in the year tried to reach me on Kimmy's birthday to tell me that he is positive for Ehlers-Danlos type IV. The pain I felt 13 years ago all of a sudden hit me once again. Please keep him in your prayers..........
UPDATE 1-22-11: He has his first episode at 11 pm with severe stomach pain, he crawled into the bedroom crying. He starting vomiting a short time later so I rushed him to hospital where they officially discharged him with a UTI knowing I was still concerned for his rapid breathing and belly pain. Good thing they checked his temperature as it shot up to 103 and white count fell to 1.1. I asked my husbands surgeon to come in for opinion at that time and he stated London should be rushed to UF where all of his doctors are. Yep, he had a hole in the sigmoid colon that could be seen on MRI w/ contrast. Emergency surgery was done while mom freaks out that he might not make it. 29 hours after the pain started....surgery was finished. Thank you so much Dr. Kays and his surgical team for listening to me and watching the EDS emergency cd before proceeding. What an ordeal when we got there.....the first ER Dr. didn't want to listen to me, watch the cd or talk to an expert physician. She claims that EDS patients do not normally present with abdominal pain; mom slaps forehead looks at son and says we are in big trouble. My eldest daughter calls Dr. Naz for me who in turn calls the ER physician and says who knows what resulting in a new dr. ASAP. Thanks Dr. Naz, your the best! Now we wait for Dr. Kays to reattach bowel for him. Heres hoping there are no complications on this one. Last surgery caused London to get bilateral plural effusion about 8 days after coming home landing us back at shands for another 4 days. Cool thing was; on the fourth day in ICU coming back from a break I saw little feet and pants under his curtain standing next to his bed. Expecting to see a nurse when i came in the room; no body there. Hmmmm, guess mommy is still watching over him!
I argued with the doctor on call when London was in ICU telling him that EDS patients heal slower than normal people and should not be moved for a few days. Not even 24 hours had passed and he wanted him moved downstairs already. I got a patient advocate involved and even though I tried my best the compromise was to move him to an area downstairs where he would be watched by a nurse with only three patients. At this point, we know a lot of the nurses by name and we had a good nurse which made me vey happy. When i woke in the morning and looked over at London I could tell only half of his chest was raising. I quickly jumped up and ran out to Vicki our nurse to tell her and she said she already knew and had a doctor on the way. Of course the same doctor i argued with about moving him came in and London right lung collapsed. This is only one of the problems we have dealt with making sure he has proper care. Before London had his bowel reattached the surgeon wanted to get an idea of how much of the lower bowel was left. So he was wheeled downstairs for a enema w/ contrast. Before I let the staff proceed I went over Londons medical issue and that he ruptures easily. While consoling my son and watching what the staff was doing.....poor London starts complaining of pain. I am listening to the staff question whether or not the contrast has made it into the bag yet. I asked them if they were trying to get the contrast into the colostomy bad and they said yes. I said but Its not attached to his ASS. They almost ruptured him before his anastamosis. This happened with a physician in the room with us. Are you kidding me? How many times do i have to save my son while trying to save my son? Who is the doctor here exactly? Ugh