Cary, RN

Pulmonary Hypertension and My Friend Christa

A friend of mine was recently diagnosed with pulmonary hypertension (PH). Many of you who follow this blog know Christa as well. We discussed my writing about PH because the statistics can be depressing, and I didn’t want her to feel negatively affected by my writing a very direct, factual post about this illness. Christa and I have discussed more than once that statistics are meaningless until it happens to you. So while this is a rotten illness, our friend’s life is not a statistic. People beat the odds in medicine every day, and Christa has the attitude to persevere.

Pulmonary hypertension is not the same as common hypertension, or high blood pressure. PH is an incurable condition. There are several causes, and sometimes it is idiopathic which means the cause is unknown. In Christa’s case, it was probably caused by fen-phen. Regardless of the cause or type of PH, treatments and prognosis are generally similar.

Fen-phen was a very popular diet drug until it was outlawed. Christa’s doctor prescribed this drug for her because she was overweight. She did nothing wrong, she didn’t take any illegal drugs or abuse her prescription. Many people have died as a result of taking fen-phen; that’s why it was outlawed by the FDA. Christa took this drug 20 years ago, before anyone realized how dangerous it was.

PH occurs when the arteries in the lungs have abnormally high pressure. The vessels narrow and become stiff, increasing pressure as when you put your thumb over the end of a hose. Because the pressure in your lungs is usually low, the right side of the heart easily pushes blood into them to be oxygenated. The blood then travels back into the left side of the heart and is pushed out into the body. ‘Regular’ hypertension is when the left side of the heart has to push harder to pump blood through your body. This is a more common problem.

When the right side of your heart has to pump so hard to push the blood into the arteries of your lungs, it enlarges. Just as any other muscle that is pushed to extremes, the right side of the heart can become very enlarged. The difference is when the heart muscle enlarges, it works less efficiently. The high pulmonary pressure can also cause regurgitation, meaning the blood going into the lungs can backflow a bit back into your heart.

All this pressure, enlarged heart and swirling backflow of blood raise the risk of blood clots significantly. People with PH are often prescribed blood thinners such as Coumadin to decrease the risk of clots.

Symptoms of PH are dizziness, shortness of breath, fatigue, chest pain, dry cough, edema, depression, cardiac arrhythmias, and fainting. Some people also get Raynaud’s phenomenon, which makes fingers white or dusky blue, can be painful, and is sometimes provoked by exposure to cold. Initially the breathlessness occurs with exercise, but as the disease progresses, it is present at rest as well.

Treatment depends partially on the cause, but includes various cardiac medications, supplemental oxygen, diuretics, anticoagulants, and inhalers such as Advair. Over time medications are added and can include portable medication infusion. These therapies are not curative. Eventually if a patient is a good candidate, PH may require a lung or heart/lung transplant, or rarely, heart valve surgery. Due to the complexities of organ donation, transplants are not something you can count on.

Medical treatments have improved a lot over the years, and people with PH live longer than they used to. Over time PH causes right-sided heart failure. The right side of the heart becomes too weak to push the blood against the pressure in the lungs. According to the Centers for Disease Control, prior to 1995 the survival rate for PH patients at diagnosis averaged less than three years. Treatment has improved since 1995, and so have quality of life and survival rates for PH patients.

As you can imagine, treatment for PH is expensive. Christa, like many in the U.S., does not have health insurance. She must pay for oxygen tank rental, and hopefully will be able to get financial help to buy an oxygen concentrator. With the portable concentrator she will not run the risk of running out of oxygen as she does now, because it takes room air and concentrates it to give her the extra oxygen she needs. Medications and doctor visits are costly, and PH treatment requires special doctors who are up to date on current research. An inexperienced treating physician can literally cost a PH patient her life.

PH is a terrible disease with no real cure. It causes disability and early death, but first it bankrupts patients, even when they have health insurance. The diagnosis and initial education is a shock to a person who is already having physical difficulty getting through her day; when you add the emotional toll this diagnosis causes, it is understandable that people with PH need all the support they can get.

Many thanks to Christa for allowing me to mention her in this post. Love you, girl.

