She arrived walking, her sandals dusty. Her small heart, failing her, literally bounding out of her chest, somehow carried her three hours from her village and up the mountain to our small hospital in remote Achham, Nepal. I first saw her after being called by Chanakya Timilsina, our Health Assistant, who asked that I evaluate her since her heart was racing. She peered around her mother’s legs anxiously. I controlled my impulse to listen her heart right there as is often practiced here, and asked that she go to the emergency department. There, I listened to her story. She had presented initially quite ill with upper respiratory tract symptoms and a throat infection 6 weeks ago. At that time, she described difficulty with breathing, exercise tolerance, and palpitations over the course of several years. Her blood tests did not suggest acute rheumatic fever. Our medical team determined she had chronic rheumatic heart disease and started her on a regimen of penicillin every three weeks as well as daily atenolol and furosemide (heart medications). Since then, she has been symptomatically somewhat improved, had received her shot three weeks ago, and she presented again to follow-up for her penicillin shot.
I do want to provide some of the medical aspects of her evaluation for my medical colleagues, as I think it is instructive both about our resources and about the sheer injustice of her situation. I also think it exposes some of my own weaknesses and limitations as a doctor, which I hope my more experienced colleagues will appreciate, and how underqualified I myself am to be caring for this child in the middle of Achham. I do apologize to our lay readers for the jargon. She was small, anxious appearing, and ambulating. Her height was 121 cm and her weight 19 kg (approximately -1SD weight-for-height on the WHO standard). Her pulse was initially recorded as 140 on admission after her long walk, though on my repeated exams she was 100s-110s and was regular. Respirations 20. Blood pressure was 100/70. She had no rashes or nodules, and her hair appeared healthy. Her oropharynx was clear and mucus membranes were moist. She had shotty anterior and posterior cervical lymphadenopathy. Her chest was clear to auscultation bilaterally, and her breathing non-labored. On cardiac examination, she had a palpable thrill with visible right heart heave. She had a loud P2 without splitting, a gallop rhythm (likely S4), a 1/4 diastolic murmur at the base, a harsh, 3/6 holosystolic murmur at the LLSB (suggestive of tricuspid regurgitation) and a blowing systolic murmur at the apex radiating to axilla (suggestive of mitral regurgitation). She had jugular vein pressure was approximately 12 cm H20 though I was not confident in my assessment. Her PMI was not displaced. Her abdomen was soft without organomegaly. She had no peripheral edema, she had 2+ distal pulses that were not bounding, and she had no clubbing or splinter hemorrhages. She was quite thin but without noticeable muscle wasting.
With our QBC machine, we found a white count of 16 (80% neutrophils), Hgb of 14, and platelets of 380. Her ESR was 5.0. Her BUN was 8.0, Cr 0.6, and potassium 3.8 from our i-Stat machine. ASO titers were again negative. A previous chest X-ray had demonstrated left atrial enlargement and pulmonary vascular congestion. We performed a repeat PA and lateral which was slightly overpenetrated but approximately the same. There was no consolidation. We elected to use our ultrasound’s cardiac
probe to try to generate some images for our cardiology colleagues. I will admit that I was torn about this owing to the fact that I am neither a sonographer nor a cardiologist, but we felt it was worth doing in case it could provide some useful information. Note also that the cardiac probe has been partially eaten by a mouse which further compromises image quality. So this is clearly not the kind of evaluation that this child deserves, and I do think that there is a risk of this being a “toy” rather than of actual value, given our lack of qualifications to use it. But we felt it was worth it and the risk of ultrasound is nil. Our Health Assistant and Sonographer Uday Kshetriya performed the exam with me. On subcostal views, we noted a large dilated left atrium, dilated right atrium and mitral regurgitation and triscupid regurgitation jets on Doppler. The valves both appeared thickened. The aorta seemed normal, and we could not get a Doppler jet on it.
As such, our assessment was a 10 year old girl with long-standing symptoms of heart failure likely from rheumatic heart disease, with right-sided heart failure, tricuspid and mitral valve regurgitation, and potentially mild aortic insufficiency, presenting for follow-up from her village three hours away from the hospital by foot, recently started 6 weeks ago on a regimen of atenolol 25 mg daily, furosemide 20 mg daily, and every three-weekly benzathine penicillin 0.6 million units. She has symptomatically improved on this regimen, and today I felt she was at an appropriate fluid volume. We considered other measures for her heart failure, including enalapril and digoxin and changing her diuretic/beta-blocker regimen, but felt that at this juncture, given our challenges in monitoring and follow-up (no EKG, no local kidney, heart rate, and blood pressure monitoring), we would leave her regimen as is. We would discuss with our colleagues about arranging for surgery owing to the severity of her disease and felt that she would be a good surgical candidate since her functional status was quite good, as she was not malnourished and able to walk three hours to reach the clinic.
I asked Agya Poudel, our Director of Community Health, to discuss with the mother her diagnosis and follow-up. My poor Nepali language skills were particularly unhelpful in understanding the mother’s Achhami Nepali, but it was clear that the mother was quite appropriately emotional about her daughter’s state. She had heard previously from us that she needed treatment in Kathmandu, but had not had the resources to get there. Her concern was moving in an almost therapeutic sense for both Agya and myself, having seen the “ke garne” (what can one do?) resignation of so many parents here when facing the illness and death of their children. We asked that she come back next week to travel back with us to Kathmandu, and in the meantime we have started emailing and calling our cardiology colleagues. Within a few hours time, we were able to secure two free tickets via our partnership with Buddha Airlines. Such will be the long process of trying to help her remarkable but ailing heart.
These are the cases that both inspire and expose us. This little girl is surviving against the most abhorrently unjust physiologies, suffering a condition that should have been eradicated 60 years ago, walking three hours to a hospital that could offer her so little. She certainly deserves far more than my clinical skills. But we will get her to Kathmandu and we will get her a surgery. And we will continue to build better systems within our hospital to better serve our patients.
In the meantime, this ten year old’s heart, holding her mother’s hand, started to carry her way back to her village. It would be dark by the time she reached home.
Duncan Maru, MD, PhD is one of the co-founder’s of Nyaya Health. He is currently a resident in the Internal Medicine – Pediatrics program and fellow in Global Health Equity at Brigham and Women’s Hospital and Children’s Hospital of Boston.