Acute Chest Syndrome


What is the mechanism behind acute chest syndrome if it's not due to an infection? How is it triggered? And how can you differentiate?


Acute chest syndrome is just what it says it is: a syndrome.  It is a new pulmonary infiltrate, elevated temperature, and chest pain.  There is no way to tell whether this is a bacterial or viral infection or really a pulmonary infarct and acute chest syndrome.  Infections cause VQ mismatch so an infection will shunt blood away from the area of infection, decreasing oxygen and leading to hemoglobin polymerization and a pulmonary infarct.  So infection and infact are related.  You can also have fat emboli from bone necrosis going to the lung obstructing vessels decreasing oxygen and leading to the same severe acute chest episode.  Fat emboli are worse as they can to the lung, brain, kidney leading to multiorgan failure.  The treatment is blood transfusion.  Either simple or red cell exchange depending on the severity.  There are other therapies: inspirometer use, bronchodilators, and oxygen if necessary.  It is a leading cause of death in adults.



Is the breathing exercise machine used only during a crisis of acute chest syndrome or is it required during other circumstances too?


All people with sickle cell disease who have acute chest syndrome should have pulmonary function testing when they have completely recovered to determine whether they need to have other therapy for their pulmonary disease.  Asthma (airway hyper reactivity) is common in sickle cell disease and increases the probability for developing acute chest syndrome.



Avascular Necorsis/Hip Replacement


I am from Nigeria. I was born in August 1981 with HbSS. I have this pain in my left femur head which they said is Avascular Necrosis of the femur head. This pain has been on since 2009. I need your help.


Avascular necrosis (AVN) is "staged" with x-rays.  The stages go from 1 to 6, progressively getting worse.  If you have had AVN for seven years it is probably in the upper middle range of the scale.  Without knowing anything about your history or your hip, I would guess that you probably need to have a hip replacement.  In Nigeria (and anywhere else) this is not a "little" surgery.  You would need to have blood transfusions before and maybe even after or during the surgery.  Though blood transfusion during or following the surgery is unusual.


I you can't get the surgery, then what can you do and what can you expect.  You should have physical therapy.  Which are exercises to keep your hip as functional as possible.  You may need to have a shoe lift.


Exercises.  This will not stop the progression of the collapse of the head of the femur, but could (though not always) give you more mobility.  If there is no way to have a physical therapist evaluate your hip you should do GENTLE hip exercises to increase the mobility of your hip.


Shoe Lift.  As the femoral head collapses your leg becomes progressively shorter, not by a lot, but it is one of the reasons you may be limping.  If you can have someone do this: lie down on your back, straighten both of your legs out as far as you can.  If there is a difference in the length of your legs you need to have a shoe lift.  A physical therapist can help you with this and get the right size.


What is going to happen over time?  Occasionally AVN reaches a certain stage and does not change for years and years.  It always hurts, you always have a limp, but it does not get worse.  If you have AVN for a long time the head of the femur fuses with the pelvis.  You then do not have mobility of the hip joint, but you have less pain.


Hope this is helpful and you are able to see a physical therapist or an orthopedic doctor.



My daughter is 16 and has developed an inward curve on her right knee and has started hip pains. Will her knee ever get straight? For her hip, can we delay hip replacement?


Avascular necrosis of the hip is common in sickle cell disease.  I cannot comment on the inward curve of your daughter’s right knee without looking at imaging and doing an examination, your primary care physician or hematologist would be able to do that.  In addition if you have a referral to a physiatrist (physical therapy specialist) that person could offer advice if they have experience with sickle cell disease.


Hip replacement timing is a personal decision.  Either you have so much pain and limitation that you feel you would be able to have a better life with a replacement or you have such limitation of movement of the hip (fusion of the hip bone to the pelvis which will happen over time) that doing normal activities is not possible.


A complication that occurs with avascular necrosis are changes in the muscles and tendons in the leg, particularly on the side with the avascular necrosis.  The muscles will become weak and smaller due to the fact you are using that leg less, and the tendons that attach the muscles to the bones will become shorter (contracted).  It is important to have physical therapy and to do stretching and strength exercises to keep the muscles and tendons in good shape for the eventual surgery and recovery.  Due to these changes and the fact that the head of the femur collapses slowly over time the leg with the avascular necrosis will become shorter and you might need a shoe insert to be able to walk with less of a limp.

If you have had physical therapy and your tendons and muscles have in good shape and the difference between the length of the legs is not extreme,  the orthopedic surgeon can sometimes lengthen the leg during the hip replacement so you do not need a shoe insert after the surgery.


Another issue with chronic avascular necrosis is osteoporosis.  If you do not use a limb not only do the muscles and tendons become weak and shorter, your bones lose their strength and become weaker.  This can be a problem during the surgery as you need to have strong bones to have the hip replacement hardware placed by the surgeon.


Bottom line: Even if you delay surgery you have to do physical therapy to prepare for the eventual surgery to be as successful as possible.  You need to have a full evaluation by a physiatrist and orthopedic surgeon to get advice.



Are there any recent advancements for the treatment of avascular necrosis in the hip? I have type SC disease and avascular necrosis or osteonecrosis of the right hip but I'm nervous about getting a hip replacement at 26 and want to weigh my options.


Avascular necrosis of the hip is, unfortunately, a common problem for people with sickle cell disease.  It can occur in children as young as ten years old. The most common time for occurrence is in adolescence.  Depending on how advanced your avascular necrosis is there are surgical options. You should consult an orthopedic doctor you trust who has experience with hip replacement.  It is not essential that the surgeon have experience with sickle cell disease, but that would be optimal.


Very important is the preoperative and postoperative care you receive, a knowledgeable hematologist should be involved with your care before and after surgery.  It will probably be recommended you receive a blood transfusion prior to surgery and you may need blood during the surgery.  Blood transfusions during a first hip replacement are unusual unless you have very severe hip disease.


A report from UCSF Benioff’s Children’s Hospital in Oakland indicated that physical therapy can be effective in delaying hip replacement for as long as three years in some patients, compared to a procedure called a “coring”.  Hip replacement is a choice you make with your primary care provider and an orthopedic surgeon.  It depends on your level of disability and pain.  The most important part of the surgery is what you do after surgery.  You should have a good physical therapist and do the exercises prescribed.  Hip replacements don’t last forever, there are some activities you will be advised not do after a hip replacement.



Blood Clots


Any tips on preventing blood clots for my 3 year old son in hospital for a pain crisis?


Thrombosis (blood clotting) is a complication of having sickle cell disease.  It is usually not a problem with small children, but rarely can occur.  The best therapy for pain and for preventing blood clots is getting up and walking and using your inspirometer when you are in bed.  A three year old probably can’t use and inspirometer, but can use a straw to blow bubbles into a glass of water, which is good lung exercise for a young child.  This will help keep the lungs “open” and can help prevent acute chest syndrome during a pain episode.





What can I do to stop having yellow eyes?


The sclera (the white part of the eye) can turn yellow if you have high levels of bilirubin.  Bilirubin is a breakdown product of hemoglobin in red blood cells. The most common reason for having yellow eyes (doctors call this scleral icterus) is due to rapid blood breakdown in your body called hemolysis.  Some people tend to have more hemolysis, some less.  People with more blood breakdown can have yellow eyes most of the time, or just when the blood breakdown is increased.  Yellow eyes does mean that something is going on with your blood or liver.


If you do not usually have yellow eyes and you notice that your eyes are yellow and your urine is dark, you should call your healthcare provider to let them know.  They may want to do blood tests to see why you are having scleral icterus.  Even if you have had this before you should still call.


Besides blood breakdown you could also have a problem with your liver.  High levels of bilirubin can occur with some types of liver problems.  If you have pain on the right side of your abdomen, it could be a sign of gallstones.  Another reason to call your healthcare provider.  You cannot tell if it is your blood breaking down or you liver that is causing yellow eyes, unless it is something you have seen your health care provider about and you know what the cause is.  Unexpected things happen when you have sickle cell disease, you should always call your healthcare provider or see them if you have something you are worried about.


People who have sickle cell disease need to drink A LOT of water.  Your kidneys make more urine than people who do not have sickle cell disease and you can become dehydrated quickly if you are not drinking enough WATER, not soda or other beverages.  If you do not drink enough water that would not be the cause of your yellow eyes, but over time drinking water might decrease the yellow tint to your sclera.


If this is new or more intense or you have other symptoms (tired, dark urine, pain), you need to see your health care provider as soon as possible.



Leg Ulcers


What can I do to speed up the healing of leg ulcers?


Leg ulcers can be very slow to heal.  If you have deep ulcers or have had them for a long time you should get a referral to an skin ulcer clinic from your primary care physician.  If they are very deep sometimes you need a skin graft to help them heal.  There are experimental therapies for skin ulcers as well.


One of the problems with sickle cell disease is there is very little research for treating these common problems, so even though there are case reports of a therapy working it is not a sure thing unless there has been a reasonably large study.  A knowledgeable sickle cell physician should know about these therapies and whether they are worth a try in your case.





I was diagnosed with sickle cell retinopathy on my left eye 5 years ago. I cannot see clearly with it only, blurred vision. Here in Nigeria there's nothing that could be done. Do I still have hope?


A twenty-year study of retinopathy in Jamaicans with sickle cell disease revealed that this complication was five times more common in hemoglobin SC disease compared to SS disease.  By the age of about 25 years, 43% of people with SC disease had retinopathy compared to 14% with SS.  There is a possibility of regression (there was some improvement) of this disease, about 16%; and a possibility of progression (becoming worse), about 14%.  Visual loss was uncommon (1 of 82), though these individuals were only followed until age 26 years.  Twenty years before this study a smaller study of people untreated for retinopathy reported that progression was most common between 20 and 40 years of age and spontaneous regression was more common in hemoglobin SS disease.


What does this mean for you?  If you have stable visual loss and are older your vision may remain unchanged.  If you are younger you may have regression or progression, this cannot be predicted.  If you have access to hydroxyurea consider this therapy as an option.  If there is any possibility to see an eye specialist you should do that.


Hydroxyurea is discussed in the answers to other questions.



Other Complications


Why does sickle cell disease produce H-shape Vertebra?


Veterbral disease in sickle cell anemia has not been very well studied.  There are a few theories about the biconcavities and step-like depressions of the vetebra seen in sickle cell disease.  The most obvious is the relationship between osteoporosis (Adams-Graves, 2014) and and collapse in older adolescents and adults contributing to this finding.  Another theory is the fact that the ends of the vetebra are supplied by the main nutrient artery of the vetebra and this becomes narrowed leading to decreased blood supply to the vertebra limiting growth (Diggs, 1973), while the other areas of the bone are supplied by perpheriting vessels.  Diggs also looked at the histology of some postmortem vetebra and did not appreciate infarcts in the vetebra bodies.


It is clear that this pathologic finding increases with age and can be seen in young children. I could find no studies looking at vetebral disease in sickle cell anemia by MR.



I have a two year old granddaughter with sickle cell anemia.  She has monthly transfusion.  At the age of of 4 months she had an episode with her spleen and that’s when they started the monthly transfusions. Why would her physician want her spleen removed?  Is this something that is usually recommended?


A life-threatening splenic sequestration even can happen again if the spleen is not removed, or you can develop hypersplenism leading to a chronically low hemoglobin.  As with many things in medicine you cannot know exactly what is going to happen with every patient.  The issue with severe splenic sequestration is that it occurs rapidly and can be fatal.


Transfusions are given to prevent another episode while the child is receiving all of their immunizations.

Most children with hemoglobin SS have functional asplenia by the age of about five years, so having a splenectomy for a child with sickle cell disease is almost (but not the same) as having your spleen lose function.


It is recommended that children who have a splenectomy take penicillin for the rest of their lives due to the risk of infection by pneumococcus.


You should have all of the recommended immunizations including the 23 valent pneumovax and meningococcal vaccine before an splenectomy.


There is risk for everything, but most people think the risk of a second life threatening splenic sequestration warrants surgery.  Of course there are children who have splenic sequestration that is not life threatening (or maybe was) and do not have a splenectomy and do okay.  You have to decide how much risk you are willing to take with your beautiful granddaughter.



I was born with SC and in 2013 I was diagnosed with DVT. Are they related? Is that common? I'm now on blood thinners everyday. I went from 160 lbs to 215. My small frame can't handle all of this extra weight and I have NO energy.  Please advise!


Yes, there is an association between sickle cell disease and DVT.  Most of the articles I have seen deal with hemoglobin SS and S beta thalassemia, where there is an increased risk for venous thromboembolism.  Thrombotic events are much more common in people who have indwelling ports versus those who do not have a indwelling intravenous line.  There is an association with elevated pulmonary artery pressure as well.  You may have had an cardiac echocardiogram to measure your pulmonary artery pressure, this is an important screening test in adults.


African Americans in general have an increased risk of DVT compared to other populations, there is some evidence (not great) that the gene responsible has been isolated.


There is one article on weight gain after DVT, but that article attributes the weight gain to decreased activity after having the DVT.  I will do a literature search and see if I can find any other information about weight and anticoagulants, but I could not today.  I will send you another note if I find anything.


It is difficult to lose weight, but it is extremely important for everyone; especially if you have sickle cell disease.  Increased weight actually increases your risk of DVT.


There is now evidence that moderate exercise is good for people with sickle cell disease (see other answers about weight and exercise).  If you want to lose weight you will have to change your lifestyle and your diet.  If you are going to do that you should see your health care provider to see if they can refer you to a diet counselor and monitor your progress.  I don't know what you are on for an anticoagulant, but if you do lose weight the dose might have to be decreased.


Personal experience:  The mother of one of my patients lost A LOT of weight, she stopped eating at Fast Food Restaurants (did all her cooking at home with no fried foods) and stopped drinking juice and soda.  You have to drink a lot of water if you stop the sugary drinks.  It is easier if you have a friend who will support you with your change in diet and exercise.  The exercise does not have to be extreme, just walking and getting out as much as you can everyday would be great.



What are the possible treatment of skin disease in a sickle cell patient of 5 years old? No other symptoms for now.


It is difficult to know a treatment for skin diseases in sickle cell disease as there are so many reasons for a skin rash or other skin disease.  Generally speaking the treatment of skin disease in sickle cell is no different than skin disease in other children when they are as young as your child.


Thank you for your question, I wish I had a better answer.



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