Cluster Headache: What You Need to Know

It is an exciting time in the migraine and headache disease world of treatment research. In addition to CGRPs being approved for the prevention of migraine in 2017, one of these CGRP drugs, Emgality, was recently approved in the US for decreasing the frequency of episodic cluster attacks. In the midst of these recent medical breakthroughs, I had the pleasure of speaking with Clusterbusters president, Bob Wold, about his personal journey with cluster headache and how it lead him to create an incredible organization for people with cluster. In part one of this interview series, Bob shares his long journey to receiving an accurate diagnosis. Do you recall your first cluster attack? Yes, I remember it perfectly even though it was 40 years ago. It was a nice weekend day in the spring and I was outside my house playing football with my 6-year-old son. My head started to hurt and I came inside, sat down, and it proceeded to feel like it was going to explode. There weren’t any other attacks leading up to that. It was an hour of terrible pain that then disappeared. It was as surprising as the pain was in that it just came and went like that. I remember it so clearly. What was your diagnosis journey like? First, we thought they were sinus related It took me 4 years before I ever heard the word “cluster headaches”. After that first attack, I went to my general practitioner that I had been seeing and he thought it was likely sinus related so he prescribed an antihistamine, Tavist-D, which was an over the counter at the time. After a while, it seemed to work because my headaches stopped, so I assumed that is what it was. The headaches came back in the fall and I was sure I had another sinus problem so I went and got the same medication again but this time it didn’t help at all. I had tried a few different medications and the headaches went away so I figured that it was sinus issues that just took longer to clear up. Then, we thought the pain was coming from my teeth Then they came back again and I thought for sure it was my teeth because the pain travels down into my lower jaw and it feels like a toothache. It felt like the pain was starting in my jaw and going up to my temple rather than the other way around. So I went to my dentist who took some x-rays and couldn’t see anything but ended up doing a root canal on one of my molars. A week later the attacks were still there so I went back to the dentist who said “maybe we didn’t get all of the root.” So I went through a second root canal but that didn’t work. I went back in a third time and the dentist pulled the tooth. The pain didn’t stop at all. [native] Through all this time, did the doctors ever ask you to describe the pain? I don’t remember all the questions they asked or specifics on how I described how intense the pain was. This is a problem with cluster headaches: trying to describe the indescribable. I don’t know at that point if we had even zeroed in on the timing - that they were always the same time or that they lasted the same amount of time. We were still trying to figure out where the pain was originating from. After the dentist pulled the tooth, he gave up and said there was nothing left for him to do so he suggested chiropractor. I tried seeing a chiropractor and getting accupuncture About three years after my first attack and trying to receive treatment from my general practitioner and then the dentist, I went to see the chiropractor and explained what was going on. He was confident he could clear it up and said, “If I haven’t cleared these up for you in three visits, you probably have a brain tumor.” After three visits with this guy my clusters were getting worse so I thought, “Well, shit, I have a brain tumor.” After the chiropractor made things worse, I was going to acupuncture. The only relief I got from that was the massage tool and TENs unit he used on the back of my neck. You can use a TENs unit on your head but you’re not supposed to put the electrodes on both sides of the head at the same time. But during a cycle, that’s exactly what I did. I put the electrodes on both sides of my temples, turned it up all the way and thought, "I’ll fry it out". I turned it up all the way where I actually froze my entire body and it didn’t take away any of the pain. After a scan showed there was no brain tumor, I was back to square one After the chiropractor gave up and said I had a brain tumor, I had a scan and was expecting I had a brain tumor. When the doc came in he was happy to tell me, “there is no brain tumor.” I was actually disappointed in hearing I had no brain tumor. At that point, I would have been happy to hear that they found out what it was. I was still back at square one. My family found the Diamond Headache Clinic where I saw Dr. Freitag. He was the first one to mention “cluster headaches”. He said it was obvious, “you definitely have cluster headaches. That was 4 years into my life with cluster headaches. That was probably my 8th cycle by the time I got to the Diamond Headache Clinic. I would go 6 months out of every year in cycles. I’d be in a cycle for 3 months, out of it for 3 months, and then back again. Three months of constant pain? Yes, my normal cycles were 6 attacks a day. Depending on what medication I may have I could never get it down under 3-4 takes a day. Prednisone cut the attacks down but I had to be on high doses for a while. When I’d cut back on the prednisone, the clusters would come back worse than when I started. The last time I took prednisone and was off of it I was getting 12 attacks a day for an hour each. 1 hour on and 1 hour off. I hadn’t slept in a month. -- Please stay tuned for part 2, where Bob shares what it's like for people who get