What happened to Star and Elhers Danlos Syndrome

This is the story of what happened to my 17 year old daughter Star, and how her Elhers Danlos Syndrome caused her to end up in hospital for nearly four days.

Friday 19th August began with a visit to the GP with Star. She had been for blood tests the week before and the GP said they would help her with her tummy problems. When we got there a different GP told us of all the vitamins that she’s lacking from her blood tests. Then she looked at me accusingly and asked, ‘doesn’t she ever eat?’ So, once again I explained about her tummy problems and how we are trying to find the foods she can actually eat without making her really poorly. So the GP wrote a request for a specialist to see her at the hospital.

Later her boyfriend came around and she was one happy girl. She was looking forward to going out for her birthday and excited about her gifts and special day. Then on Friday evening she told me she hadn’t been able to pee all day. I thought it was a bit worrying so I told her I’d get some medical advice in the morning and said she should get some sleep. I was completely shattered myself and I know she was too. She lasted a couple of hours, then my eldest daughter told me Star was crying in the bathroom.

Her tummy was swollen and she was in a lot of pain so I called 111. They told they could send an ambulance but it would take about 11 hours!! But they said she needed to be in hospital within the hour. We quickly got dressed and off we went in the early hours of Saturday morning along the dark and quiet roads.

Accident and Emergency

The A&E was not so quiet though. There was even some woman lying on the floor across the entrance refusing to go home. There were security guards that had to drag her away just so we could get in. Poor Star wanted to go back home straight away. We didn’t even get to say goodbye to her Dad as we were quickly hustled inside.

We booked in and was seen by a nurse after 2 hours. Star was really hurting, but there were a lot of really sick people there. When she was finally called things moved pretty quickly. She was taken into a room for an ultrasound and they could see how bad things were. She had more than 1300ml of urine in her bladder. Another hour and she could have easily burst it. A catheter was put in, the experience was a complete nightmare for Star, but she was incredibly brave. We have lots of coping strategies when things are tough to get through and she started talking none stop about the circus we’d seen on holiday, telling the nurse every minute detail. It was her way of taking her mind off things and it worked.

Then she was moved to cubicle where we spent 7 1/2 hours. Star managed to sleep a little. I did nod off a few times in wheelchair. I was dreaming of the wheelchair that Graham had sent me a picture of the day before, a reclining one with a headrest. Problem is, we could only afford it because it came from China and the reviews of the ordering and delivery process were dire. A wheelchair like that in this Country would cost us three times as much. Anyway, I digress!

Eventually she was moved up to a ward, but they didn’t have a bed. She was put in a reclining chair! In a lot of pain and not knowing what was going on she became distressed. I am used to these chairs as they are the type I have my treatment in, so I showed her how to adjust it so it became just like a bed and she was a bit more comfortable then.

The mobile phone was both a blessing and curse. Her boyfriend had no idea how poorly she was and ended up upsetting her by not giving her enough attention. By this time I’d been in the hospital with her for 14 hours and I was getting tired so Graham came and took over for me.

I came home and felt so bereft, my girl was so poorly, and I was so tired. The big kids cooked me dinner, Graham had already fed the younger ones. Then I went to bed. I heard from Graham that Star finally had a trolley bed on the ward so she could sleep. So, I went to sleep too.

In the morning, Graham called me to say that Star had been given an enema because they believed it was her bowels that had had caused her bladder to stop working. It felt good to have some kind of answer. I did a pile of washing, cleaned up and waited to hear about when she would be home. But then Graham called and said they were keeping her in another night and I packed a bag and went to the hospital to take over.

The Next Stage Was Messy!

Star had been given two enemas, please try not to imagine what that meant. Her catheter was still in and she was in a lot of pain. She was now in a proper bed in the older part of the hospital. It was a long way from the new hospital with restaurants, cafe and shop. My hopes of popping away for five minutes to get something to eat myself were dashed as it would have taken me a long time. Not only that but my wheelchair is become cranky (hence us looking at new ones) and I was scared to go anywhere alone in case it broke again.

