I think Wendy Nohoemi and Herlyn Querubin (boy in heart failure) are going to vie for the distinction of most difficult patient to leave behind.
Wendy came to see me for the first time not even two weeks ago. Despite the fact that she was 4-years-old, her mom carried her into the room strapped to her back and she was crying. Wendy and her mom arrived just before the lunch break, typically when the last couple of patients who don’t have numbers (appointments) squeeze through the door for a quick little visit (of course, just like back home, it seems these “little” visits often are quite the opposite). Wendy looked frail and sick. She sat on the exam table stiff and seemed to be bracing against the possibility that I might touch her anywhere. I started to get her story from mom, which only served to confuse me.
Wendy had been sick for 6 months. The list of problems she’d had over that time seemed something of a random list of symptoms inconsistent with any type of disease. She had had swellings over her forehead that then passed to her neck and then her arm and then her legs and now her feet. The swellings would form suddenly, last for minutes, then resolve. They hurt but weren’t red. They were 2cm or so. They would develop 4 or 5 times a day but they had resolved over the prior week, ever since Wendy got an injection from a curandero (Mayan medicine man). Mom brought the vial (first time I’ve seen that happen this year!): lincomicina. I’d never heard of this medicine (later found out it is essentially clindamycin) but it’s hard for me to imagine that any injection was enough to stop these swellings that had been coming daily for 6 months. I started to wonder if I could get a reliable history from this mom. She recounted that Wendy had complained of pain in various parts of her body off and on over the 6 months but there were rarely changes that she could see or sometimes there was just swelling. I started to question the history even more when she said she had taken Wendy to a doctor in town one morning after Wendy had woken up unable to get out of bed, but when they got to the doctor’s office she was fine, pain-free, and he didn’t see anything wrong. This was three months into her illness. She said that over the 6 months, there'd been fevers most days. Also, Wendy could no longer walk and there were days when she wouldn’t get out of bed because of her pain and she hasn't had an appetite and has lost weight. At this point, I was starting to take this much more seriously. Mom reported that now it was her neck that was hurting and she can’t turn her head to the right. I asked if Wendy had seen any other doctors over this time. She’d seen one just a month ago -- had travelled all the way to Panjachel to see a pediatrician. The doctor had done some tests (and mom brought the papers!!!!). She’d diagnosed a UTI on UA (lots of whites but neg nitrites, 10 RBCs) and Ascaris from a stool sample. The blood count showed 13.6K WBCs with 81%N, 13%L. Plts were 426K and there was a mild microcytic anemia (Hct 30.6). She treated the UTI and worms appropriately but apparently didn’t have an explanation for the chronicity or severity of the problem and didn't treat the anemia.
I took a deep breath and moved on to the exam thinking this was probably going to be some form of tropical disease that I wouldn’t recognize, much less know how to evaluate. As I mentioned before, the girl was scared and trembling but seemed to be trying to let me examine her. Her right neck hurt (maybe along the SCM or deeper?) and she couldn’t turn it but there was nothing to see. Just shoddy cervical lymphadenopathy. HEENT was normal. Heart, lungs, abd were normal. When I had her sit up, she was very tentative and guarded. She wimpered. Her knees looked knobby, maybe big but not red. She was so thin, I wondered if that accentuated the large appearance of her knees. Her ankles looked a little swollen and they hurt but they weren’t red either. Her fingers even looked a little swollen but they weren’t red. She stood, but tentatively. I had that “this is bad” feeling but for some reason, it didn’t hit me right away. I called Rafael in for his opinion and as we were waiting for him, I asked mom if she thought it might have been Wendy’s joints that hurt her. Mom didn’t know what joints were but I pointed out those particular body sites and, yes, those were what seemed to hurt. It’s odd to me that it took me even that long to figure out what had been hurting but, with that, it all started to come together: polyarticular arthritis. I summarized the story for Rafael and it seemed to suddenly fit. What hadn’t seemed to make any sense before now fit together. He agreed. Now what? It was clear mom was very poor, she was from a small, rather far away hamlet, and we were dealing with something serious.
