Hematology DXR Case I Terry Brooks 3 yrs old Puffy eye 3 days ago he was running around and

at night he complained his knee was hurting and then later on he complained that his eye is getting puffy Has had leg pain before No cancer Has sister with anemia Has been hospitalized for diarrhea, ear infection, twice for sore legs Child was cut out Diet does not eat very well still uses bottle. Likes chicken and some mashed potatoes No previous history of blood disorders Gave him IV in hospital Does not bruise easily He plays with other kids but fights a lot On Tylenol No allergies She works in the tobacco fields Won t let people move the leg In a old house Pesticides No discoloration of eyes or mouth Always sick Nobody has similar problems Just had puffy leg pain Had belly pain Does not know father s medical history Mom has arthritis Has had a fever for a while Tennessee No mood swings in the kid Both my parents are black Has not been diagnosed with STD NO HIV diagnosis Has been told by the DOH to go for check ups Not yellow looking X-ray of the belly done one time Does not remember pre-natal screening No rashes seen on body Has seen bug bites on body Dx Sickle Cell Anemia CLL Megaloblastic anemia Ricket s Lyme Disease Lead poisoning Vitals 100/60 a little high 102 temp Hr 160 Height 38.5 Weight 27.5 lbs Pulse 160 27 lbs

If not growing then pituitary or thyroid problem could be occurring Need to ask about milestones in a patient Heart Tachycardia no murmurs Lungs Sound clear Abdominal exam Hold the patient down Active bowel sounds Hepatosplenomegaly is present Lymph nodes Scattered lymph nodes palpable Not firm though Mouth Eye Ears Negative for erythema Joints Pt not bending his left knee Warm too touch No indication of localized effusion or source of infection Swollen knee present definitely Blood Work CBC Fever WBC high Has a left shift Anemic kid Platelets are adqueate in number Bleeding Time- Normal Blood smear o You see poikoloytes o Have sickle cells o Howell Jowell body seen in spleen dysfunction o Target cells present Spleen is not working well anymore Has high HgbS- two thirds of blood is sickleing Gram Stain on eye Salmonella in eye Immunocompromised due to spleen dysfunction Bleeding into knee Eye is near brain Need to worry about meningitis or Do a CT or blood culture also to determine what is going on in the eye and if any secondary spread of infection is occurring IV put into patient keep the pt in hospital. Need to feed the patient Give pt Normal Saline and give patient electrolytes and protein if patient needs this Ceftriaxone can be given to kill the Salmonella The spleen is auto-infarcting also Pupil looks fine There is a few strands of yellow stuff There is no sclera injection

Won t open mouth

Get hemolytic anemia- spleen, CNS, bones, liver kidney and lung are usually affected

Push social service in this patient Need long term help and financial support Need to advocate for the mother and child This child has sickle cell anemia complicated by a Salmonella orbital cellulitis. A major factor contributing to his health status is a poor social situation and lack of continuity of his care. Sickle cell anemia is a chronic hemoglobinopathy transmitted genetically and marked by moderately severe chronic hemolytic anemia with periodic acute episodes of painful "crises" and increased susceptibility to infections, especially S. pneumoniae. The heterozygous condition (Hb A/S) is called sickle cell trait and is usually asymptomatic, with no anemia. The condition is autosomal recessive affecting mostly the black population. It is equally distributed between male and females and spans all ages. It affects a variety of organ systems including: Hemolytic, Lymphatic, Immunologic, and Musculeoskeletal. Common signs and symptoms include pallor, while the signs of anemia present early in infancy. In early childhood painful swelling in the hands and feet present progressing to pain in bones, joints, abdomen, back and viscera. Patients have a functional asplenia with an increased susceptibility to infections, especially pneumococcal sepsis and Salmonella osteomyelitis. Throughout life they exhibit a chronic hemolytic anemia and in children, there is a delay in physical maturation. The anemia is lifelong. In the second decade, the number of crises diminishes but complications occur more frequently. Some patients die in childhood of CVA or sepsis. Most patients live to early or mid adulthood, though few live past age 50. The most common causes of death are infections, thrombosis, pulmonary emboli, and renal failure. The prognosis may be modified in the future when gene therapy and/or messenger RNA repair become a reality. Given the multiple clues from the history, finding sickled cells on the peripheral smear plus the eye culture results should lead to a consideration of the major diagnostic problems. The history and multiple significant illness in a young African American child raises a strong suspicion of sickle cell anemia which can be confirmed by a hemoglobin electrophoresis and inferred by an examination of the peripheral blood smear. Culture of the eye exudate grew Salmonella. The causes of fever in this patient were several including sickle cell anemia, the superimposed cellulitis, a septic knee joint, otitis, pneumonia or endocarditis. This ill child needs a thorough exam of all major organ systems, determination of the severity of his anemia, plus treatment with at least analgesics, antibiotics and IV fluids. Any hope of improvement in his long term prognosis is probably directly linked to a rapid improvement in his social situation, marked by thorough compliance and follow-up. Treatment of sickle cell disease has a strong focus in disease prevention and health care maintenance. In addition to the standard pediatric immunizations, pneumococcal vaccine should be given at 2 years of age. Follow-up boosters are administered at ages 5 and 10 years. Though an influenza vaccine may be beneficial it remains optional at this time, Prophylaxis with oral Pen VK is started at age 2 months and continues through age 4. Patients should be evaluated in the office at 2-month intervals until age 2 when it can be extended to every three months. At office visits, a CBC and reticulocyte count should be obtained. Iron and folate supplements are given and these levels should be monitored routinely as well. The mild pain episode can be managed at home with proper education. Prevention of crisis should be stressed and can be undertaken by avoiding cold, providing adequate hydration, avoiding over exertion to the point of exhaustion, avoiding exercise at high altitudes and avoidance of alcohol. Once pain occurs the fluid intake should increase to 150 mg/kg/24hrs. Application of moist heat to the painful area can increase comfort and Tylenol or Ibuprofen helps with mild pain. Moderate pain at home is best treated with codeine (1mg/kg/q4hrs.) More severe symptoms are best treated on an inpatient basis. Once admitted to the hospital, patient treatment includes analgesics, hydration, and the correction of any hypoxia or acidodic states. The presence of fever warrants the addition of antibiotics until culture results are obtained. This patient and family would greatly benefit from the intervention of social services. Income and food assistance would enhance their quality of life and moving to a home with better heat control and less overcrowding and exposure to disease would decrease the patients chances of painful episodes. Arranging for child development opportunities (head start, daycare) as well as parental education on developmental milestones would assist the patient in progress toward his normal development. Lastly, counseling for parents with children presenting with debilitating illnesses should be offered.