An Infected Heart Valve

I remember walking into this patient’s room like it was yesterday, even though it has been over a decade ago.  I remember this young thirty-some aged patient so well because of all of his tattoos he had, as well as his scraggly long hair tied into dreadlocks, which had seen better days.

“Hi, I’m Sharon and I’ve been asked to come and see you due to your being a new admission to our infectious disease service.  I see that the cardiology service has already seen you and they have asked for a cardiovascular consult.  Have you had your echocardiogram (ultrasound assessment of the heart, which shows its size, capability, ejection percentage, valve functions) yet?”

A ‘no’ answer came from the depths of the patient’s throat.  It was a clear, low bass voice that would have been wonderful and harmonious in a men’s quartet, if he had ever chosen to use it in a singing capacity. As he replied, he never moved from his position of laying on the hospital bed with the white sheet pulled up to his chin.  “When are you scheduled for it?”

Again this deep voice replied from underneath his dreadlocks, “I think they told me sometime later this day.  I think the doc from the cardiology service told me that they have to have one of the head docs do it, so they told me they would come and get me later to have it done.”

“Oh, okay, that means that one of the cardiology attendings will be doing it, so that means they will be sedating you, i.e. putting you to sleep.  Then they will put the ultrasound transducer down your throat.  You’ll be asked to swallow the transducer and then be quiet and not move, hence the use of sedation.”

“Umm,” came the reply from underneath the dreadlocks.

I swallowed hard, this patient interview was not going very well.  The patient was not exactly willing to engage in the needed conversation.   I was going to have to pull every shred of information out of him.

“Okay, how long have you been sick?”

Again, with little to no movement on his part, he answered, “about a couple of weeks.”

“Okay, what’s a couple of weeks to you?  Three weeks, four weeks, six weeks, what?”

“Umm, about a month I think.”

“Okay, when did your fevers start?

“About the same time as I started feeling bad, a month ago.”

“How high has your fever gone?”

“How am I supposed to know that?  I don’t take my temperature at home,” he replied somewhat perturbed.

“Hmm, alright.  Can I ask you whether you take any medications on a regular basis?”

 “No.”

“What do you do for a living?”

“I work as a day laborer when I feel like it, mostly carpenter work.”

“Ok, do you have any previous medical history, like any previous surgeries, or have asthma for instance?”

“Another ‘no’ came from beneath the dreadlocks.

“Do you use intravenous drugs at all, like inject heroin or some other street drugs?”

With that he grabbed his dreadlocks with both of his hands, pushing them aside from his face and partially sat up.  “Lady, I enjoy the highs I get from using my heroin.  Don’t you dare think of judging me for it, you hear me?”

“Sure do.”  

Seeing that I wasn’t getting very far with our new admission to the infectious disease service, I decided to change tactics.  I would just go read the cardiology history and physical and finish filling in the patient’s history from what they acquired.

“I need to do a physical exam on you, so would you mind sitting up for me?”

The patient reluctantly sat up as he crossed his legs.

I proceeded to do my physical exam.  The patient had numerous tattoos over his body, probably a total of ten.  One of them was a snake, another one was an anchor (typical of what former Navy vets have), another one was what I thought was probably a name of a former girlfriend.  Then there were several others.

The patient also had splinter hemorrhages underneath his dirty fingernails.  His hands were very calloused, which I assume was from his carpentry work.  His lungs were clear but his heart sounds had a definite 3/6 murmur heard over his tricuspid valve (one of the valves on the right side of the heart and can be involved in patients who have infective endocarditis.)  He also had numerous needle tracks on his forearms.

“Can you lay down for me so that I can do your abdominal exam, please?”

A grunt came from the patient’s throat as he laid back down on his hospital bed.

I found that he had a mildly enlarged spleen, with the rest of his exam being negative.

“I’m done now.  I’m going to go look up your blood work that they drew earlier on you while you were down in the emergency room.  I’ll be back later on with my attending.  Until then the nurse will be into hang another intravenous bag of antibiotics that they started on you while you were in the emergency room.  Do you have any questions for me?”

The patient grunted, no, so I left his room and walked over to the nurse’s station to look up his lab results.

Risk factors

Intravenous drug abuse

Prosthetic heart valve patients

Valvular heart disease (aortic stenosis, mitral valve prolapse, etc)

Intravascular catheters (usually used for cancer chemotherapy, hemodialysis)

Implanted cardiac devices (pacemakers, etc)

Surgical wounds

Presenting signs and symptoms of a patient with endocarditis:

Enlarged spleen

Fever (generally over 100.4 F)

Weight loss

New heart murmur

Skin lesions (raised macules or papules that have clotted off: called janeway lesions, or osler’s nodes which are small blood clots underneath the skin), splinter hemorrhages found on the nail beds, or submucosal hemorrhages found on the eyelids.

Eye changes can include roth spots which are swollen hemorrhages on the back of the eye (retina).

