Daktari wa damu

Welcome back to Daktari Cases.  These cases are NOT Ebola Virus Disease, and there are not (yet) any cases in Kenya.  We rejoice with Kent Brantley and Nancy Writebol in their recovery, and continue to remember those who are suffering in West Africa, and the brave men and women who are serving them.

No, today’s cases are examples of some mystery cases that I’ve seen of late that have ended up being outside my areas of expertise.  Life here stretches beyond your predetermined boundaries in many respects, and here are a couple of stories to illustrate.

John is a 52-year-old man from the other side of Nairobi with a 3-year history of a rash.  This itchy outbreak began on his hands and progressed to involve the entirety of his upper body, trunk, and lower extremities.  He had been seen at many health facilities, given many empiric treatments, and even had some response to topical steroids.  He had also variously been diagnosed with fungal infections of the skin and even leprosy, or a WTDNOS (weird tropical disease not otherwise specified), and that’s how he ended up coming to my attention at Tenwek.

 

After a glance and a gasp, I put on my gloves and had a feel.  A wise dermatologist once told me this, “If you know what it is, you don’t have to touch it.  If you don’t know what it is, for heaven’s sake, don’t touch it.”  Well, I once again ignored this good advice, and through this exam discovered that he had lymphadenopathy (swollen lymph nodes, lumps and bumps all over his body).

So, thinking there must be more here than meets the eye, and assuming it’s an indolent and potentially treatable disease, I rang over to my friends the surgeons to send off a skin biopsy.  “Tissue is the issue,” they say.  Anyhow, it turned out to be a Cutaneous T-cell Lymphoma, otherwise known as mycosis fungoides.  It turns out, since 1806 we have been mislabeling this slow-growing lymphoma as an infectious disease.  And if you’re going to get a lymphoma, this is one of the kinder gentler, and even treatable in Kenya ones.  And the treatment (here): nitrogen mustard.  If that sounds to you like a gas that killed people in trenches in World War 2, I think you’re right.

Our next patient is named Mary.  She is a 56-year-old Massai lady referred to me by my friends in an outlying clinic.  An aside, I get to go out to these clinics usually about one day per month, and it’s awesome: there’s no electricity, no running water, it’s just your wits and your stethoscope, and a handful of medicines.

Back to the story, this lady has a 1 month history of headache, with a pain in her left side “that is growing.”   So, I ask “what do you mean, ‘it is growing.’” At which she replies, “feel this.”  Protruding from the left upper quadrant of her abdomen is a subcutaneous American football.  But I’m not fooled, there’s not a real football in there, this is a massive spleen.  Ah, massive splenomegaly, the hallmark of many tropical infectious diseases.  This is a slide from a really smart guy from Mayo Clinic who taught us in Greece at the CMDA conference.

So, I ordered a complete blood count, and this is what we found.  So, I’m astonished by all of these results.  But most startling is her hematocrit.  We live in a land of chronic anemia, and it’s not surprising to see a patient walk into the hospital with some general malaise and a hematocrit of 7%.  This lady has a hematocrit of 70%.  She has the red blood cells of at least 3 Massai ladies.  This is abnormal.  The disease is called polycythemia vera.  It’s a myeloproliferative disorder (confusion in the bone marrow that makes too many red blood cells, and can be a precursor to leukemia).  And the treatment?  Very high-tech and modern: blood-letting, also known as serial phlebotomy.  It reduces the risk of stroke, the blood gets so thick it stops up the blood vessels in the brain.  So, once per week we are removing a unit of blood and keeping an eye on her blood counts.

These stories are ironic on many levels.  And that’s why this entry is called Daktari wa damu: the doctor of blood.

TB or not TB; That is the Question

Welcome back to Daktari Cases.

This case comes complete with a soundtrack courtesy of Merle Haggard, as well as an existential reference from Hamlet Act 3 Scene 1; so the recommended gameplan, start Merle out and have him playing in the background while you read the story.

