Archive for the HCP Category

Breathlessness

On September 24, 2020 / HCP / Leave a comment

Top tips:

Breathlessness as a symptom is a subjective sensation.
Breathlessness can be caused by general causes or cirrhosis specific causes (e.g. hepatopulmonary syndrome)
Treat breathlessness if it is affecting quality of life and function. Take into account goals of care.
Opioid therapy may be helpful to manage moderate to severe breathlessness in the last days to weeks of life.

Navigation

Assess for breathlessness with validated tools
Consider non-pharmacological therapies
When to consider pharmacological therapy
Special considerations at End of Life
Tutorials
References
Give us feedback

Check out the bottom of the page for short videos from Dr. Sarah Burton-Macleod and Dr. Christopher Woodrell.

Click here to view the videos

Approach to Symptom Assessment

End-of-Life Considerations

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Step 1: Assess for breathlessness with validated assessment tools

Investigation and treatment of breathlessness should be in keeping with the patient’s goals of care.

Differential diagnoses

Most Common:

Ascites causing diaphragmatic compromise
Physical deconditioning (See CCAB exercise page)

Examples of other potential causes:

Hepatic hydrothorax (transudative pleural effusion) (See CCAB hydrothorax page)
Other cardiopulmonary diseases
Anxiety (See CCAB Anxiety page)
Anemia

Treat the underlying cause

Cirrhosis related causes – A liver specialist may need to be consulted. Some patients may meet criteria for liver transplantation or specialized treatments may be needed (e.g. hepatopulmonary syndrome, portopulmonary hypertension).

Step 2: Consider non-pharmacological therapies

Step 3: If symptoms persist, and patient is at end-of-life, Pharmacological therapy can be considered

Medication:	Recommended Dose	Additional information
Fentanyl	12.5mcg SC/SL/intranasal q1h PRN	For episodic shortness of breath (if associated with activity, take 10 mins prior to activity) Sedation, constipation Start with a lower dose for opioid naïve patients

Medication:	Recommended Dose	Additional information
Hydromorphone (Dilaudid)	0.5mg PO (or 0.2mg SC) q4h ATC and q1h PRN Max: 5 mg PO QID	For constant shortness of breath Sedation, constipation For older, opioid naive patients, start with 0.25 mg PO q 4h ATC and q1h PRN

Step 4: Special considerations at End of Life (last few days to weeks)

Introducing Dr. Sarah Burton-Macleod & Dr. Christopher Woodrell

Video 1 – The breathlessness algorithim in End-of-life

Video 2 - The top tips that may be useful for you to know about this page including assessment and non-pharmacological considerations

This section was adapted from content using the following evidence based resources in combination with expert consensus. The presented information is not intended to replace the independent medical or professional judgment of physicians or other health care providers in the context of individual clinical circumstances to determine a patient’s care.

Authors (Alphabetical): Amanda Brisebois, Sarah Burton-Macleod, Ingrid DeKock , Martin Labrie, Adriana Lazarescu, Noush Mirhosseini, Mino Mitri, Kinjal Patel, Aynharan Sinnarajah, Puneeta Tandon

Thank you to pharmacists Omer Ghutmy and Meghan Mior for their help with reviewing these pages.

For a comprehensive review on breathlessness, please refer to the links below.

Goals of care
Anxiety
Physical Activity
Hydrothorax
Ascites
Opioid Considerations document: In development

References:

References:

Davison SN on behalf of the Kidney Supportive Care Research Group. Conservative Kidney Management Pathway; Available from: https//:www.CKMcare.com.
European Association for the Study of the Liver. Electronic address: easloffice@easloffice.eu; European Association for the Study of the Liver. EASL Clinical Practice Guidelines for the management of patients with decompensated cirrhosis. J Hepatol. 2018 Aug;69(2):406-460. doi: 10.1016/j.jhep.2018.03.024. Epub 2018 Apr 10. Erratum in: J Hepatol. 2018 Nov;69(5):1207. PMID: 29653741.
Krowka MJ, Fallon MB, Kawut SM, Fuhrmann V, Heimbach JK, Ramsay MA, Sitbon O, Sokol RJ. International Liver Transplant Society Practice Guidelines: Diagnosis and Management of Hepatopulmonary Syndrome and Portopulmonary Hypertension. Transplantation. 2016 Jul;100(7):1440-52. doi: 10.1097/TP.0000000000001229. PMID: 27326810.
Walling AM, Wenger N. Palliative care for patients with end-stage liver disease. In Uptodate, Mar 06, 2020.

Indwelling Pleural Catheters (IPCs) for Refractory Hepatic Hydrothorax

On September 23, 2020 / HCP / Leave a comment

In patients with refractory hepatic hydrothorax, therapeutic options may be limited. Patients often require frequent thoracentesis.

Although Indwelling pleural catheters (IPC) are typically used in malignant pleural effusions, they can be considered in patients with hydrothorax related to cirrhosis.

Placement is generally ordered after consultation with a liver ± pulmonary specialists, weighing the risks vs benefits.

Step 1:

Evaluate whether the patient is a candidate for other standard of care therapies:

TIPS
Liver transplant

In the setting of malignant effusions, chemical pleurodesis may be a reasonable option. With a cirrhosis related hydrothorax, pleurodesis is not generally recommended. If it is considered, it should be done only after multidisciplinary discussion involving a liver specialist, pulmonary and interventional radiology.

