During our wonderful dinner with the hematologist (see previous post) my mom stayed home with the littles (hey, I was going to take full advantage of the free wine and dinner at Ruth's Chris). Thanks mimi! She mentioned to me that I needed to put away Camdyn's little chair because she caught her just before she tried to stand up in it. I heard her and intended on doing it the next morning when she was awake.
Saturday morning we were all getting ready for Caden's basketball game when we heard a thud and then a cry. Sure enough she had pulled out that dang chair and I assume (did not see it happen) she stood up and fell forward. She hit her forehead on the door jam. It did not look good. No blood just a big goose egg. I called the hemo on call and was on hold for a couple minutes before I just decided to take her in.
We had previously been instructed to go only to Santa Rosa's ER. They took her in quickly. The ER doctor did not seem concerned about the bump and was confident there was not internal bleeding but called hematology to be extra careful. It was decided to give her an infusion to just be on the safe side. As much as I hated this for her it out weighs the alternative of an internal head bleed.
The infusion was much faster and less traumatic than the first. Excellent nurse got a vein on the first poke. She never showed any symptoms or problems. It was nice knowing she was at normal levels for the next 18 hours too. She was left with a nasty bruise on her forehead but thankfully that is all.
I wish, wish, wish I could keep her bruise free (especially her little face.)
Blog Title
Friday, February 4, 2011
Meeting with an expert
We were invited to a dinner with a leading expert in the field of rare bleeding disorders last week. It was a very nice evening at Ruth's Chris Steakhouse. We were treated to a wonderful, yummy meal and truly appreciated the opportunity to met with Dr. Mathew's from NM. No surprise, we were the only VWD-III family there. I met two severe hemophiliacs, one seven one 23. It is the most comparable bleeding disorder to VWD-III we are told. I had such a peace talking to these brave boys. Both agreed that neither feel different than their friends and feel they are totally normal. Both play sports (no football) and do everything that they want to. One is on Prophylaxis- his mom infuses him every Sunday and Wednesday. No port- just the butterfly IV. They first apply numbing creme and he does not even feel the poke any more. He is the seven year old...he promised me it did not hurt :)
I saw my first port in a 2 year old with mild hemophilia. Neither of the severe cases had it but the mild did??? Goes to show the type does not exactly predict problems. That baby resulted with a brain bleed after birth (again thank you Jesus for my c-section that unknowingly protected my baby). For the first time I was with people that got it...my fears, my concerns, etc... They were all there once. The parents all graciously shared their experiences with us. They assured us that it gets easier and less scarier and just becomes "normal."
Michael and I were both granted some time with the doctor. He felt that her case actually sounded more like a muscular Type I. Since the only problems we have seen are after shots (muscular). He offered a better way to administer her shots that we will try this month (ugh...shot time already). He gave us all the hope in the world with her case. It was a very nice evening....meeting new friends, having a nice dinner and feeling very encouraged.
I saw my first port in a 2 year old with mild hemophilia. Neither of the severe cases had it but the mild did??? Goes to show the type does not exactly predict problems. That baby resulted with a brain bleed after birth (again thank you Jesus for my c-section that unknowingly protected my baby). For the first time I was with people that got it...my fears, my concerns, etc... They were all there once. The parents all graciously shared their experiences with us. They assured us that it gets easier and less scarier and just becomes "normal."
Michael and I were both granted some time with the doctor. He felt that her case actually sounded more like a muscular Type I. Since the only problems we have seen are after shots (muscular). He offered a better way to administer her shots that we will try this month (ugh...shot time already). He gave us all the hope in the world with her case. It was a very nice evening....meeting new friends, having a nice dinner and feeling very encouraged.
Monday, January 17, 2011
For infusion of Humate-P
In November we went into Santa Rosa for her 3 month check-up and for her next round of immunizations. As always, I took her Humate-P. Every precaution was taken to prevent a bleed but after all of that when the bandages were removed all 4 shot sites were still bleeding. It was decided that she would be given Humate-P. We used the one I brought since it was readily available.
I cried and cried. The administration was much faster and smoother than I thought it would be. 5 seconds top. And within 15 minutes, she was not bleeding anymore. The last time she got shots (the shots that lead us on this journey) she bled for 18 hours. As disappointed that I was that she could not clot on her own and maybe all this VWD talk was nonsense, seeing the ease and proficiency of Humate-P gives me a piece of mind. She has a bleed that won't stop, we give her Humate-P and everything is ok. I'll take that.