The story of Charles goes like this: he is a young man of 25 years, a local guy from Bomet who grew up in the burgeoning Kenyan middle class.  He has lived the good life for our area: always had food on the table and school fees keep him on the track to success: education is the way out of poverty here, and Kenyans take it very seriously.

Then three years ago he started to get a cough.  He shrugged it off for a few weeks, until people started to notice.  Then he went to the clinic and told them his story: he'd had a chronic cough, and was an otherwise healthy young man.  In our part of the world, that means he has pulmonary tuberculosis (TB), plus or minus HIV.  His chest x-ray showed the abnormalities on the left upper lobe, and a sputum culture was sent.  Sensitivity for sputum culture in our setting is @ 50%, so most clinical decisions are made based on clinical evaluation.  For Charles, the clinical evaluation said, "TB" and he was started on TB treatment.

He felt better over a couple of weeks, but kept taking his treatment for the full prescribed 6-month course.  4 drugs x 3 times per day.

Nothing happened for a few months after he finished his initial treatment.  He returned to work and led a normal successful life for another quarter of a year.  Then his cough came back...same symptoms as before, and he returned to the clinic.  This time the exam and the x-ray showed the abnormality on the right side of the chest.  This was deeply concerning to the clinical staff, at the back of our minds there is always the question of evolving drug resistance in tuberculosis, and when we see these HIV-negative patients returning to clinic with a relapse or recurrence, there is a fear that they are in the 2-4% of patients with drug-resistant TB in our area.  Another sputum sample is taken and is negative.

So, Charles gets second-line treatment for the new set of symptoms and radiographic findings.  This time he gets 4 drugs 3 times per day, and a daily shot of a drug called Streptomycin, a very painful and inconvenient everyday experience for 9 months.

Once again, he gets better for a few months, and then has a recurrence of his symptoms.  He gets treated again with another course of intramuscular Streptomycin.

On the fourth recurrence of his symtoms, I get involved in his care.  He is admitted to the Tenwek Medicine Teaching Service, and we go back through his story.  He has had 4 treatment courses for sputum-negative TB that seems to move around from one side of his chest to the other.  Maybe there is something else going on here?  Some of the dangers of working in Africa amidst so much TB is that you start to call everything TB.

And for this case, I wasn't convinced.  I spoke with my surgeon colleagues, and asked them to do a biopsy of his lung.  At Tenwek we can do a procedure called a video-assisted thoracoscopic surgery (VATS), illustrated here.  This gave us a piece of tissue to examine: is this TB or something else?

And it turns out this case was of something else: Bronchiolitis Obliterans Organizing Pneumonia (more handily named BOOP).  It's an inflammatory lung disease that responds to a short course of steroids, something as simple as a week of prednisone like you get for poison ivy.

Charles doesn't have TB, nor does he need the stigma and fear associated with it.

That takes us back to the existential question that we on the medicine service ask ourselves daily: TB or not TB; that is the question.

Heart Reconstruction

Welcome back to Daktari Cases.  It’s been a few weeks since I’ve posted a case; not because there has been a dearth of interesting medicine, but really because there has been a surplus.  In addition to the usual holiday busyness (and we do have a less commercialized version of that here), there was a nationwide strike of healthcare workers in government institutions.  The details and causes of which I’ll omit (because I’m sure I don’t understand them).  The result was a very full hospital and outpatient department at Tenwek, which always runs at full capacity, over the past few weeks has approached “two-to-a-bed.”  With the New Year has come a settlement, and the reopening of the government hospitals, good for the patients and a breath of air for Tenwek.

So, I’ll start 2014 with a story.  The young lady’s name is Soi.  She is a 20 year-old woman from a village about 15 kilometers away who was married last year and is expecting her first child.  She finds herself getting tired and short of breath as she enters her 4th month, and when she asks her mother, she learns that this is a part of bearing a child.  Reassured, she continues to go about her day as a young wife and mother-to-be, working in the garden, carrying firewood, cooking and cleaning.  She lives beyond the reach of the electrical lines and water system, and beyond the reach of our prenatal care.  She carries on with life in the cool lush dirt-floor equatorial sun until one day she can’t walk to the river.  Then everyone notices that something is really wrong.  And they find a driver with a car and take her to Tenwek.

