Second PAM Treatment

Well at least this time we know what to expect I guess. The last treatment was very hard on all of us especially Emily, she had no idea what was going on and she refused to sleep.

This time I think we are much more relaxed, we know what we need to pack - last time we kind of just took everything we could possibly think of and hoped for the best. I have learned little things, like pack one bag of me and Emily, there is no way I am going to make it home to shower and change like I expected to the last time.

I know that the nurses know nothing about OI or how to handle a child with OI. I know that they can not under any circumstances us a blood pressure cuff on her, and I know that I can not allow anyone to bully me. I am there to protect Emily and make sure she gets the best treatment available.

I have made an information insert to put in Emily's file so every nurse that deals with Emily has a cue of what they can and can't do. I start sounding like a stuck record every 2 hrs having to explain to a new face what they can and can't do. Here is Emily's letter to the nursing staff. My mother in-law used to do this for Mark when he had to go into hospital, guess she got tired of educating everyone as well.

Hello

My name is Emily Ann Nilsen and I will be 13 months old on the 16^th of November. I have Osteogenesis Imperfect or Brittle Bone Disease. This means my bones break VERY easily.

I am here getting treatment that will make my bones stronger so I can grow up to be big and strong!!

I have had two broken legs in the last 5 months!! Please be careful with me and I don’t want to be in a cast for Christmas.

Here are some things you can do to help me to not break any more bones while I am

Please don’t use a BP cuff on my arm as the pressure could break my humorous.

Please don’t pull on my arms or legs.

Please don’t twist me in any way especially my arms or legs.

Please don’t make me do any sudden movements.

Please don’t pick me up

Please ask my mom (Tammy), dad (Mark) or granny (Sylvia) to help with anything you may need.

Please wash your hands lots, I cannot have physio if I get sick as it will break my ribs.

Thank you for helping me to get stronger

Emily

Emily's 1st Birthday

Emily turned one on the 16^th of October. I can’t believe it’s been a whole year already. This tiny little yellow (she had jaundice) baby that came home a year ago. Is now this big happy smiling little monster that is making me so happy!!

Emily is a complete joy, I really couldn’t have wished for a more perfect child. We did so much for your birthday it was almost silly. I made cupcakes for Mark’s office, we planned a birthday party. That had more adults than children. And Emily was spoilt rotten. She got so many gifts and she enjoyed playing with the boxes and wrapping paper more than what was inside them. We had been singing happy birthday to her for about a week so she would recognise the song when we sang to her at her party and she got all shy and tried to hide her face in my shoulder.

My mother got her the most stunning Winnie the Pooh birthday cake and my mother in law worked so hard to make the day absolutely wonderful. Both her granny’s love her so much!! Emily when you are bigger you are going to love going to both grannies. I can see how much they are going to spoil you.

It’s been a hard year, and a very busy one. Emily was born, and that was a very steep learning curve. Then Mark and I got married. Then we learned that Emily had OI and her first fracture. Then we had our first pamidronate treatment and Emily had her second fracture. We have had to learn to adapt quickly, but we have had a lot of support along the way.

I was made aware of how wonderful our family is and how many people care about us. I am thankful for this and I think I need to remind myself of it more often.

Emily's First Femur Fracture

Emily fractured her left Femur on Thursday the 8^th of October, 8 days before her first birthday. I had managed to half convince myself that her last fracture could have possibly have been her last. Silly me. I know that was never going to be possible but I was trying to believe it.

She was swimming in the pool with her swimming coach, and we couldn’t explain how on earth she had managed to fracture in the water. We have now, after many hours of debating how it was possible. Decided that she must have kicked the step as she went under the water. There is no way she could have fractured with no trauma. There has to be some explanation. Then I realised that there are going to be fractures that we aren’t going to be able to explain or justify. There are probably going to be fractures that we will never know about. Mark kept fractures that he had a secret from his parents. Why should Emily be any different??

Our little angel had to have her first anaesthetic to have her first spica cast put on. I hate casts. I guess best get used to them. She was not a happy camper, well I guess no one would be in that situation. But as usual she came through with flying colours, by that afternoon she was even smiling. I wouldn’t have been but she was. The next day she was a little bit grumpy (well that is a mild description). Then the poor thing has a heat rash like nothing I have ever seen and she starts teething. I couldn’t believe it, why does this all have to happen at once!!!!!!