I suggested that we send her to a rheumatologist: maybe a big bill up front but after the diagnosis, we could manage the condition with the advice of the rheumatologist. Rafael said things didn’t work that way here. We’d need to make the diagnosis first and then hand her over to the rheumatologist for management. He suggested an ESR and RF. I said I thought we already had the diagnosis, clinically, (though and ESR would be good) but really she would need a rheumatologist. I was feeling this was way out of my league. Rafael explained the options to the mom: spend lots of money and time and go to the City to see the rheumatologist or stick with us and we’ll do the best we can (which, he said, would be good, it just might take us more time). I didn’t have that same faith and thought, “please say you will see the specialist” but of course she said she’d trust us to do the best we could and, with that, Rafael stepped out.
I sent her for an ESR and RF, gave her as many diclofenac samples as she could fit in her bag as well as some iron and asked her to come back on my next clinic day four days later. Her ESR was 127, RF negative. The lab tech noted a lot of white cells so did an impromptu count and found 31K. I went home and crammed as much information as I could about JIA (and ALL). I went to the local pharmacies to see what medications from the long list of treatment options might actually be available, if any. They didn’t have any. I wrote Jenny Frankovich, an amazing newly graduated rheumatologist at Stanford who was a resident with me. She wrote me back immediately and was incredibly helpful. She converted all that “gold standard, rich country, unlimited resources” information I’d gotten on UpToDate into a realistic list of things that would truly help us make a reliable diagnosis and begin treatment. She said Wendy had a good chance of responding to the treatment and, perhaps, the response would be marked. She even wrote her Guatemalan friend, who is head of the Children’s Oncology Hospital in Guatemala City, to solicit his help in ruling out leukemia. I was super-charged.
I called my friend, Emilio, in the City and asked him to help me get methotrexate from the National Hospital rheumatology clinic and he started making calls. I was going to the City on Friday after clinic to meet up with Emilio to head to Coban for the marathon. It was serendipitous as I otherwise would have struggled to get the cytotoxic medicine to San Lucas.
I made a list of all the things I wanted to accomplish on Friday when I saw her. My heart was racing all Thursday (I was hyper reading everything I could and planning how I might pull this diagnosis and treatment together), praying that she would come in. Mom had explained to me, tearfully, that first day (after Rafael had asked her to discuss the options with her husband and then I had queried if her husband could read when she told me she couldn’t) that her husband had abandoned her with 5 children and she was really struggling to keep food on the table. There was a big family debt and it was in her name. Her parents and siblings lived far away and her sister-in-law, the only nearby relative, was very mean to her. She’d told me how guilty she felt because she’d considered abandoning her daughter (she told me this in front of her daughter) and asked God to just take her back if she was going to be sick like this because she couldn’t keep up this fight against pain she could never see (and wondered if her daughter was inventing) as she watched her daughter just get sicker and sicker without any progress towards a diagnosis or treatment. She confessed that one day, when her daughter couldn’t get out of bed, she’d just left her there all day and told her she should just die then. Mom was very emotional, clearly exhausted and despairing. I told her she was a good mother for continuing to fight for her daughter and this would be a long fight but we could help her. I knew that it would be quite possible that she would decide not to return. I was also worried because I had told her she could come any time on Friday but, as it turned out, I had to leave clinic at 1pm for my trip to Coban so even if she came, I was worried she’d come in the afternoon and would see Rafael and I just wasn’t confident he would do the hard sell I’d planned for her to make sure she gave this whole evaluation and treatment thing a go.