Step 2:

Consider potential contraindications to IPC placement:

Contraindications	Rationale
Cognitive impairment	Patients may have challenges with the self-management activities required to care for the drain and may be at higher risk for damage or dislodgement of the drainage tube
Immunosuppressed (i.e. prior organ transplant, immunosuppression, etc.)	May increase the chance of infection
Unsafe living environment	Will limit the ability for home care to provide support in the community

Step 3:

Provide pre-procedure patient counselling:

Risk of infection
Long-term prophylactic antibiotics will be prescribed
Leakage is the most common complication
Submersing the drain (bathing or swimming) is not recommended
IV albumin may still be recommended at times depending on drainage volume and renal function
Homecare services are required for maintenance of the drain
Drainage frequency can be adjusted, and some patients may need daily drainages

pleurx.

Step 4:

Arrange IPC placement and follow up care:

Edmonton

Management Recommendations:

Drain Site Leaking

Renal dysfunction & albumin replacement

Parapneumonic infection

Skin Cellulitis

Pain during vacuum drainage

Skin Irritation

Downloadable content:

You can download these to print or view offline:

Community Care Access

Home Care Referral Form

Transjugular Intrahepatic Portosystemic Shunt (TIPS)

On September 23, 2020 / HCP / Leave a comment

Patients with cirrhosis can develop major complications such as ascites and variceal bleeding, related to portal hypertension.

A transjugular intrahepatic portosystemic shunt (TIPS) can be an effective means to decrease portal hypertension, by shunting some of the blood flow from the portal venous system into the hepatic venous system, via a stent.

Placement of a TIPS is a highly specialized interventional procedure, and should only be considered after consultation with a liver specialist.

See the ALTA consensus guidelines (PMID 34274511) for an in-depth review of TIPS in portal hypertension.

-For elective TIPS for ascites, a staged approach to TIPS creation is suggested – starting at 8 mm and then progressively dilating the stent q 6 weekly to 9 mm and 10 mm if required to optimize the clinical response.

Step 1:

Evaluate the indication for TIPS

Common Indications:

Bleeding related to portal hypertension
Refractory ascites/hydrothorax

Step 2:

Consider potential contraindications and risk for complications

Contraindications:

Severe Congestive Heart Failure
Severe untreated valvular heart disease
Moderate to Severe Pulmonary Hypertension
Refractory overt Hepatic encephalopathy
Active infection
Hepatocellular Carcinoma (location dependant)
Unrelieved Biliary Obstruction
Multiple Hepatic Cysts that preclude stent creation

Risk Factors Associated with Post-TIPS Complications:

Complication	Risk Factor
Liver Failure	Advanced Liver Disease (MELD >18) Cardiovascular disease
Encephalopathy	Prior Encephalopathy Wider diameter TIPS Sarcopenia Hyponatremia Age>65
Cardiac Decompensation	Aortic stenosis Diastolic dysfunction Prolonged QTc interval Elevated BNP
Mortality	Child Pugh C Urgent indication (variceal bleed) Bilirubin >50 umol/L Child Pugh score >13, or MELD score >30 and lactate >12 mmol/L (PMID 34018627) Pre-insertion consult by an interventional radiologist should be considered in all circumstances, but especially these

Step 3:

Perform pre-TIPS investigations

Echocardiogram
Liver Ultrasound Doppler: Evaluate liver vasculature, rule out hepatocellular carcinoma. In most cases CT may be required pre-TIPS insertion.
Labs: TBili, INR, Cr, CBC, BNP

Step 4:

Provide pre-placement patient counselling

Patients will require short post-procedure hospital admission
Risk of hepatic encephalopathy (30-45%). Treat using standard hepatic encephalopathy therapy. For patients who do not respond to standard therapy – consideration should be given to narrowing or blocking the TIPS. This decision will be based on the severity of symptoms and the potential impact on the patient’s quality of life
It can take weeks for symptoms like ascites to resolve so patients may require alternate therapy (such paracentesis for ascites) in the initial period post-TIPS
Liver failure is rare but can occur, particularly if there is advanced liver dysfunction pre-procedure.
Immediate procedural complications (vascular injury, pneumothorax, hepatic injury) are discussed in detail by diagnostic imaging.

Step 5:

Info for liver specialists to arrange the TIPS procedure

Edmonton

Step 6:

Post-procedure follow-up

Routine monitoring:

Item	Rationale	Frequency
Labs	Monitor for worsening liver & renal function, electrolyte abnormalities, hemolytic anemia	Day 1, Week 4, 8 & 12, then based on patient clinical status
Symptom assessment •Encephalopathy	Common complication post TIPS	Day 1, Week 4, 8 & 12, then based on patient clinical status.
•Ascites/hydrothorax	Monitor response to TIPS Evaluate potential to taper off diuretics as TIPS improves volume management
•Edema, shortness of breath	Monitor for cardiac decompensation
•Blood pressure and heart rate	Evaluate need to discontinue beta blocker used for variceal bleed prophylaxis (might need to continue if PPG post TIPS is > 12 mmHg)
•GI bleeding	Monitor response to TIPS
Ultrasound Doppler	Detect TIPS stenosis	Month 1-3, 6, and then every 6 months along with routine screening ultrasound performed for hepatocellular carcinoma screening.

Downloadable content:

You can download these to print or view offline:

Admission Orders

Indwelling peritoneal catheters (IPCs) for Refractory Ascites

On September 16, 2020 / HCP / Leave a comment

In patients with refractory ascites, therapeutic options may be limited. Patients often require frequent paracentesis.

Although Indwelling peritoneal catheters (IPCs) are typically used in malignant ascites, they can be considered in patients with refractory ascites related to cirrhosis (requiring paracentesis at least monthly despite diuretics).

