5 posts tagged “brittle bones”
First let me apologize. I always forget that not everyone knows us personally.
I thought I'd give a bit of an anatomy lesson, no I'm not going to talk about how this bone is connected to that. I just thought since I received so many pm's asking that it would be easier to answer the questions all at once on here.
David has a fairly rare condition that makes his bones brittle. He is not lacking calcium like in the elderly where they get osteoporosis. He is lacking collagen. Collagen is what gives all our tissues in our body elasticity. Without it all our connective tissues are impacted. Connective tissues can be anything from ligaments and tendons to bone, muscle, the sclera in our eyes, white part. David has blue sclera as opposed to white or yellowish like most people.
What the treatment does for David.
All of us have bones and our bones are renewing themselves regularly. That is a part of our normal structure. When we are injured new bone appears in the form of callus type calcifications over the injury. This is the same for David. Understand, David's bones are much thinner than ours are. If you take, for example, one of our bones....the tibia or any of the long bones, they all sort of look the same. The are on x-ray thick bone covering a center channel of bone marrow. David's bones are not the same. His are a shell of bone covering a much larger channel of marrow. So then the difference between his and ours, above and beyond the obvious difference of brittle-ness, is that his bones don't have as much material holding them together which adds to the fragility.
So, Pamidronate, and many other bisphosphonate drugs, sort of prevent the absorption of the bone we have that is being made new. As I stated above all of us are making new bone all the time. The old bone is absorbed back into our system. We are slowing the process of absorption in David's bones, essentially, and allowing his bone to build greater mass. In other words, instead of thin, brittle bones he has thicker, brittle bones now.
Of course there are down sides to every drug and one of them with the type we use on him is an initial tiredness. Also, the potential for calcium loss. David has to take added calcium and then we have to watch for things like kidney stones.
The benefits are obvious. David hasn't had a major fracture since we started 2 1/2 yeas ago, other than a toe. Also, it has helped with bone pain, something David has constantly. He is never without pain, but this drug has lessened it.
The drug is infused via an I.V. over 3 hours for 3 consecutive days. The I.V. port stays in for all 3 days. We go to the hospital at 8 and are usually home around noon.
I hope this helped answer some of the questions.
Feel free to ask more as they arise.
I've been thinking about the post I did earlier about perfume. Or was it last night? Senility!
I posted why it has become important to me that I finally listen to myself, my heart, my head. It's time to take time for me.
Many of you know I grew up in an abusive home. My greatest wish was to become normal. You know, live the so called American dream complete with white picket fence, mommy baking cookies in the kitchen, devoted father and 2 parents in the home who loved each other and the children. Of course with no modelling I had no idea how to go about this.
Back in '81 I got married to the man I thought would be my forever love. What I didn't know, couldn't know, was that I didn't love him. I didn't know what that term meant. I learned, it took the birth of my daughter, but it was too late. By then I had a baby to look after and was determined to make it work as long as he didn't hurt me or my daughter. He didn't, and so it went.
Life evolved, we bought the house with the picket fence, tried to have more babies but couldn't, took infertility drugs, went through tests all to no avail. The marriage, the devotion was slowly slipping away. It was an unspoken fact that the respect that should be a part of the relationship had completely dissolved, but I was determined to not allow life to fall apart.
8 years after the birth of our daughter we were once again in counseling. I took a trip with my sister to clear my head, she moved to Denver, and when I came home was revitalized enough to make a go of the marriage. Immediately I got pregnant and I felt beyond trapped. I had not wanted more children at this point, having gone through so much I assumed we were not going to be able to have more so birth control was never discussed.
I started spotting almost immediately on finding out I was pregnant. Maybe it wouldn't last? I went to the doctor and he set up an ultrasound. By the time I went for the ultrasound I wanted my baby. The heart beat was strong and everything appeared intact so I went home, took it easy for a few days and got back to living.