I arrived Friday morning and there was a long line of patients but I didn’t see her. After rounds, I cornered Rafael and told him I thought he’d need to see her in the afternoon (despite that fact that he had a full schedule of US, as always on Friday afternoons). I tried to catch him up as best I could on what JIA is, its prognosis, Jenny’s advice. I gave him the list of tests and x-rays I wanted and asked for his help in figuring out how we could get the smear reviewed by a pathologist. He said the problem was going to be the cost of all this. I begged him to please do me the favor of letting me pay for this as it was truly a favor for me to know that this girl could start her treatment (as odd as it seems to have to beg to be able to pay for the tests, it’s the reality as the Parrochia doesn’t want gringos creating dependence so all assistance has to go through them and look like it’s coming from them, however they feel bad about me having been here for a year as a volunteer and still accepting my money and would rather turn a patient down for the test or the medication than accept money from me. It puts me in an odd position of actually having to beg that they let me pay for the tests). I walked out for consulta and, oh my gosh, she was there in the line of patients. I had a poker face but I really could have kissed her mother for having come. I took the history again (she was doing quite a bit better with the diclofenac - mom thought it was an appetite stimulant as she was now eating better - it’s a wonder what not feeling pain all over your body can do for your appetite) and clarified what I could, got the rest of the tests and some x-rays, and I started with the first of what I knew would be a long series of educational sessions about juvenile arthritis. And I gave her a pep talk to try to build her up for the coming months of frequent visits and lots of new information (and I took the pictures shown here). I asked her to come back on Monday for a visit with the ophthalmologist for her slit lamp examination to rule out uveitis (it just so happened that a team of ophthalmologic surgeons would be in town for the week -- sometimes things just go your way). I told her we might be able to even start a medication that day as I was going to try to get it from the City over the weekend.
I had to rush out of clinic to catch (actually miss) my bus to the City. I checked email quickly and Jenny had written saying that her friend at the Oncology hospital wanted to see the smear himself. And just as I got on the (later) bus, Emilio called and said the rheumatologists wouldn’t approve the medicine without a prescription from a specialist. I asked him, over roaring bus noises, to call Jenny’s friend Dr. Antillon at the Oncology hospital to see if he could intervene on our behalf but Dr. Antillon said he would only let us buy the medicine after he saw the smear. Yikes! I called the clinic and, between cut off calls and noise so loud I could hardly hear Rafael, I came to learn that the smear had already been sent to a pathology lab in Panajachel. Rafael said something about trying to get the smear back after it had been read but I didn’t catch it before the call was cut off again and we were out of signal range.
I worried all weekend. I thought of Wendy as I ran my half-marathon. I thought about how lucky I was to even be able to run. I couldn’t stop running or that’d be ungrateful of me. I was so upset about not getting the medicine and Wendy’s mom maybe becoming frustrated with all these visits and no methotrexate.
On Monday, there she was again in the clinic line. I was amazed by this great fortune. Cesar, the lab technician, had travelled - himself - to Panajachel on Saturday to bring the smear back as it hadn’t been sent from that lab as we’d expected (I couldn’t believe he did that without my asking -- did Rafe ask him to do it?). Rafael explained that his brother was taking a trip to the City the next morning and it just so happened to be at the adult oncology hospital connected to Dr. Antillon’s Children’s Oncology Hospital. He could take the smear himself and, I hoped, pick up the medication if the smear cleared.
Wendy was doing a little better still. She was walking a few steps at a time and continued to eat better than before. Still, she’d cry when her diclofenac wore off. The ophthalmologists hadn’t shown up, yet. I asked the nurses to let us know when they did. I got to the end of my consultas and, guess what, the ophthalmologists had come and gone. I was so disappointed as the mom had paid to come in to clinic and had waited all morning. Pablo Benedicto, the clinic director, volunteered to go to the house in town where the ophthalmologists were staying and have them return. They graciously did and were more than happy to examine Wendy. She had a normal eye exam and I told mom I hoped to get the firm diagnosis and methotrexate for her ASAP but would call her with the next appointment.
I wrote Dr. Antillon a nice letter of thanks for his help and pleaded my case for him to let Rafael’s brother bring MTX back with him. The smear was negative and he called me personally with the results. And he let me buy the MTX. Everything still falling into place.
I woke up at 3am this morning to read a 50-page text on MTX. I’d been calling the mom to bring Wendy in since yesterday but the phone was off. I tried 4 or 5 more times today. No luck. So, that’s our first wrinkle. I really, really wanted to start today so I could get 3 injections in while I was here but I hope I can get in touch with her over the weekend and have her come in on Monday.
Wendy came to see me for the first time not even two weeks ago. Despite the fact that she was 4-years-old, her mom carried her into the room strapped to her back and she was crying. Wendy and her mom arrived just before the lunch break, typically when the last couple of patients who don’t have numbers (appointments) squeeze through the door for a quick little visit (of course, just like back home, it seems these “little” visits often are quite the opposite). Wendy looked frail and sick. She sat on the exam table stiff and seemed to be bracing against the possibility that I might touch her anywhere. I started to get her story from mom, which only served to confuse me.
