We had the meeting with our Gastro on Monday to discuss the results of Sarah's MRI. She has a condition called Scleroising Cholangitis, I had already read her MRI report and spoken to her General Paediatrician and we were hoping that she had Primary Scleroising Cholangitis but she however has Secondary Scleroising Cholangitis
Secondary sclerosing cholangitis (SSC) is a chronic cholestatic biliary disease, characterized by inflammation, obliterative fibrosis of the bile ducts, stricture formation and progressive destruction of the biliary tree that leads to biliary cirrhosis. SSC is thought to develop as a consequence of known injuries or secondary to pathological processes of the biliary tree. The most frequently described causes of SSC are longstanding biliary obstruction, surgical trauma to the bile duct and ischemic injury to the biliary tree in liver allografts. SSC may also follow intra-arterial chemotherapy. Sclerosing cholangitis in critically ill patients is a largely unrecognized new form of SSC, and is associated with rapid progression to liver cirrhosis.
Aside from transplantation, there are no effective treatment options for SC; the median survival of patients with SC who do not undergo liver transplantation is only about 13 months.We talked about a new medication we can try to slow down the progression of the condition and I asked the dreaded question of how long would her liver hold out before a transplant would be needed. He couldnt give us a answer but we will repeat the MRI in 6 months to see how quickly or slowly it is progressing, but he said it could be 6 months or it could take 5 years, so for now we place our heads back in the sand and he will let me know when to pull it out again.
We headed away last weekend back to my home town for the BILS 30th Bday, it was a great night (we were all in Fancy Dress!), however on Sunday Sarah spiked a raging fever and was starting to become irritable. I put it down to teething, we went to visit a few friends and headed some Sunday afternoon.
Monday morning she still had the fever, so gave her some panadol just to help bring the fever down, she was very happy in herself just had this fever that started out of nowhere, by about 10am she started getting a wheeze and at about Lunchtime she started grunting with each breath in and out. I called her Paeds know already knowing Id be making a trip through the ED. I said I had the appt with Gastro but then Id bring her to Emergency.
I could tell her Gastro Specialist was getting nervous with the grunting so we wrapped up the appointment and he came down to emergency with us.
Straight from Triage into resus, where she had the Drs abit stumped, she had a fever of over 40, grunting, resp distress but her oxygen sats were 99%, they put a IV in, got bloods and ordered a xray. Her paed team had now arrived and were sure that Sarah had Pneumonia, they wrote her up for IV anti's and said they would see us on the ward.
Well the Chest Xray come back and there was no change from her December Xray, so back to the drawing board, where was this fever and resp distress coming from?
They then though that maybe she had an ascending cholangitis infection which is a infection inside the bile ducts, but then her bloods came back and infection markers wernt indicative of that either.
So it left one thing to check and that was her urine, the hospital always do a SPA as its a sterile collection in babies, so it involves sticking a needle through the abdo and into the bladder and drawing out a sample of urine, she has had them in the past and hasnt had any issues, well this time they missed so they decided to use a cathetor.
One of the worst experiences to date, the Dr tried unsucessfully 3 times to insert the cathetor all the time Sarah was just screaming, the resus nurse had a try and thankfully she was able to get it in.
The sample looked clean, so they were again scratching there heads, her bloods were back and her White cell count was down, so they were thinking it was a viral illness, so the plan was admit for 24 hours and if fever went up again, start Anti's just incase.
By tuesday morning the grunting was gone, her blood pressure had returned to normal as it was 138/77 which is high for an adult let alone a child and her heartrate had also come back to normal range. So her Dr said we will just observe overnight again and if all is good will go home tomorrow.
So to my surprise on Wednesday they got the culture back on her urine and it had returned 2 bugs so Anti's it was to be, she was given one dose IV then switched to Oral Anti's.
Her Paed then came in to see me, we had a outpatients appt for today, but since we were in he came to our room to do it to save me coming back in tomorrow.
He started off with the chest xray. Remember how the xray had no change? Then it dawned on me. Her last xray was when she collapsed her lung after the MRI. Then he handed me the xray report
"Persistant Perobronchial thickening are present. Atelectasis bilaterally with further change at the upper right and left mid zones appears unchanged from Previousily. Appeareances are inline with Chronic Lung Disease"
So we are home.
We now add Liver Disease and Chronic Lung Disease to the list.
We are feeling very mixed emotions. The CLD is more than likely caused by the recurrent bronchiolitis, which she continued to pick up in the hospital. She will grow out of it and her lungs will get stronger again, but in the meantime she is more suspectable to respitory infections, which then triggers off her diabetes, which triggers off her Malabsorbtion and the vicous cycle continues.
I want to cry, scream, vent, and hide away.
But I dont, instead I smile and laugh because if I didnt, I would do the sentence above.