Too Much and not Enough

 As you may recall I told you that Lucy has a syringomyelia and I've been getting advice from a neurologist who is not Lucy's doctor. She thinks it would be better to ask for Lucy to be referred back to the Children's hospital as the waiting list is a lot shorter, so I'm going to contact them and see if this is possible. 

Then I get more information on syringomyelia. It's a neurological order where CSF (cerebrospinal fluid) causing a fluid filled cyst on the spinal cord, a syrinx. Apparently Lucy has two and they were picked up on her MRI. They can be harmless and never detected, but if they cause problems then they need to be treated. 

Lucy has problems as we know. Things that can be cause by syringomyelia which Lucy currently suffers from are:

• pain
• pins and needles
• bladder problems
• bowel problems
• weakness in limbs
• stiffness in limbs and neck
• loss of balance
• headaches

The most common cause of syringomyelia is chairi malformation. But it can also be caused by spinal cord injury.

Lucy has had surgery on her cervical spine, in her notes it says from injury but I know that she wasn't injured it was idiopathic but the doctors wouldn't believe me, so I said she fell off her scooter (when she hadn't but at least it got her a scan and treatment) 

It was most likely caused by her EDS (Elhers Danlos Syndrome.) 

At the time of her treatment for her cervical spine I was told quite categorically that it was NOT chairi malformation but intercranial instability. I've been looking up the surgical procedure for Chairi malformation and it's exactly what she had, decompression surgery. She also had a spinal fixation which is a procedure for Chairi malformation for children with type 1 Chairi and EDS. I was also told that there was NO leakage of CSF but now, some 9 year later we find out that there was, otherwise she would not have syringomyelia. 

I really don't know what to think. If I send her back to the Children's hospital it means that they will be more understanding about me staying with her. But it also means she'll be under many of the same doctors who now appeared to have got things really wrong. 

Treatment, if she ever gets around to it, will possibly be a draining of the syrinx using a shunt. This is the minor of the operations available and hopefully she won't have to go through another decompression surgery. 

If she doesn't get treated it could get very much worse for her. People with this condition can have no symptoms and live normal lives, but Lucy already has symptoms and they could get very much worse. 

More Catheter problems and a Good Doctor

 Yesterday it was Lucy's scheduled catheter change. She wasn't too happy but she knew it had to be done. She stayed in bed until the District Nurse arrived at 11am. 

It didn't go well. The nurse got the catheter in and Lucy said it didn't feel right. The nurse insisted it was right but when she inflated the balloon to keep it in place it fell out! She tried again and said that she'd call back in 1 hour to see if it was working. 

Lucy knew straight away that it wasn't working and in 1/2 hour she was in so much pain I was on my mobile to call the nurse back. While I was waiting to get through, the house phone rang and it was her regular nurse. Lucy is supposed to have the same nurse every time because of her autism causing anxiety. We were so relieved and the nurse came straight away. She tried 3 times to put a new catheter in but it just wouldn't work. Lucy was getting more and more distressed. The Nurse said that she needed to go to A&E as by this time she was retaining quite a lot. (i.e. she couldn't wee so her bladder was expanding.) 

Lucy was really distressed and terrified that they would keep her in hospital again. 

We got to A&E and the place was the busiest I have ever seen it, and we've been there A LOT. My heart sank and Lucy was even more distressed. BUT, a nurse came straight away, took one look at her and put her in a cubicle. If the bladder gets too full then there is the prospect of damage to the kidneys. Lucy had kidney issues before which is why she was admitted to hospital. 

Lucy was looking about six months pregnant by now because of how full her bladder was and she was in a lot of pain.

A doctor came to see us and said because Lucy was just 17 yrs old and had be catheterised for so long he wanted to know why before he put another catheter in. He came back an gave me so much information my head was whirling. Before I get to that I'll let you know how Lucy got on.

It took another three attempts and two different nurses before the catheter was in and working, and it filled the bag straight away. It was terribly painful for Lucy but she was brave and so relieved afterwards. Then we were allowed to go home. We were there less than 4 hours! We've waited that long to be seen before. 

Now, for the information overload. 

Lucy had an MRI back in October last year. They found two syrinx.

A syrinx is a rare, fluid-filled neuroglial cavity within the spinal cord (syringomyelia), in the brain stem (syringobulbia)

We were told she has one on her spine and her brain. These probably come from when Lucy had Cranial Instability when she was 7 years old. It was corrected by surgery but she's never had a single scan or xray apart from the one immediately after the hospital. 

A couple of years ago she was taken to A&E when her neck was hurting and we were told she had a cyst but it would go away. They did not check to see if it was a syrinx. I'm not still not sure if I have a cause for complaint. 

The doctor told me that Lucy had already been referred to two of the best consultants in the hospital but the waiting lists were up to a year long.

The only other information he could give me was that the consultants would decide what they can do about the syrinx but in the meantime it was preventing messages from the brain that make the bladder and bowels work. So Lucy will need to use a catheter until we do know if anything can be done, otherwise her kidneys will be damaged too. He has promised to refer her back to clinic to learn how to self catheterise. 

He also told me that Lucy's pelvic floor is in a bad way. Another consultant will be dealing with this, again we have to wait a long time for an appointment. Best case scenario is physio therapy, worst is surgery. Surgery comes with the added complication of not working because of her EDS. 

It's good to have some answers even though they are vague. It's not good to know that my poor girls body is so screwed up.