I cried and cried. The administration was much faster and smoother than I thought it would be. 5 seconds top. And within 15 minutes, she was not bleeding anymore. The last time she got shots (the shots that lead us on this journey) she bled for 18 hours. As disappointed that I was that she could not clot on her own and maybe all this VWD talk was nonsense, seeing the ease and proficiency of Humate-P gives me a piece of mind. She has a bleed that won't stop, we give her Humate-P and everything is ok. I'll take that.
First major accident
A week after her first birthday, we were on the back porch and she fell on the tile. She fell very hard. When I picked her up I expected to see alot of blood. She did not bleed at all but she did end up with a black eye. She was checked out by her hematologist and no intervention was needed. She had a black eye for a month, but to the hematologist's surprise, it never spread over to the other eye. However, months later I can still see the faint lines of her black eye. We were all amazed at how well she dealt with this hard fall. A type III should have had more problems. Go Camy girl!
What does the diagnosis mean for Camdyn?
What we know:
* we have an amazing team that is taking excellent care of her.
* we have access to Humate-P, have it at home, daycare, my parents and carry it with us when we travel, etc...
* we have given it to her once and it works and she does not have reactions to it.
* Other than bruising easily and having trouble clotting after shots, she has not had any problems.
* She sees her bleeding team every three months for check-ups.
* All immunizations (shots) are given at Santa Rosa, with her team and many precautions are taken.
* We will continue to advocate her care and seek any and all that we can in terms of advances, etc...
* For now, we only will give her Humate-P if she has a bleed that requires it. If she starts having bleeding issues in joints, etc... we will give it to her prophylactically.
* HEB is so amazingly ordering her Humate-P for us for less than $5 a dose. It costs thousands for one dose without insurance.
* She can have babies one day if she wants.
* She can do any activities that she wants, except contact sports...don't want her playing those anyways :)
* Advances are there to one day replace her missing gene and make this go away. One day that I believe will be in her lifetime!
* She has no idea that she has this and I am thankful that by the time she is old enough to understand it will already just be a way of life for her.
* I am glad that she is unable to understand our sadness and grief the last 6 months. We are finally accepting it and she will only remember it as such.
* We don't think about VWD everyday. We are trained, educated and prepared. Let's not dwell on the unknowns or what-ifs.
* She inherited it from both Michael and I. We had no family history of it beforehand.
* Caden might carry the gene (TBD) but does not have the symptoms.
* She has a social worker through Santa Rosa that will handle educating her schools, caregivers, etc... Camy will have more bruises than the average child. 30% of VW patients' parents are turned into CPS. For this reason, Megan will go in and talk to others about Camy's condition as well as train them in what to do if she has a bleed.
* I have attended a bleeding conference, met another TYPE III mother and in a mini support group with other moms.
* I got burned by the other Type III mom- she is also a drug rep for a company who sells Humate-P and I think she just befriended me to sell me Humate-P.
* I belong to the Hemophilia and Bleeding Disorder chapter of Texas. Through this organization, I will raise money and proudly walk/run for my daughter.
* She walks and falls and bumps into things and is just fine. It does not slow her done in the slightest.
* Michael and I vow to allow her to lead a normal life. We don't want to put her in a glass house and deprive her of experiences and activities.
* If she has a bleed we know First Aid to treat it...if that does not work, we grab the Humate-P and go to Santa Rosa.
* She does not bleed more or faster than someone else. She just does not clot. She also has no problem replacing the blood that she looses so far.
* I can smell her blood from a mile away. From the moment I first smelled her blood in the hospital after her PKU test, I can identify her blood immediately. It is a very strong smell to me that smells of iron. I have no doubt that it is by no accident I have this strong reaction to her smell. It has no smell to everyone else.
* Ironically, I have a strong aversion to seeing blood. I faint. I have worked very hard to overcome this for my baby.
* Michael and I will be trained to administer her Humate-P when she gets older. It is given through a small IV.
* Once she is given Humate-P, her blood is completely normal for the next 12-18 hours. It clears up her bruises too!
* Humate-P is a human blood product, not a manufactured med. There has never been a case of blood contamination from Humate-P and it has been on the market for almost 40 years.