References:

American Heart Association: Pulmonary Hypertension

Centers for Disease Control and Prevention: Pulmonary Hypertension Fact Sheet

Cleveland Clinic: Pulmonary Hypertension

Medline Plus: Pulmonary Hypertension

National Heart Lung and Blood Institute: Pulmonary Hypertension

Pulmonary Hypertension Association: About PH

U.S. Food and Drug Administration: Fen-Phen Safety Update

Grief

Grief is a bitch. There is no way around it. People grieve for many different reasons, and the process is different for each person, but the thing to remember is that grieving is a process. It isn’t an act or an event. It is a process that can take from days to years in a healthy person, and in someone who isn’t healthy emotionally it can last indefinitely.

The best-known “authority” on grieving was Elisabeth Kubler-Ross. (I put authority in quotes because the true authority is the person who is grieving.) She is a minor deity in many circles, including hospice nursing. She was a psychiatrist who was one of the first modern researchers to really look at grieving, how it works, how it affects people, and how we treat the dying. The shocking thing is that while people have been dying as long as humans have existed, this research really only started in the late fifties.

Kubler-Ross came to the US from her native Switzerland and was stunned by how terribly the health care system treated the dying and their families. We are, in all honesty, still pretty bad at it overall. That is why I went to work in hospice, and why so many nurses are huge hospice advocates. It is all about doing the best we can to make every death “a good death” for the patient, family and friends.

Once again, I have wandered off track. Kubler-Ross posited five stages of grief, and this theory has been widely accepted. They make sense as long as you remember they aren’t rules, but general stages the grieving often go through, in no particular order:

1. Denial and Isolation

2. Anger

3. Bargaining

4. Depression

5. Acceptance

Grief doesn’t only happen with death. People grieve many losses. I went through a long, kind of terrible period of grieving when I was formally diagnosed with rheumatoid arthritis. I say terrible because I spent a long time in Anger and I’m sure I was just about the most awful person to be around for a couple of years. When I had to stop working, I went through it again. I wasn’t so angry this time, but I held depression close to my chest in my crooked hands.

You might grieve a job loss, a divorce, your sibling’s mental illness; you may go through a grief process related to any loss. The trigger is often the loss of what you thought would be. If your child is born with health issues, you may grieve the loss of that ideal healthy child. The loss is the death of what could have been along with the fear of figuring out how to deal with what is.

There are many books on death and dying, on grieving actual or perceived loss. There is a website containing some of the ideas and work of Kubler-Ross and her colleague, David Kessler. One of the pages is an excellent guide to helping someone who is grieving. So often we ignore the grieving person because we don’t know what to say or do, or we have our own fears of loss that make it easier to deny what is happening. The page is titled, “The 10 Best and Worst Things to Say to Someone in Grief.” For me, the best part of the page is at the bottom:

Traits of the worst ones

They want to fix the loss

They are about our discomfort

They are directive in nature

They rationalize or try to explain loss

They may be judgmental

Not about griever

May minimize the loss

Put a timeline on loss

Traits of the Best ones

Supportive, but not trying to fix it

About feelings

Non active, not telling anyone what to do

Admitting can’t make it better

Not asking for something or someone to change feelings

Recognize loss

Not time limited

We will all go through the grief process at some point in our lives. We will each have friends or relatives who go through it as we stand by, helplessly flailing in our attempts to be there for them. The most important things I have learned in my nursing career are to listen and validate.

Just listen. Don’t think about what you will say or when lunch is or how you wish she would stop crying or anything else. Quiet your mind down, engage with the grieving person, and just listen. You do not have to fix it. You cannot fix it. Just listen.

Then validate. Everyone needs validation. Validate that you heard the person, you understand what he said, or you don’t understand and ask him to explain. The point of validation is to be there to witness, and express that you are witnessing, and that you care about what another human is going through. You don’t have to understand everything or agree with anything. You are a human being saying, “I hear you. You matter.”