I coped fine for about 4 days and then the banks burst, I just couldn’t. I was once again completely sleep deprived, exhausted and at my wits end. I could stop her hurting I couldn’t stop her crying and then I couldn’t stop me crying. Then came being angry all over again. I thought that I had gotten through most of that with the first break. I was angry with everyone including my tiny little baby. No one had done anything, they had all been incredibly supportive and helpful and kind. But I was angry. I had one completely self indulgent day where I ignored my child and husband … and then so very felt guilty about it. But after that I felt much better. I just needed a break. I needed to be able to see her and to make sure she was ok but not be responsible for her every move. My poor husband, in his completely exhausted state he was now made responsible for Emily. He was wonderful, he didn’t get angry or frustrated with me (I am sure he was, but he didn’t say anything) and I just stat and sulked. He is the most wonderful man. He is in short just amazing !!

Now I am back to being able to cope and finding things to occupy Emily’s time. I am sure it helps that she isn’t crying all the time anymore

OI Groups

I have been desperately looking for OI support groups and I have found some and they have been incredibly helpful. I recommend them to anyone looking for assistance or information.

I have also found a few blogs of parents whose children have OI. This is where I find my issue Emily has mild OI type 1 or type 4. I have come across a lot of parents whose' children are much more sever that what Emily is, and then I feel guilty for feeling the way I do about Emily having OI, it was always a possibility but it was something I so desperately hoped would never touch her. These parents are so happy to have these amazing children in their lives and they celebrate every triumph no matter how small their kids have, and I feel their joy and I am in awe.

I have the most amazing child. She is happy all the time, she smiles all the time, she hardly ever cries and she is just a joy. She eats well. She sleeps well and she is the most amazing thing ever, and then she has OI. Which I know is devastating to a lot a people and to us, but it still feels like it's tacked on to who she is as an afterthought and it doesn't define who she is or who she is going to be. I wouldn't change any part of her, but why do I feel so guilty for feeling sorry that she has OI. I know how good we have it and maybe that’s the reason. I don't really have anything to complain about. I have the closet to perfect child with OI. She doesn't think there is anything wrong with her so why should anyone else.

Maybe I need to try and look at it from a different point of view. which one i am not sure yet but I think it’s something I will work on.

Emily's first Pamidronate treatment

Emily’s first Pamidronate treatment, it had to be one of the most difficult things I have had to do. It goes against every part of me to hold my child still so they can put a drip up. She is so little and she doesn’t understand, and it breaks my heart every time. I am supposed to be protecting her!! I keep telling myself that it’s worth it that the time in hospital and the drips and the constant monitoring is all worth it. Only 12 days a year, only 12. I also think it’s harder because it’s the first. Let’s hope my theory holds true and the treatment in November is easier.

On the Saturday, they took blood in these tiny little vial’s and put the line in her foot for the drip. First he (Dr Roos) tried the left foot and the cannula didn’t hold so we tried the right. Which made me a bit nervous. Not of the line but because I had to hold it and we had only taken the splint off the day before. She didn’t not take kindly to any of this, she screamed and was most upset that this was sore. I wanted to take her away and go comfort her in the corner. Once we got through that it was ok we were ready to go. It then took 4 hrs to get the medication, as they only started the drip at 2:30pm and it ran for 6 hrs. So at the end of it I had a very tired ratty baby that was not impressed at all and they then had to flush the line which made her cry again. I hated every moment of it. I won’t go on about the night, we’ll just say that Emily does not like sleeping in strange places with strange noises. We did the this for 3 days and there were temperatures and a little vomiting and a little bit of bone ache (she was very ratty at one stage and I assume it was bone ache) But at least we got the drip going earlier in the day.

Dr Roos is the kindest man I have seen with children, he really makes you feel like he cares about your child and that the last thing in the world he would want to do is hurt your child in anyway. When he had to put the line in he actually sweats, he apologies constantly and really doesn’t want to hurt. I am confident in his care for Emily and I do trust him with her life.

Then you meet other moms with their tiny babies and they have cancer and they are on chemo and it makes me feel so guilty for feeling the way I do about OI. OI is not life threatening, she will survive this. She may break and there will be pain but it’s not slowly killing her. I will still have my little baby tomorrow and the next day. And all these little kids they are so very ill and feeling so sick and they always seem to have the energy to smile at you. I don’t know how those moms cope.

Pamidronate

Emily goes into hospital on Saturday for her first Pamidronate treatment. It’s a little scary because we haven’t done it before and although we are as informed as we could be, it’s still something new. We (Emily and I) will be admitted for 3 days. Hospital food, oh joy. But 12 days a year in hospital in comparison to 6 weeks in a cast or worse. I’ll take the 12 days in hospital.

The treatment is not a cure nor does it guarantee that she won’t break. But it is something that will make her bones stronger and my hope is that she won’t break as often. They have had amazing results with children on Pamidronate. I just want to have amazing results with my little one.