* There is another Type III girl treated at Santa Rosa. She is in her second year of college. She occasionally takes Humate-P but has never had a major problem or set-back.
* we have an amazing team that is taking excellent care of her.
* we have access to Humate-P, have it at home, daycare, my parents and carry it with us when we travel, etc...
* we have given it to her once and it works and she does not have reactions to it.
* Other than bruising easily and having trouble clotting after shots, she has not had any problems.
* She sees her bleeding team every three months for check-ups.
* All immunizations (shots) are given at Santa Rosa, with her team and many precautions are taken.
* We will continue to advocate her care and seek any and all that we can in terms of advances, etc...
* For now, we only will give her Humate-P if she has a bleed that requires it. If she starts having bleeding issues in joints, etc... we will give it to her prophylactically.
* HEB is so amazingly ordering her Humate-P for us for less than $5 a dose. It costs thousands for one dose without insurance.
* She can have babies one day if she wants.
* She can do any activities that she wants, except contact sports...don't want her playing those anyways :)
* Advances are there to one day replace her missing gene and make this go away. One day that I believe will be in her lifetime!
* She has no idea that she has this and I am thankful that by the time she is old enough to understand it will already just be a way of life for her.
* I am glad that she is unable to understand our sadness and grief the last 6 months. We are finally accepting it and she will only remember it as such.
* We don't think about VWD everyday. We are trained, educated and prepared. Let's not dwell on the unknowns or what-ifs.
* She inherited it from both Michael and I. We had no family history of it beforehand.
* Caden might carry the gene (TBD) but does not have the symptoms.
* She has a social worker through Santa Rosa that will handle educating her schools, caregivers, etc... Camy will have more bruises than the average child. 30% of VW patients' parents are turned into CPS. For this reason, Megan will go in and talk to others about Camy's condition as well as train them in what to do if she has a bleed.
* I have attended a bleeding conference, met another TYPE III mother and in a mini support group with other moms.
* I got burned by the other Type III mom- she is also a drug rep for a company who sells Humate-P and I think she just befriended me to sell me Humate-P.
* I belong to the Hemophilia and Bleeding Disorder chapter of Texas. Through this organization, I will raise money and proudly walk/run for my daughter.
* She walks and falls and bumps into things and is just fine. It does not slow her done in the slightest.
* Michael and I vow to allow her to lead a normal life. We don't want to put her in a glass house and deprive her of experiences and activities.
* If she has a bleed we know First Aid to treat it...if that does not work, we grab the Humate-P and go to Santa Rosa.
* She does not bleed more or faster than someone else. She just does not clot. She also has no problem replacing the blood that she looses so far.
* I can smell her blood from a mile away. From the moment I first smelled her blood in the hospital after her PKU test, I can identify her blood immediately. It is a very strong smell to me that smells of iron. I have no doubt that it is by no accident I have this strong reaction to her smell. It has no smell to everyone else.
* Ironically, I have a strong aversion to seeing blood. I faint. I have worked very hard to overcome this for my baby.
* Michael and I will be trained to administer her Humate-P when she gets older. It is given through a small IV.
* Once she is given Humate-P, her blood is completely normal for the next 12-18 hours. It clears up her bruises too!
* Humate-P is a human blood product, not a manufactured med. There has never been a case of blood contamination from Humate-P and it has been on the market for almost 40 years.
* There is another Type III girl treated at Santa Rosa. She is in her second year of college. She occasionally takes Humate-P but has never had a major problem or set-back.
Meeting our team at Santa Rosa
You have to remember our first experience with a hematologist and the trauma that we had been through up to this point. We reluctantly entered the hospital. I still have problems with the hematology floor being the same as the cancer. We met Trisha, Megan and Dezarae....Camdyn's amazing team. From the start it was a completely different experience. They were warm, caring and informative. I cried the whole appointment and they did not judge me for it. They did want to run their own tests. They informed us that only two labs in the US run VWD tests and it will take 1 month to get the results. They checked out Camdyn and thought she looked great. And set an appointment to see us in one month.
We were given literature to read, invited to attend a conference and given their cell phone numbers to call. Camy's blood was drawn, tests sent off and we went home. The next month was life as normal as it could be (as we prayed and cried) waiting for next month.
