- Most blood results are outstanding; but we do know the glucose fasting test (looking for CF related diabetes) was fine.
- Lung function; down significantly, but as expected since he has a current exacerbation, and he struggled to do the tests while coughing (they were better later in the day when repeated; mornings are not friends with his lungs).
- Psychology review; yep, confirmed that he gets pretty cross whenever talking about CF.
- DEXA bone density scan; results outstanding.
- Sputum lab results; outstanding.
- Dietician review; his diet is great, but he needs to up his creon dose (pancreatic enzyme replacement) and to take it more reliably.
- Chest x-ray; not too bad, considering current infection.
- Portacath (his implanted venous access device); looked OK on X-ray (there were fears that it might be broken, as he’s had pain during flushes) but he was accessed today fine. We think the pain may be related to the scar tissue he has around the site from a while back when it got quite sore.
- Physiotherapy review; all fine, but his adherence to treatments when he’s left alone to do them is lacking (e.g. lower last week, while on half term). We will also drop one of his six nebulisers a day, as they would rather he did five really well, than six badly. Plus the sixth is using DNAse twice daily, which it is not licensed for, but he had prescribed after black October (read more here) as his secretions were so ridiculously thick.
- Ultrasound; enlarged spleen and fatty liver, indicating CF liver disease. He will start a new treatment for the liver, the spleen we already knew about, and is associated with the number of infections he has had.
- Doctor review; the fact that his lung function has picked up as much as it has after black October, is great, and this year has been better than the previous year. They praised his hard work to stay well. Despite his current exacerbation, they are really pleased with him.
- He may be referred to Orthopaedics to check what appears to be a curvature of his spine. He was scanned for possible scoliosis a few years ago, and if memory serves me right, it was said then that he may have a very mild case (no action required) but apparently this is something that can become more significant in teenagers. His consultant will refer his X-rays to see what they think.
Plan; We’ve started IVs today and he had the first dose in clinic. Bloods tomorrow (and twice more next week) to check antibiotic levels in his blood. He will start treatment for the liver issue next week (just tablets). They hope he will be well enough for his sinus surgery next week, and they will do a BAL while he is under GA (hoover up some more sputum from further down, to test in the lab for infection). They may also do a bronchoscopy at the same time, subject to availability on the day. Await further test results.
Sorry, nothing particularly poetic or insightful here. The day was full on, and he came home feeling sick and has gone to bed. I feel like I have no edges; I’m all blurry. Like most years, it was a mixed bag, but it wasn’t awful either. So proud of him.
Have a great day, if not today, then tomorrow x
Two weeks of IV drugs, a stocked meds cupboard, and a dogs bottom.
