Nuisances of Defensive Medicine

Defensive Medicine, refers to the practice of recommending a test or treatment that is not necessarily the best option for the patient, but an option that mainly serves the function to protect the physician against a potential lawsuit. 

When medical students are nurtured into physicians, they are seldom taught this part of medicine but it gets imbibed into them when they begin practicing in high-risk environments and see their peers landing into lawsuits in mishaps, sometimes for petty and unimaginable reasons. Physicians from United States are at highest risk of being sued for missed diagnosis and even for a delayed diagnosis! Over years, this litigation culture has penetrated other healthcare systems as well. India is not left behind either. 

I vividly recall my freshman year in medical school, back in 2005 when we were taught that a solid history provides you over 50% of the diagnosis. Required laboratory investigations are then used to confirm your suspicion. Few years later it was reinforced again when I learned that killer pathologies such as “Acute Coronary Syndromes” are suspected based only on the history but followed by ECGs, Troponins and cardiac stress testing. This applies not only to ACS but also to most other diseases. History is the key to reach a diagnosis. 

As a trainee, I was always asked before ordering a test – Why do want this and how is this going to change your management? But things have changed now, I frequently see the blood reports first and then talk to the patient. 

Almost every physician (Emergency Medicine, Cardiology, Internists, Ortho etc.) is aware of this mantra of “solid history” but there are many who choose to do the other way around i.e. order tests/imaging to safeguard regardless of the history. In Emergency Departments across the globe, now blood tests are requested even before a patient encounters a physician. Indisputably, this saves time and averts overcrowding. But what about the non-specific positive tests that increase the length of stay exponentially and force us to order some more tests, some more imaging. Few classic examples are non-specific troponins and d-dimers and unnecessary Head CTs ordered on everyone who walks in with syncopy and has a normal CNS exam. 

Indian Healthcare is distributed between private and public sector and laypersons often believe that physicians always earn a significant share from the cost of blood tests! Investigations are also done as physicians now work with an instilled fear of litigation in their minds, at least in the private sector.

Issues with Defensive Medicine

• Fuels Overcrowding: This is becoming a global concern because more patients are using Emergency Services and we are being too cautious due to fear or litigation. EDs are growing bigger in size but it is not helping. Too many labs equal false positives i.e. watch them for more hours/admit for invasive testing. Even if we interpret every test in context of history and physical, how many of us feel safe discharging a patient without performing a CTPA but with a label of non-cardiac chest pain and elevated d-dimer. From a physician’s standpoint, I don’t see anything wrong when we practice in a defensive manner. We learn this over years as we observe what our contemporaries go through during the process of handling a lawsuit. This is normal human behavior – no one wants to invite trouble.  So what would you do - Order a few more tests or handle a lawsuit that comes with months of stress and disrupts your life? Be mindful of the fact that a lawsuit can be extremely demoralizing and distressing; it has the potential to change the personality and mindset of a doctor. It can be shattering!

• Time and Resources – A ton of time and resources can be saved if labs are ordered judiciously. For instance, Routine blood cultures for well appearing patients is becoming a norm.
• Overtreatment – Overtreatment with unnecessary Antibiotics carry the risk of adverse reactions, sometimes life-threatening. Everything comes with pros and cons, even “normal saline” and “oxygen”. 

• Risks to patients – Needless imaging such as CT scans expose to radiation. Pan CT in trauma can be disastrous if clinical background is not taken into consideration. For me, it never made any sense to do CT for trivial chest trauma who look well and are hemodynamicaly stable. And if you happen to do a scan, don’t scan their whole body just because they are on a CT table. I recall a patient with blunt torso trauma (GCS 15/15, no evidence of head trauma, Normal CNS exam) who was undergoing a CT Abdo/Chest when a doctor jumped in asked to add a Head CT. His reason was – Anyways he is on the CT table, let’s do it! Not acceptable.

• Assurance/Avoidance behaviour: As described above, assurance behavior involves the charging of additional, unnecessary services in order to reduce adverse outcomes, deter patients from filing medical malpractice claims and provide documented evidence that the practitioner is practicing according to the standard of care so that if, in the future, legal action is initiated, liability can be pre-empted. Avoidance behavior occurs when providers refuse to participate in high-risk procedures or circumstances. A surgeon who was gutsy to take an unstable patient to operation theatre may not choose to operate high-risk cases in future if he gets sued due to a bad outcome. Every physicians behaviour gets shaped based on his past experiences and training environments.