At our next appointment, we were told the tests were inconclusive. The results showed less than 10% VW factor but the lab noted that her blood clotted. They had never seen that before. Our team decided to proceed with her as Type III but we would test later on when she gets older. They will also be doing genetic testing on Caden, Michael and I later on.
Points of note for those who want to know more of the medical details:
* When she was in the hospital, she was given DDAVP and she did not clot- more justification for TYPE III.
* VW is one of the 12 factors found in every one's blood that helps us to clot. Someone with VWD does not clot normally. Type 1- decreased levels of VW but will eventually clot. Very common. Estimated that 1 in 4 people have it. Signs are heavy or long menstrual cycles, bruises easy, gums bled when brushing teeth, etc... Type II- normal levels but does not bind right to form clot. Two subtypes of Type II also exists. Type III- rare and the severest. one in one million. Must receive a recessive gene from both parents. Causes less than 10% VW factor in the blood. Patients will not clot. Must be given an infusion of VW factor (Humate-P). Can live a very normal life if condition is identified and has access to Humate P. Patients can experience internal bleeds or joint bleeds and can cause life-threatening conditions or permanent damage if not treated.
We were given literature to read, invited to attend a conference and given their cell phone numbers to call. Camy's blood was drawn, tests sent off and we went home. The next month was life as normal as it could be (as we prayed and cried) waiting for next month.
At our next appointment, we were told the tests were inconclusive. The results showed less than 10% VW factor but the lab noted that her blood clotted. They had never seen that before. Our team decided to proceed with her as Type III but we would test later on when she gets older. They will also be doing genetic testing on Caden, Michael and I later on.
Points of note for those who want to know more of the medical details:
* When she was in the hospital, she was given DDAVP and she did not clot- more justification for TYPE III.
* VW is one of the 12 factors found in every one's blood that helps us to clot. Someone with VWD does not clot normally. Type 1- decreased levels of VW but will eventually clot. Very common. Estimated that 1 in 4 people have it. Signs are heavy or long menstrual cycles, bruises easy, gums bled when brushing teeth, etc... Type II- normal levels but does not bind right to form clot. Two subtypes of Type II also exists. Type III- rare and the severest. one in one million. Must receive a recessive gene from both parents. Causes less than 10% VW factor in the blood. Patients will not clot. Must be given an infusion of VW factor (Humate-P). Can live a very normal life if condition is identified and has access to Humate P. Patients can experience internal bleeds or joint bleeds and can cause life-threatening conditions or permanent damage if not treated.
The first Hematologist
At this point, we were still in a state of disbelief, confusion and overwhelming fear. The first hematology appointment was a nightmare. The hematologist we had been sent to was cold, blunt and mean. He also happens to not be very good at what he does....wait for the rest of the story.
He basically told us that she needed more tests done and that he would have the results the next Monday (week later.) He wanted her to continue taking Amicar (which gave her horrible diarrhea that was causing weight loss). Not knowing any better, we followed his directions. Later that week, sooner than he said, we got a call from him. We were told that she had a virus that caused an inhibitor in her blood and that she would heal fully and we should never experience any other problems. How ignorance is bliss! We were ecstatic! There was nothing wrong with our baby and we went on with life. I was going to California the next week so I kept with those plans.
The following week was the worst and most trying week of my life. Two days later, the hematologist calls back and says, "I think the test was wrong. I think she has a severe problem and you need to bring her in right away." He had sent her blood to two labs and gave us the initial results without waiting to get back all of the tests. We were told that Camdyn had less than 10% Von Willebrand levels and that the lab noted TYPE III. He said that he would have to put in a port to give her meds everyday and just see how things went along. He also wanted to run more blood work. I refused. I cried. I told them I wanted a second opinion. We left.
We began researching other pediatric hematologist and Von Willebrand's.
My research terrified me. The stories that I could find of Type III were horrible. I cried most days, all day long. We did not know what to do or how to fix this. A few days later, Michael had an appendicitis. He was rushed into emergency surgery. Within a week's time, I was told my daughter had a rare and severe bleeding disorder and that my husband could die if they did not get him immediately into surgery.
Thankfully, Michael had a full recovery. When he returned to work, we received such loving outreach and support. Michael works for an amazing company. On his first day back, his company called the president of our insurance company and told him about Camdyn's condition. We received a call that afternoon that we could use the company jet and go to any hospital in the country. The insurance company would cover all medical costs and we were assigned to a special case worker. By the next day, we were given options to go to the Mayo Clinic or to try out the Bleeding Disorder Clinic at Santa Rosa Hospital in San Antonio. The case worker was actually surprised and impressed with the work that was being done right in our city. We got an appointment for the next week.
