THE TELEPHONE CONVERSATION - 1

This is a conversation a lot of girls may be familiar with and it may not necessarily be the same theme either. Sometimes we have it with our best friends and other times with total strangers. I decided to let it be over the phone between a young lady (Lydia) and her much older neighbor (Fay).

Lydia: I think I am lost.

Fay: now you are back on track. The first step to finding the right path is accepting you are lost and need direction. Tell me sweetheart, what is wrong?

Lydia: *deep breath, exhales* well, I’m not so sure where to start from. It’s like I’m in a maze so many different paths but only one will lead out. I will start however and from wherever till I get the story right. I hope you will understand.

Fay: you have my full attention and I will try my best to understand without interrupting you.

Lydia: thank you so much Miss Fay. I met this guy and I just have this feeling that he is playing me but I can’t seem to muster up the courage to leave him. I feel broken and empty after we have sex. He keeps saying he loves me, and he does want to marry me. But deep down inside I just know that he is lying yet I can’t leave him. *begins to cry* I don’t know why! I don’t know how I got this way. *sniffing*

Fay:  do you want me to come over?

Lydia: *sniffing and trying to compose self* no ma’am. It would have been lovely, but I feel so ashamed of myself. I can’t even bare to look myself in the mirror. I am dirty and …..*breaks down again*

Fay: it’s okay sweetheart. Take your time. You will be okay, let it all out, baby.

Lydia: *a couple of minutes pass before she is composed* I’m so sorry about all this. *mustering courage to smile*

Fay: no don’t be, that is the expected reaction for the way you feel and I am really proud you haven’t done worse *chuckles*

Lydia: *laughs for a bit* yeh I guess so! You see the thing is when I face him with the truth of our situation; he says no, I am being paranoid. And then one day he gave me his mobile to hold for him and out of curiosity I decided to check his inbox and sent messages. *exhaling loudly* I knew it was at my own risk, but I just had to. I saw he sent a mail to some other girl called Diana. I felt my heart crash and tears welled up in my eyes, but I smiled and acted normal. Right now, I don’t recall what the message contained but I do remember “…I love you so very much, Diana.” He has never sent me such a message. In fact he has never done anything positive for me worth remembering which is what hurts me the most. It hurts because after knowing all that I still cannot leave. Why? What more am I waiting for?

After a few weeks, I told him what I knew and he said “it’s my mother’s choice and you know I wouldn't want to offend my mother. But I promise you, if you are not married in a few years time, I will marry you.” I just shook my head and with all difficulty held back the tears that were threatening to roll down my cheeks. He must really think I’m stupid or I must be really stupid for him to have told me that with all sincerity. I wanted to scream and tell him to cut the crap. To stop the lie. To stop everything. But I just let it pass as with all other obvious lies he has told me. Is it because I think I love him or is it the need for me to be loved that is causing me to downgrade myself?

SUPPORT SICKLE CELL AWARENESS - 4

In the last post, we discussed on the patterns through which SCA can be inherited and on how to detect either before pregnancy or early in pregnancy how to determine the genotype of the offspring before birth. With the risks involved, poor and probably unavailable facilities to carry out the process, and where available is highly very expensive for an ordinary African citizen to afford, there is also the issue of for and/or against abortion, tradition, strong headed men, and the list is endless. So far the best solution will be for the couples to go and have their blood tested to know their genotype. This saves a very great deal of hurt, pain, regrets and the likes. Lakin, knowing how we humans 'don't think', sometimes knowing the genotype does not stop us from eventually (or not) being a contribution to the woes of the sickle cell community.

I use myself as an example; I have a 5 year old daughter and her genotype, due to the Goodness of God, is HbAS. Her father is HbAS and I'm HbSS ...... okay calm down please! I knew his genotype before we got married, I was the one that asked him to go do it! However all the "yori-yori" and "I have faith in God" was put aside when I found out I was pregnant. I was scared and I scared the life out of him too. He didn't know how it felt like to be living with an SCA patient and for the first  time he experienced it in 4D. I'm guessing it was among the worst times of his life; he donated blood twice to me, I would wake up in the middle of the night with so much pain from which I end up spending a week in the hospital given intravenous fluids and being transfused. The following week I would be home and after that I would be back. We had a time-table! I get admitted on Wednesday, return home the following Tuesday and be back again the following Wednesday. Life was a swing! Even the O.B.G.Y.N wards knew me...I had a special bed at the very back where it was cleaner and quieter! Okay! Reality checks: I was suffering in two different degrees; physically and psychologically. Some other time for the complete story.
Now that we know some people are stubborn and still get into offspring-bearing-relationships that could give rise to sickle cell patients, the next step is to know what to expect, how to handle it, who to seek help from and all the right/helpful things that will assist the patient. Scolding and saying "why did you?" is not going to change the situation.
You decided to go ahead with your marriage after knowing your genotype and knowing each of you is a carrier of the sickle gene. You have kids. Among them could be an HbSS child. What do you know about sickle cell anemia? How do you help the kid be healthy and able to cope with the "stress" of childhood? How do you keep yourself prepared for all the unexpected pain attacks? These are some of the important questions I hope I can help all of us understand.