Few Arguments against defensive medicine

• Documentation: Now we are doing BNPs to document heart failure in previously known heart failure patients. Amylase and Lipase and sent for every abdominal pain (RIF/Suprapubic pain as well!). This is the argument that comes with defensive medicine – We need to document that it was not pancreatitis! We are all aware that, no test is perfect in Medicine – there can always be false positives and false negatives. Therefore, it is crucial for physicians to interpret test results keeping history and physical in the background. Conversely, it can be frustrating from patient’s perspective if you tell them it can still be your heart (after two negative troponins, normal ECGs and 6 hours of wait!) 

• You never know! – I took care of this elderly gentleman lately from a nursing home. He was bed bound, aphasic, occasionally gets restless. On that day, he pulled out his urinary catheter. I thought of replacing the catheter and discharge him but ended up ordering CBC, Renal Function, LFT, Coags for him. The argument being we don’t know anything about him, he is aphasic, we do not have a history although he looks okay and hemodynamically stable. Let’s do this and make sure he is okay. This would sound like reasonable way to go for many. One of my mentors said something very interesting during a discussion about defensive medicine – Lakshay, this is what my patients want me to do and I think he was right to a great extent!
• It improves patient care: Defensive medicine certainly leads to more in-hospital admissions, more referrals and more follow up visits but what is the impact of patient care? This is what I picture  - more blood tests, more false positives, more imaging and invasive procedures (Biopsies, Angiography), unnecessary treatments, more adverse reactions, of course extra cost and utilization of resources!

In hospital Violence

Practising Medicine in India also put you at high risk of facing violence. Therefore, physicians justify their standpoint by doing every possible test. Nobody likes a bad outcome after spending a fortune and with negative prejudice against the physicians based on anecdotes  Indians are no different. In India, patient relatives prefer to settle things on the spot by creating a havoc and damaging the hospital assets. Interestingly, I overhead this conversation between a patient's family and treating ICU physician. She was very candid with the family. 

Your wife is quite critical and despite our best efforts, she may not make it. For now, she needs dialysis to support her kidneys. This might help her to recover but nothing in medicine is 100%. The cost of dialysis will be 10,000INR per session and she will require a few sessions for sure. I want you to be very clear on this. There is no guarantee that we are going to get her back even after dialysis but this will certainly give her a better chance. If things go wrong, I do not want a mob coming her and creating a havoc. I hope you understand what I am trying to explain. Any questions?

This post does not intend to justify or refute defensive medicine. Indeed it is getting difficult to practise Evidence Based Medicine in high risk environments and convincing patients about a certain test or treatment can be challenging. For instance, many want a Head CT with trivial head trauma. Shared Decision Making in one way to combat this but are we absolutely safe if we do that? 

In 2004, the case of Dr. Daniel Merenstein triggered an intensive debate in scientific journals and media on defensive medicine. Following the guidelines of several well-respected national organizations, Merenstein had explained the pros and cons of prostate-specific antigen (PSA) testing to a patient, rather than simply ordering the test. He then documented the shared decision not to order the test. Later, the patient was diagnosed with incurable advanced prostate cancer, and Merenstein and his residency were sued for not ordering the test. Although Merenstein was acquitted, his residency was found liable for $1 million. Ever since this ordeal, he regards his patients as potential plaintiffs: ‘I order more tests now, am more nervous around patients: I am no longer the doctor I should be’.

References:

• Kessler D, McClellan M. Do doctors practice defensive medicine?. The Quarterly Journal of Economics. 1996 May 1;111(2):353-90.
• Tancredi LR, Barondess JA. The problem of defensive medicine. Science. 1978 May 26;200(4344):879-82.
• Sekhar MS, Vyas N. Defensive medicine: A bane to healthcare. Annals of medical and health sciences research. 2013 Apr 1;3(2):295.
• http://timesofindia.indiatimes.com/india/Over-75-of-doctors-have-faced-violence-at-work-study-finds/articleshow/47143806.cms
• Hurwitz B (2004). "How does evidence based guidance influence determinations of medical negligence?". British Medical Journal. 329 (7473): 1024–1028. doi:10.1136/bmj.329.7473.1024.
• Atkins, D., Siegel, J. & Slutsky, J. (2005) Making policy when the evidence is in dispute. Health Affairs, 24 (1), 102–113.
• Merenstein D (2004). "Apiece of my mind. Winners and losers". JAMA. 291 (1): 15–16.
• Lapp, T. (2005) Clinical guidelines in court: it’s a tug of war. American Academy of Family Physicians Report, 2005. Available at: http://www.aafp.org/x33422.xml

Author:

              

     Lakshay Chanana

     

     Speciality Doctor

     Northwick Park Hospital

     Department of Emergency Medicine

     England

     @EMDidactic

                    

Lets talk about Headache in Adults!

