Pain, pain go away

Pain comes with the territory of sickle cell. Many SCD individuals report that they are in pain on a daily basis. On the brink of her first birthday, we experienced Riley’s first pain episode. The evening before, Riley seemed calm and more solemn. Her behavior was atypical for a newly mobile 11 month old. She was teething as well, and I assumed that teething pains were the culprit. The following morning, we happened to have an appointment at the sickle cell clinic. Clinic appointments are held every few months for monitoring and blood testing. We speak with the hematologist at these appointments as well.

As we got ready that morning, Riley seemed overly tired and was very cranky. She only wanted to nurse. Dressing her was a difficult task. As I put on her pants, she flinched her leg, as if it were hurting. It was becoming increasingly harder to get her dressed. I called my mother and said, “I don’t think we are going to make it alone. Can you ride with us?” I was so thankful for the mountain of sick leave that she had built. DH was coming to the appointment from work in the opposite direction as the clinic is an hour drive from our home.

Riley slept for most of the drive. She woke up, but my usually laughing, bubbly little girl was lethargic. Her eyes projected a sadness beyond her years. I felt helpless. I continued to nurse and hold her, being mindful not to move her more than I had to once she was comfortable.

As with most typical doctor visits, Riley was weighted and measured before we saw the doctor. While examining her, the doctor paid close attention to her right leg. He pinpointed the exact spot from which the pain came and declare, “She is in a lot of pain right now.” I could not hold back my tears. I went from feeling helpful to having an awful aching in my core. Riley was in pain and there was not anything I could do to take it away. Everyone was supportive in that moment. The doctor and nurse practitioner assured me that I had done everything right. I watched her behavior and picked up on changes. I noticed that she flinched when I touched her leg. My attentiveness helped them to accurate pick up on what was happening.

While comforting me, they spoke from the place that I had been. I had thought, “We are going to get through this. This is going to be different somehow.” I was in a form of denial and reality had crashed down on me harder than I expected. I could still hold on to those feelings, but I had to put them in perspective. Sickle cell was going to affect her. The severity, I may not know, but symptoms will occur. I realized that I had to “prepare for the worse, but hope for the best.”

While reality was setting in, my inquisitive, “can fix it” attitude began to take over. “What can I do?” “How long will this last?” “When will I know if she needs to go to the hospital?” The hematologist told me that regular protocol remained paramount if she had a fever or showed signs of distress that indicate the need for immediate medical attention. Then, he wrote a prescription for Tylenol with Codeine. The nurse practitioner handed me a size dosage chart and dropper and they discussed that I could alternate between the prescription and Motrin. I believe firmly in the body’s ability to heal itself. I half-dose OTC medication for my children and I and here I was pressed with another speed bump on my ideal road of life. As I asked questions, pertaining to safety and side effects, my questions were patiently answered and discussed pain management at home. It was stressed, “She is in pain and she will be in pain until this passes.”

The magnitude of the prescription hit me as we were at the pharmacy. We use a chain pharmacy. We pulled into the drive-thru of the first one we saw on our way back home. The pharmacist took the prescription, closed the window, spoke with another pharmacist, and returned to let us know that it would be up to an hour before the prescription could be filled. We needed to return home in order to pick up our son from school. We told him so, letting him know that we would have the prescription filled closer to home. Rather than hand us the prescription paper, he said, “One moment” and closed the window again. He came back and apologize to us. Due to the nature of the prescription, he could not return it because “it was a controlled substance”. Instead, he asked if we would like to pick it up at our home pharmacy. We told him that was fine, while it sank in further that this was serious. If Riley needed a prescribed analgesic for relief before she was a year old, where do we go from here?

This pain episode occurred over a weekend. We picked our sons up and headed home to make Riley comfortable. The next 24 hours were pretty rough. Riley did not cry; rather she whimpered, and clung to me. Her discomfort was apparent and although the medication did appear to help, it could not restore her bubbly disposition. She refused food and watched everything with a sadness in her weaken glaze. My tears and feelings of desperation could not help either. I could do no more than continue to hold and nurse her.