In a 2019 systematic review, Macken et al. concluded “Despite lack of well-designed studies, preliminary data suggests low significant complication rates; however safety and efficacy of permanent indwelling peritoneal catheters in end-stage liver disease remains to be confirmed”.

A randomized controlled trial by the same authors (PMID 32478917) published in 2020 compared large-volume paracentesis vs long-term abdominal drains in refractory ascites patients who were not candidates for liver transplantation. All participants were given ciprofloxacin 500 mg po daily as antibiotic prophylaxis. No protocolized albumin was given in the long-term abdominal drain arm. Costs and time in hospital were lower in the indwelling drain group and there was no difference in the incidence of peritonitis (6% in the indwelling drain group and 11% in the large- volume paracentesis group.

Placement is generally ordered after consultation with a liver specialist, weighing the risks vs benefits.

Step 1:

Evaluate patient candidacy for other first line therapies for refractory ascites management:

TIPS
Liver transplant

Step 2:

Consider potential contraindications to IPC placement:

Contraindications	Rationale
Candidate for liver transplant	Theoretical risk of causing a cocoon abdomen or sclerosing encapsulating peritonitis
Fluid septations	Drain will likely not be effective to manage ascites. Can rule out with ultrasound
Thin abdominal wall	Can make tunnelling the drain challenging. Pre-insertion consult by an interventional radiologist should be considered in these circumstances
Severe abdominal anasarca	Drain insertion site will be less likely to heal, resulting in higher risk for long term leaking from the site Pre-insertion consult by an interventional radiologist should be considered in these circumstances
Cognitive impairment	Patients may have challenges with the self-management activities required to care for the drain and may be at higher risk for damage or dislodgement of the drainage tube
Immunosuppressed (i.e. prior organ transplant, immunosuppression, etc.)	May increase the chance of infection
Unsafe living environment	Will limit the ability for home care to provide support in the community

Step 3:

Provide pre-procedure patient counselling:

Theoretical increased risk of SBP, although in small studies that include prophylactic antibiotics, risk does not appear greater than standard SBP
Long-term prophylactic antibiotics will be prescribed
Leakage is the most common complication
Submersing the drain (bathing or swimming) is not recommended
IV albumin may still be recommended at times depending on drainage volume and renal function
Homecare services are required for maintenance of the drain
Drainage frequency can be adjusted, and some patients may need daily drainages

Step 4:

Arranging IPC placement and follow up care:

Edmonton

Management Recommendations:

Drain Site Leaking

Renal dysfunction & albumin replacement

Spontaneous Bacterial Peritonitis (SBP)

Pain during vacuum drainage

Skin Cellulitis

Skin Irritation

Drain tunnel fistula/hernia (fluid filled)

Blood tinged ascites

pleurx.

Downloadable content:

You can download these to print or view offline:

Community Care Access

Home Care Referral Form

Home Care Management Orders

Drain Placement Orders

Alcohol Use Disorder (AUD)

On December 30, 2019 / HCP / Leave a comment

Top tips:

Abstinence from alcohol is the best way to improve outcomes in patients with alcohol-related cirrhosis. In alcohol related liver disease, even 1 or 2 drinks per day can increase mortality.
Some patients won’t be ready or able to quit completely. For those patients who struggle to quit completely, continue to support them with encouragement, motivational interviewing and pharmacotherapy to help them achieve abstinence.
It is important to screen for concurrent mental health concerns alongside addiction. Patients with these issues require further management.

Navigation

Connect Care Order panels
Connect Care General Cirrhosis Admission and Discharge Order Sets
Algorithms
- Alcohol Use Disorder Diagnosis and Treatment
Additional info
Tutorials
Patient Materials
Calculators
Downloadable content
References
Give us feedback

Order panel for Alcohol use disorder:

For adults admitted with Alcohol use disorder:

Alcohol Use Disorder in Cirrhosis Order Panel

General Cirrhosis Admission and Discharge Order Sets

*Add specific panels to general admission orders as appropriate*

For adults with cirrhosis requiring hospital admission
Cirrhosis Adult Admission Orders

For adults with cirrhosis requiring hospital discharge
Cirrhosis Adult Discharge Orders

Check out the bottom of the page for short videos from Dr. Mellinger and Dr. Ghosh!

Click here to view the videos

Diagnosis

Video about screening, brief intervention, and assessing readiness to change

DSM 5 AUD

Information about AUD relapse prevention medications

Medication:	Acamprosate
Dose:	666 mg po TID; 333 mg po TID if renal impairment (CrCl 30 to 50 mL/min) or weight <60 kg; Generally abstinence for >3 days before initiation, although studies show reduction in heavy drinking days even when initiated prior to abstinence.
Contraindications:	Creatinine clearance <30 ml/min
Considerations:	Pregnancy risk category ‘C’; As above, consider in pregnant women if potential benefit outweighs risk; Although TID dosing is cumbersome, it may be useful for patients who cannot take naltrexone due to liver disease or taking opioids or with polypharmacy because no significant interactions with other drugs; Caution if depression or suicidal ideation;
Side Effects:	GI upset ; somnolence, rarely suicidality
Monitoring:	Initial close weekly follow-up may be helpful; Monitor renal function and adjust dose if CrCl 30-50 ml/min
Health Canada for AUD:	Approved
Coverage:	Funded via Special Authorization Request Form for Income Support, AISH or AB Adult Health Benefit. View Alberta Blue Cross Drug Special Authorization Request. Limited use benefit under NIHB (abstinent >3 days and enrolled in treatment program where available, prior approval required).
Est. cost with no coverage	~ $200/mo.