From the Desk of Sagar Galwankar, MBBS, DNB, FACEE, MPH, Diplomat. ABEM, FRCP

• Headache is one of the common symptoms when patients come to the ED.
• Headache can be a Presenting Complaint when the patient arrives. "I got a Headache" and sometimes when patients are being evaluated at bedside for some other symptoms they can add the complaint "..and I also have an Headache"
• History takes paramount importance when a patient complains of Headache as a Primary Symptoms or a Co-Symptom as a part of a Series of Complaints.
• Always evaluate Headache keeping a 360 Degree approach. 
• Always address Headache. via your Thought process, History taking and Clinical Exam.

Vital Signs take a Lot of Importance and ask for them as you immediately prescribe pain medications to treat the PAIN.

Temperature, Pulse, Blood Pressure, Respiration, Pulse Ox, and Bedside Glucose are key stat Bedside Parameters which guide you to a story. Order an EKG Stat and read it.

Remember:  Gender does matter ! Pregnant Females & Females who do not know they are pregnant can come to the ED. Being Pregnant Changes the way you will evaluate these patients. Having abdominal pain, Hyperemesis gravidarum vaginal bleed can come with an headache and evaluating for abdominal emergencies and Ruling out Ectopic Pregnancy at the same time deciding about CT Head and Headache work up is a complex issue. They can also have HELP Syndrome or Eclampsia also can start with Headache.

• Age is crucial before Young Female with Headache and Cold and Cough is different from a 50 Year old with Headache and Blurry Vision.
• History taking should include: When did it start, how severe is it from a scale of 1-10, any other symptoms of Dizziness, Focal weakness, Gen weakness, Vision changes, neck pain, Syncope Seizures, Nausea, Vomiting Diarrhea, Chest Pain, SOB, Neck Pain/Stiffness, Dizziness, Vertigo have to be ruled in or ruled out.
• Past History of DM HTN CAD CVA Cancer HIV Hep B Hep C are important. Is patient on anticoagulants also is key history point.

Some Cluster approaches are:

• Fever, Tachycardia, Headache, Neck Pain: Here Headache can be as simple as a Viral Fever or as severe as early meningitis or even a URI if Cold Cough Sinus Tenderness are present.
• Headache could be a early Bleed (Subarachanoid) or even a CVA when patients have vasculitis, Bleeding disorders, Hypertension , DM.
• Headache can be due to Glaucoma or due to Otitis Media or even early Temporal arteritis.
• Headache can be segmental along a nerve for a early developing Zoster.
• Syncope, Fall, Seizure, Loss of Sensorium, Altered Mental Status with Headache all can be indicating a worse diagnosis  than how the Headache presented.
• Post Ictal Phase can present as Headache.
• Another confounder: MI/ACS can also present as an Headache so can arrhythmia or PE. So EKG Trop are Important.

There have been cases who have presented as an Headache and when you do labs there has been Low Hemoglobin and patient has a GI Bleed and the Immediate anemia has caused an headache.

Be very particular and alert when Patients says "Headache is what brought me to the ED". On the other hand there is tons of Literature of approach to Migraine in ED.

Its very important that you read the literature as there are various combinations of medications used to break the migraine. When a patient says "I have a Migraine attack" you still have to approach it as an HEADACHE.

Sometimes patients present with Neck Pain and Stiffness and we disregard it as "Slept on wrong side or Neck sprain". Evaluating for Cord Compression and keeping Dissection and SAH as a differential is equally important as much as ACS/MI or even a Retro pharyngeal abscess in a URI patient.

