An African American couple brings their 1 month-old baby girl for the establishment of care in your practice. They report that they are deeply disturbed because their baby girl is found to have sickle cell disorder with hemoglobin SS type. In order to provide the best healthcare possible for their daughter, they would like to have some information on prevention and treatment of sickle cell disease complications. Regarding the preventive and treatment strategies available for patients with sickle cell disease, which of the following statements is true?

A 20 year-old African American man comes to your office and reports fever, chill, abdominal pain and dyspnea. He developed chest pain three days ago that is progressively getting worse and back pain and leg pain this evening. His medical history is significant for sickle cell disease. He had abdominal surgery in the same hospital for recurrent hernia 10 days ago. He smokes one pack of cigarettes every day and has a history of asthma, controlled with an albuterol inhaler used on an as needed basis. He has a fever of 104⁰ F, blood pressure 110/70 mm Hg, pulse 114/minute. He has tachypnea with a respiratory rate of 37/minute and an oxygen saturation of 89% on room air. In physical examination, you see an anxious individual with increased work of breathing; hear bilateral wheezing and rales on auscultation. A chest x-ray revealed an infiltrate in the middle lobe of the right lung and an alveolar consolidation in the superior lobe of the left lung. There is no atelectasis. You reviewed the records of his most recent stay in the hospital and his chest x-ray done as part of his preoperative protocol was normal during that visit. Induced sputum test revealed lipid-laden macrophages, which are Oil Red O–positive. What is the most likely diagnosis in this patient?

Which of the following organisms is associated with aplastic crisis seen in patients with sickle cell anemia? A.Coxsackie A virus B. Roseola virus C.Parvovirus B19 D.Epstein-Barr virus E. Human papillomavirus Correct Answer is C. Aplastic anemia (AA) is a rare life-threatening disorder characterized by pancytopenia and a hypocellular bone marrow. Parvovirus B19 can cause aplastic crisis in patients with sickle cell disease. Incorrect Answers: A.Coxsackie A virus causes hand,foot and mouth disease. B. Roseola virus (HHV-6) causes sixth disease, mostly in children. D.Epstein-Barr virus causes infectious mononucleosis. E. Human papilloma virus commonly causes skin or mucous membrane warts.

A 42 year-old African American male comes to your office reporting fever, chills, tiredness, and trouble walking and putting weight on his right leg. He feels a painful swelling along the lateral surface of his right thigh. He is a known patient of your practice with a prior history of sickle cell anemia. He had multiple admissions for vaso-occlusive crises in the past. On physical examination, you see a febrile patient. The skin is erythematous over the middle of the right femur. There is tenderness along the area. A pocket ultrasound showed absence of spleen and the presence of lateral soft-tissue of the hip region showing a subcutaneous collection measuring 9.2 cm by 2.7 cm. There is also a subperiosteal fluid collection of 7 mm in size. You suspected osteomyelitis. What is the most common organism associated with osteomyelitis in patients with sickle cell anemia?

A 15 year-old African American boy presents to your office for the follow-up of his recently diagnosed sickle cell disease. He asks you that he is curious to know about the formation of Hemoglobin S in the body. How would you describe the gene defect that leads to the formation of Hemoglobin S? Change in sixth amino acid of the β chain from glutamate to valine (β6 glu → val) Change in 6th amino acid of β chain from glutamate to lysine (β6 glu → lys) Change in 26th amino acid of β chain from glutamate to lysine (β26 glu → lys) Change in 121st amino acid of β chain from glutamate to glutamine (β121 glu → gln) Change in 68th amino acid of α chain from asparagine to lysine (α68 asn → lys)

A 16 year-old African American boy presents to the emergency department complaining of severe pain in both legs and back. He climbed Mount Davis, the highest point in Pennsylvania (3213 ft) over the weekend. He reports that he enjoyed climbing the mount. As he was descending, he developed pain in his chest, legs and back. The pain has been present for 1 day and is steadily increasing in severity. He took ‘tons of tylenol and ibuprofen, and it ain’t cut it’. He denies any recent trauma. Since his pain started he feels like he should have listened to his parents, who warned him against the mountain trip because of a preexisting condition he has. He reports that he has suffered from severe bouts of chest pain, back pain and leg pain in the past when he engaged in multiple sporting events during the summer holidays. One of his cousins suffers from a similar disorder. On physical examination, he is afebrile and in some distress due to pain. He has nonspecific bilateral anterior thigh pain with no abnormalities appreciated. The remainder of his examination is normal. Laboratory tests reveal his hemoglobin level as 7.1 g/dL. Peripheral smear shows crescent shaped red blood cells. Patient was treated for his condition. As you prepare him for discharge, he informs you that next month, he is planning to travel to Tennessee to climb the Clingmans Dome in Great Smoky Mountains National Park. It is 6,643 feet in elevation. What would be the best advice you can give to this patient as he prepares for his next mountain climbing?