Listening and validating the feelings of someone who is grieving can be very uncomfortable, often agonizing. Most of us fear death or loss or even bad luck. If it can happen to someone else right in front of us, it can happen to us. That’s scary stuff. But however uncomfortable you feel, the person who is grieving feels worse, and it is our duty as human beings to be there for each other, even when we don’t like it. Nothing is more important than supporting your fellow human.

If you are struggling with grief, get professional help. Sometimes grief can be complicated by difficult relationships. Sometimes people feel very hopeless in the face of loss. Do not allow grief to shut down your life. Get help.

I recommend a few books on grief:

The Year of Magical Thinking by Joan Didion

On Death and Dying by Elisabeth Kubler-Ross

the farewell chronicles by Anneli Rufus

Social Phobia/Social Anxiety Disorder

Social Phobia or Social Anxiety Disorder is often a life-long issue, even though it usually waxes and wanes according to what is happening in a person’s life. Estimates say approximately 2-13 percent of the general population struggles with Social Phobia.

Generalized Social Phobia is not the same as a limited fear of public speaking or other limited anxieties. It can be a little tricky to tease out if there are other mental/emotional issues in play, and the main point of doing that is for diagnostic purposes, as many anxieties or phobias related to interacting with others are treated in similar ways. In other words, some of the diagnostic criteria are more academic than necessarily helpful to the sufferer.

That being said, the official diagnostic criteria (edited for space) from the DSM-IV-TR is as follows (sans criteria specifically for children):

A.    A marked and persistent fear of one or more social or performance situations in which the person is exposed to unfamiliar people or to possible scrutiny by others. The individual fears that he or she will act in a way (or show anxiety symptoms) that will be humiliating or embarrassing.

B.    Exposure to the feared social situation almost invariably provokes anxiety, which may take the form of a situationally bound or situationally predisposed Panic Attack.

C.    The person recognizes that the fear is excessive or unreasonable.

D.   The feared social or performance situations are avoided or else are endured with intense anxiety or distress.

E.    The avoidance, anxious anticipation, or distress in the feared social or performance situation(s) interferes significantly with the person’s normal routine, occupation (academic) functioning, or social activities or relationships, or there is marked distress about having the phobia.

F.    In individuals under age 18 years, the duration is at least 6 months.

G.    The fear or avoidance is not due to the direct physiological effects of a substance (e.g., a drug of abuse, a medication) or a general medical condition and is not better accounted for by another mental disorder.

H.   If a general medical condition or another medical disorder is present, the fear in Criterion A is unrelated to it, e.g., the fear is not of Stuttering, trembling in Parkinson’s disease, or exhibiting abnormal eating behavior in Anorexia Nervosa or Bulimia Nervosa.

Mayo Clinic has a simpler list of signs and symptoms of the anxiety disorder:

• Intense fear of being in situations in which you don’t know people
• Fear of situations in which you may be judged
• Worrying about embarrassing or humiliating yourself
• Fear that others will notice that you look anxious
• Anxiety that disrupts your daily routine, work, school or other activities
• Avoiding doing things or speaking to people out of fear of embarrassment
• Avoiding situations where you might be the center of attention

So what can you do about all this freaking out? There are medications, mostly SSRIs and anxiolytics/sedatives, as well as a few herbal remedies, Valerian and Kava in particular. The gold standard treatment, at least in the US, is medication along with Cognitive Behavioral Therapy.

Extremely simplified, CBT teaches you how to deal with the fear and cope with the symptoms. It doesn’t say you don’t have them; it doesn’t necessarily try to tease out the origins or blame your mother. CBT says, “Okay. When you do this, that happens. So when that happens, try this.” It is a more practical behavioral approach rather than years lying on a couch looking for clues.

I’m not dissing psychoanalysis at all. It can really help people. I’m merely describing CBT, which is a faster, more concrete behavioral approach to getting yourself under control. It is pretty successful in helping people deal with their fears and feel some ability to cope with them, even if the fears refuse to disappear.

I plan to address anxiety, panic disorders, and go more in-depth into medication for anxiety in upcoming posts.

Sources:

Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition, Text Revision, American Psychiatric Association

MayoClinic.com, Social Anxiety Disorder (Social Phobia)

Neck Question

How do I tell if my chronic (9 days straight now) neck pain is from tension or because I pulled something? Do I need to visit a doctor or a chiropractor? If so, when?