What it comes down to is:

• Vitals, Past History, Med List, Clinical Co-Symptoms, History of Complaint, Detail Clinical Exam to include total undressing of patient Neuro Vascular HFN HEENT Exam and Lab Results is crucial.
• Overdose and Drug abuse are important historical points which can indicate Cocaine abuse or even overdose unintentionally on paracetamol ibuprofen trying to self medicate with Over Counter Medications.
• CBC, LFT RFT Trop EKG UA Tox Screen and CT / MRI ESR are a part of the work up in ED.
• In a patients with Hypoglycemia or Hyperglycemia Ketoacidosis versus Toxicity v/s sepsis or infection has to be kept at back of mind.
• In HTN emergency headache can be because of raised BP and Raised BP can cause headache. Treating both is important but also is important ruling out cardiac pathologies a CT Head and look for Posterior Reversible Encephalopathy Syndrome.
• I have also read reports where patients were on anticoagulants and had neck pain and when MRI was done it has Hemomyelia into the spinal cord.
• There have been cases alcoholism where patients wake up with headache in ED but they dont know that Methanol or Toxic alcohols were also drunk and they have an Headache.
• Being very aggressive to rule out meningitis and SAH and using Spinal Taps with Clinical Co relation is important in the ED.
• Patients often return post spinal tap with headache and at this time Blood Patch becomes a choice after you have ruled out any other cause or pathology.
• Patients also have headache after Nitro given for Chest Pain.
• Fever can exacerbate Headache and Hunger can do that too.
• A TIA can be presenting as Headache being one of the Co-Symptoms.

Documentation:

• Always Document in detail the history the clinical exam and the plan for ordering tests and meds and chart your thought process and notes as you reevaluate the patient.
• That helps and maintains the continuum of care at the same time maintaining standards of care.
• Discharge is a crucial part. Here too Educating the patient and giving return instructions is key.

Do not Disregard or Less regard HEADACHE. Its a Part of the PAIN PATHOLOGIES which can cause PAIN if ignored.

Patients have Pain , treat it first but work it up and decipher the cause then treat the cause....  FOR THE PATIENTS !

Author: 

                                                   

Dr. Sagar Galwankar  

MBBS, DNB, FACEE (INDIA), MPH, Dip. ABEM (USA), FRCP (UK)

Chief Academic Officer of the INDO-US Emergency and Trauma Collaborative 

Assistant Professor 

Department of Emergency Medicine 

University of Florida, Jacksonville 

(Follow Dr. Galwankar on Twitter @SagarGalwankar)

FRCEM intermediate SAQ: It’s about momentum. How you build it. How you plan it.

I recently cleared my FRCEM intermediate SAQ exam. Many of my colleagues and juniors have been asking to share my experiences about it as the exam format is new. Also the pass percentage was very low this time (~15%)

I’d like to begin with what can go wrong. I will confess. This was not my first attempt. I had a torrid time giving my part B exams in June last year. I read a lot. I Read OHEM (Oxford Handbook of Emergency Medicine), Practiced online questions. Was I stressed about the exam? Perhaps a little too much. I was doing a lot of combined studies with friends and when I was alone the tension/ stress got the better of me. Sitting for even 10minutes with the book seemed like a great deal. And to set it off I used to browse FB/ chat/ watch YouTube just to cool things off. This caused a lot of wastage of time. The targets set for the day were not being achieved and getting procrastinated. Although I was already scoring around 100/160 in all practice tests but that was borderline. I was like ‘Read OHEM complete, revise it again’ also I had some cloudy concepts regarding dermatology/ choice of antibiotics/various fractures and injury management and many more. To eliminate that uncertainty I had to probably read and make my own notes. But how? 

The syllabus is enormous and time always seemed limited. So the exam date came closer and closer and I continued with my haphazard way of studying. I was hoping that somehow I will pass the exam. But on the night before the exam I had a sinking feeling that things were not alright. The doubts still existed. The uncertainties were still there. I could barely sleep that night. I was just hoping that somehow I will pass. But such prayers are rarely answered. Next day the exam was a disaster. The questions seemed familiar but the answers were not on my fingertips. I had to try hard to remember each and every answer. Since the accurate answers were not striking fast and smooth I was writing longer sentences to rephrase my answers. This took me longer time and the momentum never built up. You need that flow to answer some questions which are not straight forward and need some logical reasoning.

