Friday, August 21, 2015

Metabolic Appointment- New Results

So today we saw Shanti at PMH the metabolic specialist. The day was off to a bad start with Miss L not feeling great and it pouring down with rain. We arrived at 11:10am for our 11:30am appt and couldn't find parking. We literally drove around the block until 11:45am and we walked in late and stressed. Thankfully the Dr wasn't waiting and we went in pretty much straight away. The nurse checked height- 106cm and weight 17.7kg which is a great sign. When I went in I wanted to know one thing, my DNA results. August last year was when my bloods were sent off to Atlanta for testing and we had no results. Shanti said the 13 known disorders they tested for all came back negative. So that was a bust and where does it leave us?

Shanti then told me something I didn't know. Miss A's blood results over the last few years showed her MMA levels were always elevated even if her B12 levels were within the normal range. My results and Miss L's were the same. Even after B12 injections the B12 levels increased but the MMA stayed high.

MMA explanation: http://www.mayomedicallaboratories.com/test-catalog/Clinical+and+Interpretive/80289

Methylmalonic acid is a substance produced when proteins (called amino acids) in the body break down. A test can be done to measure the amount of methylmalonic acid in your blood. When your levels of MMA increase it usually indicates a problem with your B12. So Shanti is saying the MMA levels should have gone down as the B12 levels went up. The fact it didn't shows there is still an issue but to this day there has never been another case like it. Then I had to sit there listening to an explanation of genes, recessive, auto dominant, cells etc and felt like I needed an interpreter. Shanti is a very fast talker but she is pretty good at explaining things as simply as possible. What we do know is that new disorders and defects are being discovered all the time. Only a few weeks ago a new problem was discovered by scientists in the Netherlands. Fascinating stuff.



So where to now? Shanti wants to do a full gene screening on the 3 of us and probably Andrew as well. It is likely he is a carrier to allow it to be passed on. A full screening means checking all 25,000 genes! Not the 13 we know about or a select few but every single one.Straight after leaving the appt with a spinning head we went in to get another blood test. L had just had one 2 weeks ago after her Neuro appt but this was more involved. I went first and as soon as L saw where we going she got upset and worked up. I hate needles and blood tests so know exactly how she feels. By the time they asked her to climb on the bed it was on! She was in full hysterics and couldn't be calmed down. I held across her shoulders another nurse had her legs and one held her arm straight. The the plebotomist started taking the blood and L was screaming, bucking and crying shouting no! I started crying then as I could see how she genuinely feared what was happening and had no control. I haven't cried at a blood test in years. It didn't help that my poor arm was getting kicked and hit just after they took blood. When finished she hopped off the bed and started hitting me and screaming. We slowly walked out and went down to the cafe. She wasn't calm but she had stopped crying. We always try and stop into the PMH Foundation cafe as the ladies have seen us for years and know us by name. The ladies were trying to offer her cake and chocolate and she kept asking for a burger. After explaining they didn't have any she picked a sandwich. We sat down where she kept crying and tried to eat one handed. The sore arm was held straight out and she wasn't using it. Thankfully after a few minutes she was calm and eating. Definitely not happy with me or the world but
under control. We walked back to the car in the rain and started back for school pick  up. 15 minutes down the road and she fast asleep in the car. I would normally enjoy that quiet time but it allowed my brain to start stressing about everything that had gone on and that wasn't a good thing. Time to let it go (like by writing it down here) and focusing on where to now. 4 years on and we are still looking for answers.  


                                             Thursday night at 5pm she crashed poor sick girl.

                                                      Not using her right arm after the blood test.







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