He basically told us that she needed more tests done and that he would have the results the next Monday (week later.) He wanted her to continue taking Amicar (which gave her horrible diarrhea that was causing weight loss). Not knowing any better, we followed his directions. Later that week, sooner than he said, we got a call from him. We were told that she had a virus that caused an inhibitor in her blood and that she would heal fully and we should never experience any other problems. How ignorance is bliss! We were ecstatic! There was nothing wrong with our baby and we went on with life. I was going to California the next week so I kept with those plans.
The following week was the worst and most trying week of my life. Two days later, the hematologist calls back and says, "I think the test was wrong. I think she has a severe problem and you need to bring her in right away." He had sent her blood to two labs and gave us the initial results without waiting to get back all of the tests. We were told that Camdyn had less than 10% Von Willebrand levels and that the lab noted TYPE III. He said that he would have to put in a port to give her meds everyday and just see how things went along. He also wanted to run more blood work. I refused. I cried. I told them I wanted a second opinion. We left.
We began researching other pediatric hematologist and Von Willebrand's.
My research terrified me. The stories that I could find of Type III were horrible. I cried most days, all day long. We did not know what to do or how to fix this. A few days later, Michael had an appendicitis. He was rushed into emergency surgery. Within a week's time, I was told my daughter had a rare and severe bleeding disorder and that my husband could die if they did not get him immediately into surgery.
Thankfully, Michael had a full recovery. When he returned to work, we received such loving outreach and support. Michael works for an amazing company. On his first day back, his company called the president of our insurance company and told him about Camdyn's condition. We received a call that afternoon that we could use the company jet and go to any hospital in the country. The insurance company would cover all medical costs and we were assigned to a special case worker. By the next day, we were given options to go to the Mayo Clinic or to try out the Bleeding Disorder Clinic at Santa Rosa Hospital in San Antonio. The case worker was actually surprised and impressed with the work that was being done right in our city. We got an appointment for the next week.
Months 1-6
The first 6 months of her life were very normal. I did start noticing tiny little bruises on her legs and we made note that when she would scratch her face it would bleed and take a while to heal. I brought it up with the pediatrician on 3 different occasions but we were told that she was just fine and not to worry. At her 6 month appointment, I brought it up again. I had called in after her 4 month shots because I thought she bled to long after her shots. At that time I was told they must have hit a vessel and that happens sometimes and not to worry. I reiterated all my concerns about bruises and prolonged bleeding. It was the first time I ever heard Von Willebrand's Disease. Dr. Davis thought she could have it and that I should get tested first, since it is hereditary. That would be the plan. 5 minutes later the nurse came in to give her shots....
Her birth
She came into this world perfect in every way. She weighed 8.6 pounds and was instant love to our family. By God's grace, she was delivered via c-section. Camdyn's brother was almost 10 pounds and out of fear of another big baby, my doctor suggested a c-section. In hindsight this was a complete blessing. A vaginal birth could have caused a brain bleed for her and since we had no indication that she would have a bleeding disorder, this could have been a terrible scenario. But, God was taking care of us and we had an uneventful C-section.
At her birth, bleeding disorder were not at all on our radar. We had no major family problems and in fact, I had never even heard of Von Willebrand's. I did notice that she bleed alot after her PKU test but we left the hospital care-free with a perfect bill of health.
The trip to the ER and Diagnosis
By the time I got her home after her 6 month shots, her car seat was covered in blood. I called our pediatrician who was already gone for the day. The pediatrician on call suggested that I take her to the ER. She was taken back right away. Immediately the nurses were concerned about her bleeding. After pressure, gauze, etc...it continued to bleed. Tests were ran, an IV was inserted and we waited and prayed. First round of tests ruled out leukemia was a huge relief! They could not figure out why she would not clot. We were told that specialized tests would have to be done and we would not get the results for a few days. We were also told that if she did not clot after the next attempt that she would be admitted to the hospital. Finally, after 18 hours an ex-army medic used durabond and the bleeding appeared to stop. We were released and told to call a hematologist who would be getting her case on Monday morning. So, began our journey with a bleeding disorder.
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