I sometimes find it difficult to express what it is that I am feeling. I have felt really angry lately and done a few things that have been a little (to put it mildly) out of character. The other day a stranger nearly took Emily and I out in traffic, he wasn’t paying attention, lucky I was. The accident was avoided but it made me so angry. The simple wave as an apology, and it was forgotten wasn’t going to wash with me today. So I followed him (not the most intelligent thing I have ever done) to a large shopping complex near our house. I got out the car and made him apologise to my 9 month old daughter for endangering her life. He thought I was completely insane. I really felt that he needed to be shown that his not paying attention could have cost a little girl her life. A life that I have become very protective of. So strangers beware.

I feel like I want to take Emily and Mark and hide away. I don’t want to see anyone or have to talk to anyone or be polite to anyone. I just want the three of us to have some time. I don’t know maybe we need some time to heal from all the heart ache over finding out about Emily having OI. Maybe I just need to heal and I want to take my little family with me. But back in the real world I have to put my big girl panties on and just get on with it.

Why ?

I have started this blog as a way to work through all the feelings and frustration I am feeling. My little girl Emily is turning 10 months on Sunday, she has Osteogenesis imperfecta (OI) and had her first break 5 and a half weeks ago. My heart broke.

She is so small and so helpless and has never done anything to anyone, what could she possibility done to deserve this?

My husband has Osteogenesis imperfecta (OI) and Emily has inherited it. We had hoped so hard that this wasn’t the case and everything indicated that she didn’t have it. The scans while I was pregnant, the x-rays when she was born, the opinions of experts and specialists. They all said that it didn’t appear that she had it. She did.

We have always been mindful that she might have OI so she has always been handled with care, we wouldn’t even let the nurses handle her in the hospital when she was born, we tried to educate everyone that come into contact with her. How to be more careful with her and always be mindful of her arms and legs especially.

But it turned out that I was the one who wasn’t careful. I think I became over confident in handling this precious little gift and I began to let her stand in front of me and hold onto my fingers. I should never have done, she didn’t have the balance and she fell. I heard the crack and I instinctively knew she had broken. Calling my husband was almost as hard as telling him Iwas pregnant.

But let’s go back a bit:

I was never meant to fall pregnant, we had only been seeing each other romantically for about 3 months when I fell pregnant. We had however been friends for close on 10 years by that stage. I knew Mark had fears of having children and passing on OI to a child, we had talked about it. I never in a million years imagined that we would fall pregnant.

It was a arbitrary Monday and I had a suspicion that something might be up, I was 3 days late or what I thought was 3 days so I went and got two home pregnancy tests from the chemist. I called my best friend Carrie, who happened to be 700 km away and now we were going to do these tests. I was shocked when the test didn’t even seem to contemplate the diagnosis. I had taken these tests in the past and they seemed to always take a really long time to give a result. Not this time. The problem came in when I was trying to take the second test. I couldn’t pee. So I had 3 cups of tea, a litre of water and still I couldn’t pee. So besides the flood of tears that my friend was having to deal with, I was now giggling hysterically. For some reason the fact that I couldn’t get my body to perform a simple task like pee seemed absolutely hilarious to me. When I finally got my bladder to obey it kind of thought it would punish me and I peed basically on the hour for the next 4 days. But it was confirmed that I was pregnant.

And all I could think was, I will never be able to go back packing through Europe. I had never planned to go back packing through Europe but now I couldn’t and I was devastated.

Telling Mark was a completely different story. I wanted to wait till the weekend when he was going to be coming to see me (we lived 700km apart at that stage) I didn’t want to tell him on the phone. My friend Carrie pleaded with me not to leave it, she know I wouldn’t be able to keep this from him for a week. Who was I kidding. So I hung up with her and called. The conversation when a little like this. This is through my tears, and I am not a pretty crier. My voice shakes, my nose runs and I sound like someone has just driven over my puppy.

Me : Hi Mark

Mark : Hi, what’s up? Are you OK?

Me: *sniff sniff* I’m pregnant.

Mark: Oh f@#*, I’ll call you back.

End of conversation.

Now this is not what I was expecting. Apparently he was sitting chatting to his mother at that precise moment. Telling Marks parents wasn’t easy either. His mom made the telling part easy, but she was very concerned about me having the baby. Having a baby with OI seemed to be the most horrific possibility. Marks dad had a very emotional response, to put it mildly. My mother didn’t believe me, to begin with. But they all came round in the end.

While I was pregnant, everything seemed to go so well, easy. I read a lot about OI and how it affected people. But none of it really sank in too much. I knew the facts, but living it was a completely different story.