sickle cell anemia

Double-Helix, BSN, RN · Mar 7, 2012

Since I work in PICU, we see children who come in with vaso-occlusive crisis, acute chest syndrome and who are post-op cholesystectomy or splenectomy.

Our hospital also has a sickle cell clinic where children in mild vaso-occlusive crisis will come for IV fluids.

One of the primary causes of sickle cell crisis is dehydration. So our #1 treatment is fluid resuscitation with normal saline. Many of these children also need blood transfusions.

Pain management is tricky, as these kids are often in severe pain due to the sickled blood cells. Pain tends to be primarily in the joints and chest, but can be generalized as well. Our younger children are assessed with a standardized pain scale- FLACC or Wong-Baker depending on the age and development. Then they are treated with IV pain medicine as needed. Morphine is the drug of choice, but sometimes we use Dilaudid as well. Older children who can report pain will nearly always be on a PCA that delivers a continuous dose of morphine as well as a demand dose when they press the button.

One of the major complications of sickle cell disease is acture chest syndrome. This occurs most often when a child in vaso-occlusive crisis develops pneumonia, or consolidation in the lungs. Because of the sickled blood cells and the mucous congestion in the lungs, they can get a huge build up of infiltrates which severely impairs their lung function. If you look at these kid's x-rays, you will see a solid mass of while where the lung should be. They need a lot of respiratory support including high flow O2, BiPAP or CPAP and up to intubation. They also may need an exchange transfusion. In a typical blood transfusion, you give a unit of blood to the patient in addition to the blood they already have. The goal is to keep an adequate percentage of healthy blood cells to sickled cells. The desired ratio depends on the patient and the situation. For a typical surgery, you want at least a 70% to 30% healthy/sickled ration. In an exchange transfusion, you are removing the patient's blood and giving them donor blood. So you can transfuse multiple units of healthy blood at one time while removing the sickled cells.

I hope that helped. If there is any way you can get in touch with a hematologist that covers your sickle cell patients, I'm sure you will find them to have a wealth of knowledge and information.

KelRN215, BSN, RN · Mar 9, 2012

I care for children with sickle cell anemia infrequently but, when I do, it's usually because they've had a stroke or have developed moya moya disease, both of which children with sickle cell anemia are at an increased risk for. Fluids and pain management are definitely the priorities in caring for these patients.

Pain assessment in children has everything to do with their developmental level and very little to do with their disease. Infants, toddlers and some pre-schoolers are assessed with the FLACC pain scale. This also applies to developmentally delayed school-age children and teenagers. Most school-age children are able to utilize the Wong-Baker faces scale and some have been around the block so many times that they can use the standard NRS.

hiddencatRN, BSN, RN · Mar 10, 2012

Ashley, some of our hematologists recommend very conservative fluid replacement in sickle cell crisis because of potential issues with acute chest. I guess their belief is that overloading with fluids can increase the likelihood of developing acute chest? Is that something you've seen in your practice? I have frequently had patients where there are orders for maintenance fluids and pain meds but no bolus or a 10ml/kg instead of a 20ml/kg.

To the OP, I've had patients who have developed varying degrees of allergy to morphine, so frequently we will administer benadryl prior to giving morphine and when we do not, be on the lookout for reactions (I had a patient develop hives in a line along the vein within minutes of administering their morphine- they'd never had issues with it before and now will need benadryl). We also use warm packs to painful areas or a gaymar pump. We assess pain in the same way we do with other patients (FLACC, OUCHER, or number scale).