And, then miraculously, I saw a smile, a small one, albeit, but a smile! It came from Nigel, no less. He noticed Riley watching him and began making silly faces. Within a few hours, Riley was eating food again, gleefully. After dinner, she began to crawl. She starting out cautiously at first, she didn’t go far in her chase after Michael. But, she no longer wanted to only be held. She wanted to explore again. I thanked God that she was slowly returning to her true self and that the pain must be subsiding.

The following morning was met with the Riley we know in full swing. We had made it! Re-arranging ideals and adjustments for reality was okay, because we had made it. We have not experienced any further pain episodes thus far. The only evident pains that have occurred are those when mini- daredevils try to keep up with much older brothers.

Ballerina Dreams

I always knew that I wanted to be a mother one day. The first time I dreamed in pink was by proxy. As I eagerly awaited the birth of my nephew, I very much wanted him to be a girl, if for nothing more than to dress him up like my dolls. My dreams of pink were put on hold for many years. Approaching motherhood myself, my dreams expanded beyond pink. While still in my womb, I knew Nigel was a boy. The simple beauty of motherhood laid my dreams of pink aside. With Michael, I was no longer dreaming of pink and blue, but love expanding and memories abound. Utter happiness of motherhood a second time around was enough for me. As I told one of the many people that asked if I were disappointed, “Having a girl would be nice, but being a mom is much better.”

And, then there was Riley. I was sure that I would be a mom of all boys and had no expectation, until a brief moment during the ultrasound. As all the pertinent measurements and pictures had been taken, the technician asked if I wanted to know the gender. In that brief, fleeting moment, my heart began to race, and I thought, “Could it be a girl?” I could not say “yes” fast enough! As she announced that Riley was a girl, I stared in disbelief, “Are you sure?” Instantaneously, all those packed away dreams of pink re-surfaced, including the dream to have a ballerina, or at least see my daughter in a tutu ;).

At times, Riley gives me a glimpse of this dream as she prances around on her tip toes. Initially, I thought, “How cute is that?” As time wore on, I began to wonder if she could be experiencing pain. Swollen hands and feet or Hand-foot syndrome is a symptom of sickle cell in babies. The swelling is caused by sickle-shaped red blood cells blocking blood flow out of the hands and feet. Like many things, this symptom can be painful and require medical attention. I have to monitor Riley’s hands and feet and keep her hydrated as a preventive measure.

At times, I do see her grimace, but thus far, it passes before I can reach her. As she prances on, I can’t help but think, maybe she has ballerina dreams too.

In that moment

I had to decide what to say. This was the moment I had thought about and wondered how to handle the situation. I would have to decide whether to remain quiet about Riley, only telling those on a strict need to know basis, or to speak freely, without reservation.

I knew that to be open and honest, would mean many things. It would mean opening up our family to ignorant or rude comments, occupying a “pity” status to in some one else’s eyes, or imposed limitation on Riley, not by her condition, but by what other’s think her condition means.

On the other hand, to speak openly provides a forum to share and educate. The fact is many people in the general public, unfortunately including a number of medical and health professionals, are misinformed about sickle cell disease. Sickle Cell is referred to as the “forgotten disease.” Sickle Cell is a rare disorder, with roughly 1,000 US diagnosed cases in newborns annually, and those primarily diagnosed are ethnic minorities. The reality for many people is that they are not aware of the severity of the disease. Many will neither know someone with SC or be affected daily by this debilitating illness. Complicating matters, during times, one can appear “normal” while the damage to vital organs is continually occurring.

The deciding moment came when Riley was 3 mths old. Ironically, we were preparing for her first visit with the hematologist. As I was getting ready, the phone rang.

Me: “Hello”

Friend: “What are you doing?”

Me (with a million thoughts running through my head): “Getting ready to go to the doctor.”

Friend: “Ah, is the baby sick?”