Medication:	Gabapentin
Dose:	With history of Hepatic Encephalopathy: Start low dose – 100 mg po TID and titrate up as tolerated. Without Hepatic encephalopathy: 300 mg po day one, 300 mg po BID day 2 ; 300 mg po TID day 3; increasing by 300 mg po each day up to 600 mg TID on day 6 as tolerated; abstinence at 12 weeks 4.1% placebo group; 11.1% gabapentin 900 mg/day; 17% for 1,800 mg/day.
Contraindications:	Decrease dose with renal impairment. Start at lower doses with Hepatic encephalopathy.
Considerations:	Risk of dependence in post-marketing database; increased risk of CNS depression esp. with opioids and other CNS depressants; Pregnancy risk category ‘C’; Consider in pregnant women if potential benefit outweighs risk
Side Effects:	Somnolence, dizziness, ataxia, fatigue, nystagmus, tremor
Monitoring:	Routine monitoring not required; consider if renal impairment
Health Canada for AUD:	Not approved
Coverage:	Drug benefit under Income Support, AISH, AAHB, NIHB and CFS
Est. cost with no coverage	~$30/mo. partial coverage by many drug plans
Clinical pearls:	Gabapentin has some off-label utility in treating mild to moderate alcohol withdrawal which means it can be useful in early sobriety particularly in AUD patients who have comorbid anxiety and/or insomnia alongside their other symptoms. It is also another good medication for patients with comorbid neuropathic pain. Requires attention to kidney function due to renal clearance and could worsen hepatic encephalopathy due to potential sedating effects.

Medication:	Baclofen
Dose:	Initiated at 5mg TID with increases 3-5 days based on patient tolerance and absence of side effects. The maximum recommended dose for alcohol related liver disease is 15mg TID. Higher doses can be tried but with extreme caution due to limited evidence and increased risk of side effects
Contraindications:	Care must be taken in patients with both renal and liver disease. This drug should be avoided in individuals with hepatic encephalopathy. Due to a lack of studies, baclofen should be avoided in pregnant patients. Baclofen toxicity can lead to overdose. Caution must be taken for individuals with severe suicide risk.
Considerations:	The maximum recommended dose for alcoholic liver disease is 15mg TID. Care must be taken for patients with renal failure or hepatorenal failure, as baclofen is predominantly excreted renally, and impairments in excretion can lead to delirium, and drug toxicity and doses of 5mg TID max should be prescribed in this setting. Operation of heavy machinery is discouraged when first using the medication until they learn how the sedation affects them or they reach a stable dose.
Side Effects:	Severe sedation, dizziness, and/or confusion. Potentially dangerous side effects are seizures, respiratory depression with sleep apnea and potentially coma (in case of intoxication), severe mood disorders (mania or depression, with the risk of suicide), and mental confusion/delirium
Monitoring:	No specific monitoring required.
Health Canada for AUD:	Not approved
Coverage:	Drug benefit under Income Support. Covered by many private drug plans.
Est. cost with no coverage	If 80% covered by a private drug plan, cost to patient: $6/month, based on 60 mg daily. Cost to patient if no coverage: $28/month.

Medication:	Notes:
Naltrexone	In the US, naltrexone is a first-line and FDA-approved medication whose utility in AUD is often in patients trying to gain early sobriety or who commonly relapse due to its ability to reduce heavy drinking and dampen cravings. It has been shown to have a larger effect size than Acamprosate in some studies but its use is limited in liver disease patients due to drug-related LFT elevation and metabolite accumulation in hepatic insufficiency. We are not using naltrexone in patients with disease more severe than Childs Pugh A. It is contraindicated in patients using opioids.
Disulfiram	Should not be used in patients with liver disease.
Topiramate	Has some off-label utility in promoting AUD sobriety and may be adjunctively useful in patients who have co-occurring weight-related challenges. Requires attention to kidney function due to renal clearance and could worsen hepatic encephalopathy due to potential sedating effects
Varenicline	May also have some off-label effect in reducing alcohol craving and consumption alongside its treatment of nicotine dependence. It requires attention to renal function for dosing.
Ondansetron	Has been shown to reduce alcohol use as an off-label treatment though we have little experience using it for this purpose.

Pearls from the UMichigan integrated care team

Introducing Dr. Mellinger and Dr. Ghosh

Video 1 – Practical tips on how to decide if a patient with cirrhosis needs alcohol use disorder therapy, if they are ready for it and what agent to choose.

Video 2 – Practical tips to accessing support for alcohol use disorder therapy in Alberta – how to refer your patient for counselling and how to get help with prescribing relapse prevention medications.