Presence of a systemic immune disease which can involve the kidneys or joint

Laboratory findings can include:

Anemia (low red blood cell count)

Increased white blood cell counts (these cells are responsible for fighting off infections)

Protein in the urine

Blood in the urine

Increased sedimentation rate (which is non-specific for an immune disease process)

Kidney insufficiency

Increased amount of circulating immunoglobulins

Positive blood cultures for a particular microbe typically associated with endocarditis

 

I pulled up the lab work that had been done on the patient just a few hours earlier.  The patient’s name was Michael and he had presented to the emergency room with a fever of 102 F and chest pain.  His lab work showed that he had an increased white blood cell count with the predominance of neutrophils (those are the specific white blood cells which fight off bacteria), a slightly low red blood cell count (mild anemia), his urine was normal, his sedimentation rate was markedly elevated at 100 (normal is below 20), and I noticed that there were two sets of blood cultures pending. 

I picked up the phone and called the microbiology lab. 

“Hi, this is Sylvia, how can I help you?”

“Sylvia, this is Sharon on the ID service.  Can you pull up this patient’s microbiology and tell me whether anything is growing out so far?”

Sylvia came back on the phone after pulling up his results.  “Sharon I just looked at his blood plates and nothing is growing out so far.  We won’t be doing a gram stain until we see some growth.  We’ll probably have some initial results tomorrow morning when your service meets with us in the morning, like you usually do.”

“Ok, Sylvia, thanks for looking.”

“Alright, see you in the morning.”

The following morning the microbiology lab did indeed have their initial results for us.  The culture plates had started to grow out what we called ‘purple clusters.’  This meant that the patient had a gram positive cocci growing in his blood.  This was typical of either streptococcus or staphylococcus.  So the patient was indeed on the right intravenous antibiotics, he had been started on Unasyn (a penicillin) and Gentamicin (an aminoglycoside). 

I went upstairs after microbiology rounds and looked up the patient’s echocardiogram that had been done late yesterday afternoon.  His echocardiogram had been done via a transesophageal approach.  It showed that he had a large vegetation on his tricuspid valve (one of 4 heart valves), with a slightly lower than expected ejection fraction (the ability of the heart to eject blood out into the system).  His ejection fraction was 45%, with a normal being above 60%.

Microbiology:

Staphylococcal species (42%)

Streptococcal species (40%)

Gram negative bacilli (2%)

Enterococci (which used to be classified as streptococcus group D)

Fungi  (2%)

Culture negative bacteria (8%)

Other bacterial organisms (6%)

Diagnosis:

Presence of any 2 major criteria or 1 major with 3 minor criteria, or all 5 minor criteria:

1) persistently positive blood cultures of organisms typical for endocarditis;

2) endocardial involvement (new valvular regurgitation or positive echocardiogram);

Minor criteria:

1)    Predisposing condition or IVDA

2)    Fever

3)    Embolic vascular phenomenon

4)    Immunologic phenomena (i.e. glomerulonephritis, rheumatoid factor)

5)    Positive blood cultures not meeting major criteria

Patients who are suspected of having infective endocarditis will end up having a transesophageal echocardiogram (ultrasound transducer placed in the patient’s esophagus or swallowing tube) instead of a transthoracic (ultrasound transducer placed on the patient’s chest wall) echocardiogram.  The reason for this is the lack of sensitivity with the transthoracic echocardiogram (i.e. 50-80%).  The sensitivity of using a transesophageal echocardiogram is 90-94%. 

Using the transesophageal approach involves sedating the patient so that they will be comfortable, during the procedure with having the transducer placed in their esophagus.

Typical findings on the echocardiogram of a patient who has infective endocarditis can include: a new vegetation found on a valve, paravalvular abscess, valve leaflet perforation or dehiscence. 

I went into check on Michael before my team began its daily rounds. 

“Hi, Michael.  How was your night?”

He grunted, “okay, I guess.” 

“Any problems with the antibiotics?”

“No.”

“Your temperature chart shows your fever is coming down, that’s nice."

“Hmm.”

“Did the cardiology service tell you the results of your echocardiogram?”

“Yeah, they told me that there’s something wrong with one of my heart valves.  That’s all I remember.”

“Well, with your history of using IV drugs, the positive results we found on your blood cultures this morning which is growing out a gram positive cocci, most likely a skin bug, and the presence of a new vegetation on your tricuspid valve means that you have what is called endocarditis.  This means that you have an infected heart valve which you acquired from your heroin use.  You’ll have to be on IV antibiotics for six weeks.  The cardiology service will also decide whether you need to have the valve replaced, i.e. whether you will need to have open heart surgery, this will be based on how well you do and whether you end up  with heart failure. 

“Umm,’ was his response.

“Okay, well can you sit up for me so that I can listen to you?”

Again, he reluctantly sat up for me as he crossed his legs.

I didn’t find any change on his physical exam.  I left his room and proceeded to see the other patients I was assigned so that I could be ready for our daily patient rounds.  On rounds my attending didn’t have anything else to add to Michael’s care, so we eft him alone. 

A day later I was at microbiology rounds again and they advised us (my attending and my fellow colleagues) that my patient was growing out Staphylocccus aureus.  With this information, Michael was on the right antibiotics. 

I went up later on and found out that his fever was again on a downward trend and he had started to eat again, his appetite had finally returned.  He must have started to feel better because this time I found him sitting up by the side of his bed talking to a friend sitting in the bedside chair.    

We kept him in the hospital for two weeks.  His heart function didn’t worsen as he had responded to the antibiotics.  But we still needed to have him continue his intravenous antibiotics for another four weeks.  So with the knowledge that we would need to keep a close eye on him and knowing that he needed help with his drug abuse, we asked the discharge social worker to find a drug rehab placement for him.  Michael agreed to be placed at the rehab facility, which allowed us to know that he would stay on his IV therapy. 

