Presented by Richard Gordon, MD
“My chest hurts”
30 year old AAM presents to ED with 2 day history of worsening sharp chest pain located over the anterior/lateral right ribs. Patient states the pain is 8/10 in intensity, non-radiating, and exacerbated with deep breathing/coughing. No obvious alleviating factors. Patient has never experienced this kind of pain in the past. Patient also complains of mild shortness of breath. The patient states that he has had a dry cough for the last 4 days without any measured fever, but has felt warm. No recent travel, no history of DVT in the patient/family, and no history of hypercoagulability. No nausea, vomiting, or abdominal pain. Review of CIS shows the patient was in the ED 7 days ago for “sickle pain” and was treated with 2 rounds of morphine sulfate 10 mg IVP, Benadryl 50 mg IVP, and one liter bolus of normal saline, and subsequently discharged home with pain under control.
PMH: Sickle cell SS, Asthma
PSH: No previous surgery
FH: Mother died at 65 MI. Father died at 62 CVA. Brother alive 37 HTN, asthma, sickle cell trait. Sister alive 35 sickle cell anemia SS, asthma.
SH: Denies smoking, drinking, or use of illicit drugs.
Meds: Albuterol inhaler 2 puffs PRN for SOB. Advair Diskus 250/50 inhaled BID. Morphine sulfate controlled release 100 mg BID. Morphine sulfate instant release 30 mg 1-2 tab Q3H PRN for pain.
Vitals: BP:140/90 HR:115 RR:25 T:39.0 Sat:89% room air
General: Alert and oriented X3. Upright in stretcher answering questions appropriately. However, anxious appearing and looks uncomfortable breathing with accessory muscles, speaking in partial sentences.
HEENT: Head is normocephalic and atraumatic. Pupils equal and reactive to light. Extraocular movements intact. There is scleral icterus. Nares have no discharge. Oropharynx has moist mucus membranes, no exudates, no erythema. Neck is supple with no JVD, trachea midline, no adenopathy.
Chest Wall: Reproducible chest pain with palpation of ribs bilateral left greater than right. Reproducible parasternal tenderness. No crepitus.
Cardiac: S1S2 regular and tachycardic. No murmus, rubs or gallops.
Lungs: Good air entry bilateral, end expiratory wheeze, prolonged expiratory phase. Basilar rales present. No ronchi.
Abdomen: Bowel sounds present, nontender to palpation, nondistended, no masses, no organomegaly.
Extremities: Moves all four with full Range of motion. No asymmetric swelling or erythema. 2+ distal pulses. Calves nontender to palpation.
Skin: No jaundice. No rashes. No diaphoresis
Neuro: Alert and oriented X3. Pupils equal and reactive to light. External ocular muscles intact. No facial asymmetry. 5/5 strength all 4 extremities. Walks with normal gait. No obvious sensory deficits.
ABG on room air: 7.45/59/30/16/89%
HbG: 8.5 Baseline HbG 9.5
Lytes: Na-140 K-5 Cl-100 HCO3-15 BUN-18 Cre-0.8
12 lead ECG shows sinus tachycardia at rate of 110.
Chest PA shows middle lobe infiltrate.
Course in ED
Patient immediately placed on non-rebreather with saturation rapidly increasing to 100%. 3 doses of albuterol and atrovent via nebulizer were administered with improvement of tachypnea and wheezing. Patient now maintiaing 94-96% 4L NC breathing more comfortably. IV established with 1 liter normal saline bolus followed by 1.5 maintenance. Blood cultures X2 sent, followed by, IV doxycycline and ceftriaxone administration. Morphine 10mg with Benadryl 50mg and titrated to pain control. Patient was type and crossed followed by transfusion with 2 units of blood. Saturation again improved 96-98% on 2 liters. Patient admitted to hematology oncology service.
1. Which of the following is NOT considered to be one of the clinical diagnostic criteria for acute chest syndrome in a patient with sickle cell disease?
a) chest pain and hypoxia
b) new pulmonary infiltrate(s) on chest x-ray
c) recent sickle cell pain crisis
d) tachypnea, wheezing, or cough
e) temperature > 38.5
There is no current laboratory or radiographic finding to provide definitive diagnosis of acute chest syndrome. The diagnosis is made clinically with one or more of the following: chest pain, temperature greater than 38.5, tachypnea, wheezing, cough, appearance of increased work of breathing, or hypoxemia. AND new pulmonary infiltrate found on chest radiograph involving at least one complete lung segment that is not consistent with the appearance of atelectasis.
