Hello to all. I am posting on behalf of my husband who is too ill to do so. He is 64 years old, diagnosed with PSC in 1997, has been followed by 3 GI docs over the years who ordered twice yearly labs & Ultrasounds.All was good until July do this year. His Hepatic panel showed marked elevations in Bili, AST & ALT. MRai done showing considerable narrowing in 2 bile duct branches, so GI referred hubby to specialist in the big city university-affiliated hospital. Saw him 5 days later & he did 2 stents 4 days after the consult. Procedure went great. But 3 days later hubby developed diarrhea, the next day a raging temp of 103.5, so back to big city hospital’s ER & subsequent admission for lots of Zosyn & Vancomycin. Stents removed and external right biliary drain placed then home after total of 5 days in Hosp. Things went well, drain was draining nicely for a mere 4 days, then bad pain & no drainage for over 12 hours, so back to big city ER again. This time, more ABX, pain med IV and replacement of the drain tube. After 18 hrs in ER, we were good. Pain escalated over following 2 days, pain med not helpful, then overall edema increased.Saw his regular GI who quickly admitted him to local Hosp & he was transported to big city hospital, 3 days after the 2nd ER visit. He was admitted again for Acute Bacterial Peritonitis so got lots more Zosyn & Vancomycin.BP’s were low and he had taken Ibuprofen ON THE ADVICE OF THE ER ATTENDING WHO DISCHARGED HIM AT THE 2nd VISIT, and he developed Acute Renal Failure. So, dialysis catheter place and he had 2 sessions. By then his creatinine was almost 5.0! Ta out 2 hours after 2nd dialysis treatment, he developed significant ascites, to the point where he couldn’t breathe. after being told by a 1st year resident that she’d review the chart and get in contact with the attending," I said No, you will get your Attending NOW-this is very urgent. Attending came in, saw the distinction, moved him to a private room and tapped his abdomen for 6 Liters! So now we added Albumin and more IV fluids to the mix. He was stable and kidneys were getting better slowly, so now they wanted to place a 2nd biliary drain, this time on the left. He comes back from Interventional Radiology looking like death warmed over. And Guess what, his bag was draining frank blood, very small amount, but frank blood all the same. Next day, his pain increased and CT done which “suggested the tube went through the stomach.” So now, surgeon gets involved and strongly advised surgery to repair the TWO perforations, yes, TWO. Now we have a true life threatening problem because of the peritonitis and the perks, and the kidney failure. But, the awesome surgeon & his fellow did a superior job, and they also placed the left biliary tube CORRECTLY. He was moved to Surgical ICU for 24 hours for intensive monitoring then moved to the brand new cancer center because the surgeon does a lot of specially GI surgeries, many Cancer-related, and all his patients go there. So now we are at Day 14 of hospital stay. Slowly he was improving. No food or drink for about 6 days due to the stomach surgery. Kidneys were turning around and things finally going in right direction, so HD catheter removed. Snow they decided to cap off the 2 biliary tubes to “see what his liver would do,” and the day he was supposed to be discharged, he got a temp, so back the drain bags went on, and more Zosyn ( he’d been changed to oral Cipro 3 days prior), blood cultures, a thousand labs and no discharge for 3-4 days. He was still getting Albumin IV and had a few units of PRBC’s, was ambulating better, and eating better. Mind you he had 2 more paracentesis procedures between the surgery and the foiled discharge. So they kept him another 10 days, having capped off the biliary drains again and they monitored that & his creatinine, which crept back up about a week after the surgery. So FINALLY, 4 weeks to the day after the admission #2, he came home.
However, he has had a lot of muscle atrophy from being unable to ambulate very well because of everything he was going through and, OH I FORGOT, he developed a right inguinal hernia 5 days before they discharged him! So now he has to walk holding onto his right groin to reduce the pain! Mind you, he is 165 lbs usually, 5’10.5" tall, so not big or fat. Now he’s got the ascites, and the hernia, feels nauseous a lot, eats half of what he used to eat, and without the ascites, probably has lost about 8-10 lbs since Sept. 9th. Now we are almost 8 weeks out from the initial stent procedure. GI specialist keeps saying Cancer, yet the biopsies and brushings done both times say no malignant cells. We feel like this guy has done all he can do and doesn’t know what else he can do, so sort of poo-poo’d us at appt. yesterday. We are looking into getting a 2nd opinion at Cleveland Clinic (5 hrs away) because he is in limbo again…So, everything that could’ve gone wrong, did, well, with the exception of death, and we are tired, upset, discouraged, and did I mention I am a Nurse Practitioner and am on the FMLA leave, but have to return to work by Nov. 19th or I have no job security? I can’t retire until 62 & I just turned 61, so losing a year of income and the medical benefits that enhance my husband’s health I surance would be rather painful. Yet, my husband is having a terrible time of things. In one week, I do not see him getting better. His labs are all great, but he runs low-grade temps every few days, so I figure that’s the PSC talking, maybe? If anyone is out there and can offer us up some kind of opinion, idea, hope, or encouragement, we sure would appreciate it. My husband went from a very physically active robust retired man to a very sick man in less than 6 weeks, and we both are hurting. Thank you.
Oh my goodness, that sounds like a living nightmare. I am so sorry that you both had to live through that, but thank goodness your husband did live through it. It sounds like the right call to get a 2nd opinion. Is there any family that could come an live with you for a spell to get through? Please keep us informed on how it goes and what you learn at Cleveland Clinic, and please let your husband know we're out here thinking of him as he hopefully regains some strength.
