Tuesday, January 12, 2010

A Nervous Wait Yet Again

Its times like these that I am forever thankful for this blog, its a place I can come to put all my irrational fears down, a place just for me where I dont have to hide my feelings and my fears.

In early december Sarah underwent an MRI on her brain and abdo, primarily it was to have a look at her brain. As she was born so tiny she suffered an Brain bleed which is very common in micopremmies, scans at the time showed the bleed but only time would tell if it had caused any damage. Then after her cardiac surgery she started having absent seizures so they wanted to do a MRI to rule out any brain damage and CP. The good new was that there is no damage at all, a massive sigh of relief.

Since she waws undergoing the MRI under a general they decided they would also scan her abdo to check and see if she had any remaining pancreatic tissue and to detail her Anatomy.

There has been some long standing concern about her Bilary System. Of the documented cases of Pancreatic Agenesis 50% has malformations of there Bilary System and 30% had heart abnormalities. Being that Sarah didnt have any heart abnormalities ( the heart condition she had was related to prematurity - PDA ducts are very common among premmature babies), there was a high probablility that she was going to have some issue with her Bile Ducts.

I remember clearly my very first meeting with Sarah's Genetics Dr. Sarah was in NICU and a few weeks old, it was not long after John had told us that she had this rare condition and it was unlikely that she would survive as her Liver appeared to be failing rather quickly. The genecist came with these case studies and said it looked like Sarah had a problem with her bile ducts, especially given her early gut problems.

One way we would know for sure was for Sarah to have a HIDA scan and then came the bad news "She is just to unstable and small right now for it" She needed to be able to cope with a GA and she needed to be about 2 kgs.

You might all remember my countdown to 2kgs and the excitment that bought when she reached it. The 2 kg club was to be a massive milestone for Sarah. Firstly that was the magic mark in which she could have the surgery to reconnect her bowel and reverse her illeostomy and it also meant she could have the HIDA scan.

Well the surgery was booked and when she finally passed a bowel motion is was elation! Then the worse fear happened and she became septic and ill once again, she pulled through and the race was on to finally get her home. We decided together (John, myself, care manager) that since she was passing bowel motions and her LFT's were coming down we would postpone the HIDA scan.

So when the MRI results came in I had her General Medical Team come to give me the great new that her Brain results are absolutely fine.

Her endocronologist was on holidays and returned last week and she emailed us with the results.

In a nutshell, her MRI brain was normal which is great; her MRI abdomen showed no pancreas or gallbladder. We essentially knew about the absent pancreas from her ultrasound and surgical findings as a neonate, but I think it was worthwhile documenting that there was absolutely no visible pancreatic tissue given her recent swings in BGL. The absent gallbladder is not surprising as it originates from the same developmental tissue as the pancreas in the fetus. She also has some abnormalities of her bile ducts in her liver.
Her geneticist then called to say that she too had looked at her MRI and noted the Abnormalities of the Bile Ducts and explained it was called "primary sclerosing cholangitis" or PSC. It has been encouraging to know that her Enzyme counts are still just above normal and she hasnt got any real noticable Jaundice at this stage. The MRI shows that she already has beading of the Bile Ducts and the geneticist was going on a search to see if any of the other PA children had survived the Neonatal Period with PSC. She explained that there is no treatment and no cure, but a bandaid solution is a Liver Transplant, but Sarah wouldnt need one for probably another 2 years.

I have tried to be fairly optimistic and the past week Ive had numerous phone calls from RCH about these MRI results. Have you been told of the results? Have you been in contact with Gastro? Do you have any questions? Has Sarah been itching? Is Sarah well at the moment?

Well by Sunday night it was doing my head in. Why all the phone calls and email? What is the urgency? Surely if there was a serious problem her Gastro Specialist would have called, since this is his area of speciality?

So I emailed him.

My only other concern is her recent MRI results. Ive had emails and phone calls from her endocrinologist and her genetics Dr in the past week to discuss the results, but I figure that you are the expert in this area, so you would be the one to discuss the results with. We have an appt with you early March, can we discuss the results then?

From speaking with genetics I understand that it was going to be highly probable that there was going to be a issue with her bile ducts being that it comes with the syndrome, but the abnormility that was picked up on the MRI is it likely that it wont be progressive? in that its just part of the syndrome and its likely that its always been this way and its not progressive?
I guess what I wanted to hear was. "Yes Ive had a look at the MRI and we will discuss at our next appointment.

We are scheduled to see him in 6 weeks, I figured its not that far away. Sarah is currently in amazing health.

So yesterday I trotted off early in the morning to the health nurse for Sarah to be weighed, then I had another appointment and I hadnt even given my email to Tony a second thought.

When I got home yesterday our Social Worker had called asking me to call her back. So called her back we discussed a few issues at hand and then she said well Ill see you on Monday.

Monday? Whats on Monday? You must be mistaken. I have an appt with John on Wednesday and with Daryl on Thursday. Then she said did you not get Tony's message.

My heart sank. I froze for a moment, then pulled myself together and said hang on Ill check my emails.

And there is was

Dear Karina and Michael,

We certainly need to meet to discuss the MRCP results - I do have a clinic next Monday 18th Jan - would that be convenient? We could meet at 3:20 in the afternoon.
Our Social worker has said that he asked her if she can attend the appointment :( Going on previous experience there is only one reason a Social Worker is requested to attend a meeting.

So while part of me is optimistic that its simply that he has had a cancellation, I just know deep in my heart thats not the case, and what is so imprtant that it cant wait just 6 weeks until our next scheduled appointment.

So now I need to try and distract my mind for another 6 days thinking of all the possibilities of what is going to be told to us, why he has requested Social Work support at the meeting and what this all means for Sarah.

I guess its just so hard to have fought and fought and battled and battled to get Sarah to where she is today and I dont know whether deep down, I, Michael and Sarah has the strength for another battle, things have been going along so well in the last month, I just dont wont to hear any bad news right now.

2010 was meant to be the year of happiness and health and well its not looking to positive

Anyway I am signing off here, sorry for such a negative posting. I am just drained and needed to get that out somewhere


E. said...

Hey Kat,

Nothing much to say other than thinking of you guys, as usual.

Madmother said...

Please, please try and not worry on this for the next week. Do not google, do not project, wait and hear what he has to say.

Enjoy your beautiful little girl and her gorgeous smiles, and let next week bring whatever then.

I know how hard it is, but please, just try...

Tanya said...

Thinking of you & Sarah. The next six days will be very hard for your family. I hope things on Monday turn out to be more routine than you expect. xxxooo

Spiralmumma said...

Thinking of you. It all sounds so hard. Much love to you and darling Sarah xx.

Melissa said...

I don't even know what to say, sweetheart. I can feel your fear, and nothing we say will hold it at bay.

I hope that things go ok. I hope that whatever it is, the news isn't as terrible as you're thinking.

I (and so many others, clearly) will be thinking about you all.

Anonymous said...

oh sweetheart, please text me anytime please! I'm here and have tears for you and precious Sarah xxx SandyR

Donna said...

Hey Kat, just dropping by to give you the new blog address.

Thinking of you guys as always.