Acute complications of sickle cell disease .pdf

This presentation displays the acute complications of sickle cell disease. It starts by presenting a real case of a child admitted to the hospital because of one of these complications…

1. Acute Complications of
Sickle Cell Disease
By; RawanAbdulwali Al-Akwa’a

2. Presentation Outline
•The case
•Differential diagnosis
•Definition of SCD
•Epidemiology of SCD
•Pathophysiology
•Clinical features
•Management of acute complications

3. The Case
•A 10 years old male patient living in Sana’a, who is a
known case of Sickle cell anemia, came complaining of
right leg pain for 2 days and chest pain for 1 day
prior to admission.
•The right leg pain started suddenly, was severe, and
involved the whole right leg but more around the hip joint.
It was relieved by paracetamoland Diclofenac analgesics
initially but then pain is no longer relieved by them. The
pain made him unable to walk. There is also lower back
painin the right side.

4. The Case
•The chest pain was sudden, severe, central chest pain,
stabbing in character, aggreviatedby taking deep breath
and relieved by analgesics. It was associated with dyspnea
at the time of event. No cough, fever, or wheezing. No
orthopnea, PND or lower limb edema.
•The patient is suffering from fatigue, palpitations, dizziness
and headache. He has also sleep disturbances because of
pain. No fever, anorexia or weight loss.
•He has no other complaints when reviewing other
systems.

5. The Case
•Past History; he is a known case of sickle cell anemia. He
had multiple acute pain episodes and multiple hospital
admissions.
•No surgical history.
•Drug history; Folic acid and hydroxyurea
•Family history; 2 of his brothers are having SCA. One of
them has died recently after receiving Bone marrow
transplantation.
•Socioeconomic history; No habits of medical importance.
No recent travel history. Middle income and good housing
and ventilation.

6. The Case
•On Examination:
•Generally; The patient looks ill, conscious, in pain. He is
of average body built. He has pallor, mild jaundice, but no
cyanosis. No respiratory distress and no signs of
dehydration.
•Vital signs;
•RR 30
•HR 122
•Temp. 37
•Blood pressure not documented

7. The Case
•On examination:
•Chest; good air entry, vesicular breathing and no added
sounds.
•Heart; normal first and second heart sounds, no added
sounds and no murmur.
•Abdomen; Soft abdomen, no tenderness or organomegaly.
•Lower limb; right lower limb tenderness along the whole
limb. No erythema or swelling. The pulse is felt and it was
of normal volume and was symmetrical. The capillary refill
time is normal. Neurological examination was also normal.
•The left lower limb and the upper limbs are all normal.

8. The differential diagnosis
•Acute vaso-occlusive pain
•Acute chest syndrome
•Acute osteomyelitis
•Septic arthritis
•Avascular necrosis, Legg-Calve-Perthesdisease
•Slipped Capital femoral epiphysis

9. Lab investigations
CBC
Hb 9.4 WBC 9.4
MCV 88 Neutrophils 52.9
MCH 31.3 Lymphocytes 37.3
MCHC 35.6 Monocytes 6.4
RDW 17.5 Eosinophils 2.9
Platelets 340 Basophils 0.5
CRP 2
Na 137
K 4.30
Urea 31
Creatinine 0.27
ALT 18

12. Radiological results
•MRI of both hips shows:
•Preserved spherical contour of both femoral heads
with no evidence of avascular necrosis.
•Intact both hip joints with smooth articular surfaces.
•Preserved marrow signal of the imaged bones.
•Normal MR appearance of the periarticular
musculature with preserved intervening fat planes.
•No joint effusion could be seen.

13. What is the diagnosis?
•Acute painful episode as a complication of Sickle cell
disease.

14. Sickle Cell Disease

15. Definition
•Sickle cell disease (SCD) is a group of genetic disorders
that cause an abnormal Hbmolecule (HbS) that
transforms RBCs into sickle shaped cells, resulting in
chronic anemia, vasoocclusiveepisodes, pain and organ
damage

16. Forms of Sickle cell disease

18. Epidemiology
•Sickle cell anemia is the most common form of intrinsic
hemolytic anemia worldwide.
•Predominantly affects individuals of African and Eastern
Mediterranean descent.
•Africa has the highest prevalence

19. Pathophysiology
•Point mutation in the beta globin gene-Glutamic acid is
replaced with valine. 2 alpha globin and 2 mutated beta
globin subunits create pathological hemoglobin S (HbS)
•HbSpolymerizes when deoxygenated causing
deformation of the erythrocytes (sickling). This can be
triggered by:
•Hypoxia (high altitude)
•Dehydration
•Acidosis
•Stress
•Pregnancy

20. Pathophysiology
•Sickle cell lack elasticity and adhere to vascular
endothelium and causes vascular occlusion and
subsequent tissue infarction.

