Sickle cell pain crisis

87 Sickle cell pain crisis




Overview/pathophysiology


Sickle cell disease (SCD) comprises a group of hereditary blood disorders in which hemoglobin S (HbS) is the dominant hemoglobin. HbS (sickle hemoglobin) replaces normal adult hemoglobin (HbA). HbS differs from HbA in the substitution of one amino acid (valine) for another (glutamine). Under conditions of dehydration, acidosis, hypoxia, and temperature elevations, HbS changes its molecular structure and forms a crescent or sickle-shaped red blood cell (RBC). This causes the cardinal clinical features of chronic hemolytic anemia and vasoocclusion, which result from obstruction caused by the sickled RBCs and increased RBC destruction. In most instances, the sickling response is reversible under conditions of adequate hydration and oxygenation. After repeated cycles of sickling and unsickling, the RBC remains in the sickled form. The most common form of sickle cell disease is hemoglobin SS disease (HbSS), also called sickle cell anemia or homozygous SCD, in which the individual inherits a sickle cell gene from each parent.


The inheritance pattern is autosomal recessive (both parents must at least have the sickle cell trait). If both parents have the sickle cell trait, there is a 25% chance that each child will have SCD, a 25% chance that each child will have neither the trait nor the disease, and a 50% chance that each child will have the trait. Therefore, a child may be asymptomatic (except under rare circumstances) with the trait or have varying degrees of symptoms with the disease. SCD is among the most prevalent of genetic diseases in the United States, predominantly affecting African Americans and Hispanic Americans. People of Arabian, Maltese, Greek, Italian, Sardinian, Turkish, and Indian ancestry are also affected (March of Dimes, 2008). Approximately 1 in every 500 African Americans and 1 in up to 1400 Hispanic Americans in the United States has SCD. Around 2 million Americans have sickle cell trait, with 1 in every 12 African Americans having the trait. More than 1000 babies are born with SCD every year (Emory University, 2010).


Pain is the leading cause of emergency department visits and hospitalizations. It can occur as early as age 4-6 mo and unpredictably throughout a lifetime. There is considerable variation in the severity, frequency, and types of pain among and within affected individuals.



Health care setting


Primary care with possible hospitalization for infections or pain crisis



Assessment




Signs and symptoms:


Generally do not appear in infants before 4-6 mo of age because of high levels of fetal hemoglobin (HbF). Pain is the hallmark manifestation and is caused by vasoocclusion and the resulting ischemia distal to the occlusion. Pain can range from mild and transient to severe, and it can be localized or generalized, lasting from minutes to days or weeks. The acute painful episode or event is reversible and can occur in the extremities, back, chest, and abdomen. Examples include acute hand-foot syndrome (dactylitis—usually seen in children between 6 mo and 2 yr old), acute joint inflammation, acute chest syndrome (a common cause of mortality manifesting as chest pain, fever, pneumonia-like cough, and anemia, abdominal pain or gallstones, and priapism). Stroke is another form of vasoocclusive event and has a high rate of recurrence. Children with SCD are at more than 200 times higher risk of having a stroke than children without SCD (American Heart Association, 2010). Children with SCD often have nonfunctional spleens due to “clogging” from the sickled RBCs, and this puts them at increased risk for sepsis. Overwhelming infection/sepsis was the leading cause of death in young children with SCD in the past, but recent studies show this changing with declining deaths in young children since the advent of daily penicillin and routine pneumococcal vaccines for infants (Adamkiewicz et al., 2008; Quinn et al., 2010).



Physical assessment:


History and physical, including character, location, severity, and duration of pain, as well as at-home treatment. Information should be obtained about methods used in the past to treat pain crises effectively. Initial pain assessment should be done and repeated before and after analgesia.



Diagnostic tests


Note: Newborn screening for sickle cell anemia is mandated in all states. Results are sent to infant’s primary care physician. If there is any HbS, a second test is done to confirm diagnosis (NHLBI, 2010).




Hemoglobin electrophoresis, isoelectric focusing, and high-performance liquid chromatography:


Enable definitive diagnosis of SCD.



Oximetry:


Noninvasive method that will reveal decreased O2 saturation if it is present.



Chest x-ray examination:


Helps differentiate between acute chest syndrome and pneumonia.