Patient materials:

You can direct patients to the following:
Healthy Living-Alcohol

Causes of liver disease – Alcohol

211.ca

AHS Help in Tough Times

Recovery Access Alberta

Alcoholics anonymous

Alberta Health Services Addiction and Mental Health Information Page for Health Professionals

Alberta Addiction Service Providers

Link to the Edmonton Canadian Mental Health Association

Alberta Addiction Treatment Centres

Provincial Detox & Substance Use Treatment Facility Directory

Calculators:

Use these calculators to help with the diagnosis:

AUDIT-C calculator

Downloadable content:

You can download these to print or view offline:
AASLD guidance statement 2019

Incorporating Alcohol Pharmacotherpies Into Medical Practice. A Treatment Improvement Protocol

Guidelines for biological treatment of substance use and related disorders, part 1: Alcoholism, first revision

Alberta Drug Special Authorization Form

References:

Authors: Dr. Monty Ghosh, Dr. Jessica Mellinger, Dr. Kathryn Dong, Dr. Laura Evans, Dr. Nicholas Mitchell, Dr. Meredith Borman, Dr. Scott G Winder, Emily Johnson, Dr. Puneeta Tandon

References:

Crabb DW et al. Diagnosis and Treatment of Alcohol-Related Liver Diseases: 2019 Practice Guidance from the American Association for the Study of Liver Diseases. Hepatology 2019 July 17 epub ahead of print, PMID 31314133

Last reviewed November 3, 2022

Assess Disease Severity and Peri-op Risk Assessment

On December 26, 2019 / HCP / Leave a comment

Top tips:

MELD-Na, Child Pugh are key prognostic scores in cirrhosis
Decompensating events (variceal bleed, ascites, etc.) place patients at higher risk of further complications and death. Decompensation should trigger consideration for liver transplantation.
Patients with cholestatic liver disease (PBC, PSC) have additional models that can be used for prognostication.

Navigation

Algorithms
- Assessing Liver Disease Severity
- Assessing Surgical Risk
Additional info
- D’Amico’s prognostic sub-stages
- More info on the Child Pugh score and the MELD Na score including prognostic limitations
Tutorials
Patient Materials
Calculators
Downloadable content
References
Give us feedback

Check out the bottom of the page for a short video from Dr. Kamath!

Click here to view the video

D’Amico’s prognostic sub-stages

	Compensated Cirrhosis	Decompensated Cirrhosis
Stage	Stage 1	Stage 2	Stage 3	Stage 4	Stage 5
Definition	No Varices no other decomp. complications	Varices no other decomp. complications	Bleeding no other decomp. complications	Single non-bleeding decomp. complications	Two decomp. complications
1 year mortality rate	1%	3%	15%	26%	57%

More info on the Child Pugh score and the MELD Na score including prognostic limitations

Factor	1 point	2 points	3 points
Total Bilirubin (μmol/L)	<34	34-50	>50
Serum Albumin (g/L)	>35	28-35	<28
PT INR	<1.7	1.71-2.30	>2.30
Ascites	None	Controlled, on diuretics	Refractory
Hepatic Encephalopathy	None	Controlled, on medical therapy	Refractory

	Class A	Class B	Class C
Total Points	5-6	7-9	10-15
1 year survial	100%	80%	45%

MELD-Na Score	90-day Mortality
<17	<2%
17-20	3-4%
21-22	7-10%
23-26	14-15%
27-31	27-32%
≥32	65-66%

Assessing Surgical Risk

Surgical risk assessment is “as much art as science” (see editorial by Dr. Kamath at PMID 33220099).

**Before using the algorithm below, consider standard factors associated with pre-operative risk assessment – bleeding risk, cardiopulmonary risk, frailty as well as surgeon and patient preferences. If the patient is still deemed a possible candidate despite these considerations, then apply the algorithms.

Introducing Dr. Kamath

Video 1 – A brief tour through prognostication in cirrhosis & practical tips that I use for surgical risk assessment in cirrhosis

Patient materials:

You can direct patients to the following:
Patient education for cirrhosis

Stages of cirrhosis

Calculators:

Use these calculators to help with the diagnosis:

Child Pugh

MELD-Na

MELD

References:

Authors: Michelle Carbonneau NP, Dr. Patrick Kamath, Dr. Juan G. Abraldes, Dr. Puneeta Tandon

References:

D’Amico G et al. Competing risks and prognostic stages of cirrhosis: a 25-year inception cohort study of 494 patients. Aliment Pharmacol Ther 2014; 39:1180-1193. PMID 24654740
D’Amico G et al. Clinical states of cirrhosis and competing risks. J Hepatol 2018 Mar; 68(3):563-576 PMID 29111320
Northup P.G. et al. AGA Clinical Practice Update on Surgical Risk Assessment and Perioperative Management in Cirrhosis: Expert Review. Clin Gastroenterol Hepatol 2019 Mar; 17(4):595-606 PMID 30273751
Reverter E et al. The prognostic role of hepatic venous pressure gradient in cirrhotic patients undergoing elective extrahepatic surgery. J Hepatol 2019 Nov; 71(5):942-950 PMID 31330170
Arroyo V et al. Acute-on-Chronic Liver Failure: Definition, Diagnosis and Clinical Characteristics. Semin Liver Dis 2016 May;36(2):109-16 PMID 27172351
Bajaj JS et al. Acute-on-Chronic Liver Failure: Getting Ready for Prime Time? Hepatology 2018 Oct;68(4):1621-1632 PMID 29689120
Gustot T et al. Acute-on-chronic liver failure vs traditional acute decompensation of cirrhosis. J Hepatol 2018 Dec;69(6):1384-1393 PMID 30195459
D’Amico G et al. Ordinal outcomes are superior to binary outcomes for designing and evaluating clinical trials in compensated cirrhosis. Hepatology 2019 Dec PMID 31837238

Last reviewed November 3, 2022

Calculators

On September 17, 2019 / HCP / Leave a comment

Please select a calculator on the right – it will display below.