21. Clinical features
•Sickle cell trait:
•Often asymptomatic
•Painless gross hematuria due to renal papillary
necrosis: often the only symptom
•Symptoms of sickle cell disease may manifest due to
severe oxygen deficiency

22. Clinical features
•Sickle cell disease:
•Onset: 30% develop symptoms in the first year of life;
>90% by the age of 6 years
•Typically manifests after 3-6 months of age as the
production of HbFdecreases and the HbSlevels
increase.

23. Clinical features
•Acute manifestations:
1.Vaso-occlusive events:
•Acute Vaso-occlusive pain
•Stroke
•Acute chest syndrome
•Kidney infarction
•Dactylitisor bone infarction
•Myocardial infarction
•Complications related to pregnancy
•Priapism
•Venous thromboembolism

24. Clinical features
•Acute manifestations:
2. Infection:
•Pneumonia
•Meningitis
•Osteomyelitis (most common cause: salmonella spp.)
•Sepsis (Streptococcus pneumonia)
3. Severe Anemia:
•Splenic sequestration
•Aplastic crisis
•Hyper-hemolysis

25. Acute Vaso-occlusive pain
•One of the most common types of vaso-occlusive events
in SCD.
•While these episodes were previously called "sickle cell
crises", we prefer to use the term painful episodes
because not all patients are in true crisis, and pain
should not be allowed to progress to the point of crisis
for patients to receive appropriate analgesia including
opioid analgesics if indicated.

26. Acute Vaso-occlusive pain
•It is intense pain, although there is significant variability
in the severity and frequency of acute painful episodes
•Pain may be accompanied by tissue ischemia and
inflammation.
•The sites of pain can include the back, chest,
extremities, and abdomen. In young children, dactylitis
(acute pain in the hands or feet) may be the most
common site of pain.

27. Acute Vaso-occlusive pain
Potentially serious complications associated with acute
pain:
•Acute chest syndrome (over 50 percent of ACS
episodes are preceded by or occur in the setting of
acute pain)
•Acute muti-organ failure
•Sudden death syndrome
•Acute surgical abdomen
•Acute papillary necrosis
•Delayed hemolytic transfusion reaction

28. Acute Vaso-occlusive pain
•Less commonly seen events that may be initially
misdiagnosed as an acute painful event include:
•Acute coronary syndrome
•Osteomyelitis
•Gout
•Arthritis
•Acute synovitis with avascular necrosis
•Autoimmune disease
•DVT or pulmonary embolism

29. Acute Vaso-occlusive pain-
Management
•Provide prompt pain management
•Ensure Adequate hydration
•Administer supplemental oxygen if SpO2 in room air
is < 95%
•Reassess pain every 30-60 minutes and titrate
analgesic dose as needed.

30. Acute Vaso-occlusive pain-
Management
•Consider diagnostics for:
•Evaluation of known triggers (e.g. dehydration,
infection) or complications (e.g., end organ
failure)
•Evaluation of differential diagnoses in patients
with atypical features:
1.Evaluate for acute chest syndrome in patients with
prominent chest pain
2.Evaluate for infection in febrile patients.

31. Acute Chest Syndrome
•It is defined as a new radiodensityon chest imaging
accompanied by fever and/or respiratory symptoms.
•It is an acute complication of sickle cell disease (SCD)
that is potentially fatal and requires immediate
intervention regardless of the patient's age.

