And, we're off ..........

Like most parents, we eagerly awaited for Riley to do many things in those first few months. The first smile, cooing, that expression of recognition, that eludes connections and love. Those first few months were filled with so much joy and anticipation, that it is amazing the room that was there for worry and concern. With each passing day, I knew that we were closer to the rain.

Children with Sickle Cell are protected in the early months by fetal hemoglobin. Fetal hemoglobin is the hemoglobin we have produced before birth. Fetal hemoglobin physically blocks hemoglobin S, preventing it from forming the long, rigid rods that lead to sickling of the red blood cells. Weeks before birth, our bodies began to make less fetal hemoglobin and more adult hemoglobin. At birth, an infant's red blood cells contain roughly equal amounts of fetal and adult hemoglobin. As the fetal hemoglobin in the body deceases, the risk for crisis increases. Some children have their first pain crisis as young as 4-6 mths of age.

I wanted to hold on to each day a little longer as I watched Riley grow. Around 3 mths of age, I began a silent countdown in my head, bracing myself for the next three months. And, by six month, nothing had happened. I felt part relief and part fear. I was relieved that Riley was healthy. We had made it through cold and flu season without the slightest hint of a cold. In fact, the season seemed to hit Michael the hardest. But, the fear that grasped me had me a bit on edge. I began to wonder, “When is it going to happen?” “How is it going to happen?” “What do I do when we reach this point?”

I pushed these fears aside and went on with daily life, until one day, I made a decision. I had to do more than put my fears aside; I had to let them go. The fear and worry would do nothing to help me, if a crisis came. I was comforted by the scripture. I Peter 5:7 “Cast all your anxiety on him because he cares for you.” Psalms 55:22 “Cast your cares on the LORD and he will sustain you; he will never let the righteous fall.” When a fearful thought creep up, I would take a moment to praise God. I thanked him for many things, but I mainly thanked him for the wonderful works that he had begun in Riley meditating on Philippians 1:6 …being confident of this, that he who began a good work in you will carry it on to completion until the day of Christ Jesus.”

Re-directing my energies allowed calmness, peacefulness in enjoying everyday life. The fact is life truly does just happen. Preparation and attitude are key. You can plan and prepare, but you never know what surprises are ahead. The beautiful thing is that most surprises, whether pleasant or unpleasant, make like worth living. The pleasant things are icing on the cake that energize us. The not so pleasant things, mold us, shape, and prepare us. Either way, it is the attitude that makes the difference.

Can I get a little sunshine?

One of the things I despise is unwanted advice and commentary. There are times when things are best left unsaid. This is especially true when dealing with SCD. I do know that sickle cell is a debilitating, chronic illness. In addition to unspeakable pain, one is at an increased risk of having a stroke, organ damage, or losing their eyesight, just to name a few things. It is even more devastating to know that these complications can affect all ages.

What amazes me is the number of people who feel that it is open season to provide negative outlooks on Riley’s condition. At Riley’s 1 year well baby check up, the nurse did the usual task of taking her weight and measurements. She asked the usual questions as well, “Any medications?” “Penicillin” “How long?” “She has to take it twice daily for sickle cell.” Of course, the conversation could have stopped there. Unfortunately, it did not.

The nurse looked sympathetic , but her next words lacked the needed empathy that would put one at ease. “Oh, how sad. I have a friend with SC. Both he and his sister have it. They are always sick or in a lot of pain. He just got out of the hospital. They have it pretty bad.” DH and I looked at each other concurrently thinking, “Who’s going to tell her to be quiet.” I guess we both realized, if you can’t say anything nice……and remained silent, while fuming.

She finished her commentary on “all the surgeries and pain” while writing down information and bounced out of the room, merely saying, “The doctor will be in shortly” going on with her day. Once the door closed, dh looked at me and said, “Don’t you hate that!” I sarcastically replied my delayed reaction of, “Gee, thanks for the encouragement.”

Now, the reason that I despise hearing these things is not because I am in denial, far from it actually. I understand the barriers that Riley may face. My heart grieves at times just thinking about it. I cannot tell you the number of times, I think, she is in pain, the possibility of her having a stroke, needing emergency surgery, or far worse.

What angers me most about these comments is there is little thought given to what the commentator can do to encourage or help. They can breeze in to put rain in you cloud with nary a silver lining in sight. It is a glass half-empty mentality that is all too often given to families and patients affected by Sickle Cell Disease, as if looking on the dark side is the best that can be done.

Rarely will these comments be followed by something the commentator has done to personally help an individual or their family, or to raised awareness about this devastating diagnosis. It is simply a comment in the wind.

Why not get involved in the fight to break the sickle cycle? There are so many things that can be done to help. Some things are things that you may do already, such as being a regular blood donor. Many sickle cell patients, young and older alike, have a need for regular blood transfusions.

Currently, blood transfusions are most effective and proven treatment for some severe complications of sickle cell disease. Blood transfusions reduce the risk of some complications of sickle cell disease and improve symptoms of severe anemia. A blood transfusion lowers the amount of hemoglobin S red blood cells in the body. When there are fewer sickled hemoglobin S cells in the bloodstream, they are less likely to build up and block blood vessels. Blood transfusion also increases the number of normal red blood cells in the body, increasing the supply of oxygen to the body.

In addition to treating acute chest syndrome and leg ulcers, blood transfusions may help prevent strokes in infants, children, and teens who previously had a stroke or are at high risk for a first stroke.

The next time you hear that someone has sickle cell, I encourage you to uplift in word or deed with the small satisfaction of knowing that a kind word goes a long way.

Raising Awareness

Someone sent this to me in email and it brings up major points to consider concerning sickle cell anemia. Although we have come on long way in the area of preventive care and on going medical support, there are many strides to be taken until a widespread cure is found. Please take a moment to pass this info on!


Someone said to me recently, that sickle cell disease
is not the same today, as it was in previous years. In
fact, the disease is very much the same. What is
different is our knowledge of the disease. With the
benefit of more knowledge, we have been able to keep
infants and children diagnosed with sickle cell a
little more healthy than before. But the truth is,
regardless of our efforts, nothing can be done to stop
a person living with the disease from having a painful
attack. It is a game of Russian Roulette ...they don't
know which day will be a good one and which day a
painful one.

And if you are the parent of a sickler, you know all
too well the fear that one day your infant or young
child can suffer a stroke.

The mentality that this disease is not the same as it
was years ago is unfortunately the reason why so many
people continue to suffer. Please help me spread
awareness about sickle cell disease. I am launching a
video campaign to inform people about the implications
of the disease so that we won't forget those who
suffer. These are people who look like us, act like us
on well days, but on sick days, are crippled from
pain. The only way we can find a cure, is if we don't
run away from the problem AND we get support from
people like you. There is research being done, but
funding limits how fast a cure is found. Help one be
found sooner...support your local sickle cell
foundation and sickle cell research. Why? Because
about 70,000 Americans live with sickle cell. Pretty
soon, it will come close to home!

I know...

There is not a more helpless feeling than watching
your child be in pain, get stuck with needles, want it
to stop, and you have no way to make the pain go away.


Please watch my video and help me, help them.

http://www.youtube.com/watch?v=Vifx72ldUNc

Cassie

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.