Sickle Cell Crises

How long do crisis last?

 

It depends on what is causing the pain.  If you have avascular necrosis of the hip it can be painful most of the time.  If you have some other medical problem (gallstones) the pain can last hours.  If you are having a sickle cell pain event this can last about 7 days, sometimes a little more.

 

See the answer to a question on the site from someone who asks about pain medication.

 

This is a problem with pain medication if you take it for days, like you do in the hospital.  You can have hyperalgesia.  This means that you have feelings of pain from things that should not be painful or you have general pain when there is no reason for the pain (your pain episode is really resolved).  This is a common side effect of opioid medicine.  Opioids can also cause tolerance, the longer you take opioids the more you need for the same amount of pain.  There are other side effects (like depression) that can occur when you are having a pain episode.

 

This is all confusing to you and to the doctor who is treating your pain.  Pain is pain, you actually feel the pain in your brain and nervous system; you usually cannot distinguish these side effects from sickle cell pain..  It does not matter if it is hyperalgesia, tolerance, or whatever.  Pain episodes do not last for weeks.  If you have having prolonged episodes of pain you need to see someone who is a pain specialist and knows about sickle cell disease.

 

With frequent or prolonged pain you should be on hydroxyurea or chronic blood transfusions and withdraw from the opioids.

 

 

What are new advances available to adult sickle cell patients who suffer reoccurring crisis almost every month or every 2-3 months? Also has multiple allergies to pain medications.

 

Hydroxyurea is the only medication that is available currently for sickle cell disease.  If you are not taking hydroxyurea you should talk to your healthcare provider about this medication.  Look at the answers to questions about hydroxyurea to find out more.

 

There are studies of new treatments and medications if you are near a large sickle cell center find out if they are participating in a pain study.  You can look on ClinicalTrials.gov to find studies that may interest you.

 

Look at some of the other answers about pain.

 

 

Does vaso-occlusive and aplastic crises feel the same?

 

Aplastic means your bone marrow (where blood is made) is not making blood.  People use aplastic to describe severe anemia, but it is technically not an aplastic event unless it is due to bone marrow failure.

 

There is a relationship between having a severe pain crisis and having blood breakdown or hemolysis.  (I like to call them a pain episodes, they are not really a “crisis”.  Though you feel like it is a crisis.)  During a severe pain episode many people have their hemoglobin decrease and can even need a transfusion.  A friend of mine, who is much smarter and more experienced than I am (Samir Ballas, M.D.) has written about pain and severe hemolysis.  He is an expert in sickle cell pain.

 

If you have had a recent red cell transfusion, you could be having a delayed hemolytic transfusion reaction (you are breaking down the transfused red blood cells and sometimes even your own red blood cells), that can feel like a painful episode.   Or even cause a painful episode.  One of the reasons a doctor finds out that someone is having a delayed hemolytic transfusion reaction is they come to the emergency room or clinic with pain and low hemoglobin.  (As has recently been written on Sickle Cell 101 it is a good idea to have someone who knows you come to the emergency room with you when your are having pain or are sick.  You might be too uncomfortable to remember you just had a transfusion.)

 

There is something that doctors call an “aplastic crisis” and that is infection by Parvovirus B 19.  This virus infects your red blood cells before they have an opportunity to come out of the bone marrow and you become anemic in a very short time.  This period of not making blood can last a week or more, with sickle cell this leads to severe anemia.  The infection itself may not cause any symptoms.  Most people who have a Parvovirus B 19 infection and anemia need a transfusion, especially children.  Pain is not generally associated with this infection.  You can be sick and have a fever with Parvovirus, and if you become very anemic you may have pain.  I have seen children who were never sick and only tired due to severe anemia with this infection and needed a blood transfusion.

 

Your spleen can become enlarged and fill up with a lot of blood (splenic sequestration) which is another reason you could have low hemoglobin rapidly.  This is a medical emergency in children with sickle cell disease as they can become so anemic they have heart failure.  This is technically not being ‘aplastic” as your bone marrow does not have enough time to make blood splenic sequestration happens so fast.

 

 

What are the best ways to prevent and treat vaso-occlusive crisis. I'm currently in Nigeria and have been involved in SCD clinics, this seems to be one of the many common health issue patients are facing, that and wound ulcers?

 

Where it is available hydroxyurea is a medication that can prevent complications of sickle cell disease when used appropriately and monitored.  Currently there is a study in Angola, the DRC, Kenya, and Uganda in children to determine the correct starting dose and the maximum tolerated dose of this medication. This study has no results at this time.  In India (half of all people in the world with sickle cell disease live in Nigeria, DRC, and India) it has been reported that hydroxyurea at 10 mg/kg/d is a safe and effective treatment for sickle cell disease. At this dose there was a dramatic decrease in pain episodes, along with all other parameters studied. Children were examined and had laboratory testing monthly.  This dose is two to three times less that what is recommended in the United States, it would be expected that hematological side effects would be decreased at this dose.  The dose used in the United States was determined by the maximum tolerated dose (the dose at which there were significant decreases in blood counts).

 

Niprisan is a mixture of five herbs that are found in Nigeria. The medication derived from these plants has been tested in humans and in sickle cell mice and has been found to be effective in reducing pain in sickle cell disease in small studies. The medication has been manufactured in Nigeria by a private company, however, in December of 2015 the license for manufacture was withdrawn from the pharmaceutical company and the drug is now. manufactured by National Institute of Pharmaceutical Research Development (NIPRD) and is available only through this government agency.  I am not aware that the active ingredients of the herbal mixture is known.

 

It has been reported that supplementation with vitamin D has also decreased pain in sickle cell disease in small studies.

 

Besides medications diet and good hydration can help reduce pain.  The amount of water that must be consumed by people with sickle cell disease is much higher than those without sickle cell disease as there is an inability to conserve water in sickle cell disease due to damage to the kidney.  Poor nutrition, infectious disease, and relative dehydration all contribute to pain in sickle cell disease.  It has been suggested that extremes in temperature also contribute to pain episodes.

 

There is published literature that indicates arginine butyrate given intravenously can help with healing of skin ulcers in sickle cell disease.  Otherwise, wearing shoes with high tops can prevent the injury that leads to skin ulcers.  A very difficult problem for people with sickle cell disease, without a standard therapy.

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