32. Acute Chest Syndrome-C/Fs
•Chest pain
•Fever
•Respiratory distress, cough, wheezing
•Signs of vaso-occlusive pain (e.g., pain in arms or legs)
•Rib or sternal pain

33. Diagnostic criteria for ACS
•Clinical findings of one or more of the following:
•Chest pain
•Cough
•Temperature >38.5
•Tachypnea
•Hypoxemia
•Signs of increased work of breathing
•Wheezing
•Crackles
•Plus a new pulmonary infiltrate on chest X-ray that involves
at least one segment and is not due to atelectasis.
•If the CXR is normal, it should be repeated in 24-48 hrsif
there is ongoing clinical suspicion for ACS

34. Management of ACS
•Hospital admission with close monitoring and
hematology consult
•Supportive care;
•Respiratory support: Supplemental oxygen (target SpO2 >
95%), identify patients at risk of progression to respiratory
failure, NIPPV or invasive mechanical ventilation as needed.
•Pain management: opioids
•IV fluids: avoid overhydration, which can lead to pulmonary
edema
•Bronchodilators if needed
•Incentive spirometryto prevent atelectasis
•VTE prophylaxis
•Treatment of complications

35. Management of ACS
•Antibiotics;
•Obtain blood culture (two sets) and sputum culture before
starting antibiotics
•Start empiric antibiotics:
•Third generation cephalosporin (e.g. Ceftriaxone) plus a
macrolide (e.g., azithromycin)

36. Management of ACS
•Evaluation for blood transfusion;
•pRBCsindicated if Hbis > 1.0 g/dl below baseline
•May not be indicated if patient’s baseline Hbis 9 g/dl or more
•Urgent exchange transfusion in the following cases:
•Oxygen saturation < 90% even with supplemental oxygen
•Worsening respiratory distress
•Worsening pulmonary infiltrates
•Hbconcentration continuing to decline after a simple
transfusion

37. Infection
•Patients with SCD are at high risk of infection most
commonly by encapsulated bacteria (e.g. S.
pneumoniae, H. influenzae, S. typhi, Meningitidisspp.)
due to functional asplenia.
•Peventionof infection by;
•Immunization; all the recommended vaccination plus
Pneumococcal and meningococcal vaccines.

38. Infection
•Antibiotics; daily prophylactic penicillin ( Children < 3
years: penicillin V 125mg PO twice daily; children 3 years
or more: penicillin V 250mg PO twice daily)
Recommended from 1-2 months of age until 5 years old
for children with HbSS.
•Continue penicillin prophylaxis in children who have had
a splenectomy or history of invasive pneumococcal
infection.
•Erythromycin prophylaxis in children with penicillin
allergy.

39. Infection
•Routine Diagnostics;
•CBC
•Reticulocyte count
•Blood culture
•LFT and KFT
•Chest X-ray if there is chest pain, cough or tachypnea.
•Consider urinanalysis, urine culture, CSF analysis, or
synovial fluid analysis depending on the underlying
symptoms
•Do not delay empiric antibiotic therapy while
awaiting laboratory results.

40. Infection
•Management:
•Start IV fluid therapy as needed; avoid overhydration
•Start empiric antibiotic therapy parentrallywhile
awaiting diagnostic results.
•Evaluate for and treat any underlying condition, e.g.:
•Sepsis
•Meningitis
•Pneumonia; should be managed as acute chest syndrome
•Septic arthritis
•Osteomyelitis (Salmonella, S. aureus)
•Acute cholecystitis

41. Infection
•Management:
•Empiric antibiotic therapy:
•First line: IV third-generation cephalosporin (e.g. Ceftriaxone
50-75 mg/kg IV once daily or 25-37.5 mg/kg IV every 12
hours)
•Second line: Clindamycin 10 mg/kg every 6 hours)
•Suspected acute chest syndrome: Add Azithromycin 5-12
mg/kg IV/PO once daily.
•Severe illness or suspected meningitis:
•Increase the dose of ceftriaxone 80-100 mg/kg IV once daily (max.
dose 4g/day)
•Add Vancomycin(60 mg/kg/day IV divided into 4 doses given every
6 hours)

42. Acute Anemia in SCD
•It is defined as a drop in Hbof 2 g/dl or more from
baseline, or a Hbvalue less than 6 g/dl (if baseline is
unknown).
•Approach;
•Stabilize the patient and provide immediate
hemodynamic support, if needed (Pts. Can present with
high cardiac output heart failure and hemodynamic
shock)
•Send an urgent type and screen and crossmatch.

43. Acute Anemia in SCD
•Administer blood transfusion according to transfusion
indications
•Identify and treat the underlying cause (aplastic crisis,
splenic sequestration, acute hepatic sequestration,
acute chest syndrome, delayed hemolytic transfusion
reaction, infection, sepsis, and acute blood loss (not
necessarily linked to sickle cell disease))

44. Thank You