The Know How/When/What about SCA patients

The first thing every parent, relative, friend and community need to know is exactly what Dr. Kenneth Enwerem of Omega Cares Foundation said describing sickle cell patients "They are not abnormal. They are special kids with a special condition, requiring special care".

Know also that the child did not bring it upon themselves but was as a contribution from both parents.

Know that the child is experiencing pain caused by the SCA and hurt/sadness from seeing their loved ones running around not knowing exactly how to ease the SCA pain.

Know that as younger kids they are likely to experience pain more often than later on in life; in other words, the intensity reduces as they get older!

Know that as kids, we cannot stop them from playing but we as parents and adults can monitor the hours they play and the sort of games they could participate in.

Know that it is your job as a parent to make sure the teachers and friends know of your child's health condition. This is so his/her activity can be monitored while at school also.
Know that you cannot stop the crisis from arising but you can help in easing the pain when it starts.
Know during the pain, all your kid can think of is how to get rid of that part of his/her body that is aching so they get pretty much impatient/irritated with any suggestion to keep calm and/or relax.
Know that water is indeed really your child's best friend! Encourage them to drink as much as they can in a day. Some may still bed wet till a really grown age but somehow it beats having to be woken up by a scream late into the night.
Know that as the parents you are solely in charge of making sure your kid takes his/her medication accordingly, eats healthy (even if most of the time their appetite is poor) it doesn't have to be much at a time.
Know that you can tell a lot through the color of your child's urine. Be very observant! You should also notice the color of the conjunctiva; is it very much jaundiced (yellow/green) or just a little?!
Know that they want to feel and be like the other regular kids but cannot be, not because they are less talented or unintelligent, but because physical stress wears them out faster than you can finish calling out their name!
Know that your child can still learn other easy fun less physically demanding activities such as riding a bicycle, swimming (but not for prolonged hours) and short distance walkathons. These are the few I can permit myself to mention.
Know that your child may be physically restricted (due to stress) but they are intellectually very active so encourage them or better still get them into the habit of reading and writing, playing creative games too. They are still fully functional.
Know that your child should be properly dressed according to the season; when it is cold, dress them warmly. when it is hot dress them simply.
Know that as they get older, although the regularity of the pain crisis reduce, there may be other complaints of pain anywhere in the body and this is caused by the constant untimely breakdown of the RBCs. These must be taken as seriously as any pain crisis and checked up at once.
Know that even though they may no longer be so dependent on you, they still need reminders from time to time to take their medications and keep up the regular water drinking habit too.
Know that your kid will grow, learn how to handle physical stress and have added stress to the list such as emotional, financial, educational and the likes. All they need is support, love and care.
Know that despite the heavy and numerous medical burdens that comes along with being SCA patient, they can and still do live a normal life; go to school, have friends, fall in love, have careers, get married and have kids.

SUPPORT SICKLE CELL AWARENESS - 3

So far I have written generally on sickle cell disease, how it comes about and the types concentrating on sickle cell anemia, it's symptoms and the main causing element of it; erythrocytes. I think we are making progress.

Knowing that the hemoglobin carries the genes through which SCA is inherited from both parents, I would like to briefly explain it.

AS and AS		male
		A	S
female	A	AA	AS
	S	AS	S

AS and SS		male
		A	S
female	S	AS	SS
	S	AS	SS

AA and SS		male
		A	A
female	S	AS	AS
	S	AS	AS

AS and AA		male
		A	S
female	A	AA	AS
	A	AA	AS

AA and AA		male
		A	A
female	A	AA	AA
	A	AA	AA

SS and SS		male
		S	S
female	S	SS	SS
	S	SS	SS

 
AA(yellow) - normal hemoglobin

AS(orange) - sickle cell trait/carrier

SS(red) - sickle cell anemia

For diagrams 3, 5 and 6, every pregnancy is expected 100% HbAS offspring, HbAA offspring and HbSS offspring respectively.

