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.

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 reliefe 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.