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5 year old female - headache and vomiting

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You can't have any labs, you don't do those in an ambucab. You have to transport her somewhere that you can do the labs at. But if you ask me nicely :punk: I'll probably give you some. but you have to say you are going to bring your ultrasound machine to me too.

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Well since no-one else is playing here's what happens - my scenario, my rules!!!!!

You get word that your helicopter is acting funny so it's out. the fixed wing is on a transport and won't be at the local airport for 4 hours so the local doc int he box hospital is what you got.

You transport her by ground to that hospital.

You get there and this is what they find:

The patient appears pale and icteric. She displays stupor alternating with irritability. Her heart rate is 136 beats/min, blood pressure is 103/62 mm Hg, temperature is 98.06°F, and respiratory rate is 16 breaths/min.

Upon heart examination, regular heart sounds are noted (S1, S2). A grade 2/6 systolic murmur is heard all over the precordium.

Figure 1.

Figure 2.

Figure 3.

The abdomen is soft and nontender, and bowel sounds are positive. The spleen is palpated 3 cm below costal margin.

The patient's cranial nerves are intact. She has normal power and sensation over the right side (upper and lower limbs). She has left-sided hemiparesis and hemianesthesia (upper and lower limbs).

Laboratory findings are as follows:

  • Hemoglobin level: 6.5 g/dL

  • White blood cell count: 24,000 cells/mL

  • Platelet count: 500,000 cells/mL

  • Prothrombin time/partial thromboplastin time: normal

  • Routine chemistry: normal

The diagnosis is what??????

Sickle Cell Anemia with Cerebrovascular Accident. Here's the rest of the article that I based the scenario on:

The patient was diagnosed with sickle cell anemia complicated by a right-sided cerebral stroke. Consequently, she underwent acute exchange transfusion and was later moved to lifelong transfusion therapy to prevent stroke recurrence. She was also given deferasirox (Exjade®) (30 mg/kg/d) orally once daily to prevent transfusion iron overload and its complications.

Stroke is one of the leading causes of morbidity and mortality among children with sickle cell anemia. Stroke can affect as many as 30% of children with this condition and can manifest clinically in 11% of all patients with sickle cell anemia.[1] Stroke also manifests as silent cerebral infarction on MRI in another 17%-22% of patients.[2] The risk for overt stroke for children with sickle cell anemia without primary stroke prophylaxis is more than 200-fold higher than in the general population; this risk may be even higher in adults.[3]

Silent cerebral infarct can occur as early as the first year of life, and its prevalence increases with age. Approximately 40% of adolescents with sickle cell anemia have silent cerebral infarcts, and adults are likely continue to acquire new or enlarged silent cerebral infarcts.[4] Cerebral infarcts tend to be ischemic in children and hemorrhagic in adult patients.

Well-documented risk factors for ischemic stroke in sickle cell anemia include prior transient ischemic attack, low steady-state hemoglobin levels, high white blood cell counts, hypertension, and a history of acute chest syndrome.[1] However, the etiology of stroke in sickle cell anemia remains unclear. Aside from the absence of lipid deposition and plaque formation, many of the histopathologic findings in cerebrovascular lesions in sickle cell anemia resemble those found in stroke patients in the general population.[5]

Cerebral infarcts in patients with sickle cell anemia are best detected by transcranial Doppler (TCD) ultrasonography, MRI, and magnetic resonance angiography (MRA). TCD ultrasonography is a reproducible and noninvasive method that aims to find narrowed internal carotid or middle cerebral arteries in asymptomatic children by detecting a signature high-flow pattern. Children with elevated blood flow velocities (≥ 200 cm/sec) have an astonishingly high rate of stroke: approximately 10,000 per 100,000 patient-years. TCD can reveal areas of high flow across the internal carotid artery and middle cerebral arteries that correspond to areas of stenosis; these are risk factors for stroke. Patients with abnormal MRA findings and higher blood-flow velocity on TCD ultrasonography are at an even higher risk for stroke.[6]

Cerebral infarcts in patients with sickle cell anemia should be treated by exchange transfusion on diagnosis, with lifelong transfusion therapy. Exchange transfusion is an effective but possibly underutilized therapy in the acute and chronic treatment of sickle cell anemia. The aim is to maintain or reduce the hemoglobin S level to below 30%. It can provide needed oxygen-carrying capacity while reducing the overall viscosity of blood and thus reducing complications. It is mainly indicated in acute infarcted stroke, acute chest syndrome, multiorgan failure syndrome, the right upper quadrant syndrome, and possibly priapism.