Treatment:

IV antibiotics and/or surgery:

Based on the patient’s microbiology and the sensitivity of the bacteria (typically bacteria although it could be a fungal infection) the patient’s is given at least 4-6 weeks of IV antibiotics to address the infection.  The duration of IV antibiotics is based on the sensitivity of the organism to the antibiotic and the rate of which it is typically known to be killed. 

For gram positive organisms (such as Staphylococcal, Streptococcus) patients are given a penicillin with gentamicin or Vancomycin with gentamicin.  Those patients who have a gram negative bacteria they are generally treated with ampicillin with gentamicin. 

Most patients (50%) will end up needing to have open heart surgery during their initial hospitalization.  Indications for surgery include:

1)    severe left sided valvular regurgitation, fistula formation and/or resultant heart failure

2)    evidence of persistent infection despite appropriate antibiotic therapy

3)    presence of a prothetic valve

4)    presence of an intracardial abscess or fistula

5)    recurrent emboli being thrown from a large infected vegetation on the heart valve

Even today with all of our advances in medicine and surgical options there still remains a high mortality (20%) for patients diagnosed with infective endocarditis.  Mortality these days is generally due to the patient resultant heart failure

 The cardiology service and us followed Michael over the next several weeks.  He stayed in his rehab facility and actually attempted to stop his heroin use.  He finished out his six weeks of intravenous antibiotics.  We ended up seeing him at 3 months after his discharge and at that time his tricuspid valve was healing, his heart murmur was less pronounced.  He was still being followed by the cardiology service to make sure that his heart function did not worsen.  But no one was surprised when he didn’t show up for his one year follow-up with the cardiology service, so what happened to him at this time, was anyone’s guess.

An Itchy Skin Rash

I walked into the exam room after knocking on the door. 

“Hi, I’m Sharon, I’m a physician assistant who is helping out in this clinic until they can find another permanent provider.”

“Hi, I’m Kim.  I was told Merilee has left, and the medical assistant told me that you would be in.”

“Well, it’s nice to meet you.  You’re here for your annual pap smear, is that correct?”

“Yes.”

“Okay, well let me start with your past medical history first.  Do you take any prescription medications or have any medical history?”

“I just take a birth control pill, that’s all.”

“You work at the local junior college?  What do you do?”

“I teach business classes.”

“Hmm, ok, well then do you have any new allergies, or previous surgical history that’s not on your medical record with us?”

“No.”

“Do you have any new concern, or something you need me to look at?”

“No, . . . wait, I do.  I’ve been coming here for the past seven years for my annual exam and every year I’ve asked the medical provider what’s wrong with my hair/scalp.  Every year I’ve gotten a different answer, one person told me that it was nothing, another told me that it was just dandruff and to use dandruff shampoo, another didn’t even look at my hair/scalp.  I think you get the picture, seven different answers, none of them right.  I can’t stand how itchy my scalp/hair is and I know it’s not dandruff!  I’ve tried all kinds of over the counter medications for my scalp and nothing has worked.  I’ve done dandruff shampoo, T-gel shampoo, tried using the 1% hydrocortisone cream, all to no avail.”

“Seven years you haven’t had an answer?”

“Yeah, I even asked for a referral to a physician who sees skin disorders and they wouldn’t give it to me.” 

“Okay.  I’ll make sure to take a look at your hair/scalp.  Anything else?”

“No.”

“Alright.  Well why don’t you go ahead and change into the paper gown and  put the paper cover over your lap, I’ll be back in to do your exam in a few minutes. “

After Kim changed over to the paper gown, I came back into the room with a small cup of water.  I asked Kim to swallow some water as I palpated her thyroid.  I then took a good look at her hair and scalp.  Her ears had yellow to red greasy raised macules on her earlobes.  She had scaly, yellow to red plaques on the edges of her hairline.  Her hair had a horrible case of dandruff.  All through her scalp her skin was erythematous and oily. 

Seborrhea dermatitis is a skin rash that affects 3-5% of the general population.  It typically affects areas of the skin that has sebaceous (oil) glands in it, i.e. face, scalp ,earlobes and/or trunk.  Lesions are usually described as being yellow tinged to red raised macules.  On the scalp patients also have lots of dandruff.  The lesions are very pruritic (itchy). 

Seborrhea is generally associated with a fungus found on the skin, Pityrosporon ovale.  Therefore by getting rid of the fungus with anti-fungal shampoo works to address the skin rash typical of seborrhea. 

  

“Kim, you have seborrhea dermatitis.   It’s very typical that it affects your scalp/hair line.  It can also affect other areas of your skin, such as your face or trunk.   Do you have any other skin lesions that are itchy?”

“Yeah, now that you mention it, I’ve got itchy lesions on my abdomen. “

“Okay, well let me look at them.”

Kim lifted her paper gown out of the way and showed me the additional skin lesions she had on her abdomen.  They were raised, yellow toned red plaques, again typical of seborrhea.

“Kim, these lesions are also seborrhea.”

“So, what do I do about all of this seborrhea?”

