Peds 30 Flashcards
Which 3 hemoglobin (Hgb) patterns are associated with a diagnosis of sickling disorders on the newborn screen (NBS)?
- FS
- FSA
- FSC
Which are two most common surgeries in children with sickle cell disease?
- Cholecystectomy (gallbladder remove)
- Tonsillectomy
Why is penicillin given to children with sickle cell disease on a prophylactic basis?
To prevent infections that can lead to sepsis
What are 4 expected complications of sickle cell disease?
- Jaundice
- Anemia
- Stroke
- Respiratory problems )ie. pneumonia, vaso-occlusion in lung parenchyma - acute chest syndrome)
What are 2 important health maintenance priorities during a 2 year WCC for a child with sickle cell disease?
Vaccinations - 23-valent pneumococcal polysaccharide vaccine (pneumococcal polysaccharide vaccine) & Meningococcal conjugate
Transcranial doppler to assess risk for stroke
Gerardo is a 2-year-old male with sickle cell disease who presents with acute fever, chest pain, and respiratory distress. Exam is significant for hypoxemia; rhonchi and decreased basilar breath sounds with dullness to percussion bilaterally; and increased splenomegaly. CXR shows bilateral infiltrates and cardiomegaly. CBC shows WBC 21,500 and platelets 450,000. Using the faces pain scale, he says his pain is 10/10.
What’s the leading dx and management plan?
Acute chest syndrome
Admit inpatient
- start IV fluids at maintenance rate (correct dehydration with bolus/fluids if needed)
- manage pain (narcotics/NSAIDs)
- abx (ceftriaxone, azithromycin)
- incentive spirometry
What should your management plan consider and/or include for a child with sickle cell disease, admitted for acute chest syndrome?
IV fluids maintenance rate
Opioids for pain
Packed RBC transfusion
Antibiotics (ceftriaxone IV, azithromycin PO)
A 5-year-old male with sickle cell disease presents to clinic with a chief concern of severe chest pain for the past day. His mother notes that he has been breathing quickly and that she measured his temperature this morning to be 100.5 F. He describes the pain as an 8/10 on the faces scale. He is tachypneic on exam and has an oxygen saturation of 97% on room air. Chest exam reveals normal lung sounds bilaterally, and he has some reproducible tenderness over his chest wall. A chest x-ray is performed and demonstrates clear lung fields and a cardiac silhouette that is within normal limits. What is the most likely cause of the chest pain?
A. Acute chest syndrome B. Congestive Heart Failure (CHF) C. Pneumothorax D. Rib infarction E. Sepsis
D. Rib infarction
Rib infarction is correct because the patient has chest pain in the setting of a history of sickle cell disease with normal oxygen saturation and clear lung fields on chest x-ray. Rib infarction may lead to a picture similar to ACS as the pain can lead to hypoventilation, which may result in atelectasis and the characteristic radiographic findings of ACS. However, no radiographic findings are present in this patient. As a result, rib infarction is the most likely etiology.
A 2-year-old patient with sickle cell disease presents for a well child evaluation. She has a history of three sickle cell vaso-occlusive crises in the past, including dactylitis and bone pain. She has been symptom-free for a few months and today she is feeling well. She is meeting her developmental milestones. She had an upper respiratory infection recently, but seems to be getting better now. She is up to date on her standard vaccinations up to 2 years including a full course (four doses) of Prevnar. What would you do for her today?
A. A dose of pneumococcal polysaccharide vaccine (Pneumovax).
B. Chest x-ray
C. HPV vaccine
D. Quantiferon gold test
E. Right upper quadrant (RUQ) abdominal ultrasound
A. A dose of pneumococcal polysaccharide vaccine (Pneumovax).
You are notified that a 10-day-old patient in your practice had a newborn hemoglobin screen positive for sickle cell disease. Pregnancy and delivery were uncomplicated. Mother is 19 years old and works as a nurses’ assistant at a nursing home. When questioned, she says she remembers her grandmother died of chest pain and a lung infection. Which of the following should be ordered next for the baby?
A. Antibiotics B. CBC C. Chest x-ray D. H. influenza type b vaccination E. Transcranial Doppler
A. Antibiotics
This newborn has sickle cell disease. Confirmatory testing should be performed using high-performance liquid chromatography (HPLC) or DNA probe. In the meantime, she should be placed on penicillin prophylaxis. Her grandmother likely passed away from acute chest syndrome, a common complication of this disease. Due to decreased splenic function and consequent decreased resistance to infection with encapsulated organisms (Streptococcus pneumoniae, Haemophilus influenzae type b, Neisseria meningitidis), infants and young children with sickle cell disease are at increased risk for sepsis. When given to infants with sickling disorders, penicillin significantly decreases the risk of mortality from overwhelming sepsis. Prophylaxis is usually continued until the child is five or six years of age (after this, there is little data to support its use except in patients who have had documented sepsis and bacteremia, or who have had their spleens removed).
A 2-year-old male with sickle cell disease is brought to the ER by his parents because he is not feeling well. His mother reports that he has been very tired for the past week. Vitals show a temperature of 39°C, BP of 120/75 mmHg, RR of 24 bpm, and pulse of 104 bpm. On physical examination he is ill appearing. His conjunctivae appear pale, and his sclerae are anicteric. Lungs are clear to auscultation. His abdominal exam is benign (nontender, nondistended, with no organomegaly). His extremities are nontender upon palpation. His nailbeds appear pale. Stat CBC reveals Hgb: 4.5 g/dL, Hct 15%, WBC 1800, and platelets 88,000. Mother is concerned as she has never seen him so ill before. What is the most likely cause of his new symptoms?
A. Acute chest syndrome B. Aplastic anemia C. Hemolytic crisis D. Splenic sequestration E. Vaso-occlusive crisis
B. Aplastic anemia
Parvovirus B19 causes aplastic anemia, especially in children with shortened RBC half-life. A mild anemia associated with parvovirus often goes unnoticed in healthy children. However, in patients with decreased RBC survival, such as sickle-cell patients, anemia can develop very rapidly and severely, along with decreases in the other cell lines (resulting in leukopenia and thrombocytopenia). In the above presentation, the presence of fever in a child with sickle cell disease should also raise concern for significant bacterial infection from encapsulated organisms like pneumococcus. This patient should have a blood culture and be started on empiric antimicrobial therapy pending screen labs and cultures.
What is the inheritance pattern for sickle cell disease?
Autosomal recessive - each parent has one copy of the recessive gene
What are 8 s/s that you should teach parents to look for that needs emergency medical attention?
Fever, splenic enlargement, slurred speech, chest pain, rapid breathing, increased pallor, increased jaundice, priapism
What are 3 processes that can lead to acute chest syndrome?
What are 3 things the provider needs to rapidly address?
Pneumonia, intrapulmonary sickling, pulmonary fat embolism
Infection possibility, pulmonary/respiratory concerns, pain
