My 10 year old step-daughter (who was diagnosed with cf at 18months) had an enlarged spleen that was noticed on her chest x-ray, which led to and ultra-sound and blood work. They have told us that she now has cf related liver damage. They have started her on a medicine to thin her bile. This is her 3rd hospitalization in 1 year. Can anybody shed some light on how their/or their child's liver damage has effected them! We have looked on-line but that is just basic info not the real life version! Thanks!!
CF liver damage in a 10year old
- Thread starter SunnyK
- Start date
DS has been on actigall (urisidiol) since he was a baby. When in the NICU they noticed his liver levels were elevated and he was put on this medication. His annual CF blood work includes liver tests, which I thought was a common practice at CF clinics being that the liver, especially with double df508, can become affected. We were told that when he was a teen they'd probably monitor his gallbladder via ultrasound as well. Hopefully the medication will do the trick.
I've been on actigall from the phase III study and I know it has made a difference. I had liver damage when I started and we monitor my liver through blood work every other regular clinic visit, and an ultrasound every other year. So far, it has really worked well and the damage rate has slowed.
Odd thing to note, but finding out when I was 11 that my liver was already damaged really turned me off drinking once I reached legal age. Why aid in the damaging process?
Odd thing to note, but finding out when I was 11 that my liver was already damaged really turned me off drinking once I reached legal age. Why aid in the damaging process?
TreasureGoddess
Member
my son (14 yrs) has an enlarged liver & spleen and has been on actigal (ursodial) for about 4-5 years. He plays sports whenever he can. We monitor liver with annual sonograms, blood tests where they check liver levels (can't remember what part of the blood work they test or what they call that) and occaisionally he has an upper GI scope to check for damage to the esophagus which can sometimes happen with liver issues. It's just another pill and another thing we watch. We have a GI doc that specializes in CF kids and check in with him 2-3 times/year and for sure when my kiddo is hospitalized. Ask lots of questions at your next appt and also check into seeing someone in GI that works with people with CF. Ask them if it's safe for sports, but I'm pretty sure it should be. 
eganhouselisa
New member
I started on Actigall when I was in 5th or 6th grade. My spleen was also enlarged, but remained largely unaffected symptom-wise until I was about 25 when I would always get ascites and multiple other issues which landed me in the hospital quite a lot. Also, when I got in college had regular endoscopies to check for varices. When I was 29 I had a liver transplant (from my amazing living done (my mom's best friend)). My 7th year post liver transplant is coming up on Jan. 10th! I had a hard time finding out anything about CF and liver transplant - there is a lot out there about LUNG transplant, but not LIVER transplant related to CF. If you have any further questions, feel free to email me at: lisacantwell1212@gmail.com.
Take care!
Take care!
LittleLab4CF
Super Moderator
I was told to take milk thistle for liver toxicity. My liver isn't in bad shape like your little ones by any comparison so I would at least mention milk thistle to the doctor before giving it. Milk thistle isn’t overly powerful it is not harmful and does a decent job of liver detoxification. Consider adding to the daily routine.
LL
LL
juliefaler
New member
My daughter passed away March of this year at the age of 28. She had an enlarged liver and extremely enlarged spleen. She was put on actigall around the age eight. She too remained largely unaffected symptom wise. She was told to be careful of her abdomen. Her sports consist of soccer, dancing and swimming. Her GI doctor monitored her and she had blood tests to check her liver levels, annual ultrasounds and endoscopies. She was 19 when they decided her varices were at risk for bleeding and had them banded. They continue to monitor them to insure the banding was working. She had low platelets and they would give her platelets whenever she had procedures done. She was evaluated for a lung and liver transplant. The lungs were driving the transplant. As they were planning for the transplant, it became evident the extremely enlarged spleen was causing problems for the lung team as well as the liver team. The spleen was also causing pressure on the heart. After much consideration her only option/chance was to have surgery and first remove the spleen. Then list her for transplant. Obviously, removing the spleen comes with its own set of problems. Surgery was extremely challenging, they did find a accessory spleen (baby spleen) which is rare. Which could of benefit her. Unfortunately her lungs did not handle the surgery well, and her infections came out in full blast. She had no drugs left to find the infections and God called her to heaven a few week later.
TreasureGoddess
Member
juliefaler,
I'm so very sorry for all your family has gone through. Thank you for sharing your daughter's story. It's scary and sad, but it does help to know what things can happen so us other parents/people with CF & Liver & Spleen issues can know things we should be asking about or watching for. Hugs to you in this sad, sad time. I truly hope you and your family are able to remember good memories through the holiday season to help (as well as every day, who am I kidding?) It is a situation I don't know how I'll handle should the time come for us. Just thank you. very much.
I'm so very sorry for all your family has gone through. Thank you for sharing your daughter's story. It's scary and sad, but it does help to know what things can happen so us other parents/people with CF & Liver & Spleen issues can know things we should be asking about or watching for. Hugs to you in this sad, sad time. I truly hope you and your family are able to remember good memories through the holiday season to help (as well as every day, who am I kidding?) It is a situation I don't know how I'll handle should the time come for us. Just thank you. very much.