“Well, I’m going to give you a prescription for you to pick up a prescription strength 2% anti-fungal shampoo which is used in patients with seborrhea.  It should work really well for you.  Then I’ll give you prescription strength steroid cream to put on these lesions on your abdomen.  The shampoo should start working in about two weeks , if you find that you need additional help let us know.  You can also see a dermatologist (a physician who sees patients with skin disorders) if what I’ll give you hasn’t totally gotten rid of the seborrhea.  Fair enough?”

“So I do have ‘cradle cap’?”

“Cradle cap?”

“Yeah, I remember seeing a picture of an infant with cradle cap.  His scalp was just a sold red color with some yellow to red macules on them.  The description said it was ‘cradle cap.’ “

“Ah-h, okay.  In an infant , seborrhea  is called ‘cradle cap.’  With adults we call it seborrhea dermatitis.  So you were on the right track with your thinking. “

“Thanks for the encouragement that I can find the right information on the internet.”

“No  problem.”

I finished up the rest of her physical exam and sent her off to the lab to have her thyroid hormone level tested.  I also gave her a refill of her birth control pills as well as the two prescriptions for her seborrhea.  About a month later I heard from one of the medical assistants in the office that she had seen Kim in one of the local restaurants eating dinner with her husband.  Kim asked her to relay to me that the shampoo/steroid creams were really working for her and she wasn’t itching anymore.   That was news, I was glad to hear.      

What's With My Cough?

I was working in a family practice/urgent care clinic when I went into see a established patient of the clinic.  He was from India, having emigrated to the U.S. some 20+ years ago to acquire a better life for his young and growing family.  He had been working as an industrial engineer all these years and had recently just retired. 

I knocked on the exam door and then went in to introduce myself. 

“Hi, I’m Sharon, I’m working in this clinic for a few months to help out until they hire another permanent provider.  And you are,  A-jit?  Did I pronounce that correctly?”

“Yes, A-jit, with the emphasis on the first syllable.”

“Okay, Ajit, what can I do for you?”

“Well, I’ve noticed over the past 2-3 months that I’ve become short of breath when I’m out walking in the evening with my wife.  I’ve never been short of breath before when we walked, but it seems to be happening more frequently now.”

“Do you have a history of asthma?”

“No, I only have some problems with my blood pressure, which I take a daily medication for.  Nothing else.”

“So no other medications except your blood pressure med?”

“That’s right.”

“Do you have any other medical problems beside your blood pressure?”

“No.”

“Do you have any other symptoms, like coughing with your shortness of breath?”

“Hmm, I do wake up in the morning with some coughing on occasion, but I think that’s related to my drippy nose, I sometimes have problems with allergies.”

“Okay.  How about any smoking history?”

“Never!”

“Any alcohol intake?”

“I may drink one or two glasses of wine before dinner with my wife when we go out to eat, but that’s it.”

“Uh, it says here on your information sheet that you’re retired.  What did you do for a living?”

“I was an engineer.”

“Ok, what kind of an engineer?”

“An industrial engineer for  Dow Chemical Plant.  Where I worked they manufactured plastics of all kinds.”

“So were you on the manufacturing floor, where they were mixing all of the solvents, in otherwords coming in contact with the organic solvents they used to make the plastics on a daily or weekly basis?”

“Yeah, I was usually on the floor with the industrial technicians on a daily basis, making sure everything was running the way it should.”

“How many years did you work for Dow?”

“A total of 22 years, I just retired a few months ago.”

Working up a new patient who complains of shortness of breath needs to have the two main bodily systems worked up:  heart (cardiac) or lungs (pulmonary).    Cardiac disease can cause shortness of breath if the heart is not pumping correctly, or if the patient has a problem with one of their heart valves not working correctly. 

Pulmonary disease (lungs) can cause shortness of breath due to the lungs not being able to exchange air correctly or there being inflammation in the bronchial (airway passages) tubes which can cause an obstruction to air passing to and from (which is the reason that asthma exists). 

So when we (as the medical provider) have a patient come in and complains of shortness of breath we have to work up both systems.  In the office, we do a EKG (electrocardiogram) to look for problems related to the heart (it gives us a good estimate of heart chamber size as well as it’s rhythm) and also order pulmonary function testing to look for problems related to lung function. 

“Okay, well let me listen to your heart and lungs and quickly listen to your carotid arteries in your neck as well as look at your throat.  Then I’ll have the medical assistant come in and put the pulse ox finger clip on you and walk you around the clinic for 5 minutes.  That will tell me whether your oxygen levels drop while you are exercisng.”

The patient’s physical examination was normal, so I had him walk around the clinic with a pulse oximetry on his finger.  His pulse ox started out at 94%, and after walking for 5 minutes it dropped down to 90%.  So I explained to him that we were going to give him a albuterol nebulizer treatment and see what would happen to his oxygen level.  After his nebulizer his pulse oximetry went up to 97%. 

“I’m feeling better,” he stated. 

“I’m glad.  Before I sent you home, I also need to get a EKG on you to make sure that your shortness of breath is not related to your heart function.”

The patient’s EKG was normal. 

“Alright, Ajit, I’m going to send you home with a prescription for an albuterol inhaler to take every day.  I’m also going to set you up to go over to the hospital so you can get pulmonary function testing (PFT) done.  I should have those results by the time you come back into clinic in 3-4 weeks and then we will know whether you have asthma or COPD (chronic obstructive pulmonary disease).  So I’ll see you back in a few weeks.”