I meet her in casualty (the ER), and she is clearly in distress.  Her initial vital signs are a heart rate of 164, BP 94/68, RR 30, pulse oximetry 82% on room air.  These are her initial chest x-ray and electrocardiogram.  Her heart is beating faster than it can fill up with blood and she is going into heart failure.  We have to slow her heart down or this downward spiral will get out of control.  We have to decide to try to use medicine to slow her rate down, or to cardiovert her with the electric shock of a defibrillator (it’s not a good idea to shock a patient when you don’t know how long she’s been in this rhythm (foreshadowing: it could cause a stroke), not to mention that I don’t want to shock a mom and baby).  After some calling around, we locate some drugs that were left from the last cardiac surgery team at Tenwek that will ease her heart rate down, and give her some diuretics to pull off some of the fluid that has collected in her lungs.  This happens over the course of the first night, and so as the beautiful African equatorial sun rises on the next morning, we start to ask “Why?”

Nothing in my medical education to date gave me an explanation of why an otherwise healthy 20-year-old woman would have this tachyarrhythmia as she enters her second trimester.  But the first storm has passed, and it’s time to get to the thinking part.  As I repeat her exam, and now that her heart rate is down around 100, I can hear new sounds: the “whoosh-thump-whoo” of a mixed systolic and diastolic heart murmur.  We are blessed at Tenwek to have echocardiography capability and this is what her echo looked like.  It shows an extraordinarily scarred and narrowed mitral valve with just a teeny jet of blood flowing through it, along with a very large blood clot in her left atrium.  This is a picture of severe mitral valve stenosis from longstanding rheumatic heart disease.  RHD is a disease of poverty from repetitive bouts of untreated strep throat.  She has been living, miraculously, with this severe heart disease, likely since her childhood.  Yet in pregnancy, her blood volume increases by about 50%, to accommodate the baby.  The way her body changes to nurture the baby has overwhelmed her damaged heart.  And the strain on her body will accelerate over the next few weeks; she won’t survive pregnancy without open heart surgery, and having that large clot in her left atrium gives her the added risk of a major stroke.

This is one of those times when I’m thinking about what it would be like to work in a walk-in clinic in Tennessee: “Oh, you have a backache, I’m sorry; here are some painkillers.” or “Is that a sniffly nose?  Should I give you some antibiotics (editorial note: NO!)” 

Anyway, I snap out of that daydream after about 1.4 seconds and call in a lifeline: Dr. Russ White, cardiothoracic surgeon extraordinaire.  I am SO thankful to work in a place like Tenwek with such awesome colleagues.  Yes, he’s seen this before. Yes, it’s a tough case with a lot of risks no matter what we do.  And Yes, we can do open heart surgery, replace her valve, remove that dastardly blood clot, and give her a chance to live.  The downsides: there’s very little or no chance that the baby will survive being on cardiopulmonary bypass, and there’s a fair chance that clot will travel up into her brain during the operation and cause a stroke, and the family will likely have to go bankrupt to pay for the operation and ICU care (even though it’s the cheapest open heart surgery in Africa).  We have a few days to weigh the risks/benefits while we try to optimize her health, and the family involves their community in a fundraising drive and all-night prayer meetings. 

At this point, we decide to go ahead with the surgery, and this is the point in the story where you come in.  Because many of you are our supporters, and I told the hospital finance department that we will pay to make this surgery happen.  The community in their village gave, the family sacrificed greatly, and we filled in what was lacking.  To the Operating Room…

The procedure was long and complicated.  The valve was deeply scarred into the surround heart muscle, the clot was hardened and calcified, making it difficult to remove (but able to be removed intact).  The baby didn’t make it through the procedure, and there were times when the survival of the mother was in doubt.  But this time when they shocked her to restart her heart, it beat in a smooth, healthy, regular 88 beats per minute.  Several days of recovery later, she walked out of the hospital and caught a ride back to her village, with a story of a miracle.  Reminds me of a story that the prophet Ezekiel told
-->: “I will give you a new heart and put a new spirit in you; I will remove from you your heart of stone and give you a heart of flesh.” (Ezekiel 26:36).  And to those of you who are our supporters, this is your story too.  Thank you for giving and praying, so that we can be here, and giving so that people like Soi can be healed.  A piece of her new heart comes from you.

