Nicholas, age 3, so far so good 8 weeks after

Personal stories of individuals and families affected by Kawasaki disease
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Nicholas, age 3, so far so good 8 weeks after

Post by lothar » Sun Aug 07, 2011 12:00 am

Our story:
Sat: our 3 year old comes down with a fever and stomach ache, won't eat. We phone the advice nurse at our ped office and she diagnoses stomach virus over the phone, says he doesn't need to be seen, just kept hydrated.

Sun: Still feverish, he grows a single, gigantic lymph node in his neck. Convinced it's an ear infection in the ear just above the node, I take him to an urgent care clinic. The MD there diagnoses an ear infection and perscribes pennicilin, but also tells me that he can't see anything in the ear because there's too much wax. We start him on it, and it doesn't have any effect.

Mon: Fever still coming and going, after his 3rd dose of the pennicilin, I notice that his lips are swollen. I take him to the ped office thinking that he's developing an allergy to pennicilin and I'd need to get an Rx for a different antibiotic. The nurse practitioner spends a lot of time examining him and his tongue, and calls in an MD who does the same. They leave for a long time, then she comes back and says that the strawberry tongue, swollen lips, red eye and fever are consistent with KD. She gives me an Rx for a different antibiotic and tells me to call back if his fever is still there the next day.

Tues: Fever still there on and off, lymph node still there, both eyes red now. I take him in to Children's hospital and we sit in the ER for hours while they take blood and do an ECG (but they don't do an echo.) His CRP is 5 and sed rate over 100 (if I recall correctly). Rash is coming and going, but nothing really bad. They admit him to the ward at 9 PM and don't want to start IVIG until they rule out adenovirus and Epstein-Barr. By this point, I have a copy of the guidelines for diagnoses and treatment published by the American Heart Assn, and I push the resident really hard to initiate IVIG then based on the symptoms Nicholas already has (he has all the symptoms besides coronary abnormalities). She tells me that there's a 'safe period' until day 10, before when no damage begins, that the IVIG treatment itself has risks and so they have the time to get lab tests for the differential diagnosis. The attending physician does not come in to examine him. Nicholas is suffering tremendously -- not responsive, not eating and irritable.

I was and remain suspicious about the justification for this delay, and the deviation from the published guidelines.

Weds: The attending finally shows up in the morning, and he and I go over the guidelines together. He defends the decision to delay the IVIG with anecdotes from his prior practice, but no evidence of superior outcomes. (I wish he had come in armed with solid, evidence-based arguments for this departure from the guidelines, such as "This morning I reviewed all of the adenovirus cases we've had here over the past 20 years, and 50% of the time there is no discharge from the eye and there is one large lymph node, just like your son, so this test to rule out adeno is really needed even with this set of symptoms." but there was nothing of that level of rigor.) I question the idea of the 'safe period' and ask him about the study that idea was based on (as far as I can tell, a study done in China on patients who already had coronary problems on day 4) -- he doesn't answer.

I'm deeply disappointed with his shallow command of the KD literature, so I make some calls to see about getting Nicholas transferred to another ped hospital. By the time I make any progress, they have the lab results back (negative, obviously) and are ready to start the IVIG, so we stay where we were. There is a further delay of the IVIG while they finally do the echo. I have to leave to take care of the other kids, so my wife is there when they finally start the IVIG, and 30 minutes later Nicholas is awake, eating, making jokes at her; 2 hours later the lymph node is down and his eyes aren't red. The pediatric cardiologist is a wonderful contrast to that attending physician who can't be bothered to actually read KD papers or conform to the AHA guidelines: the cardiologist has read everything and I mean everything. He answers all of our questions, fills us in on the studies which have been shown to improve outcomes and which issues Nicholas faced which have not been studied. I wish this guy had been in charge of administering Nicholas' care all along.

At some point in here, the nurse practitioner who first suspected KD (on day 3!) phones to ask about Nicholas. I burst into tears while thanking her for seeing it so soon.