After the exam I was a little broken. Not knowing exactly where to start again. But I knew the problem was not only the knowledge per se but also how I need to plan and prepare for it. As the saying goes Proper Planning Prevents Poor Performance I realized I have to learn to study alone. I had to learn to sit still and. Have some self control so as to achieve my daily targets. Learn to stay calm and not panic. And for that I did try meditation. No rocket science, but a simple way of learning how to stay in the moment.

So now let’s come to the point. How to prepare for the new format of FRCEM SAQ intermediate exam. We will go through it retrospectively. Describing the exam process and proceeding backwards to the time now.

THE EXAM

This exam is not just a theory paper. The paper is designed to test your clinical acumen. Most of the questions are clinical case based scenarios supplemented with images. The exam has 60 questions of 3 marks each divided into 3 (1 mark each) or 2 SAQs (2+1marks). Time duration is 3hours. So that makes 180mins for 180 marks. You should be at the exam centre atleast 30mins before the starting time or at the reporting time as suggested. You will be required to verify your identity and then wait till you are called in and seated according to your candidate numbers. You will be provided with pencils/erasers/sharpeners and refreshments like chocolates/fruit juice/ water. You will be required to enter your details in the sheet provided. The timer/clock will be displayed where you can easily see it.

TIMING the exam

60 questions in 3hours. 180 marks and 180mins. You should aim to complete 10 questions in every 25mins. Hence try to finish all 60 in 150mins. Do not pause or wait too long at a question of which you are not very sure of or are doubtful. Keep moving. Keep time to come back for a second round. Mark with pencil the question you have left and have to review. There were candidates who failed marginally this time because they could not read all the questions! Do not make that mistake. You fail the exam even if you fall short by 1 mark or 10 marks. If you feel stressed out during the exam take a deep breath and exhale through mouth, take a sip of water and start again. Answers may strike you a little later when you have developed a flow. Keep moving. Time is of essence. I had only 15mins left for the second round but I think I managed to answer atleast 8-10 stems (8-10 marks then)

1 day before the exam and the morning: This day is very important. Every hour should be planned for. You have to reach the peak level of your preparation and stay calm no matter what. Things to take care of:

TRAVEL and STAY: If you’re travelling to a different city reach atleast by evening. Try to find a place of stay within 5kms of the exam venue as it may save you good time in the morning and helps in getting a peaceful sleep.

READING: You have to reach your peak level of preparations on this day. You cannot be carrying all of the study material or planning to revise everything on last day. So filter out. Prepare your notes of all the important material that may be volatile like drug doses/ scores/ treatment protocol/fracture names/investigation findings or whatever you think is vital. You should plan it properly. And DO NOT read anything past midnight. Just shut it off. Anything you read after that will do more harm than good. Do something to take your mind off the exam. Chill. Unwind.

SLEEP and the MORNING: People say to take a good night’s sleep before the Exam. Of course it is important but I find that advice very futile. I myself have never been able to get a sound sleep before a major exam and this was also no exception. But I think more important thing is to not fret over if you’re not able to sleep. We as emergency physicians can save lives even if we have been awake for 24hours straight. Stressing over lack of sleep does you more harm than lack of sleep itself. And when it’s the morning take a power shower that washes off all tiredness and anxiety and sets you all ready for the exam. Take a good breakfast. Get into the cab or whatever transport you’ve arranged and GET SET GO.

The WEEK before the exam: 6 days excluding the penultimate day

PLAN. EXECUTE. REPLAN. DO. KEEP DOING. This is how I will describe the last week.

PLAN your leaves/ duties well in advance. Arrange duty replacements or take leaves. I suggest breakup the last week or last 6 days as first 4 days and last 2 days of the week. You should have gone through your books atleast twice before entering this week. Try to finish subject wise in the first 4 days of the week. Break the syllabus into 4days and cover all that can be. (Syllabus available from

https://www.rcem.ac.uk/docs/Exams/2.2%20FRCEM%20Intermediate%20Certificate%20Information%2 0Pack.pdf)

Last two days try to read the high yielding and must know topics. Build the momentum and prepare the material you will revise the last day. For example I had notes written for antibiotics/ antidotes with doses/ treatment regimens and guidelines/ scoring systems/ ECG abnormalities/eponymous fractures etc.

Take practice tests: Simulate yourself atleast once according to the exam scenario – 3hrs & 60 questions. May be sit in a group or do it alone. You can use online question banks for this purpose. This exercise is most important to time yourself. Use pencil while writing answers as you will do in the exam.