For diagram 4, in each pregnancy a 50% chance of HbAS offspring or 50% chance of HbAA offspring is expected.

For diagram 2, each pregnancy has a chance of 50% HbAS offspring or 50% chance of HbSS offspring.

For diagram 1, each and every pregnancy has a 25% chance of HbAA offspring, 25% chance of HbSS offspring or a 50% chance of HbAS offspring.

A lot of us make the mistake of thinking "if we have 4 children only one will be HbSS and one will be HbAA while the other two will be HbAS". It's wrong! The genetic ratio is for each and every time a couple will be having a child. If for instance a couple plan on having 3 kids and he is HbAS while she is HbSS every time she gets pregnant, that particular fetus could either have inherited a 50% probability of being HbAS or 50% chance of being HbSS. That means after all the kids have been born (not at once of course), the three of them could all be HbAS or HbSS or 2 out of 3 could be HbAS while 1 is HbSS or 1 could be HbAS while 2 could be HbSS. There is no saying this amount will come out with HbSS or this amount won't have HbSS. It remains unknown. However, modern improvement in medical sciences has come up with forms of prenatal diagnostic techniques that are used to determine if a fetus has any form of genetic disorder before it is born. 

-Chorionic Villus Sampling (CVS) entails the use of samples from the placental tissue of the fetus from as early as 8 weeks till 12 weeks of gestation. CVS is used for testing chromosomal abnormalities and other specific genetic disorders (including SCD/SCA) especially when the parents are known carriers of the defective gene and/or there is a family history of a known genetic disorder.

As with everything in life, there are risks involved in such tests and some exemptions such as women with known HIV statuses because the chances of the fetus getting infected is greatly increased. Not to mention it is expensive.

-Pre-implantation Genetic Diagnosis (PGD) as the name suggests the process is used to test for or detect genetic disorders before implanting the embryo into the uterus of the lady. This means it is carried out via in vitro fertilization. A greater advantage of this process is that it totally eliminates the option of pregnancy termination otherwise known as abortion  and also more chances of being sure about having offsprings that will be free of any form of genetic disorder, in our case sickle cell. This also comes along with it's own risks and is equally really expensive.

I think I should briefly point out that in vitro fertilization enables for more than one fertilization at a time from the same couples which allows for greater chances of having healthy embryos. Fertilized embryos can also be stored by freezing however it usually does not have a successful survival birth rate.

THE GIFT OF HEALTH

Health is one of the best gifts The Good Lord has provided us with. It is one of the things that we will give account of on the Last Day. Times without number I have heard/read if you take care of your health/body when you're young, it will take care of you when you get old.

Living with sickle cell anemia awakened my consciousness to the beauty and perfect order in which we were created. It made me more interested in reading about health related issues; sickle cell and other wise. Most of all it made me a very observant mother.

When my Aisha and I were discharged from the hospital, I would spend the entire night looking at her, I was overwhelmed by the Greatness of God. I couldn't get enough of the little life that came from me. I never thought I would be blessed with such a miracle. I know, pessimistic Fatima...but after what I went through, I don't think anyone can blame me. Whenever she had a cold (which was often) or I 'thought' she was running a fever, I'd go to the doctor. Oddly enough I always went to the pediatrician's on a Thursday! Her father found it very annoying...which is typical man character I'm not even going to mention how very African it is when he decides to prescribe what I should give her. Thank you very much but I will go and have her checked out. It could be nothing but why risk it besides, it's safer to get the drugs and in the right dosage to be given at the right time from the doctor.

I remember when she had cradle cap, I never knew what it was, never heard of it even! We went to her pediatrician and when the HOD saw me, she started laughing and said "What is it this time, Mrs. Abdallah?" After explaining the color changes I noticed on her scalp when her head was wet (sweat or bathing) and showing her Aisha's head, she looked at me and said "Wow! This is a classic case of seborrheic dermatitis! I wish more parents would be as observant as you are." Okay, I have to admit, my ego was a few inches from being super nova'd! But I looked alarmed, such big grammar ....so she said it's cradle cap probably seeing the look on my face and I thought "Oh that makes things better; tomaytoes...tomahtoes!" Good thing she knew how enthusiastic about my girl I was so she further broke it down "it's a fungal infection. Very common in infants. I will write down a shampoo for you to use for a week." Sigh of relief!