And, here it was, the moment, I dreaded.

I simply said, “No, we are going to see a specialist. Riley has sickle cell.”

As the road divulged, I chose our path.

“Don’t hurt my sister”

Did I really just hear those words come out of Michael’s mouth? I watched in amazement and with pride as Michael told Nigel this. The lively room filled with laughter, Nigel giggled and said, “She’s my sister too.” “Mom, Michael thinks I am hurting Riley.” Nigel had lifted Riley up and startled her, prompting the response from Michael, and ending in a giggle fest. I looked on at the beautiful scene with a joy filled heart.

From the age of 3, Nigel wanted a sibling. He mentioned this often, told other’s that “Mommy and Daddy are having a baby” casually, and found cute ways to show how a sibling would fit in (like the time he told us that we couldn’t stay at a hotel because they were “only for families with two or three kids”). When we eagerly showed him the ultrasound video of Michael, his reply was, “I knew it!” When Michael joined the family, Nigel was as loving and caring as a six year old could be. He even forgave the fact that Michael upstaged his sixth birthday party and looked at Michael’s birth as a gift. There are days, that the romanticized view of siblinghood are far from gone, but in its place is a bond that grows stronger with each passing day.

Michael did not have the same opportunity to yearn for a sibling afforded to Nigel. Instead, he was jolted from both his baby grandchild and baby child seats at the age of one. 2006 was a remarkable year for my family. The first girls in 25+ years joined our family. First being my niece, and four month later Riley. Weeks short of his second birthday, Michael was a big brother.

The transition in the early days was fairly normal. Nigel beamed; Michael rejoiced with everyone around him. And, then things took a turn. Michael was the “mama’s baby.” His attachment to me was evident in the early months. Once Riley came, Michael was sadden and confused. He didn’t know how to share me and had no desire to let nature teach him naturally. He tried many times to take Riley out of my arms or to sit on her while she was in my lap. Suffice to say, Riley was not welcomed by Michael with the same fanfare in which Nigel enthusiastically showed him.

As the month grew on, Michael reluctantly accepted that Riley was non returnable. And, then something remarkable happened. Michael would set aside and watch how Nigel interacted with Riley. He soon began to imitate Nigel, and as Riley smiled, cooed, and reached out to him, I watch his heart continue to melt. Once Riley was mobile, the light bulb went off that “This is someone I can play with” and there has been more and more non-stop activity and interaction every day. I often hear, “Come on Wiley, WILEY” on a regular basis now.

The memories they are building strengthens their bond. But, protectiveness is emerging from these connections. Both boys show a desire to keep Riley safe (of course Nigel more times Michael ;)).

During the initial diagnosis, I did not ponder what the potential impact on Nigel’s and Michael’s lives may be. The first time I thought about this, Riley was roughly one month old. Nigel and Michael had appointments for their annual check-ups. In the past, I chose to selectively vaccinate (vax), and neither of the boys had had the flu vaccine. This year was different. With SCD, all thoughts of selective vax went out the window.

I braced Nigel for getting his shot. Nigel had previously asked about the medicine that Riley took. He was concerned that she was so little and taking medication and inquired as to when she would “get better.” We discussed SCD briefly. We also talked about how important it was to be healthy (wash our hands thoroughly, eat healthy, etc) for Riley. That night, Nigel prayed for Riley, specifically for her health, on his on. When I broached the subject again a few weeks later, I recalled our earlier conversation and talked about why he would need to get a shot this time at his appointment. As the appointment came to an end, Nigel was nervous and scared. He put on his “strong face.” As he was injected, he watched intently and didn’t move an inch. When it was over, he began crying. As I consoled him, I thanked him for being such a wonderful big brother and momentarily the waterworks were disappeared. I saw a hint of a smile, a bit of pride, as he turned to look at Riley. Watching the subtle, yet powerful exchange, I reflected on how blessed they were to have each other. I knew the days ahead would be filled with many things, but the greatest being love.