Lastly all you need to do from the time now till you enter the last week:

Go through the curriculum and identify the areas that you find difficult to understand or remember. Read them through standard text books of your preference or some reliable internet sources. Clear your concepts and preferably make small notes about them. Organize and Simplify.

Oxford handbook of Emergency Medicine: Each and everything. Read atleast 3-4 times cover to cover. Mark sentences which could be possible questions.

Oxford handbook of Acute medicine (especially Dermat/ Rheumat/ Onco/ Practical Procedures/ Infectious diseases/ventilator modes)

I also suggest to google search images of the clinical conditions which involve a rash and take screenshots of them. Try to correlate the image with the definition of rash/ diseases. Similarly go through images of ECGs and radiographs. Go through as many images as possible.

Updated NICE/ SIGN guidelines for topics enlisted in curriculum. Also read Medicolegal and social aspects example Rape/ Violence/Abuse/ Consent/discharge advices. Go through Critical care basics like: Ventilator management/ modes/ permissive hypercapnea/ weaning strategy etc.

Online resources: There are few websites like ‘mcemprep.ac.uk’ and others which provide sample questions for you to practice. It is reasonable to subscribe them atleast 3 months prior to exam. Make a target of doing 10-20 questions/ day. Simultaneously try to finish off those topics from the books you refer.

Also I came across a facebook page named ‘MRCEM examination resources’ which gives out valuable information time to time. You may choose to follow it.

FINALLY the question... when to start preparing - Although there is no alternative to daily reading but you should ideally start preparing 6 months prior to exam date. Late starters may choose to begin with 3 months in hand but that’s a little risky. I also think people should have atleast 2.5yrs of experience in Emergency Medicine when they plan to attempt the exam. But remember it is never too late to begin. Plan properly whatever time you have. If you have months then plan your days. If you have days left then plan your hours. If you have hours then just take a chill pillBelieving in self and staying positive never hurt anyone.

That’s all about my opinion. I hope that helps! Cheers and All the best Guys.

 

Scores Final by Akshay Bhargav on Scribd

Author:

                                              Dr. Akshay Bhargav MBBS, DEM, MRCEM

Akshay is an emergency medicine enthusiast. Originally from Kanpur, he did his graduation from Kasturba Medical College, Manipal and his post graduation residency in Emergency Medicine from Apollo Hospitals, Hyderabad. He Loves teaching via simulation methods. His dream is to spread emergency medicine awareness among masses and improving standards of ED care in the country. As a student has always hated examinations but thankfully chose never to give up.

Angioedema - Bradykinin v/s Histamine

Angioedema
Angioedema is a Non-Pitting edema of reticular, dermal, subcutaneous and submucosal layers commonly affecting tongue, lips and upper airway (occasionally genitilia, abdomen, extremities)

Etiology

Allergic

• Mast Cell/IgE mediated - Type I hypersensitivity reaction
• Urticaria in common and a trigger is often present, acute onset
• Responds to Anaphylaxis medications

Non-Allergic 

• HAE (Hereditary Angioedema)
• AAE (Acquired Angioedema)
• Pseudo allergic 
• Idiopathic 
• ACEi related Angioedema 

Pathophys based classification
1. Histamine Mediated (Released from Mast cells or basophils)
Allergic or immunologic cause
2. Bradykinin Mediated
Hereditary or Acquired or ACE inhibitor induced edema
3. Idiopathic Angioedema 

Urticaria or Itching means histamine mediated reaction

Bradykinin affects more of deeper structures - not a/w itching but associated with pain and swelling

Presentation

Dysphagia, Change in voice, Abdominal pain, lump in throat, stridor, dyspnea 

Types:

1. Hereditary Angioedema  (HAE) - Accumulation of Bradykinin

• Autosomal Dominant (ask for family history). Can have airway or extremity swellings
• May present with recurrent abdominal pains due to mucosal swelling, urticaria is rare

1. Type I: due to C1 inhibitor (C1-INH) deficiency - increased bradykinin - 85%
2. Type II: Defective C1 inhibitor - need to test functionality 15-20%
3. Type III: due to defective factor XII (Normal levels and fiction of C1-I) - Rare

HAE often present with Erythema Marginatum - serpeginous erythematous rash but not pathognomic