“Thanks for your help.  I go to the front desk and they’ll have my order form for the pulmonary function testing?”

“Yes, I’ll go give it to Laura, who will order them for you.”

“Okay.”

Giving a patient an albuterol inhaler by nebulizer helps to open up their bronchial tree (airway passage) so that they are able to exchange oxygen and carbon dioxide easier.  When a patient responds to albulterol this tells me that they have either new onset asthma or COPD.

Pulmonary function testing involves the patient exhaling air into a machine as forcefully as they can.  They also exhale air forcefully after they have received a bronchodilator treatment (generally an albuterol nebulizer).  These two results are then compared to look at the difference and to assess the response to bronchodilation medication.  These results are also compared to what would be expected given the patient's age and height (which is measured as the forced volume capacity).

 

Risk factors for COPD include:

smoking (80% of all patients)

occupational exposure to organic or inorganic dust particles, particulate matter, gas fumes which over time can causes inflammation of the airways

alpha 1 anti-trypsin enzyme deficiency (a gene disorder)

atopy (allergic reaction to inhaled particles)

tuberculosis survivors

Two weeks later I received the patient’s PFT testing.    This revealed that his lung function was not in the normal range.  He had airflow obstruction with his forced expiratory volume(air volume)  as compared to his forced vital capacity (FEV₁/FVC) was < 70% which was diagnostic of COPD (chronic obstructive pulmonary disease). 

Ten days later the patient was back in the clinic for his follow-up appointment.  His pulse oximetry was normal, after walking around the clinic for five minutes, his pulse oximetry didn’t drop at all.  He had been using his albuterol inhaler three times a day with a good response at home. 

“My coughing is gone, I’m able to walk with my wife every night without any problems.  I feel like I’m back to normal,” stated Ajit. 

“I’m glad to hear that.  Stay on your inhaler then.  Your pulmonary function testing came back and showed that you have COPD, which I believe is related to all of your occupational exposure to dust, organic particle matter and the like while you were working for more than 20 years at Dow Chemical.  So, we’ll need to see you every six months for follow-up and if you have any more problems with your breathing make sure to come back in and be seen, okay?”

“Sure thing.”

There are many medications which can treat COPD they include:

bronchodilators (airway passageways) such as albuterol inhalers, anticholinergic inhalers

steroids via inhalers to deal with the inflammation response

roflumilast (a new drug family that can be used in patients with severe COPD)

Patients are also advised to stop smoking (if this is the case), receive all needed vaccines (especially the annual flu vaccine), and antibiotics when they come down with upper respiratory infections.   Patients with severe COPD are also given oxygen supplementation.

Patients are treated in a step wise fashion with albuterol being the first medication we use, adding on steroid inhalers, long acting bronchodilators, etc. as the patient may need them. 

A Problem with Wheat Products

I walked into the exam room to greet a long time 58 year old male patient of the family practice I was doing some temporary work at. 

“Hi, my name is Sharon, I’m a physician assistant helping out in this clinic for a few months.  How can I help you?”

“I’m, John, nice to meet you.  I here because I just keep losing weight without trying.  I’m having a lot of diarrhea, abdominal pain and gas.  Every time I eat, I immediately have to go visit the restroom facilities due to another diarrheal episode.  I even wake up during the middle of the night and need to use the restroom due to my diarrhea.”

“How much weight have you lost?”

“Fifteen to eighteen pounds so far over about 3 months.”

“Are you having any fevers with your symptoms?”

“No.”

“Any nausea or vomiting?”

“No.”

“When was the last time you had a colonoscopy done to look at your large bowel?”

“I just had a colonoscopy done three years ago, it was normal.”

“Do you have any family history of cancer?”

“My older sister had breast cancer, she just died last year, she was in her 60s.  Other than that no one else. “

“Do you have any history of acid reflux, in other words do you take any medications like prilosec, zantac, or nexium?”

“No.”

“What about smoking or alcohol history?”

“I’ve never smoked and I only occasionally drink a glass of wine with my dinner.”

“Okay, good.  What medications do you take?”

“I only take a blood pressure medication, I think it’s called lisinopril?”

“You would be correct.  Lisinopril is a blood pressure medicine.   Any other meds?”

“No, I just put some hydrocortisone cream that comes over the counter on my elbows and knees every once in a while when my itchy skin becomes unbearable, it helps a little bit.”

“Do you have any other medical history besides your problem with blood pressure? “

“No.”

“Have you had any surgeries?”

“Oh, yeah, I had a hernia repair about 20 years ago and I had my appendix take out when I was a kid.”

“Okay.”

“Are you staying hydrated, drinking plenty of fluids every day?”

“Yeah, with all this diarrhea, I’ve made sure to drink plenty of water every day.”

“Do you ever look at your diarrhea in the toilet water before you flush it away?”

“Once or twice I did.”

“Did the diarrhea stools float on top or sink to the bottom of the toilet?”

“Now that you mention it, they floated.”

“What do you typically eat during a 24 hour time period?”

“Nothing unusual.  Cereal for breakfast with my coffee, a sandwich and fruit for my lunch and then usually meat and potatoes with a salad for dinner.  But lately I haven’t wanted to eat much of anything due to my having so much diarrhea.”

“How many stools per day do you typically have?”

