Did you know your or your spouse’s genotype and its implications before you got married?
Not at all. We found out when I was about 4 months pregnant we were both haemoglobin AS. It was a shock.
How many children have you and how many are affected with sickle cell disorder (Hb SS)?
I have three children; two girls Marriam Carol Zawedde Mulumba, 8 and Aliah Naginda Mulumba, 2 and one boy, Mark Abudallah Mulumba, 6. Marriam is the only one with Hb SS.
When was she diagnosed with sickle cell?
She was diagnosed at 3 days in Maryland, USA.
Your first child was born with SS. You went ahead and had two more children. Were you not concerned they might be affected too?
It was always in the back of my mind when I was pregnant with my other two children. But my husband and I had determined that we were not in position to determine the destiny of the seed in my womb.
We were always ready to live with whatever God gave us. We may have been spared many, many trips to the hospital for having these two sickle-cell-free children, but it would have made no difference in our love for ourselves and for our children. We were offered genetic counseling and an opportunity for pre-selective in vitro implantation of sickle-cell-free embryos but we declined.
Why did you decide to give Marriam the option of bone marrow transplant?
For us, bone marrow transplant gave a flicker of hope for a child severely affected by sickle cell. At the age of 3 years, while visiting the sickle cell clinic at the Children’s Hospital, Michigan, the doctor informed us of a bone marrow transplant (BMT) but said it was a new therapy with a high mortality rate and she could not recommend it for our daughter.
Nevertheless, doctors advised us to keep the cord blood from my son born in 2003 in a cord blood bank. In 2007, after I joined the US Air force, the Air Force, doctors also made arrangements to save and keep the cord blood from my daughter born in 2007.
In 2008, our daughter’s condition was getting worse with multiple hospitalizations. She often complained of severe abdominal pain. Also frequent back, chest, feet, and finger pain. The pain became so persistent that it was unrelieved by strong medications such as morphine or Dilaudid. Generally, she appeared fatigued. Her appetite was poor. She was not interacting with her siblings in the house like you would expect a 6 year old. At the time, the pain interfered with her school attendance, so she had to be home-schooled. In June 2008, the trans-cranial Doppler studies (TCD), a test for the possibility of stroke in children, showed high affinity for a cardio-vascular accident (stroke). At that time, she frequently complained of severe headaches, dizziness, and a feeling of the house spinning around.
What options were you given?
The neurologist gave us the option of beginning chronic blood transfusions or hydroxyurea. We then asked our US Air Force specialist team of doctors the possibility of a bone marrow transplant. Marriam’s oncologist, Major Dr. Howell gave us all the information we needed for the transplant to take place. We finally decided to move forward with bone marrow transplant. Incidentally Dr. Howell used to refer to sickle cell as the worst condition a child could suffer from.
What were your feelings as the child went in for the procedure?
First of all, we came to terms with God. We knew that He gave us this angel to care for, and we knew that whatever happens will be His will.
The Air Force team of oncologists/haematologist provided our daughter and family with careful massive preparation five months prior to the procedure. The fact that we had sibling donor match gave us added confidence. We were very well aware of the possible complications of the procedure. The team of doctors, nurses, social workers, child development specialists, physical therapists, occupational therapists, housekeepers and counselors all boosted our confidence during the procedure.
The nurses administered different types of drugs to wipe out Marriam’s immune system and prepare it for the transplant. Chemotherapy was given in a period of about 21 days. As a nurse, I was very scared and sick to my stomach knowing what happens when patients receive massive doses of chemotherapy. She developed oral thrush, which lasted about one week. She experienced extreme fatigue and diarrhea.
The hardest part for me was fear of the unknown of what would happen next as a result of all this. It was also difficult to witness her hair falling out and seeing her bald.
The most nerve-racking, most anxious moments were after the transplant and you had to calculate whether her new immune system was functioning.
Were you aware of the possibility of failure and its consequences?
Yes, we were very well aware of the possible complications of the procedure. My husband and I are both in the medical field, which made it more terrifying. We were hopeful there would not be a failure. We were going to rely completely on American advanced medical technology.
How did the family support Marriam in the aftermath of the transplant?
After the transplant, as parents, we teamed up to make sure our daughter took medications, ate, drank fluids, kept active, and felt supported. In addition, we did all the nursing care at home. I changed her dressing on the double broviac catheter which was placed in her chest every other day. We washed her linen every day and cleaned her room with bleach and water to kill bacteria. We made sure she had her own bathroom and toilet which was also frequently cleaned and sterilized on each occasion she used it.
Marriam stayed in the hospital for 21 days after the transplant and was also home-bound for about 6 months. We avoided all visitors to the house. Children visitors in particular were not allowed. Post-transplant care was a marathon.
How much did the transplant cost and how did you fund it? Exactly where did it take place?
The transplant cost about USD 250 000 (two hundred and fifty thousand dollars), including up to three months of post-transplant care. The US Air Force paid fully for the procedure. The transplant took place at Methodist Children’s Cancer.