She would have been four years old on March 31, 2007. We were planning her birthday party (she wanted a Tinkerbell party). Instead, we cremated her last Sunday.
This is a rather long story of joy, heartbreak, agony and what-if – all rolled into one.
It is also a story about the triumph and tragedy of our medical system
For nearly four years, she brought nothing but sunshine and joy into our lives and the lives of everyone she touched – her friends, relatives, her school teachers and casual strangers who met her at Sesame Place.
And then she delivered her heartbreak.
She was diagnosed with a simple urinary tract infection on Friday. Her pediatrician recommended that she go to the ER should she have vomiting at night since that may be a sign that the infection is spreading to the kidneys. No big deal, just needs a booster shot of antibiotic.
So, she throws up her food at midnight and her dad takes her to the ER. Standard tests, shot of antibiotic and we are back home. She has a rash and high temperature, but the ER thinks nothing of it.
The child has been very healthy. Nursed for three years, she is in the 99th percentile in everything. She has fought off other infections before. Her biggest concern now is whether she can go to school on Monday. She desperately wants to.
It is important for any physician to be able to early identify potentially life-threatening disorders in diseases that may lead to severe, permanent disabilities, in particular if they are treatable. Hemophagocytic lymphohistiocytosis (HLH) is such a disorder, although rare.
Saturday goes by with regular doses of Tylenol and ibuprofen. No big deal, but fever persists.
On Sunday, the fever spikes to 105. The Pediatrician is concerned – take her to the ER and have her checked out. So off we go to a facility that is designed to deal with emergencies but has to see a child with a fever because that is the only medical facility open on Sundays in this country with the ‘best healthcare system in the world’.
More tests, more bloodwork, (more co-pays) but nothing definitive. This time, she is discharged from the ER with a diagnosis of ‘viral infection’. Second visit in three days, cause of illness unknown but patient is discharged, and not admitted for observation.
Monday’s visit to the pediatrician raises no alarms. Fever is still high after the visit, rash is persistent but nobody thinks anything of it. After all, she is almost four and four-year-olds always get some kind of fever or the other – or so we are told.
The most common early findings are fever, hepatomegaly, and splenomegaly (Table 1). Other early symptoms include a skin rash, lymph node enlargement, and neurological abnormalities.
She is not keeping much food down and her parents are now concerned. Three days of high fever, persistent rash and vomiting. Alarm bells are ringing louder now, but only in the pit of the mother’s stomach. The medical system is not concerned. She goes back to the ER on Tuesday night with a spiking fever of 105 and accompanying shivers.
One of the best facilities in the country, she has come back for the third time in four days and still no attempt to admit her. This time the diagnosis is strep throat. What of the fever? Probably a reaction to the antibiotics, so stop the antibiotics. The urinary tract infection is cleared up anyway.
The splenomegaly and hepatomegaly are usually pronounced and progressive. The rash is uncharacteristic, transient, and often associated with high fever. Lymph node enlargement develops in less than half of the patients but may occasionally be marked.
The child sleeps through Wednesday. She wakes up at about 5 pm and is unable to stand. She falls over backward
Now the volunteer EMT dad has alarm bells ringing very loud. Another trip to the ER, this time in an ambulance and an IV to combat dehydration. The almost four year old is starting to look scared. Finally, a smart doctor in the ER catches on to the abnormal blood results, but by this time, the seizures have started. The child starts to lose consciousness. The doctors are now scrambling to identify a cause. Nothing fits – the blood work and clinical symptoms do not match viral meningitis, bacterial meningitis, West Nile virus, Epstein Barr, Reyes syndrome ... dad is scared, mom is terrified and the doctors are puzzled.
A spinal tap early Thursday morning shows increased intracranial pressure, but fluid is clear.
Although the signs of central nervous system (CNS) involvement may be pronounced already early, it is more common that they develop later during the course of the disease (Table 1). However, it is important to be aware that these symptoms sometimes may dominate the clinical course. The picture may include irritability, bulging fontanel, neck stiffness, hypotonia, hypertonia, and convulsions. Cranial nerve (VI-VII) palsy, ataxia, hemiplegia/tetraplegia, blindness, and unconsciousness may also develop, as well as nonspecific signs of increased intracranial pressure.
In the spinal fluid, a moderate pleocytosis with mainly lymphocytes may be found (5-50x106/L), as well as elevated protein levels (Table 2). Note that caution with lumbar puncture must be taken with regard to a possibly increased intracranial pressure. MRI or CT of the brain may show abnormalities, in particular later in the course of prolonged disease, representing areas of past or ongoing inflammatory activity or demyelinization areas. Bleeding, atrophy and brain edema may be found. Hyperdense areas on CT may falsely be interpreted as calcifications.
It is important to treat the disease prior to the development of permanent neurological disabilities. However, it may be difficult to establish the diagnosis. Differential diagnoses presenting with CNS problems include, among others, encephalitis, degenerative cerebral disorders, and neurometabolic disorders.
She was finally admitted to the ICU early Thursday morning. At this point all communication with the outside world has been lost. Dad has forever lost his vivacious, happy, buoyant, ever-smiling wonderful three year old. Mom has lost the apple of her eye, her pride and joy and her Sunshine but does not even know it yet.
Post intubation, she goes into cardiac arrest. Rapid CPR and chemical intervention brought her heart back but her pupils were fixed and dilated.
Sandhya Yamini Suresh died on Friday at 5:22 pm.
Exactly a week after she had been first diagnosed with a simple, uncomplicated urinary tract infection.
Oh, we had all the medical coverage we could want. She was seen in one of the best children’s hospital in the country.
But then the what-if’s began – what if they had admitted her on her second visit? What if there had been a medical database that listed her symptoms and associated it with the condition that seemed to have killed her – HLH? What if there was a medical database that linked all ERs nationally?
What if the system had been focused on delivering care and not focused on managing cost?
Would it have made a difference? We don’t know.
All we know is that the hospital did not think it was appropriate or necessary to admit a patient after a third visit to the ER in five days.
The triumph, if I can call it that, of the medical system knowledge base is that this condition is well documented. A simple google search pulls up the pathology of the disease.
The tragedy was that it was unknown to most of the specialists who were responsible for her care.
It was finally diagnosed (it is still a working hypothesis at this point) by a Pediatric Hemato-Oncologist.
I have been here at DailyKos for a while. I have watched Kos’ personal blog as he documented his wife’s pregnancy. I have read and shared nyceve’s diaries on our medical systems. I have watched MSOC fight her demons in public. I have cheered them on in their quest.
Now I am asking for the support of this community. Is there a HLH support group? Google does not seem to know of any. The literature calls this an ‘underdiagnosed disease’, which if identified early can be managed, corrected and even cured.
How can we get overworked ER doctors to focus on anomalies when they have an assembly line of non-emergent cases that come to the ER simply because there is no other alternative? How can we get doctor’s offices to stay open on weekends?
Thank you for listening.
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