“Hmm, let me think.  Hmm, probably about eight a day.”

“Okay, why don’t you sit up here on the exam table and let me listen to you.”

Once he got up on the exam table, I listened to his chest, heart and then asked him to lay down so I could do his abdominal exam.  His heart and lungs sounds were fine, his abdominal exam showed that he had lost weight (he was gaunt), but he didn’t have any tenderness or masses felt.  I took a look at his elbows and knees and noticed that he had an erythematous  rash which was raised with circular macules in groups.   The patient said that they were very itchy and sometimes burned. 

“Alright, John let’s start with getting some studies done on your diarrheal stools.  Do you think you could bring in a sample either later today or in the morning?”

“Sure.”

“Okay, well then I’ll have the the laboratory technician give you a container to collect it in and the instructions to keep it refrigerated overnight if you are not able to bring it back in this afternoon.  It will take about a week to get the results back, so when you go back out front you can make an appointment to come back in next week to be seen.”

“Okay, thanks for your help.”

“No problem.  Just keep drinking lots of water until we get this figured out.”

“Sure thing.”

Risk factors for celiac sprue disease include:

--genetic disease , typically occurs in Caucasians of northern European origin

Typical presenting signs and symptoms of celiac sprue include:

--diarrhea

--abdominal pain

--abdominal gas

--anemia (low red blood cell count, which are responsible for carrying oxygen from the lungs to the tissues/cells)

--skin rash

--weight loss

--osteoporosis (low bone density)

--arthritis (inflammation/pain of joints)

--infertility

--liver damage

--kidney disease (kidneys are responsible for creating a person’s urine)

After he left I went back and advised the laboratory technician of the stool studies I needed done when he brought his sample back in.  I ordered electrolytes (sodium, potassium), stool fat, fecal white blood cells (which would tell me whether his diarrhea was of an infectious etiology) and stool osmolarity.   A few days later his results came back and showed that he had a high fecal fat concentration, no white blood cells, with normal electrolytes.  So I knew I was dealing with a malabsorption disorder.  This could either be from his pancreas or it could be celiac sprue disease.

Seeing that he didn’t have a history of heavy alcohol use I doubted he was having problems with pancreatic insufficiency.  So this left his having celiac sprue disease as being the most likely diagnosis. 

John returned to clinic 2 days later and I told him what I was thinking and to confirm whether he had celiac sprue disease I would need to order some antibody testing (a blood test) along with a complete blood count (patients with celiac sprue disease can have anemia, or a low blood count due to their malabsorption). 

John understood and had the blood testing done.  I explained to him the diet requirements of a patient who has celiac sprue disease.  It involved staying away from any food that included gluten (which is the external portion of wheat protein).  I gave him several internet sources for patients who have celiac sprue disease. 

“I’m going on vacation to Florida for the next two weeks,” John stated.  “So I’ll make an appointment to see you after I get back.” 

“Alright, enjoy your time in Florida with your family.”

A few days later I received his lab results back.  His endomysial antibody was positive as was his IgA anti tissue transglutaminase.  He was slightly anemic (i.e. had a red blood cell count that was just under the normal range).    So I hoped that his changing his diet was working to address his symptoms.  With these positive lab results, it told me that his skin rash was another typical sign of celiac sprue, dermatitis herpetiformis. 

Diagnosing celiac sprue is done through:

--blood testing for the two antibodies involved which are IgA endomysial antibody and IgA tissue transglutaminase antibody.  (Both of these antibodies are immune globulin A related, i.e. IgA.  The second antibody is against an enzyme which breaks down glutamine.)

--pathological biopsy of the patient’s skin rash which has to come back showing dermatitis herpetiformis or acquiring a biopsy of the inner layer of the small intestine which will show flattened villi (the villi are what are responsible for absorping food nutrients).

A little over two weeks later, John re-appeared in clinic with his wife. 

“Sharon, this is my wife, Linda,” said John.

“Hi, nice to meet you,” I said.

“Hi.”

“Well, John I have your test results back and they show what I was expecting, you do indeed have celiac sprue disease.”

With that I proceeded to hand him the lab results and pointed out the positive antibody testing as well as his slight anemia. 

“With these positive results, it means that your skin rash which is so itchy and bothersome to you is a skin disorder that is related to celiac sprue disease.  It is called dermatitis herpetiformis.  As you get your celiac disease under control, your skin rash will go away.  Or I can give you a medication to make it go away quicker.”

“No, that’s okay, I don’t need anymore medications to have to take every day.”

“I have all that information you gave to John the last time he was here and we’ve been pulling off all sorts of additional information off of the internet about celiac sprue.  I’ve really tried to help him adhere to the gluten free diet, but it’s not easy.  I’m getting really good at reading food labels these days,” stated Linda.

“I can just imagine that it is a major change in your diet these days.  But tell me honestly, John how are your symptoms now?”

“My diarrhea is almost gone, I only have 1-2 loose stools a day now.  The abdominal discomfort I was having is gone.  And Linda says that I haven’t lost anymore weight, but I certainly haven’t gained any back either.  I think I’m beginning to feel better, I’m not as tired, I know that.”

“Well then you’re making some progress.  But staying on a gluten free diet is hard.  So let me make a referral for you to see the nutritionist over at the hospital.  She can help you come up with meal plans, food items to buy and give you some additional resources that you haven’t come across as of yet.” 