C) Though, it is common for a sickle pain crisis to have occurred prior (usually about 2 days) to onset of acute chest syndrome, it is not currently one of the accepted diagnostic criteria.
2. Which of the following statements related to acute chest syndrome in sickle cell anemia is correct?
a) bronchodilators have no role in the acute treatment.
b) most common chest x-ray finding is new infiltrate in bilateral upper lobes.
c) only exchange transfusion provides benefit.
d) pulmonary infection is rarely the inciting cause.
e) transfusion of packed red blood cells can significantly improve the patient’s condition.
A) Studies suggest that patients with sickle cell disease have increased airway reactivity. In addition to this, increased airway reactivity further compounds the problem of sickle cell anemia secondary to hypoxia. With this in mind, it is recommended inhaled beta agonist therapy be administered in all patients whom may have acute chest syndrome, even those with no wheezing. B) The most common x-ray finding in acute chest syndrome is bilateral lower lobe infiltrates. D) In a large multicenter study it was found through bronchoalveolar lavage the second most common inciting event of acute chest syndrome is infection. Most commonly Chlamydia pneumoniae followed by Mycoplasma. C) Exchange transfusion can be reserved for those with a PaO2 less than 70 and refractory to supplemental oxygen. E) Simple transfusion does provide a risk of increasing the blood viscosity and further exacerbating the vaso occlusive event (particularly to levels greater than 10). However, studies show similar improvement in oxygenation with both simple and exchange transfusion.
3. Which of the following statements regarding acute chest syndrome is INCORRECT?
a) acute chest syndrome is a leading cause of hospital admission and mortality in sickle cell patients.
b) adequate pain control and incentive spirometry are important adjuncts to therapy.
c) choice of antibiotic should cover for atypical and encapsulated organisems.
d) fat embolism is a leading cause.
e) hydration at 2-2.5 times the maintenance fluid requirement is indicated for treatment.
A) Statistically, acute chest syndrome is the leading cause of mortality in those with sickle cell anemia. In terms of illness requiring admission acute chest syndrome is second only to acute pain crisis. B) Pain control and incentive spirometry is an excellent adjunct to managing the acute chest patient. Splinting leads to poor ventilation which raises risk for atelectasis, hypoxia, and secondary infection. However, caution should be used as over medication can lead to poor respiratory drive and therefore poor ventilation as well. Some studies suggest Non-invasive Positive Pressure Ventilation is better tolerated with better outcomes. C) Pulmonary fat embolism and infection are believed to precipitate most cases of acute chest syndrome. With regards to infection Chlamydia pneumoniae is the most common organism found followed by Mycoplasma. It is also important to keep in mind that sickle patients can have a poor functioning spleen which increases risk for infection by encapsulated organisms. Therefore it is important to cover both atypical and encapsulated organisms in a patient with acute chest syndrome. D) Pulmonary fat embolism accounts for up to 25% of acute chest syndrome cases. Intramedullary bone infarction is believed to be the source of pulmonary fat embolism. Free fatty acid associated enzymes (eg. Phospholipase A) lead to pulmonary and lung parenchyma damage. E) Intravenous fluid therapy should be used with caution. Bolus should be reserved for those that are hypotensive. Greater than one and a half times the fluid maintenance rate rarely (if ever) should be considered for fluid resuscitation. Over hydration provides a serious risk for iatrogenic pulmonary edema.
- A patient with history of sickle cell anemia that presents with cardiopulmonary symptoms think acute chest syndrome. If new infiltrates on x-ray plus above symptoms treat for acute chest syndrome.
- Administer inhaled beta agonist even with no signs of reactive airway disease.
- Low threshold for transfusion. Geared towards clinical improvement as opposed to hemoglobin correction. Important to not exceed hemoglobin of 10 as to avoid hyper viscosity and worsening of pain crisis. Exchange transfusion reserved for PaO2 less than 70 refractory to supplemental oxygen.
- If emergent exchange transfusion unavailable may try the following:
1. phlebotomize 500ml
2. infuse 300ml normal saline
3. phlebotomize 500ml
4. infuse 4 units packed red blood cells.
- Pain control is important but not too much! Incentive spirometry also a good adjunct. Non-invasive positive pressure ventilation if patient is still splinting. For patients on a vent avoid PaO2 much higher than 100 as this can suppress erythropoiesis.
- Cover for atypical and encapsulated organisms.
This case discussion presented by Richard Gordon, MD