Thurs: they discharge him the next day, we have a 'welcome home' ice cream party. He is not quite himself, still. I sleep with him in his bed that night, and at 3 AM I wake up because he's so hot. His fever is 103. We phone the attending, who tells us to bring him back in. Terrified, I drive him back in; he's awake and really excited to be driving at night. He falls asleep, and won't eat or drink this time. The second round of IVIG works its magic as the first did, and he perks right up again. This presents a problem we're delighted to have: he's awake, alert, bored and stuck to his IV pole. We entertain him as best we can in his bed, and he is such an angel.

Friday: they want to keep him overnight again after the 2nd round of IVIG, so they discharge him for the last time on day 7.

His 2 week echo is clean, thank god. I know how fortunate we are, especially after reading this forum. However, I still worry about how this might affect him when he's 35 or 55. And I realize that we're not totally out of the woods yet: he has his 8 week echo this week. Fingers crossed.

Just this past week, beginning of week 7, he caught a cold (cough, runny nose, mild fever for 2 days) and he showed some KD-type signs: that one lymph node was the only one that got enlarged during the cold, and his eyes got a little red again. I was getting paranoid that he'd have a recurrence, but after reading this forum it appears that viruses bring back some KD type symptoms commonly.

I have been reading and reading and reading, trying to get some hint of the long term questions, without much luck. I know that he'll have some endothelial dysfunction, but what does that really translate into? Is the degree of endothelial dysfunction you get from a 'mild' case of KD more like that you get from smoking for 2 years, 10 years or 50 years? Why do the guidelines call for an echo of the coronary arteries, and no imaging of the other arteries (especially the ones in the brain?)

Anyway, he is himself, and he is happy. He still talks about the hospital, and about the IV line he got -- the most traumatic part for him was being stuck with a needle. I am trying to make sense of it, and my own reactions to it. This is a serious disease, but with treatment, according to the literature, fewer than 1% of patients suffer lasting consequences. For some reason, that number doesn't comfort me, and it sure as hell didn't comfort me at the time. We are lucky, luckier than the kids with cancer I'd see toting their IV poles around the cafeteria when we were in the hospital, and luckier than many of the people who post here. I am grateful for that, but I still get that sinking dread in the pit of my stomach whenever he gets a fever.

I realize that this is part of parenting, and that one has to take the bad with the good, and that this is not an original or interesting thing to say, but boy oh boy does this suck. This is really hard, and there's just nothing good about it, like getting hit by a car. As a result of Nicholas' having KD, other parents who have lost a child or whose children have to deal with vasculitis, heart defects and cystic fibrosis have starting discussing their kids' conditions and their reactions with me. This is our first serious "close call" with our kids, and the first time any of them had to be in the hospital. I guess you just adapt to the vulnerability eventually, because you have to.

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Re: Nicholas, age 3, so far so good 8 weeks after

Post by toffeegirl » Sun Aug 07, 2011 3:07 pm

I self diagnosed my daughter in the hospital she had been sent to on Day 3 of her illness. At that point they were still treating her with IV antibiotics for a severely infected lymph node despite the emergence of other symptoms consistent with KD. With hindsight I can see that they were concerned that she may have Toxic Shock Syndrome...hence the quick infusion of antibiotics whilst they were awaiting her blood test results. I had the same conversation re: the delayed treatment with IVIG as they did not treat my daughter until around 6pm of day 5 despite her having in excess of 8 symptoms consistent with KD. The consultant I spoke to explained that the attendant risks associated with the treatment were such that they were reluctant to treat a 3 yr old child without full confidence that it was the right diagnosis. When pressed they elaborated that as Immunoglobulin is a blood product there is always a possibility that there may be problems in the future that they could not have known about now, that it can have nasty side effects and also the risks associated with high dose aspirin.