This could be very long and involved, so I’ll do multiple spine posts. I worked neuro/neurosurgery and then in an ortho/spine clinic, but that doesn’t make this area any less grey. I can say a few things with certainty.

I didn’t post your name, but I know you are young, as in twenties young. This makes it more likely you have a muscle strain or are having some kind of muscle spasms. My first guess would be what I like to call computer neck (this website gives a good explanation of anatomy and muscle groups, but don’t feel like you need to buy anything). When you sit at a desk or computer and you put your head forward a little and round your shoulders, it puts a lot of strain on that entire area from between your shoulder blades on up across your shoulders and into your neck. If you strain this area, not only will you have what can be a really painful neck, but you can also end up with headaches because the muscle spasms can go up the back of your neck, up the back of your head and over the top to your forehead.

You should always go to the doctor ASAP if you have neck pain that is associated with numbness/tingling, burning or jolting electrical sensations in your arms (this is called cervical radiculopathy). This can indicate a pinched nerve, possibly due to a disc problem or vertebral fracture. Another symptom that should send you straight to the doc is neck issues with upper body or full body weakness or new coordination issues.  That can mean pressure on your spine (this is called cervical myelopathy).

Any time you have a fall and crack your head or whiplash your neck, you should be seen by a doctor and have some sort of imaging, like xray or MRI. You can have a tiny fracture and not realize it. If it stays in place you might be fine, but if it is unstable, meaning it moves around or if a little piece of bone breaks off and is loose, you put your spine at risk. No one wants to do that.

If you go to an ortho or neurosurgeon and they determine you have spasms or a little disc problem that is not a risk to your spine or spinal nerves, they will most likely prescribe physical therapy. PT can make a huge difference, even if you only go to a few visits. It can help you avoid surgery, teach you better posture to prevent problems in the future, and what to do if you have those spasms again to head them off before nine days go by.

People still argue about chiropractors. They can really help if you have no problems that make it unsafe for you to go. For instance, if you have rheumatoid arthritis, you have to be very careful about allowing a chiropractor to adjust your neck. Go to a chiropractor with a good reputation in your area. Check the license look up to be sure they are licensed and have no complaints. If you have a lot of pain after an adjustment, or if you have visual changes, dizziness or any other symptom of compromised blood flow in your neck after an adjustment, go to the ER. It is not common, but there is a syndrome some people have if a neck adjustment is not done correctly where blood flow is compromised to your brain. That can be dangerous.

Another thing to consider is acupuncture. Acupuncture can be really great for neck pain if it is a strain and not something more serious. Some chiropractors are also certified in acupuncture, and some of them do the acupuncture with electrical stimulation. I have personally been to someone like that, and it did more for my chronic neck pain than anything else I have tried.

Lastly I will say if you are miserable, or if you feel very worried, always go to the doctor. Trust your knowledge of your body. One of the strongest signs that something is very wrong with a person’s health is their feeling that something is seriously wrong, or a feeling of impending doom. So trust your gut unless you are a silly person.

Ambien Question

I’ve had a couple of different medically trained people inform me that Ambien is only “designed for short term use” and is generally prescribed because insurance companies prefer it to be used based on cost.

I’m “lucky” that I had an adverse reaction to the drug so I was moved onto something “better”, however it makes me concerned that so many people take Ambien on what appears to be a long term basis.

What are your thoughts on the subject?

I am going to apologize now for what is most certainly going to become a miniature rant on medication.

Ambien is a non-benzodiazepine hypnotic, and is suggested for short-term use, just like every other hypnotic or sedative ever made. They are all recommended for short term because they all have potential for abuse, and many are thought to further disrupt your sleep cycle over time.

 As a newer drug, Ambien is actually more expensive than most of the old-line sleepers. One of the reasons it is prescribed a lot is because it is newer. Just like most people, doctors like to use the newest, shiniest, most promising things they find. This is, in my opinion, partly due to pharmaceutical companies beating us over the heads with their newest drugs, but also because the newer drugs tend to have fewer side effects, or at least they are presented that way. Another reason Ambien is popular is there is purported to be less risk of abuse compared to other sedatives and hypnotics. It is thought to be less addictive, and the odds of having to keep increasing your dose to get the same effect are lower than with things like Valium or Halcion.

