On Tuesday last week, we had our first appointment with the haematology consultant to discuss my son’s care going forward. The meeting went very smoothly, and it was beneficial in quelling most of my worries.
Our appointment was scheduled for 10 am at Kings College Hospital. We got there early at a comfortable 9.45 am and proceeded to the main entrance. At the reception, we were greeted by a member of staff who gave me some sanitiser to cleanse my hands and a fresh face mask – thank you COVID-19. An assistant directed us to the third floor, which houses the pediatric haematology department.
On getting there, the receptionist signed us in and directed us to the waiting area. Soon after, a nurse called for us and then led us to another room to measure my son’s height and weight. I was ecstatic to find out that he has gained another two pounds and is a healthy 12lbs 2 oz. He has been gaining weight consistently since he was born, which is fantastic for a child with sickle cell anaemia! My breast milk is clearly doing a tremendous job! LOL
Soon after his height and weight measurements, the haematology consultant came to fetch us. I expected our meeting to last half an hour at most, but it went on for almost two hours! It was partly my fault because I had so many questions and concerns that I needed to clear up. The consultant was very charming. She listened attentively to all my concerns and answered all my questions to my satisfaction.
Key points from the appointment
One of the main things that have worried me since finding out my son’s diagnosis was the notion that he would have to take penicillin daily until he was five. I know that antibiotics can harm the gut microbiome, which plays a crucial role in immunity. I was also concerned that the daily use of antibiotics would make him antibiotic-resistant and more prone to infections by penicillin-resistant bacteria.
The haemotologist reassured me that the dose he is prescribed is minimal and should have no adverse side effects. He has to take 1.25ml of penicillin twice a day, which works at about half a teaspoon a day. It sounds like a tiny dose, but if you think about it, he is only two months old. Over five years, it adds up, and that sounds pretty scary!
So do the benefits outweigh the risks? Well, it seems like it.
Penicillin is necessary to prevent infections from Streptococcus pneumonia bacteria. This bacteria can cause meningitis and sepsis in children (< 5 years old) with sickle cell anaemia and can be fatal. Before the introduction of penicillin prophylaxis, the rate of infection and, death in children from this bacteria was very high.
A landmark clinical trial in 1986 showed that daily penicillin reduced the rate of infections and death by around 84%, and since then it has become the standard treatment for infants and children with sickle cell anaemia. Children with sickle cell anaemia in African countries do not currently receive penicillin in childhood, so the death rate is still as high as 90%. That said, children in African countries are exposed to infections from other bacteria, viruses and parasites, especially malaria, which may be the cause of death.
In terms of more susceptibility to infections by other resistant bacteria, the haematologist assured me that it wouldn’t be the case. The type of penicillin he needs to take only aims to prevent Streptococcus pneumonia infections. It is not very effective at killing many other bacteria, and it will not prevent infections from them. If he has a bacterial infection, he will need treatment with a much more potent antibiotic as this one is proverbially “useless”.
Infections are the leading cause of pain crisis in childhood. In children, crisis manifests as swelling in the hands and feet (dactylitis). Infections can be due to coughs, colds or any other mild or severe infections in the community. You can’t prevent all infections, but you take proper precaution like keeping up to date with vaccinations, washing your hands regularly, avoiding sick people, eating a healthy diet and getting enough sleep.
The haematologist told me that we need to be careful about infections, particularly parvovirus infections. Parvovirus infection is sometimes called slapped-cheek because of the red rash that develops on the face. It is very contagious and can lead to a sickling crisis in children with sickle cell anaemia.
If there are any signs of a fever (temperature above 38°C), we need to go to accident and emergency without delay. Fevers in children with sickle cell anaemia can be severe, so it is crucial to get medical care ASAP.
Maintaining a comfortable temperature
Extreme heat or cold is bad news for sickle cell anaemia. Going from extreme heat to extreme cold is also a bad idea. The change in temperature can lead to a sickling crisis. So it is important to keep warm even if that means wearing more layers than everyone else in cold conditions. It is also crucial to avoid being in disease conditions for prolonged periods.
Figuring out the weather in the UK is a nightmare without sickle cell. I am going to have to pay extra attention to the weather now because of my son. My plan is to always have an extra layer whenever we are out just in case it gets suddenly cold.
Regular appointments and transcranial doppler
We are going to have regular appointments during this first year to ensure my son is growing well and thriving. At some point, he will have a transcranial doppler to check the blood flow in his brain. Children with sickle cell anaemia have a higher than normal risk of stroke in childhood. The haemotologist told me that by having this scan early and regularly, they can put in measures to prevent a stroke if he is found to have any blood flow abnormalities.
We spoke very briefly about hydroxyurea and stem cell transplantation. He is considered too young for either of these treatments, but they are options that we can explore if and when the need arises. I have done some research about hydroxyurea, and I have a lot of reservations about it. I know some people do very well with it, but it is an option that I would prefer not to explore for my son.
My thoughts overall
I found the appointment very insightful. In truth, I knew most of what the consultant was going to say, but it was very useful to be able to ask questions about things I had concerns about. The doctor also reasurred me that the majority of their patients have severe crisis only once a year or two years. Some don’t have any crisis at all or only deal with pain that can be managed at home. I would much rather that my son didn’t have any crisis at all. I don’t know if that’s impossible, but I’m going to do everything in my power to ensure that’s the case.
Many people who live with this condition do not have access to good medical care or to doctors that know much about the condition. I hope this blog post provides some insight into the options available for your child. This may be a challenging journey, but I am confident that we can manage it effectively with the correct tools and precautions.