Linda stated, “that’d be great, I need all the help I can get.”

 

“Okay, John now one more thing is that most physicians would advise you to see an gastroenterologist (digestive disease physician) so as to undergo a endoscopy, which is where they put you to sleep and then put a scope down your swallowing tube, into your stomach and then on into the first portion of your small bowel.  It’s in the small bowel that they can acquire a biopsy of your bowel wall which will show the pathological changes seen with celiac.  This is the gold standard for diagnosing celiac.  Other than that I can send you to a dermatologist (a physician who specializes in skin diseases) and have then take a biopsy of your skin rash.  If the skin biopsy comes back positive for dermatitis herpeteformis then this would also seal the deal and tell us with 100% certainty that you have celiac.  But doing either one of these is up to you.”

“Neither one of them sound like much fun to me.  Based on the lab results and my response so far to my dietary changes I do believe I have celiac.  Therefore, I’m not going to do the biopsies,” John said.

“Okay, well let’s see what happens with your continued adherence to a gluten free diet.  It usually takes several weeks before you will have total resolution of your symptoms.  Meanwhile, I do need you to begin taking a multi-vitamin every day, this will address your anemia.  Make sure it has some iron in it, because your celiac disease has mad you slightly low in iron.  How about coming back into clinic and seeing me in a few weeks after you’ve seen the nutritionist and had some additional time to change your diet.  We can also repeat your blood work then and make sure that your anemia is gone.”

“That sounds fine.  So I’ll see you in a few weeks.”

Typical treatment for celiac sprue involves:

--nutritional consult, the nutritionist can go over a gluten free diet (patients have to stay away from wheat, barley, rye and oats, or any food product that has these elements in it).  They also give the patient internet sites, cookbooks for gluten free diet eating, etc.

--blood work to assess for vitamin/iron deficiencies due to the patient’s inability to absorp the necessary nutrients.

--medication to take care of the skin rash (if the patient requests it). 

--treatment of other symptoms such as their kidney involvement, infertility problems, arthritis, etc.

Six weeks later, John returned to the clinic.  He had come in the previous day to have his blood counts drawn and they showed his anemia was gone, which I was happy about. 

I walked into the exam room and greeted John. 

“Hi, John, how’s life treating you these days?”

“I’m better.  My wife and I met with the nutritionist twice and she has been really helpful with Linda, going over meal planning and the like.  My diarrhea is totally gone.  And I think I even gained 3 pounds.  But staying on a gluten free diet is not easy, that’s for sure.  Oh, and look my skin rash is even getting better.”

“Wow, I’m totally impressed, I have to admit it.  Good for you, John.  You came in yesterday and got your blood work drawn, which shows  your anemia is now gone, so you can stop your multi-vitamin.”

“Good.”

“Well, unless you have any other problems with your celiac disease, I don’t think you need anymore follow-up.”

“Alright.”

Over the next few months, John continued with his adherence to a gluten free diet and slowly started putting weight back on.  As he did, his usual energetic zest for life returned. 

    

Yet, Another Blood Clot

I walked into the exam room, not expecting to see or hear what was about to occur.  After knocking on the door, I walked in and introduced myself to the new patient.

“Hi, I’m Sharon, I’m a physician assistant and I’m here helping out for a few months.  How can I help you?”

“Hi, I’m Norma.  This is my husband, Steve.  I came in because I have pain and swelling in my left leg.  It’s just gotten worse over the past week.  Now I can’t walk on it.”

“Hmm, I see.  So have you had any fevers with this?”

“No, but I’ve been sick for the past month at home.  I was running 102 fevers a month ago for almost a week.  I’ve felt so bad for the past month from whatever I had that I haven’t gotten out of bed.  But now with my leg the way it is, I had to come in.”

“Okay.  What other symptoms did you have for the past month?”

“I was totally wiped out, no energy at all.  Didn’t have an appetite, just wanted to sleep all the time, but when I woke up I didn’t feel refreshed at all.  I also had some sinus drainage and a little bit of a sore throat.  But that all was clearing up when my leg started to bother me a week ago, and since then it has just gotten worse.  I’m self-employed, so I was trying to get some work done on my laptop computer at home, but I’ve only been able to do about 3-4 hours each day.”

“Has this ever happened before to you?”

“Yeah, now that you mention it, I had a problem when I was pregnant 25 years ago.  I had a blood clot in this same leg, with pain and swelling.  But it wasn’t this bad, this hurts all the way up to my hip.”

Risk factors for having blood clots include:

--history of immobilization or prolonged hospitalization/bed rest

--recent surgery

--obesity

--prior episode of venous thromboembolism (blood clot)

--lower extremity trauma

--malignancy (i.e. cancer)

--use of oral contraceptives or hormone replacement therapy

--pregnancy or postpartum status

--history of stroke

--age of > 75 years

--presence of an acute infectious disease

--female sex > male sex

Many physicians use the Wells Score to assess the pretest probability that a patient has a deep vein thrombosis (blood clot).  The patient is given 1 point each for any of the following:

--active cancer

--paralysis, recent immobilization of the lower extremity

--recently bedridden for more than 3 days or major surgery within 4 weeks

--localized tenderness along the distribution of the deep venous system

--entire leg swollen

--calf swelling by more than 3 cms. when compared to the asymptomatic leg

--pitting edema

--collateral superficial veins

Wells Score gives a high probability of a deep venous thrombosis if the patient has 3 or more of the above present.  They have a moderate probability is they have 1-2 of the above, The patient has a low probability of their score is 0. 