Ambien does have weird side effects in some people. It can cause sleep-eating and other sleep behaviors that don’t typically occur in people who aren’t taking this type of medication. Sleep and insomnia are complicated, and not completely understood yet, so trying to fix problems with sleep can be pretty difficult. There are also reports that coming off of Ambien after taking it for an extended period can cause real problems in some people, including seizures if you don’t wean off and just suddenly discontinue.

Knowing all of the above, though, Ambien does have fewer potential problems than some of the older drugs, and many people have insomnia that just doesn’t respond to things like sleep hygiene or even “more natural” supplements like melatonin (see table below). Torturers use sleep deprivation as a tool, as do brain washers. Insomnia is a serious problem. So if you are one of the people who suffers from ongoing sleep issues, drugs like Ambien can be a real godsend.

I think the key is to work with your doctor and be honest about your situation. Try sleep hygiene first. Deal with any issues that can cause secondary sleep problems, such as chronic pain, depression, or anxiety. If you still have difficulty sleeping, Ambien is a decent drug for a lot of people. You have to be aware of its limitations as with any drug, and if it doesn’t work for you, try something else.

I can go on and on about this or any drug, but philosophically we have problems in the US related to medication use. We over prescribe, but we also demonize. Modern medicine and modern pharmaceuticals make our lives longer, healthier, and give us better quality of life. But we have this love/hate relationship with them. We think we should be able to muscle on without taking anything. We have this idea that drugs aren’t natural, and natural is better. But arsenic is natural, and so is suicide related to untreated depression and insomnia.

I don’t advocate unnecessarily taking medication, but I don’t think it helps when we develop unreasonable fear and hatred of meds, either. There are trade offs for everything in life, including nearly any medical treatment or lack thereof. Read up on any medication prescribed for you. Research every diagnosis you are given. You don’t have to be in health care to do this. Keep open communication with your health care providers and work as a partner in your own care. You owe it to yourself to be as informed as possible.

FDA-Approved Hypnotics for Insomnia

Duration    Agent    Trade Name    Dose    Half-life    Comments

Benzodiazepine

Long acting   

Flurazepam    Dalmane    15-30 mg    48-120 h    Do not use in older adults due to long half-life
Quazepam    Doral    7.5-15 mg    41 h    Do not use in older adults due to long half-life

Intermediate acting   

Estazolam    ProSom    1-2 mg    10-24 h    Sleep maintenance
Temazepam    Restoril    7.5-30 mg    3.5-18 h    Sleep maintenance
Lorazepam*    Ativan    0.5-4 mg; 1 mg in elderly    12-20 h    May be used if duration of action meets patients needs

Short acting   

Triazolam    Halcion    0.125-0.5 mg    1.5-5.5 h    Caution, rebound anxiety; not first-line agent

Nonbenzodiazepine
Intermediate acting   

Eszopiclone    Lunesta    2-3 mg; 1 mg in elderly and hepatic impairment    6 h    Sleep onset and maintenance

Short-to-intermediate   

Zolpidem    Ambien    5-10 mg; 5 mg in elderly or hepatic impairment    2.5 h    Primary use, sleep onset
Zolpidem ER    Ambien CR    12.5 mg; 6.25 mg in elderly or hepatic impairment    2.8 h    Primary use, sleep onset and maintenance

Short acting   

Zaleplon    Sonata    10 mg, 5 mg in elderly or hepatic impairment or use with cimetidine    0.9-1 h    Primary use, sleep onset; maintenance up to 4 h

Melatonin Receptor Agonist
Short acting   

Ramelteon    Rozerem    8 mg    1-2.6 h    Primary use, sleep onset

* Not FDA approved for sleep

Table from Primary Insomnia: Treatment and Medication on Medscape.

NOTE: Medscape requires a sign-in, but it is free and an excellent source of current, reputable medical information.