Wendy had been sick for 6 months. The list of problems she’d had over that time seemed something of a random list of symptoms inconsistent with any type of disease. She had had swellings over her forehead that then passed to her neck and then her arm and then her legs and now her feet. The swellings would form suddenly, last for minutes, then resolve. They hurt but weren’t red. They were 2cm or so. They would develop 4 or 5 times a day but they had resolved over the prior week, ever since Wendy got an injection from a curandero (Mayan medicine man). Mom brought the vial (first time I’ve seen that happen this year!): lincomicina. I’d never heard of this medicine (later found out it is essentially clindamycin) but it’s hard for me to imagine that any injection was enough to stop these swellings that had been coming daily for 6 months. I started to wonder if I could get a reliable history from this mom. She recounted that Wendy had complained of pain in various parts of her body off and on over the 6 months but there were rarely changes that she could see or sometimes there was just swelling. I started to question the history even more when she said she had taken Wendy to a doctor in town one morning after Wendy had woken up unable to get out of bed, but when they got to the doctor’s office she was fine, pain-free, and he didn’t see anything wrong. This was three months into her illness. She said that over the 6 months, there'd been fevers most days. Also, Wendy could no longer walk and there were days when she wouldn’t get out of bed because of her pain and she hasn't had an appetite and has lost weight. At this point, I was starting to take this much more seriously. Mom reported that now it was her neck that was hurting and she can’t turn her head to the right. I asked if Wendy had seen any other doctors over this time. She’d seen one just a month ago -- had travelled all the way to Panjachel to see a pediatrician. The doctor had done some tests (and mom brought the papers!!!!). She’d diagnosed a UTI on UA (lots of whites but neg nitrites, 10 RBCs) and Ascaris from a stool sample. The blood count showed 13.6K WBCs with 81%N, 13%L. Plts were 426K and there was a mild microcytic anemia (Hct 30.6). She treated the UTI and worms appropriately but apparently didn’t have an explanation for the chronicity or severity of the problem and didn't treat the anemia.
I took a deep breath and moved on to the exam thinking this was probably going to be some form of tropical disease that I wouldn’t recognize, much less know how to evaluate. As I mentioned before, the girl was scared and trembling but seemed to be trying to let me examine her. Her right neck hurt (maybe along the SCM or deeper?) and she couldn’t turn it but there was nothing to see. Just shoddy cervical lymphadenopathy. HEENT was normal. Heart, lungs, abd were normal. When I had her sit up, she was very tentative and guarded. She wimpered. Her knees looked knobby, maybe big but not red. She was so thin, I wondered if that accentuated the large appearance of her knees. Her ankles looked a little swollen and they hurt but they weren’t red either. Her fingers even looked a little swollen but they weren’t red. She stood, but tentatively. I had that “this is bad” feeling but for some reason, it didn’t hit me right away. I called Rafael in for his opinion and as we were waiting for him, I asked mom if she thought it might have been Wendy’s joints that hurt her. Mom didn’t know what joints were but I pointed out those particular body sites and, yes, those were what seemed to hurt. It’s odd to me that it took me even that long to figure out what had been hurting but, with that, it all started to come together: polyarticular arthritis. I summarized the story for Rafael and it seemed to suddenly fit. What hadn’t seemed to make any sense before now fit together. He agreed. Now what? It was clear mom was very poor, she was from a small, rather far away hamlet, and we were dealing with something serious.
I suggested that we send her to a rheumatologist: maybe a big bill up front but after the diagnosis, we could manage the condition with the advice of the rheumatologist. Rafael said things didn’t work that way here. We’d need to make the diagnosis first and then hand her over to the rheumatologist for management. He suggested an ESR and RF. I said I thought we already had the diagnosis, clinically, (though and ESR would be good) but really she would need a rheumatologist. I was feeling this was way out of my league. Rafael explained the options to the mom: spend lots of money and time and go to the City to see the rheumatologist or stick with us and we’ll do the best we can (which, he said, would be good, it just might take us more time). I didn’t have that same faith and thought, “please say you will see the specialist” but of course she said she’d trust us to do the best we could and, with that, Rafael stepped out.
