8. The Itching

‘Morning, John.’ I handed him a cup of coffee.

‘Good morning, Dr. Diaz. Have a great day.’ he smiled.

It was a Friday, the last day of November’s outpatient week. John was the security guard of the clinic and was always super friendly. I would bring him coffee every morning on my outpatient days. I have to say, I was a little sad that the week was ending already.

I looked at the schedule and saw that Dana was the first appointment of the day. That made me smile to myself. Dana White was my first outpatient on residency and just the sweetest woman. She was about 50 years old and came in regularly to control her hypothyroidism and rheumatoid arthritis, which were stable for the past year. Today was her scheduled follow-up.

‘Hi, Dana! It’s good to see you.’ I said while she was entering the room. She was an incredibly elegant middle-aged woman.

‘Hi, darling! I am happy to see you too. Have you been taking care of yourself? Are you eating right? You look a bit skinny.’

I laughed. ‘I am fine, Dana, but thank you, I’ll keep that in mind.’ Truth be told, I had lost a few pounds. What can I say, not that much time or energy to eat right. ‘Now tell me, how are you doing?’

‘Well, my symptoms are still under control with the medication, nothing new on that. But my skin has been itching a lot this last month. I’ve tried taking some antihistamines at home, but it didn’t work.’

‘What about Gildo?’ Gildo was her cat and favorite person in the world (yes, person).

‘Oh, he is as cute as ever.’ her face lit up. ‘But I don’t think the itching is related to him. I’ve had Gildo forever and he never gave me allergies. And I also took him to the vet recently.’

Mrs. White told me she couldn’t connect the itching to any activity or exposure. She also denied fever, rashes, or any other symptoms. On the physical exam, there was nothing of note. Dana also brought some of her latest exam results, which showed a mild anemia, probably from her chronic diseases, and a high alkaline phosphatase. 

I wasn’t sure about what to do next. Actually, I had no idea where to start. I excused myself from the room and told Dana I would be back soon. In the hallway, I could see Peter leaning over the reception’s balcony. I could bet he was looking for a pen, he never ever could remember where he put them. 

‘Peter, can you help me with something?’

‘Ah, hey, Lisa. Sure. And by the way, could you...’

‘Yes. You can have my pen.’ 

Peter smiled. ‘What do you need?’

‘Middle-aged woman, has a history of Hashimoto’s hypothyroidism and rheumatoid arthritis, both under control now. She started itching a month ago, everywhere.’

‘Hum... Allergy?’

‘I don’t think so. And it doesn't get better with antihistamines.’

‘Anything else?’

‘Her ALP is elevated and that’s about it.’

‘Bummer. Is she jaundiced?’

‘Not really. Maybe slightly if I push it. But that’s not a bad guess actually. Some hepatic or biliary problems could cause itching.’

‘Yeah, but that’s her only symptom right... I don’t know’.

‘Maybe something autoimmune, giving her history.’

‘Indeed.’

‘But what?’

He thought for a bit. ‘Why don’t you call her rheumatologist? She must have one right, and maybe he can give you a light on it.’

‘Huh. Smart.’

‘When is your next appointment with Dr. Ross, Dana?’ I said as I opened the door.

‘In a couple of weeks, I believe.’

‘Would you mind if I give him a call now? I am not sure what is causing the itching, but I think it could be something autoimmune, like your other conditions.’

‘Not at all, Lisa.’ She smiled. ‘Tell him I said hi.’

I asked her to wait for me again and went to call him outside. Thankfully, Dr. Ross answered his phone and was very kind to me. I told him about Mrs. White’s itching and that I thought it could be something autoimmune so I wanted to hear his thoughts on it. 

‘You may be right, Dr. Diaz. Did she show you any exams? If I am not mistaken I’ve ordered some regular check-ups on her last visit.’

‘Actually, she did. The only thing different was her alkaline phosphatase, which was elevated.’

‘Hum. Have you ever heard of something called Primary Biliary Cirrhosis? Or Primary Biliary Cholangitis, same thing.’

I thought for a bit. ‘Maybe.’ It did ring a bell but not enough that I could remember anything else about it.

‘Look it up and see if you agree with me. I think it’s a strong possibility for Dana. If you like, you can order an antimitochondrial antibody from her, and I will check it on her next visit here.’

‘Ok then, will do. Thank you, Dr. Ross.’

Before heading back to the room, I read a little about the disease. Like Dr. Ross had said the pruritus and high ALP were highly suggestive of PBC. There is no cure for it, but some drugs can help slow the progression of the disease. Most likely she would begin to take ursodeoxycholic acid along with symptomatic drugs. And also quit anything that could damage the liver, like alcohol. The hepatic damage throughout the years can eventually develop into portal hypertension and cirrhosis, needing a liver transplant.

My heart broke a little bit while I was reading. It’s not that it was a lost cause. Chances were she would probably live with it without shortening her life. But I didn’t want to tell Dana she probably had yet another condition to deal with. Deep down I wanted Dr. Ross to tell me some simple explanation for her symptom that didn’t involve bad news. I thought about how I would deliver them to Dana. Of course we didn’t know anything for sure yet, but I would have to talk to her about it.

‘I’m back.’ I said as I entered. She could tell I was a little unsteady.

‘It’s okay, darling. You can tell me.’ She smiled.

‘We don’t know for sure yet.’ I sat at the table. ‘But Dr. Ross and I really do believe it is an autoimmune condition.’

‘Go on.’ she said when I paused.

‘It’s called Primary Biliary Cirrhosis. We would like to run some tests to confirm it. Dr. Ross will check those exams in your appointment with him and we’ll take it from there. It’s possible that you’re going to need a liver biopsy besides the ultrasound. For now, I would like to start you on cholestyramine, so we can try to relieve this itching.’

‘Good. What else?’

‘Well, Dr. Ross will probably start another medication too. And we will have to take good care of your liver as it can be really damaged throughout the progression of PBC.’

‘Noted. What else?’

I chuckled at that. ‘Isn’t that enough, Dana?’

She reached for my hand, ‘I’ve had so much worse than this, Lisa. It’s just another fight for me to beat.’ She smiled broadly. ‘For us to beat.’

...

Want to know more about Primary Biliary Cirrhosis?

https://rarediseases.org/rare-diseases/primary-biliary-cholangitis/

Want to read a real case of Primary Biliary Cirrhosis?

https://www.gponline.com/primary-biliary-cirrhosis-case-study/gi-tract/gi-tract/article/1385036

Clinical Board

Ab: antibody; Bx: biopsy; Tt: treatment.

Bonus Board

 

This condition can be easily confused with another one with a similar name! Learn about this other disease below!

ERCP: Endoscopic Retrograde Cholangiopancreatography; Tt: treatment.

Remember: think BILIARY for WOMEN, and SCLEROSING for MEN.