ABC of Sickle Cell Crisis

ABC of Sickle Cell Crisis

ABC of Sickle Cell Crisis

The Science: Oxygen the Common Factor
As the red blood cells pass through a low oxygen environment within the capillaries, abnormal hemoglobin S forms rigid fibers in the red blood cells causing them to deform into a sickle shape. These red blood cells are easily destroyed, as they travel through the blood vessels causing severe anemia.

The red blood cells stick to the blood vessels creating an inflammatory reaction and attracting white blood cells and platelets. All of these cells block the flow of blood into the organs causing severe pain and damaging the organs.

The Occurrence

90% of sickle cell crisis occur most often at night or during sleep because decreased breathing at night or duringsleep causes a low oxygen environment for the red blood cells which results in a sickle cell crisis.
5 % of sickle cell pain crisis occur due to cold weather and the remaining 5% of crisis occurs due to infection.

Prevention

Using oxygen prior to going to sleep will prevent 90% of sickle cell pain crisis. Use oxygen from an oxygen cylinder oran oxygen concentrator at a low rate of 1.5-2 liters/ minute. Use especially when you have had a long day, you are tired or you do not feel well.

Aircraft are pressurized to 8000 ft., which is a low oxygen environment, that will result in a sickle cell crisis either duringthe flight or after. You must use oxygen when you fly. Request permission from the airlines to take an FAA approvedoxygen concentrator such as Sequal Eclipse.

Cities or towns at elevations of 2000 ft and above e.g. Las Vegas, Nevada or Denver, Colorado have a low oxygen environment. Visiting or living in such high altitude cities will result in more frequent crisis. If you have to be there, use oxygen when you sleep to avoid waking up in a crisis.

Avoid cold environments, dress warm and especially keep your extremities warm. Cold weather delays the transit time of the red blood cells as they travel through the capillaries, and offload oxygen. This will cause them to deform into asickle shape and result in a crisis.

Taking new medications like Voxelotor (Oxbryta) that allows the abnormal Hemoglobin S to retain oxygen, will result in less destruction of the red blood cells and increase the hemoglobin to near normal or normal.

Treatment

Rapid administration of pain and anti-inflammatory injections e.g. Dilaudid/Morphine and Toradol /Diclofenac within the first 15 minutes of a crisis, while at home, under pulse oximetry and video monitoring, will abort 90% of crisis. It will change a crisis from a 3-week crisis with multi-organ damage to a 30-minute crisis with no organ damage.

Your doctor should prescribe the same dose of pain and anti-inflammatory injection medications that you have tolerated before in the hospital, teach you and your caregiver how to administer it (IM or SQ), and set up a system to monitor you remotely at home, when you have a crisis.

Failure to abort the crisis in 30 minutes means there are other issues causing the crisis such as infection and requires an immediate visit to the hospital or medical center for medical intervention.

Cure
Bone Marrow Transplant or Gene Therapy

Author: Sota Omoigui MD
Board Certified – Anesthesia and Pain Medicine
www.medicinehouse.com

https://www.researchgate.net/publication/338984448_Sickle_Cell_Pain_Crisis_Clinical_Guidelines_for_the_Use_of_Oxygen

https://medicinehouse.com/prevention-and-treatment-of-sickle-cell-pain-crisis/

https://
www.practicalpainmanagement.com/pain/other/how-manage-acute-pain-crisis-sickle-cell-disease-practical-recommendations

https://openhematologyjournal.com/VOLUME/4/PAGE/15/FULLTEXT/