As well as this good tidbit of info:

The Stroke Prevention in Sickle Cell Anemia (STOP 1) trial was performed in 1998 with a goal of assessing the effect of transfusion therapy in preventing the first stroke episode in patients with sickle cell anemia.[7] In this study, 130 children with sickle cell anemia, high risk for stroke (two measures on TCD ultrasonography), and comparable baseline characteristics were enrolled. Sixty-three children were randomly assigned to the intervention arm (receipt of transfusions), and 67 children were randomly assigned to the control arm (no receipt of transfusions).

The study was terminated early because it demonstrated such a large benefit of transfusion. A clinical alert was issued by the National Heart, Lung, and Blood Institute. The alert stated that screening and prophylactic transfusion must be performed in children with sickle cell anemia aged 2-16 years who are at high risk for stroke and have not yet had a stroke. The conclusions of the STOP 1 trial were clear: Long-term transfusions significantly reduce the risk for stroke in children with sickle cell anemia who have risk factors on TCD ultrasonography.

After the remarkable results of the STOP 1 trial, new questions were addressed, including when to start and stop blood transfusions in high-risk patients. The STOP 2 trial sought to determine the effect of discontinuing transfusions in patients with sickle cell anemia who were once at high risk for a stroke, had been receiving transfusions for more than 30 months, and currently had normal blood-flow velocities on TCD ultrasonography.[8] This study was stopped 2 years after initiation, when 79 children of a planned enrollment of 100 had undergone randomization.

Among the 41 children in whom transfusions were stopped, high-risk results on TCD ultrasonography developed in 14 and stroke developed in 2 others within an average of 4.5 months of the last transfusion. In contrast, no events of the composite endpoint occurred in the 38 children who continued to receive transfusions. The study team concluded that discontinuation of transfusion for the prevention of stroke in children with sickle cell anemia results in a high rate of reversion to abnormal blood-flow velocities on Doppler studies and stroke. Thus, stopping transfusions in patients who are at high risk of stroke is unsafe.

Moreover, an increase in serum ferritin was found, suggesting the development of iron overload in patients who are being treated with lifelong transfusions. Alloimmunization was seen, but no risk for infection was detected.

Owing to the conclusive results of the STOP 1 and STOP 2 trials, all patients with sickle cell anemia with abnormal blood-flow velocities on TCD ultrasonography were placed on lifelong transfusion therapy. Because the frequent transfusions cause iron to progressively accumulate in these patients, they began developing complications from iron overload.

And also this bit of info but this is really for the doc's amongst us as the article is from Medscape. But good info if you think you are a paraGAWD

All children with homozygous sickle cell anemia or sickle beta zero thalassemia who are younger than 16 years should undergo annual screening with TCD ultrasonography. Values greater than 170 cm/sec are worrisome, and patients with a value higher than 200 cm/sec should be started on indefinite transfusion therapy.

Iron overload can be measured using several techniques. Biopsy is the criterion standard for measurement of liver iron concentration, but this technique is invasive. Serial measurement of serum ferritin levels is practical but has questionable accuracy. MRI is a noninvasive and reliable technique for measurement of liver iron concentration. On the basis of the degree of iron overload, these patients should be treated with iron chelation therapy.

Three chelators are available: deferoxamine (parenteral), deferiprone (oral), and deferasirox (oral).[9]Most knowledge on the efficacy and safety of these chelators in reducing iron overload comes from studies in patients with thalassemia who had iron overload and in patients with sickle cell disease. Currently, most evidence supports the use of deferasirox in treating iron overload because of its safe profile, easy means of administration, good rate of patient adherence, and efficacy in decreasing excess iron in the body.[10]

Link for the entire case is found here: http://reference.medscape.com/viewarticle/844659

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I thought it was sickle cell with some cerebral involvement, I've just been a tad busy :)

Edited by scubanurse

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I thought it was sickle cell with some cerebral involvement, I've just been a tad busy :)

having a baby doesn't count for busyness factor

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Interesting case and not that uncommon.

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I love MedScape - they often have cases that can be broken down to EMS level scenarios. This was one of them.

I believe you can also get CEU's from them as well.

Edited by Ruffmeister Paramedic

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