It was a hot night, or should I say morning, Sept. 13th, Friday at 4 am. I had not been able to get to sleep. I was hot and miserable, uncomfortable as hell so I wandered the house. Then the pain, just a little nudge at the small of my back but it grew and I knew immediately what was going to happen. I woke my husband. We took Michelle to grama's and headed to the hospital. This was the day my world was forever changed.
David greeted the world the morning at 8:12. He weighed nearly 9 pounds and had a squished nose. He was handed to me and I put him to my breast. He didn't suckle. I didn't know why because Michelle had. I didn't know anything but I felt something. My labor nurse took him from me to clean him up and then she came and I saw her lean over the doctor and point to David's mouth. I knew in my soul something was wrong. It was like being in slow motion. I felt like I was trying to speak but no one heard me or saw me. I wasn't there but I was trying to get their attention.
I got taken back to my room and David was taken to be 'weighed, measured have the normal tests', whatever.
About 45 minutes later David was brought to me, but not until after Rick, my doctor, came to talk to me. He said David had something called Pierre Robin Sequence, that he needed to be shipped off to another hospital because his breathing kept stopping and they needed to have him in an ICU where they could better evaluate him. I was not to be released, oh no. I had to stay where I was. I remember shaking my head and saying it was not possible his breathing was a problem; until I held him and he turned blue. I went into shock. I didn't know what to think. I didn't know how.
That was the beginning of a very long journey. Over the next 2 years we went from genetics counselor to plastic surgeons, David had to have the cleft palate, part of the 'sequence', repaired. We thought we had it licked. He was finally growing, developing, he was beautiful.
Surgery for the palate happened when David was nine months old. He was a little late to walk, 14 months but all other milestones had been on a similar schedule to his sister's so we let him grow and explore. Until the day I took him grocery shopping with me.
David and I arrived home from a store trip. We had this little step to our back door and I had my arms full. David was 1 1/2 and thrilled to be moving under his own steam. I started into the house and made it inside the kitchen door when I heard a scream I have since learned to associate with horror. I turned to find David sitting at the bottom of the stair holding his leg rocking back and forth screaming and white as a ghost.
I went to David, kneeled down, leaned towards him and was trying to look at his leg. I couldn't see anything but I did see a bruise starting on his shin. I leaned over him and picked him up, careful to hold his leg so I didn't hurt it, went inside and called his dad. He came home, took a look and came with me to bring David to the doctor.
David came home with his first cast. Our doctor had told me to not worry. He said it was a fluke. David would get over it and life would be great. I never felt ok about it though. Moms really do always know. Sure enough, as soon as the cast on his leg came off another one went on to his other leg. I was beginning to think I was a horrible mother, not paying enough attention to David.
This time the doctor did a thorough check up and made a recommendation to us. He suggested that we take David to be seen at Children's hospital in Seattle. He said there was a doctor there who specialized in disorders like he suspected David had. We went home and dismissed the idea. We were getting ready to move to a new state, had just sold our house and were going to try to make a new start because things had gotten very rocky in our marriage. Neither of us was ready to admit there was even the slightest possibility of something more being wrong with David, but I did know. Deep down inside I knew.
We moved to Oregon. We had just gotten settled when David decided to walk across the floor, in stubbing his toe he broke the first metatarsal in his foot. A new cast and the realization that we could live in denial no more. We made the appointment to go to Seattle. Within 6 weeks we knew what was wrong and why the fracturing. We also knew it would continue for his life. David has a fairly rare disorder called Osteogenesis Imperfecta. Brittle Bones. His type is the most mild form.
For a long time David would fracture something on a fairly regular basis. Every couple of months we would make the trip to the hospital and more xrays. One of the worst times was a fracture of the radius and a dislocated elbow at the same time. Until 3 years ago. Another devastating time and one that has brought home once again the need for me to not take for granted this life I've been blessed to have.
