The Next Diagnosis

Hannah's quarterly visit in September 2007 also included her annual blood work and chest x-ray.  Her chest x-ray looked great and her pulmonary function test was off the charts good.  Only one problem, her liver enzymes were abnormal.  We hoped that it was just a lab error but a repeat test in December indicated that Hannah did have CF-related liver disease.  So here we go again, Hannah had been so healthy.  No one could tell she had CF, she played softball every summer since she was four, she was in the 95th percentile for weight (unheard of for a CF kid). 
What did this mean?  We made an appointment with a gastroenterologist at the end of December.  She ordered an ultra sound on Hannah's liver.  The results showed that her liver was enlarged and her bile ducts were partially clogged. She started taking a drug called ursodiol to help the liver.  The Doctor said that they had great results with the medication and we would repeat the utlra sound in six months.  Let's pray and deal with this new challenge.  By June the medicine had worked wonders ,and her liver was back to normal.
Hannah was really struggling in the beginning of 2008 with her respiratory health.  She was coughing a lot and spent weeks on oral antibiotics by April she was tired all the time and the coughing was getting worse.  When she started coughing so hard she would vomit, I knew we were headed to another hospital stay.  She hadn't been hospitalized for a lung problem since 2004.
Finally, she coughed and threw up a little blood.  To the emergency room we went, we actually went twice in 24 hours and she was finally admitted early in the morning on Sunday, April 6, 2008. 
When the Doctor came through that afternoon, he said she had a scratch inside her nose that caused the blood and she would probably go home the next day.  I was exhausted and not really in the mood to fight.  I new a CF specialized pulmonologist would be in on Monday and I would wait to see what he had to say.
On Monday, we were told that in the emergency room her blood glucose was 528.  She also took a pulmonary function test and her PFT's were down.  She was scheduled for a PICC line and a visit from Endocrinology.  She would spend the next two weeks on antibiotics.  She was also diagnosed with CF-related diabetes.  It was almost as bad as the original diagnosis for CF. 
Our little girl would now endure several insulin shots per day on top of everything else she had to do to live.
It wasn't long before Hannah was giving herself shots and it quickly became part of her life.  I have read that only 25% of people with CF-related diabetes live to be 30. 
Not a day goes by that I don't get that sick feeling in my stomach thinking about losing her.  We can't do enough.
Hannah would be hospitalized again in November 2008 for an intestinal blockage and would end the year with pneumonia and more antibiotics.
Here is Hannah's daily routine:
Wake up and check glucose
Take liver pill, prilosec and 3 enzymes with a glass of milk.  Use rhinocort nasal spray for her sinus problems. Take Miralax.
Do breathing treatments 15-20 minutes for albuterol, 15 minutes of hypertonic saline and another 30 minutes for
the vest which vibrates her chest to help clear her lungs.
Time to eat, check glucose, count the carbs, take more enzymes and inject insulin
She will check her glucose before every meal or snack and take insulin based on the amount of carbs she is eating.  Hannah will give herself insulin shots taking as many as 8 shots per day.  She will also take 3-4 enzymes each time she eats.
For dinner she takes another liver pill, prilosec, a special vitamin ADEK, 4 enzymes and another dose of Miralax because she has a high risk of another intestinal blockage.
Before bed, she repeats the albuterol and hypertonic saline, does the vest, plus she will do 15-20 minutes of pulmozyme a drug that helps thin the mucus then she checks her glucose one last time and takes lantus a long acting insulin meaning one last shot before bed.
Remember she is still a kid in school and needs to do homework, baths etc. too.  She plays softball in the spring.

Check the "cure 4 hannah" blog for the latest on Hannah and the entire Donoghue clan.