I learnt along the line not to feel shy of any doctor; they are my Health Google I can tell them all the things that I feel is wrong with me and they will get the right answer to my mumbo-jumbo! They are God's gift to us. We can only appreciate them if we appreciate our lives and appreciate that God indeed has created a remedy for every ailment. Not all sicknesses have cures but they have aides that help us lead a healthy life style. And when one knows what ails them and takes the proper measures to control it, they have conquered the fear of being slowed down by it! We should never be ashamed or nonchalant about our health even though we could be mocked, misunderstood, misjudged and judged at the same time, face difficulties in various aspects of life and even get discouraged sometimes to carry on. We must remember no one is without a health condition be it slight "irrelevant" allergies or the inability to produce kids. We must always remember someone out there is worse off than us therefore we work harder to be better than the other person healthier than us. Health is really survival of the fittest!

At the end of it all we are still going to our Creator but we go knowing we appreciated the Gift of Health. 

SUPPORT SICKLE CELL AWARENESS - 2

Erythrocytes or Red Blood Cells (RBCs)

RBCs are the main or most common component of the blood. It delivers oxygen from the lungs to the entire body this blood is known as oxygenated blood and returns with the waste, carbon dioxide, which is known as deoxygenated blood back to the lungs for another cycle.

Mature normal erythrocytes are elliptic, flexible, biconcave disks; which means they are not perfect circles, move/bend to fit through the tiniest blood vessels and are dented on either side of it. They are produced by the bone marrow every 120 days. RBCs contain a protein known as hemoglobin. This hemoglobin (Hb) is what medical scientists use to determine the human genotypes; HbAA,  HbAS, HbSS. It is derived from two words, heme; which contains iron that binds to oxygen (in the lungs) and globin; which is made of a series of amino acids (trust me you don't want to know more than this about globin). 

This makes hemoglobin a very important and necessary component of the RBCs. Without it, the cells of the body will not be able to have oxygen supply in them and carbon dioxide taken out of them. I guess it is safe for me to finally make it clear that the hemoglobin is responsible for the shape/form of the RBCs. That they can change form also known as genetic mutation. This mutation in hemoglobin gave rise to what is known as hemoglobinopathies. This is generally the term used to describe hereditary blood disorders/diseases......that's right, including the Sickle Cell Disease!

We have arrived!

A-NORMAL RED BLOOD CELLS
B-ABNORMAL RED BLOOD CELLS

In sickle cell anemia (SCA), the RBCs hardly last more than 90 days, are majorly sickle or S or C shaped instead of being elliptic; making it abnormal mature erythrocytes. These properties make it highly inflexible and therefore very difficult to pass through tiny blood vessels. This also means oxygen supply to every cell in the body is usually limited. And this can cause anemia,  jaundice, fatigue, shortness of breath, exhaustion, pain crisis, and even damages to internal organs.

Yes, SCA is that horrible and scary!

Just A Note: There is a type of hemoglobin produced in babies by the spleen known as fetal hemoglobin (HbF) this is the main oxygen carrier in human fetuses from when they are in the uterus till about 6 months after delivery. This HbF differs from the mature Hb such that it has greater affinity or binding abilities to oxygen. The production starts sometime between the end of the first trimester and the beginning of the second trimester (3-4 months) and is greatly, greatly reduced after 6 months of age.

SUPPORT SICKLE CELL AWARENESS - 1

It is almost time for World Sickle Cell Day which is on the 19th June, 2013. A day dedicated to all the sickle cell warriors/survivors, their parents, friends, siblings, relatives, their doctors and nurses, offspring,  friends of friends, sisters/brothers of friends of friends ...the circle of life to be brief. You may wonder how or why it affects such a wide range of people, the answer is simple; Sickle cell is one of the most common diseases of the world. According to the World Health Organization, in some regions of the world (Africa), up to 25% of people are healthy carriers of the sickle gene. 

Before I get into a lot of confusing details, I want us to first know that Sickle Cell Disease(SCD) is an inherited blood disorder passed from BOTH parents to their offspring. This means that each parent has to be a carrier of the sickle gene. We also need to know that sickle cell disease comes in various forms which include:

-Sickle cell trait (HbAS)

-Sickle cell anemia (HbSS)

-Sickle cell with hemoglobin C disease (HbSC)

-Hemoglobin S-beta-thalassemia

-Compound heterozygotes which include HbSO, HbSE and HbSD.