Order Sets

On September 17, 2019 / HCP / Leave a comment

Advance Care Planning

On September 17, 2019 / HCP / Leave a comment

Top tips:

Advanced care planning is an iterative and longitudinal process of patient-centered medical decision-making that is guided by patients’ personal goals, values, and current health circumstances to elicit their goals and preferences for medical care
Identifying, documenting, and preparing surrogate decision-makers early in the disease course is a key component of advance care planning in cirrhosis care prior to patients losing capacity
Goals of care should be reassessed regularly during routine care but especially after sentinel events such as new clinical deteriorations or acute changes in health status or home/social circumstances
Anticipatory guidance for medical decision-making should include an assessment of preferences for future treatment options (e.g. endoscopic procedures, renal replacement therapy, mechanical ventilation, cardiopulmonary resuscitation) and place of care in the event of progressive or terminal illness

Navigation

The ACP process in cirrhosis care
When to initiate ACP discussions for patients with cirrhosis
How to initiate ACP discussions for patients with cirrhosis
6-Step SPIKES Model
Optimizing outpatient processes for ACP
Tutorials
Patient Materials
Links
References
Give us feedback

Thank you to Dr. Woodrell, Dr. Patel, and Dr. Ufere for your efforts creating the content on this page!

The ACP process in cirrhosis care

Figure 1. The ACP Process and Key Elements

Figure 1. The ACP process in cirrhosis care. Adapted [reprinted] from “Advance care planning (ACP) for specialists managing cirrhosis: A focus on patient-centered care,” by Amanda Brisebois, Kathleen P. Ismond, Michelle Carbonneau, Jan Kowalczewski, and Puneeta Tandon, 2017, Hepatology, 67(5). Copyright year 2018 by All Authors.

Talking points:

Assessing goals/values/perspectives
Assessing readiness
Early identification and documentation of surrogate decision-maker
Preparation of surrogate decision-maker

Discussing prognosis
Reviewing cirrhosis complications
Document GOC including preferences for future medical care and place of care

When to initiate ACP discussions for patients with cirrhosis

Table 1. Suggested Clinical Milestones and Life Events That Should Trigger ACP Discussions for Patients with Advanced Liver Disease

Liver-Specific Factors	Patient Factors	Major Life Events
New liver-related complications (ascites, hepatic encephalopathy, variceal bleeding, hepatocellular carcinoma)	Advanced multimorbidity	Recent ICU admission
New diagnosis of refractory ascites	Poor performance status and/or progressive frailty	Recent unplanned hospitalization for a liver-related complication
New diagnosis of acute kidney injury or hepatorenal syndrome	Ongoing substance use disorder	Loss of a spouse or primary caregiver
Recently delisted or declined for liver transplantation	New comorbid diagnosis of cancer, cardiovascular disease, neurological disease, or other life-limiting condition	Advanced age

Note. Reprinted [adapted] from “Advance Care Planning and Goals of Care Discussions in Advanced Liver Disease,” by Nneka N. Ufere, 2021, Current Hepatology Reports, 20, 77. 2021 by Author(s).

How to initiate ACP discussions for patients with cirrhosis

Conversation starters for ACP discussions

Topics	Conversation Starters
Patient perspectives	Level of detail —Are you a detail-oriented learner or do you prefer a general overview and plan?Learning style —Some people are visual leaners while others process conversations best. Which learning approach suits you the best? Values and fears —What is most important to you in life? —What aspect of health would need to be taken from you, for you to feel that living was worse than dying?
Education	—Meeting for the first time about a new illness that you have been diagnosed with can be overwhelming —We have created educational pamphlets we can provide for you, which can help guide our future discussions. —Our clinic communicates with your other health providers, so that we all know your wishes and details regarding your health
Readiness	Readiness to discuss ACP and GCD —Very ill patients with cirrhosis cannot always make their own health care decisions. We like to talk about these things early on to understand what you value in case such a situation arises. How would you feel discussing how cirrhosis may affect you in the future? Readiness to choose a surrogate —If you were to get very sick, is there anyone you trust to make medical decisions for you? Does this person know what is important to you?
Focus of care	—Recently, you had a complication of cirrhosis and were quite unwell. I would like to explain how cirrhosis may affect you in the future. —It is important to understand how disease-modifying treatments and symptom control therapies are used together an illness.
Prognosis	Discuss in general terms —To make sure that both you and your family are prepared, I like to address both the best-and worst-case scenarios regarding how your cirrhosis may progress. Can we talk about these now? —Some patients ask me how their cirrhosis will affect how long they live. Are you interested in this type of information?
Review cirrhosis	Disease complications —The investigations that we have done show that your disease has worsened. I would like to discuss how that affects what treatments and therapies are going to help you live the best-quality of life possible End-of-life wishes —What would be important to you in your last months of life? —Where would you prefer to die?
Resources	—Many communities have additional supports for patients with medical, social, psychological, and spiritual needs. Would you be interested in learning about some of these resources?
Documentation	—This depends on where patient is located. Various websites describe local and regional Goals of Care Medical Orders. — In Alberta, Canada, Conversations Matter describes local requirements
Review and compare	—Please tell me what you understand of your decisions with respect to your GCD. What impact do they have on your life? I want ot ensure that the designation properly reflects your values and wishes —Since we last met, have you had any experiences that may change your view about living with cirrhosis? — Are your wishes outlined anywhere else, such as in a Will or legal document? If so, we should look at it to ensure your wishes are consistent with your medical documents