diagnosed with cluster 40 years later today. Spoiler alert: it can still take years for people to get diagnosed and Bob shares that many still see a dentist and have dental work done first, unnecessarily. [lead_gen showIfLoggedIn="1" campaign="cluster_migraine" source="site" title="Sign up for emails from {{site-name}}:"] In early June 2014, I was sitting with my mom in my headache specialist's office, nervously wringing my hands while speaking to the headache nurse coordinator. I rarely got to see the specialist herself. The nurse – I'll call her Connie – calmly took notes and gazed into her computer screen as I described my recently experienced Worst Headache Ever. She said, “Now you say when you went to the hospital the first time, you were put on oxygen?” I nodded. "How did it affect you?" I told her I had felt calmer afterward. “Were you having symptoms that aligned more with a cluster headache than a migraine?” I responded that I didn't think so other than the unbelievable severity of the pain. It had been just as long in duration, I hadn't had several attacks over a short time period, I didn't have an eye that had watered. What I did have was the worst pain I'd ever experienced and an inability to lie down and be still, which is more common for cluster sufferers than for someone with a migraine. I'd had to pace, rock, "take the beast for a walk", as those with cluster headaches tend to describe their need to move during an attack. Adding oxygen to my migraine treatment plan Well, regardless, maybe using oxygen at home is something that could benefit you.” Connie said, and tapped on her keyboard. Sheets began ejecting from the printer. “8 to 9 liters per minute,” she explained, and handed me the prescription and several pages of information. She then outlined the new treatment plan which would be to first take an additional dose of my daily magnesium supplement and use 20 minutes of oxygen, then if pain still persisted, use a Sumatriptan injection (not a tablet). Using oral Sumatriptan was supposed to be a last resort. If those didn't work or I got into a situation where pain continued to recur, I was to call their office and schedule IV infusions of DHE. Trying it on for size I was doubtful, but intrigued by the oxygen idea. John and I took the written prescription to a medical supply office the next day, and learned some of the vocabulary involved, most notably that the mask type is non-rebreather and that a large machine called a concentrator or regulator would also be required, as well as lengths of tubing and various other tools. I received several cylinders (tanks) of oxygen in a silver cart with wheels. When we were all together that evening, John and I showed the girls the oxygen mask and talked about what it was for, and I put it on for them so that it wouldn't seem scary, and let them try it on too. [caption id="attachment_83633" align="alignnone" width="285"] Trying on the mask for my daughters, so they could get used to it.[/caption] A brief history of oxygen treatment for cluster headache According to the National Headache Foundation, oxygen treatment for headaches was first studied in 1939 when Mr. Charles Rhein of Linde Air Products reported to Dr. Francisco Alvarez at the Mayo Clinic that he had successfully treated severe headache patients with pure oxygen. In 1940 Dr. Alvarez released his own study of 100 patients with “migrainous” headaches who were treated with 100 percent oxygen through a nasal type mask and a flow of six to eight liters per minute, in which 80 percent of those were reported to be completely or significantly relieved. However, in the thirties and forties studies were much less rigorous, and there was nothing to distinguish migraine from cluster headaches. Recent work has been done almost exclusively on the effect of oxygen inhalation on cluster headaches, most significantly with Dr. Lee Kudrow, who found that 75 percent of the cluster headache patients he studied had their pain relieved by inhalation of oxygen, with increased success the earlier in the attack it is used. Oxygen therapy and migraine research As far as migraines, research is scarce. I did find a rather unhelpful collection of studies from 2008 originally published in the international journal The Cochrane Library, which evaluates research in all aspects of health care. Those studies only examined the effects of hyperbaric oxygen therapy on migraine, which would require a special chamber; normobaric therapy (the type we are discussing here) was only studied on cluster headaches. Obviously, hyperbaric chambers are not going to be available to most people. Kerrie Smyres, another Migraine.com advocate, was able to send me a journal article she'd located when researching oxygen for migraine, from Cephalalgia in 2012. In this article, authors Jürgens, Schulte and May discuss a patient with typical migraine (no aura) and autonomic symptoms (runny nose, drooping and watering eye) who had mistakenly been diagnosed with cluster headache. She did receive relief from oxygen use; however, her pain would return after thirty minutes. The authors wondered about this "success" and the connection, perhaps, with her autonomic symptoms. Cluster headache sufferers often have these same symptoms. The authors conclude that there is clinically significant evidence that oxygen inhalation is more effective in migraine with cranial autonomic symptoms than that without, but that only cluster headaches seem to be completely relieved by oxygen therapy. The downside of oxygen masks As for me, whenever I felt a migraine coming on I would dutifully go take another dose of magnesium and go up to the bedroom, recline against pillows, put on the mask and turn on the oxygen. (And of course this was only