Some Days Are Harder Than Others

Greetings from the Daktari Cases blog (we've decided to marry the clinical cases with the family life and missions blog, beware medical images and descriptions.  Previous cases: www.daktaricases.wordpress.com).  One of my goals in writing is to introduce you to and connect you to our work here.  And that includes the tragedies, as well as the victories and the comedies.  Our patients come to the hospital desperately sick, often on the brink of death; and our inpatient medicine mortality rate averages 10%.  Of the 40-50 patients that I see every day, 4-5 of them will die in the hospital.  These are a couple of their stories.

Joan was a 37-year-old woman who came to the hospital after having a septic abortion.  All abortion in Kenya is illegal, and when it is done by the hands of a non-medical practitioner it is called a “criminal abortion.”  When I was asked to see her in the ICU, she was unable to speak, and I will never know why this happened.  I was asked to see her in the ICU for multi-organ failure and sepsis.  

 The source of her infection was a necrotic uterus, an all-to-common complication of these illegal “procedures.”  As we attempted to resuscitate her with IV fluids and antibiotics, there was an ongoing discussion with the obstetricians about whether or not she would have to have a hysterectomy to remove her uterus, her womb, and the source of her overwhelming infection.  Through the course of the day, a repeated CBC showed that her hemoglobin and platelet counts were dropping precipitously.  Her body was consuming her own blood products (a condition called DIC: disseminated intravascular coagulation).  The treatment for this condition is with replacement of those blood products; ideally with FFP (fresh frozen plasma) or cryoprecipitate, neither of which are available at Tenwek.   

What we can give is fresh blood, straight out of the donor, through the screening process (to insure against transmission of HIV, hepatitis etc) and into the patient.  And there was no fresh blood.  Her blood was typed: A-positive.  Do you know who else is A-positive?  I am.  By this time, it’s getting late in the day, and I was on-call the night before, and I’m tired.  The only chance this woman has to live is to receive fresh blood during the night and then have surgery to remove her necrotic uterus after the transfusion.  So we come up with a plan: I go to the blood bank, give my blood for the transfusion, and then we set-up the OR for the moment the transfusion is accomplished.  After the blood bank, I go home, pretty well spent.  That night, during her second transfusion, her heart stops beating, the code team comes to resuscitate her, and is ultimately unsuccessful.  And in one week last month 3 pregnant women died: one of AIDS complications, one of status epilepticus, and one of a stroke from a rheumatic mitral valve.  Prior to moving here, I had never seen a pregnant woman die in the hospital.

Stanley Rotich was a Good Samaritan.  He was 28-years-old and had cared for his brother dying of AIDS complicated by tuberculosis in their 10x10 foot one-room home.  It only takes one deep breath in the right circumstances to acquire TB, and when  Stanley was brought to our hospital, he was in respiratory failure, requiring high flow oxygen to keep him alive. This was his chest x-ray, which showed miliary TB, one of the most aggressive and advanced forms of TB, spread through the bloodstream.  He remained in our ward for 5 days, and this image is copied from the clinician and chaplain notes of his last 18 hours of life before he passed.  This is one of the cases that are so hard to understand that challenges my clinical judgment, and my faith.  Is this a tragedy?

These are some of the cases of note from the past few weeks.  The stakes are very high here; the acuity of these patients once they get to the hospital is almost always a matter of life and death.  There are no cases here of indigestion in an 85-year-old great-grandmother that undergoes a nuclear stress test to insure that it’s not a subclinical heart attack.  Sometimes I miss those cases.  But this is my dream job- to be living out my true vocation (calling).  Thank you for being interested enough to read these stories, to pray for us, and to support us.