Highlights
Assessment 1 – Part C: Case Study of Joi Anderson
Joi Anderson, a sixteen-year-old female, arrives at the Emergency Department (ED) with her grandmother Sharyn complaining of bilateral thigh and hip pain. This pain has been present for one day and is steadily increasing in severity. Paracetamol and Ibuprofen have not relieved her symptoms. Sharyn denies Joi has had any recent trauma and states she hasn’t partaken in any excessive exercise. Joi reports feeling “tired all the time”, breathless and that “it hurts when I go to the toilet to wee”.
When asked if she has needed to go to the toilet more often, she replies yes. Sharyn also reports that Joi has had similar pain to this before which required hospitalisation when Joi was four and was diagnosed with sickle cell anaemia. Joi sees a haematologist once every one to two years for check-ups and has not required further treatment. No one in Joi’s family has had similar complaints. Sharyn explains that Joi’s father, Ben, is a fly-in-fly-out (FIFO) worker and has just returned to work for the next four weeks. Joi’s mother, Chloe, works long hours, so Sharyn lives with them to help with Joi’s cares.
Medical Past History:
Sickle cell anaemia – diagnosed aged 4, controlled since the age of 6.
Medications:
Nil.
Allergies:
Nil.
On examination:
• Temperature 37.5 °C
• Orientated to person time and place (GCS 15)
• Conjunctiva and mucosal membranes slightly pale in colour
• Capillary refill sluggish (5 seconds).
• Nonspecific bilateral anterior thigh pain with no abnormalities detected, pain score 3/10 • All other findings within normal limits
Investigations ordered:
• Blood test - FBE including reticulocyte count, blood group and cross match, U&E's and LFT's • Ward urinalysis
• Urine BHCG test
Significant findings from the above investigations were:
• WBC: 17 X 109/L
• Hb: 71g/L
• Urinalysis – Leukocyte esterase, nitrates, protein and blood present
• Urine BHCG negative
Based on blood test results and clinical features, Joi is diagnosed with sickle cell disease and is currently experiencing a sickle cell pain crisis. This pain crisis was triggered by a urinary tract infection, indicated by her symptoms of urinary frequency and dysuria and the elevated WBC count. The low Hb is indicative of sickle cell anaemia. Doctor’s orders for this sickle cell pain crisis includes:
• Place Joi on bedrest
• Pain management – PRN oral panadiene forte
• Clear fluids as tolerated
• IVT – 0.9% Sodium Chloride and 5% Glucose at 43 mL/hr and IV antibiotics as charted • Monitor oxygen saturations and administer oxygen 2 L/min via nasal prongs as required
The ED doctor contacted the haematologist asking for a review of Joi. The haematologist agreed with the ED doctor’s management of Joi’s sickle cell pain crisis and provided reassurance to Joi’s grandmother Sharyn. The haematologist also suggested there may be a need for a blood transfusion to help Joi’s anaemia and to lessen the blood’s thickness allowing it to flow easier to decrease disease symptoms and prevent complications. Sharyn immediately stated “no, Joi can’t have a blood transfusion, it is against our religion. We belong to the Jehovah witness faith”. The haematologist explained to Sharyn the positives of having this blood transfusion if needed but Sharyn continued to state “no, she will not have a blood transfusion. I will never give consent to this”.
Once Joi is stable and comfortable, the registered nurse speaks to Sharyn to obtain further information about Joi’s situation. Sharyn informs the registered nurse that she moved into the family home to care for Joi as both parents (Ben and Chloe) work long hours in order to make the repayments on their house each fortnight. Ben is a fly-in-fly-out worker and works four weeks away and is then home for one week before returning to work. Chloe works six days a week and is currently maintaining two jobs. Sharyn enjoys spending time with Joi but says it’s exhausting at times and she misses spending time with her friends. She also has hypertension, and type one diabetes and is finding her health deteriorating due to her caregiver role.
Sharyn also explains that they belong to the Jehovah Witness faith and they are very active in their church. She takes Joi to church meetings twice a week and mass on Sundays. Joi enjoys interacting with the other children in the faith and holds strong traditional Jehovah Witness beliefs. Sharyn speaks passionately about her religious beliefs and explains that she will “not allow anyone to do anything to Joi against these beliefs”. When asked if Joi participates in any out-of-school activities, Sharyn states that the family can’t afford for Joi to do any other activities, so it’s only the activities at the church that Joi is involved in other than school activities. Sharyn is concerned that Joi’s condition will mean more expenses, more caregiving roles for her and less involvement in the church community.
New information obtained:
As days pass, Joi is starting to feel better and is requiring less pain medication. As a result, Joi is scheduled for discharge tomorrow. As part of the discharge planning, the nurse (you) has a meeting Chloe asks many questions about sickle cell disease. She is very concerned about her daughter and what will happen to her in the future. You are aware that there are many serious complications that Joi could experience. Additional questions that were asked by Chloe and Sharyn include:
• “I have no idea what Joi should be allowed to do so she can have some fun”. • “Does Joi need to get any more vaccinations? I hate for her to get more needles. She cries and I know it hurts her”.
• “Should Joi be on any regular medication for her condition”.
• What happens if she has pain again? How do I manage it?
• What happens if I have another child? Will that child also have sickle cell disease?” • We are planning a trip to the Rocky Mountains in Canada next winter. Joi will be able to come along won’t she?”
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