possible due to the fact that I'd recently had to stop working.) The mask sometimes bugged me, putting pressure on my sensitive nose and leaving indents on my cheeks. I would struggle to relax, and breathe somewhere between deeply and normally while the big machine I'd been given vibrated noisily. The oxygen coming through the mask didn't taste odd like I'd feared it might. However, only once or twice out of six months of use I was able to get up after the oxygen session and not need to take medication for the migraine that continued to progress. I kept at it anyway, even bringing the machine and a small cylinder on vacation with me, but the honest truth is that it just didn't work to abort a migraine or decrease pain. For a while I even tried using it daily as a prophylactic measure, but I stayed as chronic as ever. I believe it was probably somewhat beneficial in that it caused me to take a break, retreat, and breathe deeply, which would be a good idea for anyone at any time, really, but particularly when a migraine is coming on. Twenty minutes of relaxation while practicing some mindfulness or biofeedback is literally just what the doctor ordered, or what my pain psychologist ordered, anyway. However, the oxygen itself was probably superfluous. Asking your doctor about oxygen therapy In conclusion, it's quite clear that oxygen inhalation is a very effective therapy for treating cluster headaches, but there is much less evidence to support its use for migraine. Dr. George Sands from the Beth Israel Medical Center in New York City states on the National Headache Foundation website that he sees some relief with oxygen inhalation in about half of his migraine patients. Side effects are rare, though Dr. Kudrow reported 25 percent of his cluster patients had rebound clusters after oxygen use. (I'm sorry, but really? What doesn't cause rebound headaches?) Oxygen is not a commonly used treatment for migraine and it may be difficult to overcome the initial resistance from one's doctor or insurance company; however it is also a natural, rather risk-free therapy without many of the worries most of us have when trying a new medication. I myself ended up retiring my oxygen inhalation accessories and returning my machine with the empty tanks by the end of that year. Would oxygen provide some relief for you? I have no idea, but there's no harm in talking to your doctor if you feel it might be worth a try. [lead_gen showIfLoggedIn="1" campaign="cluster_migraine" source="site" title="Sign up for emails from {{site-name}}:"] Cluster headache is an excruciatingly painful headache that can occur in groups or clusters. Considered perhaps the most painful of all headache types, they often begin an hour or two after going to sleep and awaken people in the middle of the night.1 What is cluster headache? Cluster headache is considered a primary headache disorder. Cluster headache is part of the trigeminal autonomic cephalgias (TAC) group of primary headache disorders.1,2 The primary head pain symptoms of cluster develop on one side of the head, in the trigeminal nerve area, and have an array of symptoms that develop on the same side of the head as the headache, including watering eyes and drooping eyelid.1 How often do cluster headaches occur? Characterized by periods of daily painful headaches, cluster headache typically occurs at the same time each day with intense pain coming on quickly and often stopping as suddenly as it starts. Cluster headache can happen numerous times in a day where the pain experienced is severe and the symptoms produced are similar each time they develop.1-5 Cluster headache can be exhausting but when each headache ends typically there are no ongoing or residual symptoms. They end as suddenly as they come on.1 Pain and cluster headache Cluster headache is cyclic, the head pain occurs in groups, and can be short or long term. People experience the same type of severe, unilateral pain (one-side) from a head pain that develops 1 to 4 times a day. People often describe this as a stabbing pain in one eye or behind their eye. These pain symptoms can last from 15 minutes to 2-3 hours, with the average lasting around half an hour.1-3 Types of cluster headache Cluster headache is classified into two types: episodic and chronic. Episodic cluster headache Head pain that occurs in groups that last between 1 week and 1 year, separated by pain-free periods of remission, are considered episodic.2 These cluster periods are separated by months, sometimes years, of remission during which there is no further development of cluster headache.3 Chronic cluster headaches People with the chronic form experience cluster headache with no period of relief or remission for more than a year, or a break period that is so brief (lasting less than a month) that it is not considered a remission. It is estimated that fewer than 20% of those experiencing cluster headache have a chronic form.2,3 [native] History of cluster headache Cluster headache was first described in 1641.3 They have been identified by different names including after a London neurologist, Dr. Wilfred Harris (1926) and US neurologist Dr. Bayard Taylor Horton. Other names include: familial cluster headaches histamine cephalalgia vasogenic facial pain ciliary neuralgia erythromelalgia of the head erythroprosopalgia of Bing Horton’s headache Harris-Horton’s disease migrainous neuralgia (of Harris) Cluster headache causes and triggers Cluster headache has been associated with disruptions to the biological clock which is controlled by the hypothalamus area of the brain. This is why many people with cluster headache are awakened at night from the onset of an episode. There is no singular cause for cluster headache, though genetics may play a