• Triggers for HAE: Trauma, Medical Procedures, Stress, OCPs, Infection
• Clinical diagnosis but also show reduced C4 levels
• HAE does not respond to Anaphylaxis medications

2. Acquired Angioedema (Accumulation of Bradykinin)

Antibodies against C1 inhibitor  

AutoAb lead to reduction in C1-INH

Type 1- lymphoproliferative disorders (a/w lymphomas)

Type 2 - Autoimmune

Labs - Decreased levels of C1q (HAE - Normal C1q levels)

3. Pseudo allergic - not IgE mediated but mimics allergic AE

NSAIDs/Opioids/IV Contrast

4. Idiopathic - Unclear cause but common type of AE

All labs are normal

Chronic Urticaria can be a feature  

5. ACE inhibitor induced Angioedema - Accumulation of Bradykinin

• More prevalent in African Americans, Females, Elderly 
• ACEi interfere with bradykinin metabolism, therefore bradykinin mediated 
• Can present as "isolated genital swelling" or "peritonitic abdomen"
• Can develop with first dose or after years
• Other meds that can cause - Sitagliptin, CCBs, ARBs, Alteplase (tPA), COX inhibitors
• Patients who are on ACE inhibitor and get tPA are more likely to get tPA
• Can be unilateral as well!! (Look at the image above)
• ACEi Angioedema does not respond to Anaphylaxis medications

Recently I heard about a case where physicians did not give FFPs but intubated a patient with unilateral oral edema and also got a neck CT done. This patient was on ACEi for 6 years. FFP is a reasonable treatment option and must be considered prior to intubation. 

Consider sending a C4 level for undifferentiated angioedema (even if they are on ACEi, ACEi can unmask underlying Angioedema)

Differentials:

• Lip Abscess
• Panniculitis 
• Ludwig's Angina 

Treatment

Supportive Care

• Only a few of them require intubation
• Majority resolve with observation and treatment. AIRWAY is our concern. 
• Be concerned for potential airway compromise - Stridor, drooling, respiratory distress, change in voice 
• Be prepared for a difficult airway (Fiberoptic, Surgical)

Allergic Angioedema (Think and treat like Anaphylaxis)

• Epinephrine 0.3-0.5mg IM 1:1000
• Epinephrine drip/Steroids/Antihistaminics
• Fluids

HAE

Anaphylaxis cocktail does not help but often tried 

1) Traditional Rx - FFP (acute and chronic Rx), Antifibrinolytics (chronic), Androgens such as Danazol (chronic Rx)

Rationale for FFP: FFP contain C1-INH, other complement factors and kinin (dose 1-4 units for HAE). Few case reports mention worsening of angioedema. 

2) Newer Therapies

• C1 INH concentrates (Berinert, Cinrynze) - For acute treatment 
• Ecallantide (Plasma kallikrein inhibitors) - 30mg SQ, very expensive 
• Icatibant (Bradykinin B2 receptor antagonists) - 30mg SQ

ACEi Angioedema

• Anaphylaxis cocktail does not help but often tried 
• Discontinue ACEi
• Inconsistent data on ACEi induced Angioedema treatment 

Rationale for FFPsin ACEi Angioedema:

• FFP's supply Kininase II which functions similar to ACE and degrades bradykinin
• Inexpensive, easily available, improves symptoms
• Risks - Volume overload, ?Possible worsening of Angioedema

Admit if

• Past h/o angioedema
• Tongue/pharynx/laryngeal edema
• Lack of improvement in ED

Take Home

• Strongly consider FFPs prior to intubation in any patient with Bradykinin induced Angioedema
• Don't be surprised if Anaphylactic cocktail does not work for Bradykinin related reactions
• Get ENT/Anesthesia involved early. This is not the time to learn intubation!

References

• Temiño, Viviana M., and R. Stokes Peebles. "The spectrum and treatment of angioedema." The American journal of medicine 121.4 (2008): 282-286.
• Lewis, Lawrence M. "Angioedema: etiology, pathophysiology, current and emerging therapies." The Journal of emergency medicine 45.5 (2013): 789-796.
• Winters, Michael E., et al. "Emergency department management of patients with ACE-inhibitor angioedema." The Journal of emergency medicine 45.5 (2013): 775-780.

Author:

              

     Lakshay Chanana

     

     Speciality Doctor

     Northwick Park Hospital

     Department of Emergency Medicine

     England

     @EMDidactic