Although not a churchy type person I am spiritual in a sense. I do believe in karma, to a certain extent and I also believe we have an obligation to learn from everything that either happens to us or that we make happen. I believe many of us go through life with our eyes closed to what is around us. Life is to be explored, in every way, every moment of every day. We should live with vigor. We also need to take ownership and be authentic. Where have I heard that word lately? ;-)
Just over three years ago I had taken David to the doctor. His back had been hurting and it was the second time in the not too distant past so I figured what they heck. Let's go see if there is a problem. In the meantime winter had come on with a vengeance and while David had become a boy who had learned to accept his limitations, we had not prevented him from living his life the way he wanted, translated that means David was riding bikes, running around though he wasn't participating in gym because his back had been sore for awhile. WInter time was a difficult time, though because all things are not created equal and ice is death for David. He knew it then and knows it more now.
The gym teacher had asked if David would like to play ref for volleyball at lunch time. David said he did and we agreed.
Day 1 back to school after winter holidays David was set to start refereeing so had taken a lunch with him to school. At 12:15 I got a phone call from the school. I was used to it. I had received hundreds of them. This one was different.
Mrs. Breuker? Yes. David has fallen. He has hurt his back. We have called the paramedics. Could you please come?
I don't remember walking out the door. I don't remember driving to the school. I just remember walking in and seeing David lying on the hard wood floor. He had a white face. He wasn't moving, eyes were closed. I ran over to him falling to the floor. I bent over him and asked him where he was broken. He said, "Mama, my back hurts, but so does my arm and my finger on my other hand.
The paramedics arrived. I told them David had brittle bones and so they were extra careful to not move him to fast, not to mention they had to be careful getting a vein for the I.V. and also rolling him onto the board. David spent the next 3 months recuperating. He was right. He had compression fractures in his back and also the ulna in his right arm and the middle finger on his left hand. That required surgery. It also required more on an emotional level than I have ever had to give.
David had a scribe, provided by the school. David maintained his straight A average. The accident, which was caused by stupidity on the gym teachers part for having David stand on a table with poor balance, changed David from a carefree child. You may wonder how he possibly could have been a carefree child with all his history but he was. He was coddled and loved. Respected by all who got to know him, looked up to, amazingly enough, by his peers. Now David lived with fear. He was faced with the knowledge that injury could cause him paralysis. Something none of us had considered. David at 12 was facing and dealing with issues most of us don't have to face until we're old.
So, what has this long, long story to do with why I sit here trying to make my own life have value and trying to live in each moment? I'm a little slow. It took me this much time, and a lot of other stuff, to realize I only get one chance. I maybe don't have the choices I think I have but what I always have is the ability to face each minute head on and grab a hold. I don't have the right to dilly dally along. The world isn't going to wait. I have learned this from a child. I have learned this from my hero.
If you made it to the end, kudos! It was a helluva ride as I lived it, too.
I got a treadmill for Christmas and I'm grateful. The weather has been so cold, not something I want to go into too often that's for sure. I also got a swiss ball. All in all I am very busy with exercise.
Tomorrow I take David in to see the doctor. We couldn't come to a consensus about how to handle the wisdom tooth issue. Skully was contacting McGonnigal to see what he had to say about the osteonecrosis, or the concern we should have or not. Honestly I think being concerned is not something we have to push aside. Skully immediately wanted us to consider seeing a maxillofacial team but I have real issues with that. It's not that David hasn't seen them before, he did. Try getting them to understand that he has more than one disorder and doing surgery on his jaw or any other part of his body will be impacted by his brittle bones and his pierre robin. They can't and don't get it. It's frustrating to me. I had a doc tell me to just be his mom and let them worry about his care but I can't make him a guinea pig.