They were very confident that the 10 day window was a comfortable one. As it stands I have since read a study that suggests that treatment in 5-7 days actually increases the chances of requiring a 2nd round of IVIG although it doesn't appear to significantly reduce the chance of coronary complications compared to treatment at day 10. As it turned out my daughter did indeed have 2 rounds of IVIG, the first of which she had a partial response to and the second had no effect on the KD but did give her Aseptic Meningitis! Having had to watch her then suffer the trauma of that whilst still suffering significantly from the KD I can fully understand their desire to be as sure as they possibly can be that she did require the treatment. As traumatic and painful as it is as a parent to watch your child suffer I do feel the Dr's are caught between a rock and a hard place with this. There are no diagnostic tests, it is just a collection of symptoms over a time period and the treatment is not a risk free cure.

I guess the difference with our experience is that I actually had 3 Consultants on board and so felt we were being given the time of well qualified staff. As it turned out my daughter spent 21 consecutive days in hospital all told and we are due back in tomorrow. She was referred to a specialist Children's Hospital after failing to respond to the second dose of IVIG. Well, actually I threw my toys out of the pram when they started talking about a blood transfusion for her anaemia whilst she was still clearly not over the KD. Having had the lecture re: unwarranted use of blood product I found it some what hard to stomach the idea that they were prepared to then give her a blood transfusion to try and treat what is a clearly documented symptom of KD without making any further attempt to deal with the underlying cause.

Within an hour of being at the Children's Hospital she was on IV steroids and the difference was incredible. The reaction that most kids have to IVIG that we had not witnessed finally appeared and within 24 hours she was a different kid. I don't know if it is different here in the UK but as well as heart ultrasounds she has also had every other major organ checked plus chest x-rays. I don't know if that is because it took her such a long time to finally shake the illness or whether it is standard practice here but I certainly feel a lot happier knowing that it is not just her heart they looked at. All is clear so far and now it is just a fingers crossed waiting game I guess.

Like your little boy, her main gripe is the needles and if that is the worst that comes out of this then I will be one happy Mummy. I have come to the same conclusion as you...that we are lucky. Luckier so far than many of the guys on here, luckier than the kids in the Oncology unit I walked past every day and it is difficult, having watched your child suffer to remember that. I too am struggling to find any comfort in statistics or the phrases used by professionals when they say certain outcomes are "very unlikely" or "in the vast majority". After all, it was "very unlikely" that Emily would get this at all as "the vast majority" do not! Like I keep saying even with a 0.01% chance some unlucky sod has to be the 0.01 and just getting KD in the first place is pretty incredible odds. Seeing Emily now in the 20% of KD sufferers that do not respond to IVIG , I am watching that window get smaller and smaller and it scares me. To have had 2 heart scans come back clear after all of this makes me breathe a huge sigh of relief on the one hand but fills me with a massive dread on the other as I wait for what I feel must be the inevitable damage as a result of her lack of response. I still cannot believe she can be that lucky. Our next ultrasound is in 3 weeks and I think if we get through that one we should be in the clear as far as you ever can be with this illness.

Sorry for hijacking your thread! Didn't realise quite how much I had to say :shock: Once I started, I couldn't stop!

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Re: Nicholas, age 3, so far so good 8 weeks after

Post by lothar » Fri Aug 12, 2011 10:15 pm

Disclaimer: this is all just 'as I ready the literature', I might not have it down correctly, and I'm not a medic.

The IVIG seems pretty darn safe these days. If there's no IgA, the chance of a good outcome with no anaphalyxis is increased. The stuff they used on Nicholas was 'affinity column purified' and is IgA free, essentially, and the chances that there would be a prion or virus in that is vastly lower than with whole blood. You may not have a preparation of IVIG this pure in the UK, not sure (not every manufacturer uses affinity column purification.)

The study showing that IVIG before day 5 didn't improve outcomes was done on 'typical' KD patients; that is, those kids all had an abnormal ECG or echo upon diagnosis. Your kid and mine never had an abnormal finding on any cardiac study, so ours were 'atypical KD patients.' The results of the study you refer to might not apply to our kids, because the kids in that study had murmurs, gallops or ballooned out coronaries when they walked into the clinic. This 'safe window' idea is a dangerous one, IMO.

Also, my son was suffering, he couldn't open his eyes because they were so red, etc -- significant morbidity.

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