I sent her for an ESR and RF, gave her as many diclofenac samples as she could fit in her bag as well as some iron and asked her to come back on my next clinic day four days later. Her ESR was 127, RF negative. The lab tech noted a lot of white cells so did an impromptu count and found 31K. I went home and crammed as much information as I could about JIA (and ALL). I went to the local pharmacies to see what medications from the long list of treatment options might actually be available, if any. They didn’t have any. I wrote Jenny Frankovich, an amazing newly graduated rheumatologist at Stanford who was a resident with me. She wrote me back immediately and was incredibly helpful. She converted all that “gold standard, rich country, unlimited resources” information I’d gotten on UpToDate into a realistic list of things that would truly help us make a reliable diagnosis and begin treatment. She said Wendy had a good chance of responding to the treatment and, perhaps, the response would be marked. She even wrote her Guatemalan friend, who is head of the Children’s Oncology Hospital in Guatemala City, to solicit his help in ruling out leukemia. I was super-charged.
I called my friend, Emilio, in the City and asked him to help me get methotrexate from the National Hospital rheumatology clinic and he started making calls. I was going to the City on Friday after clinic to meet up with Emilio to head to Coban for the marathon. It was serendipitous as I otherwise would have struggled to get the cytotoxic medicine to San Lucas.
I made a list of all the things I wanted to accomplish on Friday when I saw her. My heart was racing all Thursday (I was hyper reading everything I could and planning how I might pull this diagnosis and treatment together), praying that she would come in. Mom had explained to me, tearfully, that first day (after Rafael had asked her to discuss the options with her husband and then I had queried if her husband could read when she told me she couldn’t) that her husband had abandoned her with 5 children and she was really struggling to keep food on the table. There was a big family debt and it was in her name. Her parents and siblings lived far away and her sister-in-law, the only nearby relative, was very mean to her. She’d told me how guilty she felt because she’d considered abandoning her daughter (she told me this in front of her daughter) and asked God to just take her back if she was going to be sick like this because she couldn’t keep up this fight against pain she could never see (and wondered if her daughter was inventing) as she watched her daughter just get sicker and sicker without any progress towards a diagnosis or treatment. She confessed that one day, when her daughter couldn’t get out of bed, she’d just left her there all day and told her she should just die then. Mom was very emotional, clearly exhausted and despairing. I told her she was a good mother for continuing to fight for her daughter and this would be a long fight but we could help her. I knew that it would be quite possible that she would decide not to return. I was also worried because I had told her she could come any time on Friday but, as it turned out, I had to leave clinic at 1pm for my trip to Coban so even if she came, I was worried she’d come in the afternoon and would see Rafael and I just wasn’t confident he would do the hard sell I’d planned for her to make sure she gave this whole evaluation and treatment thing a go.
I arrived Friday morning and there was a long line of patients but I didn’t see her. After rounds, I cornered Rafael and told him I thought he’d need to see her in the afternoon (despite that fact that he had a full schedule of US, as always on Friday afternoons). I tried to catch him up as best I could on what JIA is, its prognosis, Jenny’s advice. I gave him the list of tests and x-rays I wanted and asked for his help in figuring out how we could get the smear reviewed by a pathologist. He said the problem was going to be the cost of all this. I begged him to please do me the favor of letting me pay for this as it was truly a favor for me to know that this girl could start her treatment (as odd as it seems to have to beg to be able to pay for the tests, it’s the reality as the Parrochia doesn’t want gringos creating dependence so all assistance has to go through them and look like it’s coming from them, however they feel bad about me having been here for a year as a volunteer and still accepting my money and would rather turn a patient down for the test or the medication than accept money from me. It puts me in an odd position of actually having to beg that they let me pay for the tests). I walked out for consulta and, oh my gosh, she was there in the line of patients. I had a poker face but I really could have kissed her mother for having come. I took the history again (she was doing quite a bit better with the diclofenac - mom thought it was an appetite stimulant as she was now eating better - it’s a wonder what not feeling pain all over your body can do for your appetite) and clarified what I could, got the rest of the tests and some x-rays, and I started with the first of what I knew would be a long series of educational sessions about juvenile arthritis. And I gave her a pep talk to try to build her up for the coming months of frequent visits and lots of new information (and I took the pictures shown here). I asked her to come back on Monday for a visit with the ophthalmologist for her slit lamp examination to rule out uveitis (it just so happened that a team of ophthalmologic surgeons would be in town for the week -- sometimes things just go your way). I told her we might be able to even start a medication that day as I was going to try to get it from the City over the weekend.
