My two children both with Sickle Cell Disease type Sickle beta + thalassemia. Every time I look up information on the Internet, look at brochures, books and etc this disease always shows an African American person. I understand that this disease is most common among the race but I want people and society to know that this disease CAN AFFECT ANY RACE!
Sickle Cell Disease can affect ANY race! My name is Vikki and my husband and I am a Caucasian couple with 2 beautiful boys that have from Sickle Beta Thalassemia Plus. I am of Italian decent and have a thalassemia trait. When I was pregnant I was tested and my doctor wanted to test my husband for it as well. I received a call several days later that he has a sickle trait. I said “No way! My husband has blonde hair and blue eyes.” We thought at the time that this disease was only a black disease. We now have 2 sons that have sickle beta thal plus, one with blonde hair and blue eyes. We are expecting our third child and will not know until after she is born if she also has the disease. We were told that this disease is a more mild form of Sickle Cell and can but usually does not experience the same problems that someone with SCD does.
Unfortunately our oldest, 4 years old, has experienced severe crisis in his back and legs. We have spent time in the hospital due to the crisis and fevers. Our youngest, 2 ½ has only had his hands swell with mild pain. I think that was what the doctors tried to prepare us for, not what our oldest experiences. When I am watching my children run and play it is always in the back of my mind if it will cause a crisis for him. Is he over doing it? Is he going to dehydrate? Hydration is the biggest factor for us and is what saves us at times. Although they have sickle beta thal plus, we still live with this disease EVERYDAY!
Since I began writing this letter my youngest came down with a virus with symptoms of a fever only. Had him checked out but the next day he was very irritable and pale. I called the doctor at about 4:45 when he woke from a nap very lethargic and I noticed him falling over a couple of times. I brought him in to the Children’s Hospital as advised by his doctor and his hemoglobin was down to 4. His normal is about 9.2-9.5. We experienced our first blood transfusion and he has been tested and confirmed to have been affected with the Parvovirus which I was not aware of. My next son became sick on Dec 8 for only 2 days but on the 12th woke with stomach pain and slight paleness. None of this was uncommon for him after being sick. At night I gave him a bit of Tylenol with codeine for the pain and he woke at 10:15 acting very “strange”. He punched at his stomach and was not able to stand. I paged the doctor but during the day I spoke to the Nurse to let her know about him and told her he was playing but was just a bit tired with mild pain to his stomach. He said to bring him into the ER for a blood test. I left immediately and thank God I did. By the time I got to the ER his lips were blue! He went into respiratory arrest and he had splenic sequestration. His hemoglobin dropped down to 1.8! Yes….1.8!!! Doctor said he has NEVER seen that and the next few hours for him were critical. Here my son is in PICU on a ventilator and my husband and I are told is very critical. WHY!! Because of a virus—Parvovirus. My son was in PICU for 18 hours and moved to the Oncology room for 2 ½ more days. He amazed his doctor and he told me along with every nurse that was there that he is our Miracle baby!!! My children are affected by this blood disorder and we almost lost both of our children from just a childhood virus! THIS DISEASE DOES AFFECT ANY RACE! Today though they have both fully recovered and doing very well. I encourage everyone to find out all they can about this disease (SC). I heard of the Parvovirus but did NOT know that a simple childhood virus could have taken our children from us!
Again, I want people to see other faces of people who suffer from this disease and to let everyone know it is NOT only a black disease. It can and it does affect ANY race!
Vikki Glarum Contact: vikkiglarum@hotmail.com
A Response
My name is Silvia and although it pains me to read that your two sons have this unfortunate disorder, I was surprise to read about them, because they are white….like me….and yes we can have sickle cell just like any African American. Different maybe, but sickle for sure!
I am 41 years old, White Female, Italian, Mother & Father from Sicily, and I too have Sickle Beta Thal. I have been under the care of this great Oncologist here in Houston and after being under his care for 16 years, my sister and I are still the only 2 “white” patients he cares for with Sickle Beta Thal…He even mentions us in his conventions, because I guess we are kind of rare, two of a very small number worldwide.
I have lived a pretty normal life considering the obstacles I have had to endure, (the most major ones have been, Avascular Necrosis which led me to have a total left hip replacement last year, and cataracts on both of my eyes a couple of years ago) but let me tell you that although it’s not easy, it’s possible. I had my spleen removed when I was 9, but overall, I am HERE!!!! Alive and kicking… in pain sometimes, but kicking!!!!