If you’re planning on going to Cleveland I would suggest University Hospital, next door to the Clinic, and see Dr.Post. I was diagnosed in 2010 and with the help of Dr. Post, Gastro Doc.,Dr.Fulton, Infection Doc. (3 klebsiella infections),Dr. Segal, Surgeon who replumed my ducts in 2010, and Dr. Banker, drain tube magician, who started with 10f then 12f, 14f, 16f and ending with 18f size drain tubes to enlarge my last good duct in 2013 iquite well
I appreciate the information. Should we contact the GI doctor, Dr. Post first? also, is there a place for us to stay when/if we are accepted as patient by them? Cleveland Clinic had some sort of program for that. Just wondered. We live about 5-6 hours from Cleveland. Thanks!
AADD72 said:
If you’re planning on going to Cleveland I would suggest University Hospital, next door to the Clinic, and see Dr.Post. I was diagnosed in 2010 and with the help of Dr. Post, Gastro Doc.,Dr.Fulton, Infection Doc. (3 klebsiella infections),Dr. Segal, Surgeon who replumed my ducts in 2010, and Dr. Banker, drain tube magician, who started with 10f then 12f, 14f, 16f and ending with 18f size drain tubes to enlarge my last good duct in 2013 iquite well
Sure will! Thank you so much for your kind words!
mom to psc teen said:
Oh my goodness, that sounds like a living nightmare. I am so sorry that you both had to live through that, but thank goodness your husband did live through it. It sounds like the right call to get a 2nd opinion. Is there any family that could come an live with you for a spell to get through? Please keep us informed on how it goes and what you learn at Cleveland Clinic, and please let your husband know we’re out here thinking of him as he hopefully regains some strength.
My story is quite similar to Gma's except I have made it through the next step, liver transplantation (at age 65), with many other complications. I was hospitalized for a total of 106 days. Fortunately my wife has been a very good caretaker. I am now 13 months post transplant and finally feel that the transplant has done it's job. I too had a dramatic weight loss pre-op but I have gained back almost 1/2 of the lost weight. Controlling the bowels has been a problem - for me, avoiding fats (esp. milk fats), a cup of guava tea ea. morning, and immodium 4X a day helps a lot. One of the reasons I had so many complications is that I got too sick but no livers of my blood type became available for several weeks. I was seen by the Cleveland Clinic (very impressed) but I had my surgery elsewhere.
Keep fighting, and prayers and good luck to you both!
EAD3
PS: See my posting on Dec. 7, 2013 for some other advice regarding transplants.
Prior to your transplant, my husband asks what type of options were presented to you, I.e. external biliary drains (ongoing) reattempt of stenting, etc. Just wondered what the cycle of events was for you once your PSC totally wrecked your liver. A summary is fine! Thanks so much!
Dear Gma,
I am hesitant to dive too deeply into the complications I had because it paints liver transplantation with bad colors and we try to stay positive at this website. Liver transplantation is a miracle and I'm glad I had it done. I had an elevated white count pre-op for unknown reasons which also contributed to the delay in my transplantation. Most people have a much smoother post-op course. Most people are out of the hospital in a week or two and feel better very shortly after transplantation.
Stay Strong,
EAD3
Also Stephen, the brushings & biopsies were of the bile ducts done during the surgery to repair the stomach perforations that Interventional Radiology caused when they couldn't advance the biliary tube into the left bile duct. My husband almost died from that and the complications that caused. The 1st brushings were done at time of initial ERCP 8/22/14 and were negative. We're aware that it is vitually impossible to brush all the bile ducts to get THE sample that shows cancer. But the Surgeon who repaired the stomach also re-did the botched left biliary drain and did biopsies & more brushings while he had Jim opened up. He spent 2 hours getting the drain placed appropriately. That surgeon does a lot of specialized GI surgeries, including cancer surgeries, and he stated there is no cancer there. Keep in mind that Jim has been followed by GI for 17 years and it wasn't until this August that his Bili started to go up & subsequent MRI showed the considerable narrowing, hence the ERCP with balloon dilation & stent placements that failed and ended up causing acute bacterial peritonitis, with a month-long hospitalization. That followed a 1 week hospitalization a few days after the stent placement-stents were removed & biliary tube #1 placed-the rest in in my initial discussion. It was bad..... I believe the surgeon because he was in there, looked at everything and did the bile duct biopsies. My husband did have a liver biopsy in 2011 that showed cirrhosis, about Stage 2-3 at that time. I know, as a Nurse Practitioner, that liver biopsies are not the gold standard for diagnosing PSC, but I thank you for your reply. I'll keep you posted as to my husband's progress in this situation.
Stephen Cox said:
I am sorry to read this horrendous story. This is so typical of PSC, something happens quickly but Doctors are never quite sure what is happening. A second opinion is definitely in order. GI says Cancer but tests don't support it.
Biopsies and brushings can be very inaccurate. When they do a liver biopsy, they can be removing a pice to review from a healthy part of the liver. As you know, the liver is a big organ. Brushings as well can be very tricky. So these two tests in my view are not enough, by themselves, to verify what is going on.
I hope your husband had a physical exam as well looking for Jaundice, ( the type that can only be seen in the corner of his eyes, complete blood work, check his liver for swelling or hardness.
I would get into the Cleveland clinic soon. Hopefully they will determine what exactly is going on. Please keep us informed. I wish you the very best.
What an astonishing experience! I feel for you and your husband. Cleveland Clinic is just about the best. The doctors debated whether I had bile duct cancer. I got a third opinion at Mayo in Rochester, MN. It was only abnormal cells.
I was hospitalized nine times with liver failure, but always bounced back in 2-3 days on Zosyn and Vanco. Finally, I was very sick. I have a new transplant liver now and am healthy again. I will keep you in my thoughts and prayers.