I had to rush out of clinic to catch (actually miss) my bus to the City. I checked email quickly and Jenny had written saying that her friend at the Oncology hospital wanted to see the smear himself. And just as I got on the (later) bus, Emilio called and said the rheumatologists wouldn’t approve the medicine without a prescription from a specialist. I asked him, over roaring bus noises, to call Jenny’s friend Dr. Antillon at the Oncology hospital to see if he could intervene on our behalf but Dr. Antillon said he would only let us buy the medicine after he saw the smear. Yikes! I called the clinic and, between cut off calls and noise so loud I could hardly hear Rafael, I came to learn that the smear had already been sent to a pathology lab in Panajachel. Rafael said something about trying to get the smear back after it had been read but I didn’t catch it before the call was cut off again and we were out of signal range.
I worried all weekend. I thought of Wendy as I ran my half-marathon. I thought about how lucky I was to even be able to run. I couldn’t stop running or that’d be ungrateful of me. I was so upset about not getting the medicine and Wendy’s mom maybe becoming frustrated with all these visits and no methotrexate.
On Monday, there she was again in the clinic line. I was amazed by this great fortune. Cesar, the lab technician, had travelled - himself - to Panajachel on Saturday to bring the smear back as it hadn’t been sent from that lab as we’d expected (I couldn’t believe he did that without my asking -- did Rafe ask him to do it?). Rafael explained that his brother was taking a trip to the City the next morning and it just so happened to be at the adult oncology hospital connected to Dr. Antillon’s Children’s Oncology Hospital. He could take the smear himself and, I hoped, pick up the medication if the smear cleared.
Wendy was doing a little better still. She was walking a few steps at a time and continued to eat better than before. Still, she’d cry when her diclofenac wore off. The ophthalmologists hadn’t shown up, yet. I asked the nurses to let us know when they did. I got to the end of my consultas and, guess what, the ophthalmologists had come and gone. I was so disappointed as the mom had paid to come in to clinic and had waited all morning. Pablo Benedicto, the clinic director, volunteered to go to the house in town where the ophthalmologists were staying and have them return. They graciously did and were more than happy to examine Wendy. She had a normal eye exam and I told mom I hoped to get the firm diagnosis and methotrexate for her ASAP but would call her with the next appointment.
I wrote Dr. Antillon a nice letter of thanks for his help and pleaded my case for him to let Rafael’s brother bring MTX back with him. The smear was negative and he called me personally with the results. And he let me buy the MTX. Everything still falling into place.
I woke up at 3am this morning to read a 50-page text on MTX. I’d been calling the mom to bring Wendy in since yesterday but the phone was off. I tried 4 or 5 more times today. No luck. So, that’s our first wrinkle. I really, really wanted to start today so I could get 3 injections in while I was here but I hope I can get in touch with her over the weekend and have her come in on Monday.
I am so thrilled with the possibility of being able to help Wendy. She is a precious, sweet child who may have gone undiagnosed and likely untreated (at least not treated with more than NSAIDS) until she was left chronically severely disabled and in unrelenting pain. I don’t really believe that God changes the course of events day-to-day but I have to say that it has felt like God has pulled out all stops for this to come together and I honestly feel tearful when I think of how significant this is. Now, I just don’t know how I’m going to walk away from this case. How can I leave and trust that she will continue to get her medicine and her monitoring? What’s the chance that when I come back to visit in 5 years, I’ll find her as a happy, active 9-year-old? In the next 3 weeks, I have to come to terms with all of this.
1 comment:
am proud of you kate! great work!
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