Girl with the s-shaped spine

Nicolina Njeri, 6, when aiming to shock, will contort her long limbs so her legs touch the back of her head with ease. The bubbly little girl has known so much pain, thanks to a malformation of her spine. Her father Peter Nyoike narrates.

Nicolina weighed only 2.4 kg when she was born in October 2011. We thought the weight was something we could easily manage with supplements. But six months later, she had barely added any more weight. After we introduced her to other meals six months later, we noticed she ate very little. She also started developing a very fast heart rate. Sometimes, she would convulse and on taking her to the hospital, she would be diagnosed with chest infections. As Nicolina turned three, we were worried that she had not started walking. Nurses at the hospital also thought that she was too tall. Her toes were especially very long and they suspected her tall frame and her weak legs were responsible for her delayed walking. They recommended an X-ray which revealed that her spine was slightly bent. This discovery was, however, dismissed as the medics thought it could be due to her sleeping position when performing the procedure. Doctors prescribed antibiotics and we were well on our way.

To allay our lingering doubts, we visited a pediatrician who confirmed that Nicolina’s spine was indeed s-shaped. By that time, the s-shape wasn’t visible on her outer form, making the doctor’s pronouncement a puzzle. He explained that the condition was very rare and referred us to a paediatric cardiologist in Nairobi. The cardiologist conducted a cardiac echo (uses sound waves to create pictures of the heart) and found that her aorta was big and that it was sucking a lot of blood, overworking the blood valves. She was put on hypertensive medication to reduce her heart’s intensity. The medic also found out that Nicolina had Marfan syndrome, a condition found in taller than normal individuals that also affects the heart, eyes, stomach and the spine.

Small stomach and no sleep

Our daughter’s stomach is also smaller than normal, a condition the doctors explained was behind her poor eating habits. Even at six years, she can barely take five spoons of food. She easily develops acidity and complains of stomachache. Additionally, the pupils of her eyes are detached from the lenses making her unable to see far off objects. Doctors said an eye correctional surgery wasn’t possible as it was bound to affect her heart. Nicolina hardly sleeps. She sometimes goes to bed at 5am and even then sleeps for a very short while. Additionally, she always complains of pain in her left shoulder and left leg where she is most bent.

Live with it?

Doctors said she could lead a normal life with this condition if managed well. But managing her heart, eyes and stomach is a challenge as some drugs, say, those that treat her stomach, affect her heart. Besides, admission to hospitals often leads to nosocomial infections but getting her a medic at home is very expensive. Her monthly checkups set us back by Sh25, 000 and her medication costs a pretty dime too.  My wife has had to quit her job to give her full attention.

Our worry now is that her spine is becoming more bent as she grows older. In January this year, it began affecting her posture. We visited a neurosurgeon in Nairobi who recommended a spine MRI (Magnetic Resonance Imaging). Results indicated that the curve was becoming so wide that it was causing other complications to vital organs. The results were sent to Italy for analysis where doctors said she needed urgent surgery to help tame her spine’s distorted growth. One option was a surgery in India that would see her get fixed with a rod to straighten her spine. In this procedure, she was to go for follow-up surgeries every six months to adjust the rod until she attained the age where she wouldn’t grow any taller. This seemed very complicated to us. The other option, which we have settled on, will be performed here in Kenya by medics from Italy and Turkey. Nicolina will be fitted with a rod that grows within the spine and keep it in shape. The costs add up to Sh3 million. We know it is a delicate process and we are preparing ourselves as best as we can.  We are talking to parents who have seen their children through major surgery as we do our best to raise funds for the procedure.