So What Now?
Your child has just turned six-months’ old, and they are having their first sickle cell crisis. Since learning the diagnosis, you have dreaded this day, waiting… worrying…anticipating. Now it is here, and it seems worst than you imagined. The incessant crying from pain, black and blue skin, the swollen limbs and jaundice eyes is all too much to take. The dreaded first crisis is upon you, but do not fret, help is always a hospital drive away. And remember, you are not the first or last parent with a sickle cell baby in crisis, you will get through this.
Despite the severity of your child’s pain, you will find the hospital can manage the situation, and most importantly, stop your child’s agony within minutes of the doctor seeing him. Handling the first crisis is essential, but having a long-term plan, and philosophy of approach is most important.
Every new sickle cell parent needs a “sickle cell disease care wish list.” In a perfect world, the care wish list should include:
1) A pediatrician with strong knowledge of SCA, as a new patient remember not all doctors are created even.
2) A pediatric hematologist that treats sickle disease. Some hematologists never handle sickle disease. They have the knowledge to treat your child, just not the desired level of experience you would want.
3) A Children’s Hospital. A pediatric ward in a traditional hospital is fine, but a children’s hospital brings unmatched expertise.
4) A school (pre-school and beyond) that willingly makes all the health accommodations your child may require. All schools will make an effort to comply with federal legal requirements; however, the difference between a mandated partner and a willing collaborator is immeasurable.
Where’s the Beef?
If you are a parent of a sickle cell disease newborn or toddler, and you are looking for help navigating the disease, you have likely found very little to aid you in your endeavors. Yes, you may find books, but there isn’t much of a selection available, yes, you may find a film or two, but one of the better-known films “A Warm December” is over 40 years old. If you are looking to join a group, the national organization The Sickle Cell Disease Association of America (SCDAA) is excellent, with active local organizations – doing the people’s work, but let’s just hope a chapter’s near you. The one refuge for everyone everywhere is the extensive online community. It is international and quite active. As far as sites, the usual suspects Twitter and Instagram are significant, but Facebook is the first place to start. You will find many kindred spirits to share your circumstance. You will quickly learn that you are not alone, and your feelings and numerous questions have been felt and asked before. As a result, the SCD online community is currently the best place to share. Be wary when others offer medical advice, as there is a lot of misinformation floating out there. So, take your medical questions to your private doctor to ensure that you are receiving sound advice.
The Family Dynamic
It is also crucial for parents to know how sickle disease can adversely affect the family dynamic. Despite the severe impact on families, this issue receives little attention, but thankfully, it has been the subject of extensive research. Studies show the primary caretaking parent (usually the mother) of the ill child can have compromised relational issues with their spouse and other children. Research suggests the primary parent will also have reduced social interaction outside of the family. The secondary parent often expresses dissatisfaction because their spouse fails to meet their individual needs. These are common issues within families with chronic illness children, sickle cell included. The key is to remember the strength of the family unit is a critical component in managing the child’s disease successfully. A fractured family with competing desires and goals will only complicate the situation. Available research suggests that the family can affect how the patient responds to ongoing illness. The more psychosocial stressors within the family, the less the patient positively copes with the disease. It is so significant; studies show it affects the willingness of the patient to comply with medical directives. The more social support available to the patient from the family and other significant others, the more likely the patient is to comply with the treatment regimen. So it is not only personally beneficial for a parent to focus on family first; it is advantageous to the ill child.
As a young child my mother, in conjunction with my doctor, limited almost all my strenuous physical activity. I was sheltered, kept out of gym class, alienated from my peers and prevented from doing anything physical. Yes, it kept me safe, but psychologically it reinforced the idea that I was different, fundamentally, lesser than everyone else. “Physical activity as tolerated” is the phrase often used on a patients discharge form. It is also a good philosophy for raising your child. Ultimately, you should seek medical advice on what your final choices are regarding strenuous activity, but remember you are not just trying to prevent a sickle crisis; you are trying to raise a well adjusted human being, either way, there are some things to consider.
One of the most important things a parent can do raising a sickle cell disease child is determine the child’s “triggers,” what actions will almost certainly cause a sickle cell crisis. I have never met a person with sickle cell disease that does not have triggers, and doctors in ER’s always ask what trigger caused your current crisis. So learning what causes a sickle cell crisis is critical to anyone with the disease. Is it weather related, swimming in the ocean or cold pool water, flying in either small or large airplanes, excess physical activity? Maybe it is all of them or none of them, but either way, it is more important to learn those triggers then it is to restrict all your child’s physical activity.
As the parent of a sickle cell disease child, there is a real reason for hope in 2018. Medical advancements have ensured that many of the sickle disease symptoms, like pain control and infections, are better controlled than ever before. Research is at an all-time high, and new drugs are in the pipeline and sure to make an impact in the next decade or so. There is also blood stem cell and bone marrow transplants available to some. So the disease is terrible – and there will be hard days ahead, however, there are many reasons to be optimistic.
It is prudent to be vigilant about your child’s healthcare; it is even more prudent to remember your child has sickle cell disease, but they are so much more than the condition they have. They have sickle cell disease, but sickle cell does not have them. Let your child know; all humans have a personal cross to bear, sickle cell anemia just happens to be theirs.