With faith, love and determination, and obviously the care of a good hematologist, your boys should be alright. I carried two normal children (they are now 9 & 14) and had natural childbearing I had then knowing they would have the trait, but not the illness and are both doing great. My husband was tested before we decided to have children. I had a high risk doctor monitoring both pregnancies, but did extremely well on both of them.
There are siblings in my family and only 2 of us have the disease, myself and my youngest sister who is now 32, then I have two older sisters that carry the trait and have children that have nothing, thanks to the fact their husbands are clear of the illness, and last I have a brother who is clean as a whistle….My younger sister also has two beautiful children and is she is doing alright considering…
I graduated from Sam Houston State, I worked for 15 years +plus and travel once or twice a year every year around the world…..Sometimes limping with pain, but nothing will stop me from making memories for my husband and children….I have had my share of crises in the hospitals and now that I am older things are better. For one, I know what to expect; two, I have the patience now to explain to the hospital staff that I am not a “junkie” hoping to get high in the hospital, that yes, you NEED Strong pain medication and they should do the blood test right away when you go to the ER for a crisis and check for sickle activity and call your doctor RIGHT AWAY before they laugh at you and doubt you because you are “white” and the ER doctors think you are crazy or they are in the wrong room, because they are expecting a black person when they walk into the room, (pure ignorance)…
I have learned to pace myself, know my limits and take care of any infections right away, to avoid major crisis, and I have learned to live with the pains and control them with medications, without abusing them for sure. I want to enjoy my children. That is my #1 GOAL in this life I have called a journey. They are my gifts and I cherish them each and every day. It’s hard, but it can be done!
My hemoglobin runs at an average of 8-9 which is good for me. I have had a few blood transfusions, not too many, though. My lowest had been around 3.5. That was definitely panic mode for my doctors. My blood type is O+ which is the Universal type…I can give to everyone A or O (if I could!), but only O+ can give to me. Just for your info, my sister had to have “30 bags” o blood transfused in her in “one month” alone when her liver was acting up with the sickle cell. Wild, but she came out of it well. Everybody can be very different… I am older than her, and I have never had 30 in whole life yet..knock on wood! With her, her symptoms seem to be more about the lungs and organs, mine so far more about the bones and joints, I have lived with constant mild body pains since I can remember. My heart is slightly enlarged, but pumping just great. My liver slightly enlarged, but nothing to be concerned about…check it periodically for your enzymes count, keep them leveled… not too much Tylenol is a good key. My thyroid is high, but under control.
I am not going to lie, some days are really hard, but there are some that are so good you forget you are sick. I don’t obviously know you, but the number one advise I can give you is be patient, strong and loving to them and FOR them and for you….If they have crisis, they will need you at the hospital constantly… don’t leave them alone…
The pain is so excruciating at times, that unless they have a great support doctor and staff, they will think your boys are there to get high and that is the most insulting thing you can endure when you are in so much pain. They need you to push them and to watch that they get proper amount of medical care….the best way to administer the pain medicines is to do it in intervals of smaller but frequent dosages, instead of a big shot every 4 hours…or so, you’d want to control the low/high peaks of the pain by doing in smaller dosages where you can stabilize the pain by repeating them in 2 hours or so…The morphine, Demerol whatever that may give them works well right away, but wears off extremely fast leaving you with hours of extreme pain in between dosages and you can’t get any more pain medicine for 2-3 LONG hours for fear that they stop your breathing functions…also, the patient self-pump works well, if they can give you boosters in between. That is the only way I have been able to make it thru at times without cursing out the world… Regardless if you are black or white, that if you have sickle cell, it’s painful, and we need respect and be treated with dignity. We are not there for pleasure. Professionals know as soon as they do your labs…trust me!
My crisis usually last 7-10 days and then it takes me about a week to recuperate and get my strength at home. I have had as many as 3 major ones in one year, I have also gone 5 years or more without one; so, to me in my case, they are very unpredictable. My sister is just as wacky in that sense…. Infections & Stress usually number one triggers for us. I do live with some kind of pain almost every day of my life and I can control much of it at home and with my doctor.
Anyways, I hope your sons are well and healthy…I hope they both have great lives ahead of them and I hope my short story in this email has helped you in some way to have a little insight in the life of someone else that knows exactly about what you may go thru. Three, I learned, not to ask HIM why me? There is always something that could have been worse. I accept it and it is what makes me who I am today, but it does not define me and I try to make sure it does not control me too MUCH… It doesn’t matter if you are black or white, this illness knows no COLORS, only pain!
I will put you and your family in my prayers and if you can, and you don’t mind, please update me on your boys. I truly hope they are doing great!!!!
May God bless you and your family,
Silvia