Complete blood count with reticulocytes:


May show increased white blood cells (WBCs) with infection. The life span of the normal RBC is decreased from 120 days to 10-14 days, so bone marrow compensates with increased production. Reticulocyte count gives an indication of RBC production by the bone marrow (reticulocytes are immature RBCs).



Blood culture:


Infection may have triggered crisis. Sepsis is the leading cause of death in children younger than 5 yr.



Basic metabolic panel:


If signs and symptoms of dehydration are present, helps assess degree of dehydration and need for electrolyte replacement.





Nursing diagnosis:


Acute pain

related to tissue anoxia occurring with vasoocclusion


Desired Outcomes: For mild-to-moderate pain, child states or demonstrates that pain has decreased within 1-1½ hr of receiving oral medication. For severe pain, child states or demonstrates that pain has decreased within 24 hr of intervention/treatment. Pain is less than 2 on a 5-point scale such as FACES scale or less than 4 on a 10-point scale such as FLACC or numeric scale.




























ASSESSMENT/INTERVENTIONS RATIONALES
After establishing a pain scale appropriate for child (FLACC, FACES, Oucher, Poker Chip, or numeric), assess pain before and after analgesic is administered (within 10-30 min after IV medication administration and within 1-1½ hr after oral medication administration). Assess pain level q2-4h unless on continuous infusion of pain medication, in which case assess qh. A developmentally appropriate pain scale helps monitor degree of pain and effectiveness of pain medication.
Assess hydration status q4h: level of consciousness (LOC), anterior fontanel if child is younger than 2 yr, oral mucous membranes, abdominal skin turgor, and urine output. This assessment helps detect and prevent/treat dehydration, which causes vasoocclusion/pain. A child who is dehydrated may exhibit decreased LOC, sunken anterior fontanel (if younger than 2 yr), dry or sticky oral mucous membranes, tented abdominal skin, and decreased urine output.
Plan schedule of pain medication around the clock, not prn. Consistent use lowers total amount of medication with better control. Prolonged stimulation of pain receptors results in increased sensitivity to painful stimuli and will increase the amount of drug required to relieve pain.
Do not administer meperidine (Demerol). Demerol increases risk of normeperidine-induced seizures, especially in a child with SCD.
Carefully apply warmth to affected area. Warmth may be soothing to child, but it should be applied judiciously because ischemic tissue is fragile.
Do not apply cold compresses. Cold promotes sickling and vasoconstriction.
Use nonpharmacologic pain control measures as appropriate for child. Optimally, comfort measures will distract child from the pain and augment effects of pharmacologic measures, but should not be used to replace them. Examples include distraction (watching TV or playing games), deep breathing, relaxation exercises, music, touch, imagery, and massage.




Nursing diagnoses:


Risk for ineffective cerebral tissue perfusion


Risk for deficient cardiac tissue perfusion

related to vasoocclusion and anemia


Desired Outcomes: Within 2 hr following treatment/intervention, child’s oxygen saturation is maintained at greater than 95% or at level prescribed by health care provider. There is no evidence of long-term complications from lack of oxygen.

























ASSESSMENT/INTERVENTIONS RATIONALES
Assess respiratory status and mental status q2-4h and prn. Frequent assessment ensures early detection of changes in respiratory status. Tachypnea and increased work of breathing (WOB) are early signs of hypoxia. LOC is a good indicator of oxygen perfusion to the brain.
Monitor pulse oximetry continuously. This is a noninvasive method of assessing oxygen saturation and noting changes promptly.
Administer oxygen as prescribed to keep oxygen saturation levels at greater than 95% or at level appropriate for individual child. Delivering oxygen when child is hypoxic eases WOB. However, it does not reverse the sickling process, and long-term use can depress bone marrow activity and increase the anemia.
Elevate head of bed to a comfortable level for child. This facilitates chest expansion by decreasing pressure on the diaphragm.
Ensure incentive spirometry q1-2h while child is awake. This treatment facilitates deep breathing and decreases the incidence of acute chest syndrome.
Administer packed RBCs as prescribed. This treatment improves tissue perfusion by correcting anemia.
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Jul 18, 2016 | Posted by in NURSING | Comments Off on Sickle cell pain crisis

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