“So, tell me what happened 25 years ago?”

“Umm, I think I was about 6 months along when the blood clot showed up.  I had to be in the hospital for a few days while they started me on heparin.  Then when I was discharged, I was sent home on twice daily sub-cutaneous injections of heparin that I had to do until my daughter was delivered, then they stopped the heparin.”

“Did they do any follow-up labs with you to figure out why you had the blood clot?”

“No, they just discontinued the heparin.”

“ Alright, well let me ask you whether you have any drug allergies or take any medications right now?”

“I’m not allergic to anything I know of.  I do take lisinopril for my high blood pressure and fish oil for something to do with my cholesterol?  Is that what you call it, cholesterol?”

“Well, fish oil would be addressing one part of your cholesterol panel, specifically the triglycerides, so yes, you are correct in stating it is for your cholesterol.”

“Well, that’s all I know, what can be done about my leg?”

“We’re going to have to start you back on heparin if indeed you do have another blood clot.  But I also need to quickly listen to your lungs, heart, feel your abdomen, pulses, etc.  Then I’m going to send you over to the hopsital lab for them to draw stat labs on you.”

“Okay.”

I proceeded to do her physical exam and with the exception of her left leg everything was normal.  Her left leg had increased heat to it, it was swollen, I couldn’t palpate a dorsalis pedis pulse (the pulse on the top of your foot), nor could I palpate a pulse on the back of her knee.  Her pulse at her hip was decreased at 1+ (normal is 2-3+). 

After I finished up her physical exam, I excused myself from the room and went out to the front desk area and asked one of the office staff to hand me a lab/radiology request form for the hospital.  I quickly filled it out, requesting blood work which included a d-dimer (fibrinogen) and if the d-dimer was positive then they were to do a ultrasound study of her left leg. 

Physical findings for having a blood clot include:

palpable cord (reflecting a thrombosed vein), calf or thigh pain, unilateral swelling of the leg, increased warmth to the affected limb, redness and/or superficial venous dilation. 

Laboratory findings for having a blood clot include: a positive d-dimer (a test for the fibrinogen or clotting factors in the blood)which has to be above 500.  Patients also need to have their total red blood cell count, platelet count (small pieces of blood product that helps form the clot), and coagulation studies (how fast does the patient’s blood clot) done.  They should also have their kidney function tests done as well as a urinalysis.  Then while they are initially on heparin for the first few days they need to have their blood counts re-done daily to make sure that they are not having a problem with the heparin inducing a low platelet count. 

To diagnose how large the clot is (after the d-dimer comes back > 500) an ultrasound doppler study is done.  This assesses the circulatory system of the affected limb and can quantify how large the clot is (where does it start and where does it end). 

An hour later I received a fax from the hospital, the patient’s d-dimer was 4998, dramatically positive.  So I called them back and advised them to proceed with the doppler ultrasound of her leg.  the doppler showed her to have a continuous blood clot from her foot almost up to her hip (from her anterior tibial vein up into her popliteal vein and into her femoral vein.  It did not involve her iliac vein).   With those results,  she was admitted to the hospital and put on IV heparin.  She was kept on this for 5 days at which time she was switched over to daily Xarelto and discharged home to be followed up in the clinic.  She came into the clinic two weeks later and was doing better.  She was now able to walk on her left leg, but with discomfort. 

Treatment for blood clots include:

either in-patient our out-patient treatment with heparin (can be IV or sub-cutaneous injections)

close monitoring of response to make sure the blood clot does not progress

If the patient is going to be switched over to Coumadin then they are on both heparin and Coumadin together, if the patient is going to be switched over to Xarelto then the heparin can be stopped after the first dose of Xarelto (rivaroxaban) has been taken.  There are also two other agents which can be used besides Xarelto and these include dabigatran and apixaban.

Her leg was still swollen, and had some increased heat to it (but not as much as before her admission).  Her pulse at her hip was now more prominent and there was the possibility of her having a pulse at her knee (but that was questionable).  She was followed in the clinic with slow resolution of the large clot in her leg.  At 3 months, the Xarelto was discontinued, she was put back on subcutaneous heparin for two weeks and then had all of her work-up done for her presumed inherited thrombophilia (a predisposition for forming blood clots).

To ascertain the cause of the blood clot it is first determined whether it is a:

--congenital/inherited deficiency (factor V Leiden, protein C deficiency)

--acquired  (following surgery, trauma, antiphopholipid antibody, prenancy)

--associated with systemic disease (cancer, systemic lupus, inflammatory bowel disease).

Once the determination is made that the blood clot is likely inherited then pursuing their abnormal blood clotting factor should be done. 

Patients who have experienced their first blod clot are generally not treated with long term Coumadin beyond 3-6 months.  If the patients have a second episode, they are generally treated long-term (i.e. indefinitely). 

After all of her laboratory work-up was done, she was immediately switched back to daily Xarelto.  Several days later all of her work-up came back and she was positive for Factor V deficiency.  Due to the fact that this was her second episode of having a blood clot, she was continued indefinitely on her daily Xarelto.