role. While the cause for cluster headache is unknown, there are some triggers that have been identified such as foods, medications, seasonal changes, and being at high altitudes.1,4,5 Cluster headache treatments There is currently no cure for cluster headache. The approach to treatment generally involves treatment for immediate pain relief as well as a plan for prevention. Therapeutic options for cluster headache treatment include using a 2-pronged approach to both manage episodic pain while reducing the number and severity of attacks.4 These involve both medication and lifestyle modifications.1 Medications There are a variety of medications prescribed to prevent cluster headache attacks that range from over the counter anti-inflammatories to prescription steroids, triptans, and calcium channel blocker medicines. They can be taken on a prophylactic basis which means they can delay or prevent the onset and intensity.2,5 Finding relief When a cluster headache develops, immediate relief is most frequently the desired goal. Some people use injectable triptans or hormones to bring about pain reduction. Breathing pure oxygen during a cluster headache attack is another approach that may offer some relief, especially when attacks occur at night when oxygen may be more available and convenient.2,4 Cluster headache is believed to decline with age such that individuals experience less frequent bouts and prolonged periods of remission in between episodes.3 Talk to your doctor If you experience a sudden change in headache type or pain, seek immediate medical help. If you think you are experiencing cluster headache or migraine but have not been diagnosed, call your doctor for an appointment, or ask for a referral to a specialist. Most likely you will need to see a headache specialist or neurologist to get a confirmatory diagnosis.1,4 Resources for cluster headache Cluster headache resources are available through the National Organization of Rare Disorders (NORD). Cluster headache affects a small percentage of the population; between 200,000 and 1 million people.2 It had been considered a rare condition but recently researchers have asked for this condition to be reevaluated because of its increasing prevalence. According to the National Institutes of Health (NIH) a rare disease is defined as a condition that affects fewer than 200,000 people. [poll nexus_id="4835"] [lead_gen showIfLoggedIn="1" campaign="cluster_migraine" source="site" title="Sign up for emails from {{site-name}}:"] ,In part 1 of an interview with Bob Wold of Clusterbusters, we learned how long it took for Bob to receive his cluster diagnosis. We wondered what it was like for people receiving a cluster headache diagnosis 40 years late rand if there has been any improvement. Would you say there’s any improvement for people who are getting diagnosed with cluster today? It’s getting a little better and hopefully, some of the stuff we’ve been doing is making it better - bringing awareness to the condition, all the advocacy work, and teaching doctors about it. Forty years ago most doctors hadn’t even heard of the term. Even neurologists would be lucky if they ever saw 1-2 cluster patients in their entire career so trying to find someone who was experienced was difficult. I’ve talked to a lot of people who waited 6-8 years before getting a proper diagnosis. Some people are getting diagnosed with cluster headache right away by a neurologist. But four years is common and the midrange for time to diagnosis. So if a patient makes it to a neurologist they could get the diagnosis right away? I see people all the time who say they think they have clusters, they’ve been to 8 doctors and been ignored for years, and they finally get a referral to a neurologist. They think they’re going to see an expert who will know how to treat them. Once again, the odds are they’re going to go to a neurologist who has never seen a cluster headache patient in their life. Are primary care doctors capable of spotting cluster? If you’re lucky enough have one who has had patients who have had cluster before they may recognize it. For the most part, neurologists are diagnosing cluster headache. Dr. Peter Goadsby found that 40% of people with cluster headaches go to a dentist and have dental work done before getting diagnosed with cluster headache. Are dentists being educated? There should be. Dental conferences are huge. These conferences are number 1 on my list - give me a microphone to educate them. I just want to tell them don’t start pulling teeth, learn about cluster headaches. Why are people still being misdiagnosed? We’re slowly getting things turned around. Part of the problem when people finally get diagnosed with cluster headaches, they don’t want to talk about it. They don’t want their job, family, or friends to know. The first thing you do during an attack is leave the room so no one sees us going through this pain. We hide it from those we love and from our jobs. Even when the internet came around, people don’t even really put it out there on social media. Cluster headache is misunderstood and very difficult to explain to people. “No it’s not a headache, they’re different from migraine, it’s not something I can work through.” The people with the disease were keeping it quiet. Trying to get people to talk about is one of the largest challenges. There are sports figures and celebrities who have it but don’t want to advertise it. -- Please stay tuned for part 3, where Bob discusses what it was like being prescribed over 70 different medications for cluster headache over two decades. [lead_gen showIfLoggedIn="1" campaign="cluster_migraine" source="site" title="Sign up for emails from {{site-name}}:"]

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