Can I marry someone with SS?

Dear Doctor, As part of our pre-marriage counselling, it turned out that she is sickling positive, with SS as her electrophoresis result, though she is 31 years of age, has a six-year-old son and has always remain slim, she never showed any symptoms of sickle cell disease ever since I knew her even till now.

I am sickling negative, with AC as my electrophoresis result. I'm also aware of the consequences we will face should we go ahead to marry, especially, with respect to her condition and our children should we decide to give birth. But she is of the view that we should believe and trust God as Christians and go ahead to marry and that nothing will happen.

All I want now is a professional and an independent person to advise us on the pros and cons if we go ahead to marry.
AG and worried man, Obuasi

Dear AG, I have a lot of mails from people in your position, to marry or not to marry and it's always a tough one so far as the heart is involved.

In your case faith is also exacting its pound of flesh so I will stick to the scientific evidence and leave the decision for you to make.

From your mail, your fiancée is a sickle cell disease patient with two abnormal haemoglobin combination both of the S type.

From what I explained last week, she has sickle cell anaemia (SCA) because both abnormal haemoglobins are S making her combination SS.

Again, as explained last week, people with the SS combination tend to have severe form of the disease compared to people with SC or SF.

For the benefit of those who did not read last week's column, the underlying abnormality is that in SCA, the red blood cells (RBC's) are abnormally shaped. This abnormality can result in serious infections, chronic anaemia, and damage to body organs.

Each person can have different forms of complications from the disorder. Some remain relatively stable, while others are frequently hospitalised.

I may not be able to explain fully why she has never had any problems. Although with SS combination, there may be other internal factors that protect her from manifesting severe symptoms.

Also, she might have been very compliant with her routine medications and I'm not even sure if she is on hydroxyurea to help keep her stable. Whatever it is, she seems to have kept herself well.

The crises in sickle cell tend to be brought about by stressors. This can be an infection, vigorous exercise, extreme cold etc. maybe she knows her stressor and has avoided them very well.

One important stressor in your situation is the possibility of pregnancy should you both decide to have children.
I'm not sure what the experience was with her child but every pregnancy can be very stressful and will need specialized care throughout.

Your combination of AC makes you a carrier or trait of the disease and marrying somebody with the SS increases the odds of having a child with SC combination to 50 per cent in each pregnancy.
The possible outcomes of each pregnancy in your case is that either your child is AS or SC with each pregnancy. This means that any child delivered from your marriage would either be a carrier or trait or with sickle cell disease with the SC genotype.

Marriage, beyond the blissful nature is also stressful. Due to uncertainties of life, certain unforeseen circumstances may crop up that may challenge the union.

From your mail, there can be two outcomes, either her resilient nature may continue to cushion her throughout the stress with no symptoms or she may tip to having crises during stressful episodes.

It would be difficult to predict which way it will go and that's something to consider. Even better may be that the children from your union will have SC as the worst combination to pick.

People with SC tend to manifest milder form of the disease in general even though some may have severe manifestations.

Again, it would be extremely difficult to predict which direction it will go in your case.

Symptoms of sickle cell disease can vary and range from mild to severe.  Most people with sickle cell disease have anaemia and may develop one or more of the following conditions and symptoms as part of the disorder:

Acute chest syndrome - Inflammation or trapped red blood cells in the lungs.
Aplastic crisis - Bone marrow temporarily slows its production of RBC's due to infection.

Hand-foot syndrome (dactylitis) - Painful swelling of the hands and feet.

Infection - People with sickle cell disease are at an increased risk for certain bacterial infections.

Painful crises - Can occur in any part of the body and may be caused by cold or dehydration. The pain may be so severe that hospitalization would be required.

Splenic sequestration crises - The spleen becomes enlarged by trapping the abnormal RBC's. This leads to fewer cells in the general circulation and worsens the anaemia with sometimes fatal consequences.

Stroke - Poor blood blow can occur in the brain when sickle cells block small blood vessels. This may lead to stroke.
Other possible complications can include: leg ulcers, bone and joint damage, gallstones, dney damage, painful prolonged erections in males, eye damage and delayed growth.

Besides a bone marrow transplant, there is no known cure for sickle cell disease. Transplants can become a complicated procedure and are not advised for everyone.

To qualify the person would need bone marrow from a "matched" donor with a low risk of being rejected. There are significant risks towards the procedure, along with a high chance of rejection of the transplanted marrow.

Bone marrow transplant is currently in its infantile stages at the Accra Regional Hospital and even after transplant, the risk of transmission to their children still persists.

On a positive note though, even without a cure, people with sickle cell disease can lead very normal lives, your fiancée typifies that.

Certain types of medication can be taken to help manage the pain, and immunizations can prevent infections. With the pneumococcal and meningococcal vaccines introduced into our expanded programme on immunization, daily vitamin supplements, such as Folic acid and availability of hydroxyurea which help to stabilize people with the disease, the horizon remains bright.

I must add that it can be emotionally challenging sometimes caring for a child with the disease especially with the frequent hospital admissions and severe complications that sometimes bother on fatalities.

A lot of counsellors will advise couples in your situations not to go ahead with the marriage if you desire to have children.  Even though the chance is 50%, it can translate to all children that you have coming down with the disease in the worst possible outcome.

This notwithstanding, there are other couples with your combination that have decided not to have children and have enjoyed their marriage.

The choice still remains yours.
astom2@yahoo.com
A member of Paediatric Society of Ghana