SPIKES Framework

Setting	Setting up the interview •Arrange for some privacy •Involve significant others if the patient chooses •Invite other relevant parties: Interpreter, social worker, surgeon, palliative care physician •Sit down with the patient •Make a connection and establish rapport with the patient •Manage time constraints and interruptions
Perception	Assessing the patient's perception •Use open-ended questions to determine the patient's level of comprehension about his or her medical condition (i.e. "What have you been told about your medical situation so far?")
Invitation	Obtaining the patient's invitation •Assess the patient's readiness to receive information •Discuss information disclosure at the time of ordering studies (i.e. "I have some updates on your condition to discuss. Would it be all right if I share these with you now?") •If the patient does not want to know details, offer to answer any questions they may have in the future or to talk to a caregiver
Knowledge	Giving knowledge and information to the patient •Consider a warning prior to disclosing bad news to the patient (i.e. "Unfortunately, I do not have good news" or "I'm sorry to tell you that...") •Start at the level of comprehension and vocabulary of the patient •Try to use nontechnical words and avoid medical jargon •Avoid excessive bluntness •Give information in small chunks, and periodically reassess the patient's understanding •When the prognosis is poor, avoid using phrases such as "There is nothing we can do for you" as patients may have other important therapeutic goals outside of curative therapy such as good symptom relief
Emotion	Addressing the patient's emotions with empathic responses •Observe for any emotion on the part of the patient •Identify the emotion experienced by the patient by naming it to oneself-use open ended questions to query the patient as to what they are feeling •Identify the reason for the emotion - if you are not sure, ask the patient •After you have given the patient time to express his or her feelings, show the patient that you recognize and empathize with his or her emotional response
Strategy and Summarize	Presenting a clear plan for the future with the patient •Assess whether the patient needs clarification on what has been discussed •Ask the patient if he or she is ready for a discussion regarding next steps in management •Set out a medical plan of action

Adapted from Baile et al, Oncologist 2000

Physician communication skills training

Patient facing educational tools

Best Case/Worst Case Tool

Best Case/Worst Case

Optimizing outpatient processes for ACP

Alberta Tools

U.S.A Tools

Billing guidance

Tips for ACP Billing in Hepatology Practice

Health care professionals who may furnish and bill ACP: Physicians, clinical nurse specialist, nurse practitioners, physician assistants
Since ACP services are voluntary, Medicare beneficiaries (or their legal proxies when applicable) should be given a clear opportunity to decline to receive ACP services. Beneficiaries, family members and/or surrogates may receive assistance for completing legal documents from others outside the scope of the Medicare program in addition to, or separately from, the physician or NPP.
Examples of appropriate documentation would include an account of the discussion with the beneficiary (or family members and/or surrogate) regarding the voluntary nature of the encounter; documentation indicating the explanation of advance directives (along with completion of those forms, when performed); who was present; and the time spent in the face-to-face encounter
To bill for advance care planning services provided during an Evaluation and Management (E/M) office visit or inpatient consult/follow-up visit, providers can use the modifier code 25 and the following ACP codes as follows:
- CPT Code 99497: Can be used to bill for advance care planning performed by a qualified health care professional for the first 30 minutes (minimum of 16 minutes) spent face-to-face with the patient, family member(s), and/or surrogate. This includes the explanation and discussion of advance directives such as health care proxy or MOLST forms. This include, but does not require, the completion of such forms.
- CPT Code 99498: Can be used for each additional 30 minutes (minimum of 16 minutes past the first 30 minutes documented) of advance care planning performed by a qualified health professional. List separately in addition to code for primary procedure
- Visits that are dedicated to ACP alone may also be billed separately using CPT codes 99497 and 99498

Quick reference billing for ACP
FAQ: Billing ACP services

Patient materials:

You can direct patients to the following:
Personal Directive
Power of Attorney
Living Wills
Patient Educational Brochures

References:

This section was adapted from content using the following evidence-based resources in combination with expert consensus. The presented information is not intended to replace the independent medical or professional judgment of physicians or other health care providers in the context of individual clinical circumstances to determine a patient’s care.

Authors: Dr. Nneka Ufere, Dr. Arpan Patel, Dr. Christopher Woodrell, Dr. Amanda Brisebois, Dr. Rebecca Sudore, Dr. Puneeta Tandon

References:

1. Tandon P, Walling A, Patton H, Taddei T. AGA Clinical Practice Update on Palliative Care Management in Cirrhosis: Expert Review. Clinical Gastroenterology and Hepatology. 2021;19(4):646-656.e3. doi:10.1016/j.cgh.2020.11.027. PMID 33221550
2. Brisebois A, Ismond KP, Carbonneau M, Kowalczewski J, Tandon P. Advance care planning (ACP) for specialists managing cirrhosis: A focus on patient-centered care. Hepatology. 2018;67(5):2025-2040. doi:10.1002/hep.29731.PMID 29251778
3. Ufere NN. Advance Care Planning and Goals of Care Discussions in Advanced Liver Disease. Current Hepatology Reports. 2021;20(3):77-84. doi:10.1007/s11901-021-00565-x.
4. Baile, W. F., Buckman, R., Lenzi, R., Glober, G., Beale, E. A., & Kudelka, A. P. (2000). SPIKES-A six-step protocol for delivering bad news: application to the patient with cancer. The oncologist, 5(4), 302–311. https://doi.org/10.1634/theoncologist.5-4-302PMID 10964998

Fatigue

On September 17, 2019 / HCP / Leave a comment

Top tips:

Fatigue is very common in cirrhosis, and is often multifactorial
Potential contributing factors include pain, pruritus, anxiety, depression
Treat the patient’s fatigue if it is affecting their quality of life or daily functioning
At the end of life (last few weeks or days), as the patient’s condition deteriorates, certain non-pharmacological interventions will become unrealistic (e.g. exercise), and pharmacologic interventions may become less effective. Reassess patient’s GOC as appropriate

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Assess for additional modifiable contributing factors
Consider non-pharmacological therapies
When to consider pharmacological therapies
Special considerations at End-of-Life
References
Give us feedback

Knowing your
Patient

Approach to Symptom Assessment

Symptom Management Principles

Drug Dosing
in Cirrhosis

End-of-Life Considerations

Expand all Collapse all

Step 1: Assess for possible modifiable factors contributing to fatigue

Step 2: Consider Non-pharmacological management

Step 3: If symptoms persist, consider Pharmacological therapy
(there is limited evidence to support this)

Medication:	Recommended Dose	Additional information
Methylphenidate (Ritalin)	2.5mg to 5mg PO qAM and noon	Limited data in cirrhosis Adverse effects include dry mouth, dizziness, anxiety etc.