Lets not forget the normal genes; HbAA. It may come in handy much later. *bright smile*.

My write up is mostly going to be referring to the sickle cell trait (HbAS) and especially sickle cell anemia (HbSS). Generally and under normal circumstances, HbAS  people do not exhibit symptoms of sickle cell disease and  are therefore known or called carriers. That's right because they have one normal gene (A) and (carry) one sickle gene (S). On the other hand, HbSS or sickle cell anemia(SCA) being the most common form of SCD and also the most severe form has a wide variety of symptoms exhibited by the patients or sufferers from this disease.

Some of the symptoms include:

-severe chronic anemia (hence the name)

-pain crisis or sickle crisis (vaso occlusive crisis)

-priapism (painful erection of the penis)

-acute chest syndrome

-bacterial infection due to low immunity

-jaundice

-stroke

-splenic sequestration

-organ(s) damage

-and leg ulcers among other things. 

Some SCA patients have delayed growth, pronounced forehead (like mine) *covers face*, dactylitis (inflammation of the finger or toe) ..... to be honest naming all the symptoms and complications of SCA will make this write up boring to read. I will however in following write ups try as much as I can to break them down for my very eager readers and friends that have asked me on numerous occasions to explain SCA to them.

Then again, before all these things occur, I wonder if we remember the part I mentioned "SCD (including SCA) is an inherited blood disorder"? How does it occur? How does one get it from both parents?

The blood. I don't know if it should be heading or not but that is where the whole story lies, from the beginning to the end. 

The blood is a bodily fluid which transports nutrients and oxygen to the entire body. the human blood is made of many components; erythrocytes or red blood cells (RBCs), leukocytes or white blood cells (WBCs), platelets, eosinophils, blah, blah, blah .... our main business are the erythrocytes (RBCs).

A FRIEND LIKE YOU

Today 2nd May 2013, is the birthday of a very dear sadeeq of mine. We attended Bayero University, Kano together although we were in different departments, we were in the same year and got to meet through a common course we chose in our first year. I have to say he is among the very countable set of people I have not and will never ever regret knowing in my lifetime. I know that he has positively affected the lives of other people that he has been blessed to meet but I still think I got the most from knowing him. He is known by all as Aluta Imam or Mallam. He is Usman Idris Usman.

Thirty years on earth is a big blessing and 10 years from it, I think that was a good deal for me *wink* I will try as much as I can to tell you, my dear readers about Mallam from my own perspective, how we met and other times we were opportune to meet. Although I know he will have a more detailed memory of it all.

The first time we interacted was during our very first lecture of Geography Map Reading GEO 1305. I arrived the class late because I couldn't find where it was located easily and by the time I got there there was no space left for me to sit but I wasn't very well going to stand for 3 hours so I looked around and found a space I could contain myself, somewhere close to the front row. The only 'problem'; it was mostly male students sitting and with the way I was dressed it would seem very inappropriate for me to be found in their midst.  But we were all there to learn so we were just going to have to manage each other till we finished. I went straight up to him and his friends and politely asked them to create a little space for me. That was ten years ago! As days went by, he got to know me better......I was so engrossed in my own little 'water-world' I hardly bothered to ask him questions the way he 'interrogated' me. It did me much good too.
I had not only the type of brother Islam has asked us to be to each other, I had a guardian, an adviser and a friend in deed! He worried about my academics, about my religious life, the friends I had around and everything else. What I admire the most is the way  he had studied my moods and character. He learnt I am very independent in my decisions and once I make up my mind over something, there is no going back. He would still take his time to convince me otherwise. He could tell my mood from the way I spoke/replied him.  There was a time I had to actually look for him and when we finally met, he said "from the way you replied me the last time we spoke I knew you were in a very bad state of mind so I decided to give you your space till you felt you needed to see me!" Amazing! It is very rare to find a person that has taken the time to know you not by what people say about you but by observing you and not judging. A rare gem!

I was opportune to meet with him during my last visit to Nigeria. He is now married to a very cheerful, lovely lady, Fatima Umar! May Allah bless their union with togetherness and rightly-guided children, ameen.
May Allah make his years ahead more rightly guided and blessed than his past years, ameen. Have a very peaceful birthday.