David is 15 and he wants to lead a life like everyone else. He has dreams and goals. Wishes. Right now his fondest wish is to have a girlfriend but he's kind of shy and he looks to the wrong kind of girls for relationships. He's kind of stuck on the blonde dingbat type. David got voted the kid with the biggest vocabulary at school in his yearbook so you can see why I find the dingbat idea a bad one. He's just caught up in who is considered 'hot' in his school. I keep telling him that what he needs to look for is someone with a good heart who is decent and kind but I was young once and not the traditionally 'hot' girl so I know some guys just can't get beyond the physical. I hope he realizes the right girl exists and that he will find her when the time is right and not before. In the meantime we have teenage growing pains. Emotional, not physical, for once.
I don't know. I really don't know. Now the new year is here I have to officially do something about the treatments David gets. David has pamidronate treatments every 3 months for bone density. The problem we are faced with is osteonecrosis of the jaw bone. Big words, big impact.
David is 15, he is my baby, he has brittle bones. He has been getting pamidronate infusions for over 2 years and the problem right now is that he also needs to have two wisdom teeth removed. There is some cause for concern with regard to the drug he gets intravenously and the breakdown of the jawbone, not to mention whether his jaw could withstand the removal of the teeth to begin with. His jaw has already been broken. Now I have to try to coordinate with the doctors and the oral surgeons and get some sort of consensus so we can move ahead with treatment or not.
I hate being in this position. I hate having to make a choice. My heart is in agony. I always worry I will make the wrong choice. I don't want David to pay for my making a wrong choice. He pays everyday and that is hard enough for me to watch.
I was reading a post,potential blog friends, and it occurred to me I had not defined what would be my blogging terms. Obviously it's not as though I really need to do that because I can apparently keep track of anyone I want and others can keep track of me. Still, what are my standards? Do I have any? Right this minute what seems to happen is as I happen across someone who is interesting to me I add them to my neighborhood without titling them in any way. Is this not OK? I have no idea what other people do but it has been how I handle it. My Vox Blog
So I was thinking what would matter to me and what kind of people would I want to be friends with in a blogosphere? Clearly I would choose people who are interesting. People who I can either live vicariously through or who have lives that I can relate to in some way. Ok, so that established doesn't that mean I need to define myself in some way? I mean I have already added or listed my interests but shouldn't I say who I am and what matters to me? Am I not obligated to be up front about what matters in my world? I think I should definitely define myself better here. I just don't know if it really matters long term because people don't read who I am they read what I post about. I don't know if other people go back months reading about bloggers or if they just happen across a post that is interesting and add that person. And how do other people find each other?
Who I Am.
I'm mid 40's
Female
Married for a second time for 7 years.
Have 5 kids in total, 2 bio, 3 step. All are grown except the youngest who is 15 and has a rare bone disorder.
My husband and my son are my world. They take so much time I rarely feel I have any left for me.
1 year ago almost my husband was diagnosed diabetic. We have altered our lives and lost tons of weight.
We quit smoking over 3 months ago.
I'm healthier than ever in my adult life but still 'not there' yet. It will come with work.
I love cats, like doggies. (Hubby is the opposite)
I don't have a career other than caring for my sons special needs and being a house mom.
I like caring for my home. I like having beautiful gardens.
I like not being poor. (I was growing up)
I was abused as a child and have made my life about raising children safely and with love.
I never had a goal for myself other than to be a mom though at times I have wished to be a career girl.
My husband will retire in the next few years and we have been looking for property to buy.
I look forward to spending retirement years with my husband. I love him so much. Ron is my best friend.
I don't have tons of friends. I have a few. One of my best friends is an alcoholic and I don't see her much.
Another close friend lives far away too but she has the best heart and space doesn't matter with friends like her.
I have a lot of family living in the USA and I miss them, especially my daughter. (I live in Canada)
I love music. Especially jazz, though I'm no connoisseur.
I love tech gadgets, though I don't collect them...don't have enough money for all I would want!
If you find me interesting, add me. If you don't, move on. I don't need 50 pseudo friends I'd rather have 5 real ones. You and I both have better things to do with our time than read someone we could care less about. Take care....that's all that matters.