We had a dreadful night. The enemas were really doing their job and the staff was really scarce (Sunday shift) so I ended up cleaning her up all night long myself. I had been given a fold up bed to use but I don’t think I managed more than two hours sleep all night. Poor Star was completely shattered too. She just wanted it all to be over so she could go home.

On Monday we saw a doctor and he bore good news. He said that they were going to remove the catheter and if Star could go to the toilet by herself then she could go home.

She was so happy but also scared. But the removal of the catheter was nowhere near as bad as putting it in. Then she went to the bathroom and pee’d by herself. She was ecstatic and we talked about going home. It was just two days to her birthday and she wanted to be home by then.

The Bad News

Then on Monday afternoon, after we’d told Graham to get ready to pick us up, we were told that she needed a scan before going home. Things moved quickly and a porter came and took her over to the new hospital with me trying to keep up behind. The scan was very detailed. We were a bit worried because she’d been told to drink a litre of water but couldn’t hold it so she’d already been to the toilet. But the sonographer said her bladder had 365 ml of urine in it so it was good for getting pictures of what was going on. Then Star was told to go and empty her bladder. While she was gone the sonographer said that she couldn’t tell me what was going on as it wasn’t her job, but not to get my hopes up about going home that day. Then Star came back and her bladder still had 335 ml of urine in it. Star was really upset and told the sonographer that she was wrong, she’d been to the toilet and there couldn’t be that much still inside. As we left the sonographer gave me a really sad look behind Star’s back and shook her head. Star was crying now, and I felt like crying too. What was wrong with my girl?

We had to wait for a doctor to tell us what was going on but they still hadn’t arrived by 7pm so we knew she wasn’t going home that day. I consoled her with the fact that she’d be sleeping without the catheter in. Then a junior doctor arrived and told us that Star had to have a new catheter put in. At this point Star really lost her calm and insisted that she wasn’t having it. I know the scan had been bad but I also know that Star was peeing by herself, even if she wasn’t emptying her bladder. I’d also heard one of the nurses say that catheters were not needed unless there was more than 400ml of urine in the bladder. So I stood by Star and we both refused the catheter.

I then spent the night worrying that I’d done the wrong thing, especially when her tummy began swelling again. Then at 4 am she went to the toilet and cried with relief as she had a really big wee. She also went again at 6.30 am and we took a sample bowl so we could prove she was going to the toilet. A nurse came in and scanned her bladder and she had just 60ml left. She said that meant she didn’t need another catheter and we just had to wait for the Doctor.

Elhers Danlos Syndrome.

Did you get this far? It’s a long story, but it doesn’t end here. Star was diagnosed with Elhers Danlos Syndrome when she was nine years old. The geneticist was also certain that she didn’t tick the boxes and wanted to do more tests. She was convinced that Star had Marfan Syndrome and ordered regular heart checks for her.

When the Doctor came to see us on Monday morning she was convinced it was her Elher’s Danlos Syndrome (EDS) which had caused her problems. She said her bowel, kidneys and bladder had all stopped working and couldn’t really tell us why. But it’s something that can happen with EDS. She was allowed to come home but is facing a lot more hospital visits.

She needs to have an MRI too see if there is anything stopping her brain from sending the correct signals to her organs. This could be an after affect from her spinal surgery when she was 8 years old.

She also needs to learn how to self catheter herself at home if this happens again. She’s not happy with this but she was assured that it’s nowhere near as bad as having it done in hospital. Some people have to live with this and it looks like she will be joining them. The doctor was talking about a self catheter once a week.

She will be having these complicated tests on her bladder muscles which sound quite horrid but I’ll be there with her to get her through.

We also have follow up scans on her kidneys and ovaries as they were both damaged while she was sick. They believe the ovaries will be fine, but are a little concerned about her kidneys. We’ll just have to wait and see how the scan goes.

Then we are waiting on an appointment for her bowels that the GP has requested. Maybe it’s bowel problems that caused all of this to happen.

Hopefully we can get through all these tests and get her sorted on the right medication for her future. She has her enrolment in college to complete this week so fingers crossed.

Here’s a photo of Star waiting to come home on Tuesday. She made it back in time for her birthday!