14F sickle cell anaemia

THIS IS AN ONLINE E LOG BOOK TO DISCUSS OUR PATIENT'S DE - IDENTIFIED HEALTH DATA SHARED AFTER TAKING HIS / HER /GUARDIAN'S SIGNED INFORMED CONSENT .HERE WE DISCUSS OUR INDIVIDUAL PATIENT'S PROBLEMS THROUGH SERIES OF INPUTS FROM AVAILABLE GLOBAL ONLINE COMMUNITY OF EXPERTS WITH AN AIM TO SOLVE THOSE CLINICAL PROBLEMS WITH COLLECTIVE CURRENT BEST EVIDENCE BASED INPUT 

Blog of her previous admission- 

https://venkata-phaneendra.blogspot.com/2022/02/13-yr-f-with-sickle-cell-crisis.html

History - patient was apparently asymptomatic until 4 years of age 

Then she developed fever, pain abdomen and was taken to nilofur hospital where patient was investigated and was diagnosed to have sickle cell anemia 

Since then she's been up for the Followup 

Her admissions previously for bronchopneumonia in the initial years with frequency of 2-3 times per year 

Patient was taken to the thalassemia and sickle cell anaemia society and was having blood transfusions since then every 6 monthly 

And is started on benzathine pencillin prophylaxis 

Hydroxyurea

Folic acid and other supportive medications

She's on regular follow up at the society every month for the same 

Her visits to the hospital has been increased since past 3 years with episodes of 

Acute chest syndrome

Vasoocclusive crisis

Thanks to Dr.Madireddy.Keerthi for her valuable inputs summarised in the images below.

Family history -

14year old female Born to parents -non consanguinous marriage 

Father- ambulance driver by occupation

Mother - housewife

With first male child died at the age of 3 months 

Index patient is the second child 

3rd child- daughter with no similar complaints

Both Parents were investigated accordingly and was found to have sickle cell trait 

Daughter- homozygous sickle cell anaemia

Present admission- 

14 F is brought to casualty with complaints of 

Pain in Left upper limb since 1week

Fever since 5 days

H/O slip and fall 2 weeks while running near farm at her home

Fever - low grade , intermittent 

Associated with headache 

No diurnal variation

Not associated with chills or rigors

Not associated with cough cold pain abdomen vomiting loosestools

Pain - left shoulder and elbow 

Dragging type of pain 

Non radiating

Associated with swelling 

Occasionally pain at right lower limb

History of blood transfusions +

Last transfusion was done - February

History of similar complaints in the past 

Previous hospital admissions 16

Bronchopneumonia

Sickle cell crisis

Vasoocclusive crisis

Managed conservatively and is maintained on drugs 

Hydroxyurea

Calcium 

Folicacid 

Prophylactic pencillins and vaccinations

O/E- 

14 year old female lying comfortable on bed

Conscious coherent cooperative

Well oriented to time ,place and person 

Pallor+

Icterus+

Ln-

Pedal edema -

Vitals- 

Temp - 100F

Bp- 100/70 mmHg

Pr- 110bpm regular

Sat- 96 on RA--- > today-91 RA --> 2l 02(98)

Grbs -113mg/dl

Rr- 12cpm ( thoracoabd)

Peripheral pulses present( ul &ll )

Local examination- 

Left Upperlimb examination -

Skin - smooth shiny

No scars ,ulceration

No pigmentation or colour changes

Pain+ during movement of shoulder ,elbow 

( especially during abduction and extension)

Tenderness+ at midshaft at forearm & shoulder

Pulses palpable ( brachial and radial)

Right lowerlimb- 

Discolouration + 

CVS- S1,S2 + 

RS- BAE + ,NVBS heard

Per abdomen - soft ,non tender 

Bowel sounds + 

No organomegaly

CNS- 

Pupils NSRL

Sensory normal

Tone , power -normal

Gait - ? antalgic 

No cerebellar signs 

Investigations-

Xray left elbow -

Diagnosis- 

14F with sickle cell anaemia with

? Vasoocclusive crisis of left upperlimb and right lowerlimb

Plan- 

1 . Adequate hydration ( >2lts/day orally)

2. IVF NS,DNS,RL

3. Inj monocef 1g/iv /bd( given for 2 days)

4. Tab Dolo 650 /po /tid

5. Tab Ultracet 1/2 /po/bd (SOS)

6. Strict temp charting 

7. Vitals monitoring ,inform SOS