Special considerations at End-of-life (last few days to weeks)

We gratefully acknowledge the Physician Learning Program for their design assistance.

Authors (Alphabetical): Amanda Brisebois, Sarah Burton-Macleod, Ingrid DeKock , Martin Labrie, Noush Mirhosseini, Mino Mitri, Kinjal Patel, Aynharan Sinnarajah, Puneeta Tandon

Thank you to pharmacists Omer Ghutmy and Meghan Mior for their help with reviewing these pages.

Helpful Links:

References:

Davison SN on behalf of the Kidney Supportive Care Research Group. Conservative Kidney Management Pathway; Available from: https//:www.CKMcare.com.
Bajaj JS, Ellwood M, Ainger T, Burroughs T, Fagan A, Gavis EA, Heuman DM, Fuchs M, John B, Wade JB. Mindfulness-Based Stress Reduction Therapy Improves Patient and Caregiver-Reported Outcomes in Cirrhosis. Clin Transl Gastroenterol. 2017 Jul 27;8(7):e108. doi: 10.1038/ctg.2017.38. PMID: 28749453; PMCID: PMC5539344.
Blockmans D, Persoons P, Van Houdenhove B, Bobbaers H. Does methylphenidate reduce the symptoms of chronic fatigue syndrome? Am J Med. 2006 Feb;119(2):167.e23-30. doi: 10.1016/j.amjmed.2005.07.047. PMID: 16443425. PMID: 16443425
Dyson JK, Elsharkawy AM, Lamb CA, Al-Rifai A, Newton JL, Jones DE, Hudson M. Fatigue in primary sclerosing cholangitis is associated with sympathetic over-activity and increased cardiac output. Liver Int. 2015 May;35(5):1633-41. doi: 10.1111/liv.12709. Epub 2014 Dec 4. PMID: 25363895; PMCID: PMC4737110.
Ian Gan S, de Jongh M, Kaplan MM. Modafinil in the treatment of debilitating fatigue in primary biliary cirrhosis: a clinical experience. Dig Dis Sci. 2009 Oct;54(10):2242-6. doi: 10.1007/s10620-008-0613-3. Epub 2008 Dec 12. PMID: 19082890.
Jones DE, Newton JL. An open study of modafinil for the treatment of daytime somnolence and fatigue in primary biliary cirrhosis. Aliment Pharmacol Ther. 2007 Feb 15;25(4):471-6. doi: 10.1111/j.1365-2036.2006.03223.x. PMID: 17270003.
Jopson L, Dyson JK, Jones DE. Understanding and Treating Fatigue in Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis. Clin Liver Dis. 2016 Feb;20(1):131-42. doi: 10.1016/j.cld.2015.08.007. PMID: 26593295.

Archive for the HCP Category

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Investigation and treatment of breathlessness should be in keeping with the patient’s goals of care.

Introducing Dr. Sarah Burton-Macleod & Dr. Christopher Woodrell

References:

Step 1:

Evaluate whether the patient is a candidate for other standard of care therapies:

Step 2:

Consider potential contraindications to IPC placement:

Step 3:

Provide pre-procedure patient counselling:

Step 4:

Arrange IPC placement and follow up care:

Management Recommendations:

Downloadable content:

Step 1:

Evaluate the indication for TIPS

Step 2:

Consider potential contraindications and risk for complications

Step 3:

Perform pre-TIPS investigations

Step 4:

Provide pre-placement patient counselling

Step 5:

Info for liver specialists to arrange the TIPS procedure

Step 6:

Post-procedure follow-up

Downloadable content:

Step 1:

Evaluate patient candidacy for other first line therapies for refractory ascites management:

Step 2:

Consider potential contraindications to IPC placement:

Step 3:

Provide pre-procedure patient counselling:

Step 4:

Arranging IPC placement and follow up care:

Management Recommendations:

Downloadable content:

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Order panel for Alcohol use disorder:

General Cirrhosis Admission and Discharge Order Sets

Diagnosis

Introducing Dr. Mellinger and Dr. Ghosh

Patient materials:

Calculators:

Downloadable content:

References:

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Assessing Surgical Risk

Surgical risk assessment is “as much art as science” (see editorial by Dr. Kamath at PMID 33220099).

Introducing Dr. Kamath

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Calculators:

References:

Select a calculator:

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The ACP process in cirrhosis care

Figure 1. The ACP Process and Key Elements

When to initiate ACP discussions for patients with cirrhosis

Table 1. Suggested Clinical Milestones and Life Events That Should Trigger ACP Discussions for Patients with Advanced Liver Disease

How to initiate ACP discussions for patients with cirrhosis

Physician communication skills training

Patient facing educational tools

Optimizing outpatient processes for ACP

Patient materials:

Helpful Links:

References:

Top tips:

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References: