TJMS Q&A FEBRUARY 1, 2017

Black America Web

How does sickle cell affect A1C reading?

The A1C is not a reliable test for diabetes when someone has the sickle “S” red blood cell gene.  The laboratory test, Fructosamine is better to measure the sugar level in individuals with hemoglobin S and therefore a better screening for diabetes.

What causes sickle cell?

The sickle gene “S” developed as a process of evolution to combat malaria.  People with sickle cell trait have immunity to the malaria parasite. It’s a good gene when it is all by it self.

Sickle cell disease occurs when a person has two sickle genes that they get from each parents.  There are many sick cell genes, S, B, E, O, etc.

Do people that carry the sickle cell trait have any symptoms?

Typically no.  However, there is not a national registry for sickle cell to know for sure.  There are specific situations when symptoms can occur.  When a person is in a low oxygen area or situation: high altitude, diving, extreme exertion, there can be more sickling which leads to oxygen deprivation and trapping of sickle cells in the gall bladder and spleen.  This occurred with Ryan Clark, Pittsburgh Steelers football player, when he traveled to Denver to play. He eventually has his spleen and gallbladder removed.

There is a rare kidney cancer that is associated with sickle cell trait.  It is very rare so we don’t want to cause panic in people.  Once it is detected, it is fatal and people  die within a year.  Again, it is very rare.  Some people will present with blood in the urine.  You will want to see your doctor for further testing.

1.  How does having the sickle cell trait affect you when you deep sea dive or skydive?

2. What happens to people who has sickle traits when they go into areas with high elevation or deep sea diving? 

3. I have sickle cell trait and had a hard time as I climbed mountains in Denver. How does the trait affect people in high altitude areas or deep sea diving? Can anything be done to prepare when engaging in those activities?

4.  My daughter carries the sickle cell trait. What is it about deep sea diving that we have to be cautious about because we are going to Destin Florida for vacation and she is a swimmer and she would like to go deep-sea diving.

Diving and skydiving are environments with less oxygen.  Less oxygen may cause the sickle cells to start sickling in large numbers and rapidly.  People typically get sick very quickly when this occurs.

 

What can you do: 

Know your body and KNOW YOUR SICKLE CELL TRAIT STATUS:  If you start feeling light headed, dizziness, short of breath, have a fast heart rate, pay attention to what your body is telling you. 

 

Seek immediate medical attention

If you have sickle cell disease, you will probably need oxygen if you travel above 7000 feet (2100 meters). If you have sickle cell trait (some people do not know they do), altitude-related complications (eg, infarction of the spleen) can occur, even at altitudes below 9000 feet (2700 meters).

Here is the complete reference about what to look for and how to prevent complication.

 If you live below 5000 feet (1500 meters), avoid ascending rapidly. On the first night, avoid sleeping above 9000 feet (2800 meters).

  • If you plan to travel above 9800 feet (3000 meters), do not increase your sleeping altitude more than 1600 feet (500 meters) per day as you go higher. Plan a day of rest for every 3200 feet (1000 meters) you ascend. On this rest day, do not over-exert yourself.
  • Climb high and sleep low. Hike to a higher altitude during the day and return to a lower elevation to sleep at night. This will help you adjust to the altitude.
  • If you plan to ski, hike, or climb, do not over-exert yourself during the first few days at altitude. Pacing yourself is very important. Avoid alcohol and sleeping pills, especially as you are adjusting to the altitude in the first two days.
  • Staying or hiking at elevations above 4900 feet (1500 meters) in the weeks before you ascend may allow you to ascend faster.
  • If you drink caffeine (coffee, tea, soda) regularly, do not stop drinking it before or during your trip. Caffeine is safe at high altitudes, and stopping it suddenly can cause symptoms similar to AMS.

Is it true that Sickle Cell people have characteristics of high shoulder and protruding forehead over eyebrows?

I am not aware of bones that are deformed in sickle cell like you could see in Thalassemia or hemophilia.  

    How do we donate to sickle cell research?

    The Foundation for Sickle Cell Disease Research is actively participating in research.  We want to make sure that people are aware of clinical trials and why it is important to participate. 

    You can donate to the Foundation at fscdr.org  

      My husband has sickle cell trait and I don’t. He didn’t even know he had it. All 3 of our children have the trait. Why? Will it always pass on to a
      child?

      With each pregnancy, the chance of passing the gene to your offspring in the same

      • Dad = S (sickle red blood cell gene) and A (normal red blood cell gene) = AS
      • Mom = A (normal red blood cell gene)  and A (normal red blood cell gene) = AA

      With this combination there is a 50/50 chance of having a baby with AA (normal red blood cells) or AS (sickle cell trait red blood cells) with each pregnancy. Your children may pass the gene on to their children.

      U mentioned sickle cell trait & high altitude. My daughter, age 11 has the trait, how will she be affected when flying?

      Commercial airplanes are pressurized, so flying is not a problem unless flying in an unpressurized plane.

      In what ways can sickle cell trait affect you when having cosmetic surgery? Is it safe?

      The risks related to the type of surgery will be given to you, you should inform your surgeon, anesthesiologist  and other members of your care team that you have sickle cell trait and ask if there are additional precautions for the type of surgery you will have and sickle cell trait.  If you have a history of any complications during or after surgery, let your care team know.  

      What’s the symptoms for Sickle Cells in adults?

      Because sickle cell disease is a disease of oxygen deprivation, every organ can be affected.  Symptoms are varied but pain is the most common.  Other complications can occur in the lungs, heart, skin, kidney, brain, skin (ulcers).

        I’m trying to understand if the Doc was saying that a person can only get sickle cell if both parents have the trait? Or from one parent who actually has the disease fully?

        Each parent passes a gene to their baby.  There is  a variant of normal blood A, which is blood C.  Blood AC is a normal variant of normal blood.  However, if one parent passes the C gene and the other parent with Sickle Cell Disease or Sickle Cell Trait passes the S genes, the baby could have SC blood which is also sickle cell disease.

          If a pregnant mom has the sickle cell disease does that necessarily mean the child will have it or will it complicate the pregnancy?

          Pregnancy is considered high risk and pregnant females should be have a high risk OB/GYN physician as part of their care team.  

           A mother with sickle cell disease does not pass sickle cell disease to her baby.  She would pass one sickle blood gene and dad would have to pass the other sickle blood gene for the baby to have sickle cell disease.

            I’m a woman with sickle cell and I wanted to know if a woman in my condition will still be able to have kids just as easy as a normal female without sickle cell?

            Pregnancy rates depend on many factors in all women.  If you have a chronic disease like sickle cell disease or Lupus where we know there can be complications, its important to have a care team to review your medical history and monitor you and the baby closely throughout pregnancy.  

            How can you tell if you have sickle cell anemia or the sickle cell disease? I am a carrier of the trait. I have three children and of those three two of my children have the trait as well.

            Since the late 1980’s all babies born in the US are screen for the sickle cell gene (both carrier and disease).  It is rare for someone to have sickle cell disease and not be aware that they have it.  There are milder cases that are missed.  Although these are rare, ask your doctor for hemoglobin electrophoresis.  This is a blood test will tell you your percentage of sickle blood gene and red blood cell gene. 

             You could have passed the sickle gene to your children.  However,  the gene could have been passed from Dad also if he has sickle cell trait.

            Are there any long-term research studies completed on multigenerational families impacted by sickle cell and the impact upon life and lifestyle?

            The risks related to the type of surgery will be given to you, you should inform your surgeon, anesthesiologist  and other members of your care team that you have sickle cell trait and ask if there are additional precautions for the type of surgery you will have and sickle cell trait.  If you have a history of any complications during or after surgery, let your care team know.  

            What’s the symptoms for Sickle Cells in adults?

            Because sickle cell disease is a disease of oxygen deprivation, every organ can be affected.  Symptoms are varied but pain is the most common.  Other complications can occur in the lungs, heart, skin, kidney, brain, skin (ulcers).

              I’m a 55 yr. old Black female with Sickle Beta Plus Thalassemia. My 2 sons have the Sickle Trait and my daughter has the Beta Thalassemia Trait. How did that work out like that?

              Each parent passes a gene to their baby.  There is  a variant of normal blood A, which is blood C.  Blood AC is a normal variant of normal blood.  However, if one parent passes the C gene and the other parent with Sickle Cell Disease or Sickle Cell Trait passes the S genes, the baby could have SC blood which is also sickle cell disease.

                For mor information visit fscdr.org or call us at 965-397-3251.  We can help you no matter where you are located.